HIV, HAART and dentistry: what you need to know.
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Anti-HIV Agents↗
Biomedical subjects
M O'Halloran
Publications and source records attributed to M O'Halloran.
Assessment of pancreatic function in screened infants with cystic fibrosis.
Previously we have reported that 37% of infants with cystic fibrosis diagnosed by neonatal screening with the dried blood spot immunoreactive trypsin assay have pancreatic sufficiency. However, 34 of the 78 infants had pancreatic function tests an average 2.3 years after diagnosis, thus it was possible that the percentage with neonatal pancreatic sufficiency was underestimated, due to the loss of pancreatic function with time in some infants. To assess this hypothesis we have assessed pancreatic function at the time of diagnosis in a further 20 infants since the completion of the previous study. Results of fecal fat determinations and/or pancreatic stimulation tests indicate that 10 (50%) of these infants have pancreatic sufficiency. Combining these results with those of the previous study, 31 of 64 patients (48%) have pancreatic sufficiency at this early age. We have also monitored the progression of pancreatic disease in the 39 patients with pancreatic sufficiency recognized to date. Eleven have developed pancreatic insufficiency and require enzyme replacement therapy. Five others have shown further improvement of colipase secretion with age. We conclude that the dried blood immunoreactive trypsin screening program for cystic fibrosis does recognize patients with pancreatic sufficiency, and at diagnosis nearly half the patients are in this category. To date, 28% of patients with pancreatic sufficiency have demonstrated a variable decline in pancreatic function with age.
Cystic Fibrosis↗
Successful treatment of dihydropteridine reductase deficiency, with an interesting effect of 5-hydroxytryptophan deficiency on sleep patterns.
We report a child in whom dihydropteridine reductase deficiency was diagnosed prenatally because of an affected sibling and who was treated from birth with apparent good response. This family has been reported before (Firgaira et al., 1980, 1981, 1983; Lipson et al., 1984; Cotton et al., 1986; Dahl et al., 1988). The parents are Lebanese Muslims who are first cousins, and their first and third children, both male, are well. The third child was diagnosed as normal in utero (Firgaira et al., 1983). The second child, the subject of an early report (Lipson et al., 1984), has dihydropteridine reductase deficiency. The dihydropteridine reductase is nonfunctional due to an amino acid insertion (Howells et al., 1990).
5-Hydroxytryptophan↗
Pancreatic function in infants identified as having cystic fibrosis in a neonatal screening program.
The use of the dried-blood immunoreactive-trypsin assay for the detection of cystic fibrosis in newborns has been questioned on the grounds that it may fail to identify patients with enough pancreatic function to have normal fat absorption. To investigate this possibility, we assessed pancreatic function in 78 patients identified in a neonatal screening program as having cystic fibrosis. The diagnosis of cystic fibrosis was confirmed by abnormal results on a sweat chloride test. The results of measurements of fecal fat excretion, pancreatic-stimulation tests, and estimations of the serum level of pancreatic isoamylase indicated that 29 of the 78 children (37 percent) had substantial preservation of pancreatic function. These children (median age, four years) had growth that was close to normal and comparable to growth in children with severe pancreatic insufficiency who received oral enzyme therapy. Pancreatic insufficiency subsequently developed in 6 of the 29 patients, at 3 to 36 months of age. We conclude that the serum immunoreactive-trypsin assay used in neonatal screening programs identifies patients with cystic fibrosis who have sufficient pancreatic function to have normal fat absorption and that a substantial proportion of infants identified as having cystic fibrosis are in this category.
Child, Preschool↗
Nitroprusside in children after cardiopulmonary bypass: a study of thiocyanate toxicity.
Thiocyanate levels, an indicator of nitroprusside toxicity, were studied in 22 children after repair of structural heart disease during cardiopulmonary bypass. At the total dose (2.6 +/- 2.3 mg/kg) and time (34.4 +/- 19 h) ranges of this study, no evidence of toxicity was detected, despite this total dose exceeding recommended maximum in some patients. Nitroprusside infusion, as described, in children with normal hepatic and renal function is safe and may not warrant routine assessment of thiocyanate levels.
Aspartate Aminotransferases↗
Distribution of plasma vitamin E levels in supplemented very low birthweight infants.
