Transition of children with membranoproliferative glomerulonephritis to adolescence and adulthood.

BACKGROUND: Many chronic renal diseases in children, including membranoproliferative glomerulonephritis (MPGN), often continue into adulthood, and these patients require continuing management. Despite the importance of the topic, there has been limited discussion about the problems of transition in children with continuing renal disease. We report our experience in patients with MPGN, as they matured from childhood to adolescence and adulthood, so-called "carry-over" cases. METHODS: The clinical course of diffuse MPGN in 27 children was retrospectively reviewed. Patients were over 18 years old at the end of follow-up. RESULTS: The mean follow-up period was 12.6 years; 20 children (74%) were identified by school urinary screening. The clinical course was favorable, and none of the patients progressed to end-stage renal failure during follow-up. However, eight patients (30%) continued to demonstrate proteinuria; two patients were nephrotic. Four patients were non-compliant and discontinued medication by themselves. Three patients were still on low dose of alternate-day (ALD) prednisolone. Twenty patients finished the treatment and were followed for an average of 4.6 years. Only one demonstrated trace amounts of proteinuria 1 year after discontinuing ALD prednisolone. CONCLUSIONS: MPGN often continues during maturation from childhood to adulthood, and patients are usually referred to adult nephrologists. Good communication between pediatric and adult nephrologists is important. In addition, more in depth explanation and reeducation about their disease and its management are helpful when these patients reach adolescence. These measures will improve their care and help to assure compliance with their medication regimen.

Immediate effect of Shakuyaku-kanzo-to on muscle cramp in hemodialysis patients.

We administered 2.5 g of Shakuyaku-kanzo-to granule to 61 patients who had muscle cramp during hemodialysis (HD) sessions and examined its immediate effects. We selected 10 patients who wanted to take the drug at home, out of cases, for whom the drug was effective on the study described above and had them take the drug in the same way at the beginning of muscle cramp at home examined the effects. In the study during HD sessions, muscle cramp and its associated pain disappeared in 5.3 +/- 3.9 min on average in 54 out of 61 cases. In the study of patients who took the drug at home, muscle cramp disappeared within 10 min in all cases. Shakuyaku-kanzo-to is thought to be very useful for muscle cramp during HD sessions of hemodialized patients because it has immediate effects by its oral administration on the occasion of cramp. With regard to the muscle cramp, which appears at home after HD sessions, the patients can cope with it by taking the drug by themselves. This is an epoch-making therapy, for it was impossible to cope with muscle cramp except in hospitals because the therapy of muscle cramp was limited to intravenous infusion of hypertonic solutions of dextrose, mannitol, and saline during HD sessions.

[Treatment of adynamic bone disease with the complete replacement from calcium carbonate to sevelamer hydrochloride].

The aim of this study was to examine the therapeutic effect of hypocalcemic stimulation caused by sevelamer hydrochloride (SH) administration on adynamic bone disease (ABD). The subjects were 28 maintenance hemodialysis (HD) patients who had remained in ABD state in spite of no administration of vitamin D(3) since HD induction (15 males and 13 females;12 diabetic patients and 16 non-diabetic patients). The mean age was 61.8+/-9.5 years and the mean HD duration was 5.5+/-3.9 years. The calcium concentration in the dialysate was 3.0 mEq/L. We made the final daily dose of SH after two months the same as the first daily dose of calcium carbonate (CC) in the following manner. At first we administered only CC at breakfast and lunch and SH at supper. And for the next two weeks we administered CC at breakfast and SH at lunch and supper. And for the final two weeks we administered only SH. After that we increased the dose of SH as much as possible. We evaluated the therapeutic effect of the above treatment on ABD using intact-osteocalcin (iOC) [Teijin. Tokyo] as a marker before and 6, 12 months after the beginning of the replacement. If iOC Ievel of 30 to 70 ng/mL showed normal tumover bone (NTB), 5 cases (17.9%) changed into NTB in 6 months. 9 cases (32.1%) changed into NTB in 12 months and one case (3.6%) changed into ostitis fibrosa in 12 months. It is thought that SH is effective for the treatment of ABD but we have to be careful for ostitis fibrosa.

Asymptomatic hyperleukocyturia in hemodialysis patients analyzed by the automated urinary flow cytometer.

The number of urinary white blood cells (WBC) is useful marker of urinary tract infection in patients without renal insufficiency. On the other hand, it is unclear what urinalysis data are normal in hemodialysis (HD) patients. The occurrence and numbers of WBC in 75 asymptomatic HD patients and 133 healthy individuals as controls were determined by the automated urinary flow cytometer (UF-100, Sysmex Co., Kobe, Japan). The finding of hyperleukocyturia (>10 WBC per high power field of 400x microscopic magnification) was observed in 46.7% of HD patients. Urinary WBC significantly increased with decrease in the urinary volume. It may not be appropriate to indiscriminately apply the diagnostic criteria of pyuria for patients with normal renal function to dialysis patients because the number of urinary WBC was closely related to the urinary volume in HD patients.

Hypocomplementemia and membranoproliferative glomeruloneophritis in children.

BACKGROUND: The 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. METHODS: A 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. RESULTS: Serum 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. CONCLUSIONS: The incidence of hypocomplementemia detected in school urinary screening and new cases of MPGN continue to decrease in our experience.

Kidney disease quality of life of Japanese dialysis patients who desire administration of sildenafil and the treatment of erectile dysfunction using sildenafil.

