Sonographic nomogram of paediatric renal size in Pusat Perubatan Universiti Kebangsaan Malaysia (PPUKM).

INTRODUCTION: A person's childhood is an important period of growth, and also one's most vulnerable, as one can be exposed to various pathologies, for example those that could affect the growth of one's kidney. Asians are physiologically different from Caucasians, and the nomogram renal size obtained from a Western population (mostly of Caucasians) is not be suitable for representing Asian children. As such a nomogram on paediatric renal size derived from Malaysia is needed. METHODS: A total of 109 (64 males and 45 females) aged 0-12 in Pusat Perubatan Universiti Kebangsaan Malaysia (PPUKM) took part in this study. They underwent ultrasonography of both kidneys, and their demographic and anthropometric data were collected. The mean and standard deviations of the renal length and renal volume according to their age groups was calculated, and the final data was compared to the ones reported by Rosenbaum et al. (1984). RESULT: Body weight and Body Surface Area (BSA) of the children reported the strongest correlation with renal size. Significant differences were found between local and the data from Rosenbaum et al (1984). A nomogram on paediatric renal size based on children in PPUKM was then created. DISCUSSION: Ultrasonography is regarded as the standard method for determining renal size. Body weight and BSA were both strongly correlated with renal size. It was shown that the widely used nomograms derived from data obtained from Caucasian was not suitable to represent the population of Malaysian children.

Health-related quality of life in paediatric spina bifida.

INTRODUCTION: The average incidence of spina bifida (SB) in Malaysia is 0.43 among 1,000 live births. The burden of the disease and its impact on the overall development and health though tremendously improved, remains significant. Therefore, current patient management strategies must include quality of life (QOL) measures. METHODS: This was a prospective, cross-sectional study on spina bifida children aged 5-20 years, attending the paediatric spina bifida clinics of Universiti Kebangsaan Malaysia Medical Centre Kuala Lumpur and Hospital Tuanku Jaanku Seremban. Scores were obtained using the validated disease specific Parkin QOL questionnaire. Univariate and multivariate analysis were used to investigate factors that were determinants for these outcomes. Results were expressed as beta coefficient and 95% confidence intervals (95%CI). RESULTS: A total of 54 children and adolescents aged between 5-20 years completed the questionnaires. Presence of neurogenic bowel (p=0.003), neurogenic bladder (p=0.041), shunt (p=0.044), non-ambulators (p=0.007) and being the only child in the family (p=0.037) were associated with lower QOL scores. Multivariate analysis showed presence of neurogenic bowel (ß=0.375, 95%CI: 0.00, 0.15) and being the only child in the family (ß=0.250, 95%CI: 0.04, 0.17) explained 22.1% of the variance in the QOL mean percentage scores. CONCLUSION: Being a single child in the family was the only socio-demographic variable associated with lower QOL scores. Although several clinical factors appeared to contribute significantly to QOL in spina bifida children, the presence of neurogenic bowel had the greatest impact.

Transition care readiness among patients in a tertiary paediatric department.

INTRODUCTION: A smooth transition of healthcare for young people with chronic illnesses from paediatric to adult healthcare services is important to ensure optimal outcome. At the moment, there are no standard guidelines to assess a patient's readiness to transfer care. METHODS: A cross-sectional study using a self-administered questionnaire, adapted from UNC (University of North Carolina) TRxANSITION self-assessment tool was conducted to evaluate patients' transition care readiness in paediatric haematology and paediatric diabetes clinic. RESULTS: A total of 80 patients (37 thalassaemia and 43 diabetes) with the mean age of 21.2 (SD±4.3) years, were recruited during the 3-month study period. Majority of the patients have basic knowledge regarding their medications, and were able to comply with their follow-up. The mean total score obtained by the respondents on this questionnaire was 15.3 (SD±3.59). Self-management skills and knowledge on disease were the two poorly scored section; with mean score of 3.78 (SD±1.38) and 4.28 (SD±1.20) respectively. Overall, only 21 (26.2%) respondents obtained high score (score above 75th percentile). Seventy-five percent of the respondents admitted that they were not ready for transfer to an adult healthcare service yet at the time of the study. CONCLUSION: We suggest that patients with high score should be prepared for transition to adult facility whereas those with a low score need to be identified to ensure provision of continuous education.

