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Biomedical subjects

A J W Millar

Publications and source records attributed to A J W Millar.

At least 19 recordsLinked to original sources

Current issues in the management of intestinal failure.

Successful long term parenteral nutrition has transformed the prognosis for children with irreversible intestinal failure in the last three decades, but has also highlighted the long term complications: intestinal failure associated liver disease; recurrent catheter sepsis; and impaired venous access. Recent advances in small bowel transplantation and non-transplant surgical techniques now offer hope of sustained survival in the future without parenteral nutrition.

Child↗

Four decades of conjoined twins at Red Cross Children's Hospital--lessons learned.

Conjoined twins represent a rare but fascinating congenital condition, the aetiology of which remains obscure. Over the past four decades, the paediatric surgeons at Red Cross Children's Hospital have been involved in the management of 46 pairs of conjoined twins, of which 33 have been symmetrical and 12 asymmetrical. Seventeen symmetrical twins have undergone separation with 22 children (65%) surviving; all of the live asymmetrical twins survived separation. We describe the important features of this unique cohort, outline our approach to management and present the results of this approach. We consider some of the ethical and moral dilemmas we have confronted, and discuss the prenatal diagnosis, obstetric implications and postnatal care of these children, including the relevant investigations and anaesthetic and surgical management. Specific aspects related to the cardiovascular system, hepatobiliary and gastrointestinal tracts, urogenital tract, central nervous system and musculoskeletal system are highlighted.

Adolescent↗

Overview of a paediatric renal transplant programme.

INTRODUCTION: Renal transplantation is the therapy of choice for children with end-stage renal failure. There are many challenges associated with a paediatric programme in a developing country where organs are limited. METHODS: A retrospective review was undertaken of 149 paediatric renal transplants performed between 1968 and 2006 with specific emphasis on transplants performed in the last 10 years. Survival of patients and grafts was analysed and specific problems related to drugs and infections were reviewed. RESULTS: On review of the total programme, 60% of the transplants have been performed in the last 10 years, with satisfactory overall patient and graft survival for the first 8 years post transplant. At this point, transfer to adult units with non-compliance becomes a significant problem. Rejection is less of a problem than previously but infection is now a bigger issue--specifically tuberculosis (TB), cytomegalovirus (CMV) and Epstein-Barr virus (EBV) infections with related complications. A wide variety of drugs are available for tailoring immunosuppression to minimise side-effects. CONCLUSION: It is possible to have a successful paediatric transplant programme in a developing country. However, to improve long-term outcomes certain issues need to be addressed, including reduction of nephrotoxic drugs and cardiovascular risk factors and providing successful adolescent to adult unit transition.

Adolescent↗

Liver transplantation at Red Cross War Memorial Children's Hospital.

UNLABELLED: The liver transplant programme for infants and children at Red Cross War Memorial Children's Hospital is the only established paediatric service in sub-Saharan Africa. Referrals for liver transplant assessment come from most provinces within South Africa as well as neighbouring countries. PATIENTS AND METHODS: Since 1987, 81 children (range 6 months-14 years) have had 84 liver transplants with biliary atresia being the most frequent diagnosis. The indications for transplantation include biliary atresia (48), metabolic (7), fulminant hepatic failure (10), redo transplants (3) and other (16). Four combined liver/kidney transplants have been performed. Fifty-three were reduced-size transplants with donor/recipient weight ratios ranging from 2:1 to 11:1 and 32 children weighed less than 10 kg. RESULTS: Sixty patients (74%) survived 3 months-14 years post-transplant. Overall cumulative 1- and 5-year patient survival figures are 79% and 70% respectively. However, with the introduction of prophylactic intravenous ganciclovir and the exclusion of hepatitis B virus (HBV) IgG core Ab-positive donors, the 1-year patient survival is 90% and the projected 5-year paediatric survival is > 80%. Early (< 1 month) post-liver-transplant mortality was low. Causes include primary malfunction (1), inferior vena cava thrombosis (1), bleeding oesophageal ulcer (1), sepsis (1) and cerebral oedema (1). Late morbidity and mortality was mainly due to infections: de novo hepatitis B (5 patients, 2 deaths), Epstein-Barr virus (EBV)- related post-transplantation lymphoproliferative disease (12 patients, 7 deaths) and cytomegalovirus (CMV) disease (10 patients, 5 deaths). Tuberculosis (TB) treatment in 3 patients was complicated by chronic rejection (1) and TB-drug-induced subfulminant liver failure (1). CONCLUSION: Despite limited resources, a successful paediatric programme has been established with good patient and graft survival figures and excellent quality of life. Shortage of donors because of infection with HBV and human immunodeficiency virus (HIV) leads to significant waiting-list mortality and infrequent transplantation.

