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Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is classified into three types based on disease severity: classic salt-wasting, classic simple virilizing, and nonclassic. Adrenomedullary dysplasia and epinephrine deficiency have been described in classic CAH, resulting in glucose dysregulation. Our objective was to investigate adrenomedullary function in nonclassic CAH and to evaluate adrenomedullary function according to disease severity. Adrenomedullary function was evaluated in response to a standardized cycle ergonometer test in 23 CAH patients (14 females, age 9-38 years; 6 salt-wasting, 7 simple virilizing, 5 nonclassic receiving glucocorticoid treatment, 5 nonclassic not receiving glucocorticoid), and 14 controls (7 females, age 12-38 years). Epinephrine, glucose, and cortisol were measured at baseline and peak exercise. CAH patients and controls were similar in age and anthropometric measures. Patients with nonclassic CAH who were not receiving glucocorticoid and controls experienced the expected stress-induced rise in epinephrine, glucose, and cortisol. Compared to controls, patients with all types of CAH receiving glucocorticoid had impaired exercise-induced changes in epinephrine (salt-wasting: p=0.01; simple virilizing: p = 0.01; nonclassic: p=0.03), and cortisol (salt-wasting: p=0.004; simple virilizing: p=0.006; nonclassic: p = 0.03). Salt-wasting patients displayed the most significant impairment, including impairment in glucose response relative to controls (p=0.03). Hydrocortisone dose was negatively correlated with epinephrine response (r = -0.58; p=0.007) and glucose response (r = -0.60; p=0.002). The present study demonstrates that untreated patients with nonclassic CAH have normal adrenomedullary function. The degree of epinephrine deficiency in patients with CAH is associated with the severity of adrenocortical dysfunction, as well as glucocorticoid therapy.

The initial promising results with alternating chemotherapy regimens (mechlorethamine, vincristine, procarbazine, and prednisone/doxorubicin, bleomycin, vinblastine, and dacarbazine [MOPP/ABVD]; lomustine, melphalan, and vindesine [CAD] plus MOPP plus ABV) combined with intermediate-dose radiation therapy (RT) have been sustained with further follow-up; 82.2% of patients (152 of 185) achieved a complete remission (CR), and overall survival is 71.7% +/- 4.4% at 8 years (median follow-up is 55 months among the survivors). No statistically significant differences were found in CR percentage, CR duration, or survival between stages IIB, IIIB, and IV patients. For that reason, stepwise Cox regression analyses to identify the important prognostic factors were performed on overall survival, tumor mortality, freedom from disease progression, and survival following disease progression. Pretreatment characteristics were also tested for association with the probability of achieving CR, CR duration, and death due to other causes. Characteristics that were consistently associated with an independently unfavorable prognosis were low hematocrit, high serum lactic acid dehydrogenase (LDH), age more than 45 years, inguinal node involvement, mediastinal mass greater than .45 of the thoracic diameter, and bone marrow involvement. Patients with two or more unfavorable characteristics were much more likely to fail treatment (median survival, 62.4 months) than those with none or only one unfavorable factor (greater than 95% survival). This striking difference between the low- and high-risk groups remained even if the comparison was restricted to patients less than or equal to 45 years of age. These results provide a basis for selecting the young patients at high risk of failure for more intensive initial treatment with either autologous bone marrow rescue or hematopoietic growth factors.

The purpose of our study was to describe the MR features of testicular adrenal rest tissue in patients with congenital adrenal hyperplasia and to compare the usefulness of MR imaging with that of sonography for the detection of testicular adrenal rest tissue.Nineteen patients with congenital adrenal hyperplasia underwent MR imaging and gray-scale and color Doppler sonography of the testicles during the same visit to our institution. Findings were compared.MR features of testicular adrenal rest tissue included the following: isointensity (71% of the masses) and slight hyperintensity (29% of the masses) relative to normal testicular tissue on T1-weighted images; hypointensity relative to normal testicular tissue on T2-weighted images (100% of the masses); and diffuse enhancement on contrast-enhanced T1-weighted images (85% of the masses). MR imaging and sonography revealed the testicular lesions equally well. Eleven (58%) of 19 patients had normal findings on MR imaging and sonography. Eight (42%) of 19 patients had 14 intratesticular masses detected by both imaging techniques.MR imaging and sonography are equally useful in the detection of testicular adrenal rest tissue. Because sonography is more accessible to most institutions and is less expensive, it is the imaging technique of choice for the detection of testicular adrenal rest tissue.

This study was designed to identify risk factors for mortality and morbidity of extremely premature infants born in the surfactant era. The study cohort included 194 infants born at29 weeks' gestation at one regional tertiary center between 1983 and 1986. Forty-one infants died. Blinded neurodevelopmental assessments were performed on 149 of 153 (97%) survivors at a mean age of 52 months. Thirty-one (21%) survivors had major impairments: 15 had mental retardation, 8 had multiple impairments, 7 had cerebral palsy, and 1 was blind. Logistic regression analysis identifies five significant risk factors for mortality: grade III or IV intraventricular hemorrhage, birth weight800 gm, 5-minute Apgar scoreor = 3, male sex, and absence of surfactant therapy. Significant risk factors for any major impairment included sepsis (relative risks [RR] = 6.4), male sex (RR = 3.1), and nonwhite race (RR = 2.8). Hydrocephalus requiring shunting was a significant risk factor for cerebral palsy (RR = 16.4) and neonatal retardation (RR = 16.0). Nonwhite race (RR = 7.3), sepsis (RR = 6.8), and male sex (RR = 3.7) also were significant risk factors for mental retardation. Confirmation of these risk factors should facilitate development of targeted interventions for optimizing long-term outcome.

The introduction of the MOPP (mustine, oncovin, procarbazine, and prednisone) combination by DeVita and colleagues (Longo et al. 1986) represented a dramatic advance in the treatment of patients with advanced Hodgkin’s disease. The complete remission (CR) rate at Memorial Hospital was at the lower end of those reported in the literature, perhaps reflecting the inclusion of advanced Hodgkin’s disease patients with a worse prognosis than those seen in other centers.