Primary biliary cirrhosis(PBC) is a chronic cholestatic liver disease of unknown origin. The small and medium sized intrahepatic bile ducts are destroyed by an inflammatory process, which, it has been suggested, is of the autoimmune type. It is strongly associated with the presence of antimitochondrial antibodies, predominantly IgM and IgG. The liver changes are classified into four stages, of which stage IV represents the development of cirrhosis, which required orthotropic liver transplantation in the longrun. The prevalence rates was reported 128 per millon in Sweden , but the disease is relatively rare in Oriental area. In medical treatment, long-term administration of ursodeoxycholic acid improves both clinical and biochemical signs, slows the progression of the disease and reduces the complication requiring liver transplantation. We report two cases of PBC, one with histologically proven cirrhosis, and the other with bile duct destruction consistent with stage III and hypothyroidism.
Antibodies ; Bile Ducts ; Bile Ducts, Intrahepatic ; Fibrosis ; Hypothyroidism ; Immunoglobulin G ; Immunoglobulin M ; Liver ; Liver Cirrhosis, Biliary* ; Liver Diseases ; Liver Transplantation ; Prevalence ; Sweden ; Ursodeoxycholic Acid

OBJECTIVE: The purpose of this study was to evaluate the relationship between uterine size and surgical outcomes in women undergoing total vaginal hysterectomy. METHODS: We reviewed the medical records of the 170 patients who underwent total vaginal hysterectomy from February 2001 to November 2003. These patients were stratified into two groups; Group I, patients with uterine weight of between 300 gm to 1000 gm; Group II, patients with uterine weight of <300 gm. The two groups were compared for ages, parity, uterine weight, previous abdominal operations, concurrent surgical procedures, postoperative discharge day, bleeding amount, perioperative hemoglobin concentration change and operative time. RESULTS: 1. There were no significant difference between the two groups with respect to postoperative discharge day, concurrent surgical procedures, bleeding amount and perioperative hemoglobin concentration change. 2. The rate of surgical complications were similar in the two groups (Group I 12.5%; Group II 13.1%) (P=.660). 3. Operative time for vaginal hysterectomy was slightly longer for women in Group I than Group II (Group I 78.4 +/- 27.1 minutes; Group II 62.9 +/- 22.7 minutes) (P=.176), but the difference between the two groups in operative time was not statistically significant. CONCLUSION: The vaginal hysterectomy is as safe and effective for the woman with enlarged uterus (300-1000 gm) as for the woman with uterine weight of <300 gm. Uterine enlargement (uterine weight > 300 gm) is not an absolute contraindication to vaginal hysterectomy.
Female ; Hemorrhage ; Humans ; Hysterectomy, Vaginal* ; Medical Records ; Operative Time ; Parity ; Postoperative Care ; Uterus

PURPOSE: This study evaluated the feasibility of a laparoscopic approach in children with generalized peritonitis secondary to perforated appendicitis. MATERIALS AND METHODS: We retrospectively analyzed the medical records of patients who underwent laparoscopic appendectomy with drainage for generalized peritonitis secondary to perforated appendicitis at our hospital between September 2001 and April 2012. Laparoscopic outcomes were compared with outcomes of an open method for perforated appendicitis. RESULTS: Ninety-nine patients underwent laparoscopic appendectomy (LA) for generalized peritonitis from perforated appendicitis, and 87 patients underwent open appendectomy (OA) for perforated appendicitis. Wound infection was more common in the OA group (12.6%) than in the LA group (4.0%; p=0.032). The incidence of intestinal obstruction during long-term follow-up was significantly higher in the OA group (4.6% vs. 0.0% in the LA group; p=0.046). LA was possible in most patients for whom LA was attempted, with a conversion rate of 10.8%. Conversion to OA was affected by the preoperative duration of symptoms and the occurrence of intraoperative complications. CONCLUSION: LA is feasible for use in children with generalized peritonitis from perforated appendicitis, with reasonable open conversion and perioperative complication rates comparable to those of the OA group.
Appendicitis/*complications/*etiology/*surgery ; Child ; Child, Preschool ; Female ; Humans ; Laparoscopy/*methods ; Male ; Retrospective Studies ; Treatment Outcome

