Acute cardiomyopathy and multiorgan failure in a patient with pheochromocytoma and neurofibromatosis type 1.

Pheochromocytomas are catecholamine secreting tumours of the adrenal gland, discovered in 0.1% of patients with hypertension. Our case highlights an atypical presentation of pheochromocytoma in a patient with Neurofibromatosis type 1 who developed cardiogenic shock with multi-organ failure. The patient demonstrated reversible dilated cardiomyopathy during her hospital stay, and her blood pressure fluctuated widely. Discovery of right adrenal mass followed by biochemical testing facilitated the diagnosis. Judicious medical management led to an uneventful surgical removal of the tumour followed by marked stabilization of her blood pressure. We discuss the characteristics of pheochromocytoma associated with Neurofibromatosis type 1 via reversible cardiac dysfunction.

Acromegaly caused by growth hormone releasing hormone (GHRH) secreting tumor in multiple endocrine neoplasia (MEN-1).

We are presenting the clinical features, diagnostic work up and treatment of acromegaly caused by Growth hormone releasing hormone (GHRH) secreting neuroendocrine tumor (NECT) in a case of multiple endocrine neoplasia type 1 (MEN-1). A 36 year old man, known case of MEN-1 presented with acromegalic features. He has high IGF-1, GH and very high GHRH levels with a pancreatic head tumor and pituitary mass. He had high GHRH arteriovenous gradient across pancreatic tumor and underwent tumor resection, Post operative GHRH level fell dramatically. Tumor had high GHRH m-RNA level. Acromegalic patients with MEN-1 should be screened for ectopic GHRH secretion. Measurement of GHRH arteriovenous gradient across NECT or mRNA for GHRH in resected tumor can confirm the ectopic source. Treatment of choice is surgical resection of the tumor. Somatostatin analogue is an alternative because of its dual action in the pituitary gland and the NECT. Life long surveillance is needed as recurrence chance is high.

The Interactions Between Diabetes Mellitus and Myelodysplastic Syndromes: Current State of Evidence and Future Directions.

Diabetes mellitus (DM) and cancer are disorders of global importance. Multiple epidemiologic studies show that diabetic patients have an increased risk of developing cancer of different types. Myelodysplastic syndromes (MDS) are among the most common hematologic malignancies and include a heterogeneous group of hematopoietic neoplasms characterized by dysplastic changes, low blood counts, and an increased risk of progression to acute myeloid leukemia. Potential epigenetic and metabolic interferences between DM and MDS have been reported but are poorly understood. DM and MDS share some predisposing risk factors such as obesity. Patients with MDS and DM can experience worsening of diabetic control due to multiple factors that exacerbate hyperglycemia and insulin resistance such as stress, infections, adjunct drugs (e.g. steroids to control nausea), and others. In addition, accurate assessment of glucose control in diabetic patients who have MDS can be complicated. Alternatively, DM when associated with end-organ damage can complicate management of MDS, increase risks of complications, and limit the applicability of intensive therapeutic interventions. Here we review the current knowledge of the interactions between DM and MDS at the pathogenetic, clinical and epidemiologic levels, discuss how this knowledge could be used therapeutically to improve the outcome of patients affected by both conditions, and delineate important unmet needs that should be addressed in future research.

Safety and efficacy of an algorithm-based protocol in the management of diabetic ketoacidosis.

OBJECTIVE: To determine the efficacy of an algorithm-based protocol to manage diabetic ketoacidosis (DKA). METHODS: Our study is a retrospective chart review of patients with DKA managed before and after implementation of an algorithm-based DKA protocol at a tertiary care hospital. RESULTS: There were 88 patients managed 1 year prior (control group) and 70 patients managed one year after (study group) implementation of the algorithm-based DKA protocol. The DKA resolution time was significantly shorter (11.5 [8.1 to 17.1] hours versus 8.5 [5.8 to 12] hours; P = 0.008) and the hypoglycemic events were significantly less (P = 0.042) in the study group in comparison with the control group. There was no difference in the potassium abnormalities and rate of decline of glucose. A survey on a scale of 1 to 10 found the majority of physicians and nurses rated the protocol as safe (83%) and effective (96%). Fifty-four percent of the nurses, however, found the protocol difficult to follow. CONCLUSION: Our study showed that implementation of an algorithm-based protocol reduced the DKA resolution time and hypoglycemic events without compromising electrolyte imbalance, and was associated with improved clinical measures of DKA management.

Evaluation of secondary causes of bone loss in a primary care setting.

OBJECTIVE: To evaluate the clinical and laboratory work-up for secondary causes of bone loss in a primary care setting. METHODS: We conducted a retrospective review of medical records of 100 patients with either osteoporosis or osteopenia, who presented to a university-based primary care clinic. Patients with chronic kidney disease or a history of organ transplantation were excluded, as were premenopausal women. RESULTS: Age at menopause was ascertained in 43% of female patients. Only 2% of patients were asked specifically about symptoms of malabsorption, whereas a history of malignant disease or its treatment was elicited from 24%. Of the overall study group, 50% were asked about a history of thyroid disease and 18% about a history of liver disease. Testicular examination was documented in 40% of male patients. Serum calcium and creatinine, complete blood cell count, and thyroid function tests were evaluated in 100% of patients. Vitamin D status was assessed in only 1 patient; no study patient had a 24-hour urine collection for determination of calcium excretion. Serum parathyroid hormone was measured in 7% and serum phosphorus in 10% of patients. Sixty percent of male patients had their testosterone levels assessed. Although the serum creatinine level was determined in all patients, only 1% had a formal estimation of the creatinine clearance or glomerular filtration rate. CONCLUSION: The evaluation of secondary causes of bone loss was notably inadequate in our study population. Because most patients with osteoporosis or osteopenia are managed in the primary care setting, a distinct need exists for consensus guidelines and recommendations from professional endocrine organizations to advise primary care physicians in the appropriate diagnostic evaluation for secondary causes of bone loss in such patients.