Page 44 of 73 WHIPPLE'S ILLNESS
Only one case of the child of this rare illness is described. Assume that the activator is the rhabdoid bacterium. At this disease many bodies and systems of an organism are surprised, but anyway the small bowel is involved in process owing to what there is malabsorption. The arthralgia, fever and a polyserositis are often observed. The biopsy of a duodenum reveals accumulations of PAS positive macrophages, in own plate it is possible to see bacilli. Antibiotics give very good effect, but it is necessary to enter them a long time. INTESTINES LYMPHANGIECTASIA
This inborn defect of lymphatic system can strike considerable part of intestines, causing a steatorrhea, a proteinaceous and scarce enteropathy, hypostases and a lymphopenia. Vsasyvatelny function (except for absorption of fats with a long chain) usually is not broken. Meal with the low content of such fats can reduce losses through intestines; the specified fats can be replaced with triglycerides with an average chain as they are transported by portal system. UOLMAN'S ILLNESS
This lipidosis which is seldom found and coming to an end with a lethal outcome is shown by accumulation of lipids in many IDIOPATHIC DIFFUSION DAMAGE OF THE MUCOUS MEMBRANE OF THE SMALL BOWEL
bodies, including a small bowel. Except vomitings and a gepatosplenomegaliya, the steatorrhea caused by obstruction of absorbent vessels is observed.
At some children suffering from long heavy malabsorption, the clinical picture does not keep within a framework of the known diseases. In such cases it is necessary to think of two syndromes which cornerstone damage of a mucous membrane of a small bowel is.
The "total" malabsorption arising right after the birth and causing heavy diarrhea and insufficiency of food is characteristic of a family enteropathy. Inability to support normal balance of feedstuffs does such patient completely dependent on parenteral food. In bioptata of a duodenum the sharp flattening of fibers (fig. 12-19,B) comes to light. Unlike a gluten illness of a crypt are not extended, and mitotic activity, apparently, is reduced. Family character of a disease at the heart of which as believe, defect of regeneration of an intestinal epithelium lies is in most cases traced. Despite an intensive maintenance therapy and use of corticosteroids, most of patients perishes.
Other rare idiopathic syndrome — permanent defeat of fibers — causes the expressed chronic malabsorption at the earliest age. Heavy generalized disturbance of absorption arises in several months after the birth against a good shape of the child. In the beginning usually suspect an acute infectious disease, but process lasts many months, even years. Dietary measures do not influence disease. A morphological feature of a disease is shortening of fibers; as well as at a gluten illness, crypts are extended, numerous mitoses are noted. Corticosteroids can give a positive effect.