1.Clinical Presentation of Congenital Adrenal Hyperplasia in Selected Multiethnic Paediatric Population
Subashini Chellappah Thambiah ; Zalinah Ahmad ; Zarida Hambali ; Malina Osman ; Munira Mohd Zain ; Fuziah Md Zain ; Janet Hong Yeow Hua
Malaysian Journal of Medicine and Health Sciences 2015;11(1):77-83
A clinical descriptive study was done to determine the sociodemographic, laboratory and clinical
characteristics of patients with congenital adrenal hyperplasia (CAH) referred to Hospital Putrajaya, a
tertiary endocrine centre in Malaysia. Electronic laboratory data of 51 CAH patients were obtained. The
demographics and clinical details of the study population were acquired from a questionnaire completed
by parents of participants. There were 25 males (49%) and 26 females (51%), of which, 58.8% were
Malays. Median age of participants was 4 years whilst median age at diagnosis of CAH was two years.
Parental consanguinity was documented in three patients (5.9%). Patients originated from Johor (19.6%),
Selangor (19.6%), Negeri Sembilan (17.6%) and Kedah (13.7%). Majority of patients were diagnosed
after one week of life (80.4%) although more females were diagnosed under the age of one week
compared to males (p=0.041). Most females presented with ambiguous genitalia (42.3%) [p=0.001]
whereas 72% of males presented with salt wasting (p=0.003). No significant associations between race
and all other variables, though interestingly three Malay patients presented with ambiguous genitalia
and hypertension. Equal gender distribution noted as expected in an autosomal recessive condition,
although not in keeping with other Asian countries. Early diagnosis in females attributed to obvious
genital ambiguity at birth. Varied clinical presentation, although in minority, necessitates genetic studies
for prompt diagnosis and treatment. Considering that majority of patients presented with salt wasting
and the age at diagnosis was delayed, the introduction of a neonatal screening programme is essential
in Malaysia.
Adrenal Hyperplasia, Congenital
2.Glycaemic changes among children and adolescents with Type 1 Diabetes Mellitus before and during Ramadan fasting using continuous glucose monitoring
Sze Teik Teoh ; Suhaimi Hussain ; Janet Yeow Hua Hong
Journal of the ASEAN Federation of Endocrine Societies 2022;37(2):49-59
Objectives:
This study described and compared glycaemic changes with the use of the following Continuous Glucose Monitoring (CGM) metrics: time in range, time in hyperglycaemia and time in hypoglycaemia from retrospective CGM data among children and adolescents with Type 1 Diabetes Mellitus (T1DM), before and during Ramadan to better understand the impact of fasting during this season.
Methodology:
This study was conducted in 2 tertiary centres: Hospital Putrajaya (HPJ) and Hospital Universiti Sains Malaysia (HUSM) from February to May 2020. Muslim T1DM patients between ages 8 to18 who intended to fast during Ramadan were given Ramadan-focused education. CGM iPro2® (Medtronic) was used before and during Ramadan, complemented by finger-prick glucose monitoring or self-monitoring of blood glucose (SMBG).
Results:
Of the 32 patients, only 24 (12 female) were analysed. Mean age was 13.6 ± 3.1 years old, mean HbAlc was 9.6 ± 1.9% and mean duration of illness was 5.4 ± 3.4 years. Majority (91.7%) were on multiple dose injections (MDI) while only 8.3% were on continuous subcutaneous insulin infusion (CSII). All fasted in Ramadan without acute complications. Retrospective CGM analysis revealed similar results in time in range (TIR), time in hyperglycaemia and time in hypoglycaemia before and during Ramadan, indicating no increased hypoglycaemic or hyperglycaemic events related to fasting. Glycaemic variability before Ramadan as measured by the LBGI, HBGI and MAG, were similar to values during Ramadan.
Conclusion
Ramadan fasting among T1DM children and adolescents, by itself, is not associated with short-term glycaemic deterioration. T1DM youths can fast safely in Ramadan with the provision of focused education and regular SMBG.
3.Different forms of hypothyroidism in infants with Maternal Graves’ Disease: A case series
Alexis Anand Dass Lordudass ; Jeanne Sze Lyn Wong ; Nalini Selveindran ; Janet Yeow Hua Hong
Journal of the ASEAN Federation of Endocrine Societies 2024;39(1):120-124
Infants of mothers with Graves’ disease (GD) may develop central hypothyroidism (CH) due to exposure of the foetal hypothalamic-pituitary-thyroid axis to higher-than-normal thyroid hormone concentrations, primary hypothyroidism (PH) due to transplacental passage of maternal thyroid stimulating hormone receptor antibody (TRAb), antithyroid drugs (ATD) or thyroid dysgenesis secondary to maternal uncontrolled hyperthyroidism. We describe two infants with PH and four infants with CH born to mothers with poorly controlled Graves' disease. All infants required levothyroxine and had normal developmental milestones. While national guideline consensus for high thyroid stimulating hormone (TSH) on neonatal screening is well-established, thyroid function tests (TFTs) should be serially monitored in infants with low TSH on screening, as not all mothers with Graves’ disease are diagnosed antenatally.
Infant
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Hypothyroidism
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Congenital Hypothyroidism