MyMedR

Fulltext Final height in growth-hormone-treated children with idiopathic growth hormone deficiency: the Malaysian experience

Ooi HL, Wu LL

Med J Malaysia, 2011 Dec;66(5):479-83.

PMID: 22390105

Fulltext Insulin pump therapy in children and adolescents with Type 1 Diabetes: improvements in glycemic control and patients' satisfaction -- Hospital UKM experience

Ooi HL, Wu LL

Med J Malaysia, 2011 Oct;66(4):308-12.

PMID: 22299548

Hospital UKM (Universiti Kebangsaan Malaysia) introduced the use of insulin pump therapy in children and adolescents with Type 1 Diabetes in Malaysia in April 2004. This study aims to evaluate the effectiveness of pump therapy and its impact on metabolic control among patients from our institution. Insulin pump therapy resulted in sustainable improvement in glycemic control throughout the six years of treatment with reduction in HbA1c in the first two years of pump use was statistically significant. The BMI SDS showed an increase trend but the changes before and after pump use was insignificant. There is also high level of treatment satisfaction reported among our insulin pump patients.

Fulltext Three cases of permanent neonatal diabetes mellitus: genotypes and management outcome

Ooi HL, Wu LL

Singapore Med J, 2012 Jul;53(7):e142-4.

PMID: 22815030

Neonatal diabetes mellitus (DM) is defined as insulin-requiring DM in the first six months of life. Unlike type 1 DM, it is a monogenic disorder resulting from a de novo mutation in the genes involved in the development of the pancreas, β-cell mass or secretory function. The majority of neonatal DM cases are caused by a heterozygous activating mutation in the KCNJ11 or ABCC8 genes that encode the Kir6.2 and SUR1 protein subunits, respectively, in the KATP channel. Sulphonylurea, a KATP channel inhibitor, can restore insulin secretion, hence offering an attractive alternative to insulin therapy. We report three cases of neonatal DM and their genetic mutations. Two patients were successfully switched over to sulphonylurea monotherapy with resultant improvement in the quality of life and a more stable blood glucose profile. Patients with neonatal DM should undergo genetic evaluation. For patients with KCNJ11 and ABCC8 gene mutation, oral sulphonylurea should be considered.

Fulltext Penile length of term newborn infants in multiracial Malaysia

Ting TH, Wu LL

Singapore Med J, 2009 Aug;50(8):817-21.

PMID: 19710983

Micropenis may be an important sign of underlying hypogonadism or pituitary hypofunction in the neonatal period. Penile lengths of normal newborns have been reported in many Western populations. However, the data may not be applicable in the Asian or the multiracial Malaysian population. Our study aimed to establish the normal penile length and testicular volume in term newborn infants in the major ethnic groups in Malaysia.

Fulltext Clinical Features of Girls with Turner Syndrome in a Single Centre in Malaysia

Lee YL, Wu LL

J ASEAN Fed Endocr Soc, 2019;34(1):22-28.

PMID: 33442133 DOI: 10.15605/jafes.034.01.05

Objectives: Diagnosis of Turner syndrome in Malaysia is often late. This may be due to a lack of awareness of the wide clinical variability in this condition. In our study, we aim to examine the clinical features of all our Turner patients during the study period and at presentation.

Methodology: This was a cross-sectional study. Thirty-four (34) Turner patients were examined for Turner-specific clinical features. The karyotype, clinical features at presentation, age at diagnosis and physiologic features were retrieved from their medical records.

Results: Patients with 45,X presented at a median age of 1 month old with predominantly lymphoedema and webbed neck. Patients with chromosome mosaicism or structural X abnormalities presented at a median age of 11 years old with a broader clinical spectrum, short stature being the most common presenting clinical feature. Cubitus valgus deformity, nail dysplasia and short 4th/5th metacarpals or metatarsals were common clinical features occurring in 85.3%-94.1% of all Turner patients. Almost all patients aged ≥2 years were short irrespective of karyotype.

