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Biomedical subjects

D B Dunger

Publications and source records attributed to D B Dunger.

194 records · Page 11Linked to original sources
PubMed1984

Glutaric aciduria type I presenting with hypoglycaemia.

We present a child with glutaryl CoA-dehydrogenase deficiency (type I glutaric aciduria) who presented with bilateral subdural hydromas, and progressive choreoathetosis and dysarthria. The diagnosis was made when she was investigated for hypoglycaemia at the age of 3.5 years. Temporary adrenocortical insufficiency was also noted. Three years after diagnosis the adrenal insufficiency and hypoglycaemia have resolved and treatment with riboflavin and 'lioresal', a GABA analogue, has prevented any further neurological deterioration.

Cells, Cultured↗
PubMed1984

Patterns of growth in the hepatic glycogenoses.

Longitudinal growth data from 31 patients with hepatic glycogen storage disease (type I (8 patients), type Ib (three patients), type III (13 patients), and type IX (phosphorylase kinase deficiency) (7 patients) ) have been reviewed. All patients were below the mean for height at presentation; the mean height standard deviation scores were -2.13 (type I), -2.0 (type Ib), -2.4 (type III), and -1.6 (type IX). Untreated, most patients with type I and Ib grew slowly with no catch up growth but three patients with mild disease grew normally. Most children with type III disease grew at a normal velocity throughout childhood. Puberty was delayed and final height normal. Some of the children with type III and all of those with type IX had catch up growth throughout childhood. Intensive treatment of patients with severe forms of type I and Ib disease resulted in catch up growth, but this was not complete if the treatment was started late.

Body Weight↗
PubMed1982

Growth and endocrine changes in the hepatic glycogenoses.

The biochemical and endocrine responses of 13 patients with hepatic glycogen storage disease (HGSD) (type I-six patients, type Ib-two, type III-three, type IX-two patients) to an oral glucose load have been investigated. Longitudinal growth data was available in all patients. The height velocity standard deviation score (HVSDS) was positively correlated with the plasma somatomedin and inversely correlated with the glucose-insulin ratio, plasma cortisol and plasma growth hormone concentrations. There was no correlation between plasma glucagon and HVSDS with treatment was accompanied by a rise in plasma somatomedin and a fall in growth hormone and cortisol. In two patients the glucose-insulin ratio decreased. Growth retardation in HGSD can be explained as part of the adaptation to the inability to maintain normal glucose homeostasis.

Adolescent↗
PubMed1982

Value of the glucagon test in screening for hepatic glycogen storage disease.

The fasting glucagon test of 40 patients with hepatic glycogen storage disease (type I, 13 patients; type Ib, 5 patients; type III, 12 patients; type IX (phosphorylase kinase deficiency), 10 patients) has been reviewed. In all patients with types Ib and III the blood glucose level rose less than 1 mmol/1 but in types 1 and IX there was wide variation in response. Blood lactate levels were greater than 2.4 mmol/1 in all patients with type I and Ib 120 minutes after the administration of glucagon but were lower than 2.4 mmol/1 in types III and IX. The value of this test for screening was analysed using several criteria. From the data a simplified scheme for the investigation of patients with suspected hepatic glycogen storage disease is proposed.

Blood Glucose↗
PubMed1981

The use of four-drug combination chemotherapy (D.A.V.E.) in the treatment of advanced Wilms' tumour.

A study was begun in 1971 at St. Bartholomew's Hospital with a combination of 4 drugs, dactinomycin (actinomycin D), adriamycin, vincristine and Endoxan (cyclophosphamide) (D.A.V.E.), together with surgery and radiation, in the treatment of stage III and stage IV Wilms' tumour. Seventy-one percent of the children treated achieved complete response. The median survival from diagnosis was 19 months, and in those children achieving complete response the median disease-free survival has not yet been reached. Toxicity was not a serious problem. The study group is compared with a group of children treated at this hospital before 1971. There is an improved survival in the children treated with D.A.V.E. Children who have relapsed with stage I or stage II disease may also respond. This four-drug combination was well tolerated and effective, and confirms recent experience suggesting that intensive multiple-drug regimens may be curative even in advanced disease.

