A case of recurrent lupus nephritis after renal transplantation.

A case of recurrent lupus nephritis in an 18-year-old girl with a renal transplant is described. Serological titer of ANA and Anti-DNA were low prior to renal transplantation following pulse therapy with methylpredonisolone and high dose oral predonisolone. A living related transplantation was performed after 6 months of hemodialysis. Maintenance immunosuppressive therapy consisted of predonisolone, mizoribine and cyclophosphamide. Graft function remained stable for one and half years after transplantation. Clinical recurrence was heralded by the development of proteinuria. If the serologic activities had been analyzed, the increase in ANA and Anti-DNA titers a few months before the onset of proteinuria might have predicted a possible histopathological recurrence. Fortunately, however, despite the histological and clinical recurrence of systemic lupus erythematosus, her renal allograft has continued to function fairly well.

Comparison of growth during continuous ambulatory peritoneal dialysis and renal transplantation using conventional immunosuppressive drugs in children.

OBJECTIVE: Growth during continuous ambulatory peritoneal dialysis (CAPD) and after renal transplantation (Tx) was assessed in children under the age of 12 years. DESIGN: Growth was assessed by calculating the standard deviation score for height of normal children of the same age and gender. PATIENTS: Twenty-one patients undergoing CAPD and 13 patients who had received renal Tx at Kitasato University Hospital. RESULTS: Overall growth in those children undergoing CAPD and after renal Tx was not good, but growth during CAPD seemed to be slightly better than after renal Tx using conventional immunosuppressive drugs. Only young children who had started CAPD or received renal Tx before the age of 5 years with functioning graft showed catch-up growth, but the final height of these children may still be smaller than normal. CONCLUSION: Growth in children with end-stage renal disease is poor but growth during CAPD seems to be slightly better than after renal Tx using conventional immunosuppressive drugs.

The Japanese National Registry data on pediatric CAPD patients: a ten-year experience. A report of the Study Group of Pediatric PD Conference.

OBJECTIVE: Over the past 10 years, we have collected data on pediatric patients less than 16 years of age from the National Registry of CAPD (continuous ambulatory peritoneal dialysis). We present our experience with this population. DESIGN: The database details the patient number, age, weight, height, outcome, cause of death, reason for terminating CAPD therapy, peritonitis, and catheter survival. PATIENTS: Of the 434 patients (239 males, 195 females), 37 patients (8.5%) were under 1 year of age and 164 patients (37.8%) were under 6 years of age. About half of the patients were less than 20 kg in weight, clearly indicating that CAPD was the treatment of choice in young children. The duration on CAPD for these patients was less than 2 years for 233 patients (54%), and was 5 years or more in 48 patients (11%). RESULTS: The outcome of the total patient population of 434 as of May, 1991, is as follows: 229 patients (52.8%) were being successfully treated with CAPD, 47 patients (10.8%) died, and 78 patients (18.0%) received a kidney transplantation. The patient survival rate was 85.6% at 3 years and 81.7% at 5 years. The technique survival rate was 74.9% at 3 years and 63.5% at 5 years. The rate of peritonitis was one episode over 28.6 patient-months. The mean catheter duration was 1.68 years. Peritonitis rate, catheter survival rate, and the rate of tunnel infection were worse in children less than 6 years of age than in older children. CONCLUSION: The excellent patient and technique survival rates indicate that CAPD is an effective treatment for children with end-stage renal disease in Japan. The high infection rates in younger children indicate that extra careful management is needed for this young age group.

[A case of Trichosporon beigelii peritonitis in CAPD].

