CHAPTER VII SYMPTOMATOLOGY AND DIAGNOSIS

Previous

The identity of scurvy in the infant, in the young child and in the adult is thoroughly established and requires no further substantiation. There are, however, sufficient differences between the symptoms of adult scurvy and those of Barlow’s disease to render it advisable to consider them separately. These distinctions are due largely to the fact that the former disorder affects mature tissues, whereas the latter is engrafted upon tissues which are in the process of rapid growth and development. The symptomatology is influenced also by the striking differences in environment—the passive, shielded existence of the infant, contrasted with the active and exposed life of the adult. Although we shall, therefore, treat adult and infantile scurvy separately, it should be borne in mind that, from an etiologic and pathologic viewpoint, such a division is artificial and is resorted to merely for purposes of clarity.

Adult Scurvy.—The earliest sign of scurvy is usually a change in the complexion of the individual. His color becomes sallow or muddy, an aspect difficult to describe, but one which is characteristic, and constitutes an important danger signal to the eye of the experienced physician. About the same time the patient loses his accustomed vigor, seemingly becomes indolent and complains of tiring quickly, and of breathlessness. He may experience fleeting pains in the joints and limbs, especially in the legs, symptoms which are frequently attributed to rheumatism. At this early stage the appetite may still be normal, there is usually no loss in weight, but merely a general malaise which is significant, although in no way distinctive. Very soon the gums become sore, bleed readily, and are found to be congested, spongy, and somewhat hemorrhagic at their edges. Absolute reliance must not, however, be placed on this sign for early diagnosis, as at times it does not appear until later. Careful examination at this stage will disclose petechial spots on the body, more especially on the legs, at the site of the hair follicles, or even larger ecchymoses, depending upon the hemorrhagic tendency of the individual, his exposure to bruising, the adequacy of his diet, and secondary infection. Less frequently bleeding from the nose occurs early, or the eyelid suddenly becomes swollen and purple, or the urine shows the presence of blood.

These signs progress steadily with a varying degree of rapidity. The complexion becomes more dingy and somewhat brownish, the weakness increases so that the slightest exertion causes breathlessness and palpitation, and the gums become spongy and even fungous. If there is infection of the gums and the teeth are carious, the breath is extremely foul—a sign long associated with scurvy. Later the teeth become loose and may fall out, and the alveolar process undergoes necrosis. The surface hemorrhages increase in severity, large effusions appearing on the trunk, on the extremities, and less often beneath the mucous membrane of the mouth. A bloody diarrhoea may take the place of the constipation which is generally noted earlier in the disease. There are at this time hemorrhages into the muscles and deeper tissues, especially into the calves of the legs, giving rise to hard, brawny, tender swellings which have been termed “scurvy sclerosis.” This is sometimes the earliest sign noted by the patient and may puzzle the physician who has not met with it before. The swelling may be found in the popliteal space or at the site of the tendo Achilles, and result in lameness and contracture of the neighboring joint. Frequently there is slight edema of the ankles associated with a glossiness of the extensor surfaces of the legs. This infiltration differs from ordinary edema in being firm and not pitting on pressure. The skin is dry and rough, the follicles being unusually elevated;46 the hair likewise is dry and loses its lustre. Not infrequently subperiosteal hemorrhages occur, giving rise to exquisitely tender swellings, especially of the tibia or of the femur, or of the ramus of the lower jaw, as has been noted in connection with guinea-pig scurvy. If there are wounds or ulcers they assume a hemorrhagic aspect, the edges becoming bluish or livid and showing no tendency to heal; even scars which have existed for many years change in color and show an altered state of nutrition, and ulcers long healed break out afresh.

Nowadays, the disease usually does not reach this stage, and rarely progresses further. If, however, the patient remains untreated, he becomes progressively weaker and more lethargic; there is frequent palpitation, shortness of breath, and increasing loss of weight. The pains in the limbs render him helpless and an object of pity. Marked edema may be added to the picture as the result of starvation, so that the legs become swollen, and even the face becomes bloated. Hemorrhages into the skin as large as the palm of the hand appear on different parts of the body. The gums swell to such an extent that they overlap and may even hide the teeth and protrude from the mouth as foul fungoid growth. Death comes about in various ways. Frequently sudden and fatal syncope occurs, due to heart weakness or to the pouring out of fluid into the pleural or the pericardial cavities. Another frequent cause of death is secondary infection, resulting in pneumonia, which finally ends the suffering of the patient. The fatal outcome is thus described in the narrative of Lord Anson’s voyage:

“Many of our people, though confined to their hammocks, ate and drank heartily, were cheerful, and talked with much seeming vigor, and in a loud, strong tone of voice; and yet, on their being the least moved, though it was only from one part of the ship to another, and that in their hammocks, they have immediately expired; and others, who have confided in their seeming strength, and have resolved to get out of their hammocks, have died before they could well reach the deck. And it was no uncommon thing for those who could do some kind of duty, and walk the deck, to drop down dead in an instant, on any endeavor to act with their utmost vigor; many of our people having perished in this manner during the course of this voyage.”

The disease may develop and progress in various ways. It may remain latent for a long period and be cured by some accidental change of diet, or, as more frequently occurs, it runs a moderately acute course, and is promptly cured by means of antiscorbutics. In the days when scurvy was common and widespread it sometimes became chronic, developing into the “inveterate scurvy” of the older authors, which was notably resistant to treatment. Harvey, in his treatise published in 1685, states that “a mild scurvy may continue or be protracted to ten, twenty, or thirty years.”

In addition to the general picture of the disease which we have presented, mention should be made of other less common symptoms. As is well known, one of the characteristic signs of scurvy is hemorrhage. Indeed, in many of the systematic treatises of medicine it is classified as a hemorrhagic disease. Besides the bleeding into the gums, skin and bones, hemorrhage into the stomach may take place, giving rise to hÆmatemesis, or there may be hemorrhage into the eye, under the conjunctiva or into the anterior chamber, leading to the destruction of the eyeball. A very unusual form is meningeal bleeding, giving rise to symptoms of apoplexy. It may be stated in general that hemorrhage dominates the picture of scurvy. Eruptions which in normal individuals are simply macular or papular, assume a hemorrhagic character when occurring in a scorbutic individual. This phenomenon was noted in the recent war in connection with the eruption of typhus fever, and has been observed by military and naval surgeons in numerous expeditions.

Scurvy reduces the nutritional state of probably all the cells and tissues of the body. If the resistance is still further lowered by exposure, nutritional disturbances will result more readily than where the tissues are normal and well nourished. For this reason we believe that scurvy may predispose to frostbite. Reports of congelations occurring in the trenches in the course of the World War tend to confirm our opinion that scurvy was a predisposing factor in many of these cases. This has been true in other wars. For example, Munson writes that “during the Crimean War the temperature was never very low and a report of the times suggests that the large number of congelations observed among the soldiers might well be regarded as gangrene owing to a scorbutic tendency exaggerated by the cold.”

In connection with the involvement of the gums, another typical symptom of scurvy, it should be remembered that this sign may appear late and therefore fail to be of value for early diagnosis, and that it occurs also in purpura and thus may lead to error. This is especially the case if there is pyorrhoea. As is well known, hemorrhages of the gums appear only where teeth are present, and are absent in the edentulous gums of old people as well as in babies who have no teeth. Immerman is probably correct in believing that an injury is always necessary to produce a hemorrhagic lesion in scurvy, and that this explains the early involvement of the gums and also their non-implication in the absence of teeth.

It is a common belief that separation of the epiphyses occurs only in infants and young children, and not in the scurvy of adults. This, however, is not correct, as in severe adult scurvy there is frequently a separation of the epiphyses of the long bones of the lower extremities or of the ribs, the latter resulting in a sinking of the sternum.

The pulse is sometimes slow and feeble, having been recorded as low as 40 beats per minute, but more frequently is rapid, in the neighborhood of 140. It is, however, almost invariably unduly excited by emotion or by mild physical activity. Frequently there is a low type of fever, which has been termed “scorbutic fever,” but which probably should be regarded as a complication of the disease rather than as an intrinsic symptom.

There is little tendency to the formation of pus. Although the lymphatic glands are frequently enlarged and effusions into the tissues and into cavities of the body are by no means uncommon, they show little tendency to become purulent. In the severe cases described by the older authors, the breaking down of the glands in the inguinal region—buboes—is frequently noted. The urine is apt to be scanty, becoming much more profuse following treatment. Perspiration is also retarded.

