An association between primary sclerosing cholangitis and ulcerative colitis is well known. But, primary sclerosing cholangitis with ulerative colitis has been rarely reported in children. The prevalence of primary sclerosing cholangitis among ulcerative colitis patiens is 3% in children. Primary sclerosing cholangitis is characterised by inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts. The diagnosis of primary sclerosing cholangitis based on biochemical, histologic and cholangiographic criteria. A twofold or greater elevation of serum alkaline phosphatase is required to suspect this diagnosis. The definitive diagnosis of primary sclerong cholangitis can usually made by cholangiography. The prognosis varies. No adequate treatment exists although a number of potential treatments have been evaluated. We experienced a case of primary sclerosing cholangitis with ulcerative colitis in a 14 year old girl. She was admitted with a history of intermittent bloody diarrhea and jaundice over a two year period. Hepatosplenomegaly and cholestasis had been noted. Abnormal liver function tests were noted. AST was 117U/l, ALT 179U/l, alkaline phosphatase 603U/l, gamma-GT 366U/l, total bilirubin 5.5mg/dl. An endoscopic retrograde cholecystopancreatography showed evidence of strictures, beading, and irregularities of intra and extrahepatic biliary system. Liver biopsy showed histologic findings compatible with a sclerosing cholangitis and evidence of periductular fibrosis. She sufferred from persistent cholestasis and sign of portal hypertension. She had developed recurrent episodes of variceal hemorrhages which had been successfully managed several times endoscopic variceal ligations.
Adolescent ; Alkaline Phosphatase ; Bile Ducts, Extrahepatic ; Bilirubin ; Biopsy ; Child ; Cholangiography ; Cholangitis ; Cholangitis, Sclerosing* ; Cholestasis ; Colitis ; Colitis, Ulcerative* ; Constriction, Pathologic ; Diagnosis ; Diarrhea ; Female ; Fibrosis ; Hemorrhage ; Humans ; Hypertension, Portal ; Inflammation ; Jaundice ; Ligation ; Liver ; Liver Function Tests ; Prevalence ; Prognosis ; Ulcer*

Intestinal neuronal dysplasia(IND) is a disease characterized clinically by symptoms of intestinal obstruction and pathologically by hyperplasia of the submucosal and myenteric plexuses with formation of giant ganglia. Chronic intestinal pseudo-obstruction is a clinical diagnosis, composed of myopathic form and neuropathic form, and normal intestinal histology. Intestinal neuronal dysplasia is a neuropathic form of chronic intestinal pseudo-obstruction. The clinical presentation and the course of IND is very variable. We experienced a case of intestinal neuronal dysplasia in a 5 year-old boy who had suffered from recurrent abdominal pain and vomiting for 3 years. Small bowel series showed multiple intestinal dilatations and delayed excretion of contrast media. He underwent exploratory laparotomy. However no mechanical causes for markedly dilated intestine were found and he received loop ileostomy. However, he suffered from recurrent vomiting and abdominal pain. So, he received repair-operaton. The pathology of surgical specimen showed hyperplasia of the submucosal and myenteric plexuses with giant ganglia. We report this case with a brief review of the related literatures.
Abdominal Pain ; Child, Preschool ; Contrast Media ; Diagnosis ; Dilatation ; Ganglia ; Humans ; Hyperplasia ; Ileostomy ; Intestinal Obstruction ; Intestinal Pseudo-Obstruction ; Intestines ; Laparotomy ; Male ; Myenteric Plexus ; Neurons* ; Pathology ; Vomiting

