Parental Knowledge on Nephrotic Syndrome and Disease Relapse in children.

INTRODUCTION: Parental knowledge on nephrotic syndrome and disease relapse is important for early recognition and treatment of relapse to prevent the complications. Parental knowledge on nephrotic syndrome was reported to be inadequate from published studies. To date, there is no study on parental knowledge on childhood nephrotic syndrome in Malaysia. This study is thus aimed at to determine the level of knowledge on NS and disease relapse among parents of children with nephrotic syndrome and determine factors that influence knowledge on nephrotic syndrome and disease relapse. STUDY DESIGN AND METHODS: This was a cross-sectional study conducted in Paediatric Nephrology Clinic, Hospital Selayang from November 2016 to November 2017. Seventy-eight parents were recruited based on universal sampling. Selfadministered questionnaire in Bahasa Malaysia and English was designed through focus group discussion with five subject matter experts and validated through content validity. Data was analysed using IBM SPSS Statistics 23.0. RESULTS: Majority of parents or guardians (91%) were able to answer more than 50% of the questions correctly. Of these, 56% were able to answer more than 75% of the questions correctly. A 'cut-off' of 75% was defined as good knowledge. Parents of children with frequent relapses had higher parental knowledge and this was statistically significant (p=0.025). CONCLUSION: Parental knowledge on nephrotic syndrome and disease relapse was still inadequate as only 56% parents had good knowledge. The main areas of deficit in parental knowledge were related to medications, infections, home urine dipstick monitoring, and recognition of warning signs during relapse.

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.

Diabetic ketoacidosis at diagnosis of type 1 diabetes mellitus in Malaysian children and adolescents.

BACKGROUND: Diabetic ketoacidosis (DKA) is a late presentation of newly diagnosed type 1 diabetes mellitus (DM) in children. The aim of this study was to determine the clinical characteristics of type 1 DM at presentation so that appropriate actions can be taken to promote early diagnosis. METHODS: This was a retrospective cohort review from a patient registry database. Data on all patients younger than 20 years old diagnosed with type 1 DM who had been registered with the Malaysian Diabetes in Children and Adolescents Registry (DiCARE) from its inception in 2006 until 2009 were analysed. RESULTS: The study included 490 children and adolescents, out of which 57.1% were female. The mean (SD) age at diagnosis was 7.5 (3.7) years, which increased from year 2000 to 2009 [6.6 (3.3) years to 9.6 (3.5) years; p = 0.001]. An increasing percentage of DKA at diagnosis was observed from year 2000 (54.5%) to year 2009 (66.7%), which remained high and leveled between 54.5% and 75.0%. DKA was more common in patients with normal weight (p = 0.002) with no significant association with age, gender, ethnicity and status of family history of diabetes mellitus. CONCLUSION: An increasing trend of age at diagnosis of patients with type 1 DM was observed. Besides that, proportion of DKA at diagnosis had remained high over the past decade. This study found that normal weight was associated with status of DKA, thus more detailed investigations are required to determine the risk factors for DKA.

Diabetic ketoacidosis at diagnosis of type 1 diabetes mellitus in Malaysian children and adolescents

Background: Diabetic ketoacidosis (DKA) is a late presentation of newly diagnosed type 1 diabetes mellitus (DM) in children. The aim of this study was to determine the clinical characteristics of type 1 DM at presentation so that appropriate actions can be taken to promote early diagnosis. Methods: This was a retrospective cohort review from a patient registry database. Data on all patients younger than 20 years old diagnosed with type 1 DM who had been registered with the Malaysian Diabetes in Children and Adolescents Registry (DiCARE) from its inception in 2006 until 2009 were analysed. Results: The study included 490 children and adolescents, out of which 57.1% were female. The mean (SD) age at diagnosis was 7.5 (3.7) years, which increased from year 2000 to 2009 [6.6 (3.3) years to 9.6 (3.5) years; p = 0.001]. An increasing percentage of DKA at diagnosis was observed from year 2000 (54.5%) to year 2009 (66.7%), which remained high and leveled between 54.5% and 75.0%. DKA was more common in patients with normal weight (p = 0.002) with no significant association with age, gender, ethnicity and status of family history of diabetes mellitus. Conclusion: An increasing trend of age at diagnosis of patients with type 1 DM was observed. Besides that, proportion of DKA at diagnosis had remained high over the past decade. This study found that normal weight was associated with status of DKA, thus more detailed investigations are required to determine the risk factors for DKA.