Adolescent↗

Colonic atresia: spectrum of presentation and pitfalls in management. A review of 14 cases.

Fourteen cases of colonic atresia seen over a 38-year period are reviewed with particular reference to clinical presentation and pitfalls in management. Seven had Type I atresia, two Type II and five Type IIIa. Ten had associated gastrointestinal anomalies. Management varied considerably. Six had primary colonic anastomosis. Two of these developed complications due to unrecognized distal hypoganglionosis, two had associated jejunal atresias resulting in short bowel syndrome, and two had primary anastomosis protected by proximal ileostomies. Seven had a staged repair with initial defunctioning enterostomy with only one complication, an unfixed mesentery that later resulted in midgut volvulus. The only mortality was a patient in which a jejunal atresia repair leaked as a result of a missed colonic atresia. Operative strategy should depend on the clinical state of the patients, the level of atresia, associated small bowel pathology and exclusion of distal pathology. Primary anastomosis would only rarely be advised with a circumspect approach. Long-term outlook, as in small bowel atresia is generally excellent.

Colon↗

Liver injuries in children: the role of selective non-operative management.

INTRODUCTION: This review article on the management of blunt liver injury in children is based on the authors' experience of 311 patients over a 22-year period. MATERIAL AND METHODS: All children presenting to our institution with confirmed blunt liver trauma were studied retrospectively. Hospital folders of 311 patients were analysed. Information was gathered about the clinical presentation, associated injuries, grade of injury, transfusion requirements and haemodynamic stability to examine factors influencing outcome. RESULTS: The age of patients ranged between 3 weeks and 12 years (mean of 7 years). Injuries as a result of motor vehicle accidents (MVAs) were the most common (268; 232 pedestrian and 36 passenger), other causes were falls (26) assaults or child abuse(15), bicycle handle bar injury (2). One hundred and thirty-six patients sustained an isolated hepatic injury and 175 had multiple injuries. Associated injuries included 147 head injuries, 131 fractures, 66 thoracic and 143 intra-abdominal (74 spleen, 45 renal, 4 pancreatic and 4 hollow viscus). Two patients died soon after arrival, 21 underwent laparotomy, 13 of which were liver related, while 288 were treated non-operatively. One hundred and six patients required blood transfusion (mean of 21.3 ml/kg); 30% of the nonoperative group and 100% of the operative group. There were three fatalities from the operative group (1% total mortality), one secondary to a severe, head injury, one liver haemorrhage and one from multi-organ failure DISCUSSION: The vast majority (93%) was successfully treated non-operatively with only 4% coming to liver related laparotomy, complications were lower, transfusions less and the in-hospital occupancy was shorter. Complication rate was 8% and mortality was 1%. CONCLUSION: We confirm the success selective non-operative management of blunt liver trauma as adopted by this institution 20 years ago. It is now proven treatment in an appropriate centre. However, the challenge is to identify the severely injured child early and institute aggressive resuscitation and expedite laparotomy when indicated.

Accidental Falls↗

Gastroesophageal reflux strictures in children, management and outcome.