BACKGROUND: Optimal treatment for recurrent primary central nervous system lymphomas (PCNSLs) has not been defined yet and there is no general consensus about the salvage chemotherapy after high-dose methotrexate (HD-MTX)-based chemotherapy. The purpose of the present study was to evaluate the efficacy and safety of procarbazine, lomustine, and vincristine (PCV) chemotherapy for recurrent PCNSLs. METHODS: We reviewed eight immunocompetent patients (five males/three females, mean age: 56 years) who received salvage PCV chemotherapy (procarbazine 60 mg/m2, days 8 through 21: CCNU 110 mg/m2, day 1: vincristine 2 mg, days 8 and 28) for recurrent PCNSL and two patients switched to PCV chemotherapy due to severe adverse effects of HD-MTX chemotherapy. Radiologic responses, survival, and adverse effects were analyzed. RESULTS: Of the eight recurrent PCNSLs, three patients (37.5%) showed radiologic complete response, one patient (12.5%) showed partial response, and four patients (50%) showed progressive disease after PCV chemotherapy. Median progression free survival (PFS) from the first administration of PCV to relapse or last follow-up was 7 months (range 5-32 months) and median overall survival was 8 months (range 2-41 months). The two patients who switched to PCV chemotherapy showed PFS of 9 and 5 months from the beginning of PCV to relapse. The common side effects were thrombocytopenia, neutropenia, and peripheral neuropathy. There were 4 grade III or IV myelo-suppression, but no fatal complications, including severe hemorrhage or infection, were observed. CONCLUSION: Salvage PCV chemotherapy has a moderate anti-lymphoma activity for recurrent PCNSLs after the HD-MTX-based chemotherapy with tolerable toxicity.
Central Nervous System* ; Consensus ; Disease-Free Survival ; Drug Therapy* ; Female ; Follow-Up Studies ; Hemorrhage ; Humans ; Lomustine* ; Lymphoma* ; Methotrexate ; Neutropenia ; Peripheral Nervous System Diseases ; Procarbazine* ; Recurrence ; Salvage Therapy ; Thrombocytopenia ; Vincristine*

The mechanism by which propofol exerts its action is poorly understood, but may involve a non-specific effect on lipid membrane and has been shown to potentiate GABA-mediated synaptic inhibition. And, midazolam also acts through GABA receptor mediated increased chloride conductance. The aim of this study was to evaluate the dose response of midazolam, propofol and combination of these drugs, and determine possible interaction between two drugs in patients. The effect of propofol on the dose response curve for midazolam was studied in 260 nonpremedicated ASA physical status I or II female patients who were scheduled for elective operation. The response to the verbal command was used as an end-point of hypnosis. Dose response curves for midazolam, propofol, and their type of interaction was determined using Instat software package, nonlinear regression analysis, and algebraic(fractional) analysis of interaction. ED of midazolam and propofol was 0.11 mg/kg, 1.13 mg/kg and ED95 was 0.18 mg/kg, 1.67 mg/kg respectively. ED50 of combined drug(midazolam+propofol) in comhined dose response curve was 29% of each drug and the type of interaction between two drugs was found to be synergistic.
Anesthesia* ; Female ; Humans ; Hypnosis ; Membranes ; Midazolam* ; Propofol* ; Receptors, GABA

BACKGROUND: Although chemotherapy-related hepatic injury has been reported in colorectal cancer liver metastasis (CRLM) patients, the morphologic changes caused by chemotherapeutic agents and the effect of chemotherapy on postoperative outcome remain ill-defined. A comprehensive review of the morphologic changes in the post-chemotherapy non-neoplastic liver was performed and the clinical effect of preoperative chemotherapy in CRLM patients was analyzed. METHODS: Hematoxylin-eosin, Masson's trichrome and reticulin-stained slides from non-neoplastic livers obtained from 89 CRLM patients were analyzed, and the clinicopathologic features were correlated with the status of chemotherapy exposure. RESULTS: Histopathologic features of sinusoidal injury (sinusoidal dilatation, centrilobular perivenular fibrosis, parenchymal extinction lesions, small vessel obliteration, and hepatocyte plate disruption) were significantly more frequent in oxaliplatin-exposed livers (p<0.05). The extent of sinusoidal dilatation was positively correlated with increasing numbers of chemotherapy cycles (p=0.022). Abnormal preoperative liver function tests were more frequently seen (p<0.05) and postoperative total bilirubin was higher in the chemotherapy group (p=0.008). Postoperative morbidity was more common in the chemotherapy group (p=0.044). CONCLUSIONS: Sinusoidal injury is frequently seen in oxaliplatin-treated livers, and its presence, especially when extensive, should be documented in surgical pathology practice. The recognition of sinusoidal injury may provide helpful guidelines for surgeons in deciding the extent of hepatic resection.
Bilirubin ; Colorectal Neoplasms ; Dilatation ; Drug-Induced Liver Injury ; Fibrosis ; Glycosaminoglycans ; Hepatocytes ; Humans ; Liver ; Liver Function Tests ; Neoplasm Metastasis ; Pathology, Surgical

