Severely dysplastic abdominal muscles

Introduction Orbinsky Syndrome is a dysplasia of the abdominal muscles, often associated with limbs, urinary tract, gastrointestinal tract and heart deformities.

Cause

Pathogenesis:

The pathogenesis of this disease has not been fully elucidated. That is to say, the incidence of FHF is mainly primary immune injury, and secondary hepatic microcirculation disorders, with the study of the role of cytokines (Cytokine) on vascular endothelial cells and the role of liver microcirculation disorders in the pathogenesis, The Schwartz response is thought to be involved in the pathogenesis of FHF. Cytokines are a group of biologically active protein mediators that have been secreted by lymphokine research, such as tumor necrosis factor (TNF), interleukin-1 (IL-1), and lymphotoxin (LT). Among them, TNF is a product of endotoxin-stimulated mononuclear macrophages, and can act on vascular endothelial cells and hepatocytes, which can lead to Schwartz reaction, and thus TNF is considered to be one of the main pathogenesis of FHF. In addition, endotoxemia can increase hepatocyte necrosis and cause visceral damage (such as renal failure) is also an important cause of disease.

an examination

Related inspection

Abdominal shape examination abdominal skin examination abdominal plain film abdominal perspective abdominal wall tension

symptom:

The main clinical manifestations are that the abdominal wall is as thin as paper, the organs inside the abdomen are faintly visible, and the touch by hand is clearer. If the patient does not use the arm force, the patient cannot be transferred from the lying position to the sitting position. Abdominal pressure is insufficient when coughing, and there are often respiratory infections. The skin at the lower part of the rib is red. The incomplete type of abdominal wall is normal, showing only bilateral cryptorchidism and urinary malformations.

Urinary malformations can be expressed as:

1 complete urethral obstruction, renal failure after birth, urinary surgery drainage still oliguria, short-term death from uremia.

2 severe upper urinary tract structure and functional damage, uremia progresses rapidly, for acute kidney or ureteral stoma drainage, the ureter is obviously dilated and lacks peristalsis.

3 urinary tract obstruction is mild, and the symptoms in childhood are not serious.

Differential diagnosis

Identification:

For children and young people should be identified with chronic hepatitis, fulminant hepatitis or cirrhosis.

diagnosis:

The main clinical manifestations are that the abdominal wall is as thin as paper, the organs inside the abdomen are faintly visible, and the touch by hand is clearer. If the patient does not use the arm force, the patient cannot be transferred from the lying position to the sitting position. Abdominal pressure is insufficient when coughing, and there are often respiratory infections. The skin at the lower part of the rib is red. The incomplete type of abdominal wall is normal, showing only bilateral cryptorchidism and urinary malformations.

Urinary malformations can be expressed as:

1 complete urethral obstruction, renal failure after birth, urinary surgery drainage still oliguria, short-term death from uremia.

2 severe upper urinary tract structure and functional damage, uremia progresses rapidly, for acute kidney or ureteral stoma drainage, the ureter is obviously dilated and lacks peristalsis.

3 urinary tract obstruction is mild, and the symptoms in childhood are not serious.

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