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Biomedical subjects

F Harris

Publications and source records attributed to F Harris.

At least 73 records · Page 4Linked to original sources

The Liverpool Congenital Malformations Registry.

Data collection and validation for the Liverpool Congenital Malformations Registry (LCMR) are described. Founded in 1960, the LCMR increased its area of surveillance in 1979 to include five health districts in Liverpool and its environs with approximately 20,000 births per annum. The LCMR is now one of the members of the European Congenital Anomalies Register (EUROCAT). Multiple sources of ascertainment are employed, the most useful of these being OPCS notifications, hospital discharge letters and data from specialised paediatric units. In spite of several difficulties encountered in data collection the data base is an invaluable tool both for routine monitoring of prevalence rates and as a starting point for epidemiological research.

Congenital Abnormalities↗

Clinical evaluation of oral mexiletine therapy in the treatment of ventricular arrhythmias.

The effect of oral mexiletine therapy on ventricular arrhythmias was evaluated in 58 patients in whom conventional drugs had been unsuccessful. Mean daily dose of mexiletine was 652 mg (range 250 to 1,500) and mean duration of therapy was 14.4 months (range 0.1 to 34.4). Mexiletine was associated with a decrease of 52% in total premature ventricular complexes in 24 hours compared with control (6,841 +/- 1,053 [SEM] versus 3,248 +/- 734, p less than 0.005) and 19 patients (36.5%) had a greater than 83% decrease in ventricular ectopic rhythm. The drug was discontinued in 6 of these 19 patients because 5 of them (26%) experienced side effects after a mean period of 29.6 weeks (range 0.83 to 63.2) and sudden death occurred in 1 patient (5%); this indicates effective suppression of ventricular ectopic rhythm without significant side effects in 13 (25%) of 52 patients during long-term therapy. Adjustment of drug dosage to achieve therapeutic blood levels resulted in an efficacy on ventricular ectopic rhythm similar to that obtained with the maximal tolerated dose. There was no correlation between drug dose and therapeutic effectiveness. Mexiletine was associated with a 48% decrease in episodes of ventricular tachycardia (345.5 versus 179.3/24 h) and 5 of 10 patients with a history of cardiac arrest remained free of symptomatic ventricular tachyarrhythmias for 14.8 months (range 3.7 to 24.3).(ABSTRACT TRUNCATED AT 250 WORDS)

Administration, Oral↗

Epidemiology of facial clefting.

An analysis was performed of patients with facial clefts notified between 1960 and 1982 to the Liverpool Congenital Malformations Registry. From 1960-82 there were 325 727 births in the area surveyed and 544 cases of facial clefting were notified. When 88 patients with recognised syndromes and multiple congenital anomalies were excluded, the overall prevalence of facial clefts alone was 1.4 per 1000 total births. This group was then classified further into 137 cases of cleft lip alone, 166 cases of cleft lip and palate, and 153 cases of cleft palate alone. The prevalence of these groups per 1000 total births is 0.42, 0.51, and 0.47 respectively. There were some fluctuations in annual prevalence with rises being observed in the mid and late 1960s and mid and late 1970s. There was a noticeable male predominance in the cleft lip and cleft lip and palate groups of 1.52:1 and 1.98:1 respectively, with a 1:1 ratio in the cleft palate group. There were no significant differences in birthweight and mean maternal age in the three groups. In the cleft palate group, however, there was a significant trend towards an increase in the frequence of conception in the second half of the year. There was a maternal history of epilepsy in 4.4% of the cleft lip and 3% of the cleft lip and palate groups but only in 1 patient (0.6%) in the cleft palate group. The study illustrates the importance of environmental factors in the aetiology of facial clefting.

Cleft Lip↗

Benign intracranial hypertension following severe hyponatremic dehydration in congenital adrenal hyperplasia.

A case of salt-losing congenital adrenal hyperplasia with severe hyponatremic dehydration is presented. Clinical signs and symptoms of cerebral edema with elevated intracranial pressure were present. Conventional treatment was started, and after initial concern regarding future head growth and development, there was a good outcome with normal development at 1 year of age. This course is suggestive of benign intracranial hypertension. Possible mechanisms are discussed with a review of the relevant literature.

Adrenal Hyperplasia, Congenital↗

Slipped upper femoral epiphysis and primary juvenile hypothyroidism.