The serial transverse enteroplasty (STEP) procedure is a novel technique to lengthen and taper the bowel in patients with short bowel syndrome. The advantages of STEP include not only technical ease and simplicity, but also the ability to repeat the procedure. Herein, we report a case of extreme short bowel syndrome that was successfully treated by the second STEP procedure. A 3-day old newborn girl underwent STEP because of jejunal atresia with the small bowel length of 15 cm, but her bowel elongation was not enough to escape from short bowel syndrome. At the age of 6 months, she underwent a second STEP procedure. The bowel lengthening by the second STEP made her tolerable to enteral feeding with body weight gain and rescued her from short bowel syndrome. This case showed that second STEP is very helpful in treatment of extreme short bowel syndrome.
Digestive System Surgical Procedures ; Female ; Humans ; Infant ; Intestinal Atresia/surgery ; Short Bowel Syndrome/radiography/*surgery

Congenital atrichia is an anomaly characterized by congenital absence of hair in varying degrees as well as the ypopigmentation & poor development of the abnormal residual hair on the scalp, eyebrows, eyelashes & body. Two cases of congenital atrichia in siblings were reviewed. The first case was associated with congenital megacolon and skin biopsy of scalp (esp. parietal region) was non-specific. The second case was associated with bilateral inguinal hernia. Congenital atrichia is inherited by autosomal dominant or recessive. Acording to the limited pedigree data, our cases were X-linked recessive inheritance We report these cases with a review of related literatures.
Biopsy ; Eyebrows ; Eyelashes ; Hair ; Hernia, Inguinal ; Hirschsprung Disease ; Humans ; Pedigree ; Rabeprazole ; Scalp ; Siblings* ; Skin ; Wills

Congenital atrichia is an anomaly characterized by congenital absence of hair in varying degrees as well as the ypopigmentation & poor development of the abnormal residual hair on the scalp, eyebrows, eyelashes & body. Two cases of congenital atrichia in siblings were reviewed. The first case was associated with congenital megacolon and skin biopsy of scalp (esp. parietal region) was non-specific. The second case was associated with bilateral inguinal hernia. Congenital atrichia is inherited by autosomal dominant or recessive. Acording to the limited pedigree data, our cases were X-linked recessive inheritance We report these cases with a review of related literatures.
Biopsy ; Eyebrows ; Eyelashes ; Hair ; Hernia, Inguinal ; Hirschsprung Disease ; Humans ; Pedigree ; Rabeprazole ; Scalp ; Siblings* ; Skin ; Wills

We have experienced a case of pseudocyst of transverse mesocolon in 3-year-old male child. Operative findings showed that the pseudocyst was originated from the transverse mesocolon, and was not connected to the pancreas. A colonic duplication has been also found incidentally near the pseudocyst. On microscopic examination of specimen, ectopic pancreatic tissue was noted in transverse mesocolon. We think that this pseudocyst may be originated from the ectopic pancreas of the transverse mesocolon. To our knowledge, this is the first case of pseudocyst originated from ectopic pancreas associated with colonic duplication, and the pathogenesis is discussed.
Child ; Child, Preschool ; Colon* ; Humans ; Male ; Mesocolon* ; Pancreas*

