Browsing by Author "Tsau Y.-K."

Background: Acute lobar nephronia (ALN) is a severe nonliquefactive inflammatory renal bacterial infection, and requires a longer duration of treatment. The aim of this prospective study was to investigate renal scarring after ALN and to examine the risk factors for renal scarring in children with ALN compared with those with acute pyelonephritis (APN). Methods: Patients with computed tomography-diagnosed ALN were enrolled and randomly allocated, with serial entry, to either a 2-or 3-week antibiotic treatment regimen. Age-and gender-matched APN patients served as comparators. Patients underwent dimercaptosuccinic acid scintigraphy at least 6 months later to assess renal scarring. Results: A total of 218 children (109 ALN, 109 APN) were enrolled. The incidence of renal scarring was similar between 2-and 3-week treatment groups and was higher in ALN patients than in APN patients (89.0% vs. 34.9%, P < 0.001). Renal scarring was prone to occur in children with higher inflammatory indices and longer duration of fever before and after treatment. Multiple regression analysis on independent variables showed that only ALN was significantly associated with a higher incidence of renal scarring. Conclusions: Our results showed a new finding that ALN is associated with a very high incidence of renal scarring, in comparison to APN, irrespective of the duration of antibiotic treatment. ? 2010 by Lippincott Williams & Wilkins.

Background/Purpose: This aim of this study was to assess the clinical manifestations, the microorganisms involved and their antibiotic resistance in children hospitalized due to acute lobar nephronia (ALN) and non-ALN community-acquired urinary tract infections (UTIs). Methods: We retrospectively reviewed the records of 265 previously healthy children hospitalized due to a first-episode of community-acquired febrile UTI between July 2004 and June 2007. Based on the results of renal ultrasonography and computed tomography, they were divided into ALN and non-ALN groups. Their demographic and clinical characteristics, distribution of microorganisms, and their antimicrobial resistance were analyzed. Results: Of the total number of cases of children admitted with a first-episode community-acquired UTI, 19.2% (n=51) were diagnosed as ALN. Children with ALN were older (1.86 years vs. 0.81 years; p < 0.01), had longer periods of fever before admission (4.7 days vs. 1.4 days; p < 0.01), higher peak body temperatures (39.5°C vs. 38.9°C; p < 0.01), higher white cell counts (18.86 × 109/L vs. 15.08 × 109/L; p < 0.01) and higher C-reactive protein levels (9.0 mg/dL vs. 3.5 mg/dL; p < 0.01) compared with non-ALN children. Fever also persisted for longer after the start of antibiotic treatment in the ALN children (2.7 days vs. 1.4 days: p < 0.01) and they required longer hospital stays and incurred higher medical costs. The major pathogen found in ALN was E. coli (90%). The E. coli isolated from ALN children was more resistant to cotrimoxazole and ciprofloxacin than those from non-ALN children. Conclusion: ALN is not uncommon in children with a first-episode febrile UTI. They have a prolonged clinical course, higher inflammatory parameters, longer hospital stays and incur higher medical costs. E. coli is the major pathogen isolated from these children. ? 2010 Taiwan Society of Microbiology.

Background: Intermittent hemodialysis (IHD) is the most efficient form of renal replacement therapy (RRT) for removing toxic substances from patients' bodies. However, the efficacy and safety of IHD in infants and young children with inborn errors of metabolism are still not clear. Methods: This retrospective study included patients with urea cycle disorders, maple syrup urine disease, and methylmalonic acidemia who received IHD or non-IHD RRT at our hospital between 2001 and 2012 to remove ammonia, leucine, or methylmalonic acid. Both the efficacy and safety of the RRT were evaluated. Results: Thirty-five courses of RRT, including 25 courses of IHD and ten courses of non-IHD RRT, for 15 patients were included in the analysis. Before 2006, non-IHD RRT procedures, including peritoneal dialysis (PD) and continuous venous-venous hemofiltration (CVVH), were the most often used; from 2006 onwards IHD was used. There was one procedure-unrelated death. Catheter penetration occurred in one course of IHD. The efficacy data revealed that both the median duration of dialysis and the median 50 % toxin reduction time were shorter in IHD than in non-IHD RRT. Conclusions: In infants and young children with inborn errors of metabolism, IHD is safe and more efficient than non-IHD RRT at removing toxins. ? 2013 IPNA.