Conclusion: Although short stature is a universal finding in Turner patients, it is usually unrecognised till late. Unlike the 45,X karyotype, non-classic Turner syndrome has clinical features which may be subtle and difficult to discern. Our findings underscore the importance of proper serial anthropometric measurements in children. Awareness for the wide spectrum of presenting features and careful examination for Turner specific clinical features is crucial in all short girls to prevent a delay in diagnosis.

Persistent neonatal hypoglycaemia as a result of hypoplastic pituitary gland

Yong SC, Boo NY, Wu LL

Br J Hosp Med (Lond), 2006 Jun;67(6):326.

PMID: 16821748 DOI: 10.12968/hmed.2006.67.6.21296

Case series of testicular adrenal rest tumours in boys with congenital adrenal hyperplasia: A single centre experience

Karen Leong SW, Wu LL

Med J Malaysia, 2019 02;74(1):92-93.

PMID: 30846672

Testicular adrenal rest tumours (TART) are aberrant adrenal tissue within the testes (1). Although benign, they can lead to obstruction of the seminiferous tubules and infertility in patients with congenital adrenal hyperplasia (CAH). We report six boys who developed TART, a complication of CAH. Diagnosis was confirmed by ultrasound and testicular vein sampling of elevated 17-hydroxyprogesterone (17-OHP) levels. Glucocorticoids dosages were increased 1½-2 folds to suppress size of the aberrant adrenal tissues. Despite reductions in 17-OHP, the lesions remained unchanged. Three patients had testis-sparing surgery to excise the TART and to preserve normal testicular tissues.

Mutational characterization of congenital adrenal hyperplasia due to 21-hydroxylase deficiency in Malaysia

Balraj P, Lim PG, Sidek H, Wu LL, Khoo AS

J. Endocrinol. Invest., 2013 Jun;36(6):366-74.

PMID: 23027774 DOI: 10.3275/8648

Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21-OHD) is a common autosomal recessive disorder. Our objective was to identify the 21-hydroxylase active gene, CYP21A2 mutations in Malaysian 21-OHD patients using different techniques.

Episodic vomiting and headache in children: consider occipital epilepsy

Lope RJ, Wong SW, Wu LL

J Paediatr Child Health, 2010 Apr;46(4):204-6.

PMID: 20412414 DOI: 10.1111/j.1440-1754.2009.01662.x

Children with occipital seizures often have ictal autonomic symptoms such as pallor and vomiting and lack motor manifestations. This has lead to misdiagnosis of occipital seizures in children. The following case report highlights the clinical features of a child with occipital epilepsy misdiagnosed as having migraine. The aetiology of symptomatic occipital epilepsy will be discussed.

Fulltext Audit of newborn screening programme for congenital hypothyroidism

Zarina AL, Rahmah R, Bador KM, Ng SF, Wu LL

Med J Malaysia, 2008 Oct;63(4):325-8.

PMID: 19385494 MyJurnal

Newborn screening for congenital hypothyroidism (CH) was implemented in Hospital UKM in December 2004 using cord blood sample. From the audit over a period of 25 months, a total of 13,875 newborn babies were screened with a coverage of 98.8%. From this cohort, the mean recall rate was 0.32%; unfortunately the mean percentage of recalled babies that came for retesting was only 79.5%. In addition, the mean sample rejection rate was high, i.e. 2.2%. Two babies were diagnosed to have CH. These findings implied that whilst the coverage of screening was good, there is a need for regular surveillance of performance of both clinical and laboratory personnel. In addition, a more concerted effort should be carried out to promote community awareness of such a programme.

Measurement of androstenedione levels by an in-house radioimmunoassay

Lo MS, Ng ML, Wu LL, Khalid BA

Malays J Pathol, 1996 Jun;18(1):53-8.

