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Journal : Paediatrica Indonesiana

Management of Lowe syndrome: a case report Risky Vitria Prasetyo; Heru Setiawan; Ninik Asmaningsih Soemyarso; Mohammad Sjaifullah Noer; Irwanto Irwanto; Prastiya Indra Gunawan; Rozalina Loebis; Sri Andreani Utomo; Ni Wayan Tirthaningsih
Paediatrica Indonesiana Vol 55 No 3 (2015): May 2015
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (2093.872 KB) | DOI: 10.14238/pi55.3.2015.176-84

Abstract

Lowe syndrome (the oculocerebrorenal syndrome of Lowe, OCRL) is a multisystem disorder characterized by anomalies affecting the eyes, nervous system and kidneys.1-3 The disorder was first recognized by Lowe et al. in 1952, and described as a unique syndrome with organic aciduria, decreased renal ammonia production, hydrophthalmos, and mental retardation. In 1954, renal Fanconi syndrome was recognized as being associated with Lowe syndrome and in 1965, a recessive X-linked pattern of inheritance was determined.2,4 Lowe syndrome is a very rare disease, with an estimated prevalence in the general population of 1 in 500,000. According to the Lowe Syndrome Association (LSA) in the USA, the estimated prevalence is between 1 and 10 affected males in 1,000,000 people, with 190 living in the year 2000. The Italian Association of Lowe Syndrome estimated that there were 34 Lowe syndrome patients (33 boys and one girl) living in Italy in the year 2005.2,4,5 It almost exclusively affects males.6 Physicians may not be familiar with Lowe syndrome due to its rarity.4
Risk factors for hypertensive crisis in children with acute glomerulonephritis Sherly Yuniarchan; Risky Vitria Prasetyo; Ninik Asmaningsih Soemyarso; Mohammad Sjaifullah Noer
Paediatrica Indonesiana Vol 56 No 2 (2016): March 2016
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (283.518 KB) | DOI: 10.14238/pi56.2.2016.101-6

Abstract

Background Hypertensive crisis occurs in 1-4% of the hypertensive pediatric population, mostly due to acute glomerulonephritis (AGN). Some factors have been suggested to affect blood pressure (BP) in children, such as age, sex, race/ethnicity, obesity, and socioeconomic status, but little is known for risk factors for hypertensive crisis in AGN.Objective To analyze the risk factors for hypertensive crisis in children with AGN.Methods Retrospectively, we studied possible risk factors for hypertensive crisis in children with AGN at Dr. Soetomo Hospital from 2007 to 2011. Hypertensive crisis was defined as systolic BP ≥180 mmHg or diastolic BP ≥120 mmHg (for children ≥ 6 years of age); and systolic and/or diastolic BP >50% above the 95th percentile (for children aged <6 years). We evaluated the demographic and clinical characteristics as potential risk factors. Statistical analysis was done with Chi-square, Fisher’s exact, and logistic regression tests. Variables with P <0.25 in the univariable analysis were further analyzed by the multivariable logistic regression model. A P value of < 0.05 was considered statistically significant.Results There were 101 children included (mean age 9.7 (SD 2.17) years), with a male-to-female ratio of 2.7:1. Hypertensive crisis occurred in 42 (41.6%) children, of whom 8 had hypertensive urgency and 34 had hypertensive emergency. Proteinuria was seen in 53 children with AGN (52.5%) and was the significant risk factor for hypertensive crisis in our subjects (OR=2.75; 95%CI 1.16 to 6.52; P=0.021). Gender, clinical profiles, ethnicity, nutritional status, blood urea nitrogen (BUN), and glomerular filtration rate (GFR) were not significant risk factors for hypertensive crisis.Conclusion Proteinuria is the significant risk factor for hypertensive crisis in children with AGN.
Persistent proteinuria as an indicator of renal disease in HIV-infected children Yuni Hisbiiyah; Risky Vitria Prasetyo; Dwiyanti Puspitasari; Ninik Asmaningsih Soemyarso; Ismoedijanto Moedjito; Mohammad Sjaifullah Noer
Paediatrica Indonesiana Vol 56 No 6 (2016): November 2016
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (104.857 KB) | DOI: 10.14238/pi56.6.2016.343-9

