Infantile Atrophy

INFANTILE ATROPHY, a term that first appeared in pediatrics in the 16th century, is used to denote a severe, often fatal, chronic nutritional disorder in children, accompanied by phenomena of sharp emaciation and exhaustion of the organism. In 1874, the French clinician Parrot described a special form of severe atrophy under the name athrepsia; the French pediatric school adopted this new term, however, expanding its meaning compared to Parrot's description, and applies it in those cases of chronic nutritional disorder for which German authors have retained the older name of atrophy. In our country, both terms were used in parallel until recently, denoting the same clinical picture. At the beginning of the current century, the Berlin pediatrician Finkelstein introduced a new term for the concept of infantile atrophy—decomposition (see)—emphasizing with this name the presence of a gradual change in cells and tissues toward their loss of functional capacity and normal resistance. This term gained great popularity, but very recently (June 1927), the IV All-Union Pediatric Congress, in its (temporary) classification of nutritional disorders, again approved the term infantile atrophy to denote a severe form of chronic nutritional disorder in children. Etiology and pathogenesis. The immediate causes of infantile atrophy are: 1) acute and chronic nutritional disorders, especially those accompanied by diarrhea; 2) prolonged starvation and malnutrition; 3) infections, mainly chronic; and 4) unhygienic living conditions for the child—lack of light and fresh air, overheating of the organism, all kinds of care defects, i.e., the entire complex of conditions constituting the essence of so-called hospitalism (see). However, in these cases, true severe infantile atrophy affects almost exclusively artificially fed children, in whom, especially in the first three months, infantile atrophy develops rapidly even as a result of disorders that do not seem particularly severe at first glance. Endogenous factors—congenital weakness due to prematurity or pathological constitution—also have a certain significance in the etiology of infantile atrophy, but they recede into the background compared to the indicated external factors. Regardless of the causes from which atrophy develops, it essentially represents starvation of the organism, depending either on insufficient delivery of nutritional material from the outside or on the organism's inability to digest and assimilate the food provided to it. As studies of metabolism in atrophic patients show, parallel to the weakening of assimilation processes, there is an intensification of dissimilation processes. The organism of atrophic patients lives at the expense of its own reserves, first of all fats, and only then proteins and salts. In severe cases of infantile atrophy, according to studies by Ohlmüller, up to 90.9% of all body fat and 30.7% of proteins disappear. Besides adipose tissue, the thymus gland, muscles, and skin atrophy most strongly; to a lesser extent, in descending order, the intestines, heart, brain, liver, and bones. Regarding the essence of the pathological processes underlying atrophy, almost nothing is known yet. Previously, the cause of infantile atrophy was considered to be atrophy of the intestinal mucosa, but later authors (Heubner, Gerlach, Faber, Kuskov) proved the incorrectness of this view. Pfaundler sees the center of gravity in the disturbance of intermediate metabolism; Maslov and Rudnev, pointing to a significant weakening of the power of gastric and pancreatic juice enzymes, assume a deep lesion of the enzymatic apparatus both in the intestine and outside it. Symptoms and course. The cardinal, most striking symptom of infantile atrophy is the disappearance of the subcutaneous fat layer and, accordingly, the emaciation of the child. Since subcutaneous fat disappears in a certain order—first on the abdomen, then in the chest and back area, limbs, buttocks, and lastly on the face—in mild and moderate degrees of atrophy, emaciation is clearly visible only on a completely undressed child. The ribs, shoulder blades, and iliac bones protrude sharply, clearly outlining the skeleton; the limbs are thin and slender; the roundness of the buttocks disappears, and the ischial tuberosities clearly show through the skin. In more severe cases, the torso and limbs give the impression of a skeleton covered with thin skin, and the face has a characteristic senile appearance. The head is disproportionately large in comparison with the emaciated and pointed face; the skin of the forehead is gathered into wrinkles and folds, the cheeks are deeply sunken, and the mouth therefore seems especially large; the nasolabial folds (plicae naso-labiales) are sharply defined. Through the thin, pale, and graying skin, the cheekbones