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Biomedical subjects

Kensuke Harada

Publications and source records attributed to Kensuke Harada.

At least 19 recordsLinked to original sources

Triggers of relapse in steroid-dependent and frequently relapsing nephrotic syndrome.

An awareness of the triggers of relapse is critical for the control of steroid-dependent, frequently relapsing nephrotic syndrome (SDFRNS). We have investigated the triggers, usually described as 'episodes', to such relapses within a temporal context. Thirty-five patients with SDFRNS were analyzed retrospectively. A total of 442 relapses occurred in 2499 patient-months. The relapses were classified into two groups: those with episodes (E+) and those without episodes (E-). There were 135 E+ relapses and 296 E- relapses. The common cold was the most common episode (52%) of E+ relapse, followed by school events (18%). These E+ relapses occurred almost evenly throughout the 4 weeks between each follow-up visit. Conversely, 161 (55%) of the 296 E-z relapses occurred within the 3-day period preceding the patient's appointment (relapse-related hospital visit, RRHV). McNemar's test revealed that the concentration of relapses in this period was statistically significant (P < 0.00011). In addition, 15 out of 26 RRHV without additional therapy showed a spontaneous remission. From a chronological perspective, the common cold and school events as well as up-coming hospital visits may trigger relapses in SDFRNS patients.

Adolescent↗

B-flow sonographic demonstration for assessing carotid atherosclerosis in young patients with heterozygous familial hypercholesterolemia.

PURPOSE: To determine whether bloodstream swirls detected with B-mode flow (B-flow) sonography at the region of the carotid bifurcation can predict the early progression of atherosclerosis, we prospectively examined the relationships between the size of bloodstream swirls as identified with B-flow sonography, the local mechanical behavior of the common carotid artery (CCA), and some cardiovascular risk factors in young patients with heterozygous familial hypercholesterolemia (FH). METHODS: Thirty-eight young patients (mean age 25.8 +/- 14.1 years) with FH were evaluated for the following parameters: age, sex, body mass index, degree of dyslipidemia, presence of a plaque, intima-media thickness (IMT), elastic modulus (Ep) and internal diameter of the CCA via duplex scan, wall shear stress, maximal diameter of the bloodstream swirl (Sm) at bifurcation, maximal diameter of the CCA (Cm) at the level of the swirl's center, and diameter ratio (Sm/Cm) via B-flow sonography. RESULTS: Bloodstream swirls of varying size at the site of bifurcation were observed in all patients. Six (15.7%) out of 38 patients exhibited plaques at bifurcation. All plaques were located at the far wall and were in contact with the distal portion of the bloodstream swirl as observed with B-flow sonography. Univariate analysis revealed that Sm/Cm and Sm were positively correlated with age, presence of a plaque, IMT, and Ep and were negatively correlated with shear stress. Multivariate analysis revealed that Sm was significantly correlated with age (P < 0.01) and Ep (P < 0.05). CONCLUSIONS: Our results suggest that a bloodstream swirl visualized at the carotid bifurcation with B-flow sonography may be an indicator of a local mechanism for the age-related progression of atherosclerosis in young patients with FH.

Adolescent↗

Cockayne syndrome with recurrent acute tubulointerstitial nephritis.

A 12-year-old girl, who had been diagnosed as having Cockayne syndrome (CS), was admitted for emaciation and dehydration. On admission the patient had mild chronic renal failure (glomerular filtration rate: GFR 50 mL/min) and hyperuricemia. After rehydration, allopurinol was commenced for her hyperuricemia. Then, her renal function rapidly deteriorated (GFR 20 mL/min) with enhancement of proximal tubular dysfunction and hypertension. A renal biopsy showed that the patient had acute tubulointerstitial nephritis (ATIN). Based on this diagnosis, allopurinol was stopped and prednisolone was started (2 mg/kg per day), following which the renal tubular function improved. However, the proteinuria intensified to become nephrotic syndrome. After 1 month the patient developed a gastric ulcer. Famotidine was commenced but GFR deteriorated and renal proximal tubular dysfunction re-occurred. The renal pathology was evaluated by referring to the previous reports of renal pathology in CS. It is suggested that rapid deterioration of the renal function in CS patients might be the result of ATIN. In addition, the present nephrotic syndrome seemed to be accompanied by ATIN, as in other reports.

