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Biomedical subjects

D B Dunger

Publications and source records attributed to D B Dunger.

At least 181 records · Page 10Linked to original sources
PubMed1988

Somatomedin-C/IGF-I measured by radioimmunoassay and somatomedin bioactivity in adolescents with insulin dependent diabetes compared with puberty matched controls.

Plasma concentrations of insulin like growth factor-I in the adolescent diabetic remain controversial. IGF-I levels were determined by radioimmunoassay in 81 insulin dependent adolescent diabetics (49 boys and 32 girls) and compared with 75 puberty stage matched normal controls. Plasma somatomedin bioactivity was determined by cartilage bioassay in a smaller group of 10 normal and 25 diabetic subjects. IGF-I concentrations increased in the normals during puberty and there were no observed differences between the sexes. (P1, 0.77 +/- 0.08; P2, 1.33 +/- 0.2; P3, 1.59 +/- 0.16; P4, 1.76 +/- 0.16; P5, 2.24 +/- 0.09). IGF-I also increased in the diabetics reaching a peak level at stage three in both girls and boys. Combining the data from both sexes the diabetics had significantly lower levels of IGF-I compared to controls at stages; (P1, 0.67 +/- 0.05, ns. P2, 0.92 +/- 0.1, p less than 0.05; P3, 1.16 +/- 0.09, p less than 0.01; P4, 0.73 +/- 0.11, p less than 0.001; P5, 1.13 +/- 0.11, p less than 0.001). Plasma somatomedin bioactivity was elevated in the normals but inhibitory responses were observed in all but two of the diabetic subjects, highlighting the presence of inhibitory factors in diabetic plasma. HbA1C levels rose in the diabetics during puberty, however using covariance analysis there was no relationship between IGF-I and HbA1C when changes relating to puberty were excluded. The importance of controlling for pubertal stage as opposed to age is noted when assessing IGF-I status in the diabetic adolescent.

Adolescent↗
PubMed1987

Lack of effect of lithium carbonate in patients with glycogenosis Ib.

Lithium carbonate has been observed to induce neutrophilia in psychiatric patients and has been used in a number of childhood neutropenic disorders. We tried lithium carbonate in three children with glycogenosis Ib to see if the drug would alleviate the neutropenic complications of the disorder. Mean absolute neutrophil counts rose in one patient but not in the other two. Despite high-dosage schedules, serum lithium levels were highly erratic. Two patients developed potentially severe side effects, including polyuria, diarrhea, and altered mental status. One patient developed pneumonia despite a neutrophil count rise in response to therapy. Lithium carbonate is not useful in patients with glycogenosis Ib.

Adolescent↗
PubMed1987

Two cases of Winchester syndrome: with increased urinary oligosaccharide excretion.

We present our findings in two unrelated patients with the characteristic clinical and radiological features of the Winchester syndrome. The histological findings in gum and skin biopsies taken from one of the subjects, indicated excessive collagen turnover (active phagocytosis, an active endoplasmic reticulum, and an abundance of fibrillogranular material of probable collagen origin). An abnormal oligosaccharide was detected in urine from both patients which was identified as a trisaccharide containing one fucose and two galactose residues. The finding of this oligosaccharide may prove a useful marker in other cases of this rare syndrome and may help elucidate the underlying biochemical defect.

Child↗
PubMed1987

Neurohypophyseal peptide function during early postoperative diabetes insipidus.

Neurohypophyseal function has been investigated in 11 children undergoing pituitary or suprasellar surgery. All had corticosteroid and thyroxine replacement; 9 developed diabetes insipidus (DI) within 1-12 h of operation. At the onset of DI, the plasma vasopressin (AVP) concentration was 3.9 +/- 1.2 pmol/l, considerably higher values usually associated with cranial DI (less than 0.9 pmol/l). AVP fell significantly to 1.1 +/- 0.2 pmol/l by the second day of DI. There was a similar change of levels of the AVP prohormone/carrier peptide, neurophysin I, but plasma oxytocin did not change significantly. High performance liquid chromatography of plasma at the onset of DI revealed a major peak that coeluted with synthetic AVP and two smaller peaks of AVP immunoreactivity. Seven patients required very large doses of desamino-8-D-arginine vasopressin (DDAVP) during the first day; 4 needed smaller doses on day 2. Water deprivation tests were performed on days 6 and 14 after operation in 5 patients with prolonged DI (2 with a triple response). There were no differences in plasma AVP on the two occasions but urinary AVP excretion rate was significantly higher on day 6 (2.4 +/- 0.8 pmol/h) than day 14 (0.7 +/- 0.3 pmol/h). It is concluded that early postoperative DI is not due to decreased levels of circulating AVP but may be related to the release of biologically inactive precursors from the damaged neurohypophysis. These may lead to renal refractoriness to AVP. There is a higher urinary AVP excretion rate on day 6 than day 14 after operation in both patients with a triple response and those with uninterrupted DI. Other factors may determine whether or not a transient resolution phase of DI occurs.

Adolescent↗
PubMed1987

The plasma arginine vasopressin response to insulin-induced hypoglycaemia in children with short stature is related to age and the onset of puberty.

The plasma arginine vasopressin (AVP) response to insulin-induced hypoglycaemia was investigated in 27 children with short stature. None had diabetes insipidus. Six patients were excluded from further analysis because of hypothalamo-pituitary dysfunction. Of the remainder, 14 were prepubertal (Tanner 1) and seven were pubertal (Tanner 2-4). Both groups had similar height velocity retardation. There was a significant rise of AVP of 3.4 +/- 1.3 pmol/l at 30 min in the pubertal group (P less than 0.05) but no significant change in prepubertal patients. There was a significant relationship between chronological age and AVP response 30 min after insulin (r = 0.45, P less than 0.05) and a closer correlation between bone age and AVP response (r = 0.62, P less than 0.01). The data suggest that insulin-induced hypoglycaemia does not reliably stimulate AVP secretion in children and that this response is related to age and pubertal stage.