and jawbones clearly protrude. The oral mucosa is bright or brick-red. Against the background of the emaciated face, large eyes stand out with a completely conscious, often even lively gaze; sometimes, mostly in severe cases, the gaze is lethargic and indifferent. The skin, with clearly visible veins, is thinned everywhere and has lost its elasticity. Through it, slightly enlarged lymph nodes, especially in the groin, are easily palpable. The atrophied muscles of the limbs, clearly visible to the eye due to the absence of fat, are easily palpable as thin, flabby bundles. Sometimes, in severe atrophic patients, muscle hypertonia is observed. The abdomen is distended; often, shortly before death, it rapidly subsides. The meteorism common in atrophic patients depends partly on the abundant development of gases in the intestine and partly on the weakness of the atrophied intestinal musculature; at times, their peristaltic movements are clearly visible. Body weight drops sharply. The more severe the degree of atrophy, the more the child's weight lags behind the weight of a normal peer, not reaching even half of the latter in severe atrophic patients. This weight loss occurs either more or less gradually or in irregular jumps. Weight gain can sometimes depend on the appearance of subcutaneous edema. Growth retardation is less noticeable, but it too can reach several centimeters during a prolonged course of the disease. Cardiac activity is weakened; heart sounds are muffled, sometimes barely audible. The pulse is slowed (60–70 or fewer beats per minute), soft, and weak. Blood pressure is lowered. The blood composition in cases of moderate severity presents phenomena of anemia: the number of erythrocytes is reduced to 3–4 million, hemoglobin to 60–80%. The leukocyte formula is unchanged. According to Marfan, in the most severe cases, depending on the loss of water and the corresponding thickening of the blood, the number of formed elements and hemoglobin is increased. Breathing is superficial and not entirely rhythmic; in the further course, the respiratory rate slows down, reaching 40–30 and even 20 respiratory movements per minute. Very characteristic for atrophic patients is a low and very unstable body temperature with a strong tendency toward rapid cooling. The hands and feet, as well as the nose, are mostly cold to the touch and often cyanotic. Urine is normal. In the final, pre-death stage of the disease, signs of dehydration of the organism are added. The eyes sink in, are half-closed by the eyelids, and the pupil narrows. The fontanelle sinks, and the skull bones sometimes overlap one another. In this stage, the development of sclerema is often observed. Death can occur completely unnoticed, sometimes after a prolonged agony. With a favorable turn in the course of the disease, the weight stops falling, remains stationary for some time (2–3 weeks and longer), and then slowly increases. In its course, infantile atrophy, in addition to the indicated symptoms, is in most cases accompanied by a whole series of painful phenomena, mainly from the digestive apparatus, skin, and respiratory organs. Dyspeptic disorders—lack of appetite, vomiting, diarrhea, increased weight loss, and in severe cases, intoxication phenomena—in many atrophic patients invariably follow any change in food toward an increase in its quantity or higher caloric content. Along with these cases, there are also atrophic patients with a normally functioning digestive tract. Their appetite is not only preserved but, apparently, even pathologically increased. The child has normal stools, sometimes even constipation is observed; there is neither vomiting nor regurgitation, and yet their weight falls uncontrollably, and the atrophy progresses rapidly. A severe complication for an atrophic patient is often the appearance of thrush on the oral mucosa, usually accompanied by a worsening of appetite, as well as ulcers on the palate. In second place in frequency are complications of the skin. The mildest irritations—wet diapers, excrement, insect bites, pressure from an uncomfortable bedding—cause severe, stubbornly non-healing diaper rash, erythema, and bedsores on the skin, which is weakened in its nutrition. Pustules, boils, and abscesses easily develop on the skin, spreading rapidly across the surface and into the depths, and easily leading to septic infection. The primary localization of these pyodermas is most often the back of the head. A very frequent and fatal complication of infantile atrophy, which turns out to be the immediate cause of death, is the development of hypostatic pneumonia in the posterior parts of the lower lobes. These bronchopneumonias in atrophic patients often proceed without an increase in temperature, almost without cough and shortness of breath, and therefore can easily remain unnoticed.