Urinary tract infection and bacteriuria in children performing clean intermittent catheterization with reused catheters.

STUDY DESIGN: This study was designed as a comparative cross-sectional cross-over trial on children performing clean intermittent catheterization (CIC) with reused catheters for 1 or 3 weeks. OBJECTIVES: To determine the incidence of symptomatic urinary tract infection (UTI) and bacteriuria (defined as colony count of ⩾105 colony forming units per ml of a single strain of organism) in these two different frequencies of catheter change. SETTING: Multidisciplinary children's neurogenic bladder clinics at two tertiary care hospitals in Kuala Lumpur Malaysia. METHODS: Forty children aged between 2 and 16 years performing CIC for at last 3 years were recruited. Medical and social data were obtained from case files. Baseline urine cultures were taken. All children changed CIC catheters once in 3 week for the first 9 weeks followed by once a week for the next 9 weeks. Three-weekly urine cultures were obtained throughout the study. Standardization of specimen collection, retrieval and culture was ensured between the two centers. RESULTS: At baseline, 65% of children had bacteriuria. This prevalence rose to 74% during the 3-weekly catheter change and dropped to 34% during the weekly catheter change (Z-score 6.218; P<0.001). Persistence of bacteriuria (all three specimens in each 9-week period) changed significantly from 60 to 12.5%, respectively (P<0.005). There was no episode of UTI during the 18-week study period. CONCLUSION: Reuse of CIC catheters for up to 3 weeks in children with neurogenic bladders appears to increase the prevalence of bacteriuria but does not increase the incidence of symptomatic UTI.

Contrast enhanced Voiding Urosonography (ce-VUS) as a radiation-free technique in the diagnosis of vesicoureteric reflux: Our early experience.

OBJECTIVE: Contrast-enhanced ultrasound has become increasingly utilised as an alternative imaging modality for the diagnosis of vesicoureteric reflux (VUR) in paediatric patients. The study objective is to evaluate the efficacy of contrast enhanced Voiding Urosonography (ce-VUS) compared with fluoroscopic micturating cystourethrography (MCU) in the detection of VUR. METHODS: This prospective study was carried out between July 2011 and January 2013 on paediatric patients who underwent MCU. All consented patients would undergo ce- VUS prior to MCU. We documented the epidemiology details, the number of Kidney-Ureter (K-U) unit studied, baseline renal and bladder sonogram, as well as presence of VUR on ce-VUR. The technique for ce-VUS was standardized using normal saline to fill the bladder prior to administration of SonoVue® (2.5 ml) to assess the kidney-ureter (K-U) unit. Dedicated contrast detection software was used to discern the presence of microbubbles in the pelvicaliceal system (PCS). The findings were then compared with MCU. RESULTS: 27 paediatric patients were involved in the study [17 males (63%) and 10 females (37%)] involving 55 K-U units (one patient had a complete duplex system). MCU detected VUR in 10 K-U units while ce-VUS detected VUR in 8 out of the 10 K-U units. There were 2 false negative cases (both Grade 1) with ce-VUS. The sensitivity, specificity, accuracy, positive predictive value, and negative predictive value of ce-VUS were 80%, 98%, 95%, 89% and 96%, respectively. CONCLUSION: ce-VUS is a sensitive and specific radiation-free alternative for the detection of VUR in the paediatric population.

Renal doppler assessment in differentiating obstructive from non-obstructive hydronephrosis in children.

