Atheroma

Atherosclerosis is a clinical manifestation of pancreatic insufficiency and neutropenia syndrome in children. Children with pancreatic insufficiency and neutropenia syndrome were reported by Shwachman in 1964, so they are also called Shwachman syndrome, Shwachman-Diamond syndrome. Essence is congenital pancreatic insufficiency, accompanied by hypoplasia of the bone marrow hematopoietic system. Most infants and young children develop disease in 2 to 10 months, have a familial tendency, may belong to autosomal recessive inheritance. Mainly caused by insufficient exocrine function, such as reduction or lack of amylase, protease, lipase, manifested as loss of appetite, nausea and vomiting, diarrhea, atherosclerosis, steatosis or celiac disease. Extra-intestinal manifestations may be physical developmental malnutrition, such as short stature, long bone epiphyseal developmental disorders. Due to the abnormal development of the bone marrow, neutrophils are reduced and resistance is reduced, and respiratory infections, otitis media, and sinusitis are likely to be complicated by anemia and thrombocytopenia.