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Biomedical subjects

R W Smithells

Publications and source records attributed to R W Smithells.

At least 37 records · Page 2Linked to original sources

Onset and severity of hearing loss due to congenital rubella infection.

Of 111 children born in 1978-82 with congenital rubella infection, confirmed by the detection of rubella specific IgM shortly after birth, 68 were reported to be hearing impaired when notified to the National Congenital Rubella Surveillance Programme (NCRSP). The average age at which the diagnosis of hearing impairment was confirmed was 11.6 months. Recent audiograms showed a hearing loss that was usually uniform across all frequencies, equal in both ears and severe, averaging 93 dB across the range 250-4000 Hz. Despite the early diagnosis of congenital rubella and the known risk of deafness, only eight of 57 infants had been tested for auditory evoked responses in the first 6 months of life; all eight had severe hearing loss. Evidence of progressive hearing loss was found in only one of the 57 children. Definitive hearing tests (as distinct from screening tests) were frequently delayed until after the first birthday. Newborn babies identified as being at high risk of congenital deafness should have a full audiological assessment in early infancy.

Age Factors

Neural tube defect recurrence after 'partial' vitamin supplementation.

A total of 227 mothers enrolled for periconceptional multivitamin supplementation because of previous neural tube defect (NTD) births took vitamins for less than the recommended minimum period (at least 28 days before conception until two menstrual periods have been missed). Of 213 examined infants/fetuses born to these partially supplemented mothers, two had NTD, one of whom followed four previous NTDs. The observed NTD recurrence rate is similar to that observed in fully supplemented mothers. A further 14 mothers started supplements before the second missed period but after the normal time of neural tube closure. Three of their offspring had NTD. The significance of this apparently high recurrence rate is discussed.

Ascorbic Acid

Temporal relations between maternal rubella and congenital defects.

The Time relations between maternal rubella infection in pregnancy and the presence and type of defects in the children were determined from the records of 422 children with confirmed congenital rubella, registered in the National Congenital Rubella Surveillance Programme. In the 106 children born after laboratory-proven maternal infection, no defects were recorded following infection after the 17th week of pregnancy, but in the remaining 316 children defects followed infection reported to be as late as 33 weeks. The striking difference underlines the importance of serological investigation of pregnant women who present with a rash or a history of contact with rubella. With proven infection later than the 16th week the risk of fetal damage seems to be very small. Of 148 children followed up to school age, 40 (27%) attended normal schools.

Abnormalities, Multiple

Congenital rubella in babies of south Asian women in England and Wales: an excess and its causes.

The incidence of congenital rubella was found to be 2.3 times higher in Asian than non-Asian births in England and Wales. This was attributed in part to higher susceptibility to rubella in Asian than non-Asian women, as shown by antenatal serological data from public health laboratories in Leeds, Luton, and Manchester. Examination of the ethnic origin of pregnant women requesting laboratory testing after contact with rubella or rash and of women with laboratory confirmed rubella in pregnancy also suggested that the disease was being underdiagnosed in pregnant Asian women. Failure to prevent congenital rubella by termination of infected pregnancies may therefore contribute to the increased incidence of the syndrome in Asians. Health education programmes about the dangers of rubella in pregnancy and of the need for vaccination can readily be promoted in the Asian community through existing ethnic organisations. Protection of other ethnic minorities likely to be at similar increased risk may require a vaccination programme aimed at national elimination of rubella.

Asia

Weber-Christian panniculitis.

Two cases of Weber-Christian panniculitis with onset at 7 months and 3 years 6 months are described. Both show evidence of disturbances in immune mechanisms. The family of the first case has a high prevalence of coeliac disease, and the mother of the first patient also suffers from alopecia areata and dermatitis herpetiformis.

Child, Preschool

High-performance liquid chromatographic determination of carbamazepine and carbamazepine 10,11-epoxide in plasma and saliva following solid-phase sample extraction.

A rapid, sensitive and simple to operate high-performance liquid chromatographic method for the simultaneous determination of carbamazepine (CBZ) and carbamazepine 10,11-epoxide (CBZ-EP) in plasma and saliva is described. The drug and its metabolite are extracted from both plasma and saliva using commercially available reversed-phase octadecylsilane bonded silica columns (Bond-Elut C18, 2.8 ml capacity). Separation of CBZ and CBZ-EP was achieved by reversed-phase chromatography, using a mobile phase consisting of acetonitrile-methanol-water (19:37:44) at a flow-rate of 1.8 ml/min in conjunction with a Nova-Pak C18 column. The analytical column, in Radial-Pak cartridge form, was used in combination with a Z-module RCSS and protected by a Guard-Pak precolumn module containing a Guard-Pak mu Bondapak C18 insert. Using ultraviolet detection at 214 nm, levels in the region of 50-100 ng/ml for CBZ and CBZ-EP can be measured with only 250 and 500 microliters of plasma and saliva, respectively. The method, which has been used to determine steady-state concentrations of the drug and its metabolite in paediatric patients receiving CBZ monotherapy, is also suitable for pharmacokinetic studies.

Carbamazepine

The effects of maternal folic acid and vitamin C nutrition in early pregnancy on reproductive performance in the guinea-pig.

1. The effect of different intakes of folic acid (FA) and vitamin C on pregnancy in the Dunkin-Hartley guinea-pig was examined. Female guinea-pigs were subjected to three graded intakes of FA and vitamin C ('deficient', 'intermediate' similar to recommended daily intakes (RDI), and 'supplemented') during early gestation and up to the time of neural tube closure (17th day of gestation), and then returned to the RDI of these vitamins. 2. Plasma and blood cell concentrations of these vitamins were measured once before and at the end of the dietary treatments. Reproductive performance was assessed in terms of the number of resorbed and aborted embryos and weight and size of the live fetuses on the 36th day of gestation. 3. The short-term deficiency of either of these two vitamins, insufficient to affect maternal health, had a dramatic effect on the reproductive performance. 4. The RDI of FA was significantly less effective than the supplemented intake in preventing embryonic deaths. The RDI of vitamin C produced lighter and smaller live fetuses than the supplemented intake. 5. The implications of these findings with regard to vitamin status in early pregnancy in man are discussed.

Animals

Recurrent neural tube defects, risk factors and vitamins.

Data from our trial of periconceptional vitamin supplementation for the prevention of neural tube defects have been analysed to assess the influence of various factors on recurrence rates of neural tube defect. Our data suggest that the risk of recurrence of neural tube defect is influenced by the number of previous neural tube defects, area of residence, immediately prior miscarriage, and interpregnancy interval. None of these factors, however, contributed any significant differential risk between supplemented and unsupplemented mothers. Hence we conclude that the highly significant difference in recurrence rates of neural tube defect between supplemented and unsupplemented mothers was due to vitamin supplementation.

Abortion, Spontaneous