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Biomedical subjects

Masataka Honda

Publications and source records attributed to Masataka Honda.

34 records · Page 2Linked to original sources
PubMed2004

[Two cases of autosomal dominant polycystic kidney diseases who presented bilateral enlarged kidneys and severe hypertension in the neonatal period].

Autosomal dominant polycystic kidney disease(ADPKD) is rarely observed in the neonatal period. We report 2 cases of ADPKD who showed bilateral enlarged, hyperechoic kidneys and severe hypertension. It is difficult to differentiate ADPKD from autosomal recessive polycystic kidney disease (ARPKD) based on the initial clinical presentations in this period. In both cases, bilateral enlarged kidneys and severe hypertension were detected without oligohydramnion and respiratory distress. The mother of case 1 has polycystic kidneys. The father of case 2 was diagnosed as ADPKD. Case 2 had heart failure due to hypertension. Angiotensin converting enzyme inhibitor (ACE-I) was administered to both patients and resulted in good control of blood pressure. ADPKD in the neonatal and very early infantile period has diverse clinical courses. In general, although severe cases are rare, some cases have renal failure and/or hypertension as we reported. We emphasize that both the prompt diagnosis of ADPKD and the start of medication are of great importance in the neonatal and very early infantile period. We recommend that neonates and infants with a family history of ADPKD undergo screening including physical examinations, blood pressure measurements and urinalysis.

Diagnosis, Differential↗
PubMed2003

NPHS2 mutations in sporadic steroid-resistant nephrotic syndrome in Japanese children.

Podocin is an integral membrane protein encoded by NPHS2, which is mapped to 1q25-31 and is exclusively expressed in glomerular podocytes. NPHS2 mutations are responsible for autosomal recessive familial steroid-resistant nephrotic syndrome (SRNS) with minor glomerular abnormalities or focal segmental glomerulosclerosis (FSGS), which is characterized by early childhood onset (age less than 6 years) and rapid progression to chronic renal insufficiency. This gene mutation is also responsible for an adolescent/adult onset form of autosomal recessive familial FSGS with heavy proteinuria. It has been demonstrated that sporadic SRNS and heavy proteinuria are also due to NPHS2 gene mutations. We isolated genomic DNA from 36 Japanese children with chronic renal insufficiency caused by SRNS or heavy proteinuria, and analyzed all eight exons and exon-intron boundaries of NPHS2 using the polymerase chain reaction and direct sequencing. The age at onset of disease was 3.9+/-0.5 years. There were 29 patients with SRNS and 7 with heavy proteinuria without nephrotic syndrome at the onset, but all patients developed chronic renal insufficiency 4.6+/-0.8 years after the onset. A new homozygous missense variant of NPHS2, G34E (G101A) in exon 1, was detected in 1 of 36 patients. However, this homozygous variant was also found in 1 of 44 normal controls, suggesting that the mutation is a polymorphism. Two silent variants (T954C and A1038G) in exon 8 of this gene were also identified in some of the patients and normal controls, indicating that the silent variants are also polymorphisms. There was no significant difference in the genotypic and allelic frequencies of T954C and A1038G polymorphisms between the patients and normal controls. In conclusion, NPHS2 gene mutations are not a major cause of chronic renal insufficiency caused by sporadic SRNS or heavy proteinuria in Japanese children.

Adult↗
PubMed2003

Alport syndrome-like basement membrane changes in Frasier syndrome: an electron microscopy study.

Frasier syndrome (FS) is a rare disease characterized by male pseudohermaphroditism and slowly progressing nephropathy. FS originates from heterozygous mutation in the intron 9 splicing donor site of Wilms' tumor suppressor gene (WT1). Focal segmental glomerular sclerosis is common in FS, but there have not been so many detailed pathologic investigations. The authors examined the kidneys of 3 patients with FS. The results showed that nephropathy started as mesangial proliferative glomerulonephritis, and later a concomitant focal segmental lesion developed. In all cases, electron microscopy results showed widespread thinning, splitting, and lamellation of the glomerular basement membrane, which mimicked hereditary nephritis. Throughout adulthood, WT1 protein expresses on glomerular podocytes. Recent reports described that podocytes expressing WT1 play an important role in maintaining the glomerular basement membrane. Hereditary nephritis-like glomerular basement membrane findings in FS suggest that one of the important functions of podocytes is to form and maintain the glomerular basement membrane.

