Effect of peritoneal dialysate on bioelectrical impedance analysis variability in pediatric patients receiving peritoneal dialysis.

BACKGROUND: Previous adult studies have yielded conflicting results regarding whether the presence (D +) or absence (D-) of peritoneal dialysate affects the accuracy of bioelectrical impedance analysis (BIA) measurements. The aim of this study was to investigate whether the accuracy of BIA data varies between D + and D- measurements in children. METHODS: This cross-sectional study recruited chronic kidney disease stage 5 patients aged 3 to 18 years who received peritoneal dialysis. Body composition was assessed by multifrequency BIA, and values were compared between D + and D- measurements using the intraclass correlation coefficient (ICC). RESULTS: Fifty paired BIA measurements were collected from 18 patients with a mean age of 13.6 ± 4.1 years and a mean dialysate fill volume of 1,006 ± 239.7 ml/m2. Sixteen out of 17 BIA parameters (94.1%) exhibited excellent correlations between D + and D- measurements (ICC values = 0.954, 0.998). There was a trend of increased fluid status, including extracellular water, edema index, and overhydration, in D + measurements, with mean differences (95% CIs) of 0.5 (0.4, 0.6) L, 0.002 (0.001, 0.002), and 0.1 (0.1, 0.2) L, respectively. Soft lean mass and fat-free mass were higher in D + measurements, with mean differences (95% CIs) of 1.4 (1.2, 1.6), and 1.6 (1.4, 1.8) kg, respectively. In addition, patients older than 10 years had a stronger correlation between D + and D- measurements than younger patients. CONCLUSIONS: A total of 94.1% of BIA parameters exhibited excellent correlations between D + and D- measurements, especially patients older than 10 years. We recommend that BIA measurements be collected from children regardless of the presence of peritoneal dialysate.

Dysnatremia and subsequent sodium level changes following various intravenous treatments in infants with acute gastroenteritis.

Acute gastroenteritis is one of the main causes of electrolyte imbalance in infants. We aimed to determine the frequency of and factors associated with dysnatremia at presentation and establish the ideal intravenous treatment scheme. The records of hospitalized infants aged 1-12 months with community-acquired acute gastroenteritis between January 2017 and March 2021 were retrospectively reviewed. Factors associated with dysnatremia at presentation were analyzed by multivariable logistic regression analysis. Subsequent sodium levels 4-24 h after intravenous fluid treatments, which were categorized into 2 groups, were determined in the subgroup of infants with normal sodium levels at presentation. A total of 347 infants with a median age of 8.0 (5.0-10.0) months were included. The frequency of dysnatremia at presentation was 14% (hyponatremia 12% and hypernatremia 2.0%). Severe dehydration was associated with dysnatremia at presentation (p = 0.048). Among 68 infants with normal sodium levels at presentation, the median sodium change was highest in the 5% dextrose in saline group, with changes of + 3 (0.5-5) and + 1 (- 2 to 2) mmol/L in infants who received 5% dextrose in saline and 5% dextrose in 1/3-1/2 saline, respectively (p = 0.001). Four out of 47 infants (8.5%) developed hyponatremia while receiving 5% dextrose in 1/3-1/2 saline. None of those who received 5% dextrose in saline developed subsequent dysnatremia.   Conclusion: The frequency of dysnatremia at presentation among infants with acute gastroenteritis was 14%. Severe dehydration was associated with dysnatremia at presentation, so electrolyte levels need to be assessed in these patients. The use of isotonic solution did not promote acquired dysnatremia. This study supports once more that current guidelines recommending isotonic solution for children, and, especially, infant rehydration, are important also for infants in Thailand. What is Known: • There were a wide variation in the incidence of dysnatremia at presentation in children with acute gastroenteritis in previous pediatric series. • The AAP guidelines recommend using isotonic solution in children with acute illness from 28 days to 18 years of age to prevent acquired hyponatremia. What is New: • The incidence of dysnatremia at presentation in infants with acute gastroenteritis was 14% (hyponatremia 12% and hypernatremia 2.0%). • The use of isotonic solution did not promote acquired dysnatremia in infants with acute gastroenteritis.

Model for predicting high-grade vesicoureteral reflux in young children presenting with febrile urinary tract infection.

