Polyostotic Fibrous Dysplasia: Response to Zoledronic Acid Jia Cheng Ong, Ahmad Hadif Zaidin Samsudin, Suhaimi Hussain Malaysian Journal of Medicine and Health Sciences, 2025 Fibrous Dysplasia (FD) is a rare developmental bone disorder in which fibro-osseous tissue replaces normal bone tissue. FD can manifest as either monostotic or polyostotic. Polyostotic FD is often associated with McCune-Albright syndrome (MAS). Treatment of FD varies due to its diverse clinical manifestations. Bisphosphonate therapy, with its antiresorptive properties, is commonly used as part of the treatment. Two commonly used bisphosphonates are pamidronate and alendronate. However, literature on the use of intravenous zoledronate is limited. In this report, we share our experience using zoledronate to treat polyostotic fibrous dysplasia in a 10-year-old girl.
A Focal Form of Diazoxide-resistant Congenital Hyperinsulinism with Good Response to Long-acting Somatostatin Suhaimi Hussain, Sarah Flanagan, Nurshafinaz Salmah Mohd Fezal Journal of the Asean Federation of Endocrine Societies, 2024 A four-year-old female who was born term via spontaneous vaginal delivery with a birth weight of 3.4 kg had an onset of persistent hypoglycaemia at the 6th hour of life. She was diagnosed with congenital hyperinsulinism based on high glucose load, negative ketone and a good response to glucagon. Genetic workup revealed the presence of ATP Binding Cassette Subfamily C Member 8 (ABCC8 genes) mutation which indicated a focal form of congenital hyperinsulinism. She was resistant to the standard dose of oral diazoxide but responded to subcutaneous somatostatin. At the age of 3 years and 6 months, multiple daily injections of somatostatin were replaced with a long-acting monthly somatostatin analogue. With the present treatment, she had better glycaemic control, normal growth and was able to stop tube feeding.
R243W Mutation in Thyroid Hormone Resistance Syndrome Beta: A Case Report Jia Cheng Ong, W Mohd Hilmi W Omar, Tuan Salwani Tuan Ismail, Krishna Chatterjee, Suhaimi Hussain Journal of the Asean Federation of Endocrine Societies, 2024 A three-year-old female with a history of recurrent tonsillitis was investigated for failure to thrive and global developmental delay. Clinically, she had a triangular face with low-set ears and intermittent tachycardia. She had growth failure with her weight under the third centile while her height was within normal limits. Other systemic examinations were unremarkable. The presence of an elevated free T4 (FT4) with an inappropriately high thyroid stimulating hormone (TSH) in this patient raised the clinical suspicion of Thyroid Hormone Resistance Syndrome. DNA sequencing confirmed the diagnosis, which showed R243W gene mutation in Thyroid Hormone Receptor-Beta1 (THRB1).
Endocrine Disorders in Childhood Brain Tumour Survivors: A Single-Centre Study , Nurul Wahidah Ramezan, Suhaimi Hussain, , Norsarwany Mohamad, , Najib Majdi Yaacob, and Journal of the Asean Federation of Endocrine Societies, 2024 Objective: The study aims to determine the prevalence and risk factors for endocrine disorders in childhood brain tumour survivors. Methodology: Included in the study were 124 childhood brain tumour survivors aged 18 years old or younger with either stable disease or in remission, and had survived for at least 2 years after diagnosis. Demographic data (age at diagnosis, gender, ethnicity, socioeconomic status), clinical clues for endocrine disorders, anthropometrics (weight, height, midparental height), pubertal staging, tumour-related characteristics, treatment modalities and endocrine laboratory measurements at diagnosis and during follow up were obtained. Logistic regression was applied to evaluate risk factors for endocrine disorders in childhood brain tumour survivors. Results: The prevalence of endocrine disorders in childhood brain tumour survivors was 62.1%. The risk factors were high BMI [adjusted odds ratio (OR) 1.29, 95% CI: 1.12 to 1.5], high-risk site [adjusted odds ratio (OR) 7.15, 95% CI: 1.41 to 36.3] and chemotherapy [adjusted odds ratio (OR) 0.18, 95% CI: 0.05 to 0.62]. Conclusion: The prevalence of endocrine disorders in childhood brain tumour survivors in our centre was 62.1%. The significant risk factors were high BMI, tumour location (suprasellar and intrasellar) and chemotherapy.
Thyroid autoimmunity and autoimmune thyroid disease in Malaysian girls with Turner syndrome: An understudied population Yee L Lee, Azriyanti A Zaini, Arini N Idris, Raja A Abdullah, Jeanne SL Wong, Joyce SS Hong, Suhaimi Hussain, Poi G Lim, Song H Lim, Noor SM Nor, Loo L Wu, Muhammad Y Jalaludin Journal of Paediatrics and Child Health, 2023 AIMS Knowledge on the spectrum of thyroid disorders amongst Turner syndrome (TS) patients in Southeast Asia is limited. This study aimed to evaluate the prevalence of thyroid autoimmunity, the spectrum of autoimmune thyroid disease and association with age and karyotype amongst Malaysian TS girls. METHODS A cross-sectional study was conducted at 11 paediatric endocrine units in Malaysia. Blood samples for antithyroglobulin antibodies, antithyroid peroxidase antibodies and thyroid function test were obtained. In patients with pre-existing thyroid disease, information on clinical and biochemical thyroid status was obtained from medical records. RESULTS Ninety-seven TS patients with a mean age of 13.4 ± 4.8 years were recruited. Thyroid autoimmunity was found in 43.8% of TS patients. Nineteen per cent of those with thyroid autoimmunity had autoimmune thyroid disease (Hashimoto thyroiditis in 7.3% and hyperthyroidism in 1% of total population). Patients with isochromosome X and patients with 45,X mosaicism or other X chromosomal abnormalities were more prone to have thyroid autoimmunity compared to those with 45,X karyotype (OR 5.09, 95% CI 1.54-16.88, P = 0.008 and OR 3.41, 95% CI 1.32-8.82, P = 0.01 respectively). The prevalence of thyroid autoimmunity increased with age (33.3% for age 0-9.9 years; 46.8% for age 10-19.9 years and 57.1% age for 20-29.9 years) with autoimmune thyroid disease detected in 14.3% during adulthood. CONCLUSION Thyroid autoimmunity was significantly associated with the non 45,X karyotype group, particularly isochromosome X. Annual screening of thyroid function should be carried out upon diagnosis of TS until adulthood with more frequent monitoring recommended in the presence of thyroid autoimmunity.
