Yoshiki Kakizaki

Interstitial cystitis and ileus in pediatric-onset systemic lupus erythematosus.

Abstract A girl aged 11 years presented with autoimmune hemolytic anemia with thrombocytopenia, and subsequently developed severe abdominal pain, vomiting, and pollakiuria. X-ray findings of her abdomen demonstrated paralytic ileus with intestinal wall thickening. Intravenous pyelography revealed bilateral hydroureter with mild hydronephrosis and contracted bladder. Pathological examination of her bladder revealed interstitial cystitis, with evidence of focal deposition of IgG and C3 in a granular pattern on small blood vessel walls. She was diagnosed as having systemic lupus erythematosus (SLE) associated with paralytic ileus and chronic interstitial cystitis. Although initiation of high-dose prednisolone therapy resulted in a gradual improvement in clinical symptoms, reducing the dosage of prednisolone caused a relapse. To our knowledge, the combination of paralytic ileus and chronic interstitial cystitis is quite uncommon in pediatric-onset SLE.

Acute tubulointerstitial nephritis associated with piperacillin therapy in a boy with glomerulonephritis

An 11‐year‐old boy with glomerulonephritis developed acute renal failure 4 days after beginning piperacillin (PIPC) treatment. Renal biopsy revealed acute tubulointerstitial nephritis (ATIN) with marked eosinophils. A lymphocyte stimulation test (LST) for PIPC demonstrated an extremely high LST index of 626%. The serum levels of immunoglobulin E and eosinophil cationic protein also showed a significant increase at 9021 IU/mL and greater than 150 μg/L, respectively. These observations suggest that a hypersensitivity reaction might play a role in the pathogenesis of ATIN. This is the first report to describe PIPC‐induced ATIN in a child.

Efficacy of long-term alternate day prednisolone therapy in childhood IgA nephropathy

BackgroundRecent reports suggest that long-term oral prednisolone with or without cytotoxic agents may decrease proteinuria and histologic alterations, and preserve renal function in patients with proteinuric IgA nephropathy.MethodsTo evaluate the efficacy of corticosteroid therapy in children with IgA nephropathy, we performed a retrospective study of patients seen during a 10-year period. Twenty-three of 64 children with IgA nephropathy met study criteria. They received prednisolone without cytotoxic agents for 2 years at an initial dosage of 1 mg/kg (maximum 60 mg) every other day for 3 to 12 months, and a tapered dose thereafter. Renal biopsies were done at presentation and repeated at a mean interval of 22 months.ResultsAt the initial presentation, urine protein excretion and histologic indices, such as the mean activity score and the mean chronicity score, in the patients were 0.9±0.7 g/day, 4.2±1.1, and 4.2±1.6, respectively. Urine protein excretion and the activity score decreased significantly at the second biopsies (0.3±0.3 g/day, 2.2±1.0, (P<0.01), respectively), while the chronicity score did not change. A percentage of mesangial area occupying glomeruli did not increase between the biopsies (23.5±4.6% vs. 23.4±4.1%). At the latest observation at mean interval of 50 months, no patient showed renal impairment. No serious clinical toxicity was seen.ConclusionIt is suggested that alternate-day prednisolone therapy without cytotoxic agents may be of benefit and cause less clinical toxicity in children with moderately proteinuric IgA nephropathy. A prospective controlled trial is needed to confirm these preliminary findings.

Chronic urticaria associated with aseptic meningitis: an atypical urticarial vasculitis?

The patient was a 7‐year‐old girl with early onset urticarial cutaneous lesions and was later complicated with aseptic meningitis. Her skin lesions occurred in the infantile period and were diagnosed as urticaria, but did not disappear with antihistamines and were recurrent and persistent. In addition, she had experienced an episode of headache about once a month since 1991, when she was 4 years old, and was diagnosed as aseptic meningitis. All studies including skin biopsy for urticarial vasculitis (UV) and systemic lupus erythematosus (SLE) were negative except for the data from non‐specific inflammations. A systemic corticosteroid therapy dramatically reduced her symptoms. An unusual clinical course for this patient is described. It might suggest that this case is a presentation of the disease entity of UV, chronic urticaria and possibly SLE. To our knowledge, a similar case has not been previously reported.

IGA NEPHROPATHY IN A PATIENT WITH UNILATERAL RENAL AGENESIS

Shinobu Waga, Department of Pediatrics, Hirosaki University School of Medicine, 5-Zaifucho, Hirosaki, 036 (Japan) Dear Sir, The clinical relevance of the observation that reduction of renal mass in rats promotes the development of proteinuria and progressive renal failure due to focal glomerular sclerosis (FGS) is controversial. Similar mechanisms were postulated in the patients with unilateral renal agenesis, oligomega-nephronia, or unilateral small kidney, but Novick et al. [ 1 ] suggestet that patients with a solitary kidney had an increased risk for progressive nephropathy after partial ne-phrectomy. These observations suggest that patients with a solitary kidney need further loss of nephrons in the remaining kidney due to resection or other structural abnormalities to be comparable to the rat model. Therefore, exposure of glomerulonephritis to a solitary kidney may also be relevant, but there have been few reports. Since IgA nephropathy is a disease with mesangial proliferation, which may cause progressive renal insuffici-cency, it is interesting to know how IgA nephropathy in a solitary kidney influences the outcome of renal function, and how the disease itself is influenced by a solitary kidney. We describe a boy with agenesis of the right kidney who had IgA nephropathy associated with nephrotic syndrome. A 9-year-old boy was found to have heavy proteinuria and hematuria on 27th July 1992. Proteinuria or hematuria had not been discovered by annual screening for uriniary abnormality at school in the previous 4 years. On admission, he weighed 28 kg and, within a short time, gained 5 kg. Blood pressure was 114/40 mm Hg. No skin or mucosal lesions were detected. His testes

Sequential measurement of mesangial matrix area occupying the glomerulus in children with IgA nephropathy

BackgroundIgA nephropathy is the most common form of primary glomerulonephropathy in children it has a variable clinical course, from spontaneous remission to progression to renal death. It has been reported that predominant mesangial hypercellularity is characteristic of early lesions, and that it changes to a gradual matricial increase, with sclerosis, according to the disease progression.MethodsA sequential measurement of the ratio of mesangial matrix area to glomerular area (M/G) was done in 5 children with moderately proteinuric IgA nephropathy, who underwent 3 consecutive, repeat renal biopsies. A prompt initiation of alternate-day prednisolone therapy (an initial dosage at 1 mg/kg, maximum 60 mg) after the first renal biopsy was done in 4 cases. The remaining patient received this therapy after the second renal biopsy.ResultsA sequential measurement of the M/G in the former cases did not show an increase between the biopsies, while measurement of the latter one showed a progressive increase. Moreover, the case that had an increase in the M/G showed renal impairment at the third biopsy.ConclusionAlthough a small number of cases were examined, a sequential measurement of the M/G in children with moderately proteinuric IgA nephropathy may be a valuable indicator for a more precise evaluation of clinical outcome in a clinical setting.