BACKGROUND: The objective confirmation of subjective symptom of patient is important in the primary care consisted largely by functional disorders of which mechanisn could not explain the symptoms clearly. Definite diagnostic method is not established yet for the functional disorders desptie the fact that various investigations have been done. So, we tried to reveal the relationship between the value of indicator drop(ID) from electroacupunctrure point accordint to Voll and the clinical diagnosis and subjective symptom by using noninvasive electroacupuncture diagnosis according to Voll. METHODS: Among the patients of three university level hospital health care center from April to June 1997, consenting 203 persons were enrolled. Various laboratory finding and ID from EAV were measured by double blind test method. In parallel, Subjective symptom was classified by each organ. Validity was tested by the relationship between the gastrofiberscopy finding and the stomach control measurement point ID. RESULTS: There were statistically significant ID increases in the CMPs of endocrine, lung, circulation system, gastrointestinal system, kidney and bladder compared to alboratory findings or subjective symptom by the comparison between the variables of the assessed clinical or laboratory findings and organ specific CMP score. Especially, the ID increase of stomach shows 79.3%-90.9% positive predictive value to positve findings of gastrofiberscopy when gastofiberscopy is defined to confirmation test of gastritis, gastric ulcer and duodenal ulcer. CONCLUSION: Our results show that the noninvasive electrodiagnostic method result by measuring EAV of organ system is related statistically to subjective symptoms and laboratory findings. Also they show that it could be useful tool as a clinical diagnostic method. We suggest that further study is needed to reveal organ specific sensitivity, specificity, positivE and negative predictive value by using ccnfirmation method of organ specific disease.
Delivery of Health Care ; Diagnosis ; Duodenal Ulcer ; Electroacupuncture ; Gastritis ; Humans ; Kidney ; Lung ; Primary Health Care ; Sensitivity and Specificity ; Stomach ; Stomach Ulcer ; Urinary Bladder

19 years old girl presented with bilateral 1, 4th brachymetatarsia. We have used an autograft interposition technique to lengthen the brachymetatarsia involving first and fourth metatarsal. The technique was to graft the bone fragment from the relatively long second and third metatarsal bone to be used as an autograft to the short first and fourth metatarsal bone. The method is superior in not having the necessity of a long term external fixator which is needed using callotasis method and in also avoiding the inconvenience of performing an allograft. Absence of donor site complication during autograft from iliac bone is also an advantage to be mentioned. Our technique can therefore be ascertained as a successful method in both cosmetic results and improvement of symptoms including reduction in length of recovery.
Cosmetics ; External Fixators ; Humans ; Imidazoles ; Metatarsal Bones ; Nitro Compounds ; Osteogenesis, Distraction ; Tissue Donors ; Transplantation, Homologous ; Transplants

PURPOSE: Treatment for esophageal atresia has advanced over several decades due to improvements in surgical techniques and neonatal intensive care. Subsequent to increased survival, postoperative morbidity has become an important issue in this disease. The aim of our study was to analyze our experience regarding the treatment of esophageal atresia. METHODS: We reviewed and analyzed the clinical data of patients who underwent surgery for esophageal atresia at Severance Children's Hospital from 1995 to 2010 regarding demographics, surgical procedures, and postoperative outcomes. RESULTS: Seventy-two patients had surgery for esophageal atresia. The most common gross type was C (81.9%), followed by type A (15.3%). Primary repair was performed in 52 patients. Staged operation was performed in 17 patients. Postoperative esophageal strictures developed in 43.1% of patients. Anastomotic leakages occurred in 23.6% of patients, and recurrence of tracheoesophageal fistula was reported in 8.3% of patients. Esophageal stricture was significantly associated with long-gap (> or =3 cm or three vertebral bodies) atresia (P = 0.042). The overall mortality rate was 15.3%. The mortality in patients weighing less than 2.5 kg was higher than in patients weighing at least 2.5 kg (P = 0.001). During the later period of this study, anastomotic leakage and mortality both significantly decreased compared to the earlier study period (P = 0.009 and 0.023, respectively). CONCLUSION: The survival of patients with esophageal atresia has improved over the years and the rate of anastomotic leakage has been significantly reduced. However, overall morbidities related to surgical treatment of esophageal atresia still exists with high incidence.
Anastomotic Leak ; Constriction, Pathologic ; Demography ; Esophageal Atresia ; Esophageal Stenosis ; Humans ; Incidence ; Infant, Newborn ; Intensive Care, Neonatal ; Prognosis ; Recurrence ; Tracheoesophageal Fistula