The pathogenesis of slipped upper femoral epiphysis is unknown but the condition has been linked with various endocrine disorders. Nine patients with slipped epiphyses in association with primary juvenile hypothyroidism are presented. In all patients, slipping occurred or symptoms developed in the affected hip before the hypothyroidism was diagnosed. A generalised pathology was suggested by the absence of trauma (8 patients), by bilateral slipping (6 patients), and by obesity and short stature in all patients. All cases had delayed skeletal maturation and characteristic metaphysial changes were seen on their radiographs. The clinical diagnosis of juvenile hypothyroidism can be difficult but it merits consideration in patients who have a slipped upper femoral epiphysis in association with short stature, obesity, delay in skeletal maturity, or any one of these.

Adolescent↗

Potential uses of human pancreatic growth hormone-releasing factor 1-44 amide.

A family of growth hormone releasing peptides have been isolated and characterized from human pancreatic islet cell tumours. We have compared the growth hormone release in normal volunteers and patients with various hypothalamo-pituitary disorders, following direct stimulation of the pituitary using 50 micrograms of the most potent homologue, hp GRF 1-44 amide i.v. with that following indirect stimulation using oral clonidine 0.15 mg/m2, which depends on intact hypothalamic mechanisms. These tests both produced a wide variation in GH response in normal volunteers, considerable GH release following hp GRF 1-44 amide but little after clonidine in idiopathic GH deficiency, and indistinguishable, negligible responses in patients with craniopharyngiomas and pituitary tumours associated with GH deficiency. Two untreated acromegalics showed GH increments in the normal range despite elevated basal levels. It is concluded that hp GRF 1-44 amide is of limited diagnostic value by itself, but may be of considerable therapeutic use in patients with idiopathic GH deficiency.

Acromegaly↗

Adverse drug reactions in medical inpatients.

Fifteen of 268 children admitted to a general medical ward suffered a definite or probable adverse drug reaction to their treatment. In 6 of these children the reactions were preventable. Anticonvulsants were the most common cause of an adverse reaction.

Anticonvulsants↗

Occult pneumococcal bacteraemia and febrile convulsions.

Over two years 29 children had bacteraemia due to Streptococcus pneumoniae at this hospital. In 15 previously healthy children the site of infection could not be identified, and in most of them, bacteraemia was not suspected clinically. All 15 had high total white cell (greater than or equal to 17 x 10(9)/1) and neutrophil (greater than or equal to 11 x 10(9)/1) counts. Twelve children were under 4 years of age, and of these, 10 had been admitted because of a simple febrile convulsion and one had a prolonged febrile convulsion. Occult pneumococcal bacteraemia has been reported in the USA for more than 10 years, but no series has been reported from the United Kingdom. Occult pneumococcal bacteraemia may be an important cause of febrile convulsions. Persisting bacteraemia and the development of focal infections, including pneumococcal meningitis, have been reported. Meningitis did not occur after occult bacteraemia in our patients. Studies to date have been retrospective, and thus the true incidence of the complications and the best treatment are not clear. A prospective study of children with febrile convulsions could provide answers.

Age Factors↗

The incidence of Down's syndrome over a 19-year period with special reference to maternal age.

The incidence of Down's syndrome in the Liverpool and Bootle areas from 1961 to 1979 was investigated. A total of 319 liveborn cases was ascertained over this period. Using 3-year moving averages, the incidence of the condition fell gradually from 1.62 per 1000 livebirths for 1961 to 1963 to 1.09 per 1000 livebirths for 1977 to 1979. This trend is significant at the 0.1% level. Over the same period the mean maternal age of Down's syndrome births fell gradually from 36.7 years in 1961 to 29.0 years in 1979. This trend is significant at the 1% level. There was a contemporaneous decrease in the proportion of total births to women over 35 years in the study area. Cytogenetic analysis was performed on 175 out of the 319 index cases (54.9%). Of these, there were 161 trisomies (92%), 11 translocations (6.3%), and three mosaics (1.7%). Between 1969 and 1979 four terminations of pregnancy for Down's syndrome were performed, all for trisomy. Quinquennial age specific incidences for Down's syndrome were calculated for the years 1960 to 1964, 1965 to 1969, 1970 to 1974, and 1975 to 1979. There have been no statistically significant changes over this time. It is suggested that the fall in incidence of Down's syndrome can be explained by the fall in mean maternal age.

Adult↗