INTRODUCTION: High-grade vesicoureteral reflux (VUR) is associated with long-term renal outcome, and can be missed if voiding cystourethrogram (VCUG) is not performed. The current febrile urinary tract infection (UTI) guidelines have narrowed down the indications for VCUG. This study aimed to determine the predictors and develop a model to predict high-grade VUR (grade III-V) in young children presenting with febrile UTI. METHODS: We retrospectively reviewed 324 children aged 2-60 months who presented with first or recurrent febrile UTI and underwent both renal and bladder ultrasonography (RBUS) and VCUG during 2004-2013. Multivariate logistic regression was used to identify independent predictors of high-grade VUR, and a predictive model and scoring system were developed. Sensitivity and specificity of the predictors were evaluated. RESULTS: Sixty-two (67.4%) of 92 children with VUR were high-grade VUR. The independent risk factors for high-grade VUR were recurrent UTI (p < 0.001), non-Escherichia coli (non-E. coli) pathogen (p = 0.011), and abnormal RBUS (p < 0.001) including pelvicalyceal dilation only (p = 0.003), and with ureteric dilation (p < 0.001). Abnormal RBUS showed highest sensitivity (58.1%), while ureteric dilation had highest specificity (96.6%) to predict high-grade VUR. Combination of these predictors improved the specificity but decreased the sensitivity. The prediction score was calculated, as follows: recurrent UTI = 3 points + non-E. coli = 1 point + pelvicalyceal dilation only = 1 point + pelvicalyceal and ureteric dilation = 3 point. A cutoff score of 1 showed sensitivity of 82.3% and specificity of 63.7%. The specificity would increase (87.8, 92.7, 97.7, 100, 100, and 100%) for higher scores of 2, 3, 4, 5, 6 and 7, respectively. DISCUSSION: High-grade VUR could be predicted by simple model using single or combined predictors. Sensitivity and specificity varied according to each predictor. The specificity of ureteral dilation was as high as recurrent UTI. The weighing score for each presenting predictor and the summation of the scores provided more detailed in sensitivity and specificity. CONCLUSIONS: Children with febrile UTI who have the predictor(s) of high-grade VUR are recommended to undergo VCUG. The high-grade VUR prediction score may help for decision-making regarding whether to perform VCUG in a particular child.

Screening and management of bladder and bowel dysfunction in general pediatric outpatient clinic: a prospective observational study.

BACKGROUND: Bladder and bowel dysfunction (BBD) is a common disorder in children that is often associated with psychosocial and behavioral problems. Data specific to BBD in Asian children are comparatively scarce. Accordingly, this study aimed to investigate the prevalence of BBD and the response to standard urotherapy among Thai children attending the general pediatric outpatient clinic of Siriraj Hospital - Thailand's largest national tertiary referral center.  METHODS: Children aged 4-12 years were recruited to complete the Dysfunctional Voiding Symptom Score (DVSS) questionnaire to screen for BBD during 2018 to 2020. Standard urotherapy, which consists of education and behavior management, was prescribed to those with a DVSS score that suggests the presence of BBD. Enrolled children and their caregivers were followed-up at 3 and 6 months. The Strengths and Difficulties Questionnaire (SDQ) was completed at baseline. DVSS scores at baseline, and at 3 months and 6 months after standard urotherapy were compared using repeated measures analysis of variance (ANOVA). RESULTS: A total of 1,042 children completed the DVSS during the study period, and 90 (8.6%) were deemed to have BBD. The mean age of BBD children was 6.9 ± 2.2 years, and the female to male ratio was 2.9:1. The most common symptoms were defecation frequency (80.0%), difficult defecation (80.0%), curtsying (74.4%), urgency (68.9%), infrequent voiding (43.3%), and daytime incontinence (32.2%). Forty-one BBD children completed the SDQ, and 32.5% had scores suggestive of hyperactivity problems. Among the 24 BBD patients who were followed-up after 3 and 6 months of standard urotherapy, there was a significant improvement in DVSS results (9.5 ± 3.1 at baseline vs. 6.9 ± 3.4 at 3 months vs. 4.4 ± 3.9 at 6 months; p < 0.01). Nine of 12 patients with urinary incontinence showed complete response at 6 months. The overall Bristol stool score significantly improved from 2.6 ± 0.7 at baseline to 3.2 ± 1.0 at 6 months (p = 0.03). CONCLUSIONS: BBD is a prevalent condition among Thai children that is often associated with emotional and behavioral problems. Standard urotherapy prescribed in a general pediatric outpatient clinic setting yielded favorable outcomes in Thai children with BBD.

Prevalence of cognitive dysfunction and its risk factors in children with chronic kidney disease in a developing country.