A peculiar symptom reported in connection with numerous epidemics of scurvy, both on sea and on land, is nyctalopia or night-blindness. The patients can see fairly well during the day, but have very little vision as soon as darkness develops. This phenomenon has puzzled many observers, as nothing abnormal has been found on examination of the eyes. Recently O’Shea, who met with many cases of this nature among soldiers, has reported that in an ophthalmic examination of 22 cases the only abnormality was pallor of the optic disc in 3 cases. This weakness of sight is due to the general nutritional weakness and has been reported in connection with other exhausting and nutritional diseases—for example, hunger edema. More rarely there is day-blindness.

As a complication, dysentery may be mentioned. This has been described by Schreiber and others in scurvy epidemics occurring in the course of the World War. Jaundice may appear, and might be expected to occur more often in view of the marked congestion of the upper duodenum found so frequently at necropsy.

Pericarditis, hydrothorax, pleurisy with effusion, pneumonia, are common complications of severe forms of scurvy. Lind reports that the dominant complication varies in different epidemics; that on one cruise many cases of diarrhoea would occur and on another many pulmonary infections.

O’Shea reports the exceptional case of a man who was operated upon for acute appendicitis. A large hemorrhage in the wall of the cÆcum was found, as well as some other hemorrhages in the peritoneal cavity. This report is interesting, not so much from a diagnostic standpoint as because “contrary to what might have been expected, scorbutic cases when operated upon showed no particular tendency to hemorrhage.”

Infantile Scurvy.—The stereotyped picture of infantile scurvy and the one which this term commonly suggests, is that of the acute form of the disease. In acute infantile scurvy we have to do generally with a poorly-nourished, pale infant with a peculiarly alert and worried expression. As we approach its bed it whimpers or cries out in terror. Frequently its posture is characteristic, as it lies quietly on its back with one thigh everted and flexed on the abdomen. Examination shows that one or even both thighs are swollen and exquisitely tender, or that there is merely tenderness, the baby shrieking at the slightest pressure upon the lower end of the femur. If teeth are present, the adjacent gums are red, swollen and bleed readily. This is the syndrome which the medical student is taught to carry away to guide him in his everyday practice. It is the acute, florid type, and presents a striking picture, but must not be regarded as the common form of the disorder. If we are to diagnose infantile scurvy early and not overlook its more subtle manifestations, the classic textbook description must be augmented by portrayals of types of the disorder which are less crude and more difficult to recognize—of “subacute” and of “latent” scurvy.

The commoner form, which we have termed “subacute infantile scurvy,” comprises a large number of symptoms which are inconclusive individually, and frequently escape correct interpretation. The affected baby is usually in the second half of the first year of life, and does not gain in weight or gains but slightly for weeks. It may be fairly well nourished, but is pale or sallow, with perhaps slight edema of the upper eyelids. The mother or nurse complains that the child is irritable and peevish, and that the appetite is poor or capricious. The gums show a lividity or slight peridental hemorrhage, which on subsequent examination may be no longer visible, and may have consisted merely of a rim of crimson edging the borders of the upper gum, perhaps behind an upper incisor, as Still pointed out. On closer examination it may be observed that the papillÆ of the tip of the tongue are markedly congested, and that a petechial spot is to be seen on its frenum, on the palpebral conjunctiva, or here and there on the surface of the body, more especially where there are erosions, eczema or other skin lesions. Attention may be called to tenderness of the lower thighs, which in some instances is definite, in others so ill-defined and fleeting that it is impossible to convince oneself of its significance or even reality. There may be slight edema over the crests of the tibia, of a kind which does not pit on pressure. The knee-jerks are almost always markedly exaggerated. The urine is diminished in volume but is generally normal or contains a trace of albumen and red and white blood-cells. The pulse is frequently rapid, and becomes markedly rapid and irregular on the slightest excitement. The respirations are also rapid (Fig. 15).

These symptoms do not constitute a rigid entity, but are subject to manifold variations. The syndrome may be rendered less typical and clear by the fact that the infant has gained steadily rather than lost in weight, as is sometimes the case if the food has been insufficient during the first few months of life. Roentgenograms of the bones may show the “white line” at the epiphyses first described by Fraenkel (Fig. 20) or a thickening of the periosteum. However, too great reliance should not be placed on these signs in making an early diagnosis of this disorder, as neither is invariably present.

An instance of subacute scurvy, which in many respects is typical, is the following:

I. F., girl, was seen when 3 months old, weighing somewhat over 8 pounds. She was given Schloss milk, 4 ounces, and then 5 ounces every three hours, and did well, weighing 11¼ pounds two months later. As she failed to gain for some weeks, although getting 6 ounces of food, it was thought that this might be due to the fact that she was getting pasteurized milk and had never received an antiscorbutic. Autolyzed yeast had been tried as a prophylactic antiscorbutic, but failed to bring about a gain. When, however, orange juice was substituted for the yeast, a prompt growth-reaction resulted, a gain of 1¼ pounds in four weeks. Accompanying this lack of gain in weight there were many of the other symptoms enumerated above; irritability, pallor, slight tenderness of the lower ends of the femora, albumin and a few red and white cells in the urine. The pulse- or heart-beat was frequently over 150, and the respiration 60 (Fig. 15). The diagnosis of subacute scurvy was substantiated by the prompt subsidence of all symptoms when orange juice was administered.

Infantile scurvy may be dormant for a long time. The diagnosis of latent scurvy is based mainly on the reaction to specific therapy, on the marked improvement when orange juice, tomato, potato or other antiscorbutic food is given. The symptoms themselves are suggestive, and do not enable an absolute diagnosis to be made. In our experience with many cases of this kind the usual course has been as follows: The infant has been generally from 6 to 9 months of age, and fed for a considerable period on pasteurized milk, which may or may not have been prepared with cereal decoction. Nor has it been material whether gruels also had been given. When about 6 months of age the baby ceased to thrive, to gain satisfactorily, to look healthy, and to feed as it should. The most careful investigation or physical examination has failed to solve the difficulty. On the other hand, the history of a diet of heated milk, especially if the quantity was not large, considered in conjunction with the pallor and poor appetite, the increased knee-jerks, and perhaps a rapid pulse and respiration (the cardiorespiratory syndrome), has awakened suspicion. Orange juice or canned tomato, prescribed in such cases with a view to diagnosis as well as to treatment, frequently brings about a magic result. The following case, the weight chart of which is reproduced (Fig. 14), is fairly typical of this abnormal nutritional state:

H. S., boy, born December 15, 1915, was artificially fed until January 4, 1916, when he weighed 6½ pounds. He was given 28 ounces of Schloss milk a day. (This was prepared from pasteurized milk which was not heated a second time. It contains per litre (quart) 140 c.c. of whole milk, 140 c.c. of 20 per cent. cream, 50 g. of dextrimaltose, 5 g. of plasmon, 0.2 g. of potassium chlorate, and 700 c.c. of water.) By March 1 he weighed 9 pounds, and gained three-quarters of a pound more in the course of this month. During April he gained only 4 ounces. As will be seen from the chart, there was almost a cessation of gain from April 10 to May 3, although yeast was added to the diet. May 2, orange juice was given. The weight advanced at once, the color and the general appearance improved, and an eczematous condition of the face rapidly healed. It will be noted from the chart that the gain occurred, although the food intake remained the same.

Epicrisis: A baby 4 months old with latent scurvy, which existed since he was at least 3 months of age.

This condition of latent scurvy is probably the commonest type of the disorder, especially in the larger cities where almost the entire milk supply for infants is pasteurized. It usually passes unrecognized. Most infants fortunately are given orange juice by the time they are 6 months of age, and may receive a small amount of vegetable or potato before they are much older, so that they are protected from serious harm in this way. But there is no doubt a considerable number, especially those peculiarly susceptible, who quite unbeknown to anyone pass through the state of latent scurvy.

If this large group of cases were included in the incidence of infantile scurvy, we should not look upon it as a disorder which occurs rarely during the first six months of life.

When scurvy goes unrecognized or untreated for a long time, or the antiscorbutic content of the food is exceptionally small, or the patient unusually susceptible, the disorder may progress and resemble the advanced cases described in connection with the adult type of this disease. Happily such instances are rare. One of the most typical and vivid descriptions of an extreme case of infantile scurvy is that reported by Vincent:

The infant lay in its bed extremely apathetic and barely conscious. Its face was ashy gray in color, the respirations were extremely frequent, the pulse-rate was 144 per minute, and the temperature 103.2°. When touched it moaned feebly, and made no attempt at movement. The mouth was kept open, the lower jaw hanging away from the face. There was a complete absence of muscular tone, so that the infant appeared to be quite incapable of voluntary movement.