Epidermal nevus syndrome is seldom encountered, and its association with hypermelanosis and the chronic syndrome of inappropriate antidiuretic hormone secretion (SIADH) has never been reported. A male neonate who developed intractable seizures and hyponatremia soon after birth is reported. He had alopecic patches on the scalp at birth. Large areas of skin hyperpigmentation, and epidermal nevi developed gradually. The clinical picture of hypotonic hyponatremia, high urine osmolality, elevated urine sodium, and euvolemia was compatible with SIADH. The seizures did not correlate with the hyponatremia, and no other cause for the seizures could be identified. The hyponatremia became chronic and was treated with a direct supply of sodium chloride. The development of the patient was markedly delayed at the last visit when he was 1 year of age. It is suggested that hypermelanosis and chronic SIADH may also be a variant presentation of epidermal nevus syndrome. (C) 2000 by Elsevier Science Inc.

This retrospective case-control study compared the tolerability and efficacy of linezolid between patients with and without renal insufficiency (serum creatinine ?1.3 mg/dL for women and ?1.5 mg/dL for men). All patients with Gram-positive infections treated with linezolid for more than 7 days were included. Data were collected from medical charts and differences between patients with and without renal insufficiency were analysed. Sixty-two patients (40 men), with a mean age of 56.9 years, were enrolled in this study. At the start of linezolid treatment, 17 patients (27.4%) had impaired renal function. Patients with renal insufficiency had a higher prevalence of diabetes mellitus compared with those with normal renal function (64.7% vs. 22.2%; P = 0.002). At the start of therapy, patients with renal impairment had a higher frequency of elevated blood urea nitrogen (51.0 ± 21.1 mg/dL vs. 18.3 ± 9.7 mg/dL; P < 0.001), elevated serum creatinine (2.3 ± 0.7 mg/dL vs. 0.9 ± 0.3 mg/dL; P < 0.001) and decreased initial haemoglobin (9.2 ± 1.5 g/dL vs. 10.4 ± 1.7 g/dL; P = 0.017). Development of severe thrombocytopenia (<100 × 109/L) was significantly more common in patients with renal insufficiency (64.7% vs. 35.6%; P = 0.039). The incidence of linezolid-associated thrombocytopenia was higher among patients with renal insufficiency. When patients with renal insufficiency are treated with linezolid for more than 2 weeks, the platelet count should be monitored at least twice a week owing to the increased likelihood of thrombocytopenia. ? 2006 Elsevier B.V. and the International Society of Chemotherapy.

Background/Aim: Renal replacement therapies (RRTs) have been used for the acute management of inborn errors of metabolism. Hemodialysis is the most effective modality. The aim of this article is to demonstrate that high-volume hemofiltration can offer an alternative way to effectively remove small molecules. Methods: Eight patients presented with acute neurological deterioration due to ammonia or organic acid accumulation. Different RRTs were applied, including continuous venovenous hemofiltration (CVVH, n = 7), continuous arteriovenous hemofiltration (CAVH, n = 2), continuous venovenous hemodialysis (CVVHD, n = 1), intermittent hemodialysis (HD, n = 1), and peritoneal dialysis (PD, n = 2). Results: Ammonia 50% reduction time in HD was 1.7 h while in CVVH it was 2-14.5 h. The greater the ultrafiltration flow was, the sooner patients regained consciousness. CAVH, CVVHD or PD was not sufficient enough. Conclusion: CVVH also has a good clearance for organic acid and ammonia if applying high-volume hemofiltration (>35 ml/kg/h). It can be therefore be considered as an alternative therapy if infant HD is not available. Copyright ? 2007 S. Karger AG.