UNLABELLED: Esophageal reflux (GER) strictures are frequently diagnosed late and require a prolonged management programme depending on the severity of the stricture. Management protocols include medical therapy, bouginage, fundoplication, stricture resection and even interposition grafting. Our preferred method is to delay the anti-reflux surgery until the esophagitis is medically controlled, adequate enteral intake with weight gain is achieved and the oesophageal narrowing adequately dilated. We review the results of the approach over a 27-year period (1977-2004). METHOD: Thirty-one children were treated (mean age at diagnosis 35 months). Diagnosis of GERD was made on barium meal and confirmed by pH studies, gastroesophageal scintigraphy and oesophagoscopy. Stenosed site, its length and nature (i.e. response to dilatation) were documented. Dilatations were carried by prograde, balloon and string-guided techniques. Three fundoplication techniques were used (Boix-Ochoa, Toupet and Nissen). RESULTS: Twenty-two strictures were in the lower third, seven in the mid-third and two in the upper third of the oesophagus. Thirteen (42%) had associated hiatus hernia (HH). Twenty (64%) had a stricture length>3 cm. Twelve strictures were so severe (tight) as to require gastrostomy and string-guided dilatation. An average 5.5 dilatations were required prior to surgery. Only six children did not require post-surgery dilatation. Twelve required more than five post-operative dilatations. Reasons for stricture persistence were identified as failed reflux surgery in seven, candida oesophagitis in two, HIV infection in one and severity of fibrosis in three (two requiring stricture resection). At average follow-up of 5 years, all patients have restored growth without further symptoms. CONCLUSION: Strictures are a major complication of GER requiring prolonged and intensive management in most cases. Reasons for persistence of stricture after anti-reflux surgery can be identified and require early intervention. Long-term follow-up is essential but results have been good in our hands.

Child↗

Nephrotoxic effects of immunosuppressant therapy in pediatric liver transplant recipients.

Drugs used for immunosuppression have been implicated in causing numerous long-term side effects including nephrotoxicity, glucose intolerance, and hyperlipidemia. In this study, we reviewed our pediatric liver transplant recipients in terms of glomerular filtration rate (GFR) as well as fasting glucose and lipid profiles. To date, 79 pediatric liver transplantations have been performed at our center: 24 transplantations of at least 5 months to a maximum of 7.3 years posttransplant are reviewed herein. The mean time posttransplantation was 2.1 years. Nine boys and 15 girls showed a distribution of 19 mixed race, 3 black, and 2 white patients. The mean age at the time of transplantation was 6.6 years (0.8-13.3 years) with 8 cases under the age of 3 years. All recipients started with Cyclosporine Neoral (CSA) as first line, but, at the time of testing, immunosuppression included 5 children on CSA and 19 on Tacrolimus. Radionuclide 51 Cr-EDTA Glomerular Filtration Rates (GFR) showed a range from 21 to 220 mL/min/1.73 m2 (mean 96.1, median 89.8). Seven cases had a GFR less than 75 mL/min/1.73 m2. Twenty-one children were on antihypertensives agents: 15 children on 1 agent and 6 children on 2 agents. On full fasting lipid profiles, the total cholesterol ranged from 2 to 7.9 mmol/L (mean 4.4). Only 1 child is currently on statin therapy. Fasting glucose ranged from 3.2 to 5.9 mmol/L (mean 4.1) No difference was observed in glucose values between CsA and Tacrolimus. Thus, immunosuppressive therapies, such as the calcineurin inhibitors, are known to cause nephrotoxicity, which is of concern in pediatric liver transplant recipients. Almost all our patients currently require antihypertensive therapy. At present, the renal function is adequate in the majority of the group, but this study needs to be extended to other pediatric liver transplant recipients with particular emphasis on those who are more than 5 years posttransplantation.

Adolescent↗

Liver transplantation for children: Red Cross Children's Hospital experience.