Purpose: Recently the incidence of vancomycin resistant enterococcus (VRE) infection and colonization has increased in the hospitalized patients. The purpose of present study is to examine the clinical significance of VRE infection and colonization in liver transplantation (LT) patients and to investigate the outcome. Methods: Among 194 patients who underwent LT from January 2001 to July 2004, 15 patients had at least one report of culture positive of VRE (VRE(+)). We compared the clinical outcome of 15 VRE(+) patients with those of VRE(-) patients. Results: One year mortality was higher in VRE(+) patients than VRE(-) patients (27% vs 4%; P=0.0001). Causes of death were primary graft non-function, infective endocarditis, MRSA sepsis and CMV pneumonitis. Mean duration of hospital stay was 102+/-63 days in VRE(+) patients, which shows significant difference with 51+/-30 days in VRE(-) patients (P=0.008). Cases who underwent gastroscopy (1.00+/-1.51 vs. 0.12+/-0.47; P=0.04) and percutaneous catheter drainage (1.33+/-1.49 vs. 0.40+/-1.05; P=0.03) were significantly more frequent in VRE(+) patients. The findings of gastroscopy were upper gastrointestinal bleeding (n=4), and bile duct obstruction (n=1). The contents of percutaneous catheter drainage were hematoma (n=3), bile juice (n=3) and ascites (n=1). Conclusion: VRE infected patients experienced more frequently gastrointestinal tract complications including bleeding or biliary complication and they showed higher 1-year mortality rate, although these patients died of causes other than VRE infection.
Ascites ; Bile ; Catheters ; Cause of Death ; Cholestasis ; Colon ; Drainage ; Endocarditis ; Enterococcus* ; Gastrointestinal Tract ; Gastroscopy ; Hematoma ; Hemorrhage ; Humans ; Incidence ; Length of Stay ; Liver Transplantation* ; Liver* ; Methicillin-Resistant Staphylococcus aureus ; Mortality ; Pneumonia ; Sepsis ; Transplants ; Vancomycin*

OBJECTIVE: Microscopic and endoscopic transsphenoidal approach (TSA) are major surgical techniques in the treatment of pituitary adenoma. Endoscopic endonasal transsphenoidal approach (EETSA) has been increasingly used for pituitary adenomas, however, its surgical outcome particularly in functioning pituitary adenoma has been debated. Here, we investigated the endocrine outcome of the patients with growth hormone (GH) and adrenocorticotropic hormone (ACTH) secreting pituitary adenoma treated by EETSA. METHODS: We treated 80 patients with pituitary adenoma by EETSA since 2004, of which 12 patients were affected by functioning pituitary adenomas (9 GH, 3 ACTH, 0 PRL; 9 macro, 3 micro). Surgical outcome of those patients treated by EETSA was compared with that of the 11 functioning pituitary adenoma patients (8 GH, 3 ACTH; 8 macro, 3 micro) who underwent sublabial microscopic TSA between 1997 and 2003. RESULTS: Imaging remission based on postoperative MRI was achieved in 8 (73%) and hormonal remission in 5 (45%) of 11 patients treated by sublabial microscopic TSA. Imaging remission was observed in 10 (83%, p=0.640) and hormonal remission in 10 (83%, p=0.081) of 12 patients by EETSA. CSF leakage was noticed in 2 (17%) of EETSA group and in 2 (18%) of sublabial microscopic TSA group. Panhypopituitarism was observed in 1 (9%) of EETSA group and in 3 (27%) of sublabial microscopic TSA group. CONCLUSION: EETSA appears to be an effective and safe method for the treatment of functioning pituitary adenomas.
ACTH-Secreting Pituitary Adenoma ; Adrenocorticotropic Hormone ; Endoscopy ; Growth Hormone ; Growth Hormone-Secreting Pituitary Adenoma ; Humans ; Hypopituitarism ; Pituitary Neoplasms

BACKGROUND/AIMS: Levosulpiride is the levo-enantiomer of sulpiride, a well-known antiemetic, antidyspeptic and antipsychotic drug. This study was undertaken to investigate the effects of levosulpiride on esophageal symptoms, esophageal peristalsis, and lower esophageal sphincter pressure (LESP), as well as evaluate the side effects in patients with nonspecific esophageal motility disorder (NEMD). METHODS: Thirty patients with NEMD (12 males and 18 females, with a mean age 48.6 +/- 13.0 years old) were administered 25 mg of levosulpiride t.i.d. for 2 weeks. Symptom assessment, esophageal manometry, blood biochemistry and serum prolactin concentrations were evaluated before and after treatment. For the evaluation of serum prolactin concentrations according to the dose of levosulpiride, 10 healthy volunteers were administered 25 mg or 12.5 mg of levosulpiride t.i.d., and serum prolactin concentrations were examined before and after treatment weekly. RESULTS: After treatment with levosulpiride, the symptom score was improved significantly (6.5 +/- 3.0 vs. 3.9 +/- 2.7, p < 0.05), but esophageal manometric findings were not improved. Two patients developed breast engorgement and the serum prolactin concentration was significantly elevated after treatment with levosulpiride. However, the increased serum prolactin level returned to a normal level within one week, and there was no difference in regards to the dose of levosulpiride. CONCLUSIONS: Levosulpiride did not improve the esophageal motor abnormality, but was effective for esophageal symptoms, which might be the result of the antidopaminergic effect on the central nervous system. Hyperprolactinemia developed in all patients, but it was normalized within a week, and symptoms for hyperprolactinemia were seen in only a few cases.
Biochemistry ; Breast ; Central Nervous System ; Esophageal Motility Disorders* ; Esophageal Sphincter, Lower ; Female ; Healthy Volunteers ; Humans ; Hyperprolactinemia ; Male ; Manometry ; Peristalsis ; Prolactin ; Sulpiride ; Symptom Assessment

