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1/222. Idiopathic thrombocytopenic purpura after a living-related liver transplantation.

    BACKGROUND: Idiopathic thrombocytopenic purpura (ITP) is a rare complication after liver transplantation. We describe three cases of ITP in pediatric patients after a living-related liver transplantation (LRLT). methods: Of 266 patients who underwent an LRLT between June 1990 and June 1996, severe thrombocytopenia developed in three pediatric patients after transplantation, and ITP was also diagnosed. The original disease was biliary atresia in all cases, and the patients were given a partial liver graft from a living-related mother and subsequently treated with tacrolimus and low-dose steroids as an immunosuppressive regimen. RESULTS: The duration until the onset of ITP after transplantation in the three cases was 1 day, 3 months, and 13 months, respectively. The platelet-associated IgG levels increased in all cases. A preceding viral infection was suspected in two of the three cases. All patients were treated with intravenous gamma globulin with a transient recovery of thrombocytopenia in two cases and a sustained recovery in another. CONCLUSIONS: Transplant clinicians need to be aware of the possibility of ITP complication because a sudden onset of severe thrombocytopenia can occur even in patients who are apparently doing well after undergoing an LRLT.
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ranking = 1
keywords = atresia
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2/222. Left ventricle to pulmonary artery conduit in treatment of transposition of great arteries, restrictive ventricular septal defect, and acquired pulmonary atresia.

    Progressive cyanosis after banding of the pulmonary artery in infancy occurred in a child with transposition of the great arteries and a ventricular septal defect, and a Blalock-Taussig shunt operation had to be performed. At the time of correction a segment of pulmonary artery between the left ventricle and the band was found to be completely occluded so that continuity between the left ventricle and the pulmonary artery could not be restored. A Rastelli type of operation was not feasible as the ventricular septal defect was sited low in the muscular septum. Therefore, in addition to Mustard's operation, a Dacron conduit was inserted from the left ventricle to the main pulmonary artery to relieve the obstruction. Postoperative cardiac catheterization with angiocardiography indicated a satisfactory haemodynamic result. The patient remains well 11 months after the operation. This operation, a left ventricle to pulmonary artery conduit, may be used as an alternative procedure in patients with transposition of the great arteries, intact interventricular septum, and obstruction to the left ventricular outflow, if the obstruction cannot be adequately relieved.
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ranking = 4
keywords = atresia
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3/222. tetany, tetanus or drug reaction? A case report.

    An 8-year-old child was given perphenazine to prevent vomiting after surgery and developed an acute dystonic reaction; There were features of both tetany and tetanus without any of the classical features of extrapyramidal disturbance. The diagnostic difficulties are discussed. The reaction was treated successfully with i.v. diazepam. The prescribing of anti-emetics after surgery is examined critically with special reference to children.
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ranking = 0.093012958287767
keywords = anus
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4/222. Primary antiphospholipid syndrome: a cause of catastrophic shunt thrombosis in the newborn.

    This is a unique report of systemic-to-pulmonary artery shunt thromboses secondary to primary antiphospholipid syndrome and antithrombin iii deficiency in a neonate with cyanotic congenital heart disease. This infant with tricuspid atresia experienced thromboses of two modified Blalock-Taussig shunts en route to a bidirectional cavo-pulmonary shunt and potential future Fontan operation. Chronic warfarin anticoagulation has prevented additional thrombo-embolic events.
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ranking = 1
keywords = atresia
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5/222. tetanus after a resection for a gangrenous perforated small intestine: report of a case.

    We report herein the case of a 75-year-old man who developed severe tetanus 24 h after the resection of a gangrenous perforated small intestine. It seemed that the tetanus was caused by a spillage of the intestinal contents harboring clostridium tetani; however, this was not identified by a culture. The diagnosis of tetanus was made only when opisthotonus in this patient became evident and normal tetanus treatment proved to be successful.
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ranking = 0.14882073326043
keywords = anus
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6/222. Right ventricular thrombosis early after bidirectional Glenn shunt.

    thrombosis in the right ventricle occurred early after bidirectional superior cavopulmonary shunt in 2 patients with pulmonary atresia with intact ventricular septum and major right ventricular coronary artery communication, and perioperative brain infarction occurred in 1 patient. Clinicians should be aware of the hazards of this potentially lethal complication, and transfusion of platelets and fresh plasma should be minimized. Although the hemodynamic state is good, echocardiography should be performed frequently and strict anticoagulation should be started as early as possible.
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keywords = atresia
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7/222. Combined Rastelli and atrial switch procedure: a 10-year follow-up.