BACKGROUND: The liver transplant program for infants and children at the Red Cross Children's Memorial Hospital is the only established pediatric service in sub-Saharan Africa. Since 1985, 250 infants and children have been assessed and 155 accepted for transplantation. METHODS: Since 1987, 76 children (range 6 months to 14 years) have had 79 liver transplants, with biliary atresia being the most frequent diagnosis. The indications for transplantation include biliary atresia (n = 44), metabolic (n = 7), fulminant hepatic failure (n = 10), redo transplants (n = 3), and other (n = 15). Three combined liver/kidney transplants have been performed. Forty-nine were reduced-size transplants with donor: recipient weight ratios ranging from 2:1 to 11:1, and 29 children weighed < 10 kg. RESULTS: Fifty-six (74%) patients survived 3 months to 12 years posttransplant. Cumulative 1- and 5-year patient survival data are 79% and 70%, respectively. However, with the introduction of prophylactic intravenous gancyclovir and the exclusion of hepatitis B virus (HBV) IgG core Ab-positive donors, the projected 5-year pediatric survival has been >80%. Early (<1 month) post-liver-transplant mortality was low, but included: primary malfunction (n = 1); inferior vena cava thrombosis (n = 1); bleeding esophageal ulcer (n = 1); and sepsis (n = 1). Late morbidity and mortality was mainly due to infections: de novo hepatitis B (5 patients, 2 deaths); Epstein-Barr virus (EBV)-related posttransplantation lymphoproliferative disease (12 patients, 7 deaths); and cytomegalovirus disease (10 patients, 5 deaths). Tuberculosis (TB) treatment in three patients was complicated by chronic rejection (n = 1) and TB drug-induced subfulminant liver failure (n = 1). CONCLUSIONS: Despite limited resources, a successful pediatric program has been established with good patient and graft survival figures and excellent quality of life. Shortage of donors due to HBV and human immunodeficiency virus (HIV) leads to significant waiting list mortality and infrequent transplantation.

Adolescent↗

Hepatoblastoma--evolution of management and outcome and significance of histology of the resected tumor. A 31-year experience with 40 cases.

BACKGROUND/PURPOSE: The aim of this study was to retrospectively evaluate and compare the clinical features, treatment strategy, pathology, and outcome of all patients with hepatoblastoma treated at an African hospital over a 31-year period (1970 to 2001). METHODS: Forty patients with hepatoblastoma were divided into 3 groups according to the treatment given. Group I (1970 to 1983, 14 patients) had no protocol therapy; group II (1984 to 1988, 6 patients) received protocol treatment according to Children's Study Group (CCSG) guidelines; group III (1989 to 2001, 20 patients) received SIOPEL protocol therapy. All available clinical, surgical, radiologic, and pathologic data were reviewed and analyzed. RESULTS: Overall patient survival was as follows: group I, 14%; group II, 50%, and group III, 80%. Deaths in group II were caused by chemotherapy-induced immunosuppression only. Prognostic data for group III showed that all tumor-related deaths could be predicted by identifying multifocal disseminated growth patterns (P =.001) or vascular invasion (P =.001) in resected tumors. Of the 40 diagnostic tumor biopsies performed, 2 significant complications (1 death, 1 intraperitoneal tumor seeding) occurred. Histologic criteria evaluating these biopsies were not predictive of overall survival. CONCLUSIONS: The introduction of protocol therapy has resulted in a marked improvement in survival. Immunosuppression-related sepsis in our setting resulted in unacceptable mortality in patients treated according to CCSG guidelines. A diagnostic biopsy in hepatoblastoma is of value but not without complications. Preoperative chemotherapy followed by complete surgical excision according to International Society of Paediatric Oncology guidelines yields excellent results with a current survival rate of 80%.

Antineoplastic Combined Chemotherapy Protocols↗

Liver transplantation for children--the Red Cross Children's Hospital experience.

