Axonal Polyneuropathy in a Man Treated for Pulmonary Cocci: A Case of Acute Intermittent Porphyria.

BACKGROUND Acute intermittent porphyria (AIP) is a rare autosomal dominant disorder that is part of a group of acute porphyria disorders usually found in females of reproductive age. Although clinically there is low penetrance, with 90% of genetically diagnosed individuals never experiencing an acute flair, consequences of acute flairs may lead to devastating results. Debilitating paresis, seizures, respiratory failure, and even death may result from AIP. Early detection is key in preventing these devastating manifestations. CASE REPORT A 67-year-old Hispanic man with a past medical history of pulmonary Coccidioides on fluconazole presented with bilateral thigh pain for 2 days. At baseline, the patient had no limitations, but now was limited to minimal walking due to his thigh pain subsequently progressing to diffuse weakness after the administration of IV Solumedrol. Over the next few months, EMG was notable for acute-on-chronic sensorimotor axonal denervation in upper and lower extremities, without evidence of myositis. Urine porphobilinogen was 58 mmol/L, which is 29 times the upper limit of normal. Treatment was started with hemin 4 mg/kg/day for 4 days. CONCLUSIONS Over our patient's clinical course, he was affected by a severe manifestation of repeated acute porphyria attacks, which started as anterior thigh pain and progressed to diffused weakness disproportionally affecting the muscles of the upper extremities. Although the patient was in his late 60's at the initial onset of AIP, his diffuse Coccidioides infection, use of azoles, and steroids likely contributed to his first AIP attack.

Current Challenges of Plastic Surgical Care in Sub-Saharan Africa (Maputo, Mozambique).

BACKGROUND: Limited data exist on plastic surgery practices in Sub-Saharan Africa. The aim of this study was to characterize the spectrum of disease and operative procedures at a teaching hospital in Maputo, Mozambique to help understand the challenges of providing care for the local providers and to provide contextual relevance for training through partnerships. METHODS: A mixed-methods approach was utilized to perform an ongoing needs assessment. A retrospective review was performed of plastic surgery operative records, ward admissions records, and death records in a tertiary-care hospital in Maputo, Mozambique for the period January 2015 to December 2015. RESULTS: Limited resources (equipment, block-time, personnel, and perioperative services) were observed. The most common diagnoses for the 455 patients evaluated were burns (44%) and neoplasms (17%). Congenital abnormalities accounted for only 1% of the patient diagnoses. Of the 408 procedures performed, the majority were skin grafts (43%) and skin excisions (31%). Sepsis from burns accounted for 70% of documented deaths (14/20). The mean number of days to skin grafting for inpatients was 53 days. CONCLUSION: We observed a large burden of burns and skin graft procedures at a public referral teaching hospital in Mozambique. Our findings provide contextual relevance to help focus public health efforts and improve plastic surgery training and practices.