BACKGROUND: Chronic kidney disease (CKD) is associated with cognitive dysfunction. Cognitive function in children with CKD residing in developing countries has not been previously reported. METHODS: This cross-sectional study included children aged 6-18 years with CKD stages 2-5D and kidney transplant. Cognitive function was assessed by WISC-V for children from 6-16 years of age. In adolescents 17-18 years of age, WAIS-III was used. Factors associated with cognitive dysfunction were identified using multivariable regression analysis. RESULTS: Thirty-seven children with median age 13.9 (11.3-15.7) years were recruited. The median full-scale intelligence quotient (FSIQ) was 83.0 (71.0-95.0). Below-average cognitive function (FSIQ <90) was identified in 24 children (64.8%), 24.3% of whom had cognitive impairment (FSIQ <70). Most children (94.6%) scored lower than average on at least 1 cognitive domain. Kidney replacement therapy (p = 0.03) and low family income (p = 0.02) were associated with below-average cognitive function in multivariable logistic regression analysis. Children who left school and low family income were significantly associated with cognitive function. The FSIQ of children who had left school was 12.94 points lower than the educated group (p = 0.046). In addition, every 10,000 Thai Baht (approximately 330 United States dollars) increase in family income correlated with 1.58 increase in FSIQ (p = 0.047). CONCLUSION: Cognitive dysfunction was commonly found in children with CKD. Socioeconomic factors, particularly school attendance and family income, were associated with cognitive dysfunction. Cognitive evaluation is suggested for children with CKD who have socioeconomic risk factors. "A higher resolution version of the Graphical abstract is available as Supplementary information."

Acute kidney injury without previous renal disease in critical care unit.

BACKGROUND: Acute kidney injury (AKI) is common in hospitalized and critically ill children. Apart from primary kidney disease, etiologies of AKI are usually related to systemic disease and nephrotoxic insult. This study examines the incidence, characteristics, and mortality risks of AKI in critically ill children without primary renal disease or previously known chronic kidney disease. METHODS: A retrospective cohort study was conducted of patients aged 1-18 years, diagnosed with AKI (excluding severe glomerulonephritis and previously known chronic kidney disease) in pediatric intensive care units between 2013 and 2016. Acute kidney injury was defined according to the Kidney Disease Improving Global Outcomes classifications. Cox proportional hazards regression analysis was employed to assess the relationship between the risk factors and mortality. RESULTS: Of 1,377 pediatric intensive care unit patients, 253 (18.4%) developed AKI and only 169 (12.3%) who did not have previously known renal disease were included. Of these 169 AKI patients, the mean age was 8.1 ± 4.7 years; 88 (52.1%) patients were male; and 60 (35.5%) patients had AKI stage 3. The most common etiologies of AKI were sepsis (76.9%) and shock (64.5%). Fifty-three (31.4%) of those patients died during admission. The risk factors for death were the need for mechanical ventilation (adjusted hazard ratio, 17.82; 95% CI, 2.41-132.06) and AKI stage 3 (adjusted hazard ratio, 2.32; 95% CI, 1.07-5.00). CONCLUSIONS: Acute kidney injury in critically ill children without previously known renal disease was approximately two-thirds of the overall incidence. The risk factors of in-hospital death were the use of mechanical ventilation, and AKI stage 3.

Renal outcome and risk factors for end-stage renal disease in pediatric rapidly progressive glomerulonephritis.

BACKGROUND: Rapidly progressive glomerulonephritis (RPGN), defined as acute nephritic syndrome with dramatic loss of renal function within a few months, is associated with crescentic glomerulonephritis (CresGN), which requires ≥50% crescents on pathology. The disease characteristics and renal outcome in children with RPGN, however, will differ according to the percentage of crescents. METHODS: To evaluate the renal outcomes and factors associated with end-stage renal disease (ESRD), this retrospective cohort study assessed children aged ≤15 years with RPGN at a tertiary medical center. RESULTS: Of 67 patients with RPGN, 32 (47.8%) were male; mean age was 10.6 ± 3.0 years; median follow up was 1.1 years (range, 0.02-9.17 years) and 24 (35.8%) progressed to ESRD. Post-infectious glomerulonephritis was the most frequent cause of RPGN (50.7%). The incidence of ESRD was significantly higher in patients with ≥50% than <50% crescents (57.9% vs 28.6%, P = 0.029). On Cox regression analysis, disease etiology, serum creatinine >3 mg/dL, need for acute dialysis, ≥80% crescents and ≥20% tubular atrophy and interstitial fibrosis (TA/IF) were associated with ESRD. On multivariate analysis, need for acute dialysis (HR, 2.8; 95% CI: 1.1-7.3, P = 0.041) and ≥20% TA/IF (HR, 4.8; 95% CI: 1.4-16.1, P = 0.011) were independent risk factors for the development of ESRD. CONCLUSIONS: Approximately one-third of children with RPGN developed ESRD; and need for acute dialysis and TA/IF ≥20% were independent risk factors for ESRD.