Sociodemographic determinants of glycaemic control in young diabetic patients in peninsular Malaysia.

Recent studies have shown that good glycaemic control can prevent the development of diabetic complications in type 1 and type 2 diabetes. We wished to observe the glycaemic control in patients from different centres in Peninsular Malaysia and the factors that determine it. We recruited 926 patients with diabetes diagnosed before age 40 years from seven different centres, with proportionate representation from the three main ethnic groups. Clinical history and physical examination were done and blood taken for HbA1c and fasting glucose. The overall glycaemic control was poor with geometric mean HbA1c of 8.6% whilst 61.1% of the patients had HbA1c greater than 8%. Glycaemic control in patients with type 2 diabetes varied between various centres and ethnic groups, with the best control obtained in Chinese patients. Significant predictors of HbA1c in both type 1 and type 2 diabetes include access to nurse educators, ethnic background and WHR. In type 2 diabetes, use of insulin was a significant predictor, while in type 1 diabetes, household income was a significant predictor. Socioeconomic status did not have a significant effect in type 2 diabetes. There were no significant differences in the glycaemic control in patients with different educational status. In conclusion, glycaemic control in big hospitals in Malaysia was poor, and was closely related to the availability of diabetes care facilities and ethnic group, rather than socioeconomic status.

Autoimmune markers in young Malaysian patients with type 1 diabetes mellitus.

This study determined the prevalence and significance of autoantibodies to GAD65 (GAD Ab), insulin (IAA), tyrosine-like phosphatase (IA2) and islet-cell (ICA) in a group of 213 young Malaysian Type 1 diabetics, diagnosed before the age of 40 years. Venous blood was taken at fasting, and at 6 minutes post-glucagon (1 mg i.v.). IAA was detected in 47.4%, GAD Ab in 33.8%, IA2 in 8.9% and ICA in 1.4% of the subjects. When based on post-glucagon C-peptide level of 600 pmol/L, 172 (80.7%) patients had inadequate pancreatic reserve, while the remainder 41(19.3%) showed normal response. The autoantibodies, either alone or in combination, were detectable in both groups of patients; higher prevalence in those with poor or no beta-cell function (73.3% versus 46.3%, p = 0.0001). Although the prevalence of GAD Ab was highest in newly diagnosed patients (< 5 years), unlike IA2 and ICA, the marker remained detectable in 24-25% of those patients with long-standing disease. Nineteen patients could probably belong to the "latent autoimmune diabetes in adults (LADA)" subset, where pancreatic reserve was adequate but patients had detectable autoantibodies and insulin-requiring. On the other hand, 68 of the 213 patients (32%) were seronegative, but presented with near or total beta-cell destruction. Thus, as has also been suggested by others, there is indeed etiological differences between the Asian and the Caucasian Type 1 diabetics, and, there is also the possibility that other, but unknown autoantigens are involved in causing the pancreatic damage.

Prevalence of glutamic acid decarboxylase antibodies amongst young Malaysian diabetics.