Adolescent↗
PubMed1980

Effect of naloxone in a previously undescribed hypothalamic syndrome. A disorder of the endogenous opioid peptide system?

A syndrome of disordered hypothalamic function with abnormal control of temperature, appetite, and thirst, hyperprolactinaemia, and inappropriate vasopressin release is described in a 13-year-old boy who, in addition, had insensitivity to pain and a more general disorder affecting mood, sleep, and control of respiration. A disturbance of the opioid peptide system is postulated. Naloxone reversed central analgesia, altered urine fluid and electrolyte excretion, modified the hormonal response to gonadotrophin-releasing and thyrotrophin-releasing hormones, and improved the auditory and visual reaction times. Specific opioid antagonists may have a therapeutic role.

Adolescent↗
PubMed1980

Renal tubular acidosis and nerve deafness.

Two brothers are described with renal tubular acidosis and nerve deafness: the elder also had rickets and hypokalaemia. The parents were unaffected. Studies of urinary acidification and bicarbonate excretion were consistent with a distal tubular abnormality. This report strengthens the view previously proposed in similar cases that nerve deafness and renal tubular acidosis constitute a genetic entity. Examination for nerve deafness is indicated in any child with renal tubular acidosis.

Acidosis, Renal Tubular↗
PubMedDate not supplied

A comparison of morning and bed-time ultralente administration when using multiple injections in adolescence.

Ten adolescents took part in a randomized sequential crossover study comparing two insulin regimes: (1) three preprandial injections of soluble insulin from a pen injector plus ultralente given before bed; (2) morning soluble insulin with ultralente, plus two preprandial injections of soluble. Nine patients completed the study. Metabolic profiles were performed at the end of each 3-month treatment period. Glycosylated haemoglobin levels did not fall significantly on either regimen, and there were no differences between the changes in HbA1 concentration on the two regimens. However, blood glucose concentrations tended to be lower with bedtime than with morning ultralente, and were significantly different at 0800 h (13.1 +/- 2.3 mmol/l vs 17.9 +/- 1.9 mmol/l, p less than 0.02) and 1000 h (14.5 +/- 1.4 mmol/l vs 18.6 +/- 0.7 mmol/l, p less than 0.03). Plasma free insulin concentrations were not different. Total daily insulin dose and the proportion of ultralente given in each regimen did not differ. Results indicate that the rise in early morning fasting blood glucose concentrations was less when ultralente was given at bedtime rather than in the morning.

3-Hydroxybutyric Acid↗
PubMedDate not supplied

An unbiased glucose clamp method using a variable insulin infusion: its application in diabetic adolescents.

A simple, unbiased insulin-varying glucose clamp program is described. The aim of the program was to utilize a continuously updated array of data to predict insulin requirements for normoglycaemia. In assessing Type 1 diabetes the quantity of insulin required for maintenance of basal euglycaemia can be more clinically informative than other clamp methods. We present a method which uses an iterative computer program to predict changes in insulin infusion rate required for glucose clamping. After initial parameter estimation, the program uses no fixed algorithm but makes predictions according to previous blood glucose responses to infusion rates. The program has flexible data entry, graphic display, and running statistics including mean infusion data, mean glucose levels, and their respective standard deviations. Data for 26 consecutive overnight clamp studies have been analysed. The median coefficient of variation of glucose values at the end of the clamp was 4.1% (range 1.4-12.0%). The mean bias during the last 2 h was 0.20 +/- 0.24 mmol l-1. Cross-correlation showed that insulin had its maximal effect on the rate of decline of glucose after 15 min, and the nadir of glucose occurred 45 min after a change in insulin infusion rate.

Adolescent↗