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Biomedical subjects

A Shun

Publications and source records attributed to A Shun.

At least 19 recordsLinked to original sources

Routine contralateral orchiopexy for children with a vanished testis.

AIM: The aim was to determine whether routine contralateral orchiopexy is justified in a child with a vanished testis from intrauterine torsion. METHODS: A retrospective study of thirty-one consecutive boys with a vanished testis who underwent contralateral orchiopexy with the sutureless technique over an eight-year (1995 - 2002 inclusive) period was carried out. Operative findings were analyzed. All patients have been followed to date and interviewed by telephone. RESULTS: Of the thirty-one patients, 22 had testicular abnormalities (71 %). Five patients (16 %) had abnormalities that could predispose them to metachronous torsion. These included bell clapper deformity (3), horizontal lie (1), and ectopic testis (1). Other abnormalities were abnormal epididymal-testicular fusion (2), hydrocele (2), and one testis did not show compensatory hypertrophy. CONCLUSIONS: Sixteen percent of patients had testicular abnormalities that could predispose them to metachronous torsion. There was no morbidity from the operation and no post-orchiopexy torsion on follow-up. Therefore, routine contralateral orchiopexy in a boy with a vanished testis appears to be safe and effective. A large multicenter trial should be done to investigate these preliminary findings. If confirmed, contralateral orchiopexy should be advocated.

Child↗

Pulmonary hypertension--a new manifestation of mitochondrial disease.

Mitochondrial respiratory chain (RC) abnormalities in children can present as multiorgan disease, including liver failure, usually within the first year of life. Cardiorespiratory complications have previously been described in association with RC defects; however, to our knowledge no cases of pulmonary hypertension have been described. We discuss two patients with proven mitochondrial RC liver disease who developed severe pulmonary hypertension, one subsequent to cadaveric orthotopic liver transplantation, the second in the neonatal period. It is our contention that pulmonary hypertension should now be included as another potential manifestation of paediatric mitochondrial disease.

Adenosine Triphosphate↗

Biliary dyskinesia: is the problem with Oddi?

We report our experience of cholecystectomy for treating symptoms suggestive of biliary disease in association with a decreased gallbladder ejection fraction (GBEF) but without evidence of cholelithiasis. Five children with normal biliary ultrasounds were evaluated between January 1990 and December 2000 for recurrent upper abdominal pain. Based on a cholecystokinin (CCK)-provoked GBEF of less than 50% and the absence of any other gastrointestinal pathology, patients underwent cholecystectomy with operative cholangiography for presumed biliary dyskinesia. Pathological examination demonstrated chronic inflammation in all cases. Two patients had complete resolution of their symptoms, but three had persistent pain following surgery. Biliary dyskinesia seems an uncommon cause of persistent abdominal pain in childhood. Cholecystectomy was not always effective in relieving symptoms. Biliary scintigraphy with CCK provocation should not be used as the sole criterion for cholecystectomy. Sphincteric manometry may be valuable in the assessment of this small group of patients to avoid inappropriate intervention. The future perhaps lies in better understanding of the physiological action and pharmacological control of the sphincter of Oddi.

Biliary Dyskinesia↗

Pancreatic trauma in children.

BACKGROUND/PURPOSE: The aim of this study was to determine the etiology, associated injuries, and outcome of children with pancreatic injuries. METHODS: A retrospective review was conducted of children under 16 years with pancreatic trauma admitted to the Children's Hospital at Westmead (CHW) from January 1983 to September 2002. Deaths were reported to the New South Wales Paediatric Trauma Death Registry (State Registry) from January 1988 to September 2002. RESULTS: Sixty-five cases were identified: 46 patients were admitted to CHW, and 22 deaths were reported to the State Registry (including 3 deaths at CHW). The median age was 6 years (range, 1 to 14 years). Boys accounted for 60% (n = 40) of cases, decreasing to 50% (n = 11) of those that died. Motor vehicle injuries (MVI) were the most common mechanism, accounting for 40% of survivors and 77% of those who died. Children were restrained incorrectly in 48% of all cases and in 67% of those who died. Significantly more children who died had head, chest, and abdominal visceral injuries, compared with those who survived. Death occurred as a result of head injuries in 68%, with only 3 deaths directly attributed to pancreatico-duodenal injuries. CONCLUSIONS: Pancreatic injuries remain uncommon in children. The most frequent mechanism was MVI, with incorrect use of passenger restraints an important contributing factor. Whereas mortality was mainly caused by other injuries, 3 deaths were directly attributable to pancreato-duodenal trauma.