The distribution of plasma Vitamin E (VE) was determined in 25 very low birthweight (VLBW) infants who were supplemented with 100 mg/kg per day of alpha-tocopherol acetate, given intragastrically. Their mean birthweight was 917 g and mean gestational age was 28 weeks. Mean plasma VE levels after 1 and 6 weeks' supplementation were 2.7 mg/dL (s.e.m. = 1.0) and 6.4 mg/dL (s.e.m. = 1.4), respectively (the difference was not significant). There was wide variability in plasma VE levels in these infants despite being on an identical dose of tocopherol. Plasma VE was less than 0.5 mg/dL in 12% of samples, 0.5-3.0 mg/dL in 32%, 3.1-5.0 mg/dL in 18%, and 5.1-20 mg/dL in 38%. Fifteen of the 25 infants had at least one level in the range which has been associated with an increased incidence of septicaemia and necrotizing enterocolitis (greater than 5.0 mg/dL). These data suggest that if a policy of VE supplementation for VLBW infants is chosen, monitoring of plasma VE levels appears necessary so that the dosage can be adjusted in order to maintain plasma VE within the optimal range. This study's dosage regimen of supplementing infants with 100 mg/kg per day of VE was associated with a high incidence of elevated plasma VE levels and it is concluded that it is not advisable to use such large doses of VE in the premature newborn.
Female↗
The selenium status of children with phenylketonuria: results of selenium supplementation.
The selenium status of children with phenylketonuria on a synthetic low phenylalanine diet was assessed. Correlation between blood selenium and red cell glutathione peroxidase was unsatisfactory (r = 0.65) due to the poor discrimination of red cell glutathione peroxidase with a low selenium diet. No symptoms of deficiency were observed. Supplementation with 50 micrograms per week of selenium as brewers yeast tablets over a period of 6 months significantly increased the blood selenium of the phenylketonuric children. Plasma Vitamin E levels were within normal limits. The supplementation effectively doubled their selenium intake to 15-17 micrograms per day, which is probably sufficient for this group with an adequate Vitamin E status, though considerably lower than the recommended minimum intake of 50 micrograms per day.
Adolescent↗
Diagnosis of cystic fibrosis.
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Cystic Fibrosis↗
Serum bile acid concentrations during pregnancy and their relationship to obstetric cholestasis.
We have prospectively studied changes in serum postprandial cholylglycine (CG) concentration during 297 pregnancies. We found an increase in CG concentration from 0.3 mumol/L at 15 weeks' pregnancy to 0.6 mumol/L at 40 weeks' pregnancy. Although this increase was statistically significant (p less than 0.005), median concentrations of CG remained well within the normal range (0-1.5 mumol/L). However, 10% of the group showed markedly elevated serum CG concentrations at 30 weeks' pregnancy, and the CG level in this group continued to rise during the third trimester. Pruritus was significantly more common in the group with elevated CG concentrations (48%) than in the group with normal CG levels (20%) (p less than 0.005). Serum CG was a much more sensitive predictor of pruritus during pregnancy than other biochemical liver tests. Elevated CG levels were found more commonly in Mediterranean and Asian patients than patients of other ethnic origins (p less than 0.025). No statistically significant associations were found between elevated CG concentrations and maternal age, number of previous pregnancies, pruritus during previous pregnancies, contraceptive-induced cholestasis, and fetal maturity. We conclude that obstetric cholestasis is probably much more common than previously suspected and that consideration should be given to the measurement of serum bile acids in all pregnant individuals with unexplained pruritus.
Adolescent↗
Prospective study of liver function in children following multiple halothane anaesthetics at short intervals.
In the 4 years January 1981 to December 1984, 186 children received 1362 halothane anaesthetics. Twenty-five patients were anaesthetized with halothane at least 10 times in a year. No patient developed postoperative jaundice. Sixty-nine children were exposed to two halothane anaesthetics within 28 days on 149 occasions and serial estimations of serum liver enzyme concentrations were obtained. Minor increases in the concentrations of serum AST and ALT were recorded in 10.6% and 4.7% of patient entries, respectively. Postoperative GGT and SAP concentrations were increased in 2.7% of patient entries. Patients in whom enzyme values were increased before the first anaesthetic were no more likely than other subjects to develop further changes.