The mouth presented a horrible appearance. No sign of the teeth could be discovered, though it was stated that several had appeared. All that could be seen was a purple mass, which was so extensive that on superficial inspection it was difficult to distinguish between the upper and lower jaws, despite their wide separation. Scattered over this purple mass were areas of necrosing tissue, the odor of which was extremely unpleasant.

Petechial hemorrhages were distributed over the back and limbs, and a large patch of extravasated blood was found in the region of the left hip.

Tenderness was present in all the limbs, as manifested by moaning and by the facial expression. There was a general enlargement over both humeri throughout their length; the ulna and radius did not appear to be thus affected, but the index-finger of the right hand was enlarged, especially at the junction of the metacarpal bone with the first phalanx, the enlargement being at each side of the joint. In the legs the signs were extreme. At both knee-joints the skin was tightly stretched over the swollen epiphyses; the tenderness also was greater than at any other part.

Bleeding from the gums and nose had occurred; no history of hÆmaturia could be obtained. The motions were semisolid, green, and offensive. During the last twenty-four hours the infant had refused food.

The baby was given large amounts of lemon juice and subcutaneous injections of salt solution and the necrosing surfaces of the gums were scraped and swabbed with boracic solution. By the third day the pulse was 100, the temperature 99.8°, the odor from the mouth scarcely noticeable, and the general condition distinctly improved. It continued to improve and to gain in weight and when seen at the end of the sixth week of treatment it was doing well and was quite happy.

It will be well to consider in detail the signs and symptoms which may develop in the course of scurvy.

Hemorrhage.Hemorrhage of the gums is one of the characteristic signs of scurvy. For a reason not clearly understood it involves first and foremost the tissues about the upper incisors. If, however, we fix our attention too narrowly to this region we may be led into error; in several instances we have first encountered hemorrhages about the molar or the canine teeth, which had been overlooked because the anterior part of the gums had been found normal. Where teeth are absent or not in the course of eruption hemorrhages do not appear. At the onset the gums may be merely deep red or bluish red, especially if they overlie upper incisors which are close to the surface. Hemorrhage is particularly apt to occur where the edges of the teeth have just broken through the mucous membrane. In this connection the question arises as to whether every hemorrhage of the gums in infants is to be considered a sign of scurvy. This is a matter of some diagnostic importance. We have seen hemorrhages of the gums at the site of erupting molar teeth where, as prolonged observation proved, not even latent scurvy existed. This sign should not, therefore, be regarded as pathognomonic. In two infants entirely free from scurvy we have noted slight hemorrhage of the gums overlying incisor teeth. It should be well understood that such an occurrence is most exceptional; it is to be attributed probably to bacterial invasion or to a constitutional hemorrhagic condition. One of these cases was the following:

The infant was 8½ months old. It had been nursed by the mother up to this time and was well nourished, but when first seen had some fever, probably due to a grippe infection. About ten days later distinct linear hemorrhages of the gums were noted over the two upper incisor teeth. No treatment was instituted for this condition, and it healed within a week. There was no subsequent sign of similar hemorrhage or of other scorbutic manifestation in the months that the baby was under observation.

The localization of the hemorrhage in the gums is due largely to trauma, occasioned by the sharp contact of the jaws or of the nursing-bottle. Local infection plays almost no rÔle in infants, although in the adult where there is caries of the teeth it frequently incites hemorrhage. Dental caries and gingival infection may lead to local hemorrhage, even where the nutritional conditions are normal.

Subperiosteal hemorrhage is a sign distinctive of infantile scurvy, although it must be borne in mind that it may take place in the scurvy of adults. It involves most frequently the lower end of the femur and the tibia, but occurs in connection with the humerus, the mandible, the scapula and other bones.47 The hemorrhage usually manifests itself as a swelling which appears suddenly at the lower end of the femur or femora. It is brought about by trauma, at times in the course of diapering, or by manipulation in testing for local tenderness. The swelling is very tender, and varies in size from an enlargement which is difficult to appreciate, to one which renders the leg fully twice its normal circumference (Fig. 18). It may involve merely a small part of the long bone or extend up or down the shaft for a long distance. As might be supposed from the nature of this lesion, the enlargement persists for weeks, frequently long after the gums and the general symptoms have disappeared. During this period it becomes harder and less tender, and may develop the consistency of bone; it is in this stage that such swellings have been diagnosed as new growths, and that incision or even amputation of the leg has been resorted to. In subacute cases the swelling—which must be regarded as hemorrhagic rather than scorbutic—may be absorbed gradually in spite of the fact that no antiscorbutic food has been given. This has led to the mistaken conclusion that the scurvy has been cured without dietetic treatment.

Subperiosteal hemorrhage may be clearly seen by means of the fluoroscope or in X-ray photographs (Figs. 16 and 17). The shaft of the bone appears surmounted by an elongated blood-clot, which is more or less distinct according to its age and density. It may become calcified, as clearly seen in figures. More often the periosteum undergoes calcification or ossification, especially near the site of the separation of the epiphysis. This gives rise to a bizarre radiographic picture which may be difficult to interpret—the opaque strip or streamer being almost unrecognizable as periosteum (Fig. 17).

Subperiosteal hemorrhage and separation of epiphysis. Roentgenogram Fig. 16.—Infant 11 months old. Separation of lower epiphysis of femur. Fraying of end of femur and head of tibia. Subperiosteal hemorrhage surrounding lower part of shaft of femur, with calcification of periosteum and of clot. Periosteal “tags” and “streamers.” Roentgenogram Fig. 17.—Infant 11½ months old. Separation of lower epiphysis of femur with marked subperiosteal hemorrhage. Typical periosteal “tags” or “streamers.” The connection of these “streamers” with the periosteal layer is evident.

Hess and Unger observed that in several instances where subperiosteal hemorrhage had been diagnosed, X-ray examination disclosed that the swelling of the thigh was due mainly to infiltration of the muscles and subcutaneous tissue. It is surprising how an infiltration of serum gives rise to a swelling which resembles in appearance and consistency the classical subperiosteal tumor.

The skin, mucous membranes and subcutaneous tissues are frequently the sites of hemorrhage. There is a difference of opinion as to how frequently petechial hemorrhages occur in scurvy, particularly as to whether they are encountered early in this disorder. Great variation in this regard may be noted in individuals and in groups of cases occurring at different times. In the cases reported in 1914 by Hess and Fish, petechial hemorrhages were frequently an early sign, to such an extent that they led to a study of the blood and blood-vessels in this disorder. The hemorrhages in this “scurvy epidemic” were the result of a complication of scurvy with an infectious disease. It is not necessary, however, for infection to exist to bring about a rupture of the small vessels. The idiosyncrasy of the individual has to be considered as well as the fact that infants have a tendency to develop minute skin hemorrhages, especially such as have an exudative diathesis. In the course of scurvy, petechiÆ may be found not only in the skin, but in the mucosa of the mouth, especially overlying the hard palate, and also in the palpebral conjunctiva, identical with the minute petechiÆ so significant of general sepsis. In addition to these minute hemorrhages larger ones are not infrequently found in various parts of the body, especially in the neighborhood of the joints. They appear as discolorations of various intensities and shades, and are often interpreted as being merely the result of bruises. These have been encountered most often about the knee-joint, on the forehead, or in the concha of the external ear, where they may best be seen by means of transmitted light.

A form of hemorrhage which must be especially mentioned, although it is very infrequent, is that taking place into the orbit, leading to a proptosis of the eyeball, usually the left (Still). This sign should be borne in mind, as it occurs occasionally before other symptoms have rendered the diagnosis clear, and may lead to a diagnosis of tumor.

As mentioned above, hemorrhages into the muscles or between the muscle planes are very common in adults, leading to hard swellings, the typical “scurvy sclerosis.” Such effusions occur much less frequently in infants, due probably to their lack of activity. In addition to these hemorrhages there are serous effusions of the muscles similar to those which are found in the pleural and pericardial cavities. These effusions are very striking at necropsy, when one incises the muscles—for example, the muscles of the thigh. During life they are frequently mistaken for subperiosteal hemorrhages.

Less frequently there are hemorrhages into the internal organs. These, however, play a comparatively insignificant rÔle in the symptomatology of this disease. At postmortem examination we find numerous hemorrhages of the pleura, pericardium and peritoneum, which rarely produce symptoms during life. Still records a case with marked abdominal pain and swelling, which he believed to have been due to hemorrhage into the wall of the intestine. As previously mentioned, O’Shea met with a case of hemorrhage into the cÆcum which was mistakenly operated upon for appendicitis. HÆmothorax and hÆemopericardium occur, especially associated with local inflammatory processes of tuberculous nature. The clinical aspect of hemorrhage of the gastro-intestinal and the genito-urinary tracts will be considered elsewhere.