Abdominal Injuries↗

Small bowel perforation in the premature neonate: congenital or acquired?

To determine the potential aetiological factors of small bowel perforation in the premature neonate, we performed a retrospective chart review of those neonates with spontaneous intestinal perforation (SIP) of the small bowel seen in our tertiary paediatric hospital between January 1980 and December 2000. Data were collected on gestational feto-maternal health, medical interventions prior to perforation and the subsequent operative and laboratory findings. There were 23 patients with SIP of the small bowel over the 21-year review; 65% were male. There were 7 twin pregnancies but no cases linked to maternal drug abuse. The median gestational age was 27 weeks, the median birth weight 973 g, 19 neonates required ventilation, 15 steroids and 13 indomethacin. The median age at diagnosis was 7 days, heralded by rapid development of abdominal distension in 22 patients. Surgical intervention in addition to insertion of a peritoneal drain was required in 19 patients. Positive microbiological cultures of blood or peritoneal fluid at operation were documented in 8 patients; 5 grew Staphylococcus epidermidis and 4 Candida species. Perforations were located in the ileum in 20 and the jejunum in 1. Deficiency of the muscularis propria was found in 6 patients. Of the 6 deaths, 2 neonates had significant co-morbidity in addition to extreme prematurity. Small bowel SIP occurs in the premature neonate after the first week of life and usually presents with abdominal distension. Putative risk factors identified included twin gestation, neonatal ventilation, use of steroids and indomethacin, infection with Staphylococcus epidermidis and Candida species and deficiency of enteric smooth muscle.

Comorbidity↗

Intra-operative pulse oximetry can help determine intestinal viability.

The intra-operative assessment of intestinal viability when dealing with ischaemic bowel remains a challenge. Reliable healing of small-bowel anastomoses, using pulse oximetry to exclude critical ischaemia, has been shown in the canine model. In this study, intra-operative pulse oximetry (PO) was used to help determine intestinal viability 48 h after de-torsion of a volvulus. Approximately one-half of the ischaemic, volved bowel was able to be preserved. Intra-operative PO can thus help preserve bowel of doubtful viability.

Anastomosis, Surgical↗

A new technique of anastomosis to avoid stricture formation in oesophageal atresia.

Anastomotic stricture is a common problem following repair of oesophageal atresia (OA). We describe a technique of oesophageal anastomosis that may prevent this problem. A horizontal incision is placed on the anterior hemicircumference of the upper pouch approximately 0.5 cm proximal to its blind ending to raise a flap. A corresponding vertical incision is made at the open end of the lower pouch to spatulate it. The flap from the upper pouch is laid into the open V of the lower pouch. This creates a wide anastomosis, and the suture line is not restricted to one plane. In 11 cases of OA, oesophageal continuity was established with this technique over a period of 10 years. Only 1 child developed an anastomotic stricture, which responded to a single dilatation. Two patients required Nissen's fundoplication for a distal oesophageal stricture. In neither of the patients did the anastomosis become stenotic. The technique described here is simple and effective. A suture line is created that is long and not in a single plane. This minimises the risk of stricture formation.

Anastomosis, Surgical↗

Inferior liver allograft survival from cadaveric donors >50 years of age?

The growing imbalance between the number of cadaveric organ donors and recipients has led to an increasing use of high-risk donors as an option to expand the donor pool. The aim of this study was to evaluate our experience with the use of older liver (donor>50 yr of age) allografts. The medical records, postreperfusion biopsies and laboratory results were reviewed of the 393 patients who underwent orthotopic liver transplantation between 1986 and 1997. The outcome of the 61 patients who received older livers (OL) was compared to that of the other 332 recipients. Increasing use of OL was evident from 1992 onwards. Recipients of OL were older than recipients of younger livers (YL, p<0.001) and more commonly had underlying chronic viral hepatitis (CVH) or fulminant hepatic failure (p<0.05). Patient and allograft survival were only slightly less in recipients of OL versus YL (p=NS). Although postperfusion biopsies showed more damage in OL than YL allografts (p<0.05), this was not associated with increased primary graft failure. OL allografts can be transplanted with acceptable results into recipients without the concern of early allograft loss. SUMMARY OF ARTICLE: This report of one centre's experience with 61 recipients of older donor liver allografts identifies recipient factors that may also have a negative impact on allograft outcome. These factors include a diagnosis of either CVH or fulminant hepatic failure at the time of transplantation. Postreperfusion biopsies of older donor allografts tend to show more damage, but this is not associated with primary non-function.

