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Biomedical subjects

N D Barnes

Publications and source records attributed to N D Barnes.

At least 37 records · Page 2Linked to original sources
PubMed1994

Intrapulmonary shunting in the biliary atresia/polysplenia syndrome: reversal after liver transplantation.

UNLABELLED: One hundred and seventy three children, including 93 with biliary atresia, received liver grafts at Addenbrooke's Hospital between 1983 and 1993. Of these, only seven developed cyanosis due to intrapulmonary shunting as a complication of their liver disease, and all seven of these had the biliary atresia/polysplenia syndrome. Intrapulmonary shunting was confirmed by a radioisotope scan in four children. Only one child with the syndrome did not have cyanosis when undergoing transplantation. Seven of the eight children are alive 6-54 months after transplantation, with normal pulmonary and hepatic function. Cyanosis recurred in one child who developed chronic rejection with liver failure. IN CONCLUSION: (a) there is a strong association between the biliary atresia/polysplenia syndrome and cyanosis due to intrapulmonary shunting; (b) intrapulmonary shunting is fully reversible after successful liver transplantation; and (c) cyanosis, once present, is progressive, and these children should be considered for liver transplantation as soon as it occurs.

Arteriovenous Fistula↗
PubMed1994

Neurological crisis in hereditary tyrosinaemia and complete reversal after liver transplantation.

A 19 month old Indian girl with tyrosinaemia developed a severe generalised neuropathy involving both phrenic nerves. Treatment with haemarginate failed to improve her condition. After liver transplantation the raised concentrations of the neurotoxin delta amino-laevulinic acid returned to normal and gradual but complete neurological recovery occurred over a period of 13 months.

Amino Acid Metabolism, Inborn Errors↗
PubMed1994

Liver transplantation for hepatic cirrhosis in cystic fibrosis.

Five children with cystic fibrosis complicated by hepatic cirrhosis received liver grafts. They all had portal hypertension with varices and three had variceal bleeding; respiratory function was only moderately impaired, but four were colonised with pseudomonas and one with aspergillus. Liver transplantation was well tolerated and there was no increase in respiratory or other early postoperative complications. Four of the children were fully well from 14 to 35 months after transplantation; the most recently transplanted had problems from a biliary stricture. In spite of the need for immunosuppression there was no increase in infection and respiratory function improved or remained stable. Once the children were stabilised after transplantation their nutrition and general health were greatly improved.

Adolescent↗
PubMed1993

Neurological and adrenal dysfunction in the adrenal insufficiency/alacrima/achalasia (3A) syndrome.

Review of 20 patients with glucocorticoid deficiency (three cases also with salt loss) associated with absent tear secretion (19 cases) and achalasia of the cardia (15 cases) revealed neurological abnormalities in 17 including hyper-reflexia, muscle weakness, dysarthria, and ataxia together with impaired intelligence and abnormal autonomic function, particularly postural hypotension. These findings indicate that significant neurological problems are common in this multisystem disorder.

Addison Disease↗
PubMed1992

Glomerulonephritis with end-stage liver disease in childhood.

Renal biopsies were done perioperatively in 18 children receiving liver grafts. All specimens showed glomerulonephritis, which was mesangial-proliferative in 15 and mesangio-capillary in 3. Of the 16 children who were alive four or more months after transplantation, only 1 showed progressive deterioration of renal function; 1 other had a subnormal but static glomerular filtration rate. In all 6 children who had proteinuria before operation, the urine became normal.

Adolescent↗
PubMed1992

Liver transplantation in 100 children: Cambridge and King's College Hospital series.

OBJECTIVE: To review the results of the Addenbrooke's and King's College Hospital children's liver transplantation programme. DESIGN: Retrospective analysis of the first 100 children to receive liver grafts at Addenbrooke's Hospital, Cambridge, from December 1983 to March 1990. SETTING: Addenbrooke's Hospital, Cambridge, and King's College Hospital, London. SUBJECTS: 153 children assessed for liver transplantation, of whom 22 died before a donor became available and 31 were considered unsuitable. 100 children received grafts, of whom 27 had second grafts. RESULTS: One year actuarial patient survival was 71%, with 57% one year graft survival. In the last two years survival rates had improved considerably, with 86% of patients and 63% of grafts surviving for at least one year. Sixty five children were alive 12 to 86 months after transplantation; 63 were well and leading normal active lives and 56 had entirely normal liver function. Children's growth and development were essentially normal, with many showing remarkable catch up growth. CONCLUSIONS: Liver transplantation offers children with terminal liver disease a high chance of a return to full quality life and normal development. Improved surgical and medical care have progressively improved survival. The timing of transplantation is critical but has been constrained particularly by the availability of donors and resources.

