Problems during and after pregnancy in female patients with biliary atresia.

Background/purpose: Advances in the management for biliary atresia (BA) have improved the prognosis and has greatly increased the number of long-term survivors. Even the long-term survivors, however, still face some problems. This retrospective review was performed to assess pregnancy-associated issues in long-term survivors after surgery for BA.

Long-term outcome in type I biliary atresia.

Purpose: This retrospective study reviews the long-term outcome of type I biliary atresia (BA).

Methods: Three hundred twenty-three patients with BA, including 50 with type I, underwent corrective surgery. The surgical results, role of cholangiograms during the corrective surgery, late complications, and current statuses were evaluated.

Concentrations of copper and zinc in liver and serum samples in biliary atresia patients at different stages of traditional surgeries.

Many patients with biliary atresia (BA) have impaired metabolism of copper (Cu) and zinc (Zn) because of the obstruction of bile ducts. An excessive Cu accumulation is cytotoxic and results in fibrosis in hepatic tissues. Since Zn works antagonistically to Cu, lower Zn concentrations may deteriorate liver damage.

Primary tracheal schwannoma (neurilemoma) in a 9-year-old girl.

The authors report a case of primary tracheal schwannoma (neurilemoma) causing symptoms of airway obstruction in a 9-year-old girl. Computerized tomography scan showed a polypoid intratracheal mass obstructing 73% of the lumen. Partial tracheal resection with primary anastomosis was performed.

Postoperative corticosteroid therapy for bile drainage in biliary atresia--a nationwide survey.

Purpose: Members of the Japanese Biliary Atresia Society were surveyed to determine their current practice regarding early use of corticosteroids after Kasai's operation.

Methods: Questions included the patient's background data, dosage, timing, complications, and outcome. Anicteric survival with the native liver was statistically compared between groups categorized by steroid dosage using Kaplan-Meier analysis.

Cholangitis as a late complication in long-term survivors after surgery for biliary atresia.

Background/purpose: A retrospective review was performed to assess the utility of diagnostic imaging (DI), efficacy of treatment, and outcome of late cholangitis in long-term survivors after surgery for biliary atresia.

Methods: Sixty-one patients surviving without liver transplantation (LTx) for more than 20 years were divided into 2 groups depending on whether cholangitis developed after age 20. Clinical factors including the type of obstruction, the age at the initial operation, and the early complication with cholangitis were compared between the 2 groups.

Biliary atresia with associated complicated anorectal and urogenital malformations.

We report on a girl with biliary atresia (BA) who also suffered with anorectal agenesis without fistula and complicated urogenital malformation. The outcome of patients with these severe anomalies is poor, but she has survived without liver and/or renal transplantation for more than 3 years. A careful treatment plan for each anomaly in addition to prevention of cholangitis and urinary tract infection is indispensable for managing these complicated anomalies.

Development of an implantable artificial anal sphincter using a shape memory alloy.

Background/purpose: The authors created a new artificial anal sphincter using a shape memory alloy (AS-SMA) to treat fecal incontinence and evaluated its validity.

Methods: AS-SMA consists of 2 Ti-Ni plates to sandwich the intestine and generates a pressure of 55 mm Hg at its resting position. With the electric power supply, the 2 metals bend to form an almondlike shape making a maximum gap of 33 mm between each other at the temperature of 55 degrees C.

Long-term efficacy of partial splenic embolization in children.

Purpose: To elucidate the role of partial splenic embolization (PSE) procedures, long-term outcome was assessed in terms of the recurrence of thrombocytopenia.

Methods: A retrospective study was performed after 41 PSE procedures in 36 patients for hypersplenism owing to portal hypertension. The underlying disease was biliary atresia in 32 patients, extrahepatic portal obstruction in 3, and idiopathic cirrhosis in 1.

Clinical significance of 99mTc-DTPA-galactosyl human serum albumin liver scintigraphy in follow-up patients with biliary atresia.

Purpose: Technetium 99m DTPA-galactosyl human serum albumin (GSA) liver scintigraphy was performed in follow-up patients with biliary atresia, and its clinical significance was investigated.

Methods: Between 1994 and 2001, GSA liver scintigraphy was performed 153 times in 57 follow-up patients. HH15, LHL15, and H/L15 (HH15/LHL15) were obtained.

A newly developed small-caliber videoduodenoscope for endoscopic retrograde cholangiopancreatography in children.

Background: In neonates and younger children, the procedure of endoscopic retrograde cholangiopancreatography (ERCP) is now optimally performed using a small-caliber fiberoptic duodenoscope. However, there have been no reports on a specially designed videoduodenoscope for pediatric ERCP.

Goal: To evaluate performance and clinical usefulness of a newly developed small-caliber videoduodenoscope for ERCP in children.

Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese Biliary Atresia Registry.

Purpose: The aim of this study was to elucidate the epidemiology and short- and long-term results of biliary atresia in Japan analyzing the data of the Japanese Biliary Atresia Registry (JBAR).

Methods: In 1989, the Japanese Biliary Atresia Society started a nationwide registry, JBAR, to investigate all aspects of biliary atresia. A total of 1,381 patients, 863 girls, 507 boys, and 11 unknown, were registered between 1989 and 1999.

The functional roles of porcine CD80 molecule and its ability to stimulate and regulate human anti-pig cellular response.

Background: The pig is currently considered to be the most likely candidate for a xenogenic-organ source. Anti-pig human T-cell response via co-stimulatory molecules has been studied with great interest. The soluble form of porcine CD80 has recently been cloned and characterized, but the sequence of the transmembrane form has not been determined.

Clinical characteristics and management of congenital esophageal stenosis: a report on 14 cases.

Purpose: The aim of this study was to review 14 patients with congenital esophageal stenosis (CES) from diagnostic and therapeutic points of view.

Methods: From 1976 to 1999, 14 patients with CES were treated at the authors' hospital. In each diagnostic examination, an esophagogram, 24-hour pH monitoring, a manometric study, and an endoscopy were performed.

Serum insulinlike growth factor-I in biliary atresia.

Background/purpose: Low level of Insulinlike growth factor-I (IGF-I) has been reported in children with chronic liver disease like biliary atresia (BA) awaiting liver transplantation. However, there has been no report on IGF-I in BA managed without liver transplantation.

Methods: The authors measured IGF-I and growth hormone (GH) in 21 postoperative BA, and 17 choledochal cysts (CC) as a control with normal liver function.

[Problems and reformation of the board of medical specialties in Japan].

The Board of Medical Specialties, which is closely related to postgraduate training in Japan, including the Board of Surgery, has serious defects in its system because it has developed on its own initiative and has been administered by a medical society. Many of the public are not aware of its existence, and its workings are difficult for them to understand. In the past 20 years, the Organization for the Board of Medical Societies (tentative English translation) has aimed to ensure sound development of the Board of Medical Specialties.