Delayed Therapeutic Response Time Predicts Renal Damage in the First Episode of Febrile Urinary Tract Infection.

Objective: To evaluate the relationship of therapeutic delay time (TDT) and therapeutic response time (TRT) with renal damage in the first episode of febrile urinary tract infection (UTI). Material and Method: A prospective study was conducted in 67 children with the first episode of UTI at the Department of Pediatrics, Faculty of Medicine Siriraj Hospital between 2008 and 2010. To assess for renal damage, dimercaptosuccinic acid (DMSA) renal scintigraphy was performed at one and six months after the acute episode. Results: Abnormal DMSA renal scintigraphy was detected in 20 (29.9%) patients. There was no difference in TDT but TRT was different between the patients with normal and abnormal DMSA renal scintigraphy at p-value 0.001. The area under receiver operating characteristic (ROC) curve for TRT was 0.76 (95% confidence interval (CI) 0.64-0.86) at p-value 0.001. The optimal cut-off value for TRT was 22 hours with sensitivity 80.0% (56.3-94.1) and specificity 63.6% (47.8-77.6). In 50 patients with no vesicoureteral reflux (VUR), there was difference in TRT at p-value 0.002. The area under ROC curve for TRT was 0.82 (95% CI 0.69-0.96) at p-value 0.004. The optimal cut-off value for TRT was 25 hours with sensitivity 88.9% (95% CI 51.7-98.2) and specificity 68.4% (95% CI 51.3- 82.5). Conclusion: TRT at or more than 22 hours predicts renal damage after first episode of UTI. In patients with no VUR, TRT at or more than 25 hours predicts renal damage. DMSA renal scintigraphy in the first episode of UTI should be considered in these patients.

Validity and Reliability of the Thai Version of Dysfunctional Voiding Symptom Score (DVSS) Questionnaire.

Background: Bladder and bowel dysfunction (BBD) is a common problem and a predisposing factor for urinary tract infection in children. Evaluation using the Dysfunctional Voiding Symptom Score (DVSS) questionnaire is widely used. The original English DVSS has been translated to several languages. Thai version of the DVSS would be helpful for early detection and management of BBD in children. Objective: To translate and adapt DVSS to Thai version and evaluate validity and reliability of this questionnaire in children aged 3-10 years. Material and Method: DVSS English version was translated into Thai according to the following methodology: translation, synthesis, back-translation, expert reviews, and pre-testing. Patients aged 3 -10 years with a history of diurnal urinary incontinence, urinary tract infection or abnormal voiding habits, presenting to pediatric nephrology or urology clinic, were recruited as study group. Age-matched children presenting to general outpatient clinic were recruited as control group. Final DVSS-Thai version was tested and re-tested at the following 1 - 2 weeks. The internal consistency and test­retest reliability was assessed with Cronbach's alpha test and intraclass correlation (ICC), respectively. The optimum cutoff score was analyzed using receiver operating characteristics (ROC) curve. Results: The back translation to English of Thai version corresponded to the original English DVSS. Age and sex between the study (n = 22) and control (n = 30) group were not different. Mean total scores of the DVSS were 11.6±4.7 VS 1.9±1.6 (p<0.001) in the study and control group, respectively. Cronbach's alpha coefficient was 0.8 and intraclass correlation coefficient (ICC) was 0.906 (p<0.001). The optimum cutoff score was 5 (sensitivity 100% and specificity 96.7%) with area under the curve of 0.998 (p<0.001). Conclusion: Thai version of the DVSS was completed by proper process and demonstrated an excellent validity and reliability. It should be used to identify BBD in Thai children.

Long-term outcome in pediatric renal tumor survivors: experience of a single center.