Abstract

Background Persistent proteinuria (microalbuminuria) has been reported to be a precursor of HIV-related renal disease. Screening allows for early management in order to prevent the progression of renal disease and decrease morbidity and mortality associated with chronic kidney disease in HIV. Several studies have been done on renal manifestation in HIV-infected children from American and African regions, but similar studies from Asia are lacking.Objective To determine the prevalence of persistent proteinuria in HIV-positive children on antiretroviral therapy (ARV) in Dr. Soetomo Hospital, Surabaya.Methods A cross-sectional study on children with HIV and treated with  highly active antiretroviral therapy (HARRT) was done from August 2014 to February 2015. Microalbuminuria was measured by the ratio of urine albumin to creatinine (ACR), while proteinuria was measured by dipstick. Measurements were performed 3 times in 4-8 weeks. All subjects underwent complete evaluation of blood tests, serum creatinine, blood urea nitrogen (BUN), CD4 counts, and urinalysis. Data were analyzed using Chi-square and logistic regression tests.Results Of 38 children on HARRT enrolled in this study, 2 subjects developed acute kidney injury (AKI), 4 subjects were suspected to have urinary tract infection (UTI), and 1 subject was suspected to have urinary tract stones. The prevalence of persistent microalbuminuria was 2.6%. There was no correlation between immunological status, WHO clinical stage, or duration of ARV and the incidence of persistent proteinuria (P>0.05).Conclusion The prevalence of persistent proteinuria is  lower in younger HIV-infected children at a non-advanced stage and HIV-infected children with normal immunological status who are on HAART. We provide baseline data on the renal conditions of HIV-infected children in the era of HAART, before tenovofir is  increasingly used as an antiretroviral therapy regimen in Indonesia.
Management of childhood Gitelman syndrome: a case study Risky Vitria Prasetyo; Putu Dian Saraswati; Ninik Asmaningsih Soemyarso; Mohammad Sjaifullah Noer
Paediatrica Indonesiana Vol 56 No 3 (2016): May 2016
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (401.636 KB) | DOI: 10.14238/pi56.3.2016.184-91

Abstract

Gitelman syndrome is a rare, autosomal recessive, renal tubular salt wasting disorder characterized by hypokalemia, and metabolic alkalosis in combination with significant hypomagnesemia and hypocalciuria.1,2 The prevalence is estimated to be 1 in 40,000 individuals. The condition affects both males and females of all ethnic backgrounds. The prevalence of heterozygotes is approximately 1% in Caucasian populations.2,3In the majority of cases, symptoms do not appear before the age of six years and the disease is usually diagnosed during adolescence or adulthood. Symptoms, such as transient episodes of muscle weakness and tetany, sometimes accompanied by abdominal pain, vomiting and fever, are often seen in Gitelman syndrome patients. Paresthesias, especially in the face, frequently occur. Remarkably, some patients are completely asymptomatic except for the appearance of chondrocalcinosis at adult age that causes swelling, local heat, and tenderness over the affected joints. Blood pressure is lower than that in the general population. Sudden cardiac arrest has been reported occasionally. In general, growth is normal but can be delayed in those Gitelman syndrome patients with severe hypokalemia and hypomagnesemia.2,4
Repeat urine cultures in children with urinary tract infection Risky Vitria Prasetyo; Ninik Asmaningsih Soemyarso; Mohammad Sjaifullah Noer
Paediatrica Indonesiana Vol 52 No 3 (2012): May 2012
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (103.106 KB) | DOI: 10.14238/pi52.3.2012.170-4

