Zulfiqar Ali Bhatti, Uzma Anees.
Porphyrias.
Pak Armed Forces Med J Jan ;53(2):242-3.

A 25 years married woman was brought with complaints of weakness of legs, constipation, pain abdomen, inability to pass urine and abnormal behavior of 07 days duration. The complaints had been intermittent for the last 07 days and intensity increased with passage of time. Past H/O appendectomy was also forwarded. In family history her elder sister was also having similar complaints. On examination she was conscious but was hyper breathing with carpopedal spasm. Pulse was 82/min, BP120/80 mmHg with normal body temperature. There were fluid filled vesicles and bullae on sun exposed parts, face, and dorsum of hands and feet, forearms and legs were observed. There was wasting in distal parts of limbs, tone was diminished along with hyporeflexia, planters were down going and hypoesthesia was also noted on limbs. Her bladder was distended and abdomen had diffuse tenderness. Rest of the systemic examination was unremarkable. She was seen by dermatologist who concurred with the provisional diagnosis. Her urine became dark brown on standing. Urine was tested with Ehrlich`s reagent with equal volume of urine, a red color was obtained. The red color due to porphobilinogen (PBG) persisted when chloroform was added, Watson-Schwartz reaction to differentiate from urobilinogen. Urine and blood for aminolevolinic acid (ALA) and PBG was positive for porphyria. She was treated with narcotic analgesic for abdominal pain and IV glucose 300g/d. Intravenous hematin was not available in market. Patient was counseled for identification and avoidance of inciting factors to fasten recovery for acute attack and prevent future precipitation. Dermatological care was provided by skin specialist.

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