Acute Disease↗

Morbidity and mortality of infants born at the threshold of viability: ten years' experience in a single neonatal intensive care unit, 1991-2000.

BACKGROUND: The purpose of the present paper was to evaluate the mortality and morbidity of infants born at 22-24 weeks gestation. METHODS: A total of 78 infants born at 22-24 weeks gestation, who were admitted between January 1991 through December 2000, were retrospectively studied. RESULTS: Seventy-one of 78 infants were enrolled in the present study. One year survival rates at 22, 23 and 24 weeks were 40.0% (2/5), 61.1% (11/18), and 50.0% (24/48), respectively. Failure of response to surfactant and air leak were associated with death in infants born at 23 weeks gestation. Low Apgar score, intraventricular hemorrhage (> or =III), and sepsis were correlated with death in infants born at 24 weeks gestation. The handicap rates of survivors born at 22, 23, and 24 weeks gestation were 100, 36.4, and 26.1%, respectively. CONCLUSIONS: The present study indicates that infants born at 22 weeks gestation, in whom pulmonary structure is established, that is, a viable lung that can exchange gas with exogenous surfactant, have a chance to survive, but neurological outcome is still poor. Every possible effort should be made to extend gestation beyond 22 weeks.

Female↗

Prediction of transfusions in extremely low-birthweight infants in the erythropoietin era.

BACKGROUND: The purpose of the present paper was to detect the clinical factors most predictive of red blood cell (RBC) transfusion in extremely low-birthweight (ELBW) infants in the recombinant human erythropoietin era. METHODS: Between 1995 and 2000, 66 ELBW infants were admitted to a level III neonatal intensive care unit. Fifty-four of 66 infants were eligible for enrollment in the present study. Infants were treated with erythropoietin 200 IU/kg per dose s.c. twice a week with 4-6 mg/kg per day iron supplement. RESULTS: The mean gestational age and birthweight were 26.5 +/- 2.1 weeks and 776 +/- 134 g, respectively. Ten of 54 ELBW infants (18.5%) died during the first 21 days. Eight of 10 dead infants (80.0%) and 27 of 44 surviving infants (61.4%) received one or more RBC transfusions. The overall requirement for RBC transfusions in the surviving infants was 3.0 +/- 3.2 per infant/hospital course (range: 0-9) . There were significant differences in gestational weeks, birthweight, initial hemoglobin value, 5 min Apgar score, phlebotomy loss, phlebotomy loss/birthweight, duration of mechanical ventilation, duration of oxygen supplement, and incidence of both intraventricular hemorrhage and chronic lung disease between the transfused and non-transfused group. The predictive variables, initial hemoglobin level (odds ratio [OR] 2.61; 1 g/dL), birthweight (OR 3.00; 100 g), and gestational week (OR 1.89; 1 week), were found to be most predictive for transfusion on logistic regression analysis. CONCLUSION: ELBW infants are still the population at greatest risk for repeated blood transfusions after introduction of erythropoietin treatment. If labor develops, it is often impossible to extend the pregnancy period, therefore efforts should be made to increase hemoglobin level at birth.

Algorithms↗

Charge selective function in childhood glomerular diseases.

The charge selectivity (CS) function in human renal disease has not been unequivocally demonstrated to date. However, the clearance ratio of IgA to IgG may be theoretically useful in estimating CS in humans, since IgA and IgG have similar sizes and tertiary structures, but distinct isoelectric points (3.5-5.5 [IgA] and 4.5-9.0 [IgG]), and Stokes-Einstein radius: 61 A (IgA) and 49-60 A (IgG). Two-dimensional electrophoresis with the following immunoblotting revealed that the considerably anionic portion (isoelectric points [pI] <4.0) of IgA, visible in serum, was absent in the urine in steroid-sensitive nephrotic syndrome (SSNS) but present in the same during IgA nephropathy (IgAN) and membranoproliferative glomerulonephritis (MPGN). A latex assay revealed the CS index (CSI) was significantly low in patients with podocyte disease (group A), including SSNS, focal and segmental glomerulosclerosis (FSGS) and Finnish-type congenital nephrotic syndrome (FCNS), but high in those with Alport syndrome (AS), IgAN, Henoch-Schönlein purpura nephritis (HSPN), and MPGN (group B). The linear regression analysis of the IgA size selectivity index (IgA SSI; clearance ratio of IgA to transferrin) and SSI (clearance ratio of IgG to transferrin), which represents the clearance ratio of IgA to IgG referring to the transferrin clearance, revealed the influence of the charge more accurately. Indeed, the slope of the regression lines of IgA SSI (y) to SSI (x) were concluded to be y = 0.39x (group A) and y = 1.05x (group B), respectively. These results suggested that the charge selective barrier among podocyte diseases (group A) is preserved to some degree, but lost in cases of nephritis and AS (group B).