Continuous ambulatory peritoneal dialysis (CAPD) was introduced to Japan ten years ago and was established as the treatment for end-stage renal disease along with HD. Although the incidence of peritonitis in CAPD has decreased by educating the patients and parents and the improvement of various devises of CAPD, peritonitis is still one of the major complications of CAPD. Fungus is a rare pathogen for peritonitis in CAPD, but it must be considered as a causative agent in cases of intractable peritonitis. This report describes the first case of Trichosporon beigelii (T. beigelii) peritonitis in CAPD in Japan. A nine year old boy with chronic renal failure due to bilateral vesicoureteral reflux was given CAPD treatment four years prior to admission. This patient had been admitted to our hospital frequently because of recurrent bacterial peritonitis. The peritonitis in CAPD was usually treated by changing the peritoneal fluid and antibiotic treatment. In this case T. beigelii was proved to be a pathogen of peritonitis by culture of CAPD fluid and also serum antibody titers. T. beigelii infection was successfully eradicated from the peritoneal cavity by administration of MCZ and by the removal of peritoneal catheter. The patient was switched from CAPD to HD. In the case of intractable peritonitis in CAPD, rare fungal pathogens such as T. beigelii must be considered as a causative agent.

[Renal osteodystrophy and growth in children undergoing CAPD].

Renal osteodystrophy (ROD) and growth were assessed in 20 children undergoing CAPD. Serum calcium and inorganic phosphorus levels were maintained with normal limits in these patients. Elevated alkaline phosphatase and c-PTH levels and abnormal radiological bone changes of ROD were observed in patients with severe ROD, but they were not correlated each other in patients with mild ROD. Fifteen patients showed the radiological bone changes of ROD with deterioration and improvement in some patients, and administration of vitamin D seems to be necessary to prevent ROD during CAPD treatment. The growth of older children was poor during CAPD, but catch up growth was observed in young children under the age of 7 years. The growth was affected by rickets in young children, but the presence of mild secondary hyperparathyroidisms seemed to have little influence on growth in older children.

[Echocardiographic assessment of cardiac function in the children of chronic renal failure with cardiomegary].

Five children (four boys and one girl) with chronic renal failure (CRF) developed congestive heart failure 0.5 to 11 years after the onset of the disease. Their ages were from 4 to 13 years old. They noticed tachypnea, tachycardia, cough, chest anxiety, general fatigue and their chest X-rays showed cardiomegaly with cardio-thoracic ratio (CTR) of from 55 to 63% and pulmonary congestion. Their echocardiograms showed no cardiomuscular hypertrophy, but the dilatation of left ventricular diastolic diameter (LVDd), and the decreased ejection fraction (EF) were observed. They were treated with water restriction, antihypertensive agents, cardiotonics and dialysis. Their clinical symptoms improved promptly, but their cardiomegary and echocardiographic findings improved gradually. The causes of heart failure in these patients seemed to be due to uremia, fluid overload and hypertension. The echocardiographic examination was useful for the management of the children with CRF in heart failure.

[Prominent subepithelial deposits detected on 2nd renal biopsy in a boy with membranoproliferative glomerulonephritis type I: a case report].

The first renal biopsy of a 7-year-old boy with microscopic hematuria, hypocomplementemia (CH50 20 U/ml, C3 33 mg/dl, C4 13 mg/dl) and otherwise normal renal function revealed lobular, but not diffuse, expansion of glomerular mesangial areas showing mild to marked mesangial cell proliferation and thickening of the capillary walls with double contours as seen on light microscopic examination. Granular C3 deposition along the capillary wall and in the mesangium was observed on immunofluorescent microscopy. Further, electron microscopy showed subendothelial dense deposits and circumferential mesangial interposition. Thus, he was diagnosed as membranoproliferative glomerulonephritis (MPGN) type I. Thereafter, high-dose, alternate-day administration of prednisolone was started. A few months after treatment, microscopic hematuria disappeared and hypocomplementemia improved. The second renal biopsy at 11 years of age showed markedly decreased mesangial cell proliferation and subendothelial dense deposits. However, C3 deposition still persisted and subepithelial dense deposits had increased in number prominently at this time. Whether MPGN type III is a distinct disease entity or a variant of MPGN type I has not yet been established. The morphological changes in this reported case might justify the notion that MPGN type III is a variant form of MPGN type I.

[Peritoneal sclerosis in children under treatment with peritoneal dialysis].

