Jeyakantha Ratnasingam

Hypothalamic germinoma masquerading as superior mesenteric artery (SMA) syndrome.

OBJECTIVE To report a case of superior mesenteric artery (SMA) syndrome secondary to hypothalamic germinoma. METHODS We describe the clinical presentation, diagnostic work-up, management, and clinical course of a patient admitted with SMA syndrome who was subsequently found to have a hypothalamic germinoma. RESULTS An adolescent boy was admitted to the surgical ward with progressive weight loss over a 2 year period and postprandial vomiting. He was diagnosed with SMA syndrome based on evidence of proximal duodenal dilatation, extrinsic compression of the distal duodenum, and a narrowed aortomesenteric angle (16°). Investigations performed to exclude thyrotoxicosis unexpectedly revealed secondary hypothyroidism and further evaluation demonstrated evidence of pan-hypopituitarism. Psychiatric evaluation excluded anorexia nervosa and bulimia. Magnetic resonance imaging (MRI) of the brain revealed a heterogeneously enhancing hypothalamic lesion, but a normal pituitary gland. Hormone replacement with hydrocortisone, desmopressin, testosterone, and thyroxine resulted in weight gain and resolution of gastrointestinal symptoms. A transventricular endoscopic biopsy subsequently confirmed a hypothalamic germinoma and he was referred to an oncologist. CONCLUSION SMA syndrome secondary to severe weight loss is an uncommon cause of upper gastrointestinal obstruction. While there have been reports of poorly controlled diabetes mellitus and thyrotoxicosis manifesting as SMA syndrome, there are no published reports to date of SMA syndrome secondary to hypothalamic/pituitary disease. Management of SMA syndrome is conservative, as symptoms of intestinal obstruction resolve with weight gain following treatment of the underlying cause. Awareness of this uncommon presentation of endocrine cachexia/hypothalamic disease will prevent unnecessary laparotomies and a misdiagnosis of an eating disorder.

Glucocorticoids suppress brown adipose tissue function in humans: a double-blind placebo-controlled study

To investigate the effect of glucocorticoids on brown adipose tissue (BAT) function in humans.

Asymmetrical bone loss in a patient with poliomyelitis: an indication for anti-osteoporotic therapy.

BACKGROUND Poliomyelitis survivors suffer from post-myelitic complications including osteoporosis that are often overlooked. METHODS We report a case of a 49-year-old lady with history of poliomyelitis with resultant flaccid paralysis of the involved limb. RESULTS The bone mineral density revealed asymmetrical severe osteoporosis in the poliomyelitic limb. Given the risk of falls and fractures, she was commenced on oral bisphosphonate therapy. CONCLUSION Poliomyelitis is an important acquired risk factor for regional osteoporosis. This condition should be detected and treated in this cohort of patients who are clearly at higher risk of fractures.

Relationship of glycated hemoglobin, and fasting and postprandial hyperglycemia in type 2 diabetes mellitus patients in Malaysia

Studies on the relative contributions of fasting and postprandial hyperglycemia (FH and PPH) to glycated hemoglobin (HbA1c) in patients with type 2 diabetes have yielded inconsistent results. We aimed to assess the relationship by using continuous glucose monitoring in a multi‐ethnic cohort.

Predictors for secondary therapy after surgical resection of nonfunctioning pituitary adenomas

Factors determining recurrence of nonfunctioning pituitary adenomas (NFAs) that require further therapy are unclear as are postoperative follow‐up imaging guidelines. We aimed to identify predictors for secondary therapy after surgical resection of NFAs and use this knowledge to inform postoperative management.

Primary hyperparathyroidism: a rare cause of genu valgus in adolescence.

A 15-year-old girl presented with a history of progressive genu valgus of both lower limbs. She had no other skeletal deformities or fractures. There was no significant family history, in particular none to suggest multiple endocrine neoplasia syndrome. Radiographs of the lower limbs confirmed severe bilateral genu valgus (Figure 1). Investigations revealed a markedly elevated serum corrected calcium of 12.4 mg/dL (normal, 8.8–10.4), phosphate of 2.8 mg/dL (normal, 3.4–5.8), alkaline phosphatase of 1136 IU/L (normal, 50–136), intact PTH of 1649 pg/ml (normal, 10–68), 25-hydroxyvitamin D of 28 ng/ml (normal, 9–52), and 24-hour urinary calcium of 196 mg/d (normal, 88–300). Further radiographs of the hand re-

