We studied androgen receptor function in cultured scrotal skin fibroblasts from eight subjects with X-linked spinal and bulbar muscular atrophy (SBMA) (Kennedy's syndrome) from four families. The neuromuscular and endocrine features were similar in all patients. High-affinity dihydrotestosterone binding (Bmax) was decreased in three patients from one family (average, 11.1 fmol/mg) similar to values in subjects with androgen resistance syndromes. Bmax was normal in five SBMA patients from three other families (average, 26.0 fmol/mg). This finding provides direct evidence for abnormal androgen receptor function in some patients with SBMA. There was some correlation between severity of neuromuscular and endocrine dysfunction, providing further evidence that the two types of manifestations are related.

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Introduction. Posterior reversible encephalopathy syndrome (PRES) is a relatively common cause of encephalopathy in the hospital setting, and the EEG findings have not been well described. The purpose of this study was to review the EEG findings in a series of patients with PRES. Methods. We retrospectively reviewed our electronic medical record database to identify patients who received a diagnosis of PRES at Tampa General Hospital from January 2016 to October 2017. The diagnosis of PRES was suspected on clinical presentation and confirmed by magnetic resonance imaging. We selected patients with PRES who had received at least 1 EEG. EEGs were interpreted by 2 board-certified electroencephalographers. Results. From January 2016 to October 2017, 19 patients were diagnosed with PRES at Tampa General Hospital. Of those, 10 received at least 1 EEG. Four patients were male, 6 were female. The ages ranged from 21 to 87 (mean was 47). The patients had the following clinical presentations: 5 with encephalopathy, 8 with seizures, 2 with vision changes, and 3 with headache (some patients had more than 1 symptom). EEGs findings were as follows: 3 were normal; 3 showed intermittent generalized slowing; 2 showed continuous generalized slowing; 3 showed background slowing; 1 showed background suppression; 1 showed generalized rhythmic delta activity (GRDA); 1 showed GRDA, plus spike/sharp-wave discharges; 1 showed generalized periodic discharges. The etiologies were as follows: 9 from hypertension, 1 secondary to eclampsia, 3 due to posttransplant immunosuppression, and 1 patient was undergoing chemotherapy (some were multifactorial). Conclusion. EEG findings in PRES are diverse, with no specific or even predominant pattern, based on this small sample size.

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Nephrotic syndrome is defined by three characteristic features including proteinuria of >3 g in 24 hours, hypoalbuminemia of less than 3 g/dL, and peripheral edema. Multiple nephropathies can result in nephrotic syndrome. Most commonly, minimal change disease is seen in children under the age of 10, while adults are more commonly found to have membranous nephropathy. Hypercoagulability and thrombotic sequela can be seen in nephrotic syndrome, regardless of underlying etiology, and thrombosis is most commonly seen in deep veins of the lower extremities and renal veins. Our case identifies an adult with previously diagnosed and treated for minimal change disease who presented with weight gain, peripheral edema, foamy urine, headache but no neurologic deficits. The patient was found to have near to complete occlusion of the entire superior sagittal sinus, near complete occlusion of the left transverse and sigmoid sinuses, and nonocclusive thrombus in the right sigmoid sinus. She was treated with heparin and IV steroids then transitioned to warfarin and PO steroids, respectively, with resolution of symptoms. This case report emphasizes on the importance of recognizing CVST as a potential complication of nephrotic syndrome at both initial presentation and relapse.

C h i l d r e n ' s H o s p i t a l , Department o f P e d i a t r i c s (C a r d i o l o q v) , Houston The e f f e c t i v e and sa& dosage o f d i g o x i n f o r premature i n f a n t s has n o t y e t been e s t a b l i s h e d. W e compared t h e d i g o x i n serum l e ve l , change i n l e f t v e n t r i c u l a r s y s t o l i c time i n t e r v a l (LVSTI) and t o x i c r r~a n i f e s t a t i o n s i n 2 qroups of premature i n f a n t s , administ e r i n g a t o t a l d i g i t a l i z i n g dose (TDD) o f 30pg/kq t o Gp.1 and a

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