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PURPOSE: This prospective study presents the results of a new approach in the treatment of primary macronodular adrenal hyperplasia (PMAH), with simultaneous total adrenalectomy of the larger adrenal gland and partial adrenalectomy of the contralateral adrenal gland (adrenal-sparing surgery). MATERIALS AND METHODS: We performed a prospective study including 17 patients with PMAH treated surgically with adrenal-sparing surgery in a tertiary referral hospital, with a median follow-up of 41 months. Clinical, hormonal, and genetic parameters were evaluated before surgery and during follow-up. All patients had at least 1 radiological examination before and after the procedure. RESULTS: Among the 17 patients, all but 1 patient had complete hypercortisolism control, and 12 recovered normal adrenal function after surgery. Significant improvement in clinical parameters was observed: weight loss (P = .004); reduction of both systolic (P = .001) and diastolic (P = .001) blood pressure; and reduction in the number of antihypertensive drugs (P < .001). Intra-, peri-, and postoperative complications were not observed. CONCLUSION: Adrenal-sparing surgery is a safe and feasible procedure to treat patients with PMAH, providing a substantial chance of hypercortisolism control without the disadvantages of lifetime corticosteroid replacement.
RESUMO
CONTEXT: Nephrocalcinosis (NC) and nephrolithiasis (NL) are described in hypophosphatemic rickets, but data regarding their prevalence rates and the presence of metabolic risk factors in X-linked hypophosphatemic rickets (XLH) are scarce. OBJECTIVE: To determine the prevalence rates of NC and NL and their risk factors in patients with XLH with confirmed PHEX mutations. METHODS: Renal ultrasonography (US) and CT were performed in 16 children and 23 adults. The images were evaluated by two blinded radiologists specializing in US and two specializing in CT. Confirmation of NC was determined with a positive result on both US and CT, whereas the diagnosis of NL was confirmed by CT alone. The presence of hypercalciuria, hypocitraturia, and hyperoxaluria was determined from 24-hour urinary samples from each patient. The glomerular filtration rate was estimated. RESULTS: NC was identified in 15 patients (38.4%), and stratification by age group showed a higher prevalence of NC in children than in adults (56.2% vs 26.1%). CT identified NL in four adults (10.2%). Patients in the pediatric group required intensive use of phosphate, started treatment earlier, and presented greater phosphaturia than those in the adult group (P < 0.01). In addition to hyperphosphaturia, which was present in all patients with XLH, hypocitraturia was the most common metabolic factor (28.2%), whereas hypercalciuria occurred in two patients (5.1%). None had hyperoxaluria. Most patients had normal renal function. CONCLUSIONS: NC was more prevalent than NL. The main metabolic factor was hyperphosphaturia, and intensive phosphate treatment appears to be a worsening factor for kidney calcification.