Medical records of 30 children with renal tumor diagnosed at Siriraj Hospital during 1996 to 2007 were reviewed. Mean age at diagnosis was 36 months; male to female ratio was 1.7:1. Clinical manifestations included abdominal mass (96.7%), hypertension (40.0%), abdominal pain (36.7%), hematuria (26.7%), postrenal obstruction (16.7%), and proteinuria (13.3%). Eight patients had Denys-Drash malformations, WAGR, Dandy-Walker malformation, or genitourinary anomalies. Twenty-seven patients (90%) had Wilms tumor. Sixteen patients were stage 3 or more at diagnosis. Rhabdoid tumor was found in 3 patients. All patients received chemotherapy, 13 patients also received radiation therapy. Acute complications included febrile neutropenia (44.4%), hypokalemia (37.0%), hyponatremia (29.6%), Fanconi syndrome (11.1%), urinary tract Infection (10.0%), and acute renal failure (7.4%). Mean follow-up time was 57.2 months. Ten patients died from progressive disease. Five-year patient survival was 69.7%. Two patients had chronic kidney disease. One of these had Denys-Drash malformations. Both patients received ifosfamide-carboplatin-etoposide protocol and abdominal radiation. Antihypertensive medications were needed in 9 patients for a mean duration of 164 days. None had persistent proteinuria or hematuria. No difference was found among mean estimated glomerular filtration rate at diagnosis, 1 year after treatment, and at last follow-up. Long-term follow-up, especially renal function, is recommended.

Tacrolimus in steroid resistant and steroid dependent childhood nephrotic syndrome.

OBJECTIVE: To evaluate the efficacy of tacrolimus (Tac) in steroid resistant and steroid dependent nephrotic syndrome (NS) in children. MATERIAL AND METHOD: Retrospective chart reviews of 18 children from outpatient clinic at the Department of Pediatrics, Faculty of Medicine Siriraj Hospital were diagnosed with steroid resistant (SR) and steroid dependent (SD) NS during 2002-2008 were enrolled in the present study RESULTS: The boy to girl ratio was 2:1. The mean age at diagnosis was 6.0 years (1-14.4 years). There were nine SR and nine SDNS. Nine patients had focal segmental glomerulosclerosis (FSGS), 4 IgMnephropathy and two had minimal change diseases (MCD). Three children did not receive renal biopsy. All patients received prednisolone at the start of Tac. The average time from the diagnosis to initiation of Tac was 3.5 years (0.2-14years). The mean duration of Tac treatment was 1.3 year (0.3-6.2 years). The average Tac trough blood level was 4.09 mcg/L (1.3-9.9 mcg/L). The average dosage of Tac was 0.09 mg/kg/day (0.03-0.2 mg/kg/day). Thirteen (72.2%) children achieved complete response (CR). Five (27.80%) children did not respond to Tac. Nine (69.2%) children could stop prednisolone whereas four (30.8%) could lower prednisolone doses. The mean time to achieve CR was 24.6 days (0.1-3 months). The mean follow up period was 3.1 years (0.2-6.4 years). There was no change in an estimation of glomerular filtration rate (eGFR). In SRNS, there were CR in four (44.4%) and five (55.6%) children that FSGS did not respond to Tac. In SDNS, all responded to Tac and four (44.4%) children relapsed while on Tac and had upper respiratory tract infection (URI). CONCLUSION: Tac is well-tolerated and effective treatment for SR and SDNS.

Combined renin angiotensin blockade in childhood steroid-resistant nephrotic syndrome.

BACKGROUND: Reduced proteinuria results in delayed deterioration of renal function and remission of proteinuria predicts a good long-term prognosis in steroid-resistant nephrotic syndrome (SRNS). The aim of this study was to analyze the effects of the combined angiotensin-converting enzyme inhibitor and angiotensin II receptor blocker in reducing proteinuria in SRNS. METHODS: A prospective study of eight patients with SRNS was conducted at the Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University from September 2003 to December 2007. Enalapril was given at 0.1 mg/kg/day and was increased by 0.1 mg/kg/day every 4 weeks up to 0.6 mg/kg/day (maximum 40 mg/day) and 1 mg/kg/day of losartan was added for 4 weeks and stepped up to 2 mg/kg/day (maximum 100 mg/day) for another 4 weeks. RESULTS: There were five boys (62.5%) and three girls (37.5%). The mean age at diagnosis was 8.3 ± 4.1 years (range 2.05-13 years) and age at enrollment was 11.7 ± 3.8 years (range 6-16 years). Renal histology revealed seven focal segmental glomerulosclerosis and one immunoglobulin M nephropathy. The results showed significant reduction on mean spot urine protein : creatinine ratio from 9.6 ± 2.3 to 3.6 ± 1.6 (P < 0.05) and 24-h urine protein from 182.8 ± 59.6 to 28.7 ± 8.2 mg/m(2) /h (P < 0.05). Urine protein reduction ratio at the end of the study was 50% (P= 0.08). Serum cholesterol, albumin, potassium, blood pressure and renal function had no significant change. No clinical and laboratory side-effects were reported. CONCLUSION: Combined high-dose angiotensin II receptor blocker to high-dose angiotensin-converting enzyme inhibitor therapy is safe and effective in reducing proteinuria in childhood SRNS. However a large-scale study should be conducted to validate this result.

