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.

Eosinophilic cholecystitis: A rare manifestation of hypereosinophilic syndrome

Eosinophilic cholecystitis (EC) is a rare form of acute cholecystitis, of which diagnosis is based on classical symptoms of cholecystitis with a presence of >90% eosinophilic infiltration within the gall bladder. EC rarely manifests in idiopathic hypereosinophilic syndrome (IHES). Here, we report two cases of EC with IHES. One is a 57-year-old male who presented with acute right upper quadrant (RUQ) pain, jaundice and fever. The initial peripheral blood eosinophil count was 2,070/mm3, and further elevated to 12,590/mm3. Acute acalculous cholecystitis with cholangitis was confirmed by computed tomography (CT). He improved with endocopic nasobiliary drainage and antibiotic therapy. The other is a 64-year-old female who presented with acute RUQ pain. She also complained of dyspnea and tingling sensation of both hands and feet. The initial peripheral blood eosinophil count was 10,400/mm3. Abdominal CT revealed findings suggestive of acute acalculous cholecystitis. She improved with systemic glucocorticosteroid therapy. No other causes of hypereosinophilia were found in either patients. Thus, cholecystectomy may not be mandatory for the treatment of EC with IHES.

A Case of Acute Cholecystitis Caused by Stenotrophomonas maltophilia Bacteremia / 대한췌담도학회지

Stenotrophomonas maltophilia is an ubiquitous aerobic Gram-negative bacillus. Hospitalization and prior antibiotic therapy are risk factors for S. maltophilia infection. This organism is isolated with increasing frequency from hospitalized patients and may cause therapeutic problems because of its intrinsic resistance to common antibiotics and the immunodeficiency status of the affected host. S. maltophilia has been reported to be commonly associated with pneumonia and urinary tract infection. However, biliary infection caused by S. maltophilia is very rare. Herein, we report on a case of acute cholecystitis that developed secondary to S. maltophilia bacteremia in a patient with hepatitis-B related liver cirrhosis and gallbladder stone.

A Large Hemorrhagic Pseudocyst in Patient with Valproic Acid-Induced Severe Acute Pancreatitis: A Case Report / 대한췌담도학회지

The occurrence of valporic acid (VPA)-induced pancreatitis is a rare condition, predominantly observed in adolescent. Also, the occurrence of VPA-associated with hemorrhagic pseudocyst is extremely rare. We report the case of a 54-year-old man who had been taking VPA for uncontrolled seizures. He was admitted to our hospital with complaints of abdominal pain and diagnosed with acute on chronic pancreatitis. There were no other causes explaining pancreatitis, and it was thought to be due to VPA therapy. Despite of cessation of VPA, there was ongoing severe abdominal pain with fever. The patient underwent follow-up CT, which revealed a large loculated fluid collection that was observed with intra-cystic hemorrhage. After treatment with percutaneous catheter drainage, he was discharged with regression of the pancreatic pseudocyst. VPA-associated pancreatitis with hemorrhagic pseudocyst is rare but possible. Therefore, this possibility should be considered in the cause of hemorrhagic pseudocyst in a patient taking VPA.

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

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.