In the scurvy of adults as well as that of infants, the nails and the hair are altered by the nutritional condition. Mention has been made of the hyperkeratosis recently emphasized by Wiltshire as an early sign, occurring especially on the thighs and legs. The skin is frequently dry, the so-called “goose skin” that is seen in some poor nutritional states. The nails are thin, brittle and lined; at times small hemorrhages will be noted beneath them. The hair also becomes thin and dry, and there is a tendency for petechial hemorrhages to develop at the roots.

In a paper on the therapeutic value of yeast and of wheat embryo the author called attention to the fact that eczema may occur in connection with infantile scurvy, and be cured by means of orange juice. We have met with eight cases of eczema in infantile scurvy, which, in almost every instance, have yielded promptly to an antiscorbutic, thus proving their scorbutic nature. A case of this kind is the following:

M. L., seven months old, was getting “Molkenadaptierte” milk, and in addition autolyzed yeast. On May 25th it developed nasal diphtheria, but soon afterward did well. On June 9th it was gaining, but its pulse was 160 and respirations 80. A few days later it developed marked eczema about the neck and to a less extent on the back and buttocks. The “capillary resistance test” was negative. Cardiographic tracings showed merely a simple tachycardia. A few days later petechial spots appeared at the site of the eczema. On June 17th orange juice was given. The appetite improved, the cardiorespiratory syndrome disappeared, and the child began to gain. The eczema also cleared up rapidly without any local treatment.

We wish to draw particular attention to this skin condition, as it is generally not mentioned, or has been regarded merely as a chance occurrence. The report of the American Pediatric Society includes two cases of eczema as a complicating condition. This symptom is of special interest in view of the fact that a similar skin lesion constitutes one of the typical signs of pellagra. In a case of infantile scurvy we have seen an eruption at the nape of the neck which was symmetrical and greatly resembled that of pellagra. Andrews refers to the occurrence of eczema in his description of infantile beriberi.

In a paper published a few years ago attention was drawn by Hess and Fish to the fact that infantile scurvy frequently is associated with the exudative diathesis of Czerny, a pathological condition which predisposes to the development of exudations of the skin and the mucous membranes. Infants suffering from this condition—intertrigo, eczema, recurrent bronchitis—seem to be particularly susceptible to scurvy and to develop it more quickly than others.

As is well known, edema constitutes a not infrequent symptom of adult scurvy. It has not, however, been accorded any place in the symptomatology of infantile scurvy. We do not refer to the edema in connection with subperiosteal hemorrhage or separation of the epiphyses of the long bones, but a mild and peculiar form which is seen early in the disease. It involves most regularly the upper eyelids, and the legs—especially the skin covering the lower part of the tibiÆ. In the latter site it differs from edema as usually encountered, in that it does not pit on pressure; it is firm, tense, causing some glossiness of the overlying skin, which is rendered difficult to wrinkle or to pinch between the fingers. Not infrequently the skin is slightly reddened, a sign of interest, in view of a similar, although much more intense, hyperÆmia seen in pellegra.

In addition to this very mild edema there may be marked swelling, resulting in what might be called, following the terminology of beriberi, “wet scurvy.” The legs, body and even the face may be swollen. This has been frequently described in adult scurvy, and occasionally in infantile scurvy. The first case of infantile scurvy described in America, that of Northrup, had marked edema of the scrotum. Edema is frequently met with in “ship beriberi,” a disorder considered by some writers to be a combination of beriberi and scurvy.

The symptom leading to the diagnosis of scurvy most often is tenderness or swelling of one of the extremities, as the antecedent clinical signs, comprising latent scurvy, are generally overlooked. These manifestations involve usually the distal end of the thigh or thighs. The tenderness is elicited most readily by pressure just above the knee, which causes the baby to wince, and to quickly flex the thigh, a reaction termed by Heubner “the jumping-jack phenomenon.” As a result of pain and tenderness, the leg lies often immobile in a state of pseudo-paralysis (Fig. 18). There may be tenderness elsewhere than in the long bones. Kerley refers to two cases showing tenderness of the spine, and we have seen a similar case. Not infrequently there is tenderness of the chest wall, the earliest symptom noted by nurse or mother being unaccountable crying whenever the baby is lifted by the thorax. This is largely due to the sensitiveness of the ends of the cartilage and bone which are pressed together at their junction.

Infant with marked scurvy. Characteristic position Fig. 18.—Infant with marked scurvy. Characteristic posture and swelling of right thigh.

An early sign of infantile scurvy is beading of the ribs—the development of a “rosary” similar to that characteristic of rickets (Fig. 19). This has recently been described by Hess and Unger in an article devoted to this subject. That this rosary is truly scorbutic and not rhachitic is proved by the fact that it recedes rapidly when antiscorbutic foodstuff is given, and that it remains uninfluenced by treatment with cod liver oil. A similar scorbutic rosary occurs in guinea-pig scurvy, but has been termed “pseudo-rhachitic.” It is important that this sign should be recognized, as it is probable that much of the confusion regarding the relationship and frequent association of these two diseases is due to considering the beading rhachitic. The interpretation of infantile scurvy as “acute rickets,” the view held previous to the writings of Barlow, was based largely on the development of the rosary. To-day the error is made of regarding early scurvy as chronic rickets; the rickets supposed to be occasioned by a diet of condensed milk is probably more often scurvy. This beading differs generally from the round knobby “rosary” usually encountered. It is more angular, the junction taking on a step-like form, as if the abutting ends of the cartilage and the bone were of unequal size, and not well fitted to each other. In the accompanying radiograph (Fig. 19) it will be noted that the “beads” present an irregular appearance.

Scorbutic beading of ribs. Roentgenogram Fig. 19.—Same infant as in figure 17. Scorbutic beading of the ribs (rosary). This developed on a diet which included cod liver oil, and decreased when an antiscorbutic was given. Note peculiar ragged appearance of “beads.”

In Figs. 6, 16 and 17 will be seen illustrations of a separation of the epiphyses of the head of the humerus, and of partial and of complete separation of the lower ends of the femora. This is a frequent lesion of fully developed scurvy in infants, children, and even in young adults. It is most frequent at the lower end of the femur, the upper end of the tibia, the head of the humerus, and the costochondral junctions. It is to these epiphyseal separations that the term fracture or infraction usually refers. Union is remarkably perfect even where no splint has been employed, and nature has effected the cure (Fig. 7). Occasionally there is some deformity, as when coxa vara develops. The callus is often remarkably large; an old callus sometimes undergoes destruction in the course of scurvy.

“White line.” Roentgenogram Fig. 20.—Radiograph. Infant 14 months of age, showing “white line” at wrist some months after cure of scurvy.

We have referred to use of rÖntgenograms in connection with separation of the epiphyses, subperiosteal hemorrhage, cardiac enlargement and beading of the ribs. In addition to its application in these connections, the X-ray may be of service to show a peculiar alteration of the ends of the long bones—the white line of Fraenkel. This is portrayed in Fig. 20. It is best seen at the lower ends of the radius and femur, and appears as a white, transverse, somewhat irregular band. Its diagnostic value has been greatly exaggerated, as it is frequently not present when the disease is advanced (observe radiographs illustrating separation of the epiphyses). This sign should therefore not be relied on for establishing the diagnosis. Furthermore, changes may be seen in connection with rickets (cases receiving antiscorbutic diet) which are very difficult to differentiate from the “white line.” It cannot be employed as a criterion of the progress of the case, as it may persist for months after all other signs and symptoms have disappeared.

The joints may be involved in scurvy. In most instances, however, where swelling of the joints is diagnosed, the lesion is periarticular. An effusion of serum or of blood does occur occasionally into the joints and has been found at operation, at necropsy, and by puncture. If these effusions are allowed to go undisturbed, to be absorbed as a result of antiscorbutic treatment, they rarely suppurate. Czerny and Keller report the articular fluid as invariably sterile.

The cardiovascular system has been given but scant attention in connection with scurvy. Adults complain not infrequently of palpitation and pain over the pericardium, or rather of a tightness or oppression in the chest. Little information is given regarding the size of the heart. Darling described enlargement of the heart, especially a right-sided hypertrophy, which he thought was pathognomonic of the Rand type of scurvy. The pulse is described in some cases as slow, and in others as rapid. In descriptions of infantile scurvy the entire subject is generally passed over without mention—for example, in the excellent report of the American Pediatric Society nothing whatsoever is stated regarding the heart’s action or the pulse. Barlow wrote: “There is nothing to note regarding the heart and lungs.”

