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Biomedical subjects

N D Barnes

Publications and source records attributed to N D Barnes.

At least 19 recordsLinked to original sources
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↗
PubMed1987

United Kingdom multicentre clinical trial of somatrem.

In a multicentre clinical trial 54 children aged 4.0 to 17.3 years, who had growth hormone deficiency that had not previously been treated, were given biosynthetic methionyl growth hormone (somatrem) 4 units three times a week by subcutaneous or intramuscular injection for one year. Height was measured every three months for at least one year before and during treatment. Forty two patients responded to treatment with an increase in growth of greater than 1.5 cm/year. The remaining 12 who grew more slowly were less obviously short and had a higher pretreatment growth than those who responded. The three who responded and the one who did not had undergone therapeutic spinal irradiation before starting the drug. If a whole year's pretreatment growth rate of less than 5 cm/year had been used as a diagnostic criterion the prediction of those who responded would have slightly improved. About two thirds of the patients developed antibodies against growth hormone and Escherichia coli protein; these were, however, of low and fluctuating titre and binding capacity, and did not influence the response to treatment. No adverse side effects were encountered. We conclude that somatrem is a safe and effective alternative to pituitary growth hormone.

Adolescent↗
PubMed1985

Clinical presentation, growth, and pubertal development in Addison's disease.

The clinical course of eight boys and six girls with Addison's disease has been reviewed. Adrenal antibodies were found in five boys and five girls, and four children showed clinical evidence of other autoimmune disease (hypoparathyroidism (three); diabetes (one)). The presentation was insidious in 12 children but acute in two. On treatment, linear growth was normal and, with the exception of one girl with theca cell antibodies, pubertal development proceeded normally in the older patients.

Addison Disease↗
PubMed1982

Vitamin D from skin: contribution to vitamin D status compared with oral vitamin D in normal and anticonvulsant-treated subjects.

1. The plasma 25-hydroxycholecalciferol [25-(OH)D3] response to measured u.v. irradiation applied thrice weekly for 10 weeks was investigated in normal and in anticonvulsant-treated subjects. 2. Levels of plasma 25-(OH)D3 achieved after u.v. irradiation were similar in both normal and anticonvulsant-treated subjects, suggesting that hepatic microsomal enzyme induction does not lead to low plasma 25-(OH)D3 concentrations. 3. Cholecalciferol was present in plasma of normal subjects in a very low concentrations (less than 5.0 nmol/l) and did not increase until plasma 25-(OH)D3 levels exceeded 62.5 nmol/l. 4. Cholecalciferol occurred in significant concentrations in plasma during whole body u.v. irradiation or during oral dosage of 62.5 nmol (100 i.u) or more daily. 5. Plasma 25-(OH)D3 concentrations reached a steady state after 5-6 weeks of u.v. irradiation or of oral intake within the usual intake range. 6. Cholecalciferol synthesis in skin calculated from the steady-state equation was 0.0015 +/- 0.0008 nmol/mJ. 7. Cholecalciferol synthesis in skin was also calculated from the oral dosage required to yield the same plasma 25-(OH)D3 concentration as u.v. irradiation and was 0.0024 +/- 0.0018 nmol/mJ. 8. Rates of cholecalciferol synthesis calculated from these data suggest that many of the population of England receive insufficient u.v. irradiation to maintain vitamin D status throughout the year.

Adolescent↗
PubMed1981

Effect of somatostatin infusion on intermediary metabolism and entero-insular hormone release in infants with hyperinsulinaemic hypoglycaemia.

The hypoglycaemia of infantile hyperinsulinism is often exceedingly difficult to control. The use of somatostatin has been advocated recently in such infants because of its effect on inhibiting insulin release, but nothing is known of the wider effects of this potent hormone in the young child. Two infants presenting at 9 weeks and 5 days of age with severe hyperinsulinaemic hypoglycaemia were studied during an infusion of somatostatin. In both infants normoglycaemia was restored with suppression of insulin secretion. An increase in blood ketone bodies occurred, but no change was seen in blood pyruvate, lactate or alanine concentrations. The plasma concentrations of glucagon, cortisol, growth hormone, motilin, pancreatic polypeptide, gastric inhibitory of polypeptide, neurotensin, gastrin and vasoactive intestinal peptide decreased markedly during the somatostatin infusion. No consistent change occurred in plasma enteroglucagon or secretin values. We conclude that somatostatin effectively suppresses abnormal insulin secretion in infants, but it has profound effects on the release of nine other hormones. Further studies are needed to define the consequences of suppressing the release of these hormones before somatostatin can be used routinely in the management of infantile hyperinsulinism.

Adenoma↗
PubMed1976

Cerebral malformation associated with metabolic disorder. A report of 2 cases.

A clinical and neuropathological study is presented of two cases each of which showed neuronal heterotopia. Microgyria was also present in one case. One patient was suffering from a degenerative disorder affecting the white matter. The other was a case of Menkes' disease. It is suggested that the antenatal damage may have been caused by an imbalance of the maternal metabolism, the predisposing factor being the mother's carrier state for a metabolic defect. This is the first report of teratogenesis in a case of Menkes' disease. It is also noted that in this case there is interference with the postnatal as well as the antenatal development of the brain.

Brain↗