Adolescent↗

Inhaled nitric oxide therapy might reduce the need for hyperventilation therapy in infants with persistent pulmonary hypertension of the newborn.

AIM: To determine whether inhaled nitric oxide might reduce the need for excessive respiratory alkalosis to maintain systemic oxygenation in infants with persistent pulmonary hypertension of the newborn (PPHN). MATERIALS AND METHODS: A retrospective historical cohort study of 34 infants with PPHN with oxygenation index (OI) of 25 or more, including 19 infants without inhaled nitric oxide (i-NO) therapy (control group) and 15 infants with inhaled nitric oxide therapy (i-NO group) was performed. The initial dose of 10 ppm of i-NO was administered and no responders received the maximum dose of 25 ppm. We evaluated the mortality rate and the change of OI index and PaCO(2) during the first 6 days. RESULTS: There were no significant differences in characteristics between groups. Two of 15 in the i-NO group and 6 of 19 infants in the control group died during the first 48 h. Baseline OI, PaCO(2) and arterial pH were similar in the two groups. OI in the i-NO group was significantly higher than in the control group between 12 and 96 h. PaCO(2) in the i-NO group was higher than in the control group between 24 and 144 h. CONCLUSION: i-NO therapy for PPHN might improve systemic oxygenation without excessive hypocapnia. However there was no reduction in duration of ventilation support or oxygen supply.

Administration, Inhalation↗

Thr-encoding allele homozygosity at codon 54 of FABP 2 gene may be associated with impaired delta 6 desatruase activity and reduced plasma arachidonic acid in obese children.

BACKGROUND: Alanine-for-threonine substitution at codon 54 (A54T polymorphism) in the fatty acid-binding protein 2 gene (FABP2) has been associated with hypertriglyceridemia and insulin resistance. Impairment in the activity of delta 6 and 5 desaturases is also supposed to be a factor predisposing the development of insulin resistance syndrome. AIM: We investigated the relationship between A54T polymorphism in FABP2 and the impairment of long-chain polyunsaturated fatty acid metabolism in obese children. METHODS: Thirty-two obese children participated. During the study, the children continued their habitual diet, which was documented in a 3-day food record using household measures. Anthropometry was performed, and serum lipid and fatty acid composition in plasma were analyzed. The polymorphism of codon 54 in the FABP 2 gene was analyzed. RESULTS: The allele frequency was 0.66 and 0.34 for Ala54 and Thr54, respectively. There were no significant differences in age, body mass index, fasting serum glucose, insulin or serum lipoproteins among the three polymorphism groups. These were also no significant differences in the intake of energy, the percentage of energy nutrients or in the dietary lipid composition. The content of arachidonic acid (AA) in plasma was lowest in Thr/Thr54 (p < 0.05). The indices of delta-6 desaturase (D6D) activity in Thr/Thr54 were significantly lower than in Thr/Ala54 or Ala/Ala54 (p < 0.05, p < 0.01, respectively). CONCLUSIONS: In obese children, Thr/Thr54 of the FABP 2 gene is associated with impaired activation of D6D and reduced AA content. The results in the LCPUFA profile suggest that Thr/Thr54 may predispose the to development of insulin resistance.

Adolescent↗

Determination of lipid composition of plasma lipoproteins in children with a rapid agarose gel electrophoresis method.

The agarose gel electrophoresis and differential staining system is an easy and quick method for analyzing the serum lipid composition of each lipoprotein fraction. It has been reported in adults that measured values obtained by this method strongly correlated with those obtained by ultracentrifugation. The aim of this study was to examine the clinical application of this method for children, in comparison with the ultracentrifugation method. The subjects were sixteen hyperlipidemic and twenty-five normolipidemic children, aged from two to eighteen years old. Cholesterol (C) and triglyceride (TG) levels were determined in serum very low density lipoprotein (VLDL), low density lipoprotein (LDL), and high density lipoprotein (HDL) fractions by both methods. Correlation coefficients between the two methods for cholesterol levels were 0.937 (HDL), 0.983 (LDL) and 0.837 (VLDL), and for triglyceride levels were 0.735 (HDL), 0.621 (LDL) and 0.964 (VLDL). We confirmed the clinical application of this method to evaluate the lipoprotein lipid profile in children as well as in adults.