Peritoneal dialysis(PD) is an established method of dialysis for children at the end stage of renal failure. Among several problems associated with PD, peritoneal sclerosis is one of the most serious complications. We examined four children on PD who were diagnosed as having peritoneal sclerosis in 1997 and 1998. Three of these cases were switched to hemodialysis(HD) and one is now undergoing a switch from PD to HD. Although all of the four patients had a history of bacterial peritonitis, they had been maintained in a fairly good condition on PD. The mean duration of PD was 9 years(4-14 years). All cases were anuric and had been maintained on PD without any problems of peritoneal function. From these observations, we should consider peritoneal sclerosis even in patients with a good clinical condition during the long-term period of PD. We recommend serial peritoneal biopsies, at least at the time of peritoneal catheter changes, to check for peritoneal sclerosis. The patients who are diagnosed as having sclerotic thickening of the peritoneal membrane should be switched from PD to HD to prevent the occurrence of life-threatening peritoneal sclerosis.

[Henoch-Schönlein purpura nephritis (HSPN) in children: comparison of the incidence and severity between two 12-year groups].

From 1971 to 1982, we treated 286 patients of HSP in Kitasato University Hospital. In these 286 patients, 137 developed purpura nephritis (47.9%). During the second 12-year period (from 1985 to 1996), we treated 34 HSPN patients among 189 HSP patients (18.0%). The clinico-pathological evaluations and comparisons in 30 cases from 1971 to 1982 and in 11 cases from 1985 to 1996 were performed, using the ISKDC grading. The numbers of patients of HSP and the incidence of HSPN both decreased in the more recent 12-year group. Furthermore, the severity of the renal histopathological findings decreased in the more recent group as well.

[Membranous nephropathy in Japanese children].

Since 1973, 15 patients, consisting of 8 boys and 7 girls, were diagnosed as having membranous nephropathy (MN). The average age at detection was 8.2 years (2-14 years). The presenting symptom was edema in 1, pyrexia in 1 and upper respiratory infection in 1 case, in the all other cases, abnormal urinalysis was detected by the school or chance urinalysis. Surface antigen of hepatitis B virus (HBs) was positive in 6 patients and negative in 9. Anti-nuclear antibody (ANA) was positive in 3 and negative in 11. In one patient, ANA was not tested. One patient who was negative for ANA was diagnosed as having SLE 4 years later. At the last follow-up, 10 patients continued to have urinary abnormalities. Among these was one case positive for HBs antigen who went into end-stage renal failure. In the other 14 patients, the serum creatinine level was below 1.4 mg/dl. All patients showed a normal mesangium or mild mesangial proliferation. The patient diagnosed as having SLE. 4 years later showed mesangial deposits at the first renal biopsy. In our experience, most patients with MN were detected by the school or chance urinalysis and six of the these had positive HBs antigen. Lupus nephritis must be ruled out in making a diagnosis of idiopathic MN.

[A case of childhood IgA nephropathy who developed rapidly progressive glomerulonephritis in one-year clinical course].

A 10-year-old girl who presented with microscopic hematuria, proteinuria and normal renal function has been followed up for the past two years. At the first examination, renal biopsy revealed focal/segmental lesions accompanying by occaisional necrosis and small crescents. Diagnosis of IgA nephropathy was given by light, electron and immunofluorescent microscopic findings. She started on the treatment with dipyridamol and was followed up for one year without any serious complications. Thereafter, she suddenly developed severe deterioration of renal function (serum creatinine 2.7 mg/dl) with nephrotic syndrome and hypertension. The second renal biopsy done at this time indicated the presence of typical crescentic glomerulonephritis with mesangial proliferation. No vasculitis was noted. She was intensively treated with steroids, anticoagulants and other medication and responded fairly well clinically. The third renal biopsy performed 5 months afterwards demonstrated marked histological improvement, but there was still present mesangial proliferation and varied degrees of sclerotic changes with fibrocellular crescents. Focal interstitial fibrosis and collapsed tubules were also seen. At present, 5 months after the last renal biopsy, she has improved much better and her serum creatinine decreased to 1.9 mg/dl, although proteinuria of 3 g/day still persists. It is suggested that only a small segmental necrosis with crescent formation in IgA nephropathy should be considered as an important indicator of disease activity in the evaluation of prognosis.

