Atherosclerotic diarrhea

Introduction Atherosclerosis is a clinical manifestation of childhood pancreatic insufficiency and neutropenia syndrome. Pancreatic insufficiency in children and neutropenic syndrome were reported by Shwachman in 1964, so it is also called Shwachman syndrome, Shwachman-Diamond syndrome. Intrinsic is a congenital pancreatic insufficiency accompanied by hypoplasia of the bone marrow hematopoietic system. More than 2 to 10 months of infants and young children, there is a familial incidence, may be autosomal recessive inheritance. Mainly due to insufficient exocrine function, such as amylase, protease, lipase reduction or lack, manifested as loss of appetite, nausea and vomiting, diarrhea, atherogenic diarrhea, steatorrhea or celiac disease. Intestinal manifestations may have physical developmental malnutrition, such as short stature deformity and development of long bone epiphysis. Due to the abnormal development of the bone marrow, the expression of neutrophils is reduced, the resistance is decreased, and the respiratory tract infection, otitis media, and sinusitis are easily combined, and the reduction of anemia and platelets can also be exhibited.

Cause

The pathological features of this disease are dysplasia of the pancreas, mainly the development of exocrine tissue, which shows that acinar cells are rare, while islet development is normal, total pancreatic secretion and HCO3- content are normal. At the same time, accompanied by dysplasia of the myeloid cell line, occasionally lack of megakaryocytes, may have dysplasia of the skeletal metaphysis, and focal calcification in the ankle.

an examination

Related inspection

Conventional white blood cell count (WBC) blood electrolyte examination

Blood routinely shows a decrease in neutrophils, a decrease in often hemoglobin and platelets. Bone marrow images show hypoplasia and stagnant blood cell maturation. The stool routine changes in a fat-like diarrhea. Biochemical tests can have a reduction in various pancreatic digestive enzymes. When the disease is complicated by pulmonary infection, it is easy to be confused with cystic fibrosis of the pancreas, but the electrolyte and chloride of the sweat gland are normal for identification.

Differential diagnosis

When the disease is complicated by pulmonary infection, it is easy to be confused with cystic fibrosis of the pancreas, but the electrolyte and chloride of the sweat gland are normal for identification.

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