Familial Mediterranean Fever With Complete Symptomatic Remission During Pregnancy.

Familial Mediterranean fever (FMF) is an inherited autosomal recessive disorder, ethnically restricted and commonly found among populations surrounding the Mediterranean Sea. FMF is the most prevalent autoinflammatory disease; is characterized by recurrent, self-limited episodes of fever with serositis; and is caused by Mediterranean fever gene (MEFV) mutations on chromosome 16. We describe a case of adult-onset FMF with complete symptomatic remission during pregnancy, without the use of colchicine. A 25-year-old woman had presented with periodic fever, abdominal pain, and vomiting since she was 21. Her abdominal computed tomography scan showed intestinal nonrotation. She underwent exploratory laparotomy and appendectomy for her symptoms 1 year prior. She had a symptom-free pregnancy period, but abdominal pain and fever recurred after delivery. Mutation analysis of the MEFV gene revealed two point mutations (p.Leu110Pro and p.Glu148Gln). We report an adult female patient with FMF in Korea with complete symptomatic remission during pregnancy.

Clinical features and treatment outcomes of skin cancer arising from burn scar: a single-institution experience.

AIMS AND BACKGROUND: This study was conducted to investigate the clinicopathological features and long-term outcomes of patients with skin cancer arising from burn scar (SCBS). PATIENTS AND METHODS: We retrospectively reviewed the medical records of patients diagnosed with SCBS between January 2000 and May 2012. A total of 44 patients were enrolled in this study. RESULTS: The median latent period between burn injury and development of SCBS was 32 years (range, 8-78 years). The most frequent sites of SCBS were the lower limbs (68.2%) followed by the upper limbs (15.9%) and trunk (11.4%). Most patients (95.4%) had squamous cell carcinoma. Of 34 patients with localized disease at the time of diagnosis, 33 patients are alive with no evidence of recurrence. Of 10 patients with regional lymph node metastasis (referred to as locally advanced disease), 4 died of disease progression and 5 are alive with metastatic disease in the lymph nodes, bone or lung. Patients with localized disease survived longer than patients with locally advanced disease ( P = 0.000). In patients with locally advanced disease, the median overall survival time was 16 months (95% CI, 2.88-29.4 months). CONCLUSIONS: While localized SCBS is a potentially curable disease, locally advanced SCBS has a poor prognosis in spite of aggressive treatment. These results suggest that early recognition and aggressive treatment are essential to improve the outcomes of SCBS.

An Elderly Man with Fatal Respiratory Failure after Eating a Poisonous Mushroom Podostroma cornu-damae.

A 73-year-old, previously healthy man presented with nausea, vomiting, diarrhea, dry mouth and febrile sensation 3 hours after eating boiled wild mushrooms. After admission, he showed progressive severe respiratory distress, pancytopenia, azotemia, hypotension, hypoxemia and consolidation of the entire left lung on chest radiography. With a preliminary diagnosis of necrotizing pneumonia, he underwent left pneumonectomy in order to remove all necrotic lung tissue. Lung histology showed extensive hemorrhagic necrosis, massive inflammatory cell infiltration, prominent proliferation of young fibroblasts and the formation of an early-stage hyaline membrane along the alveolar wall. Despite aggressive treatment, including mechanical ventilation, continuous renal replacement therapy and administration of granulocyte colony stimulating factor and broad spectrum antibiotics, he died on hospitalization day 13. Subsequently, the mushroom was identified as Podostroma cornu-damae. This is the first case of a histological evidence of lung involvement by Podostroma cornu-damae poisoning in Korea.