Abstract

Background Urinary tract infections (UTIs) are the secondleading cause of infection in children, following respiratorytract infections. Repeat urine cultures after antibiotic treatmentare routinely obtained in clinical practice to verify proof ofbacteriologic cure. The American Academy of Pediatrics doesnot recommended repeat cultures, due to increased cost anddiscomfort to patients.Objective To determine the frequency of positive repeat urinecultures after 3 days of antibiotics in children 'With UTIs.Methods We conducted a retrospective study on childrenwith UTIs who visited the Division of Pediatric Nephrology,Department of Child Health at Dr. Soetomo Hospital, Surabayafrom January 2006 to December 2011. Results of repeat urinecultures were obtained after 3 days of antibiotic treatment.Descriptive statistics were used to analyze the data.Results Of the 779 pediatric UTI cases, repeat urine cultureswere performed in 264 (33.9%) cases. Of the 264 patients whocomprised our study, there were similar numbers of girls and boys(50.4% vs. 49.6%, respectively). The mean age of patients was43.9 (SD 1.59) months and 35.5% of subjects were aged under 1year. In the initial urine cultures of our subjects, Escherichia coliwas the most common organism found, v,ith 92 cases (34.8%),compared to 58 cases (21.9%) of Klebsiella pneumoniae and 29cases (10.9%) of Pseudom.onas aeruginosa. Repeat urine culturesshowed no bacterial growth in 168 cases (63.6%).Conclusion Mostly negative repeat urine cultures v,ill probablyobviate the need of this test in daily routine practice. [PaediatrIndanes.2012,52:170·4].
Serum immunoglobulin E levels in children with idiopathic nephrotic syndrome Ninik Asmaningsih; Windhu Poernomo; M Sjaifullah Noer
Paediatrica Indonesiana Vol 45 No 2 (2005): March 2005
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (306.264 KB) | DOI: 10.14238/pi45.2.2005.55-9

Abstract

Background Children with idiopathic nephrotic syndrome (INS)have been known to have T-cell dysfunction and an impairment ofthe cytokine network that may alter glomerular permeability andthe glomerular filtration barrier. This disorder may contribute to thepresence of urinary protein loss in children with INS. The elevationof serum IgE levels has been noted in some cases, but its associa-tion with steroid-responsive nephrotic syndrome has not been fullyelucidated.Objective This study was done to investigate the association be-tween serum IgE levels prior to prednisone treatment in childrenwith INS and the outcome of treatment.Methods A prospective observational study has been conductedon 22 children with INS. Prednisone therapy was given with a doseof 60 mg/m 2 body surface area (BSA) for four weeks followed by asingle dose of 40 mg/m 2 BSA every other day for another fourweeks. This protocol was applied for steroid-responsive INS chil-dren. Children with steroid resistance were given oral cyclophos-phamide 2 mg/kg for eight weeks. IgE level measurements wereperformed prior to prednisone therapy and at remission. Data wereanalyzed using one-way ANOVA and multiple regression.Results Twenty-two children were enrolled in this study. High lev-els of serum IgE were found in 95.5% of children, with a mean of2002.5 (SD 2172.1) IU/ml. The serum IgE levels of INS childrenwith history of allergy were significantly higher than those of neph-rotic children without history of allergy (P<0.05). However, therewas no significant correlation between the serum IgE levels andthe outcome of treatment in children with INS.Conclusion The high serum IgE levels in children with INS seemto be associated with humoral immune disorder and did not haveany association with the outcome of therapy. Even though the se-rum IgE levels were significantly higher in INS children with historyof allergy, other factors that may influence serum IgE levels mustbe considered
Predictors of mortality in children with lupus nephritis Lukman Oktadianto; Risky Vitria Prasetyo; Ninik Asmaningsih Soemyarso; Mohammad Sjaifullah Noer
Paediatrica Indonesiana Vol 54 No 6 (2014): November 2014
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (102.854 KB) | DOI: 10.14238/pi54.6.2014.338-43

