Retroperitoneal Fibrosis with Duodenal Stenosis

Retroperitoneal fibrosis is a rare disease characterized by the formation of dense plaque of fibrous tissue covering the retroperitoneal structures. This disease is commonly presented as ureteral obstruction, but the involvement of duodenum is rare. We report a case of retroperitoneal fibrosis which was complicated with duodenal stenosis and was successfully treated with corticosteroids. A 58-yr-old man, who had history of aorto-iliac bypass graft due to arteriosclerosis obliterans with infrarenal aortic occlusion was admitted to the hospital with abdominal pain and a mass. Abdominal CT scan revealed the periaortic soft tissue mass encircling grafted aorta and stenosis of duodenal third portion. Retroperitoneal fibrosis with duodenal stenosis was diagnosed and prednisolone therapy was initiated. Follow-up CT scan showed that the patient responded to prednisolone therapy with eased pain, shrinking periaortic mass, and reduced duodenal stenosis.

A Case of a Pancreaticobiliary Fistula Accompanied by Pancreatic Duct Stones / 대한소화기내시경학회지

A Pancreaticobiliary fistula is a rare complication of acute necrotizing pancreatitis or pancreatic abscess. One case is herein reported of pancreaticobiliary fistula that was found following removal of pancreatic duct stones by extracorporeal shock-wave lithotripsy and endoscopic sphincterotomy. The patient was a 53-year old woman who was admitted with epigastric pain and fever. Endoscopic retrograde pancreatography revealed multiple stones in the main pancreatic duct. Pus from the pancreatic duct was drained through the major papilla during cannulation. On balloon cholangiogram obtained after removal of the stones, a direct fistulous connection between the main pancreatic duct and distal common bile duct was noted. It is speculated that pancreatic abscess or mechanical compression of pancreatic duct stones may contribute to the destruction of the intrapancreatic bile duct, which leads to the formation of a pancreaticobiliary fistula.

An Incidentally Found Emphysematous Cystitis / 대한신장학회잡지

Emphysematous cystitis is rare disease associated with gas in the bladder wall and lumen. Presentation is varied and sequelae may be minor, severe or fatal. Treatment consists of administration of appropriate antibiotics, control of blood glucose, and adequate bladder drainage. We present a case of emphysematous cystitis with diabetic woman, who did not present symptoms of cystitis but abdominal pain, nausea and vomiting. In our case, it was detected incidentally on simple radiography. After administration of susceptible parenteral antibiobcs and control of blood glucose level, her symptoms were improved.

A Case of Pulmonary Hypertension Associated with Portal Hypertension / 결핵

Since its first description in 1951 by Mantz and Craig, pulmonary hypertension associated with portal hypertension has been observed more frequently. In a recent prospective study Hadengue et al. reported 2% incidence of pulmonary hypertension in patients with portal hypertension. Thus this simultaneous occurrence can no longer be considered to be coincidental. The etiology remains unclear. It is most likely that vasoactive substances (,)normally metabolized by the liver(,) may gain have gained access to the pulmonary circulation through portosystemic collaterals in portal hypertension. In genetically susceptible individuals, these substances could lead to pulmonary hypertension by inducing vasoconstriction or direct toxic damage to the wall of the small pulmonary arteries. A recent case of pulmonary hypertension in a 49-year-old woman with portal hypertension due to liver cirrhosis is reported as well as a review of the literature.

A Case of type Ia glycogen storage disease associated with pulmonary hypertension / 결핵

A glycogen storage disease(GSD) type I is a metabolic disease caused by a deficiency in one of the components of the glucose-6-phosphatase(G-6-Pase) system. This disorder results in hypoglycemia, hepatomegaly, lactic acidemia, hyperlipidemia, and hyperuricemia. Common long(-)term complications include growth retardation, gout, hepatic adenomas, osteoporosis and renal disease. However the cardiovascular system is rarely involved, and only six cases of pulmonary hypertension associated with GSD I have been reported in the literature. We experienced a case of pulmonary hypertension with type I GSD. A 31-year-old man, who had discovered type I GSD and received portocaval shunt operation 22 years ago, was admitted to the hospital with the chief complaint of dyspnea. Echocardiographic examination and cardiac catheterization revealed severe pulmonary hypertension. Nitric oxide and oral prostacycline derivative(beraprost) were tried without acute favorable response. After one year with beraprost, dyspnea, exercise capacity and hemodynamic parameters were improved. We report this case with a review of the literature.

Nuclear Hyperploidy of Megakaryocytes and Platelet Aggregation Test in Essential Thrombocythemia / 대한혈액학회지

BACKGROUND: Essential thrombocythemia (ET) is a rare chronic myeloproliferative disorder characterized by an extremely high platelet count in the circulating blood and abnormal proliferation of the megakaryocytes in bone marrow, resulting in splenomegaly, thromboembolic or hemorrhagic complications. We studied the presence of nuclear hyperploidy of the megakaryocytes in bone marrow, the presence of abnormal response to the individual reagent on platelet aggregation test, and its clinical implication. METHODS: We analyzed the 43 cases of ET at the Asan Medical Center between January, 1989 and March, 1999. The Polycythemia Vera Study Group criteria were used to diagnose ET. RESULTS: Nuclear hyperploidy was observed at 43 cases (100%). Platelet aggregation test was done at 32 (74.4%) cases, of which 27 (84.4%) cases showed abnormal response to more than one reagent, 16 (50%) cases to more than two reagents. Abnormal response to epinephrine and collagen was most common, but 5 cases showed normal response. By individual reagent, 1 (3%) cases to adenosine diphosphate, 1 (3%) case to ristocetin, 22 (69%) cases to epinephrine, 19 (59%) cases to collagen showed abnormal response. CONCLUSION: We observe that nuclear hyperploidy of the megakaryocyts and abnormal response on platelet aggregation test are frequent in ET in this study.