2015年1月31日星期六

Clinical Manifestations of Congenital Nephrotic Syndrome

Congenital nephrotic syndrome refers to children born within three months after the onset of nephrotic syndrome, children often appear at birth or within three months after the birth of massive proteinuria, high degree of edema, hyperlipidemia, and hypoalbuminemia and so on. But its etiology, pathology and prognosis in patients with older child or adult is different, its clinical manifestations are special appearance, proteinuria, growth retardation and so on.

1. Special appearance: Common special appearance after birth, such as low nose, the eye away from the wide, low ears, skull fracture width, large anterior fontanelle and posterior fontanelle, also common hip, knee, elbow flexion deformity. Subsequently common abdominal distension, ascites, umbilical hernia.

2. Edema: half to 1 week to 2 weeks after birth, that see edema, edema and a half months, but often parents before being discovered.

3. Proteinuria: Proteinuria in children with significant and persistent, initially highly selective proteinuria, late in the disease, the selectivity decreased significantly in children and hypoalbuminemia and hyperlipidemia.

4. Growth retardation: Because protein malnutrition in children with congenital nephrotic syndrome, so often occur growth retardation, as well as some children may be associated with gastroesophageal reflux and pyloric stenosis

5. Thrombosis, embolism: Because children often has a hypercoagulable state, it can be thrombotic, embolic complications, such as seen in multiple peripheral arterial vessels, the sagittal sinus, kidney, lung, and other veins.

6. Renal dysfunction: age, renal function gradually slow decline, and the corresponding changes in blood chemistry chronic renal dysfunction, most sick children 3 years of age have the need dialysis or transplantation. kidneyfight@hotmail.com

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