PREDICTING RECOVERY OF THE HYPOTHALAMIC-PITUITARY-ADRENAL AXIS AFTER PROLONGED GLUCOCORTICOID USE

OBJECTIVE Prolonged exposure to glucocorticoids lead to hypothalamic-pituitary-adrenal (HPA) axis suppression that recovers after cessation of treatment. We aimed to identify the predictive factors for HPA axis recovery after prolonged glucocorticoid use. METHODS Retrospective review of patients who had undergone first short Synacthen test (SST) to assess HPA axis recovery after prolonged use of glucocorticoids. RESULTS A total of 61% (20/33) of patients had adequate SST response at a median time of 2 years after diagnosis of adrenal insufficiency. Those who had adequate response during SST had higher ambulatory early morning cortisol ( P<.01), shorter duration of exposure to glucocorticoids ( P = .01), and lower final cumulative hydrocortisone replacement dose ( P = .03). Age, gender, body mass index, indications for glucocorticoid use, and basal adrenocorticotropic hormone levels were not predictive of HPA axis recovery. On multivariate analysis, ambulatory early morning cortisol was the only independent predictor of adequate SST response (odds ratio, 1.02; 95% confidence interval, 1.01 to 1.04; P = .02). Using receiver operating characteristic curve analysis, ambulatory early morning cortisol of 8.8 μg/dL predicted a positive SST response with a sensitivity of 70% and specificity of 93%. CONCLUSION Early morning ambulatory cortisol could be used to decide on timely SST in order to prevent complications from unnecessary replacement with glucocorticoids. ABBREVIATIONS ACTH = adrenocorticotropic hormone; BMI = body mass index; CV = coefficient of variation; HPA = hypothalamic-pituitary-adrenal; SST = short Synacthen test.

Continuous glucose monitoring results in lower HbA 1c in Malaysian women with insulin-treated gestational diabetes: a randomized controlled trial

To determine if therapeutic, retrospective continuous glucose monitoring (CGM) improves HbA1c with less hypoglycaemia in women with insulin‐treated gestational diabetes mellitus (GDM).

PRKAR1A-negative familial Cushing’s syndrome: two case reports

IntroductionDetermining the etiology of Cushing’s syndrome is very challenging to endocrinologists, with most of the difficulty arising from subtype differentiation of adrenocorticotropic hormone–dependent Cushing’s syndrome. We present the pitfalls of evaluating a rare cause of adrenocorticotropic hormone–independent Cushing’s syndrome in the transition period between adolescence and adulthood.Case presentationA sibling pair with familial isolated primary pigmented nodular adrenocortical disease is described. The index case, a 20-year-old Chinese woman, presented with premenopausal osteoporosis with T12 compression fracture and young hypertension. Biochemical analysis confirmed adrenocorticotropic hormone–independent Cushing’s syndrome (elevated 0800 h plasma cortisol 808 nmol/L with suppressed adrenocorticotropic hormone level <5 pg/ml). Computed tomography of her adrenal glands revealed a 0.7-cm left adrenal hypodense nodule. After a left adrenalectomy, she had residual hypercortisolism (progressive weight gain, new T10 compression fracture, and not glucocorticoid-dependent postoperatively). Completion of contralateral adrenalectomy was performed upon recognition of typical histologic characteristics of primary pigmented nodular adrenocortical disease found in an initial left adrenalectomy specimen. Similarly, her younger brother developed adrenocorticotropic hormone–independent Cushing’s syndrome at age 18 years, with typical cushingoid habitus, but no osteoporosis or hypertension. His adrenal computed tomographic scans showed micronodularities over bilateral adrenal glands. He was successfully treated with bilateral adrenalectomy. Screening for Carney’s complex and PRKAR1A gene mutation was negative. Signs and symptoms of Cushing’s syndrome resolved after bilateral adrenalectomy for both patients. They were placed on lifelong glucocorticoid and mineralocorticoid replacement therapy and long-term surveillance for Carney’s complex.ConclusionsThe cases of these two patients illustrate the difficulties involved in diagnosing primary pigmented nodular adrenocortical disease, a variant of adrenocorticotropic hormone–independent Cushing’s syndrome that is managed with bilateral adrenalectomy. A high index of suspicion for this disease is needed, especially in adolescents with adrenocorticotropic hormone–independent Cushing’s syndrome who have a significant family history, features of Carney’s complex, and no resolution of Cushing’s syndrome after unilateral adrenalectomy. Patients with primary pigmented nodular adrenocortical disease can either have bilateral/multiple adrenal nodules or normal adrenal glands visualized by computed tomography. Long-term surveillance is imperative in patients with confirmed Carney’s complex and in those who have not undergone complete genetic testing to exclude this hereditary disorder.