This study determined the prevalence of glutamic acid decarboxylase antibodies (GAD Ab) in a group of 926 young Malaysian diabetics of three ethnic groups, Malay, Chinese, and Indian. Patients were clinically diagnosed to be Type 1 or Type 2 before the age of 40 years. The overall GAD Ab positivity was 17.4% (161/926), significantly higher in the Type 1 than the Type 2 diabetics (35.5%, 116/329 vs. 7.5%, 45/597, P=0.0001). Compared to GAD Ab negative patients, seropositive diabetics were diagnosed at younger age (21.2+/-0.9 vs. 27.4+/-0.3 y, P=0.0001), had lower fasting (289+/-27.4 vs. 640+/-17.6 pmol/l, P=0.0001) and post-glucagon C-peptide levels (527+/-51.8 vs. 1030+/-28.9 pmol/l, P=0.0001). There were no racial differences in the prevalence of GAD Ab; of the total Type 1, 30.8, 36.4, and 39.4% were Malay, Chinese, and Indian diabetics, respectively and of the total Type 2, 8.8, 8.2, and 4.4% were Malay, Chinese, and Indian diabetics respectively. There was a curvilinear relationship between GAD Ab and the post-glucagon C-peptide levels, suggesting that GAD Ab do play a role in the beta-cells destruction and could be an important immune marker for the LADA group. This study reconfirmed previous reports that the autoimmune mechanisms in the Type 1 Asian diabetics are indeed different from the Caucasians, and further investigations should be carried out to explain the differences.

IGF-1 and IGFBP-3 screening for disorders of growth hormone secretion.

AIM: To assess the value of plasma assays of insulin like growth factor (IGF-1) and insulin like growth factor binding protein 3 (IGFBP-3) in the diagnosis of growth hormone disorders in children and adults. METHODS: Plasma IGF-1 and IGFBP-3 were measured in 47 children referred for the assessment of short stature, 26 adult subjects with hypopituitarism and in 10 adult subjects with acromegaly. Findings were compared with results obtained in 148 normal children and 124 normal adult subjects who comprised the reference range. RESULTS: Levels of both growth factors and especially IGF-1 are highly age dependent in normal children and adults. Six of 47 short children had growth hormone deficiency and in these cases both IGF-1 and IGFBP-3 were close to the lower limit or below the normal reference range. In young children ( < 10 yr) IGFBP-3 was more informative than IGF-1, distinguishing normal short children from those with growth hormone deficiency. IGF-1 levels were raised in all 10 acromegalic adults, eight of whom had normal levels of IGFBP-3. Similarly growth hormone deficient adults were better identified (23 of 26 patients) by IGF-1 whereas IGFBP-3 was subnormal in only eight cases. CONCLUSIONS: Provided results are reviewed in relation to an age related normal reference range, both IGF-1 and IGFBP-3 are simple and convenient screening tests for assessing growth hormone deficiency in children. In adults plasma IGF-1 is the diagnostic test for a disorder of growth hormone excess. Low IGF-1 in an adult with a history of pituitary disease strongly suggests the presence of growth hormone deficiency.

Growth patterns and outcomes in congenital adrenal hyperplasia; effect of chronic treatment regimens.

AIM: To assess outcome (final height and sexual maturation), growth patterns and blood pressure in 16 children with congenital adrenal hyperplasia treated at one institution over a 30 year period. METHODS: Growth patterns and maturation were determined by retrospective review (median follow up period 14 years). Dose adjustment of corticosteroid replacement treatment, sufficient to maintain normal levels of adrenal precursor secretion, was determined using assays of urinary pregnanetriol excretion (up to 1975) or early morning measurements of plasma 17 hydroxy progesterone and plasma renin activity at intervals of 4-6 months. RESULTS: In 7 of 15 patients the growth pattern during infancy was retarded--13 not exceeding the population mean. Catchup growth as steroid dose fell with age was not usually observed. In boys, height potential was further compromised by a significant reduction in growth velocity during puberty. None of nine patients evaluated at final height had attained the target height. During the first year of life, plasma renin activity was suppressed below the reference range in six of nine infants. Despite this, and lower than normal levels of plasma renin activity in childhood, most children were normotensive. CONCLUSIONS: Avoiding salt depletion in infancy and excessive androgen secretion during childhood do not ensure normal growth patterns or normal final height. Impaired final height in congenital adrenal hyperplasia is more likely to be due to over treatment, particularly in infancy when lower doses of corticosteroids may improve height prognosis.