Pancreatic insufficiency and neutropenic syndrome in children
Introduction
Introduction to pancreatic insufficiency and neutropenia syndrome in children 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. basic knowledge The proportion of children: the incidence rate of children is about 0.006%-0.008% Susceptible people: more than 2 to 10 months of infants Mode of infection: non-infectious Complications: pulmonary edema
Cause
Pancreatic insufficiency and neutropenic syndrome in children
The pathological features of the disease are dysplasia of the pancreas, mainly the development of exocrine tissue, showing a lack of acinar cells, normal islet development, total pancreatic secretion, normal HCO3- content, accompanied by dysplasia of the myeloid cell line. Occasionally, megakaryocytes are deficient, and there may be dysplasia of the skeletal metaphysis and focal calcification in the ankle.
Prevention
Pancreatic insufficiency in children and prevention of neutropenia syndrome
Intrinsic is congenital pancreatic insufficiency, accompanied by hypoplasia of the bone marrow hematopoietic system, and has a familial morbidity tendency, which may be autosomal recessive inheritance, so there is no special precautionary measure for this disease.
Complication
Pancreatic insufficiency and neutropenic syndrome complications in children Complications pulmonary edema
The disease is easy to be complicated by pulmonary infections, such as various inflammations of the lungs, pulmonary edema, and the like.
Symptom
Children with pancreatic insufficiency and symptoms of neutropenia syndrome Common symptoms Diarrhea granulocytes reduce appetite, bone marrow dysplasia, nausea and diarrhea
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 performance can have physical Developmental malnutrition, such as short stature deformity, long bone epiphysis developmental disorder, due to abnormal development of bone marrow, showing a decrease in neutrophils, decreased resistance, and easy to concurrent respiratory infection, otitis media, sinusitis, can also show anemia and platelets Reduction.
Examine
Examination of pancreatic insufficiency and neutropenia syndrome in children
Blood routine can be seen to reduce neutrophils, often <1.5 × 109 / L, decreased hemoglobin and platelets, bone marrow shows hyperplasia and blood cell maturation stagnation, stool routine fat-lipepymic changes, biochemical examination can have a variety of pancreas digestion Reduction of enzymes.
Diagnosis
Diagnosis and differentiation of pancreatic insufficiency and neutropenia syndrome in children
When the disease is complicated by pulmonary infection, it is easy to be confused with cystic fibrosis of the pancreas. It should also be distinguished from acute pancreatitis and chronic pancreatitis.