UNLABELLED: Liver transplantation for infants and children has been available in South Africa at a single centre, the only established service in Sub-Saharan Africa, for more than a decade. Current concerns have shifted from an initial target of early post-transplant survival to quality of life in the long-term. MATERIALS AND METHODS: Since 1985, 225 infants and children have been assessed, with 146 accepted for transplantation. Sixty-nine have had 71 orthotopic liver transplants (OLTx). Biliary atresia was the most frequent diagnosis (54%) followed by acute liver failure (ALF) (15%). Waiting list mortality has remained high (23%), particularly for the ALF group (50%). Forty-three were reduced size transplants with donor: recipient weight ratios ranging from 2:1 to 11:1. Twenty-seven were <10 kg. RESULTS: Fifty (74%) survive 1 month-12 years post-transplant. Actuarial survival after 1996 since HBV core antibody positive donor livers were refused and prophylactic IV ganciclovir used has been >82%. Early post-OLTx mortality was low (5%), one primary non-function, one IVC thrombosis, one PV thrombosis, but late morbidity and mortality (20%) was mainly due to viral infection: de novo hepatitis B (five patients, three deaths), EBV-related post-transplantation lymphoproliferative disease (PTLPD) (eight patients, six deaths) and CMV disease (11 patients, five deaths). Tuberculosis prophylaxis, required in six cases, resulted in major morbidity in two and mortality in one. Poor compliance played a significant role in seven deaths. Hypertension requiring medication along with some compromise of renal function has been present in all but two patients. However, all those of school-going age (25) attend school normally and remain in good health and only three of the survivors have abnormal liver function tests. CONCLUSIONS: Successful liver transplantation is possible in a developing country with limited resources. Scarcity of virus-free donors (HBV and HIV) leading to waiting list mortality and infrequent re-transplantation along with long-term consequences of immunosuppression (infection, lymphoma and renal toxicity) remain problems. Intense education of the caregiver and close follow-up, particularly of those living at long distances has partly addressed the compliance problem.

Actuarial Analysis↗

The effects of war on children in Africa.

There is no doubt that the effects of war extend to the most vulnerable members of society, including children. Although armed conflicts occur throughout the world, the African continent seems to be a particular background for civil and international wars. The aim of this study was to identify causes of conflict in Africa and to evaluate the effect of war on children and their health in order to make practical recommendations to health care workers dealing with children in the setting of war. All articles written in the past 5 years concerning "war" and "children" were identified by means of a literature search and internet review. Contrary to common belief, the causes of conflict are complicated and multi-factorial. The effects of war on childhood are disastrous and include severe negative effects on general paediatric health status. Short-term recommendations for health care workers working with children in war include supply of emergency medical infrastructures, basic health care, rehabilitation and education. Long-term recommendations include orchestrating the relief and support efforts from both national governments and international non-profit organisations and speeding up of economic recovery. The causes of conflict in Africa are complex and unlikely to be resolved soon. The effects of war on children are horrendous in many ways, but can be limited by providing timely and appropriate health care.

Africa↗

Unusual intestinal sequelae after operations for Ascaris lumbricoides infestation.

Two children with severe bowel sequelae distal to the site of an Ascaris lumbricoides bowel obstruction are reported. It is postulated that these resulted from inflammatory reaction to toxic decomposition products of disintegrating worms. Surgery should be modified in the presence of bowel-wall injury distal to the site of ascarid worm - bolus obstruction.

Animals↗

Malrotation and volvulus in infancy and childhood.

The subject of malrotation and midgut volvulus in infancy and childhood is reviewed from the perspective of experience with 138 patients evaluated in a published series and a further 82 cases seen since. Embryology, historical aspects, clinical presentation, investigation, surgery, and outcome are discussed. The diagnosis of malrotation and volvulus should always be kept in mind when assessing any infant or child with symptoms of vomiting and pain, particularly when the vomiting is bile-stained. The diagnosis cannot be excluded if diarrhea is present, when clinical findings are bland, or even with normal abdominal radiographs. A contrast meal and follow-through should be done and, if correctly interpreted, should be diagnostic. Ultrasound examination may be helpful but is not secure enough to exclude the diagnosis. Laparotomy or laparoscopy is the only way to be sure. Malrotation with its propensity for volvulus is truly a time bomb lying within.

Abdominal Pain↗

Child rape.

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Child↗

Y-V advancement antropyloroplasty for corrosive antral strictures.

Accidental corrosive ingestion by children can result in damage to the oesophagus and stomach. With progressive scarring and fibrosis luminal obstruction occurs. Gastric fibrosis typically results from acid ingestion and occurs in the antrum because of pooling of the corrosive agents at that site. The antral stricture presents with symptoms of progressive outlet obstruction that may take up to 3 years to become clinically apparent. Surgery is necessary to bypass this obstruction. We describe three patients in whom we have successfully utilised a Y-V advancement antro-pyloroplasty as corrective surgery for corrosive antral strictures. The procedures required little dissection and two could be done early in the evolution of the stricture. Antro-duodenal patency was maintained on follow-up.

Burns, Chemical↗