BACKGROUND: to determine the usefulness of Doppler ultrasound measurement of resistive index (RI) in differentiating obstructive from non-obstructive hydronephrosis in children. METHODS: From August 2011 to November 2012, renal Doppler assessments of the intra-renal renal arteries were performed on 16 children (19 kidneys) with congenital hydronephrosis. the independent t-test was used to assess for significant difference in RI values between those with obstructive hydronephrosis (6 kidneys) and those with non-obstructive hydronephrosis (13 kidneys) as determined by dynamic renal scintigraphy. the assessor was blinded to the clinical findings and scintigraphy results. RESULTS: RI was significantly different between obstructive and non-obstructive hydronephrosis. Obstructive hydronephrosis returned higher RI values, with mean RI of 0.78. Mean RI in non-obstructive hydronephrosis was 0.70, and the difference was significant (p <0.05). the sensitivity and specificity of Doppler ultrasound were 100% and 53% respectively. CONCLUSION: Doppler ultrasound measurement of resistive index is useful in differentiating obstructive from nonobstructive hydronephrosis and provides an alternative non-ionizing investigation.

Predictors of parenting stress in mothers of children with spina bifida.

STUDY DESIGN: Prospective cross-sectional multidimensional study using clinical assessment and standard measures. OBJECTIVES: To determine the medical and social factors associated with parenting stress among mothers of children with spina bifida. SETTING: Spina bifida clinics of two tertiary hospitals in urban Kuala Lumpur, Malaysia. METHODS: A total of 81 mothers of children aged 1-18 years completed the Parenting Stress Index-Short Form (PSI/SF). Each child's adaptive skills were assessed using the Vineland Adaptive Behaviour Scales (VABS), Interview Edition. Medical and social data were obtained from direct interviews and case note reviews. Hierarchical multiple regression analysis was used to investigate factors that were determinants for high scores in the parental distress (PD), difficult child (DC) and parent-child dysfunctional interaction (P-CDI) subdomains of the PSI/SF. Results were expressed as beta coefficient (ß) and 95% confidence intervals (95% CIs). RESULTS: Single-parent families (ß 8.6, 95% CI 3.4-13.9) and the need for clean intermittent catheterization of bladder (ß 3.5, 95% CI 0.7-6.2) were associated with high PD scores. Clean intermittent catheterization (ß 3.0, 95% CI 0.5-5.5) was associated with higher DC scores. Lower composite VABS scores (ß -0.08, 95% CI -0.02 to -0.15) and mother as the sole caregiver (ß 2.6, 95% CI 0.15-4.96) was associated with higher P-CDI scores. CONCLUSION: The need for clean intermittent catheterization was the only medical factor associated with parenting stress in mothers of children with spina bifida. This was mediated by single parenthood, caregiver status and the child's adaptive skills.

Malignant hypertension in a child with phakomatosis pigmentovascularis type II b.

UNLABELLED: Phakomatosis pigmentovascularis (PPV) is a rare congenital syndrome characterized predominantly by cutaneous vascular malformations and pigmentary naevi. The most frequently reported form, Type II b, is associated with systemic involvement. Sturge-Weber Syndrome (SWS) with concomitant glaucoma, Klippel-Trenaunay Syndrome (KTS) and naevus of Ota have been frequently described, but there have only been two case reports with asymptomatic renal anomalies. CONCLUSION: We report the first case of PPV associated with SWS and naevus of Ota presenting with malignant hypertension secondary to bilateral renal artery stenosis. This case raises the possibility that the phakomatoses are not distinct entities but a spectrum of overlapping neurocutaneous, vascular and renal features.

Congenital hepatic fibrosis in a child with autosomal dominant polycystic kidney disease.

The association of congenital hepatic fibrosis (CHF) with autosomal recessive polycystic kidney disease (ARPKD) is well known and occurs in approximately 50% of cases. However the association of CHF with autosomal dominant polycystic kidney disease (ADPKD) is less well known and less well documented. We report a child with neonatal onset of hypertension due to ADPKD who later develops portal hypertension due to CHF in childhood. A review of this rare association follows.

The treatment of primary nocturnal enuresis in Malaysia.