Accidental infections, to which atrophic infants are highly prone due to weakened immunity, are also a serious complication. These infections can also proceed without elevated temperature, causing almost no reaction specific to the given infection, but they always strongly reflect on the deterioration of the general condition; The diagnosis of infantile atrophy in clearly expressed cases does not present difficulties and can often be made at first glance. It is more difficult to notice the initial stages of the disease. Here, one must take into account the degree of emaciation, the activity of the heart and lungs, and, most importantly, the decrease in the organism's endurance in relation to food. Diagnostics should not be limited to merely establishing the presence of atrophy. For prognosis and treatment, it is very important to clarify the causes of infantile atrophy and its individual characteristics. In very many cases, a causal diagnosis is possible only with the help of accurate anamnestic data. Prognosis. Atrophy is always a very serious, often fatal disease. According to Quest, the prognosis becomes hopeless if weight loss has reached one-third of the initial body weight, but weight loss in itself does not always have decisive significance. The younger the child, the more dangerous atrophy is for him, especially for children in the first 3-4 months. With the beginning of the second half-year, the chances for recovery are much more favorable. A poor prognostic sign is the appearance of dark brown (due to an admixture of blood) stools, indicating the presence of ulcers in the intestine (according to Helmholtz, mostly in the duodenum). The presence of signs of constitutional anomalies (exudative diathesis, neuropathies) worsens the prognosis. The possibility of providing the atrophic infant with breast milk and rational care is of great importance for the prognosis. Prevention of infantile atrophy comes down to the correct implementation of breastfeeding and supplementary feeding and to the strict observance of all rules of hygiene for the infant during care. Both of these in a family setting often depend entirely on the culture and conscientiousness of the parents, and therefore sanitary education of the broad masses of parents, the involvement of all infants in the sphere of child welfare clinics, and supplying them with high-quality milk are the most effective preventive measures. In relation to children living in closed institutions, the matter usually comes down to the proper organization of the latter, which eliminates the disastrous influence of hospitalism. Treatment. The only more or less reliable means in the treatment of infantile atrophy is breast milk. Only when it is completely impossible to provide it to the atrophic infant, one has to, with incomparably lower chances of success, use various artificial nutritional mixtures, predominantly protein milk, buttermilk, and, in extreme cases, skimmed, diluted cow's milk. Ordinary milk dilutions are completely intolerable to severely atrophic infants. Very small and weak children should not be put to the breast; expressed breast milk is given to them from a bottle or with a spoon; this makes it possible to accurately dose the quantity and is less tiring for the child. In the case of severe dyspeptic phenomena, a question may arise about the necessity of prescribing therapeutic fasting. But with the latter, extreme caution must be observed in atrophic infants, and in particular, it should not be prescribed repeatedly during new exacerbations, as it easily causes them a severe decline in strength, up to collapse. When prescribing fasting, the interval between feedings should in any case not exceed 8-12 hours, while it is necessary to introduce a sufficient amount of liquid (water or tea with saccharin) all the time and carefully monitor the heart and general condition. In very small or weak children, it is better to limit oneself, instead of fasting, to some reduction in the quantity of food and the prescription of skimmed breast milk. In all cases of infantile atrophy, treatment begins with small but frequent intakes of expressed breast milk (10 times a day, 20-30 g each). If daily weight losses are very large, and the general condition and appearance of the child deteriorate, it is useful to replace 1-2 intakes of breast milk with buttermilk with 2-3% flour, but without sugar. The amount of food is increased only after a few days, when vomiting and diarrhea have ceased and the weight has stopped falling, while every 2-3 days the food is increased by 50-100 g per day to the norm necessary for the given child. The daily food requirement for atrophic infants in relation to body weight is relatively higher than that of a normal child and is approximately 2/3 - 3/4 of the amount of food required by his normal peer. With the onset of improvement, the child can be put to the breast and the number of feedings reduced to 7-8. Feeding with breast milk continues, if possible, longer even after improvement has occurred, and the transition to artificial mixtures must be carried out with great gradualness and caution. It is safer in this case to first replace breast milk with protein milk with 5% sugar or diluted skimmed (separated) milk and only then transfer the child to ordinary milk mixtures or, better, mixtures acidified with lactic acid. Protein milk and buttermilk are prescribed in the same quantities as breast milk, increasing the intakes with the same gradualness and guided by the same rules. To protein milk in the first days, in the presence of vomiting or diarrhea, 3% sugar is added, increasing the content of the latter to 4 and 5% as soon as dyspeptic phenomena subside. Instead of beet sugar, in this period it is better to use dextrin-maltose preparations (Mellin's food, Soxhlet sugar), which are less susceptible to fermentation processes. In the recovery period in children older than 3 months, in addition to sugar, 1-2% flour is added if weight gain is insufficient. Buttermilk in the same quantities is initially prescribed without carbohydrates; after 2-3 days, 1-3% flour is added, and only in the recovery period or in the absence of diarrhea is another 1-2% sugar added. In a similar way, skimmed milk is given, diluted by half with rice decoction and in a weaker dilution for older children, initially without the addition of sugar, then—in the recovery period—with 2-5% sugar. American authors (Marriott et al.) have seen good results in atrophic infants from the use of acidified milk mixtures. Besides food, the atrophic infant also needs the abundant introduction of liquid in the form of drink (tea, water, saline solution) or, in case of refusal to drink, in the form of saline enemas. It is necessary to carefully monitor the temperature of the atrophic infant, protecting him from cooling (heating pads), and to monitor cardiac activity; in case of heart weakness - caffeine internally (2-4 times a day, a teaspoon of 1/8-1% solution) and subcutaneously (1/2 syringe of 10% solution 1-2 times a day). Attentive care, which protects the atrophic infant from accompanying complications from the skin and lungs, which are extremely dangerous for him, is of great importance.