Adolescent↗

Relationship between serum adiponectin level and lipid composition in each lipoprotein fraction in adolescent children.

The association of lipid composition in each lipoprotein fraction with adiponectin level in relation to body fatness was investigated. The subjects were 283 children (144 boys and 139 girls) aged 11.6 years (S.D. 1.5). Cholesterol (C) and triglyceride (TG) levels in each lipoprotein fraction were measured by a combination of agarose gel electrophoresis and differential staining. Adiponectin level was not significantly different between girls and boys. In simple regression analyses, an association of adiponectin level with lipoprotein lipid profile was shown only in girls, and not in boys at all. In girls, very low-density lipoprotein (VLDL)-C, low-density lipoprotein (LDL)-C, VLDL-TG and LDL-TG were negatively correlated with adiponectin level, and high-density lipoprotein (HDL)-C and HDL-C/TG ratio were positively correlated. Multiple regression analyses including adiponectin level and body mass index (BMI) as predictors for the lipoprotein lipid profile demonstrated that adiponectin level was a significant independent predictor of VLDL-TG, LDL-C, LDL-TG, HDL-C and HDL-C/TG ratio. In conclusion, adiponectin level correlates with lipoprotein lipid profile independent of body fatness in adolescent girls.

Adiponectin↗

A fatal case of ruptured giant coronary artery aneurysm.

A 5-year-old Japanese boy died because of a ruptured left coronary artery aneurysm (CAA). He was diagnosed as having Kawasaki disease (KD) on the 5th day from onset, with all of the principal signs. On the 7th day of illness, bilateral CAAs were already found via echocardiography, and he was treated with intravenous (IV) gamma globulin and oral ASA. However, the fever persisted and the CAA progressed rapidly. Echocardiography on the 12th illness day showed a giant (18-mm) left anterior descending (LAD) artery aneurysm. Oral propranolol and nifedipine were administered, in conjunction with warfarin/aspirin anti-coagulation therapy. On the 13th day of illness, cardiac arrest developed abruptly, and, despite cardiopulmonary resuscitation (CPR), the patient remained unresponsive and died one hour later. The final pathological diagnosis was a ruptured LAD artery aneurysm and cardiac tamponade. Microscopic investigation of the ruptured vascular wall revealed marked neutrophilic infiltration, with fewer macrophages and lymphocytes. CAA ruptures are a very rare, but fatal, complication of KD. Based on a review of previous reports on CAA ruptures, we consider it useful to distinguish aneurysms which rapidly dilate and continue to expand beyond a diameter of 10 mm with ongoing vasculitis (these CAAs can be termed "super-giant") from the more common giant CAAs limited to a diameter of 8 or 9 mm, because a decision must be made as to whether to start intensive care or to intervene surgically, in order to ensure the survival of patients with such a potentially critical complication.

Aneurysm, Ruptured↗

Acute interstitial nephritis predisposed a six-year-old girl to minimal change nephrotic syndrome.

A six-year-old girl was admitted to our hospital with acute renal failure. We made a clinical diagnosis of acute interstitial nephritis and oral corticosteroid therapy was started. Her renal failure soon recovered, and renal biopsy showed acute interstitial nephritis by light microscopy with glomerular foot process effacement by electron microscopy. Although her proteinuria was not heavy at the time of biopsy, her proteinuria subsequently increased to show nephrotic syndrome. We continued to give corticosteroids and her nephrotic syndrome went into remission 13 days after biopsy. Serological and bacteriological examination showed no evidence of known pathogen or drug hypersensitivity. The time changes in proteinuria were monitored by fractional total protein excretion (FETP) and fractional beta2 microglobulin excretion (FEbeta2MG) in order to evaluate the severity of proteinuria under different glomerular filtration rates and different proximal tubular functions. The results revealed that nephrotic syndrome had occurred during recovery from acute interstitial nephritis. This is the first case report to show the sequential occurrence of acute interstitial nephritis and nephrotic syndrome based on evidence from fractional protein excretion.

Acute Disease↗