To determine treatment outcomes in Malaysian children with primary nocturnal enuresis using both non-pharmacological methods and oral desmopressin. Data was collected prospectively from children aged 6-18 years who were referred to the Hospital UKM Enuresis Clinic. Treatment was given to those with a baseline wetting frequency of at least six wet nights/14 nights. Three modalities were offered: fluid management, reward system and oral desmopressin. Response was recorded as partial (> or = 50% reduction in WN from baseline) or full (completely dry). Seventy-one healthy children completed 12 weeks of therapy. Twenty-three children (32.4%) responded to non-pharmacological methods alone (4 full and 19 partial). Another 37 children (51.2%) responded to oral desmopressin (32 to 0.2mg, 4 to 0.4mg and 1 to 0.6mg). Thirty-two percent became dry whilst on therapy. The mean wetting frequency during treatment was significantly reduced (p < 0.01) compared to the baseline mean for both the non-pharmacological group and the desmopressin group. Discontinuation of desmopressin after 12 weeks increased the wetting frequency but this was still significantly lower than at baseline (p < 0.01). No adverse ents were recorded. Treatment of primary nocturnal enuresis in Malaysian children is both effective and well tolerated using fluid management strategies, reward systems and oral desmopressin.

Intravenous immunoglobulin in the treatment of acute disseminated encephalomyelitis.

We describe a case of acute disseminated encephalomyelitis in a child. This case is unusual in that the illness was characterised by recurrent episodes rather than a monophasic course and that the choice of treatment was intravenous immunoglobulin over corticosteroids. The rapid and remarkable recovery is highlighted and a review of the treatment for this rare condition is discussed.

Echocontrast cystosonography versus micturating cystourethrography in the detection of vesicoureteric reflux.

PURPOSE: To compare echocontrast cystosonography (ECS) using in-vivo agitated saline with fluoroscopic micturating cystourethrography (MCU) in the detection and grading of vesicoureteric reflux (VUR). MATERIALS AND METHODS: This was a prospective study of 25 children, who had MCU between 2007 and 2009. ECS was performed and findings documented prior to MCU. Baseline renal and bladder sonograms were obtained. The bladder was filled with normal saline followed by introduction of 10-20 mls of air to generate microbubbles. Detection of VUR was based on two sonographic criteria: (1) presence of microbubbles in the pelvicaliceal system (PCS), and (2) increase in dilatation of the PCS. VUR was graded as (1) Grade I: microbubbles seen in ureter only; (2) Grade II: microbubbles seen in non-dilated PCS; and (3) Grade III-V: microbubbles seen in dilated PCS. The ECS findings were compared using MCU as the gold standard. RESULTS: Of the 50 kidney-ureter (K-U) units studied, ECS detected 9 of 10 K-U units with VUR on MCU. ECS did not detect a Grade II VUR. The sensitivity, specificity, accuracy, positive predictive value and negative predictive value for criterion 1 was 90%, 87.5%, 88%, 64.3% and 97%, respectively, compared to criterion 2 which was 70%, 90%, 86%, 64% and 92%, respectively. The grading of VUR was similar on both ECS and MCU except for one case. CONCLUSION: ECS using agitated saline was a sensitive technique for the detection of VUR. ECS grading was comparable with MCU grading of VUR.

The usefulness of early ultrasonography, electroencephalography and clinical parameters in predicting adverse outcomes in asphyxiated term infants.