Adolescent↗

Gallstones and common bile duct calculi in infancy and childhood.

BACKGROUND: Gallstones and common bile duct calculi have been increasingly diagnosed in recent years in infants and children. The present study aims to review the spectrum of this disorder in the last two decades. METHODS: During the period 1979-96 a total of 102 consecutive infants and children were diagnosed in Royal Alexandra Hospital for Children with gallstones or common bile duct calculi. A detailed retrospective analysis and follow-up of these children form the basis of the present report. RESULTS: The median age at presentation was 10 years. Recurrent right upper quadrant pain was the most common clinical presentation. The male-to-female ratio was 3:2 and this male predominance was noted in all the age groups. Aetiologically three identifiable groupings were noted: idiopathic disease (n = 66), haematological diseases (n = 23) and specific non-haematological disease (n = 13). The incidence of idiopathic and haematological stones had increased two-fold in the second half of the study. The majority of children (86%) underwent surgical correction. Choledocholithiasis (CDL) was noted in 18 children (18%). Jaundice was commonly associated with abdominal pain in this group. A higher incidence of common bile duct calculi was noted in females and children less than 5 years of age (P < 0.01). Common bile duct calculi were accurately diagnosed by pre-operative imaging in all 18 children. Surgical correction was required in all except two. CONCLUSIONS: The present study suggests an increasing incidence of gallstones in children. Cholelithiasis in children occurs commonly in boys, is idiopathic in aetiology and presents with a vague right upper quadrant pain. Choledocholithiasis is not uncommon in children, occurs more commonly in girls aged < 5 years and presents with jaundice or abnormal liver function tests.

Adolescent↗

Adverse outcome of congenital diaphragmatic hernia is determined by diaphragmatic agenesis, not by antenatal diagnosis.

BACKGROUND/PURPOSE: The authors studied their congenital diaphragmatic hernia (CDH) cases retrospectively to ascertain if classical CDH and diaphragmatic agenesis (DA) have separate clinical manifestations, whether antenatally diagnosed cases behave differently from those not diagnosed antenatally, and if antenatal diagnosis before 25 weeks carries a worse prognosis. METHODS: The authors performed a retrospective review of 23 infants with CDH treated at their institution between January 1996 and March 1999. The patients were divided into 3 groups that were analyzed: DA and classical CDH, antenatally diagnosed and nonantenatally diagnosed, and antenatally diagnosed before 25 weeks and after 25 weeks. RESULTS: There were 8 cases of DA and 11 cases of classical CDH. Four infants died without operation and could not be classified. Neonates with DA had significantly longer mean duration of preoperative stabilization compared with classical CDH (5.25+/-2.76 days v 1.36+/-1.0 days) and postoperative mechanical ventilatory support (15.7+/-3.0 days v 4.9+/-3.0 days). Fifty percent of DA patients died; all classical CDH patients survived. Twelve cases were diagnosed antenatally, 6 before 25 weeks' gestation. Antenatally diagnosed cases had no statistically significant difference in mortality rates from those not diagnosed antenatally; 50% of those diagnosed before 25 weeks survived. CONCLUSIONS: DA cases require more preoperative preparation and postoperative ventilation and have a bad prognosis compared with classical CDH. Antenatal diagnosis of CDH does not convey a different prognosis. Fifty percent of CDH patients with antenatal diagnosis before 25 weeks survive.

Diaphragm↗

Portosystemic shunting for paediatric portal hypertension.