Cardiac enlargement. Roentgenogram Fig. 21.—Radiograph. Scorbutic infant 14 months of age, showing cardiac enlargement and broadening of shadow at base of heart.

In a paper written a few years ago, it was pointed out by the author that there is frequently enlargement of the heart, and more especially of the right heart. This can be elicited at the bedside and has been substantiated in numerous cases by means of the RÖntgen-ray, which demonstrates not only enlargement of the heart, but also a marked broadening at its base, at the site of the large vessels (Fig. 21). These phenomena resemble closely the description of Reinhard in cases of beriberi.

Necropsy protocols usually are incomplete and unsatisfactory in their descriptions of the heart. The excellent monograph of Schoedel and Nauwerk, however, which reports five careful necropsies, contains the following data regarding three:

1. Pericardial fluid somewhat increased, both ventricles moderately dilated, the right somewhat hypertrophic.

2. The heart showed a hypertrophy of the right and left ventricles, as well as dilatation of the right ventricle.

3. The right ventricle dilated and slightly hypertrophied, the muscles pale and tough.

In addition to this enlargement of the heart, or perhaps associated with it, there is a combination of signs which has been termed “the cardiorespiratory syndrome” (Hess). It will be noted in the above description of a case of subacute scurvy, that the pulse- or heart-beat was frequently over 150, and the respiration 60. These phenomena were noted in several instances before their significance and intimate relationship to scurvy were realized. The heart-beat not infrequently is found to be 200 per minute, and to be characterized by marked lability—increasing to an astonishing degree as the result of slight exertion or excitement. A mild febrile disturbance causing a rise of temperature to little more than 100° F. will send the pulse-rate up 30 beats. It must not be thought that this refers to severe cases; the babies we have in mind are similar to the one cited as an instance of subacute scurvy. Apparently they are not ill, but show merely some tenderness of the thighs, pallor, and the other minor signs described. The cardiographic tracings showed a simple tachycardia with an exceptionally tall T-wave in some tracings, such as is commonly seen in exophthalmic goitre (Fig. 22).

Electrocardiogram showing “cardiorespiratory syndrome” Fig. 22.—Electrocardiogram in case showing cardiorespiratory syndrome. Tachycardia with exceptionally tall T-wave.

The rapidity of respirations is perhaps a more delicate indicator of this disturbance than the pulse and has been found to be markedly affected when the latter was merely slightly increased in rate. For example, in one instance the respirations were 64, 60 and 64 on three successive days, while the pulse was 124, 141 and 136; in other words, there was a 2:1 instead of the normal 4:1 pulse-respiration ratio. The accompanying chart (Fig. 15) illustrates the phenomenon in all its details better than a verbal description. There is one point in connection with it, to which especial attention should be called. This is a reaction evident at a glance at the chart—the sharp drop in the pulse and in the respiratory rate when orange juice was given. It is the essence of the phenomenon; a therapeutic response which proves that the rapidity is scorbutic in nature.

The main involvement of the respiratory system in scurvy is the polypnoea just described in connection with the cardiorespiratory syndrome. There is no aphonia, a sign so typical of adult and of infantile beriberi, although at times the voice is abnormal and whining. The lungs frequently show some dullness posteriorly, which may be due to engorgement or to the pressure of the enlarged heart. Pneumonia is a frequent complication and edema a terminal event. Hydrothorax associated with hydropericardium is of frequent occurrence, and was noted in the early description of this disease in adults and in the first account of Barlow. These effusions rarely progress to what may be termed the clinical degree and under antiscorbutic treatment are rapidly absorbed.

It is commonly thought that scurvy does not involve the nervous system; that this is a feature which distinguishes it sharply from beriberi, another “deficiency disease.” This view is incorrect, for the nervous system is probably affected in many cases of scurvy. The rapidity and lability of the pulse, combined with the rapid respirations, would seem to be due to a disturbance of the vagus mechanism. It is true that in beriberi the vagus is involved to a still greater extent, especially its recurrent laryngeal branch which brings about the characteristic aphonia. In scurvy the knee-reflexes are generally increased. Very rarely they are absent in infantile scurvy, as described in adults. It is impossible to judge whether the pain and tenderness in infants are due in part to a sensitiveness of the nerve trunks as well as of the periosteum. Careful studies in adult scurvy should furnish an answer to this question. No methodical examination for areas of anÆsthesia or parÆsthesia, signs which occur so frequently in connection with beriberi, has been carried out in scurvy. In certain epidemics, however, pains in the limbs have been prominent symptoms.

The optic discs are generally pale in both infants and in adults, with occasional signs of neuredema. Nyctalopia, so frequently encountered, must be regarded as a circulatory symptom rather than as one of nervous origin.

In a recent paper the author described a focal degeneration of the lumbar cord in a case of infantile scurvy, the lesion involving mainly the anterior horn cells (Figs. 3 and 4). In view of this report it would be well to watch for corresponding clinical signs of involvement of the spinal cord. Herpes has been described in connection with both adult and infantile scurvy. In one of the early cases in the American literature Fruitnight reported a case with herpes in a girl five years of age. In considering the rÔle of the nervous system, mention should be made of cases where sweating constituted an important symptom. Finkelstein lays particular stress on this symptom in infantile scurvy. We have not met with it frequently; possibly it is due in part to complicating rickets.

As would be expected, the nervous system is at times the site of hemorrhage. Such lesions cannot, however, be considered essentially nervous. For instance, hemorrhage into the meninges may occur, as in the case of Sammis, where there was “a general clonic convulsion” before death, and a blood-clot 2½ inches long by ½ inch wide was found at necropsy between the dura and arachnoid. Fife reported a similar case. Finkelstein also has drawn attention to the occurrence of meningeal hemorrhage, and Hess and Fish reported obtaining bloody cerebrospinal fluid from a case with meningeal symptoms. Recently Aschoff and Koch have depicted hemorrhages in the sheath of the sciatic nerve, which undoubtedly must have given rise to symptoms during life.

In view of many of these symptoms, especially those involving the vagus, scurvy must be looked upon as a disorder which may seriously affect the nervous system. Furthermore, when we note the marked reaction brought about by the antiscorbutic vitamine—for example, the sharp fall in the rate of respirations and of pulse, as shown in Fig. 15, after giving orange juice, we must conclude that the antiscorbutic vitamine functions, at least indirectly as an antineuritic vitamine—that it must possess this character to allay the various nervous signs of this disorder.

The urinary system is frequently involved in the course of scurvy. Among 38 cases Still reports that 89 per cent. gave evidence of urinary changes and that 60 per cent. showed hÆmaturia. Finkelstein found urinary signs in at least a third of his cases. Our figures, the result of a study of subacute and mildly acute cases, correspond more nearly with those of Finkelstein.

The occurrence of pronounced renal hemorrhage as a first symptom of scurvy is emphasized in many descriptions of this disease, and has impressed itself in the minds of physicians. It is true that this occurs sometimes at the onset, as does hemorrhage into or about the joints, or hemorrhage behind the eyeball. It is well to bear these possibilities in mind, but they must be regarded as very exceptional early signs of this disorder. We have encountered frank hÆmaturia but once in the early stage of infantile scurvy. The blood emanates generally from the kidneys, although the submucous hemorrhages of the bladder as well as in the urethra, described both in man and in guinea-pigs, indicate that the blood in the urine may have its origin lower down in the tract. This bleeding should be regarded not as a sign of nephritis, but rather as a hemorrhagic manifestation. It is less frequent in adults than in infants. O’Shea reports some degree of hemorrhage in 15 per cent. of his cases (adults).

A true nephritis, however, may occur in connection with scurvy. There may be albumen and many casts, or a urine loaded with casts and cylindroids. These peculiar casts may appear suddenly, as in the alimentary intoxication of infants, and disappear just as rapidly when antiscorbutic treatment is given. The urine may contain a large number of pus cells as in pyelitis. This condition may be accompanied by irregular fever, but in two instances we have encountered it where the temperature was normal. It is to be regarded, probably, merely as one of the manifestations of secondary infection so commonly associated with scurvy. Some pus cells may continue to be present in the urine for a period of months. This is likewise true of the red cells. We have under observation at present an infant which had subacute scurvy almost three years ago and still has red blood-cells in the urine.