PMID: 10879225

An in-house radioimmunoassay (RIA) for the measurement of androstenedione levels in serum was established and validated. Levels of androstenedione were measured by RIA using serum samples from various normal population groups and patients with congenital adrenal hyperplasia (CAH). Analytical recovery and linearity results were > 95%, while intra- and inter-assay CVs were < 10% and < 22% respectively. The assay sensitivity was 0.5 nmol/l or 25 fmol/tube. In normal population groups, the highest androstenedione levels were found in preterm neonates (1.6-12.4 nmol/l), followed by adult females (1.5-10.2 nmol/l), adult males (1.6-8.0 nmol/l) and term neonates (0.8-8.8 nmol/l), while the lowest values were observed in prepubertal children (0.5-3.4 nmol/l). There were no significant differences in diurnal variation and between follicular and luteal phases. The range of androstenedione levels in untreated or poorly controlled CAH patients (7.6-355.0 nmol/l, median 42.5 nmol/l, n = 20) were significantly higher (p < 0.001) than the upper normal limit of 3.4 nmol/L for prepubertal children. The normal androstenedione reference ranges for paediatric and adult groups have thus been established.

Fulltext Hyperthyroid graves disease--a 5 year retrospective study

Tan TT, Ng ML, Wu LL, Khalid BA

Med J Malaysia, 1989 Sep;44(3):224-30.

PMID: 2626137

The clinical, biochemical and immunological features of 180 patients with hyperthyroid Graves' disease managed at the Universiti Kebangsaan Malaysia (UKM) Endocrine Clinic from 1983 to 1987 were examined. The prevalence of the disease is highest in Chinese and lowest in Indians. The female: male ratio is 2.8:1. Hypokalaemic periodic paralysis and hypercalcaemia were present in 5.0% and 1.7% of the cases respectively. Pretibial myxoedema was extremely rare. Thyrotropin - binding inhibitory immunoglobulins, anti-thyroglobulin and anti-microsome antibodies were positive in 61.5%, 25.8% and 42.3% of the patients respectively. A eumetabolic state could be achieved in the majority of patients with antithyroid drugs alone. Definitive therapy with subtotal thyroidectomy or radioiodine were needed in 31.3% of cases.

Effects of iodine deficiency on insulin-like growth factor-I, insulin-like growth factor-binding protein-3 levels and height attainment in malnourished children

Wan Nazaimoon WM, Osman A, Wu LL, Khalid BA

Clin Endocrinol (Oxf), 1996 Jul;45(1):79-83.

PMID: 8796142

The expression and synthesis of IGF-I and IGFBP-3 have been shown to be regulated by hormones and nutrition. We study the effects of malnutrition and iodine deficiency on these growth factors and the height attainment of a group of children.

Fulltext Congenital hypothyroid screening using cord blood TSH

Wu LL, Sazali BS, Adeeb N, Khalid BA

Singapore Med J, 1999 Jan;40(1):23-6.

PMID: 10361481

Clinical diagnosis of congenital hypothyroidism (CH) is difficult at birth without neonatal screening. In line with the priorities of the national health services in Malaysia towards preventive medicine, early diagnosis and treatment of CH is emphasised. We conducted a pilot study at Kuala Lumpur's Maternity Hospital between April 1995 and November 1995 to estimate the incidence of CH and also evaluated the problems associated with large-scale neonatal screening using a commercial TSH kit on cord bloodspots.

Fulltext A rare case of ambiguous genitalia

Ng SF, Boo NY, Wu LL, Shuib S

Singapore Med J, 2007 Sep;48(9):858-61.

PMID: 17728969

Genes on the Y chromosome are essential for normal sex determination and sex differentiation of male genitalia. However, genes on the X chromosome and other autosomes have been shown to be anti-testes and have a detrimental effect on this process. Addition of X chromosomes to the 46,XY karyotype results in seminiferous tubules dysgenesis, hypogonadism and malformed genitalia. We report a term male newborn with 49,XXXXY syndrome presenting with ambiguous genitalia, multiple extra-gonadal anomalies, facial dysmorphism, and radioulnar synostosis.