INTRODUCTION: The early identification of asphyxiated infants at high risk of adverse outcomes and the early selection of those who might benefit from neuroprotective therapies are required. A prospective observational study was conducted to determine if there were any early clinical, neuroimaging or neurophysiological parameters that might predict the outcome in term newborns with asphyxia. METHODS: 44 term newborns with acute asphyxia had a cranial ultrasonography (US), electroencephalography (EEG) and clinical examination performed between three and eight hours of life to determine the parameters that might predict outcome. US findings were classified as normal or abnormal (ventricular dilatation or compression and/or focal/diffuse echogenicities). EEG background activity was classified into two categories: normal/mildly abnormal/intermediate, or severely abnormal (low voltage activity or "suppression-burst"). An intrapartum score (based on graded abnormalities of foetal heart monitoring, umbilical arterial base deficit and five-minute Apgar score) and a hypoxic ischaemic encephalopathy (HIE) score (based on graded abnormalities of the neurological and respiratory status at 3-8 hours of life) was also obtained. RESULTS: At one year of life, eight infants had died, six had defaulted follow-up, five had major impairment, two minor impairment and 23 were normal. On univariate analysis, poor outcome (death or major impairment) was associated with abnormal cranial US, severely abnormal EEG and a high HIE score (greater than or equal to 15). The positive predictive value was 54.5, 100 and 100 percent, respectively, while the negative predictive value was 93.8, 80.6 and 80.6 percent, respectively. Combining these factors did not improve the predictive values. CONCLUSION: There was no added advantage in combining EEG or US parameters over a clinical neurological scoring system alone in predicting the outcome of asphyxiated term newborns.

Echocontrast cystosonography versus micturating cystourethrography in the detection of vesicoureteric reflux

Purpose: To compare echocontrast cystosonography (ECS) using in-vivo agitated saline with fluoroscopic micturating cystourethrography (MCU) in the detection and grading of vesicoureteric reflux (VUR). Materials and methods: This was a prospective study of 25 children, who had MCU between 2007 and 2009. ECS was performed and findings documented prior to MCU. Baseline renal and bladder sonograms were obtained. The bladder was filled with normal saline followed by introduction of 10–20 mls of air to generate microbubbles. Detection of VUR was based on two sonographic criteria: (1) presence of microbubbles in the pelvicaliceal system (PCS), and (2) increase in dilatation of the PCS. VUR was graded as (1) Grade I: microbubbles seen in ureter only; (2) Grade II: microbubbles seen in non-dilated PCS; and (3) Grade III-V: microbubbles seen in dilated PCS. The ECS findings were compared using MCU as the gold standard. Results: Of the 50 kidney-ureter (K-U) units studied, ECS detected 9 of 10 K-U units with VUR on MCU. ECS did not detect a Grade II VUR. The sensitivity, specificity, accuracy, positive predictive value and negative predictive value for criterion 1 was 90%, 87.5%, 88%, 64.3% and 97%, respectively, compared to criterion 2 which was 70%, 90%, 86%, 64% and 92%, respectively. The grading of VUR was similar on both ECS and MCU except for one case. Conclusion: ECS using agitated saline was a sensitive technique for the detection of VUR. ECS grading was comparable with MCU grading of VUR.

Epidemiology of childhood nocturnal enuresis in Malaysia.

OBJECTIVES: To estimate the prevalence of nocturnal enuresis in primary school children in Malaysia and to determine the factors associated with primary nocturnal enuresis. METHODS: This was a cross-sectional survey. A total of 3371 self-administered questionnaires were distributed to parents of children aged 7, 9 and 12 years attending four primary schools in the city. The ICD-10 definition of enuresis was used. RESULTS: From an overall response rate of 73.8%, nocturnal enuresis was reported in 200 children (8%), primary nocturnal enuresis in 156 children (6.2%) and secondary nocturnal enuresis in 44 children (1.8%). Fifty-three percent of those with primary enuresis had a positive family history, and 54% had two or more wet nights per week. Eighty-seven percent had not sought any form of treatment despite 74% admitting to being embarrassed. Using logistic regression analysis, only three factors were significant predictors of primary nocturnal enuresis. These were: (i) younger age (P < 0.001); (ii) male sex (P < 0.033); and (iii) Indian ethnic group (P < 0.044) compared to Chinese. CONCLUSION: The prevalence of nocturnal enuresis in urban-dwelling Malaysian children is similar to that reported from Korea and Taiwan but appears to be lower than that reported from developed countries. Predictive factors associated with primary nocturnal enuresis included lower age group, male sex and Indian ethnicity.