Oliguria is a common symptom of both adult and infantile scurvy. Lind mentioned this symptom, and in this connection remarks on the beneficent effect of antiscorbutic treatment. Charpentier called attention to the fact that in a case of scurvy the urine decreased from 1250 g. to 800 g. The report of the American Pediatric Society mentions scanty urine in 9 cases and suppression of urine in one. This sign, however, was not emphasized until recently, when Gerstenberger, and Hess and Unger drew attention to its frequent occurrence in infants. It has some diagnostic significance and should be borne in mind where a decreased excretion of urine is reported. A counterpart of this symptom is the sudden outpouring of urine frequently noted after antiscorbutic treatment has been instituted. This polyuria accounts for the loss of weight or lack of gain which sometimes accompanies unmistakable general improvement, and which is difficult otherwise to understand (Fig. 23). It is interesting to learn that oliguria occurs commonly in both adult and infantile beriberi.

Stationary weight during cure of scurvy. Oliguria followed by polyuria Fig. 23.—Joseph G., aged 9 months. Chart showing stationary weight (due to oliguria followed by diuresis) in spite of marked variation of fluid intake. A=Schloss milk; B=cod liver oil; C=egg yolk; D=1 ounce of orange juice; E=potato (orange juice stopped).

One of the earliest, as well as one of the most constant symptoms of scurvy, is a lack of appetite. It is a typical sign of latent scurvy, although occasionally we have met with cases where the appetite remained unimpaired until the hemorrhagic stage was reached. In adults there is sometimes bulimia and a marked capriciousness of the appetite. Anorexia is a true scorbutic symptom, disappearing with remarkable rapidity when antiscorbutic food is given, and not capable of alleviation by tincture of gentian or other vegetable bitters. Whether it depends upon a lack of secretions in the gastro-intestinal tract is not known, as there has been no thorough study of this aspect of the disorder. The hydrochloric acid generally is deficient in cases of scurvy. Recently McCarrison has laid emphasis on the importance of the impairment of the digestion and assimilative function in scurvy. This subject gains added interest in view of the recent reports of Uhlmann as well as of Voegtlin, showing that water-soluble vitamine acts as a stimulant for the various secretions of the gastro-intestinal tract.

As a result of McCollum’s statement that scurvy is due mainly to constipation, marked attention has been directed recently to the action of the bowels in this disorder. This question has been discussed in the chapter on etiology, and, therefore, will be referred to in this place merely from the clinical viewpoint. In our experience the activity of the bowel varies greatly in cases of latent or subacute scurvy. In a great many instances it has been normal; more often there has been slight constipation, and exceptionally there has been irregular diarrhoea. In other words, no causative relationship or parallelism could be observed between the emptying of the intestinal tract and the development of scurvy. This in general has been the experience of others. In the report of the American Pediatric Society the bowels are stated as having been regular in 74 cases, irregular in 15, constipated in 126, and diarrhoeal in 65. It may be added that we were unable to cure scurvy by means of liquid petrolatum or phenolphthalein, either in infants or in guinea-pigs, and likewise unable to protect guinea-pigs from scurvy by means of various laxatives. On the other hand, opium given in the form of the camphorated tincture did not lead to an intensification of the symptoms, although, in one case, the bowels did not move for over three days.

As complications involving the gastro-intestinal tract may be mentioned the vomiting of blood, which is stated in the above report as occurring in 2 of the 361 cases, as well as bleeding from the bowel, which was noted in 37 cases, in 12 of which there was bloody diarrhoea. However, these are late symptoms, and correspond to the mycotic ulcers which are so frequently found, especially in the large intestine, in cases of scurvy. Mention may again be made of the fact that hemorrhages may occur under the peritoneum and give rise to symptoms simulating appendicitis or general peritonitis.

Jaundice has been described in connection with certain epidemics of scurvy. To our knowledge it has not been reported in infants.

The presence of worms has been frequently reported in the bowel or in the stool of patients suffering from beriberi. There have been no similar investigations in relation to scurvy. It would be interesting to inquire into this question, as it is quite possible that a lack of antiscorbutic foodstuff may favor the presence of parasites in the intestinal canal.

Before closing this consideration of the involvement of the alimentary tract, we would call attention to the relation of stomatitis to scurvy. Among adults this is a common complication. In infants it is uncommon, due to the absence of carious teeth and secondary infection; we have encountered it in but two instances. Stomatitis is of importance in this connection, as it frequently develops on the basis of malnutrition, scurvy being one of the disorders which may constitute the substratum. Such may be the case where stomatitis occurs in epidemic form —for example, among large bodies of troops. It may be remarked that stomatitis at times was a very common disease among the soldiers in the recent war.

TABLE 4

The Platelets and Other Blood-Cells in Scurvy
Name Date Platelets Leukocytes Erythrocytes Hmgl.%
(Sahli)
Remarks
M.H. 5/3 280,000 ...... ......... ..
5/4 248,000 10,000 ......... ..
5/5 ....... ...... ......... .. Boiled orange juice given.
5/8 ....... ...... 4,300,000 35
5/16 ....... 15,900 ......... ..
7/2 ....... 6,800 5,456,000 40 Well but pale.
A.L. 5/3 300,000 ...... ......... ..
5/5 ....... 21,000 ......... ..
5/9 ....... ...... 5,480,000 ..
5/11 320,000 ...... ......... ..
5/13 ....... ...... ......... 65
5/16 ....... 11,500 ......... ..
H.C. 5/8 320,000 20,000 ......... ..
5/9 ....... ...... 5,340,000 ..
5/13 ....... ...... ......... 70
5/16 362,000 ...... ......... ..
B.B. 5/4 496,000 21,000 ......... .. A severe case.
5/13 585,000 14,000 3,200,000 70
5/18 ....... 17,600 ......... ..
7/13 ....... 40,000 7,672,000 82 Has gained well lately.
7/15 ....... ...... 7,640,000 88
H.Y. 5/15 560,000 ...... ......... ..
5/16 424,000 ...... ......... ..
7/9 ....... ...... 5,750,000 45

Scurvy is associated with an alteration of both the blood and the blood-vessels. The characteristic pallor, which is one of the most common as well as earliest symptoms, is due in a large measure to the anemia. This anemia is of the secondary type, but has definite peculiarities, and does not resemble that encountered in the course of tuberculosis, rickets or marasmus. The hemoglobin is greatly diminished, far out of proportion to the decrease in the number of the red cells. Not infrequently we will find a hemoglobin index of 0.5. Table 4, above taken from the article on this subject by Hess and Fish (1914), brings out the details of the blood-picture. It shows that there may be a polycythemica, which may persist after the other signs of the disorder have disappeared. Brandt has recently made similar observations, reporting in one instance over ten million red cells two months after treatment. In soldiers suffering from scurvy Wassermann has encountered cases where, during convalescence, the red-cell count has risen to over six or seven millions and the hemoglobin to 110 or 120 per cent. Under the microscope the red cells show poikilocytosis, anisocytosis and a lack of hemoglobin; they are slightly enlarged, with the occasional occurrence of exceptionally large cells resembling the “dropsical cells” described in connection with chlorosis. Sometimes a few nucleated red cells and myeloblasts are seen; megaloblasts are also reported.48 The blood-picture bears a remarkable similarity to that of chlorosis, a point of interest, in view of the fact that both scurvy and chlorosis have been attributed to a disordered function of the endocrine glands. The “dropsical cells” suggest a disturbance of the salt balance in the plasma. In some cases we have found a decreased fragility of the red cells, which also has been described in chlorosis.

The total number of leucocytes is slightly increased. In our cases the mononuclear cells have averaged 66 per cent., which is somewhat high even for infants. This has been the experience of Labor, who, however, also describes an eosinophilia during convalescence, a phenomenon which we have not encountered. Some describe a marked increase in the polynuclear cells, which, probably, is to be regarded as the reaction to secondary infections. There is indeed a marked difference of opinion in regard to the morphology of the blood in scurvy in adults as well as in infants. Some found a large number of one type of cell—for example, nucleated red cells, myelocytes, eosinophiles—whereas others have failed to observe an increase of these cells. The divergent reports probably should be attributed to the fact that the investigators are describing scurvy of various grades of severity, of different stages of development, or complicated by intercurrent disease.

NobÉcourt, Tixier, and Maillet have questioned whether there is always complete recovery from this anemia, which is severe from the standpoint of hemoglobin and iron. The older authors reported instances where men have been weakly and ailing for the remainder of their lives after an attack of scurvy. In some infants pallor and anemia may persist for months after apparent cure; however, this is the exception rather than the rule.

