Satoshi Hisano

Asymptomatic isolated microhaematuria: natural history of 136 children.

In a mass screening programme, 251 children with isolated microhaematuria were detected. Of these 251 children, 115 were excluded from the study because of microhaematuria, secondary to a specific cause. The remaining 136 children were diagnosed as having asymptomatic isolated microhaematuria (ASH). Of these 136 children, 23 had evidence of urinary abnormalities in their family members. Red blood cell casts were evident in 31 children at their initial visit or during the follow-up period. Ten children had one or more episodes of macrohaematuria during the study. Renal biopsy was performed in 19 children because of indications of glomerular discase, and 13 of these 19 children had mild to moderate glomerulonephritis. None of these 136 children developed hypertension or renal impairment after a mean period of 7.4 years (range 6–13 years). Thirty-five children had normal urinary findings within 6 years of their initial visit, and 100 have had persistent microhaematuria, without proteinuria throughout the follow-up period. The other child had microhaematuria with proteinuria greater than 1 g/m2 per day at the end of the study. This study suggests that the prognosis of ASH is good and that renal biopsy is not indicated for children with ASH.

Asymptomatic haematuria and proteinuria: Renal pathology and clinical outcome in 54 children

In a mass screening programme, 54 children with haematuria and proteinuria were detected and evaluated by clinical findings and renal histology. IgA glomerulonephritis (GN) occurred in 29 patients, diffuse mesangial proliferative GN (DPGN) in 16, membranous GN (MGN) in 4, membranoproliferative GN (MPGN) in 3, and focal segmental glomerular sclerosis (FSGS) was seen in 2. Of the 35 children with proteinuria less than or equal to 1 g/m2 per day, 21 with IgA GN and 14 with DPGN had only mild to moderate glomerular changes. None of these children had developed renal impairment after a mean period of 6.5 years (range 5–10 years). On the other hand, 8 children with IgA GN, 2 with DPGN, 4 with MGN, 3 with MPGN, and 2 with FSGS had proteinuria that exceeded 1 g/m2 per day. The biopsy specimens from these children showed moderate to severe glomerular changes, and 7 of these children had hypertension or renal impairment during the period of evaluation. This study suggests that a poor outcome correlates with the level of proteinuria and the severity of renal pathology in children with haematuria and proteinuria.

The Natural History of Screening Detected IgA Glomerulonephritis in Children

ABSTRACT. The clinical course of 43 children with IgA glomerulonephritis detected by mass urine screening was followed for a mean period of 8.1 years. Histological findings were graded according to the severity of glomerular and tubulointerstitial lesions. There was no correlation in the severity of histological grade and clinical outcome between subjects with microscopic hematuria and those with microscopic hematuria and pro‐teinuria nor between those with and without one or more episodes of macroscopic hematuria during the follow‐up period. None of the 35 children with proteinuria ≤ 1 g/m2/day had severe histological findings or developed renal impairment. In contrast, the 8 children with proteinuria > 1 g/m2/day had moderate and severe histological findings. Four of these 8 children developed hypertension or renal insufficiency during the follow‐up period. Our study indicates that the outcome of screening detected IgA glomerulonephritis in children correlates with the level of proteinuria and the severity of renal pathology.

Immune status of children on continuous ambulatory peritoneal dialysis

Immunological parameters including serum IgG, IgA and IgM, lymphocyte phenotypes (CD3, CD4, CD8, HLA-DR+CD3-), natural killer cell activity and lymphocyte proliferation with phytohaemagglutinin were assessed in 10 children on continuous ambulatory peritoneal dialysis (CAPD) and 10 control subjects. Live vaccines were injected into 6 of the 10 children on CAPD (4 had a combined measles-mumps-rubella vaccine and the other 2 mumps vaccine). Serum antibody titres to these viruses were measured before and after vaccination. The serum IgG level was statistically lower in the CAPD group than in the control group (P<0.01), but there was no difference in the percentage of HLA-DR+CD3-cells and in the ratio of CD4 to CD8 between the two groups. There were no differences in the other parameters between the two groups. All of the 6 vaccinated children seroconverted, and serious side effects were not noted. Our results suggest that children on CAPD have no significant immune impairment.

Epidemiologic Survey of Children with End‐Stage Renal Disease

We performed an epidemiologic study on the basis of a questionnaire survey of 162 children with end‐stage renal disease (ESRD). Sixty‐nine (43%) of our 162 children, including 25 detected at mass screening of urine, were found by chance hematuria and/or proteinuria. The three major causes of ESRD in our children were chronic glomerulonephritis (CGN) in 56, congenital anomalies of the urinary tract in 30, and nephrotic syndrome (NS) in 27. The renal pathology in 39 children with CGN or NS was focal glomerular sclerosis in 15, diffuse mesangial GN in 7, IgA GN in 5, membranoproliferative GN in 3, membranous GN in 3, and unclassified in 6. Forms of dialysis initiated were hemodialysis in 91 children, continuous ambulatory peritoneal dialysis (PD) in 66, and intermittent PD in 5. Renal transplantation was performed on 38 children, and the graft and the patient survival rates were 76% and 89%, respectively. The survival rate of our 162 children for a mean follow‐up of 8.1 years was 77%. In conclusion, an integrated program of maintenance dialysis and transplantation provides a favorable life for children with ESRD.