Childhood nephrotic syndrome is mainly caused by minimal change disease which is named because only subtle ultrastructural alteration could be observed at electron microscopic level in the pathological kidney. Glomerular podocytes are presumed to be the target cells whose protein sieving capability is compromised by a yet unidentified permeability perturbing factor. In a cohort of children with non-hereditary idiopathic nephrotic syndrome, we found the complement fragment C5a was elevated in their sera during active disease. Administration of recombinant C5a induced profound proteinuria and minimal change nephrotic syndrome in mice. Purified glomerular endothelial cells, instead of podocytes, were demonstrated to be responsible for the proteinuric effect elicited by C5a. Further studies depicted a signaling pathway involving Rho/Rho-associated kinase/myosin activation leading to endothelial cell contraction and cell adhesion complex breakdown. Significantly, application of Rho-associated kinase inhibitor, Y27632, prevented the protein leaking effects observed in both C5a-treated purified endothelial cells and mice. Taken together, our study identifies a previously unknown mechanism underlying nephrotic syndrome and provides a new insight toward identifying Rho-associated kinase inhibition as an alternative therapeutic option for nephrotic syndrome. ? 2015 Springer Basel.

Background: We previously showed a positive correlation between nephromegaly and plasma hepatocyte growth factor (HGF)/transforming growth factor β1 (TGF-β1) ratio in children with biliary atresia. The purpose of this study is to examine the possible reversibility of nephromegaly in patients with biliary atresia. Methods: We evaluated kidney volume in 13 patients with biliary atresia before and after liver transplantation, 6 patients with hepatoblastoma, and 26 healthy children. Plasma HGF and TGF-β1 levels were determined for all children. Results: We noted significant nephromegaly in children with biliary atresia before liver transplantation compared with healthy children and children after liver transplantation (P < 0.001 and P = 0.006 for intercepts, P = 0.064 and P = 0.753 for slopes by analysis of covariance, respectively). The highest plasma HGF levels and HGF/TGF-β1 ratios and the lowest TGF-β1 concentrations were found in children with biliary atresia before liver transplantation (P < 0.001). No statistically significant nephromegaly was observed in children with biliary atresia after liver transplantation or those with hepatoblastoma despite the presence of a mildly increased plasma HGF level and HGF/TGF-β1 ratio. Plasma HGF/TGF-β1 ratio correlated positively with degree of nephromegaly in all patients (r = 0.717; P < 0.001). Conclusion: Our data suggest that liver transplantation reverses the nephromegaly present in children with biliary atresia and that plasma HGF/TGF-β1 ratio may be associated with the development of nephromegaly in patients with biliary atresia. ? 2005 by the National Kidney Foundation, Inc.

Severe muscle weakness in Fanconi's syndrome is rarely the result of mitochondrial cytopathy. We describe a rare case of a 9-year-old male with early onset of Fanconi's syndrome. He developed severe proximal muscle weakness exacerbated by hypokalemia and hypophosphatemia in childhood. The muscle biopsy revealed increased accumulation of abnormal mitochondria and fat droplets in histochemical stains and electron microscopy. Mitochondrial cytopathy cannot be excluded in Fanconi's syndrome with late onset of muscular impairment. Long-term follow-up of his clinical course is suggested to understand the natural history of this unusual case. Copyright (C) 2000 Elsevier Science Inc.

To measure the kidney size in children with Kawasaki disease (KD) and to delineate the condition of nephromegaly, 20 children with KD were enrolled in our study. Kidney sizes were measured during acute stage in these patients. Twenty healthy children and 15 febrile children served as healthy controls and fever controls, respectively. To delineate the possible mechanism, we also evaluated kidney volume (KV) in 13 other patients with KD during the acute phase and after the recovery phase as well as 26 healthy children. Plasma hepatocyte growth factor (HGF) and transforming growth factor-β1 (TGF-β1) levels were determined for all children. Kidney lengths and KV in patients with KD during the acute phase were significantly larger than those of healthy children. There was no kidney enlargement in healthy controls and after the recovery phase. The ratio of patient plasma HGF/TGF-β1 during the acute phase and after the recovery phase correlated positively with the degree of nephromegaly in all patients. These results confirm the presence of large kidneys in children with KD. Our data also suggest that an elevated HGF/TGF-β1 ratio may be responsible for the transient nephromegaly in these children. ? International Pediatrics Research Foundation, Inc. 2008. All Rights Reserved.