Basement Membrane↗
PubMed2003

The impact of repeated subclinical acute rejection on the progression of chronic allograft nephropathy.

Chronic allograft nephropathy (CAN) is due to both immunologic and non-immunologic factors and results in the development of nonspecific pathologic features that may even be present in long-term well-functioning renal allografts. To investigate the natural history of CAN and potential risk factors associated with progression of these histologic lesions, this study evaluated the of histologic alterations of 124 sequential protocol biopsies performed at 2, 3, and 5 yr after transplantation in 46 patients who exhibited histologic evidence of CAN in the 1-yr biopsy. The occurrence of late acute rejection (AR) greater than 4 mo posttransplant was significantly associated with the development of histologic CAN. In contrast, early clinical AR occurring within 3 mo had no impact on the subsequent development of CAN at 1 yr. Subclinical AR was evident in association with CAN in 50%, 32%, 19%, and 16% of cases with CAN at 1, 2, 3, and 5 yr, respectively. These acute lesions correlated significantly with histologic progression defined as an increased CADI score of the follow-up biopsies. Furthermore, a group of patients who exhibited repeated subclinical AR in the sequential follow-up biopsies had a lower creatinine clearance at 5 yr after transplantation and worse long-term graft survival. In contrast, the absence of evidence of acute inflammation in association with CAN at any time point was associated with minimal deterioration in renal function or progression of renal lesions during the observation period. These results suggest that the persistence of chronic active inflammation may be responsible for the histologic progression of CAN.

Acute Disease↗
PubMed2003

[Peritoneal dialysis catheter-related complications].

UNLABELLED: The introduction of a double-cuff swan neck type catheter has reduced the frequency of peritonitis. The frequency of complications associated with insertion of this catheter has remained unknown. We evaluated these complications in patients aged < 20 years at the start of the chronic peritoneal dialysis using double-cuff swan neck catheters. SUBJECTS AND METHODS: The data from 221 double-cuff swan neck catheters of 126 patients inserted in our hospital between 1990 and 2001 were compared with 102 single-cuff straight catheters of 54 patients between 1982 and 1990. The frequency of catheter-related complications, such as dislocation, leakage with in/outflow malfunction and infection(exit-site/tunnel infection and peritonitis within a month after catheter insertion) were estimated. RESULTS: We observed 37 dislocations(17%), 37 leakages(17%) and 36 infections(16%) of all double-cuff swan neck catheters. Twenty-nine catheters were removed due to catheter-related complications: 18 dislocations(8%), 2 leakages(1%) and 9 infections(4%). Catheter removal due to dislocation occurred significantly more frequently in 12% of children who were > or = 6 years old than in 1% of children < 6 years old(p = 0.002). Eighty-three percent of dislocations could be returned by the whiplash method(alpha-replacer, JMS, Tokyo). Of all single-cuff straight catheters, 10 catheters were removed due to catheter-related complications: 4 dislocations(4%), 6 leakages(6%) and 12 infections(12%). CONCLUSION: A single-cuff straight type catheter was more frequently replaced because of leakage and infection than a double-cuff swan neck type catheter. A double-cuff swan neck catheter was more frequently replaced because of dislocation than a single-cuff straight catheter. When a double-cuff swan neck catheter is inserted particularly in older children, care should be taken to avoid dislocation.

Catheterization↗
PubMed2002

White coat effect and white coat hypertension in pediatric patients.

We evaluated the prevalence of white coat (WC) effect in pediatric age patients and that of white coat hypertension (WCH) in hypertensive pediatric patients. Two hundred and six patients (136 normotensive and 70 hypertensive patients, 107 boys and 99 girls, aged 6-25 years, mean 13.4, SD 4.7) were studied. Hypertension was diagnosed when systolic and/or diastolic blood pressure (BP) measurements with auscultatory technique were >or= the 95th percentile for sex and age. WC effect was defined as office BP minus daytime mean ambulatory BP (ABP). WCH was diagnosed in the hypertensive patients when daytime ABP values were < the 95th percentile for sex and height of reference values. There was a positive correlation between office BP and WC effect ( P<0.05). A WC effect of >or= 10 mmHg was observed more frequently in hypertensive patients (50%) than in normotensive patients (25%). Among 70 hypertensive patients, 33 (47%) had WCH. There was no significant difference in the prevalence of WCH in relation to age, gender, or the presence or absence of causes of hypertension. In conclusion, WC effect is frequently seen in pediatric patients, and is more common in subjects with higher office BP.