Abstract

Background Renal involvement during the clinical course ofsystemic lupus erythematosus (SLE) is generally considered to bethe most important factor influencing disease prognosis in termsof morbidity and mortality. Various factors have been reported toinfluence the prognosis of lupus nephritis (LN).Objective To analyze clinical signs and laboratory parameters thatmight serve as predictors associated with mortality in pediatricLN.Methods Retrospectively, medical records of children with LNat Soetomo Hospital from 1998 to 2011 were studied. Diagnosisof SLE was based on Revised American Rheumatism Associationcritera, while patients with clinical manifestations of hypertension,abnormal urinalysis, and serum creatinin > 1 mg/dL wereconsidered as lupus nephritis. Cox proportional hazard modelingwas used to assess for associations of clinical signs and laboratoryparameters with mortality. Kaplan-Meier survival analysis wasused to assess the cumulative survival from the time of diagnosisto the outcome.Results There were 57 children with LN of whom 43 (75%) weregirls. The female-to-male ratio was 3:1. Subjects’ mean age was 10.6(SD 6.87) years. The mean time of observation was 51 (SD 74.54)months and 23 (40%) children died. Age, gender, hypertension,hematuria, proteinuria, and anemia were not significant aspredictors for mortality. However, hypertensive crisis (HR=2.79;95%CI 1.16 to 6.75; P=0.02) and initial glomerular filtration rate(GFR) of <75 mL/min/1.73m2 (HR=3.01; 95%CI 1.23 to 7.34;P=0.01) were significant predictors of mortality in children with LN.The mean survival time of LN with hypertensive crisis and initialGFR <75 mL/min/1.73m2 was 36.9 (SD 12.17) months.Conclusion Hypertensive crisis and GFR <75 mL/min/1.73m2 aresignificant predictors of mortality in children with LN.
Incidence of Cholera in Children under two years of age at the Dr. Soetomo Hospital, Surabaya Haroen Noerasid; Pitono Soeparto; Bing Rudyanto; Moh. Sjaifullah Noer; Moh. Adnan
Paediatrica Indonesiana Vol 15 No 7-8 (1975): July - August 1975
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (396.526 KB) | DOI: 10.14238/pi15.7-8.1975.219-28

Abstract

Stool cultures from 510 patients, admitted to the Children's Ward of the Dr. Soetomo Hospital with gastroenteritis, were examined. Fifty-six cases were found to have para Cholera El Tor (10.98%) and 56 patients showed positive E. coli (10.98%). The clinical features of para Cholera El Tor have been discussed, and compared with E. coli infections. We also reviewed the clinical observations of other writers. Clinically Cholera El Tor and E. coli have similar signs and symptoms. They differ only in: (a) Age (El Tor is usually found in older age groups). (b)Nutritional State (El Tor is found among the undernourished), (c) State of dehydration (El Tor causes dehydration more rapidly), and (d) Faeces (El Tor excretes more faeces per movement).
HBsAg in Cord Blood Qf Newborns of HBsAg-Positive Mothers Adnan S. Wiharta; Evi Setiadi; H. M. Sjaifullah Noer; Triyatmo Rachimhadhi; Asri Rasad
Paediatrica Indonesiana Vol 34 No 5-6 (1994): May - June 1994
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (421.132 KB) | DOI: 10.14238/pi34.5-6.1994.125-8

Abstract

Vertical transmission of hepatitis B infection that may occur during pregnancy at delivery-, in infancy, and early childhood has an important role in the development of chronic hepatitis B. Intrauterine infection is suspected to occur when hepatitis B viruses cross the placenta into fetal circulation due to failure of placental tissue function. In Cipto Mangunkusumo Hospital, Jakarta, 98 (6.4%) of 1536 pregnant mothers obseiVed during 3 years (1987 -1990) showed positive HBsAg. Six (8.3%) of 60 babies of born to HBsAg positive mothers showed positive HBsAg in their cord blood, but this disappeared after one month. All babies born to HBsAg positive mothers were vaccinated on months 0, 1, 2, and 12. HBsAg in cord blood might not play an important role in vertical transmission.