A Case of Gastric Langerhans Cell Histiocytosis Showing Hypertrophic Folds / 대한소화기내시경학회지

Langerhans cell histiocytosis is a rare disease entity in which various tissue are infiltrated by proliferating histiocytes. Involvement of alimentary tract is rare (<5%), especially in the stomach. Only seven cases of gastric Langerhans cell histiocytosis have been reported in Enlglish literature. Diabetes insipidus is the most common endocrinologic complication. We report a case of Langerhans cell histiocytosis involving stomach and pituitary stalk. The diagnosis of gastric Langerhans cell histiocytosis and diabetes insipidus was based on gastric mucosal biopsy specimen from a Korean man with suspicious hypertrophic gastric folds of gastric body, water deprivation test and sella MRI.

A Case of Peutz-Jeghers Syndrome with Intussusception / 대한소화기내시경학회지

Peutz-Jeghers syndrome is a rare autosomal dominant disorder defined by hamartomatous polyps of gastrointestinal tracts and the occurrence of melanin spots on the mucocutaneous surface. This syndrome has important complications such as malignant transformation, gastrointestinal bleeding, and intussusception. About 50% of patients with Peutz-Jeghers syndrome involved Intussusception. A case of Peutz-Jeghers syndrome with intussusception was recently experienced and herein reported.

Percutaneous Endoscopic Biliary Stenting (PEBS) Using the Percutaneous Fine Needle Puncture Method in Malignant Complete Biliary Obstruction / 대한소화기내시경학회지

Nonsurgical drainage of malignant obstructive jaundice is an interesting alternative to surgical drainage in the palliative treatment of pancreaticobiliary neoplasms. Biliary drainage by endoprosthesis is as effective and better supported than percutaneous external drainage, but more difficult to control. Endoscopic retograde biliary drainage (ERBD) is a safe and effective biliary drainage procedure, and is indicated with malignant obstructive jaundice in patients on whom endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic sphincterotomy (EST) can be performed. A nonoperative method of palliation was used in patients with malignant obstructive jaundice, in whom a biliary endoprosthesis could not be placed endoscopically due to complete obstruction of the bile duct. A guide wire was manipulated through the lesion by a percutaneous transhepatic route, after puncturing the tumor by a fine needle, and retrieved from the duodenum through an endoscope. A stent was then passed through the endoscope over the guide wire across the stricture.

Blood Volume Change and Side Effects during Various Sodium Ramping in Hemodialysis / 대한신장학회잡지

Chronic hemodialysis patients frequently experience hemodialysis(HD)-related side effects caused by excessive ultrafiltration and abrupt change of osmolality. Sodium ramping in HD is known to reduce ultrafiltration-related side effects, but it frequently induces symptoms related to sodium overload. We wanted to know the relationship between blood volume changes and the side effects related to ultrafiltration during hemodialysis and whether we can individualize various sodium ramping methods according to the effect of change in blood volume( BV) and side effects of sodium ramping. We studied 9 hypotension-prone patients during HD. The duration of the study lasted for 5 weeks, each week using different sodium ramping protocols: protocol 1; dialysate [Na+] of 140mEq/L, protocol 2; dialysate [Na+] same as the predialysis serum [Na+], protocol 3; dialysate [Na+] was 20mEq/L greater than that of the patient's serum for 1hr, 10mEq/L greater than patient's serum [Na+] for 2hr and then the same as patient's serum [Na+] for the last 1hr, protocol 4; at the beginning of dialysis, dialysate sodium was ramped to 20mEq/L above the patient's serum sodium and then on a straight linear fashion lowered to the predialysis serum [Na+] at the end of dialysis, protocol 5; sodium was constantly ramped to 10 mEq/L above serum [Na+]. We measured the BV with Crit-Line IIR(In-Line Diagnostics, Corp., Riverdale, USA), the blood pressure during each HD and interdialytic weight gain. We documented subjective symptoms which occurred during the 5 treatment protocols by patient's questionnaire after each HD. The results were as follows. 1) The mean age of the patients(M:F=3:6) was 54.1years and 6 patients were diabetics. 2) There was no significant difference in the BV among the 5 protocols in both whole study population and individual. Neither was there a statistically significant difference in the BV with respect to hypotension during HD. 3) There were no episodes of hypotension(P value <0.001) with protocols 3, 4, 5 compared to protocols 1 and 2. 4) Three patients during protocols 4 and 5 experienced more thirst after HD than during protocol 1 and one patient during protocol 4, 5 had more interdialytic weight gain than the protocol 1. As a whole, patients while on protocol 4 & 5 experienced more thirst than protocol 1 but patients during protocol 3 experienced the same degree of thirst as protocol 1. In summary, sodium ramping reduced HD-related side effects but this benefit could not be explained on the basis of blood volume change measured by the Crit-Line IIR. Protocol 3 may be more appropiate sodium ramping method in 4 of the 9 patients. These data suggest that protocol 3 may be used before protocol 4, 5 when we apply sodium ramping to the patients who frequently have hypotension during HD.

A case of acquired angioedema with C1 esterase inhibitor deficiency / 천식및알레르기

Angioedema is a well-demarcated localized edema involving the deeper layers of the skin, including the subcutaneous tissue. Angioedema occurs with Cl esterase inhibitor (Cl INH) deficiency that may be inborn as an autosomal dominant characteristic or may be acquired. Acquired angioedema (AAE) is a rare disorder characterized by adult onset and lack of evidence of inheritance of the disease. Two types of AAE are known today: type I in which there are lowering of functional Cl INH, an underlying disease such as a B-cell disease, and no detectable autoantibodies to Cl INH, type II with anti Cl INH autoantibodies in the circulation without detectable underlying disease and with depressed functional Cl INH levels. We experienced a case of angioedema in a 29-year old man. He had no family history of angioedema and laboratory data showed depressed Cl-INH levels. We diagnosed the case as acquired type of angioedema. Even though we could not measure anti-Cl INH auto-antibodies, we identified the case as type II because there was no evidence of underlying disease.