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Biomedical subjects

P M Mathew

Publications and source records attributed to P M Mathew.

9 recordsLinked to original sources

Cystic fibrosis presenting with recurrent vomiting and metabolic alkalosis.

Between January 1980 and December 1987, ten Saudi Arabian children at Saudi Arabian Oil Co. (Saudi Aramco) health care facilities in the Eastern Province of Saudi Arabia had cystic fibrosis (CF). The incidence of CF in Saudi Arab children less than or equal to 14 years was 1 in 4243. Five of the ten children had hypoelectrolytaemia and metabolic alkalosis on initial presentation. Two of the five had recurrent vomiting, hypoelectrolytaemia and metabolic alkalosis alone and initially no chest symptoms. Early exclusion of CF should be part of the workup in any child, especially in an infant with hypoelectrolytaemia and metabolic alkalosis.

Alkalosis

Complications of diazoxide treatment in persistent neonatal hyperinsulinism.

Seven infants with persistent neonatal hyperinsulinism were treated in Dhahran Health Centre from 1983 to 1986. The insulin:glucose ratio (serum insulin concentration pmol/l) divided by the blood glucose concentration (mmol/l) ranged from 12 to 636, mean (SD) 177 (201). To control hypoglycaemia, diazoxide (12-24 mg/kg/day) was given in a continuous intravenous glucose infusion (12-22 mg/kg/min) on 11 separate occasions, four infants twice each and three infants once each. An increase of more than one standard deviation in the heart and respiratory rates, together with other symptoms of heart failure, was considered to be evidence of diazoxide toxicity. Cardiorespiratory failure (toxicity) occurred on eight of the 11 occasions (73%) in seven infants. The average daily fluid intake, weight change, respiratory rate and heart rate before treatment were similar whether or not the infant developed toxicity. A diazoxide toxicity index was obtained by multiplying the dose of diazoxide by the insulin:glucose ratio to relate the diazoxide dose to the severity of the disease. In all instances when the toxicity index was more than 1533 (mean (SD) 3732 (2741) cardiac toxicity developed. In contrast, infants with a toxicity index of less than 675 (mean (SD) 364 (270), had no symptoms of toxicity. Symptoms were significantly related to the severity of the disease and the diazoxide dose. It is possible to use the toxicity index to predict the risk of toxicity and to calculate a safe dose of diazoxide in infants with persistent neonatal hyperinsulinism.

Blood Glucose

Persistent neonatal hyperinsulinism.

Over a 3-year period, the diagnosis of persistent neonatal hyperinsulinism (PNH) was made in seven infants, from an unselected cohort of 18,726 births, all of Saudi Arabian origin. Thus the incidence of PNH was one in 2,675 births. The high incidence, associated consanguinity, and occurrence in siblings suggest that PNH may be inherited as an autosomal recessive disorder.

Consanguinity

Neonatal diabetes mellitus in first cousins.

The cases of two first cousins with neonatal diabetes mellitus are presented in this report. In one child the condition was transient, whereas the other has permanent diabetes mellitus. It seems unlikely that the difference in outcome was related to the degree of diabetic control early in the course of the disorder. The possibility of genetic influence in the etiology of neonatal diabetes is stressed. Chlorpropamide was not useful in curing neonatal diabetes mellitus in one case.

Blood Glucose

Hypoparathyroidism in Arab children.

Four children with hypoparathyroidism are reported. We believe that this is the first report of this condition from Kuwait or Saudi Arabia. Treatment and course of disease are discussed. Two children have hypoparathyroidism as part of DiGeorge Syndrome.

Child

Adrenal insufficiency possibly due to lipoid adrenal hyperplasia: a case report.

An infant who presented with adrenal insufficiency possibly due to lipoid adrenal hyperplasia at the age of 66 days is described. This is the first time that this very rare disorder is reported from Saudi Arabia. Adrenocorticotrophic hormone (ACTH) stimulation test is useful in the ante-mortem diagnosis of this condition.

Adrenal Hyperplasia, Congenital

Ethnic differences in congenital malformations.

Perinatal deaths and major lethal and non-lethal congenital malformations occurring in this hospital from 1979-82 inclusive were related to the ethnic group of the 15 438 mothers. The highest crude perinatal mortality rates occurred in Indian and Pakistani populations (18.3 per 1000 and 24.1 per 1000 respectively). The highest incidence of congenital abnormality also occurred in these groups (13.3 per 1000 and 12.8 per 1000 respectively), but there was considerable variation in the distribution of different malformations.

Adolescent