PURPOSE: Primary aldosteronism (PA) is characterized by hypertension (HTN), hypokalemia, suppressed plasma renin activity, and inappropriate aldosterone secretion. The purpose of this study was to analyze postoperative results on blood pressure (BP), and to determine the factors associated with resolution of HTN after adrenalectomy for PA. METHODS: One hundred eight patients (66 females and 42 males) with a mean age of 46 years underwent adrenalectomy for PA between January 1, 1996 and September 30, 2009. Their clinical characteristics and biochemical parameters were reviewed retrospectively. RESULTS: All patients had HTN preoperatively and 20 patients (18.1%) had uncontrolled HTN. Hypokalemia was evident in 89.1% of patients, cardiovascular events in 4.5% and cerebrovascular events in 8.2%. There was a significant decrease in both systolic BP and diastolic BP postoperatively, as compared with that before operation. Median systolic BP decreased from 150 mmHg to 125 mmHg at the last follow-up (P<0.01), and median diastolic BP decreased from 93.5 mmHg to 81.5 mmHg (P<0.01). Sixty two (57.4%) patients were cured of HTN and did not require any hypertensive agent, and 38 (35.1%) patients had an improvement in BP control, whereas 9 (8.3%) patients had no change in BP. Univariate analysis showed that duration of HTN and more than two HTN treatment agents were independent factors predicting sustained hypertension after surgery. CONCLUSION: The duration of HTN and the severity of HTN are factors influencing persistence of HTN after operation for a PA.
Adrenalectomy* ; Adrenocortical Adenoma ; Aldosterone ; Blood Pressure ; Female ; Follow-Up Studies ; Humans ; Hyperaldosteronism* ; Hypertension ; Hypokalemia ; Plasma ; Renin ; Retrospective Studies

This study outlines the current status of the autopsy practice and the medical records for autopsies at the Department of Pathology, Seoul National University Hospital. Total number of autopsy cases from 1954 to 1995 was 3,131. Adults aged over 17 were 371 cases and children were 2,515 cases. The demographic data in 245 cases was not available. The number of adult autopsies and its proportion among total number of autopsies during 10-year periods decreased from 144 cases (40%) during the 10-year-period from 1956 to 52 cases (3%) during the 10-year-period from 1986. The number of children cases during the same period groups increased slightly from 210 cases (58%) to 393 cases (25%). But the number of fetal cases increased rapidly from 7 cases (2%) to 1,146 cases (72%). Among fetal autopsies the proportion of fetuses died earlier than 24 weeks of gestation increased and this figure exceeds that of fetuses that died later than 24 weeks of gestation from 1992. Forty percent of the cases were submitted from the clinical departments of the Seoul National University Hospital but the remainders were referred from 73 hospitals. Final autopsy diagnoses were analysed according to the Korean Standard Classification of Disease (KCD)-3 coding system and by searching key words for all cases. Common diagnoses as coded among cases from 1990 were P9, P0, P2, Q2 and Q0. Common diseases by key words for adult cases were liver disease, tuberculosis and pneumonia. Common diseases for children cases were pneumonia, hyaline membrane disease, meningitis and tuberculosis. Through this study we could show the importance of autopsy services for fetuses. We could also establish a regular registration system for autopsies at general hospitals.
Adult ; Autopsy* ; Child ; Classification ; Clinical Coding ; Diagnosis ; Epidemiology ; Fetus ; Hospitals, General ; Humans ; Hyaline Membrane Disease ; Infant, Newborn ; Liver Diseases ; Medical Records ; Meningitis ; Pathology ; Pneumonia ; Pregnancy ; Seoul* ; Tuberculosis