A novel mutation in the beta-subunit of the epithelial sodium channel gene (SCNN1B) in a Thai family with Liddle's syndrome.

Liddle's syndrome is a rare form of autosomal dominant hypertension with early penetrance and cardiovascular sequelae. It is caused by missense or frameshift mutations in the epithelial sodium channel (ENaC) gene resulting in excessive salt and water resorption from the distal nephron, volume expansion, and suppression of plasma renin activity and serum aldosterone secretion. Treatment with an antagonist of the amiloride-sensitive ENaC, amiloride or triamterine, can correct hypertension and biochemical abnormalities in Liddle's syndrome by closing the sodium channels. Missense and truncation mutations at the C-terminus of the ENaC gene have been found in two of the three genes encoding beta- and gamma-subunits of ENaC. We report here a Thai family with Liddle's syndrome caused by a novel P615H missense mutation in the proline-rich domain of the SCNN1B gene coding for the beta-subunit of ENaC. This mutation occurs within the conserved proline-rich (PY) motif at the C-terminal end and emphasizes the critical role of this motif in ENaC internalization. The presence of severe hypertension and/or a suggestive family history of hypertension with or without hypokalemia in young children should always raise a suspicion of Liddle's syndrome.

Renal tubule function in beta-thalassemia after hematopoietic stem cell transplantation.

Advances in hematopoietic stem cell transplantation (HSCT) for beta-thalassemia major make the long-term outcome of these patients very important. Few data on long-term renal function of thalassemia patients are available. We evaluated the renal function in children after successful allogeneic HSCT for beta-thalassemia. Twenty-nine patients were included; the mean age at HSCT was 4.9 years. Mean follow-up time was 7.6 years. After HSCT, two patients developed acute renal failure and two had graft versus host disease. At last follow up, height standard deviation score (SDS) remained the same, but weight SDS had improved. Mean hemoglobin was 12.5 g/dl, and serum ferritin level was 545 ng/ml. All children had normal estimated glomerular filtration rate (GFR). One patient had hypertension and proteinuria, 10 years after HSCT. When comparing 39 children of the same age with beta-thalassemia of similar disease severity but who had not experienced HSCT, we found that the parameters of renal tubule function were better in patients that had undergone HSCT, as demonstrated by urine protein level (0.36 mg/mg creatinine vs 3.03 mg/mg creatinine, P < 0.001), osmolality (712 mosmol/kg vs 573 mosmol/kg, P = 0.006), N-acetyl-beta-D: -glucosaminidase (17.7 U/g creatinine vs 42.9 U/g creatinine, P = 0.045), and beta 2 microglobulin (0.09 microg/mg creatinine vs 0.13 microg/mg creatinine, P = 0.029). This study showed a low incidence of long-term renal impairment after HSCT and indicated that renal tubule function may be better in beta-thalassemia patients after HSCT.

Persistent hypertension in Thai children: etiologies and outcome.

A retrospective study was done in 66 children (0.21% of all admitted children) below the age of 18 years with persistent hypertension diagnosed at the Department of Pediatrics, Faculty of Medicine Siriraj Hospital from Jan 1999 to Dec 2003. Male to female ratio was 1.4:1 with 54.5% aged between 6-12 years old and 9.1% aged less than 1 year. Hypertension was found to be severe (BP more than the 99th percentile for age, sex and height) in 79.1% but most (78.6%) of the patients did not have symptoms related to hypertension. Chronic headaches were found in 10%, hypertensive encephalopathy in 8.6%, epistaxis in 1.4% and visual disturbance in 1.4%. The most common cause of hypertension was renal parenchymal diseases (62.7%) mainly lupus nephritis (26.9%), idiopathic nephrotic syndrome (16.4%) and chronic renal failure (16.4%). Other causes of hypertension included renovascular diseases (7.5%), drug-induced (7.5%), essential (7.5%), tumors (4.5%), coarctation of aorta (3.0%), bronchopulmonary dysplasia (3.0%), and pheochromocytoma (1.5%). Obesity and overweight (body mass index, BMI more than 25) was found in only 10 patients (15.1%). The proportion of children with BMI more than 25 was not different between essential and secondary hypertension (p = 0.15). Left ventricular hypertrophy was noted in 7.5%, hypertensive retinopathy in 3.0%, and hypertensive encephalopathy in 9.0%. One-third of the patients had normal BP within 1 month and another 47.0% had normal BP within 6 months of diagnosis. One-fifth of the patients also needed surgical intervention for specific underlying diseases. The authors suggest that since a large number of children with hypertension have secondary hypertension, intensive investigation and prompt management should be done in all. Obesity and overweight is not reliable in the differentiation between primary and secondary hypertension. Short term outcome of hypertension is good with medications and surgery in selected cases but long term outcome is still unknown.

