1.Conundrum in the Management of a Rare Cause of Cushing Syndrome
Wee Mee Cheng ; Lit Sin Yong ; Poh Shean Wong ; Nor Afidah binti Karim ; Noor Lita Adam
Journal of the ASEAN Federation of Endocrine Societies 2026;41(S1):32-
Introduction:
Primary bilateral macronodular adrenal hyperplasia
(PBMAH), characterized by bilateral adrenal macronodules
>1 cm, is a rare genetic disease contributing to <2% of
Cushing syndrome.
Case:
A 56-year-old male with underlying diabetes mellitus
and hypertension presented with worsening proximal
muscle weakness. Detailed neurological assessment was
unremarkable. Re-assessment revealed more apparent
cushingoid features, prompting referral to endocrinology.
Basal cortisol was markedly elevated at 957 nmol/L (145.4–
619.4 nmol/L). Unsuppressed cortisol after 1 mg overnight
dexamethasone and low-dose dexamethasone test confirmed
Cushing Syndrome. Suppressed adrenocorticotropic
hormone (ACTH <0.33 pmol/L) suggested autonomous
cortisol secretion from adrenal glands. Computed
tomography adrenal reported massively enlarged
hypodense multinodular adrenal glands of varying sizes.
Oral ketoconazole, an adrenal steroidogenesis inhibitor,
was initiated. Despite careful titration to keep serum
cortisol 400–500 nmol/L, he experienced glucocorticoid
withdrawal syndrome. Bilateral adrenalectomy was
planned but deferred as he continued to lose weight despite
persistent hypercortisolism. Extensive investigations
were conducted for possible opportunistic infections or
malignancy. His chest X-ray revealed a suspicious right
lung cavity. Positive serum galactomannan and bronchial
alveolar lavage galactomannan suggested pulmonary
aspergillosis. Voriconazole was introduced, with drug-drug
interaction judiciously addressed. He underwent bilateral
adrenalectomy and required lifelong glucocorticoid and
mineralocorticoid replacement. Histopathological report confirms bilateral macronodular adrenocortical disease. A
referral to a genetic clinic was made to explore the potential
hereditary basis.
Conclusion
PBMAH is a highly heterogeneous disease with variable
presentation and a wide spectrum of hypercortisolism.
Definitive management remained controversial and
individualized. Multidisciplinary discussion is crucial,
and genetic study is highly recommended for long-term
prognostication and familial screening.
2.Understanding Diabetes Literacy in Seremban: Predictive Factors and Clinical Implications
Wee Mee Cheng ; Nadiah Aminah Azizan ; Wan Farahiyah Wan Muhmad ; Aun Aun Chua ; Poh Shean Wong ; Lit Sin Yong ; Nor Afidah Karim ; Noor Lita Adam
Journal of the ASEAN Federation of Endocrine Societies 2026;41(S1):35-
Introduction:
Diabetes literacy represents personal knowledge and
competencies related to diabetes mellitus (DM) among
people living with DM (PLWD). It is a crucial component
for optimizing care and empowering effective selfmanagement.
Methodology:
A cross-sectional study was conducted at Hospital Tuanku
Ja’afar Seremban (HTJS) and Klinik Kesihatan Seremban
(KKS) using a validated simplified Diabetes Knowledge
Test (DKT). DKT assessed understanding in six domains,
namely, awareness, lifestyle modification, diet and monitoring, general knowledge, preventive screening, and
insulin. A score of >75% was classified as adequate diabetes
literacy. Significant predictive factors identified from univariate Pearson Chi Square were further analyzed using
multivariate logistic regression.
Results:
A total of 250 PLWDs, with equal contribution from each
centre, were recruited with a mean (SD) age of 50.3 (13.6)
years, 58.4% female, and 43.2% Malays. A total of 21.2%
demonstrated adequate diabetes literacy. The results
demonstrated the highest proficiency in the preventive
screening domain (92.6%) and the lowest in the awareness
domain (50.2%). Unemployed PLWDs had significantly
lower odds of adequate diabetes literacy (odds ratio [OR]
= 0.20, p = 0.010). Similarly, PLWDs with hemoglobin A1c
levels >10% exhibited approximately 2.6-fold lower odds
of adequate diabetes literacy. In contrast, PLWDs who
reported healthcare personnel as their primary source of
diabetes-related information had significantly higher odds
of adequate literacy (OR = 1.59, p = 0.017). No significant
association was demonstrated between diabetes literacy
and frequency of hospital admissions, medication
adherence, and diabetes-related target organ damage
Conclusion
Diabetes literacy among PLWD in HTJS and KKS is
suboptimal, with notable gaps in awareness of the disease.
Targeted, culturally tailored education strategies are
essential to address these disparities and to empower them
for effective diabetes self-management.
Literacy
;
Diabetes Mellitus
3.Rare presentation of right Adrenal Mass: Extramedullary haematopoiesis in a patient with Thalassaemia Intermedia
Poh Shean Wong ; Lit Sin Yong ; Nor Afidah Binti Karim ; Ee Leng Gan ; See Guan Toh ; Noor Lita Binti Adam
Journal of the ASEAN Federation of Endocrine Societies 2021;36(1):80-84
Extramedullary hematopoiesis (EMH) is a rare cause of adrenal mass. We present a 44-year-old woman who has thalassaemia intermedia, referred to Endocrinology clinic for huge adrenal mass. Along with a paraspinal lesion discovered in this patient, the leading diagnosis was EMH. The patient was treated with hypertransfusion and hydroxyurea, which led to a reduction in the size of the right adrenal mass and paraspinal mass. This case highlights the challenges in managing this rare condition. Although EMH is a rare cause of adrenal mass, the diagnosis must be considered in any patient with a history of a congenital hemolytic disorder, to avoid unnecessary surgical procedures.
Hematopoiesis, Extramedullary
4.Primary hypoparathyroidism and non-functioning pituitary adenoma: An incidental coexistence?
Journal of the ASEAN Federation of Endocrine Societies 2014;29(1):86-89
Primary hypoparathyroidism is caused by a group of heterogeneous conditions in which hypocalcemia and hyperphosphatemia occur as a result of deficient parathyroid hormone (PTH) secretion. The most common cause is surgical excision and damage to the parathyroid gland(s). Nonetheless, autoimmune endocrine disorder of primary hypothyroidism has been well-described in polyglandular autoimmune syndromes (PAS).1 Its association with pituitary lesion may be autoimmune lymphocytic hypophysitis as the cause for pituitary disorder. In this report, we encountered a patient with primary hypoparathyroidism who had a non-functioning pituitary tumour. It was confirmed as pituitary adenoma rather than lymphocytic hypophysitis from the histopathological examination. To our knowledge, this is the first reported case of non-functioning pituitary macroadenoma and primary hypoparathyroidism.
Hypopituitarism
;
Autoimmune Hypophysitis
;
DiGeorge Syndrome


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