The records of 22 patients who received portosystemic shunting for portal hypertension from 1985 to 1995 inclusive at the Royal Alexandra Hospital for Children (RAHC) were retrospectively reviewed. There were 11 girls and 11 boys. The average age at operation was 8 years, 3 months (range, 2 years, 3 months to 16 years, 7 months). The aetiology was idiopathic portal cavernomatous transformation (n = 9), billiary atresia (n = 4), cystic fibrosis (n = 3), documented neonatal portal vein thrombosis (n = 3), congenital hepatic fibrosis (n = 2), and portal vein obstruction after liver transplant (n = 1). The major presenting problem was upper gastrointestinal haemorrhage. Two patients had recurrent melaena from Roux-en-Y jejunal loop and caecal varices, respectively. Before receiving shunts, 12 patients had endoscopic sclerotherapy, 1 had gastric transection, and 2 had gastric varices oversewn. Portal pressure at preoperative splenoportogram averaged 28 mm Hg (range, 20 to 41). Urgent shunts were performed on 13 patients. Two disadvantaged patients had prophylactic shunts for severe hypersplenism. The types of shunts used were reversed splenorenal (n = 13), splenoadrenal (n = 6), inferior mesenteric renal (n = 1), portocaval (n = 1), inferior mesenteric caval (n = 1), and superior and inferior mesenteric caval (n = 1). In all, 22 patients had 23 shunts. The patency rate was 96% on 6 months to 10 years follow-up (average, 5.8 years). No spleen was lost. There were 2 late deaths. Two cystic fibrosis patients and one child with extrahepatic portal hypertension experienced post-shunt encephalopathy. Three patients rebled in the early postoperative period despite a patent shunt. Two patients subsequently received liver transplantation without any additional difficulties. Thus, portosystemic shunting using a method appropriate for the patient is a reliable option for treating children with portal hypertension in whom variceal sclerotherapy is inappropriate or has failed.

Adolescent↗

ABO blood group incompatibility in liver transplantation: a single-centre experience.

BACKGROUND: For most organ transplantation (Tx), ABO blood group incompatibility (ABOI) is an absolute contraindication because of the high incidence of hyperacute rejection (HAR). While HAR occurs in ABOI liver Tx (LTx), it is known that some liver grafts can be accepted. METHODS: ABO-incompatible (ABOI) liver allografts were used in seven of 355 orthotopic LTx operations performed at our institution over a 10-year period. All seven recipients were in fulminant hepatic failure (FHF) prior to Tx. RESULTS: Following Tx, all grafts functioned immediately. One patient died without recovering consciousness. Six patients recovered consciousness following Tx but three patients subsequently required re-transplantation (with ABO-compatible grafts (ABOC)) because of severe acute rejection (2) and chronic rejection (1). Hyper-acute rejection did not occur. All six patients are now well, with a mean survival of 61.5 months. When compared to 36 other FHF patients who received ABOC grafts, graft survivals were 3/7 (43%) for ABOI versus 23/36 (64%) for ABOC (P = not significant (NS)). Patient survivals were 6/7 (85.7%) for ABOI patients and 23/36 (64%) for ABOC (P = NS). The re-transplantation rate was significantly higher in the ABOI group (P = 0.001). CONCLUSIONS: The results confirm that ABOI liver grafts should be used in urgent circumstances when compatible grafts are not available. Some grafts function indefinitely, while those that fail may function for sufficient time to allow successful retransplantation with ABOC grafts.

ABO Blood-Group System↗

Use of liver allografts from carbon monoxide poisoned cadaveric donors.

Carbon monoxide (CO) inhalation leads to cerebral, cardiac, and, more rarely, liver damage. The use of liver allografts from CO poisoned donors with evidence of liver damage has not previously been reported. In this report we describe two recipients, both in fulminant hepatic failure, who received liver grafts from such donors. One donor had markedly abnormal liver function tests (LFTS), and in the other LFTS were mildly abnormal. In both, the liver appeared normal at procurement. There was satisfactory early function of both allografts, although marked patchy necrosis was seen on the postreperfusion biopsy (case 1), and on a 10 day postoperative biopsy (case 2). In both cases the changes were considered to be related to damage sustained from CO inhalation. Both allografts soon achieved normal function and both recipients are well. We conclude that CO poisoning can cause liver damage that can recover completely following liver transplantation.

Adult↗

Retrieval of organs for transplantation: experience of the Australian National Liver Transplantation Unit.

The Australian National Liver Transplantation Unit carried out 251 multiple organ-retrieval procedures between January 1986 and December 1994: 53% were in the Sydney metropolitan area, 22% in country New South Wales and the Australian Capital Territory, 21% interstate and 4% in New Zealand; mean total procurement times were, respectively, 4 hours 57 minutes, 6 hours 58 minutes, 9 hours 20 minutes and 12 hours 20 minutes. Donor operation times steadily decreased over the nine years. One-year patient and graft survival rates were 77.1% and 70.3%, respectively; primary non-function occurred in only one case. The importance of coordination of donor organ procurement and efficient organ retrieval to the success of transplantation programs is discussed.

Adolescent↗