Adolescent↗
PubMed1992

Liver transplantation in tyrosinaemia type 1: the dilemma of timing the operation.

Four children with tyrosinaemia type 1 received liver transplants. The metabolic disorder was corrected and all four had normal liver function on an unrestricted diet. Two children, transplanted at age five and seven years, proved to have occult hepatocellular carcinoma and both subsequently developed pulmonary metastases. One child was well 32 months after removal of a single pulmonary metastasis but the other child died with multiple metastases. The two younger children, transplanted at age 19 and 21 months, were well 28 and 44 months after operation, one after a second liver transplant. Our experience confirms the high risk of hepatocellular carcinoma in this disease and the potential value of early liver transplantation.

Age Factors↗
PubMed1991

Ultrafiltration for intractable ascites after liver transplantation.

A 5.7 year old boy with alpha 1-antitrypsin deficiency developed intractable ascites after liver transplantation. Conservative treatment was unsuccessful and after 18 days concentrated ascitic fluid was reinfused intravenously using a Gambro haemofilter. The ascitic loss resolved rapidly. This new method of ultrafiltration proved simple and effective.

Animals↗
PubMed1990

Pharmacokinetics of intravenous amoxycillin and potassium clavulanate in seriously ill children.

The pharmacokinetics of amoxycillin and potassium clavulanate were studied in 15 sick children after a 30 min iv infusion of 50 mg/kg amoxycillin and 5 mg/kg clavulanic acid as the potassium salt. Levels of both compounds in plasma were assayed microbiologically. Mean peak concentrations at the end of the infusion were 121.0 mg/l of amoxycillin and 12.0 mg/l of clavulanate, falling to a mean of 15.8 and 1.92 mg/l respectively after 2 h. Mean beta phase T 1/2 was 0.88 h for amoxycillin and 0.79 h for clavulanate. The elimination half-life of clavulanate in some individuals was much shorter because of higher plasma clearance. The data suggest that the treatment of some infections due to beta-lactamase producing organisms in such severely ill children may require more frequent iv administration of amoxycillin and potassium clavulanate, than in less severely affected children.

Adolescent↗
PubMed1990

Severe hypertension after liver transplantation in alpha 1 antitrypsin deficiency.

Five children with alpha 1 antitrypsin deficiency and terminal liver disease received liver grafts; all five became hypertensive and four developed hypertensive encephalopathy. There was evidence of renal disease preoperatively and renal biopsy specimens showed variable glomerulonephritic histology with IgA nephropathy in one, mesangial-proliferative changes in two, and mesangio-capillary glomerulonephritis type I in two. Four hypertensive episodes were preceded by a fall in creatinine clearance. The association of glomerulonephritis with alpha 1 antitrypsin deficiency in children is more common than has been recognised. Affected patients are prone to severe hypertension of probable renal origin after liver transplantation and the renal lesion may affect long term prognosis.

Adolescent↗
PubMed1990

Viral and toxoplasma gondii infections in children after liver transplantation.

The incidence and morbidity of viral and Toxoplasma gondii infections were studied in 40 children who underwent liver transplantation between December 1983 and February 1988. The incidence of primary and reactivated cytomegalovirus (CMV) infection was 19% and 47%, respectively; primary infection caused clinical disease in all five cases affected and was fatal in one. Primary Epstein-Barr virus (EBV) infection occurred in 10 (26%) recipients but caused only mild disease. No reactivated EBV infection was recorded and no lymphoproliferative disorders associated with EBV were found after a maximum of four years' follow up. Adenovirus infection occurred in seven (18%) patients; this was associated in one case with fatal pneumonia and fulminant hepatitis, but otherwise with only mild respiratory disease. Primary T gondii infection was detected in one patient who remained asymptomatic. Other viruses causing infection included herpes simplex, varicella zoster, and respiratory syncytial virus. Surveillance for these infections and the long term sequelae should be included in the follow up of all children who undergo transplantation.

Adenovirus Infections, Human↗