1,25-Dihydroxyvitamin D-mediated hypercalcemia in oleogranulomatous mastitis (paraffinoma), ameliorated by glucocorticoid administration.

OBJECTIVE: To report a case of oleogranulomatous mastitis (paraffinoma) in which both the hypercalcemia and the inappropriately elevated 1,25-dihydroxyvitamin D [1,25(OH)2D] levels were successfully reduced by administration of prednisone. METHODS: We describe the clinical, imaging, and laboratory findings in the study patient. Furthermore, we review the relevant literature regarding hypercalcemia in oleogranulomatous mastitis. RESULTS: A 58-year-old man with oleogranulomatous mastitis (paraffinoma)-related hypercalcemia, renal failure, and inappropriately elevated levels of 1,25(OH)2D presented to our institution. Treatment with prednisone--0.5 mg/kg of body weight or 30 mg daily--for 10 days resulted in a reduction of his serum calcium and 1,25(OH)2D levels to near-normal or normal values and a substantial improvement of his renal function. Nevertheless, the patient ultimately died 11 months later of multiple paraffinoma-related complications. CONCLUSION: In selected cases, treatment with glucocorticoids may offer a temporary relief from paraffinoma-related hypercalcemia until definitive treatment options are feasible.

Insulin pump therapy: guidelines for successful outcomes.

PURPOSE: Current forms of insulin delivery used in the treatment of diabetes mellitus (diabetes) include syringes, pens, and insulin pumps. Technical advantages of insulin pump therapy, or continuous subcutaneous insulin delivery (CSII), include precise and flexible insulin dosing. In the context of intensive diabetes management, insulin pumps can facilitate improved long-term glycemic control and reduce the risk for diabetes complications, with improved lifestyle flexibility for patients and their families. Comprehensive patient education, carbohydrate counting, and frequent self-monitoring of blood glucose or continuous glucose monitoring are necessary components of successful insulin pump therapy. Technological advances have increased the appeal of pump therapy to patients and clinicians. Physically, current insulin pumps are discreet, ergonomic, and water resistant. Meanwhile, software improvements have yielded smart pumps with features that support pump users in their daily diabetes management. Robust data analysis software packages allow patients and clinicians unprecedented insight into the quality of diabetes control. Furthermore, widespread insurance reimbursement for CSII has expanded access to therapy. CONCLUSIONS: As the number of pump users and potential users expands, diabetes educators face new challenges and opportunities to improve patients' lives with diabetes. This activity describes the rationale for insulin pump therapy, its potential advantages and disadvantages, and strategies regarding patient selection and education.

Medical management of Cushing disease.

Although transsphenoidal excision of the adrenocorticotropic hormone (ACTH)-producing neoplasm is often the treatment of choice in patients with Cushing disease, medical management is itself a useful preoperative temporizing measure, an option for long-term management in nonsurgical candidates, and an option for patients in whom surgery and/or radiotherapy have failed. Three pathophysiologically based approaches exist in the research literature--neuro-modulation to limit ACTH levels, adrenal enzyme inhibition, and glucocorticoid receptor antagonism. Unfortunately, the neuromodulatory approach involving agents such as bromocriptine, cyproheptadine, octreotide, and valproate has yielded only suboptimal results. Glucocorticoid receptor antagonism remains in its infancy but may overall be limited by side effects and a resultant increase in ACTH and cortisol levels. Adrenal enzyme inhibitors, however, offer substantial future promise in the management of Cushing disease but are limited by the potential need to use them indefinitely and by dose-tolerance effects. Although etomidate is a potential intravenous alternative for acute cortisol level control, ketoconazole has shown efficacy in the long-term treatment of patients with the disease. Metyrapone and/or aminoglutethimide can be added to ketoconazole if additional control is needed. If success is still not achieved, the potent adrenolytic agent often used for adrenocortical carcinomas, mitotane, is another alternative.

Diagnostic approach to Cushing disease.

In Cushing disease, a pituitary corticotroph neoplasm causes secondary adrenal hypercortisolism. This condition has known morbidity and mortality, underscoring the need for an efficient and accurate diagnostic approach. An 11 p.m. salivary cortisol level is a modern, simple initial screening tool for the diagnosis of Cushing syndrome. Confirmation with a 24-hour urinary free cortisol test and/or a low-dose dexamethasone suppression test may subsequently be performed. Patients with repeatedly equivocal results should be reevaluated after several months or undergo a corticotropin-releasing hormone (CRH) stimulation test following low-dose dexamethasone suppression to help rule out pseudo-Cushing states. The presence of low morning serum adrenocorticotropic hormone (ACTH) levels then distinguishes primary adrenal hypercortisolism from Cushing disease and the ectopic ACTH syndrome. Patients with moderate ACTH levels can undergo CRH stimulation testing to clarify the underlying disease because those with an ACTH-independent disorder have blunted subsequent ACTH levels. Once ACTH-dependent hypercortisolemia is detected, magnetic resonance (MR) imaging of the pituitary gland can be performed to detect a pituitary neoplasm. Normal or equivocal MR imaging results revealing small pituitary lesions should be followed up with inferior petrosal sinus sampling, a highly specific measure for the diagnosis of Cushing disease in experienced hands. If necessary, body imaging may be used in turn to detect sources of ectopic ACTH.