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, , and Journal of the Asean Federation of Endocrine Societies, 2022 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.
Neonatal Outcomes of Pregnancies Complicated by Maternal Hyperthyroidism Adlina Awanis Mamat, , Noraida Ramli, Najib Majdi Yaacob, Suhaimi Hussain, , , and Journal of the Asean Federation of Endocrine Societies, 2022 Objective This study aimed to determine the proportion, clinical characteristics, hormonal status, median time for normalization of serum thyroxine (FT4) and thyroid-stimulating hormone (TSH) and factors affecting time to thyroid function test (TFT) normalization of neonates born to mothers with maternal hyperthyroidism admitted in our institution. Methodology This was a retrospective cohort study that included 170 newborns admitted to the Neonatal Intensive Care Unit (NICU) of Hospital Universiti Sains Malaysia (HUSM) with a history of maternal hyperthyroidism from January 2013 until December 2018. We analyzed their baseline demographic and clinical characteristics, maternal thyroid status and antibody levels. Finally, we analyzed newborn thyroid function and thyroid antibodies. Results The proportion of neonates born to mothers with maternal hyperthyroidism was 0.8% (170 of 20,198 neonates within the study period). Seven (4.1%) developed overt hyperthyroidism, while four (2.4%) had thyroid storm. The median time for thyroid function test normalization was 30 days (95% CI: 27.1 to 32.8). The median time for TFT normalization was longer among neonates of mothers with positive thyroid antibodies [46.6 days (95% CI, 20.6 to 39.4)] and of mothers who received anti-thyroid treatment [31.7 days (95% CI, 23.5 to 39.9)]. Conclusion Neonates born to mothers with hyperthyroidism is uncommon. These babies were observed to have a longer time for normalization of thyroid function tests if their mothers had thyroid antibodies or received anti-thyroid treatment.
Prevalence of Metabolic Syndrome and its Associated Risk Factors in Pediatric Obesity Wan Muhammad Najib Wan Mahmud Sabri, , Rashdan Zaki Mohamed, Najib Majdi Yaacob, Suhaimi Hussain Journal of the Asean Federation of Endocrine Societies, 2022 Objective We aimed to study the prevalence of metabolic syndrome (MetS) and the factors associated with metabolic syndrome among obese children. Methodology We recruited 175 subjects, aged 7 to 18 years old, referred for obesity. We studied their demography (age, gender, ethnicity, family background), performed clinical/auxological examinations [weight, height, body mass index (BMI), waist circumference (WC), blood pressure (BP)], and analyzed their biochemical risks associated with metabolic syndrome [fasting plasma glucose (FPG), fasting lipid profile (FLP), fasting insulin, liver function tests (LFT)]. MetS was identified according to the criteria proposed by the International Diabetes Federation (IDF) for pediatric obesity. Multiple logistic regression models were used to examine the associations between risk variables and MetS. Results The prevalence of metabolic syndrome among children with obesity was 56% (95% CI: 48.6 to 63.4%), with a mean age of 11.3 ± 2.73 years. Multiple logistic regression analysis showed age [adjusted odds ratio (OR) 1.27, 95% CI: 1.15 to 1.45] and sedentary lifestyle (adjusted OR 3.57, 95% CI: 1.48 to 8.59) were the significant factors associated with metabolic syndrome among obese children. Conclusion The prevalence of metabolic syndrome among obese children referred to our centers was 56%. Older age group, male gender, birth weight, sedentary lifestyle, puberty and maternal history of gestational diabetes mellitus (GDM) were found to be associated with MetS. However, older age group and sedentary lifestyle were the only significant predictors for metabolic syndrome.
Rate of weight gain and its association with homeostatic model assessment-insulin resistance (HOMA-IR) among obese children attending paediatric endocrine clinic, hospital universiti Sains Malaysia Siti Hasmiza Che Mat, , Najib Madji Yaacob, Suhaimi Hussain Journal of the Asean Federation of Endocrine Societies, 2021 Objective We aimed to study the median time to gain weight from baseline and factors that were associated with rate of weight gain among obese children attending pediatric endocrine clinic Hospital USM. Methodology We recruited 70 participants with the mean age of 10.1 ± 2.94 years with exogenous or simple form of obesity from June 2019 until September 2020. We analyzed their demography (age, gender, ethnicity, family background), measured their anthropometry (weight, height, BMI) and monitored monthly weight increment and finally analyzed their HOMA-IR at baseline and after 6 months of follow up. Results The mean time to gain 5 kg from baseline was 16 weeks (95% CI): (15.2, 16.7). Multivariate analysis showed only HOMA-IR after 6 months was a significant predictor affecting time to gain 5 kg; Adjusted HR: (95% CI) 1.617 (1.232, 2.123), (p=0.001). Conclusion The time to gain 5 kg from baseline weight was increased 1.6 times in the presence of insulin resistance at 6 months follow up in patients with obesity. More intensive education and closed follow-up are recommended for children with obesity.
