Cyclosporin A therapy for Henoch-Schönlein nephritis with nephrotic-range proteinuria.

To evaluate the therapeutic role of cyclosporin A (CyA) for the treatment of Henoch-Schönlein nephritis (HSN), 29 patients (18 boys, 11 girls) with nephrotic-range proteinuria were analyzed retrospectively. Mean age was 8.6 years (range 2.

False positive immunoglobulin m antibody to cytomegalovirus in child with infectious mononucleosis caused by epstein-barr virus infection.

A 16-month-old boy was admitted because of cough that had lasted for 10 days. The patient showed severe hepatomegaly incidentally, and dual positivity of Immunoglobulin (Ig) M to Epstein-Barr virus (EBV) viral capsid antigen (VCA) and cytomegalovirus (CMV). On the basis of seroconversion to Epstein-Barr nuclear antigen (EBNA) Ig G positivity and reduced CMV Ig M titer with persistently negative CMV Ig G, a definite diagnosis of EBV-induced infectious mononucleosis was established 1 year 2 month later.

Doppler ultrasonographic indices in diagnosing nutcracker syndrome in children.

To elucidate the Doppler ultrasonographic cut-off value of nutcracker syndrome causing hematuria in children, we analyzed Doppler spectral findings between 15 children with nutcracker syndrome and 15 age- and sex-matched normal control subjects. A follow-up Doppler ultrasound (US) was also performed in children with nutcracker syndrome when hematuria subsided completely after a median period of 1.7 years (range: 1.

Effect of renal Doppler ultrasound on the detection of nutcracker syndrome in children with hematuria.

To assess the detection rate of nutcracker syndrome in children with isolated hematuria, renal Doppler ultrasound examinations were routinely performed on 216 consecutive children (176 microscopic hematuria and 40 gross hematuria). Renal Doppler ultrasound was also performed on 32 healthy normal children. The peak velocity (PV) was measured at the hilar portion of the left renal vein (LRV) and at the LRV between the aorta and the superior mesenteric artery.

Superimposition of nutcracker syndrome in a hematuric child with idiopathic hypercalciuria and urolithiasis.

We report a 5-year-old girl with idiopathic hypercalciuria who developed gross hematuria and left flank pain despite normalization of calciuria, a renal stone, and microscopic hematuria. She was found to have nutcracker syndrome by renal Doppler ultrasound, which revealed the significant differences of the peak blood flow velocities in the two portions of the left renal vein.

Successful use of cyclosporin A in severe Schönlein-Henoch nephritis resistant to both methylprednisolone pulse and azathioprine.

Schönlein-Henoch nephritis (SHN) usually presents with micro-haematuria or mild proteinuria but can be associated with heavy proteinuria and nephrotic or acute nephritic syndrome. Although the treatment of SHN is still under debate, it has been suggested that cyclosporin A (CsA) may be beneficial in children with severe SHN, as reported by Ronkainen et al. (Pediatr Nephrol 18:1138-1142, 2003) and Someya et al.

Catastrophic antiphospholipid syndrome in a 7-year-old girl.

Antiphospholipid syndrome (APS) has been recognized as the leading cause of vascular thrombosis in children. The syndrome may occur in isolation or in association with an underlying systemic disease, particularly systemic lupus erythematosus. Less than 1% of patients with APS present with a life-threatening condition resulting from thrombosis in multiple organs and subsequent multiorgan failure, which is defined as catastrophic APS.

Caroli's syndrome with autosomal recessive polycystic kidney disease in a two month old infant.

Caroli's syndrome is a rare congenital disorder that involves intrahepatic bile duct ectasia and congenital hepatic fibrosis, frequently seen with concomitant autosomal recessive polycystic kidney disease (ARPKD). Literature on infants with ARPKD is rare. Here, we present a case of a two month old boy who was diagnosed with Caroli's syndrome and ARPKD.

Factors affecting histological regression of crescentic Henoch-Schönlein nephritis in children.

To identify the factors affecting histological regression of crescentic Henoch-Schönlein nephritis (HSN), we retrospectively analyzed serially biopsied 20 children with crescentic HSN treated with immunosuppressants. They were classified into two groups according to the histological changes between the first and second biopsy: group I (n=10) with histological regression and group II (n=10) with no change or histological progression. Of the 20 patients, 19 showed a favorable outcome at the end of follow-up.

Doppler ultrasonographic detection of nutcracker syndrome in a young child with intussusception: a case report.

Unlabelled: We report on a 2-y-old male with intussusception accompanying nutcracker syndrome detected by renal Doppler ultrasound. Renal Doppler ultrasound revealed a significant difference in the peak velocity between the hilar and aortomesenteric portions of the left renal vein.

Conclusion: Renal Doppler ultrasound has been very useful and effective in detecting nutcracker syndrome in a young child.

Cyclosporin A therapy for severe Henoch-Schönlein nephritis with nephrotic syndrome.

To evaluate the efficacy of cyclosporin A (CyA) for treating severe Henoch-Schönlein nephritis (HSN), seven patients with nephrotic syndrome, aged 3.9-13.8 years (mean 6.

Serum IgA/C3 ratio may be a useful marker of disease activity in severe Henoch-Schönlein nephritis.

Background/aims: This study was designed to investigate whether the serum IgA/C3 ratio can be a serologic marker of disease activity in children with severe Henoch-Schönlein nephritis (HSN).

Methods: Twelve HSN patients who were treated with steroids and cyclosporine were examined. The levels of serum IgA and C3 were measured using an international reference preparation (IFCC/CRM470) and a renal biopsy was performed in all patients before and after therapy.

Can azathioprine and steroids alter the progression of severe Henoch-Schönlein nephritis in children?

To evaluate the effect of azathioprine with steroids on the clinical course and histologic parameters of severe Henoch-Schönlein nephritis (HSN), 20 patients with a median age of 9.3 years (range 4.4-17 years) and a follow-up period of 4.

Factors affecting spontaneous resolution of hematuria in childhood nutcracker syndrome.

To identify factors affecting spontaneous resolution of hematuria in children with nutcracker syndrome, 20 patients diagnosed as having nutcracker syndrome using renal Doppler ultrasound (US) were analyzed retrospectively. Sixteen patients had microscopic hematuria, and four had gross hematuria at presentation. The mean age was 10.

A systemic lupus erythematosus patient with protein losing enteropathy.

Protein-losing enteropathy (PLE) is a rare manifestation of systemic lupus erythematosus (SLE), and it is an uncommon cause of hypoalbuminemia without proteinuria. We describe a case of an 11 year-old girl who had diarrhea and periorbital edema as the initial symptoms of SLE. PLE was diagnosed by 24-hour stool alpha-1 antitrypsin clearance and (99m)Tc-human serum albumin scintigraphy.