In Malaysia, acromegaly is under-recognised with only 10-15% of the expected number of cases from prevalence estimates, having been diagnosed and managed in established endocrine centres with access to multidisciplinary care. This is mainly due to lack of awareness and standardised approach in diagnosing this disease resulting in delay in diagnosis and management with suboptimal treatment outcomes. This first Malaysian consensus statement on the diagnosis and management of acromegaly addresses these issues and is based on current best practices and latest available evidence so as to reduce the disease burden on acromegaly patients managed in the Malaysian healthcare system.

Pituitary stalk lesions can represent a wide range of pathologies. The exact cause is often unknown due to hesitancy to proceed with biopsy. We present a 16-year-old adolescent who presented with delayed puberty, short stature and bilateral cryptorchidism. He was found to have a thickened pituitary stalk of uncertain etiology with partial hypopituitarism (gonadotrophin and growth hormone deficiency) on further assessment. The presence of bilateral cryptorchidism and micropenis represents lack of “mini puberty,” a phenomenon of activation of the hypothalamic-pituitary-gonadal (HPG) axis in-utero or within the first few months of life.1 These key clinical features have been useful to establish an early temporal relationship and suggest a congenital origin of disease. This enabled a more conservative approach of surveillance to be employed as opposed to invasive pathological examination with pituitary stalk biopsy.
Pituitary Diseases ; Hypopituitarism ; Cryptorchidism ; Growth Hormone

Presentation of Cushing’s syndrome during pregnancy is extremely rare. We report a 21-year-old female with Cushing’s syndrome diagnosed at 23 weeks of gestation and had recurrent acute pulmonary oedema during the antepartum and postpartum period. She delivered prematurely via emergency caesarean section at 28 weeks of gestation. This case highlights the rare occurrence of recurrent acute pulmonary oedema during pregnancy and consequential premature birth in a patient with adrenal Cushing’s. She was diagnosed with adrenal Cushing’s during the postpartum period based on unsuppressed serum cortisol after overnight and low-dose dexamethasone suppression test with a suppressed ACTH. CT scan of the adrenal glands revealed a right adrenal cortical adenoma. The risk of complications in infants and mothers who suffer from Cushing’s syndrome needs to be handled carefully. The diagnosis of Cushing’s syndrome in pregnant women often overlaps and is difficult to establish in early pregnancy.
Pregnancy

Somatostatin analogue is useful in carcinoid crisis for symptom control. Optimal dosing of somatostatin analogues for carcinoid symptoms is not known. This case highlighted management issues using combination short-acting octreotide infusion with long-acting lanreotide during carcinoid crisis. The patient had left lung neuroendocrine tumour that metastasized to his liver and bone, post left lobectomy. Due to extensive metastasis to the liver causing recurrent carcinoid crisis, he required shorter interval long-acting lanreotide with continuous infusion of short-acting octreotide, which led to transient diabetes insipidus. Symptoms resolved with discontinuation of treatment. Somatostatin analogues, especially in combination, may inhibit the posterior pituitary resulting in diabetes insipidus. Prompt withdrawal of short-acting somatostatin analogue and initiation of desmopressin can reverse the complication. It is important to recognize this complication with combination of octreotide and lanreotide injections to avoid serious complications.
Diabetes Insipidus ; Octreotide ; Neuroendocrine Tumors ; Malignant Carcinoid Syndrome

Ganglioneuromas (GNs) are benign tumors that originate from neural crest cells, composed mainly of mature ganglion cells. These tumors, which are usually hormonally silent, tend to be discovered incidentally on imaging tests and occur along the paravertebral sympathetic chain, from the neck to the pelvis and occasionally in the adrenal medulla. Rarely, GNs secrete catecholamines.1 Adrenal GNs occur most frequently in the fourth and fifth decades of life, whereas GNs of the retroperitoneum and posterior mediastinum are usually encountered in younger adults.2 Adrenal GNs are commonly hormonally silent and asymptomatic; even when the lesion is of substantial size.3We report an incidentally detected asymptomatic case of an adrenal ganglioneuroma with mildly elevated urinary catecholamine levels in an elderly male. After preoperative alpha blockade, the patient underwent open right adrenalectomy. Upon microscopic examination, the right adrenal mass proved to be a ganglioneuroma, maturing type and the immunohistochemistry examination showed immunoreactivity to synaptophysin, chromogranin, and CD 56, while S100 was strongly positive at the Schwannian stroma. Following resection, catecholamine levels normalized, confirming the resected right adrenal ganglioneuroma as the source of the catecholamine excess. This case represents a rare presentation of catecholamine-secreting adrenal ganglioneuroma in the elderly.
Adrenal Glands ; Catecholamines ; Ganglioneuroma

Objective: This study aims to report the demographic features of patients with acromegaly the disease burden, and the corresponding treatment patterns and outcomes in Malaysia. Methodology: This is a retrospective study that included patients from the Malaysian Acromegaly registry who were diagnosed with acromegaly from 1970 onwards. Data collected included patient demographics, clinical manifestations of acromegaly, biochemical results and imaging findings. Information regarding treatment modalities and their outcomes was also obtained. Results: Registry data was collected from 2013 to 2016 and included 140 patients with acromegaly from 12 participating hospitals. Median disease duration was 5.5 years (range 1.0 – 41.0 years). Most patients had macroadenoma (67%), while 15% were diagnosed with microadenoma. Hypertension (49.3%), diabetes (37.1%) and hypopituitarism (27.9%) were the most common co-morbidities for patients with acromegaly. Majority of patients had surgical intervention as primary treatment (65.9%) while 20.7% were treated medically, mainly with dopamine agonists (18.5%). Most patients had inadequate disease control after first-line treatment regardless of treatment modality (79.4%). Conclusion This registry study provides epidemiological data on patients with acromegaly in Malaysia and serves as an initial step for further population-based studies.
acromegaly ; treatment outcomes

Objective: The primary objective was to assess beta-cell function of recently-diagnosed young-onset type 2 diabetes mellitus (T2DM) individuals using basal and stimulated C-peptide levels. The secondary objective was to examine the association between C-peptide with metabolic factors and diabetes complications. Methodology: A cross-sectional study was conducted for young-onset T2DM individuals aged 18-35 years with a disease duration of not more than 5 years. Plasma C-peptide was measured before and after intravenous glucagon injection. Demographic data, medical history and complications were obtained from medical records and clinical assessment. Continuous data were expressed as median and interquartile range (IQR). Categorical variables were described as frequency or percentage. Multivariable linear regression analysis was used to determine factors associated with C-peptide levels. Results: 113 participants with young-onset T2DM with a median (IQR) age of 29.0 (9.5) years and 24 (36) months were included in this study. The median (IQR) basal and stimulated C-peptide was 619 (655) pmol/L and 1231 (1024) pmol/L. Adequate beta-cell function was present in 78-86% of the participants based on the basal and stimulated C-peptide levels. We found hypertension, obesity and diabetic kidney disease (DKD) to be independently associated with higher C–peptide levels. In contrast, females, smokers, those on insulin therapy and with longer duration of disease had lower C–peptide levels. Conclusion Most recently diagnosed young-onset T2DM have adequate beta-cell function. Elevated C-peptide levels associated with obesity, hypertension and diabetic kidney disease suggest insulin resistance as the key driving factor for complications.
Diabetes Mellitus, Type 2 ; C-Peptide