Adolescent↗
Comparative sensitivity of serum cholylglycine concentration and bromsulphalein retention in patients with early and late alcoholic liver disease.
Measurement of serum bile acids has been claimed to be a sensitive and specific biochemical test of hepatic function. We have prospectively measured post-prandial serum glycocholate (cholylglycine) concentrations in 31 patients with alcoholic liver disease and compared these measurements with those of bromsulphalein (BSP) retention, prothrombin time, and serum albumin. In the patients with early (non-cirrhotic) alcoholic liver disease (N = 14) BSP retention was abnormal significantly more frequently than was serum cholylglycine concentration (100% vs 29%, p less than 0.001). In contrast, amongst patients with late (cirrhotic) alcoholic liver disease, BSP retention and serum cholylglycine were abnormal with equal frequency (94%). In both groups of patients BSP retention and serum cholylglycine were abnormal significantly more often than were prothrombin time and serum albumin concentrations. We conclude that moderately severe hepatocellular dysfunction is required before serum cholylglycine can become a reliable biochemical indicator of liver disease.
Adult↗
Tyrosinaemia II.
Four cases of tyrosinaemia type II (Richner-Hanhart syndrome) are reported. This syndrome consists of corneal erosions, palmar and plantar hyperkeratoses, and sometimes mental retardation. Presentation with photophobia and dendritic corneal ulceration or circumscribed palmoplantar keratoderma should alert the physician to the possible diagnosis of tyrosinaemia II. Early diagnosis is important, as the clinical picture can be modified by dietary restriction.
Adult↗
Dihydropteridine reductase deficiency: non-response to oral tetrahydrobiopterin load test.
In a child with dihydropteridine reductase deficiency (McKusick 26163) none of four separate oral tetrahydrobiopterin loading tests resulted in a decrease of the serum phenylalanine concentration. This test cannot be recommended for screening hyperphenylalaninaemic patients for dihydropteridine reductase deficiency.
Biopterins↗
Maternal hyperphenylalaninemia fetal effects.
Thirty-four children of 11 mothers with untreated hyperphenylalaninemia had a pattern of malformation consisting of prenatal and postnatal growth retardation, microcephaly and central nervous system dysfunction, increased incidence of malformations, and a peculiar facial appearance. Maternal hyperphenylalaninemia appears to be teratogenic, with a variability related to the blood phenylalanine concentration.
Adolescent↗
Erythrocyte ammonia in liver disease.
This study reports the relevance of plasma and erythrocyte ammonia concentrations in patients with liver disease. Three groups of subjects were studied: group 1, 47 normal subjects; group 2, 73 patients with liver disease; and group 3, 14 patients with portal-systemic encephalopathy (PSE). The difference in plasma ammonia concentrations between groups 1 and 2 was not significant, but for erythrocyte ammonia this was significant (p less than 0.05). Group 3 subjects had significantly elevated plasma (p less than 0.001) and erythrocyte ammonia (p less than 0.001) compared with the other two groups (Mann-Whitney U-test). In group 3, two patients had plasma ammonia values within the reference range, whereas six patients had values within the range of group 2 subjects. However, none of group 3 subjects had erythrocyte ammonia concentrations within the range of either group 1 or 2. A cut-off level of 65 mumol/l was assigned to differentiate group 3 from group 2 subjects. We conclude that erythrocyte ammonia measurement is a better biochemical index of PSE than plasma ammonia.
Adolescent↗
Twice daily methyldopa: home blood pressure recordings.
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Adult↗
Comparison of enteric-coated levodopa with levodopa-carbidopa combination. A double-blind crossover trial.
Twenty-seven patients with idiopathic Parkinson's disease completed a double-blind crossover trial which compared enteric-coated levodopa (Prodopa) with levodopa-carbidopa combination (Sinemet). It was easy to stabilize the patients' condition with either drug, and the dose-sparing effect both of the enteric-coated preparation, and of the levodopa-carbidopa combination was again noted. At the dosages used, the levodopa-carbidopa combination was objectively shown to be more effective in 71% of the patients investigated, although there was no clear personal preference for either preparation when patients compared the two parts of the trial. Both drugs play a valuable role in the treatment of Parkinson's disease.
Adult↗