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.

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."

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.

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.

The role of DMSA renal scintigraphy in the first episode of urinary tract infection in childhood.

OBJECTIVE: The role of dimercaptosuccinic acid (DMSA) renal scintigraphy in the first episode of urinary tract infection (UTI) has been the subject of debate for many years. The aim of this study was to evaluate the relationship of voiding cystourethrography (VCUG), renal ultrasonography and DMSA renal scintigraphy and to detect renal parenchymal changes by performing DMSA renal scintigraphy at 6 months after the first episode of UTI. METHODS: A prospective study was conducted in 67 hospitalized children (46 boys, 21 girls). Mean age of the patients was 0.97 ± 1.57 years (0.02-7.26 years). All children received VCUG, renal ultrasonography and DMSA renal scintigraphy. DMSA renal scintigraphy was performed at 1 and 6 months after UTI. RESULTS: Of 67 children, 17 (25.4%), 23 (34.3%) and 20 (29.9%) had vesicoureteral reflux (VUR), abnormal renal ultrasonography and abnormal DMSA renal scintigraphy, respectively. Unilateral hydronephrosis had a significant correlation with VUR at p value 0.024. In renal units, abnormal renal ultrasonography and hydronephrosis had significant correlations with VUR at p values 0.039 and 0.021, respectively. In patients and renal units, hydronephrosis had no significant correlation with abnormal DMSA renal scintigraphy at 1 month after UTI. However, abnormal renal ultrasonography and VUR had significant correlations with abnormal DMSA renal scintigraphy at p values 0.022 and < 0.001 in patients and at p values 0.024 and < 0.001 in renal units, respectively. Both in patients and renal units, VUR (Grade I-III) had no significant correlation with abnormal DMSA renal scintigraphy. However, severe VUR (Grade IV-V) had significant correlations with abnormal DMSA renal scintigraphy at p values < 0.001 and < 0.001, respectively. Seventeen patients underwent DMSA renal scintigraphy at 6 months after UTI. In addition, 15 (88.2%) developed persistent renal scarring. CONCLUSION: Abnormal renal ultrasonography and severe VUR identify renal parenchymal changes. DMSA renal scintigraphy in the first episode of UTI should be carried out in those patients. Abnormal DMSA renal scintigraphy at 1 month after UTI has a tendency to persist.

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.

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.

Pediatric systemic lupus erythematosus in Siriraj Hospital.

A descriptive study of one hundred and one pediatric patients with systemic lupus erythematosus treated between July 1985 and March 2003 in Department of Pediatrics, Faculty of Medicine Siriraj Hospital was conducted. According to existing database, there were a total of 181 patients, 101 of them (55.8%) had available data for review. The female to male ratio was 6.2:1. The mean and median ages of onset were 9.7 +/- 2.8 and 10 +/- 2.2 years, respectively (range 4-14 years). The clinical presentations were renal involvement in 87 patients (86.2%), skin and mucocutaneous involvement 77 patients (76.3%), hematological abnormalities 74 patients (73.4%), musculoskeletal involvement 32 patients (31.7%), prolongedfever 24 patients (23.8%), neuropsychiatric symptoms 21 patients (20.8%), gastrointestinal involvement 20 patients (19.8%), cardiac involvement 14 patients (13.9%), lymphadenopathy 13 patients (12.9%), and pulmonary involvement 7 patients (6.9%). The most common renal, skin and mucocutaneous, and hematological manifestations were proteinuria, malar rash, and anemia, respectively. Lupus nephritis with WHO class IV was the most common histopathological finding of the initial renal biopsies. The most common neuropsychiatric, gastrointestinal, cardiac, and pulmonary involvements were seizure, hepatomegaly, pericarditis, and pleuritis, respectively. Ninety-two percent of patients reported as having significant ANA positive results using rat liver tissue as a substrate. Sixty-six out of 94 patients (70.2%) had positive test result of Anti-dsDNA. In conclusion, the age at onset, clinical manifestations and laboratory investigation results of SLE in children at Siriraj Hospital are comparable to other studies in the Country and also to other Asian and Western studies.

Gene expression in early ischemic renal injury: clues towards pathogenesis, biomarker discovery, and novel therapeutics.

Acute renal failure (ARF) represents a common and serious problem in clinical medicine. Renal ischemia-reperfusion injury (IRI) is the major cause of ARF in the native and transplanted kidney. Several decades of research have provided successful therapeutic approaches in animal models, but translational efforts in humans have yielded disappointing results. The major reasons for this include a lack of early markers for ARF (and hence a delay in initiating therapy), and the multi-factorial nature of the disease. This review focuses on the use of cDNA microarrays to elucidate the molecular genetic mechanisms underlying tubule cell apoptosis, and to identify novel biomarkers for early renal IRI. Also presented is a comparative temporal analysis of cDNA microarray results from mature kidneys following IRI and during normal nephrogenesis. Molecular genetic evidence for the notion that regeneration recapitulates development in the kidney, and that injured tubule cells possess the capacity to de-differentiate to the earliest stages of development, is presented. The implications of these findings to the ability of the kidney to repair itself and potential strategies for accelerating recovery are briefly discussed.

Differential gene expression following early renal ischemia/reperfusion.

BACKGROUND: Acute renal failure from ischemia/reperfusion injury is associated with tubule cell apoptosis, the molecular mechanisms of which remain under active investigation. The purpose of this study was to identify apoptosis-related genes that are differentially expressed in the early periods following renal ischemia. METHODS: Mice underwent unilateral renal artery clamping for 45 minutes and were sacrificed at 0, 3, 12, or 24 hours of reperfusion. Tubule cell apoptosis was confirmed by DNA laddering and terminal deoxynucleotidyl transferase-mediated uridine triphosphate nick end labeling (TUNEL) assay. We employed cDNA microarrays to define global changes in renal gene expression. Semiquantitative reverse transcription-polymerase chain reaction (RT-PCR) and immunohistochemistry were used as confirmatory tools. RESULTS: By microarray analysis, we identified consistent patterns of altered gene expression, including transcription factors, growth factors, signal transduction molecules, and apoptotic factors. Prominent among the last category included FADD, DAXX, BAD, BAK, and p53. Up-regulation of these proapoptotic genes was confirmed by semiquantitative RT-PCR and immunohistochemistry. CONCLUSION: The results indicate that apoptosis may represent an important mechanism for the early loss of tubule cells following ischemia/reperfusion injury. Both the death receptor-dependent (FADD-DAXX) and mitochondrial (BAD-BAK) pathways are activated. The results also provide a molecular basis for the previous findings that significant intrarenal mechanisms exist to enable tubule cell repair and regeneration, as evidenced by the up-regulation of genes such as growth, proliferation, transcription, and cytoskeletal factors.