Kikuo Iitaka

Idiopathic membranoproliferative glomerulonephritis in Japanese children

The course of idiopathic membranoproliferative glomerulonephritis (MPGN) in 41 Japanese children (21 boys, 20 girls) is reported. The mean follow-up period was 8 years, 9 months; 29 children with MPGN (71%) were identified by school urinary screening; 32 patients had type I MPGN, 2 type II and 7 type III; 10 patients were treated with multiple low-dose cocktail therapy (MLD), 8 with MLD followed by high-dose alternate-day (ALD) prednisolone and 21 with high-dose ALD prednisolone alone. In 1 patient, MPGN progressed to end-stage renal failure. The serum creatinine level in all of the remaining 40 patients was ≤ 1.3 mg/dl at the last follow-up. Urinalysis was normal in 24 (59%). Of the 17 patients who still had urinary abnormalities, 4 had nephrotic syndrome. The incidence of remission of urinary abnormalities was highest in the patients treated with high-dose ALD prednisolone. Rebiopsy was performed in 33 patients, and revealed slight histological improvement in 11 (33%) patients, moderate improvement in 8 (24%), marked improvement in 5 (15%) and deterioration or no improvement in 9 patients (27%). Relatively few side effects of treatment were observed. The superior outcome of the MPGN patients in this compared with other studies may be the result of earlier detection and treatment.

Henoch-Schönlein purpura with hypocomplementemia in children

Abstract  Background : The clinical course and prognosis of Henoch‐Schönlein purpura (HSP) associated with hypocomplementemia are not clear.

Familial cases of Henoch-Schonlein purpura in eight families

Abstract Background : Familial cases of Henoch‐Schönlein purpura (HSP) have rarely been reported.

Hypocomplementaemia and membranoproliferative glomerulonephritis in school urinary screening in Japan

Yearly screening was performed from 1980 to 1985 in 92,934 school children in the first to ninth grades; a total of 370,148 urine specimens were evaluated. In 1980 and 1981, urinary abnormalities were detected in 0.23% of children examined; the detection rates at the third level of screening for proteinuria, haematuria and haematuria with proteinuria were 0.03%, 0.15% and 0.06%, respectively. The criteria for haematuria were changed from 6 to 20 red blood cells/high power field in the second screening after 1981. Between 1982 and 1985, the equivalent rates were 0.02%, 0.07% and 0.03%, respectively, and the total abnormality prevalence was 0.13%. In 19 children the serum C3 level was below 53 mg/dl; in 5 of these it was below 30 mg/dl. Of these 5 children, 4 underwent renal biopsy and were diagnosed as having membranoproliferative glomerulonephritis.

Decreasing hypocomplementemia and membranoproliferative glomerulonephritis in Japan.

Abstract The number of patients with membranoproliferative glomerulonephritis(MPGN) diagnosed in our institution and the incidence of hypocomplementemia as determined by school urinary screening programs in Japan were investigated retrospectively. Fifty-seven children were diagnosed as having MPGN between 1974 and 1997. Of these, 13 patients were diagnosed during period 1 (1974–1979) and 25 during period 2 (1980– 1985). A decreasing trend was observed during periods 3 (1986–1991) (12 patients) and 4 (1992–1997) (7 patients) compared with period 2 (P<0.05 and P<0.01, respectively). A significant difference was also noted when MPGN was compared between periods 1 and 2 (1974– 1985) and periods 3 and 4 (1986–1997) (P<0.01). Serum C3 was measured in 1,282 school children with abnormal urinary findings between 1980 and 1997. Thirty children had hypocomplementemia. The incidence of hypocomplementemia was significantly lower in period 3 (1986–1991) (9 children) and period 4 (1992–1997) (2 children) compared with period 2 (1980–1985) (19 children) (P<0.05 and P<0.01, respectively). New cases of MPGN in our institution and the incidence of hypocomplementemia as determined by school urinary screening programs are decreasing in Japan. The reason for this trend is unknown.

Steroid-sensitive nephrotic syndrome in two families

Few reports of familial cases of steroid-sensitive nephrotic syndrome (SSNS) are available. Of 123 children with SSNS, four cases in two families are presented. Two sisters and a father and his daughter developed SSNS. The incidence of SSNS siblings in Japan seems to be similar to the incidence reported in other countries. SSNS in two generations is rare. To our knowledge, cases of SSNS in a parent and a child in Japan have not been reported.

Clinical course of congenital nephrotic syndrome and Denys‐Drash syndrome in Japan

Abstract Background : The prognosis of Japanese patients with congenital nephrotic syndrome (CNS) and Denys‐Drash syndrome (DDS) is not clear.

A girl with Peters plus syndrome associated with myelomeningocele and chronic renal failure

A girl was born with sclerocornea of the right eye, corneal staphyloma of the left eye and lumbar myelomeningocele. The myelomeningocele was repaired soon after birth. The corneal staphyloma was perforated during infancy. She received keratoplasty and achieved light perception. Her right kidney revealed multicystic dysplasia and was non-functioning at birth. She had neurogenic bladder, and her renal function deteriorated gradually. Peters plus syndrome was diagnosed based on anterior ocular segment anomalies, short stature, developmental delay and characteristic face. Anterior ocular segment anomalies are rare findings, but seem to be occasionally associated with spina bifida and renal anomalies. Myelomeningocele and chronic renal failure in patients with Peters plus syndrome have not been reported to our knowledge.

Long-term follow-up of type III membranoproliferative glomerulonephritis in children

Abstract Seven patients with type III membranoproliferative glomerulonephritis (MPGN) were followed for 9–17 years. Their mean age at presentation was 11.0 years. Their urinary abnormalities were detected by school urinary screening in five, and two patients presented with nephrotic syndrome. With time, urinalysis became normal in five, proteinuria persisted in one and nephrotic syndrome persisted in one. All had serum creatinine levels below 1 mg/dl at the last follow-up. All were initially hypocomplementemic. Serum C3 levels became normal in five patients but decreased again in three without clinical changes. Mesangial proliferation was initially slight or moderate in five but, except for one patient, histological changes persisted in follow-up biopsies even though in two the urinalysis became normal. Electron micrographic studies using silver-methenamine staining revealed subepithelial and subendothelial deposits associated with basement membrane disruption and layering of the lamina densa, an abnormality typical of MPGN type III. These observations suggest long-term outcome of type III MPGN is good despite persisting changes in type III lesions.

Hypocomplementemia and membranoproliferative glomeruloneophritis in children

BackgroundThe incidence of hypocomplementemia detected in the school urinary screening program in Kanagawa Prefecture, Japan, and the number of new patients with membranoproliferative glomerulonephritis (MPGN) diagnosed in our institution were decreasing during the period between 1974 and 1997. Follow-up of this study was performed during the period between 1998 and 2003.MethodsA total of 1 230 398 urine specimens in elementary and junior high school were examined between 1980 and 2003. Serum C3 was measured in children with abnormal urinary findings. Fifty-nine children were diagnosed as having idiopathic MPGN in our hospital between 1974 and 2003.ResultsSerum C3 was measured in 1546 school children with abnormal urinary findings, and 34 had hypocomplementemia between 1980 and 2003. Among 264 children in whom C3 was measured between 1998 and 2003, only 4 had mild hypocomplementemia of 83–86 mg/dl (normal, 87–157 mg/dl). Between 1974 and 2003, 59 children (8.8%) were diagnosed as having idiopathic MPGN in our hospital, whereas only 2 were diagnosed during the period between 1998 and 2003.ConclusionsThe incidence of hypocomplementemia detected in school urinary screening and new cases of MPGN continue to decrease in our experience.