In view of the fact that scurvy frequently is classed as a hemorrhagic disease, and that hemorrhages play such an important rÔle in its symptomatology, a consideration of the factors concerned in the coagulability of the blood is of interest. In an investigation (Hess and Fish) it was found that the oxalated plasma (of blood taken directly from a vein) showed a slightly delayed coagulation time—eight to fourteen minutes. The “bleeding time” carried out according to the simple method of Duke was slightly increased. Holt reports a case where a child bled to death following incision into an epiphyseal swelling at the lower end of the femur. The number of blood platelets is increased, running parallel, as is usually the case, with the number of red cells (Table 4). This increase in the blood-platelets, recently confirmed by Tobler and by Brandt, is a very exceptional phenomenon, and was not anticipated in connection with a disorder characterized by hemorrhage. The antithrombin content of the plasma is normal.

The investigation was directed to a study of the integrity of the blood-vessels in order to account for the hemorrhages. To this end the “capillary resistance test” was devised.49 In the majority of cases this was found to be “positive” (the blood-vessels showing an increased permeability) and to become negative when antiscorbutics were given and the symptoms disappeared. This shows that the cellular structure of the vessels is altered in the course of scurvy, and indicates probably that this is an important cause of the hemorrhages. The edema of the face and ankles, the outflow of serum into the body cavities and into the muscles (Barlow) must be regarded as other evidences of the inadequacy of the vessel walls. The tendency of children with exudative diathesis to develop scurvy is perhaps still another manifestation of vascular weakness. This point of view has been strengthened recently by the pathological studies of Aschoff and Koch, who regard scurvy as a nutritional disorder in which there is a lack of some colloidal substance needed for the normal structure of the vessels.

When one makes a subcutaneous puncture in infants suffering from scurvy, a small hemorrhage very often develops at the site of the puncture wound. This is not the case when one makes a hypodermic puncture in a normal person or in a hemophiliac, although it does occur in cases of purpura. This “stick test” is not a constant sign of scurvy, but, like the capillary resistance test, was found in many cases and disappeared with the subsidence of the disorder. It shows that the cells of the skin and subcutaneous tissues are affected, and possibly that their thromboplastic power is diminished.

Nutrition and Growth.—The general nutrition suffers in scurvy as the disease progresses. It is a mistake, however, to picture the scorbutic individual, either adult or infant, as in a state of malnutrition. Not infrequently he appears well nourished, an appearance which is heightened by the slight edema of the face. Infants generally for a period of weeks or months preceding the onset maintain a stationary weight. This may be the only sign of the scorbutic condition. For example:

An infant seen in 1915 gained about one-half a pound during the months of February, March, April and May. At this time it was somewhat over 9 months of age and had never received raw milk or other antiscorbutic food. In June it was given orange-peel juice, and gained 2 pounds within a month. There were no other scorbutic signs or symptoms, and no loss of appetite during the months of February and March, although the baby was suffering from a progressive scurvy.

The growth impulse of the body throughout an attack of scurvy remains unimpaired, being merely in an inactive or quiescent state. Fig. 14 shows this very well, demonstrating that when an antiscorbutic food is added to the dietary the gain may be abnormally great—there may be supergrowth. Generally such marked increases are due to an increase in the consumption of food, following the stimulation of the appetite. However, decided gain in weight may follow the giving of orange juice or other antiscorbutic despite the fact that the intake of food is maintained at the same level.

Although it may be stated as a principle that the development of scurvy is accompanied by a failure to gain in weight, there are exceptions to this rule. Under certain conditions the weight may follow a perfectly normal course during the entire period. Fig. 24 illustrates this clinical paradox:

A baby was seen in January, when it was 7½ months of age. Toward the end of February, in spite of constant and normal gain in weight, he manifested unmistakable signs of scurvy—peridental hemorrhage over the upper incisor teeth, which were erupting, and tenderness of the lower ends of the femora. The scorbutic nature of these signs were substantiated by their prompt subsidence on the administration of orange juice. We explain the phenomenon as follows: This baby had been starved in a two-fold sense throughout the first months of its life—it had received a diet lacking in caloric value as well as deficient in antiscorbutic vitamine. Its growth impulse had been held in abeyance for months by both of these factors. When sufficient calories were supplied in the dietary, growth was no longer repressed, and a steady gain resulted in spite of the continued inadequacy of the antiscorbutic factor.

It has been shown that during the period of infancy undernourishment must be extreme to occasion stunting of growth in length. In animals Aron demonstrated that lack of nutrition led to a decrease of the fat and of the muscle of the body, but that the skeleton nevertheless continued to grow, and the ash content of the body to increase. In marasmus, or infantile atrophy, the baby usually grows in length, although its weight remains stationary or decreases. In scurvy we have shown that there is frequently a definite retardation of growth in length, an observation which has been recently confirmed by Epstein in babies which developed this disorder in the foundling asylum of Prague during the war. This fact shows how profoundly the metabolism must be disturbed by this disorder. Figure 25 portrays this retardation in growth and the sharp reaction when orange juice was added to the dietary. It also demonstrates that the growth impulse remains unimpaired and capable of quick response when the essential food factor is furnished.

Retardation of growth in length when no orange juice was given and supergrowth when given once more Fig. 25.—Showing retardation of growth in length during the period when no orange juice was given and supergrowth when it was given once more, O. J.=orange juice. O. P. J.= orange-peel juice. Lower curve represents the normal.

Fever.—Fever frequently accompanies scurvy. It is generally of a low grade, ranging between 100° and 101°, as may be seen in Fig. 15. There is a difference of opinion as to whether the rise of temperature should be considered as truly scorbutic in nature, as “scorbutic fever,” or regarded merely as a condition grafted upon the nutritional disturbance. A phenomenon which might seem to argue for its essential scorbutic character is the sharp subsidence on giving antiscorbutic food. On the other hand, this may quite as well be interpreted as due to a secondary reaction, checking the absorption of toxins or bacteria. High temperatures—for example, fever of 103° or over—are attributable to a complicating infection and should lead to careful examination for the source of the trouble; pyelitis should particularly be borne in mind. In a recent case fever of uncertain origin disappeared following the transfusion of blood.

We have already considered numerous complications of scurvy, and shall therefore not go over this ground again. Many of them are due to hemorrhages or to serous effusions in various parts of the body. Another large group in adults as well as in infants are the result of infection. The respiratory tract is particularly susceptible, pneumonia constituting the most common cause of death. In infants we meet with frequent attacks of “grippe,” widespread occurrence of nasal diphtheria, furunculosis and torpid ulcers of the skin, pyelitis, otitis, adenitis, etc. We have encountered nasal diphtheria—with typical bloody mucous discharge—so frequently in connection with scurvy, that where this local infection occurs among a group of infants they should be carefully examined for latent or mild scurvy. Aschoff and Koch recently have laid emphasis on the frequency with which diphtheria complicated scurvy among adults (soldiers). Dysentery is another complication resulting from an invasion of bacteria. Local infections occur more often in adults than in infants—cervical adenitis following gingival pyorrhoea, “bubo” of the groin following infection of the lower extremity, abscess of the calf of the leg following hemorrhage into this region.

Scurvy sometimes occurs in epidemic form, especially in the army, but also, as in Russia, among the civilian population. This results when a large group of individuals have been maintained on a limited and inadequate ration, and especially where this nutritional condition is complicated by intercurrent infection. It should not be interpreted as evidence of the bacterial origin of scurvy. A few years ago the author reported an epidemic of scurvy in connection with an outbreak of grippe in an infant asylum. Twelve infants in one ward were affected. The signs were atypical—an undue degree of hemorrhage occurring at atypical sites (Table 5). It will be noted from the table that the ages of the infants, the distribution of the hemorrhages, the development of signs (in some instances) in spite of antiscorbutic treatment, the sharply defined epidemic character, distinguish these cases from the scurvy commonly seen. This is an instance where latent scurvy was prematurely changed to acute scurvy by an intercurrent ward infection; an epidemic of grippe precipitated a pseudo-epidemic of scurvy. It is important, especially for army surgeons, to bear in mind that where latent scurvy exists a bacterial invasion will lend the disorder a hemorrhagic character. This has been noted during the recent war in connection with typhus fever on the Eastern front, and was remarked upon during the Crimean War and our War of the Rebellion. Some years ago Wherry made a similar observation in the course of experiments with the plague bacillus—guinea-pigs fed on a cereal diet developed far more hemorrhages subsequent to infection than those which received cabbage in addition.