The relation of vesicoureteral reflux and renal scarring in childhood urinary tract infection.

OBJECTIVE: Assess the relation of age and sex in vesico ureteral reflux (VUR) and renal scarring and the relation of VUR and renal scarring in childhood urinary tract infection. MATERIAL AND METHOD: A descriptive study of one hundred and twenty-six children who received renal cortical scintigraphy from 1st Jan 2000 to 31st Dec 2004 in the Department of Radiology, Faculty of Medicine Siriraj Hospital, was conducted. Ninety-three (50 males, 43 females) patients were diagnosed with urinary tract infections (UTIs) but only ninety-one of them had renal cortical scintigraphic results available. The male to female ratio was 1.16:1. The mean age of the patients was 4.33 years (SD +/- 4.17, range 7 days-16 years). During the 1st year of life the male to female ratio is 2.6:1. Fever, dysuria, and poor feeding were the most presenting signs and symptoms. Eighty-five (45 males, 40 females) patients received Voiding Cysto Urethro Gram (VCUG). RESULT: The authors did not find the correlation between the age groups and sex with VCUG results on right and left side, respectively (p = 0.856, p = 0.145, p = 0.77, p = 0.75). Ninety-one (49 males, 42 females) patients received DMSA renal scintigraphy. Fifty-two patients (57.1%) had abnormal DMSA renal scan results. However; the authors did not find the correlation between age groups and sex with DMSA renal scan results on the right and left kidneys, respectively. (p = 0.202, p = 0.416, p = 0.511, p = 0.791). The authors compared times of UTIs with and DMSA renal scintigraphy in each side of the kidney. Even though the authors did find the correlation between episodes of UTIs and abnormal DMSA on the left kidneys (p = 0.017), it was not found on the right kidneys (p = 0.081). There were 80 patients who received both VCUG and DMSA renal scintigraphy. The authors found the correlation between severity of VUR and abnormal DMSA results on right and left kidneys (p = 0.001, p = 0.01). CONCLUSION: The authors recommend that all children who have repeated UTI and/or VUR, irrespective of age and sex, should receive DMSA renal scintigraphy to detect renal scarring and follow up future complications.

Immune-mediated hemolytic anemia in pediatric renal transplantation.

The aim of the study was to demonstrate clinical course of the first reported cases of PLS in pediatric kidney transplantation and therapeutic outcome for such condition using a combination of high-dose corticosteroid and tacrolimus. We report a single case (a nine-year-old Thai boy) with end-stage kidney disease secondary to obstructive uropathy developed immune-mediated hemolytic anemia from the PLS at second week after a pre-emptive living-related kidney transplantation. The alloimmune hemolysis was a result of anti-B antibodies, derived from blood group O-donor lymphocytes. Using a combination of high-dose corticosteroid and a substitution of cyclosporin with tacrolimus, there was no further hemolysis although the anti-B antibodies remained detectable until the eighth week post-transplantation. An impairment of the graft function because of hemoglobinuria was resolved after the hemolysis was stopped. The alloimmune hemolysis caused by PLS in pediatric kidney transplantation could be controlled with a combination of high-dose corticosteroid and tacrolimus.

Difficult-to-treat nephrotic syndrome: management and outcome.

