MITOCHONDRIAL DISESES

 Mitochondrial encephalomyopathies constitute a broad group of disorders that were first described in the early 1960s, when systemic ultrastructural and histochemical studies revealed excessive proliferation of mitochondria of normal or ubnormal appearance in muscle from patients with weakness or exercise intolerance. knowledge of this group of disorder was gained following the introduction of the modified gomori trichrome stain, which revealed the presence of subsarcolemmal accumulation...

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HYPERTENSION

Hypertension is commonly associated with Wilms tumor, but hypertension secondary to renin screting Wilms tumor is uncommon. We present an infant with severe hypertension and renin hypertension, which we resistant to multiple antihypertensive agents. blood pressure was eventually controlled with a combination of captopril and losartan. Hypertension  in patients with Wilms tumor is relatively common. Sukarochana et al. reviewed 46 patients with Wilms tumor from the Children's hospital of pittsburgh...

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ESSENTIAL FATTY ACID DEFICIENCY PROFILE IN PATIENTS WITH NEPRHOTIC RANGE PROTEINURIA

The term neprhrotic syndrome defines a clinical diagnosis characterized by the precence of massive proteinuria (>40 mg/m2/h) and hypoalbuminemia (<2.5 mg/dl). Dydlipidemia is frequently associated feature and includes high levels of  cholestrol, triglycerides, and lipoproteins due to enhanced hepatic synthesis. Plasma concentrations of low molecular weight apoproteins A-I and A-II are low due to increased urinary loss. The high production of atherogenic lipoproteins carries an elevated...

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IDIOPATHIC MEMBRANOUS NEPHROPATHY IN CHILDREN

Membranous nephropathy ( MN ) is a renal disease with distinct pathologic features of normocellular glomerular wall thickening and subepitthelial electron dense deposits. MN is a common cause of nephrotic syndrome ( NS ) in adult, but this occurs infrequently in children. Idiopathic MN, a form of MN without any associated systemic diseases used to be uncommon in korean children in whom hepatitis B virus ( HBV ) infection was endemic and HBV-associated MN ( HBV-MN) was the major cause of childhood...

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