To demonstrate nephromegaly in children with biliary atresia and children with compensatory renal hypertrophy and to examine their plasma hepatocyte growth factor (HGF), transforming growth factor β1 (TGF-β1), and the difference of total kidney volume, 11 children with biliary atresia (age range 5 months to 10 years), 11 with compensatory renal hypertrophy, and 11 age-matched healthy controls were investigated. Kidney volume was measured by renal ultrasonography and plasma HGF and TGF-β1 levels were studied. To clarify the significance of nephromegaly in biliary atresia, creatinine clearance was also measured in 9 children with biliary atresia and 9 healthy children. The unilateral kidney in biliary atresia and the solitary kidney in compensatory renal hypertrophy had significantly higher kidney volumes compared with those of healthy children (P<0.001 by analysis of covariance). However, a significant increase in total kidney volume was noted only in children with biliary atresia (P<0.001 by analysis of covariance). Although this was actually associated with increased creatinine clearance (117.3±22.0 ml/min per 1.73 m2 vs. 98.3±13.6 ml/min per 1.73 m2 in controls, P<0.05), corrected creatinine clearance was not correlated with total kidney volume (r=0.199, P=0.61) in biliary atresia. Plasma HGF levels and HGF/TGF-β1 ratios were elevated in children with biliary atresia (2,648±1,215 pg/ml and 233.8±139.1 pg/ng vs. 493±131 pg/ml and 35.9± 15.7 pg/ng in compensatory renal hypertrophy and 468±194 pg/ml and 24.0±19.6 pg/ng in controls, P<0.001) and had a positive correlation with total kidney volume by multiple regression analysis (P=0.006 and P=0.002, respectively). These results show that nephromegaly in biliary atresia is associated with increased total kidney volume and a higher glomerular filtration rate, and is positively correlated with plasma HGF and plasma HGF/TGF-β1 ratio, implying a role of HGF in this situation. However, nephromegaly in compensatory renal hypertrophy may have different mechanisms in terms of normal total kidney volume, transient elevation of plasma HGF followed by normal plasma HGF, and normal plasma HGF/TGF-β1 ratio. These data also suggest a common mechanism (HGF) for initial renal hypertrophy (as in compensatory renal growth), with dysregulation of control of this process later in the course (as in biliary atresia). The detailed mechanisms for nephromegaly in these two conditions should be further clarified.

Background: Hemolytic uremic syndrome (HUS) is an uncommon cause of acute renal failure in children. In contrast to Western countries, most HUSs in Taiwan are caused by Streptococcus pneumoniae. In this article, we demonstrate the clinical courses of children with HUS in Taiwan and try to explain the pathophysiology of complications. Methods: We retrospectively reviewed the medical records of children with HUS who were admitted to the Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan, between January 1997 and February 2008. The clinical courses, laboratory data, complications, and prognosis were recorded. We divided the patients into dialysis group and non-dialysis group. The laboratory data for the two groups were expressed as mean, standard deviation, and range. Results: Nine patients were enrolled in the study, and all were diagnosed with S pneumoniae-associated HUS. One patient had meningitis, and the others had pneumonia. The mean durations of anemia and thrombocytopenia were 19.2 days and 10.2 days, respectively. Five patients received renal replacement therapy because of oligouria. The average of the peak total bilirubin levels of patients in the dialysis and non-dialysis groups were 24.6 ± 20.7 mg/dL and 3.8 ± 1.9 mg/dL, respectively. In addition to one patient who had meningitis, four other patients experienced central nervous system (CNS) complications. The mean durations of hypertension in five patients with CNS manifestations and four patients without CNS manifestations were 16.8 ± 7.8 days and 4.8 ± 6.6 days, respectively. Two patients died in acute stage, and most of the others had regained normal renal function at discharge or during follow-up. Conclusion: (1) Streptococcus pneumoniae is the most common causative pathogen of HUS in Taiwan; (2) the mean duration of hypertension in patients with CNS manifestations was much longer than that in patients without CNS manifestations; and (3) the average of the peak total bilirubin levels of patients in the dialysis group was much higher than that in the patients of the non-dialysis group. ? 2011, Taiwan Pediatric Association. Published by Elsevier Taiwan LLC. All rights reserved.

We report on a 12-year-old female patient with lipoprotein glomerulopathy (LPG) who was proven to be heterozygous for ApoE2 Kyoto (Arg25Cys). Her family members have the same variant but do not have obvious signs of renal function impairment. Six months of treatment with a statin caused significant clinical improvement in the lipid profile, proteinuria, and renal function. Our case suggests that administration of a statin is a potential therapeutic strategy for improving nephrotic syndrome in patients with LPG. ? 2012 Dustri-Verlag Dr. K. Feistle.