    We performed a combined atrial (Mustard) and ventricular (Rastelli) repair on a previously palliated patient with situs inversus, atrioventricular discordance, ventricular septal defect, and pulmonary atresia. The suitability and durability of this operative strategy is supported by the satisfactory hemodynamic and functional status of the patient 10 years later.
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ranking = 1
keywords = atresia
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8/222. Rapid two-stage repair of S,L,L, ventricular septal defect, pulmonary atresia, ebstein anomaly of the tricuspid valve, and stenotic pulmonary arteries.

    The repair of a corrected transposition of the great arteries, ventricular septal defect, and pulmonary atresia is presented. An ebstein anomaly of the tricuspid valve, dextrocardia, and severe distortion of the pulmonary arteries complicated the surgical procedure, which was performed in two stages. Reconstruction of the pulmonary arteries and a bidirectional cavopulmonary anastomosis were performed first; Rastelli and hemi-Mustard procedures completed the correction. The rationale and the possible indications of this "one and a half ventricle" repair are discussed.
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ranking = 5
keywords = atresia
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9/222. thromboembolism originated from the pulmonary artery stump after Fontan operation.

    Cerebral thromboembolism is a rare but serious complication after Fontan operation. This is the report of a patient who underwent a successful intracardiac thrombectomy for cerebral thromboembolism after Fontan operation. A 2-year-old girl was referred to us with the diagnosis of tricuspid atresia without pulmonary stenosis, normally related great arteries, and a ventricular septal defect. Although she underwent a successful Fontan operation and division of the main pulmonary artery, she developed a cerebrovascular event at 3 weeks after the operation. echocardiography demonstrated a large thrombus within the residue of the main pulmonary artery, and suggested that the thrombus had migrated into the systemic circulation by way of the ventricular septal defect. At 2 weeks after the cerebrovascular event, she underwent thrombectomy and excision of the pulmonary valve. Although she has developed slight left-sided hemiparesis, she is leading a normal life at 1 year after the operation.
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ranking = 1
keywords = atresia
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10/222. intestinal obstruction after lung transplantation in children with cystic fibrosis.

    BACKGROUND/PURPOSE: Distal intestinal obstruction syndrome (DIOS) occurs in 15% of patients with cystic fibrosis (CF). The authors reviewed their experience to determine the incidence, risk factors, and natural history of adhesive intestinal obstruction and DIOS after lung transplantation. methods: Eighty-three bilateral transplants were performed in 70 CF patients between January 1990 and September 1998. All were on pancreatic enzymes preoperatively, and none had preoperative bowel preparation. Fifty-six patients (80%) had prior gastrostomy (n = 54) or jejunostomy (n = 2). Eighteen patients (25.7%) had a previous laparotomy for meconium ileus (n = 8), fundoplication (n = 4), liver transplant (n = 1), jejunal atresia (n = 1), Janeway gastrostomy takedown (n = 1), pyloromyotomy (n = 1), free air (n = 1), or appendectomy (n = 1). RESULTS: After lung transplantation, 7 patients (10%) required laparotomy for bowel obstruction (6 during the same hospitalization, and 1 during a subsequent hospitalization). The causes of obstruction were adhesions only (n = 1), DIOS only (n = 2), and a combination of DIOS and adhesions (n = 4). Adhesiolysis was performed in the 5 patients with adhesions, and a small bowel resection was also performed in 1 patient. DIOS was treated by milking secretions distally without an enterotomy (n = 3) with an enterotomy and primary closure (n = 1) or with an end ileostomy and mucus fistula (n = 2). Five had recurrent DIOS early postoperatively. One resolved with intestinal lavage, 2 were treated successfully with hypaque disimpaction, and 2 underwent reoperation; 1 required an ileostomy. The most important risk factor for posttransplant obstruction was a previous major abdominal operation. Obstruction occurred in 7 of 18 (39%) who had undergone a prior laparotomy versus 0 of 52 who had not (P < .001, chi2). CONCLUSIONS: (1) The incidence of intestinal obstruction is high after lung transplantation in children with CF. (2) Previous laparotomy is a significant risk factor. (3) Recurrent obstruction after surgery for this condition is common. (4) Preventive measures such as pretransplant bowel preparation and early postoperative bowel lavage may be beneficial in these patients.
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ranking = 1
keywords = atresia
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