Adolescent↗
PubMed2002

The 1998 report of the Japanese National Registry data on pediatric end-stage renal disease patients.

We carried out a nationwide survey on patients less than 20 years of age with pediatric chronic end-stage renal disease (ESRD) in Japan for the year 1998. There were 582 patients who had started on renal replacement therapy before 1998, and 105 patients who had been newly introduced to renal replacement therapy in that year. The prevalence rate of the ESRD patients already on treatment was 22 per million population (aged 0-19 years) in 1998. Older patients had a higher prevalence rate than younger ones. There were 345 patients on dialysis as of 1 January 1998, and 237 patients with transplants. The major diseases causing ESRD were renal hypoplasia/dysplasia and focal segmental glomerulosclerosis. Of the 237 patients (46.9%) who had received renal transplants before 1 January 1998, 262 patients (96%) received their transplants from living kidney donors. The incidence rate for the new ESRD patients was 4 per million population (aged 0-19 years) in 1998. Older patients had a slightly higher incidence rate than younger ones. Peritoneal dialysis was used more frequently than hemodialysis under 15 years (85%-95% and 39% respectively), especially in very young patients. The major diseases causing ESRD were the same as in the patients already on treatment. The transplant rate for the year 1998 was 10 per 100 dialysis patient-years (patients aged 0-19 years) with 9 living kidney donors. The death rate was 15.6 per 1,000 dialysis patient-years (patients aged 0-19 years); the major causes of death being cardiovascular diseases and infections.

Adolescent↗
PubMed2002

Nephrotic syndrome and mizoribine in children.

BACKGROUND: The treatment of nephrotic syndrome (NS) has recently made dramatic progress. The ultimate purpose of treatment is that patients can lead a normal, disease-free life with no adverse effects from treatment. However, clear treatment guidelines remain to be established in the children with frequent relapsing NS (FRNS) and steroid-resistant NS (SRNS). The frequent use or large dose of steroids may lead to a serious adverse effect. Immunosuppressive drugs including cyclophosphamide and cyclosporin have been used to induce lasting remission, thereby sparing patients from further exposure to steroids. However, these drugs have both acute and chronic side-effects. Mizoribine is a relatively safe and effective immunosuppressant. The report discusses the role of mizoribine in FRNS and SRNS in children. METHODS: The papers in experimental and clinical studies about mizoribine in NS were reviewed. Our experiences of mizoribine were also added. RESULTS: Mizoribine has been reported as an effective and safe drug for patients with FRNS. However, the efficacy of mizoribine has differed among various reports, depending on the dose given. There have yet to be any conclusive reports on the effects of mizoribine in SRNS in children. CONCLUSIONS: Better results might be obtained if the doses of mizoribine increased in children with FRNS. When 5 mg/kg was used, no serious adverse effects were seen, therefore this dose may be safe and effective. An investigation of appropriate, effective and safe doses of mizoribine should be examined in the future. In patients with SRNS, large doses of mizoribine of more than 5 mg/kg might be effective, while the combined use of mizoribine and cyclosporin or methylprednisolone pulse therapy might also be even more effective. Further studies are called for to examine the use of large doses of mizoribine.

Animals↗
PubMed2002

Preventive effect of TAK-751S on complications of hemorrhagic colitis (results of clinical study of TAK-751S).

