Chapter VII: Symptomatology and Diagnosis (2)

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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
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Name | Date | Platelets | Leuko- | Erythro- | Hmgl. % | Remarks
| | | cytes | cytes | (Sahli) |
-----+------+-----------+--------+-----------+---------+--------------
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 | 382,000 | ...... | ......... | .. |
| 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 |
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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.

[48] Senator regarded the marrow in scurvy as being aplastic. Nobécourt, Tixier and Maillet report postmortem examinations where the marrow showed an increased number of myelocytes and nucleated red cells of various kinds. They consider the typical blood change an intense myeloid reaction of the blood.

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.

[49] A blood-pressure band, or tourniquet, is placed about the arm, and the pressure increased until the forearm becomes cyanosed and the radial pulse is almost obliterated. The pressure is then maintained at this level for 3 minutes. The principle of this test consists in subjecting the capillaries and venules to increased intra-vascular pressure to observe whether this strain results in the escape of blood. In infants the pressure was usually raised to 90 mm.; in some cases it had to be raised higher in order to entirely obstruct the return flow of the blood.

The test is considered to be "positive" when the forearm shows many petechial spots. In normal infants petechiæ were almost always absent, or there were few to be seen. This is not a specific test for scurvy, but demonstrates a weakness of the vessel walls, whatsoever may be the cause. It is found to be positive in the majority of cases of scurvy.

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.

=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.

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, Hoerschelmann 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.

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Scurvy, Past and PresentChapter VII: Symptomatology and Diagnosis (2)

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