A retrospective study was performed in 68 patients diagnosed as having idiopathic nephrotic syndrome with steroid-dependent, steroid-resistant or frequent relapse subtypes at the Department of Pediatrics, Siriraj Hospital during Jan 1996-Dec 2004. Male to female ratio was 3.3:1 and mean age (+/- SD) was 8.4 +/- 3.5 years. Mean follow up time (+/- SD) was 47.4 +/- 30.5 months. Renal biopsy was done in 60 patients, showing IgM nephropathy in 73.3%. Fifty-four patients (79.4%) received cyclophosphamide at a dose (+/- SD) of 2.2 +/- 0.5 mg/kg/d for 11.6 +/- 3.4 weeks. Negative proteinuria at 1 year was found in 70% and prednisolone was discontinued in 52%. Leucopenia was found in 9.2%. At last follow up, 34% of the patients were still in remission. Enalapril was prescribed in 50 patients for 12.4 +/- 10.0 months. Thirty-six patients also received cyclophosphamide. Remission at 1 year was achieved in 66% and prednisolone discontinued in 28%. Twelve patients (24%) were still in remission at last follow up. The results of 3 regimens: cyclophosphamide, enalapril, and cyclophosphamide plus enalapril were compared using chi-square test. Remission was significantly better in cyclophosphamide group (p = 0.014). Dipyridamole was prescribed in 14 patients due to thrombocytosis. Only 2 of 14 patients achieved remission although 11 patients received cyclophosphamide plus enalapril, and another 2 patients received only cyclophosphamide. Complications included hypertension (44%), cataract (40%), glaucoma (15%), short stature (17.6%), and obesity (5.9%). Recurrent infection was found in 69%, including dental caries (16.29%), urinary tract infection (14.7%), intestinal parasitic infestration (10.3%), respiratory tract infection (8.8%), and skin infection (7.4%). Chronic renal failure was found in 3 patients and portal vein thrombosis was found in 1 patient. We suggest that cyclophosphamide should be used as first line drug in difficult-to-treat nephrotic syndrome patients. Enalapril may be beneficial in some patients. Thrombocytosis may be associated with poor response to both medications. Difficult-to-treat patients also need long-term follow up and surveillance for complications due to disease and/or treatment.

Systemic lupus erythematosus in Thai children: clinicopathologic findings and outcome in 82 patients.

OBJECTIVES: To define the patterns of clinicopathologic findings and to identify the risk factors for renal failure and mortality of childhood-onset systemic lupus erythematosus (SLE) in Thailand. MATERIAL AND METHOD: The study is a retrospective analysis of clinical manifestations, laboratory data, and pathologic findings, treatment modalities, and outcome of 82 patients with biopsy-proven lupus nephritis (LN) with disease onset between I January 1987 and 31 December 1997. All children developed these first manifestations at the age 13 years or under RESULTS: Sixty-four (789%) patients were females and eighteen (22%) were males (ratio female/male = 3.5:1). The patients were followed for a mean period of 53.6 months (range 1 -141). The mean age at disease onset was 9.2 years (range 2-12.6). Class-IV LN, observed in 40 (48.8%) patients, was the most frequent histopathology on initial renal biopsy. Less frequent findings were class-II (30.5%), V (14.6%), I (3.7%) and III (2.4%) LN. Based on the renal histopathology and clinical presentations, patients were treated with corticosteroids alone or in combination with azathioprine or with intravenous cyclophosphamide (CYC). Methylprednisolone pulses were given in patients with clinically more severe disease. Follow-up biopsies, performed in 12 patients, showed no change in 4 patients, and were progressive in 8 patients. On final clinical evaluation, 20 patients died, 65% died from serious infections, 15% from cardiopulmonary complications, and 10% from end stage renal disease. As the whole group, survival rates were 89% and 74% at 12 and 60 months, respectively. The 5-year patient survival in class-II, class-IV and class- VLN patients were 83%, 67% and 64%, respectively. Within the group of class-IV LN, the 5-year survival in the patients treated with intravenous CYC was significantly better than those receiving prednisolone with or without azathioprine. Five-year kidney survival rates from the time of diagnosis to the endpoints of terminal renal failure were 94% for the whole group, and 100%, 96%, 91% in the class- V, class-II, and class-IV group, respectively. Initial presence of hypertension, hematuria, renal insufficiency were independent factors significantly associated with lower patient survival probabilities. There was no association of either patient and kidney survival with gender, age, cytopenia, and autoantibody level. CONCLUSIONS: Infectious complications were the most common cause of morbidity and mortality in our pediatric patients with SLE. The immunosuppressive agents used to treat SLE seemed to be a major contribution to the patient survival. With judicious use of corticosteroid, intravenous CYC in severe SLE showed superior efficacy over oral prednisolone with or without azathioprine.