BACKGROUND: The effective therapy for hemolytic uremic syndrome and encephalopathy caused by enterohemorrhagic Escherichia coli have not been established. Great attention has been drawn to the results of the clinical study of TAK-751S, performed in Canada. In Japan, a nationwide clinical study of TAK-751S had been performed since 1997 to investigate the preventive effect on the onset of HUS and the safety. METHODS: TAK-751S was administered in daily doses of 500 mg/kg for one week to 128 pediatric patients with colitis who were suspected of enterohemorrhagic Escherichia coli (EHEC) infection. RESULTS: 1. TAK-751S was confirmed to absorb Shiga toxin (Stx) existing inside the human intestine and to excrete Stx out of the body. 2. The incidence of HUS is 5.9% (4/68) and a tendency to inhibit the onset of HUS was observed as compared with the historical control. The complications of central neuropathy such as encephalopathy were observed in 3 of these patients with HUS. 3. Mild "sweating" and "nausea" were observed. There were 13 mild non-specific abnormalities of laboratory test values in 8 patients. CONCLUSIONS: From these results, it was clarified that TAK-751S absorbed and removed free Stx in the intestinal tract of pediatric patients with EHEC infection. The test drug could not inhibit the onset of HUS completely, but since HUS occurred within 48 hours after the start of administration in 3 of the 4 patients with onset of HUS, TAK-751S is a safe drug for pediatric patients with EHEC infection in which the preventive effect on HUS and encephalopathy are expected when it can be given from an early stage of the diseases. Furthermore, these results suggest that importance of rapid diagnosis of HUS.

Adolescent↗
PubMedDate not supplied

Intranasal mupirocin does not prevent exit-site infections in children receiving peritoneal dialysis.

OBJECTIVE: Exit-site infections (ESI) in patients receiving peritoneal dialysis (PD) often progress to tunnel infections and peritonitis, sometimes requiring PD catheter removal. Staphylococcus aureus (SA) is the commonest cause of ESI. In this study, we evaluated the efficacy of mupirocin nasal ointment in preventing ESI in children receiving PD. DESIGN: A single-center study. SETTING: Tokyo Metropolitan Kiyose Children's Hospital. PATIENTS: 47 outpatients [33 males; age 11.7 +/- 4.9 years (mean +/- SD)] participated in this study between April 1998 and March 1999 at Tokyo Metropolitan Kiyose Children's Hospital. The total study period comprised 399 patient-months. Nasal cultures were performed every month. Nasal carriers of SA applied intranasal mupirocin ointment twice per day for 7 days. We compared the incidence of ESI in the intervention group to 77 historical controls (48 males; age 7.6 +/- 5.1 years); the total control period comprised 2802 patient-months. RESULTS: 32 patients were identified as SA nasal carriers on one or two occasions (32/47 or 68%). The total period of SA nasal carriage was 95 patient-months (95/399 or 24%). The incidence of ESI caused by SA among all ESI cases was 20/28 (71%) in the study group and 115/151 (76%) in the control. There was no significant difference. The incidence of peritonitis caused by SA among all peritonitis cases was 3/6 (50%) in the study group and 17/42 (40%) in the control group, showing no significant difference. There was no significant difference in the overall incidence of ESI, peritonitis, or replacement of PD catheters between the study group and the control group. CONCLUSION: Intranasal mupirocin ointment did not prevent ESI. Further study is needed to develop an effective method of preventing ESI in pediatric PD patients.

Administration, Intranasal↗
PubMedDate not supplied

Suitable dialytic indicators for pediatric peritoneal dialysis patients: the alternative to creatinine clearance.

OBJECTIVE: Owing to the discord between body weight and body surface area (BSA), creatinine clearance (CCr) is predisposed to be small in pediatric patients on peritoneal dialysis (PD). Alternatively, Kt/V creatinine (Kt/V creat), which is normalized to total body water (TBW) rather than BSA, could be a better dialytic indicator. In this study, the efficiency of dialysis and the nutritional status of pediatric patients on chronic PD were examined, and the utility of dialytic indicators was evaluated. PATIENTS AND METHODS: 49 patients under 20 years old, in stable condition, and on PD were analyzed. Weekly total Kt/V of urea (Kt/V urea), CCr, Kt/V creat, and normalized protein equivalent of nitrogen appearance (nPNA) were measured for all patients and for patients under 6 years old. The target value was 2.0/week for Kt/V urea and 60 L/ week/1.73 m2 for CCr, as recommended by the Kidney Disease Outcomes Quality Initiative guidelines. The target value for Kt/V creat was set as 1.52/week, using a male model with a height of 170 cm and a body weight of 65 kg. RESULTS: The mean values of delivered Kt/V urea, CCr, Kt/V creat, and nPNA (and proportion of patients that achieved each target value) for all patients were 2.25 +/- 0.57/ week (67.4%), 53.8 +/- 19.3 L/week1/.73 m2 (26.5%), 1.83 +/- 0.73/ week (65.3%), and 1.11 +/- 0.42 g/day, respectively. The values for patients under 6 years old were 2.38 +/- 0.26/week (90.0%), 45.9 +/- 12.8 L/week/1.73 m2 (10.0%), 1.94 +/- 0.51/week (90.0%), and 1.52 +/- 0.67 g/day, respectively. Stepwise multiple regression analyses revealed that the relationship between CCr and Kt/V urea was affected by the patient's age. CONCLUSIONS: Our pediatric patients achieved the recommended target value of Kt/V urea. At the same time, the nPNA results reflected the patient's status well. However, CCr appeared to be inappropriate as an indicator for patients under 6 years old. Kt/V creat is suggested to be a better dialytic indicator for these patients.