[Extracorporeal shock wave lithotripsy in an 8-month-old infant].

An 8-month-old boy who presented with pyrexia and vomiting was referred to the pediatric clinic of Kitasato University Hospital. He was found to have hematuria, proteinuria and pyuria. Abdominal X-ray film showed calcification suggesting renal staghorn calculus in the bilateral kidneys. At 10-months and 11-months, we succeeded in treating him with extracorporeal shock wave lithotripsy (ESWL) without complications.

Hereditary nephritis associated with low-tone sensorineural hearing difficulty: a case report.

The proband was a 14-year-old girl with hematuria and proteinuria. Many members of her maternal pedigree had hematuria and proteinuria. Her mother, younger brother (age 12 years) and younger sister (age 9 years) had microscopic hematuria and proteinuria with normal renal function. Her mother had nephrotic syndrome during pregnancy and a renal biopsy was performed. Light microscopic findings of the renal biopsy specimen revealed mild mesangial proliferation and irregularity of glomerular basement membrane. The pedigree showed no chronic renal failure and no deafness. The proband had experienced microscopic hematuria and occasionally macroscopic hematuria since 3 years of age. Proteinuria increased steadily and at the age of 14 years, she had nephrotic syndrome and renal dysfunction (creatinine clearance of 57.9 ml/min/1.48 m2). Renal biopsy was performed and light microscopic findings showed segmental mesangial cell proliferation and numerous interstitial foam cells without significant findings by immunofluorescent study. Electron microscopic examination showed splitting into many layers and thinning of the glomerular basement membrane. She had no complaint of hearing. However, audiological studies detected bilateral low-tone (from 125 Hz to 1000 Hz) sensorineural hearing difficulty, ranging from 30 to 40 dB. High scores on the short increment sensitivity index (SISI) test suggested inner ear hearing difficulty. Audiogram of her brother revealed also low-tone sensorineural hearing loss. Hereditary nephritis with the characteristic lesion of the glomerular basement membrane and sensorineural hearing difficulty has been known as Alport syndrome. Alport syndrome associated with familial low-tone hearing difficulty has not been reported previously.

[A prospective controlled study of sairei-to in childhood IgA nephropathy with focal/minimal mesangial proliferation. Japanese Pediatric IgA Nephropathy Treatment Study Group].

To determine the effect of the Chinese herbal medicine, Sairei-to (TJ-114) in children with newly diagnosed IgA nephropathy showing focal/minimal mesangial proliferation, we undertook a prospective controlled study. One hundred and one patients were randomly assigned to receive Sairei-to for 2 years (group 1) or no drug for 2 years (group 2). Forty-six of the 50 patients in group 1 and 48 of the 51 patients in group 2 completed their trial. At entry, the two groups of patients did not differ in the clinical, laboratory and pathologic findings. At the end of the trial, urinary protein excretion and hematuria were significantly reduced in group 1, but were unchanged in group 2. Twenty-one group 1 patients (46%) had normal urine, but only 5 group 2 patients (10%) had normal urine at the end of the trial (p < 0.001). Blood pressure and creatinine clearance were normal at the end of the trial in all but one group 2 patient, who developed chronic renal failure. The present study demonstrates that 2-year Sairei-to treatment early in the course of disease is effective in children with IgA nephropathy showing focal/minimal mesangial proliferation.

[Acid-base disturbances in pediatrics].

Acid-base balance is regulated by respiratory and metabolic factors and also by the compensatory mechanisms of the body. This balance is often disturbed by various diseases or conditions in children. These diseases or conditions which cause acidosis or alkalosis in pediatric practice are presented and discussed in this paper with special interest in their age at presentation.