Transcapillary ultrafiltration and peritoneal equilibration test in pediatric patients

The usefulness of the peritoneal equilibration test (PET) in children is unknown. The relationship between transcapillary ultrafiltration and PET was investigated in order to evaluate the usefulness of PET in children. PET was performed on 14 patients undergoing peritoneal dialysis. Their age and bodyweight ranged from 3.8 to 23.6 years and 10.2 to 55.8 kg, respectively. The patients were divided into two groups according to bodyweight; group A patients weighed ≤ 40 kg (n = 7) and group B patients weighed > 40 kg (n = 7).

Long-term prognosis and prognostic factors of Japanese children with mesangial proliferative glomerulonephritis without IgA deposition

Mesangial proliferative glomerulonephritis without IgA deposition (non‐IgA MesPGN) is commonly detected in biopsy specimens, but the clinicopathological correlation with the long‐term prognosis still remains obscure. The aim of our study is to elucidate the long‐term prognosis and the clinicopathological prognostic factors in patients with non‐IgA MesPGN. We mailed questionnaires to 122 patients with primary glomerulonephritis who were biopsied between 1963 and 1975. Information was obtained from 109 of these 122 patients and 55 were histologically rediagnosed as having non‐IgA MesPGN. The histological alterations of glomeruli and tubulointerstitium were classified into five grades. The mean period between the biopsy and the questionnaires was 20.5 years. Six of the 55 patients with non‐IgA MesPGN developed end‐stage renal failure and histopathological alterations of renal biopsies from these six patients were classified into grade IV or V. The presence of hypertension, heavy proteinuria of over 2 + or renal insufficiency at the biopsy was related to the severe histological changes, a grade of IV or V and to a poor prognosis. The renal survival rate of all the 55 patients was 88.3% at 20 years after the biopsy, while that of the 12 patients with severe histological changes was 48.6%. Although non‐IgA MesPGN is considered to be a heterogeneous disease, we cannot ignore the incidence of this disease and thus consider it to be one of the important primary glomerulonephritides that occur in childhood.

Comparison of asymptomatic and symptomatic childhood glomerulonephritis progressing to renal failure: a report of Kyushu Pediatric Nephrology Study Group

We evaluated the clinicopathological features and the outcome of 33 children with primary glomerulonephritis (GN) as the cause of renal failure; 17 had asymptomatic (ASP) haematuria and/or proteinuria and the remaining 16 had symptoms suggestive of GN. The renal histology in the ASP group indicated IgA GN in 6 children, focal segmental glomerular sclerosis (FSGS) in 4, diffuse proliferative GN (DPGN) in 3, membranous GN (MGN) in 1, membranoproliferative GN (MPGN) in 1 and diffuse sclerosing GN in 2. In the symptomatic (SYP) group, FSGS was evident in 9 children, DPGN in 3, MGN in 2, IgA GN in 1 and MPGN in 1. There was no difference in the histological severity between the two groups. Fourteen children in the SYP group had nephrotic syndrome (NS) and/or hypertension at their initial visits. Only 4 children in the ASP group showed NS or hypertension during the period of follow-up. Eleven children in the ASP group and all in the SYP group were treated with immunosuppressive and/or antihypertensive drugs, but these did not improve the prognosis of the ASP children compared with those in the SYP group. There was no significant difference in the mean duration between the onset of the disease and the start of dialysis in these two groups. In conclusion, it is questionable whether the urinary mass screening programme in Japan will alter the outcome of children with GN.

Growth Hormone and Adrenocorticotropic Hormone Levels Increased in a Child with Diencephalic Syndrome

A Japanese male child with diencephalic syndrome due to suprasellar tumor was treated in our department. There were no symptoms suggestive of hyper‐adrenalism despite an increased adrenocorticotropic hormone (ACTH), nor was there an excessive growth even in the presence of high fasting levels of growth hormone (GH). After radiation therapy, both ACTH and GH levels reverted to normal. Thus, the abnormal hypothalamic control following destruction of a certain area of the diencephalon may have led to increases in the levels of ACTH and GH. This may be the first documentation of increased ACTH levels in a patient with diencephalic syndrome.

Low Dose Administration of Captopril in Severely Hypertensive Children

Capopril, an oral angiotensin‐1‐ converting anzyme inhibitor, was administered to 8 severely hypertensive children. Three children did not respond to other conventional antihypertensive drugs. According to our protocol, patients who weighed 10 kg or less were given one half tablet, and those of over 10 kg were given one tablet in order to alleviate hypertensive crisis. There was a dramatic drop in blood pressure (BP) withing 30 minutes of administration of an anitial low dose of captopril (0.5–1.4mg/kg). The BP was stabilized to withing normal limits for two hours, and thereafter, the BP returned to the previous level after four hours. After administration of an initial dose of captopril, the BP was controlled by contined therapy of captopril alone with the exception of two cases to whom other antihypertensive were given in combination with captopril. Four cases with low renin activity responded as well to captopril as other with high renin activity did. Although captopril was given to two cases with nephrotic syndrome, there was no exacerbation of proteinuria. The only side effect was a skin eruption in one child who received long‐term treatment. We conclude that even a low dose of captopril is very effective in the control of both hypertensive crisis and of hypertensive children refractory to ther potent antihypertensive drugs.