Adolescent↗
PubMedDate not supplied

Cholesterol ester transfer protein in children on peritoneal dialysis.

OBJECTIVES: To examine whether cholesterol ester transfer protein (CETP) activity and mass contribute to dyslipidemia in children on peritoneal dialysis (PD), and to determine whether CETP activity or mass is responsible for severer hyperlipidemia in smaller (younger) patients. STUDY DESIGN: 27 patients (18 males, 9 females; mean age 11.8 +/- 6.1 years) were enrolled. Each patient had been receiving PD for more than 6 months. Fasting blood samples were drawn and CETP activity, CETP mass, total cholesterol, triglyceride, beta-lipoprotein profiles, lipoprotein lipid profiles (cholesterol and triglyceride in lipoproteins), apoprotein profile, and serum albumin levels were measured. The results were then compared, using Student's t-test, with those for a control group. In the patient group, the relationships between CETP activity and each factor were examined using simple and multiple regression analyses. RESULTS: Total cholesterol, triglyceride, low-density lipoprotein (LDL), very low-density lipoprotein (VLDL), chylomicron, and ApoB levels were significantly higher in the patient group. Mean CETP activity levels were 106% +/- 24% in the patient group and 111% +/- 21% in the control group. No significant difference in CETP activity was seen between the two groups, but CETP mass was lower in the patient group than in the control group (2.2 +/- 0.6 microL/dL for the patient group vs 2.8 +/- 0.9 microL/dL for the control group, p = 0.01). As a result, specific CETP activity (activity/protein mass ratio) was significantly higher in the patient group (p < 0.0001). CETP activity was positively related to LDL and other atherogenic factors and negatively related to serum albumin level. No relationship between CETP activity and patient body weight was seen. CONCLUSION: Specific CETP activity was higher in the patient group compared with that in the control group, and strong correlations were found between CETP activity and atherogenic factors in the patient group. Therefore, CETP seems to be associated with lipid abnormalities in children on PD but is not responsible for the severer hyperlipidemia seen in smaller children.

Adolescent↗
PubMedDate not supplied

Terminal complement complexes in childhood type I membranoproliferative glomerulonephritis.

BACKGROUND: The role of terminal complement complexes (TCCs), which are the final products of complement activation, in the pathogenesis of human glomerulonephritis has not been completely elucidated. To clarify the clinical significance of TCCs in type I membranoproliferative glomerulonephritis (MPGN), we studied TCCs in plasma, renal tissue and urine in pediatric patients with this disease. PATIENTS AND METHODS: We measured the concentrations of TCC in plasma (n=25) and urine (n=13) using enzyme-linked immunosorbent assay. Frozen tissue from 18 renal biopsies were evaluated for the presence of TCC by direct immu-noperoxidase staining. RESULTS: At the early stage of the disease, TCC concentrations in plasma were elevated to above 0.5 arbitrary units (AU)/mL in 14 of 25 patients (high-TCC group), while the remaining 11 patients showed less than 0.5 AU/mL (low-TCC group). In the high-TCC group, TCCs were deposited more diffusely and intensely in the glomerulus, compared with those in the low-TCC group (p=0.034). Furthermore, urinary TCC concentrations in the high-TCC group were higher than those in the low-TCC group (p=0.0001). The high-TCC group showed not only a poorer response to steroid treatment, but also poorer prognosis than the low-TCC group. CONCLUSIONS: These results suggest that, in pediatric patients with type I MPGN, TCCs in circulation may play a particular role in TCC formation in the glomerulus and in urine. The TCC concentration in plasma could be used as a marker of responsiveness to steroid treatment and long-term prognosis.

Adolescent↗