Usefulness and limitations of an in-house direct radioimmunoassay for 17-hydroxyprogesterone in serum

Lo MS, Ng ML, Wu LL, Azmy BS, Khalid BA

Malays J Pathol, 1996 Jun;18(1):43-52.

PMID: 10879224

Since conventional radioimmunoassays (RIA) for measurement of 17-hydroxyprogesterone (17-OHP) in serum samples require a laborious solvent extraction step, a direct and rapid in-house RIA was developed for early diagnosis and management of congenital adrenal hyperplasia (CAH). In-house rabbit anti-17-OHP antiserum, tritium labelled 17-OHP and dextran-coated charcoal were used in assay buffer with low pH 5.1 and preheated serum samples. Both inter- and intra-assay CVs were < 10% and the sensitivity was 1.2 nmol/l or 12 fmol/tube. Results from the direct assay correlated well with values from an extraction assay, r = 0.88 in samples from CAH patients, r = 0.85 in adults and children, 0.69 and 0.40 in term and preterm neonates respectively, 0.66 and 0.63 in luteal phase and third trimester pregnancy; p < 0.001 in all groups except p < 0.05 in preterm neonates. However, results from the direct assay were two to three times higher in serum samples from CAH patients, normal adults and children, but were five to seven times higher in pregnancy and term neonates and thirty times higher in preterm neonates. The markedly elevated levels measured by the direct assay are probably due to cross-reactivities with water-soluble steroid metabolites such as 17-hydroxypregnenolone sulphate and dehydroepiandrosterone sulphate (DHEAS). Although the direct assay is only useful as a screening test for preterm babies, it can be used for both diagnosis and monitoring of treatment of CAH in all other age groups.

Thyroid function and pubertal development in malnutrition

Ali O, Tan TT, Sakinah O, Khalid BA, Wu LL, Wan Nazaimoon WM, et al.

Ann Acad Med Singap, 1994 Nov;23(6):852-5.

PMID: 7741498

Thyroid function and pubertal development of aborigines (Orang Asli) and Malays at different socioeconomic strata were assessed among 1136 subjects aged 7 years and above. Anthropometric measurements, goitre and pubertal staging were done. Serum thyroxine (T4), triiodothyronine (T3) and growth hormone were measured using radioimmunoassays (RIA) and serum thyroid stimulating hormone (TSH) by immunoradiometric assays (IRMA). It was found that serum T3 in children was significantly higher in Malays from rural areas, girls and children aged less than 13 years. However, in adults, T3 was significantly associated with anthropometric indices. On the contrary, serum T4 levels were higher among children from urban areas. In adults, serum T4 levels were significantly related to nutritional status and they increased according to the levels of social development, being lowest in remote areas and highest in urban areas. However, serum TSH levels were significantly higher in Orang Asli at all ages and among malnourished children. By using multiple regression, apart from age, gender and ethnicity, nutritional status was a significant predictor for T3 levels in children and adults. Presence of goitre was an important factor which determined the T4 levels in children and adults after controlling for other factors. It was also a predictor for TSH levels in children but not in adults. Fasting serum growth hormone (GH) levels were significantly higher among less privileged groups and decreased according to social development. Serum growth hormone was negatively correlated with anthropometric indices and had a significant association with malnutrition.(ABSTRACT TRUNCATED AT 250 WORDS)

Prevalence of NIDDM and impaired glucose tolerance in aborigines and Malays in Malaysia and their relationship to sociodemographic, health, and nutritional factors

Ali O, Tan TT, Sakinah O, Khalid BA, Wu LL, Ng ML

Diabetes Care, 1993 Jan;16(1):68-75.

