Pediatric non-lipoid reticuloendothelial proliferative syndrome
Introduction
Introduction to non-endocrine reticular endothelial proliferative syndrome in children Hand-Schüller-Christiansyndrome is a Han-Xue-Ke syndrome, also known as yellow lipoma, xanthomatic granuloma, reticuloendothelioma, lipid-like granuloma, Diabetes insipidus - exophthalmos - osteogenesis imperfecta syndrome, histiocytosis X, Schüller-Christian syndrome, Christian syndrome, etc. This disease is a clinical manifestation of skull defects, ocular protrusion and diabetes insipidus caused by tissue cell proliferation Performance and pathological changes. basic knowledge The proportion of illness: 0.03% (children with a history of genetic disease, the incidence rate is higher) Susceptible people: children Mode of infection: non-infectious Complications: otitis media pulmonary fibrosis
Cause
Pediatric non-endolipid reticuloendotheliosis syndrome etiology
(1) Causes of the disease
The cause of this disease is unknown.
(two) pathogenesis
This disease is a type of histiocytosis X (Histiocytosis X), formerly known as reticuloendothelial hyperplasia, is a group of diseases with unknown causes, pathologically differentiated tissue cells proliferating as a common point, possibly with fat And lipid-like metabolic disorders and unexplained infections, some people believe that this disease is a transitional disease between Le-Snow disease and bone eosinophilic granuloma, recently reported T lymphocyte histamine H2 in patients with this disease The lack of application of the calf thymus extract after the injection of the body successfully restored the child, thought that T may be caused by inhibition of lymphocyte defects.
Prevention
Prevention of non-endolipid reticuloendotheliosis syndrome in children
The cause is still unknown, and there is no systematic prevention method. It may have certain correlation with environmental factors, genetic factors, dietary factors, and mood and nutrition during pregnancy, so this disease cannot be directly prevented. Early detection, early diagnosis, and early treatment are important for preventing this disease. Regular examination should be done during pregnancy. If the child has a tendency to develop abnormalities, chromosome screening should be done in time, and abortion should be performed in time to avoid the birth of the diseased child.
Complication
Pediatric non-endolipid reticuloendotheliosis syndrome complications Complications otitis media pulmonary fibrosis
Otitis media, pulmonary fibrosis, secondary infection, liver and spleen lymphadenopathy.
Symptom
Pediatric non-endolipid reticuloendotheliosis syndrome symptoms common symptoms polydipsia polyuria lymphadenopathy eye swelling urine collapse eyeball abscess asthma pancreatic papular hemorrhagic papules
1. Skull defect: the earliest, the most common, can be single or multiple, skull lesions, the beginning of the scalp surface is a packet-like bulge, hard and light tenderness, when the lesion erodes through the skull outer plate, the mass changes Soft, touched with a sense of volatility, often touch the edge of the skull. At this time, the tenderness is not obvious. Occasionally, the ruptured person is easily misdiagnosed as an abscess. After that, the tumor is gradually absorbed. The large defect can touch the brain and beat with the pulse. X-ray Examination showed that the skull was a map-like defect, and the mandibular bone was more common in the facial bone. Other bones such as the pelvis, sacrum, scapula, scapula, spine and other bones also suffered defects.
2. Eyeball protrusion: About 1/3 of the patients have eyeball protrusion, which often occurs on both sides, but the side is more obvious. Because of the involvement of the tibia, it is caused by the adipose tissue after the sputum.
3. Drink more, more urine: half of the patients showed polydipsia, polydipsia, polyuria, growth and development disorders, mostly due to neurohypophyseal, invasive lesions in the lower part of the hypothalamus, or due to saddle destruction caused by compression of the pituitary.
The above triads do not necessarily appear at the same time, or only one or two of them are seen during the course of the disease, especially in early cases.
4. Hepatosplenomegaly: The child has fever, the liver and spleen lymph nodes are enlarged, and the degree of anemia is generally mild.
5. Rash: There are scattered yellow macules or hemorrhagic papules on the skin, and yellow suede is sometimes seen on the scalp.
6. Respiratory system: When the lungs are involved, there may be cough, asthma and other respiratory symptoms, and fibrotic mesh shadows may be seen in the lungs.
Examine
Examination of non-endolipid reticuloendotheliosis syndrome in children
1. Blood test: Peripheral blood hemoglobin and red blood cell counts are reduced, and white blood cell neutrophils are increased at the time of infection.
2. Bone marrow examination: visible foam cells.
3. Pathological examination: rash and lymph node biopsy, such as tissue endothelial cell hyperplasia and visible foam cells can be diagnosed.
4. X-ray examination: X-ray examination shows that the skull has a map-like defect, pelvis, sacral, femoral, scapular, rib, spine, etc., the bone also has defects, the saddle destroys the pituitary, and the fibrotic mesh shadow is visible in the lung field.
5. B-ultrasound: hepatosplenomegaly can be found
Diagnosis
Diagnosis and identification of non-endolipid reticuloendotheliosis syndrome in children
diagnosis
If there is bone defect, especially skull defect with exophthalmia, diabetes insipidus, combined with X-ray examination is not difficult to diagnose, but most cases, especially in the early stage, do not have typical symptoms, such as otitis media with prolonged unhealed, When the head mass, rash and other symptoms, should consider the possibility of the disease, early bone marrow examination and pathological examination.
Differential diagnosis
Identification with diabetes insipidus and blood diseases, laboratory tests can help diagnose.