Erectile dysfunction (ED) is common among patients on dialysis therapy. In the present study, we attempted administration of sildenafil to Japanese patients undergoing dialysis. In order to diagnose ED before prescribing sildenafil, we assessed the hemodialysis patients who desired sildenafil by using the five items version of the International Index of Erectile Function (IIEF-5). In addition, the characteristics of the quality of life in Japanese hemodialysis patients who desired sildenafil were assessed using the kidney disease quality of life (KDQOL). To all 37 male subjects (mean age of 53.8 +/- 10.4 years) attending the Outpatient Hemodialysis Unit at Atsugi Clinic (Atsugi City, Japan), it was explained by their primary doctor that the treatment of ED with sildenafil was possible. As a result, 10 patients (27.0%) desired the treatment. For eight patients, ED was diagnosed by IIEF-5 prior to prescription of sildenafil. The mean IIEF-5 scores were 6.13 +/- 4.67 points. Sildenafil was prescribed to five patients (three diabetic, two non-diabetic) and sexual function was improved in three cases. The main adverse effect was found to be ventricular arrhythmia in one case. As for KDQOL, the group desiring sildenafil showed significantly high values in Dialysis Staff Encouragement and Patient Satisfaction. Among the other nine dialysis patients (five diabetic, four non-diabetic; mean age of 58.1 +/- 8.9 years) who visited the ED department of Ishida Hospital (Asahikawa City, Japan), sildenafil was effective for all non-diabetic patients (100%) and for only one diabetic patient (20%). Among all 14 patients at Atsugi Clinic and Ishida Hospital, sildenafil efficacy rates were 83.3% for non-diabetic patients and 37.5% for diabetic patients. Non-diabetic patients without the side-effects were all responders for the sildenafil treatment. The patients who relied on the dialysis staff and were more satisfied with the general treatment in the dialysis institute desired the administration of sildenafil under the present circumstances wherein the dialysis population had few experiences of sildenafil treatment. Diabetic status is thought to be a negative factor for the response of sildenafil treatment in dialysis patients.

Blood compatibility and filtration characteristics of a newly developed polyester polymer alloy membrane.

BACKGROUND: Many synthetic polymeric membranes have been widely used for blood purification therapies for removing larger solutes and for better blood compatibility. METHODS: Five different filters with polyester polymer alloy (PEPA) membrane (Nikkiso) that included polyvinylpyrrolidone (PVP), that is, standard-pore-size FLX (PEPA with PVP(-)), standard-pore-size FDX (PEPA with PVP(+)), new FDX (PEPA with PVP(++)), larger-pore-size FDY (PEPA with PVP(+)), and new FDY (PEPA with PVP(++)), were evaluated in terms of in vivo blood compatibility and aqueous in vitro sieving coefficient (s.c.) for albumin. RESULTS: FDY showed excellent small changes in C3a concentration during the treatment right after switching from polysulfone membrane, leaving no significant changes in other blood-compatible indices. New FDY, in contrast, showed less biocompatible characteristics in terms of C3a, which may be caused by increased use of PVP. FLX (no PVP) showed the highest s.c. for albumin. With a hydrophilic agent (PVP), the s.c. was greatly reduced in FDX. By enlarging the pore diameter in FDY, the s.c. increased in accordance with the enlargement. Generally speaking, polysulfone filters with PVP(+++) reach the peak s.c. value approximately 10 min after starting the experiment. The s.c. of the PEPA membrane with no or small amount of PVP, which showed excellent blood compatibility, never showed peaks, whereas the peak s.c. values were found in new PEPA with increased amount of PVP that showed higher C3a changes. CONCLUSIONS: The separation characteristics of aqueous albumin may be related to its blood compatibility.

Long-term follow-up of type II membranoproliferative glomerulonephritis in two children.

We report the long-term follow-up of two patients with type II membranoproliferative glomerulonephritis (MPGN). One Patients was treated with high-dose alternate-day prednisolone and the other with dipyridamole. Both had favorable clinical courses over 8.5 and 15.5 years of follow-up, respectively. One patient who showed diffuse proliferative changes lost the urinary abnormalities 2 years after starting treatment. Her follow-up renal biopsies showed histological improvement. In the other patient, a boy, renal biopsy performed 6 years after the onset showed mild mesangial proliferation and a moderate matrix increase. His urine became normal 14 years after onset. Intramembranous electron-dense deposits persisted in these patients over 7 and 6 years of observation, respectively. Patients with type II MPGN are usually difficult to treat and often progress to endstage renal failure. There is, however, a group of patients who have a favorable clinical course.

Focal segmental membranoproliferative glomerulonephritis in children.

Eight patients with focal segmental membranoproliferative glomerulonephritis (FSMPGN) were followed for 5-16 years. Their urinary abnormalities were detected by school urinary screening in seven, and one patient presented with nephrotic syndrome. All but one patient were treated with alternate-day (ALD) prednisolone. With time, urinalysis became normal in six and two continued to have proteinuria with or without hematuria. Serum albumin, cholesterol, and creatinine levels were normal at the last follow-up. Serum C3 returned to normal levels in six, but remained persistently decreased in two. Mesangial proliferation and matrix changes in glomeruli without MPGN lesions were mild. Subendothelial and mesangial electron-dense deposits and deposits containing C3 along capillary walls and mesangium were observed. MPGN lesions and mesangial proliferation improved. No severe growth retardation was observed, but the duration and dosage of ALD prednisolone could be reduced further, since the patients with FSMPGN seemed to have an excellent prognosis.

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

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.