Multiple endocrine neoplasia type 2 (MEN 2) is a rare syndrome of medullary thyroid carcinoma (MTC) with pheochromocytoma and/or primary hyperparathyroidism (PHP), usually due to multigland hyperplasia. MEN 2 is associated with several RET protooncogene mutations. A 61-year-old woman with a family history of RET-positive MTC presented with a solitary thyroid nodule. Fine-needle aspiration biopsy was suspicious for neoplasm. Biochemical studies revealed basal hypercalcitoninemia (116 pg/mL [normal <26]) and PHP (serum calcium, 10.9 mg/dL; intact PTH, 113.2 pg/mL [10.0-65.0]). Pheochromocytoma screening was negative. A provisional diagnosis of MEN 2 was made, but at surgery, a single parathyroid adenoma was resected and frozen sections of several lymph nodes revealed papillary thyroid carcinoma (PTC). A total thyroidectomy was performed. Final histological diagnosis was PTC and parathyroid adenoma with no evidence of MTC. Postoperatively, RET mutation testing was positive. The basal calcitonin (CT) fell to 25 pg/mL, but peaked at 935 (normal <105) after pentagastrin infusion, consistent with occult MTC. After radioiodine ablation, CT decreased further. Octreotide scanning was negative. Faced with PHP, a thyroid nodule, and a family history of MTC, clinicians tend to diagnose MEN 2. This patient had a single parathyroid adenoma and nonmedullary thyroid cancer, which the literature actually suggests to be an association more frequent than MEN 2. Yet, there remains compelling data in favor of occult MTC, leaving open the possibility of an MEN 2 variant with the rare association of PTC.
continued) Renal ischemia was measured by using serum urea nitrogen, serum creatinine, and urine alpha glutathione-S-transferase (alpha-GST).Postclamp renal blood flow measurements were standardized as a percentage of preclamp renal blood flow. Treatment with either distal perfusion or distal perfusion with theophylline demonstrated increased renal blood flow in comparison to the controls (p < 0.001). Pulsatile perfusion with theophylline was superior to pulsatile perfusion alone (p < 0.023). Pulsatile perfusion with theophylline was superior to nonpulsatile perfusion with theophylline (p < 0.030). Creatinine 6 hours postoperative showed a significant decrease with both forms of distal aortic perfusion when compared to the control groups (p = 0.002). Creatinine values at 18 hours postoperative were not significant (p = 0.237). Serum urea nitrogen values at 6 (p = 0.239) and 18 (p = 0.460) hours postoperative were not significant. Alpha-GST values at 6 (p = 0.034) and 18 (p = 0.035) hours postoperative showed a significant difference between the control and treatment groups.Pulsatile distal aortic perfusion with theophylline is superior to nonpulsatile distal aortic perfusion with theophylline in augmenting renal blood flow following renal ischemia in the porcine model.
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