Objectives: To assess the consequences of renal ultrasonography (RUS) in the treatment of children younger than 5 years of age with a first febrile urinary tract infection. Methods: We retrospectively reviewed the results of imaging studies, including RUS, computed tomography, and voiding cystourethrography in children with a first febrile urinary tract infection during a 2-year period. Children with known urologic anomalies, other underlying diseases, or simultaneous combined illnesses were excluded. Children with nephromegaly were diagnosed with acute lobar nephronia by computed tomography. Results: A total of 390 children were included in this study. Of the 390 children, 112 (28.7%) had abnormal RUS findings. The children with abnormal RUS findings of nephromegaly, small kidney, intermittent hydronephrosis, or a double collecting system had a significantly greater incidence of vesicoureteral reflux than children with normal RUS findings. Additionally, the occurrence of high-grade vesicoureteral reflux in children with abnormal RUS findings was more frequent than in children with normal RUS findings. Conclusions: The results of our study indicate that it is worth performing RUS in children with a first febrile urinary tract infection because abnormal kidney size or other specific structural ultrasound findings should be investigated, in addition to isolated hydronephrosis. ? 2008 Elsevier Inc. All rights reserved.

Background/Purpose: Transient pseudohypoparathyroidism is a rare cause of late-onset hypocalcemia in neonates and infants. The purpose of this study was to investigate the clinical presentation and natural course of transient pseudohypoparathyroidism in neonates and infants. Methods: From 1995 to 2006, 21 patients under 3 months of age were admitted to our department because of late-onset neonatal hypocalcemia. Among these, five were noted to have transient hypocalcemia, hyperphosphatemia and elevated serum parathyroid hormone levels. Their clinical data, biochemical findings and natural course were thoroughly analyzed. Results: All five patients were boys with increased neuromuscular irritability as their initial clinical manifestation. Initial biochemical data showed calcium 1.5 ± 0.16 mmol/L, phosphorus 9.6 ± 1.5 mg/dL, intact parathyroid hormone 182 ± 93 pg/mL and tubular reabsorption of phosphorus 94.8 ± 3.7%. Two of the patients had magnesium deficiency. After reduction of phosphorus intake and supplementation with calcium and/or magnesium as indicated, the biochemical derangements resolved in 28 ± 3 days. Conclusion: Neuromuscular irritability is usually the initial clinical presentation of transient pseudohypoparathyroidism. Aside from delayed renal maturation, pseudohypoparathyroidism is also caused by magnesium. deficiency. Such a disturbance usually resolves before 3 months of age. ? 2008 Elsevier & Formosan Medical Association.

Aim: Children with steroid-dependent nephrotic syndrome (SDNS) need long-term steroid usage to maintain sustained remission. Cyclophosphamide is a well-known alternative agent to spare the use of steroids and avoid the side-effects that result from long-term steroid therapy. Most children may continue to have SDNS despite receiving cyclophosphamide. Additional alternative drugs may be needed. In the present study, the effects on SDNS of sequential treatment after cyclophosphamide usage were established. Methods: Forty-six children with SDNS were enrolled in this retrospective uncontrolled study. In addition to prednisolone, patients were treated with cyclophosphamide as a first-line alternative drug. Children who still had SDNS despite cyclophosphamide therapy received chlorambucil, levamisole or another course of cyclophosphamide. The treatment responses were recorded and the mean duration of follow up was 96 months. Results: Seventeen patients (37%) experienced no relapse after cyclophosphamide therapy. Twenty-five patients (54%) had varied responses. Only four patients showed no effect. Children who still had SDNS despite cyclophosphamide therapy received second or more alternative drugs. Cyclophosphamide with or without chlorambucil resolved steroid-dependency in 33 of 46 (72%) children who either had complete remission or developed steroid-sensitive, rather than steroid-dependent, nephrotic syndrome. Conclusion: With the exception of four patients who were lost to follow up and four who were refractory and needed other treatment, most children with SDNS could spare the steroid (complete remission or steroid sensitive nephrotic syndrome) after using one or more of these modulating agents. ? 2010 Asian Pacific Society of Nephrology.