TABLE 5

Data of Epidemic of Scurvy
Case Age, Mos Weight Site of Hemorrhages Date Diet Remarks
lbs. oz.
1.J.H. 9 6 Humerus, tibia, face. Apr.19 Breast milk (1 week); pasteurized milk previously. Grippe since end of February; nephritis; v. Pirquet negative.
10 4 Upper eyelid May9
2.L.S. 5 12 14 External ear, parietal bones, vertebral column, abdominal wall May4 Pasteurized milk formula; orange juice 1 oz. daily since April 22. Twitchings and convulsions; signs of intoxication; red blood cells in urine fever to 101° F.; v. Pirquet negative.
3.A.R. 10 12 8 Femur Apr.19 Pasteurized milk formula; vegetables for a month; orange juice longer; getting orange juice and vegetables. Grippe end of January; again in April; fever until April 17; v. Pirquet positive; gums negative.
Femur again swollen and tender. June4
4.D.E. 5 7 Both ears; parietal bones. Apr.29 Breast milk since April 19; May 30, changed to pasteurized milk. Grippe throughout March; intoxication; nephritis; no relapse although no orange juice given.
5.T.K. 10 15 13 Ear and face Apr.27 Pasteurized milk, cereal, vegetable, soup; orange juice since April 15. Two teeth; gums negative; v. Pirquet negative.
6.P.G. 4 8 4 Tibia May8 Pasteurized milk formula. Grippe end of February and first half of March gained 20 oz. during last month; v. Pirquet negative.
7.I.P. 2 6 4 Abdomen Mar.7 Breast milk for past week; pasteurized milk previously. Grippe; probable source of epidemic.

DIAGNOSIS

A correct and early diagnosis of scurvy is the more important in view of the fact that we possess a specific remedy, and that the disorder is not self-limited. Recognition generally presents little difficulties for those who have seen cases, but is a stumbling block where the symptomatology has been gleaned merely from the textbooks. It has been our experience that medical students who were conversant with scurvy from a theoretical standpoint failed to diagnose a case presented to them in the clinic. Where diagnosis is uncertain, the most important aid is an exact knowledge of the previous diet, and observation of the reaction of the patient to antiscorbutic treatment. These diagnostic points should be constantly remembered in relation to the discussion which follows, and will not be reiterated in the differentiation of scurvy from the various other diseases.

The scurvy of adults and of infants are very similar. The main difference is the subjective symptoms in the adult—pains in various parts of the body—and the fact that the gums are frequently the site of infection and ulceration, as well as of hemorrhage. It might be thought that when scurvy occurs in epidemic form it would be readily recognized, but experience shows that for months it may permeate the ranks of troops or the inmates of almshouses, and pass as rheumatism. This is the cardinal diagnostic error in adult as well as in infantile scurvy—time and again, and in spite of urgent and repeated warnings, patients continue to be treated for rheumatism. Holt writes: “In fully four-fifths of the cases which have come to my own notice this (rheumatism) has been the previous diagnosis.” Such has been our experience. The diagnosis should not be difficult. In sporadic cases, the individual has limited his diet usually on account of indigestion, or diarrhoea, or following some dietetic whim or medical advice given months previously. Where scurvy occurs en masse it may follow an inability to obtain fresh food—as during war, on shipboard, in the Tropics or in the Arctic regions—or be the result of a misplaced reliance on some article of food—for example, dehydrated vegetables. More careful investigation will disclose that the “rheumatic” pains and tenderness are not in the joints but in the muscles and tendons. The calf muscles are frequently painful and excessively tender and somewhat swollen or infiltrated; the hamstring tendons or the tendo Achilles may be sore and slightly swollen and the site of slight subcutaneous hemorrhages. In some cases there is bone tenderness, pain on percussion of the shins or of the sternum. These signs may be accompanied by, or even precede, hemorrhages in the gums. The diagnosis of rheumatism in infants indicates a lack of knowledge of pediatrics, as this disease is hardly ever encountered in babies under a year and a half of age.

It is not always easy to differentiate scurvy from purpura. In either disease the gums may be hemorrhagic and swollen, there may be scattered subcutaneous hemorrhages and pains in the limbs. Occasionally, as in a case seen a few years ago, we may be forced to resort to the dietetic test. A close inquiry into the previous diet, however, a history of previous attacks of purpura, the number, and especially the large size, of the subcutaneous hemorrhages, and above all, the diminished number of blood-platelets, should make diagnosis possible. In scurvy the platelets are almost always over 300,000 per cubic millimetre, whereas in purpura they are reduced to less than 200,000.

In the army it may be difficult to distinguish scurvy from beriberi, especially if they occur side by side as in the recent English campaign in Mesopotamia. There may be a combination of the two diseases, a picture similar to ship-beriberi, regarded by Nocht as a hybrid of these diseases. The diagnosis is rendered more difficult, as at times scurvy is associated with signs of neuritis. We shall have to depend on the involvement of the gums and the hemorrhages in scurvy, and on the hyperÆsthesia, parÆsthesia, and anÆsthesia in beriberi; marked edema points to the latter disease.

We have thus far had in mind frank and outspoken cases of scurvy. When we come to consider latent or early cases, the diagnosis is more difficult and may have to be merely tentative. All that need be added, in view of the clinical picture sketched above, is that this condition should not be forgotten in treating adults who have malaise and indefinite “rheumatic” pains and, more particularly, in relation to infants who fail to gain, whose appetite is capricious, whose disposition has become fretful and who have developed the sallow scorbutic complexion. This warning is particularly opportune at present in the United States, where pasteurized milk is fed so extensively to infants, and an antiscorbutic food is not always given.

In addition to the symptoms just enumerated, tenderness of the bones, especially of the distal ends of the femora, should be sought for, the urine should be examined carefully for red blood-cells, and perhaps the ends of the long bones radiographed for “the white line” of Fraenkel.

The experience of Comby with infantile scurvy is illuminating. Among the fifty-five cases which he has seen, the diagnosis was erroneous in forty-five, and among thirteen cases recently met with, the physician failed to recognize the disorder in all but two. The infants had been given sodium salicylate, had been treated with electricity and massage for the supposed acute poliomyelitis, or given mercury for syphilis, or incisions or trephining had been carried out for acute osteomyelitis. Some had been put into plaster casts for coxalgia or for Potts’ disease. This experience requires little comment. It should be added, however, that in the course of an epidemic of poliomyelitis, such mistakes are apt to happen, and, to our knowledge, did occur in the recent epidemic.

In regard to “the pseudo-paralysis” of congenital syphilis diagnosed as scurvy, it should be remembered that this lesion occurs almost always before the fifth month of life. A history of previous papular eruption, the bilateral enlargement of the epitrochlear glands, and the Wassermann test should suffice to establish the correct diagnosis.

Besides the clinical conditions enumerated above, we may add the following, which have been confused with scurvy: Neuritis, hemorrhagic nephritis, calculus of the urinary tract, renal tumor, orbital tumor, appendicitis, peritonitis, pleurisy and pneumonia. Holt writes as follows: “I have known two cases to be operated upon by eminent surgeons, once with a diagnosis of sarcoma and once of ostitis of both tibiÆ. Not until the subperiosteal hemorrhages and epiphyseal separations were discovered was the nature of the trouble suspected.” Recently we saw a case of fracture of the distal end of the femur diagnosed as infantile scurvy; the baby had caught its thigh between the bars of the crib and snapped the bone in trying to extricate itself. Finkelstein adds acute endocarditis, hemorrhagic septicÆmia with multiple bone swellings, and leukÆmia as having been confused with scurvy.

In an interesting account of scurvy in the Russian army during the recent war, Hoerschelman states that tired soldiers at times feign scurvy. They produce a “pseudo-scurvy” by means of scratching the gums with their nails or rubbing them with tobacco, and at the same time bring about hemorrhages of the skin by means of trauma.

Scurvy in the breast-fed infant has been fully considered under etiology. We wish merely to state again that one cannot be too cautious in venturing this diagnosis in a nursing baby. The great majority of reported cases are not scurvy, but bacterial infections, syphilis, or various forms of intoxication. In establishing a diagnosis the same principles hold as in the case of bottle-fed infants.

The most important consideration in the diagnosis of scurvy is to keep in mind the heterogeneous character of its symptoms, and the manifold diseases with which it may be confused. Surgeons should be alert to this danger when about to perform operations for osteomyelitis or bone tumor. The mistakes occur because cases are infrequently seen and because the signs, being dependent largely upon hemorrhage, occur in such varied locations of the body. Where diagnosis cannot be made from the signs or symptoms, the most important aid is a thorough acquaintance with the previous diet of the individual and observation of his reaction to antiscorbutic treatment.


                                                                                                                                                                                                                                                                                                           

Clyx.com


Top of Page
Top of Page