PMID: 8422835 DOI: 10.2337/diacare.16.1.68

OBJECTIVE: To determine the prevalence of diabetes mellitus and IGT in different ethnic groups living in the same physical environment and to find their relationship to nutritional status and dietary intake.

RESEARCH DESIGN AND METHODS: The study was conducted among Malays and Orang Asli in six rural and urban locations in Malaysia. OGTTs were performed on 706 adult subjects > or = 18 yr of age. WHO criteria were used for diagnosing diabetes mellitus and IGT.

RESULTS: The overall prevalence of diabetes mellitus and IGT among Orang Asli was 0.3 and 4.4% compared with 4.7 and 11.3%, respectively, among Malays. This increased prevalence of glucose intolerance among Malays was associated with higher levels of social development. Among rural Malays, the crude prevalence of diabetes in a traditional village was 2.8% and in the land scheme was 6.7%, whereas urban Malays had a prevalence of 8.2%. In contrast, the prevalence of IGT (10.5-14.8%) was higher among rural Malays, compared with 9.6% among urban Malays. Ethnic group, > or = 40 yr of age, an income > M$250, fewer daily activity, and obesity were associated with a higher prevalence of diabetes.

CONCLUSIONS: Diabetes mellitus and IGT, which were more common among Malays than Orang Asli, were associated with more affluent life-styles and modernization.

Fulltext Effect of gender and age on fasting serum growth hormone levels in normal subjects

Nazaimoon WM, Ng ML, Osman A, Tan TT, Wu LL, Khalid BA

Med J Malaysia, 1993 Sep;48(3):297-302.

PMID: 8183142

Fasting growth hormone (GH) level is an important reference level in dynamic tests of GH secretion. Other studies have demonstrated sex and age variation in the rate of GH secretion. We analysed fasting serum samples from 377 normal subjects (193 males and 184 females, age range 6 to 81 years old), using our in-house enzyme immunoassay. We found sex differences in fasting GH levels to be only significant in the prepubertal children (Tanner stage I), being higher in girls than in age-matched boys (p < 0.05). Both sexes showed age-dependent changes in fasting GH levels (p < 0.001); highest levels were achieved at puberty and subsequently declined with advancing age. Hence, the physiological sex difference and age-dependency in GH secretion can also be demonstrated in single fasting samples.

Factors associated with control of type I diabetes in Malaysian adolescents and young adults

Tan SM, Shafiee Z, Wu LL, Rizal AM, Rey JM

Int J Psychiatry Med, 2005;35(2):123-36.

PMID: 16240970 DOI: 10.2190/EQ71-RMWV-6CEJ-1DGM

Objectives: To examine the association between ethnicity, depression, quality of life, and diabetic control in Malaysian adolescents and young adults with type I diabetes mellitus.

Methods: Fifty-two outpatients with type I diabetes (mean age 15.5 years) who attended a Diabetes Clinic were included. The level of HbA1c was the measure of diabetes control used (better control defined as HbA1c < 10%). Other variables were measured through questionnaires (e.g., depressive symptoms, quality of life), computerized diagnostic interviews (major depression), and medical records (e.g., demographic, family circumstances, compliance with treatment).

Results: Ethnic Chinese youth showed better diabetic control than Malays and Indians (mean HbA1c 9.1%, 10.3%, and 11.0% respectively). Young people with better diabetic control (HbA1c < 10%) were more likely to have better quality of life and less likely to live in problematic families. When the cut-off for diabetic control was stricter (HbA1c < or = 8%), the young person's compliance was the main predictor of poor control. Family problems were also associated with poor control but to a lesser extent. The initial association between poorer diabetes control and depression became non-significant when quality of life was taken into account.

Conclusions: There are ethnic differences in juvenile diabetic control in this Malaysian sample which need to be understood further. Previous findings of an association between quality of life and glycemic control were verified but different definitions of good control showed different associations with individual and environmental variables. Clinicians' awareness and early intervention for psychosocial problems (for example, inadequate family support) could improve diabetes control.

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