Adolescent↗
PubMed1987

Increased renal sensitivity to vasopressin in two patients with essential hypernatremia.

Patients with essential hypernatremia maintain urinary concentrating ability despite plasma hyperosmolality and low plasma vasopressin concentrations. We investigated renal sensitivity to ultralow dose vasopressin infusions in two patients with a syndrome of hypodipsia, hypernatremia with selective osmoreceptor dysfunction, early puberty, and aggressive behavior. The patients were water loaded until a hypotonic diuresis was established. Vasopressin was infused in stepwise increments from 0.4-12 fmol/kg X min. Both patients had increased renal sensitivity to vasopressin, achieving negative free water clearance at infusion rates of 0.4 and 4 fmol/kg X min (normal greater than or equal to 6). Treatment for 3 months with 1-desamino-8-D-arginine vasopressin (DDAVP) led to an improvement in behavior and the reporting, for the first time, of a sensation of thirst. After DDAVP therapy both patients had a reduction of their renal sensitivity to infused vasopressin. We conclude that untreated patients with essential hypernatremia have increased renal sensitivity to vasopressin which is reduced by DDAVP administration.

Adolescent↗
PubMed1986

Deletion on the X chromosome detected by direct DNA analysis in one of two unrelated boys with glycerol kinase deficiency, adrenal hypoplasia, and Duchenne muscular dystrophy.

In studies of the X chromosomes of two unrelated boys with adrenal hypoplasia, glycerol kinase deficiency, Duchenne muscular dystrophy, and mental retardation, conventional G banding did not reveal any numerical or structural abnormality, but direct DNA analysis with the X short-arm probes 754, C7, and OCT revealed a deletion in 1 of these patients. It is likely that both boys have a deletion at Xp21 affecting a number of closely linked disease-specific gene loci.

Adrenal Insufficiency↗
PubMed1986

Weber-Christian panniculitis and chronic active hepatitis.

We report a 4-year-old child with Weber-Christian panniculitis who subsequently developed histologically proven chronic active hepatitis, pancreatitis and extensive lipoatrophy. An "LKM" variant autoantibody was detected in the serum and a favourable response has been seen with immunosuppressive therapy. These findings lend support to the concept of Weber-Christian being an "auto-immune" disease.

Autoantibodies↗
PubMed1986

The investigation of atypical psychosomatic illness. A team approach to diagnosis.

The experience of 12 children with atypical psychosomatic illness who presented considerable diagnostic difficulties is described. All patients were admitted to the hospital, and using a team approach, it was possible to make a confident diagnosis. No organic disease was identified, and a psychosomatic diagnosis was made. In general, atypical symptoms and signs, false-positive investigations, and differing medical opinion had compounded the diagnostic difficulties. The value of the observations made by ward staff of the childrens' symptoms and behavior is stressed.

Adolescent↗
PubMed1986

Antidiuretic effect and pharmacokinetics of oral 1-desamino-8-D-arginine vasopressin. 1. Studies in adults and children.

The dose-response relationships and pharmacokinetics of orally administered 1-desamino-8-D-AVP (DDAVP) were investigated. In water-loaded normal subjects marked reductions in urine flow rate and increases in urinary osmolality occurred. The responses were maximal 2 h after ingestion of 50, 100, and 200 micrograms, and lasted at least 6 h. Plasma DDAVP levels increased in a dose-dependent fashion and its disappearance from the plasma followed an exponential time course, with a half-life 86 to 142 min. In water-loaded adults with central diabetes insipidus (CDI), 200 micrograms oral DDAVP caused marked antidiuresis and mean urinary osmolality increased from 107 mosm/kg to 554 mosm/kg after 3 h. In these patients the effect also lasted at least 6 h. Children with CDI were less sensitive than normal subjects to the 50-microgram dosage and required 100 micrograms orally to achieve a duration of action similar to that resulting from 200 micrograms in adults. Oral DDAVP may be useful for treating some patients with CDI.

Administration, Intranasal↗
PubMed1986

Long-term treatment with diazoxide in childhood hyperinsulinism.

This paper reviews the outcome in 12 children with hyperinsulinaemic hypoglycaemia who first developed symptoms between the ages of 2 and 8 months and who were treated with diazoxide (5-20 mg/kg/day) for 2-13 years. Two cases required subtotal pancreatectomy at the ages of 5 and 10 years because of recurrent hypoglycaemia and one girl with severe retardation died at the age of 6 years while still on diazoxide therapy. Two patients aged 3.5 and 9 years are still on treatment and in 7 cases diazoxide was discontinued between the ages of 2.5 and 14 years, indicating that spontaneous remission can be expected in a high proportion of children with post-neonatal hyperinsulinaemic hypoglycaemia. Of the 9 children who started diazoxide within 3 months of the onset of symptoms, 5 are of normal intelligence and 4 are moderately retarded (IQs 63-71). In 3 children diazoxide was started 8 months to 3 years after the onset of symptoms; two are retarded (IQs 60-70) and the third was severely retarded and died aged 6 years.

Diazoxide↗
PubMed1985

Infectious and bleeding complications in patients with glycogenosis Ib.

Clinical, hematologic, and immunologic findings were reviewed in 21 patients with glycogenosis Ib. Fifteen of the patients suffered from moderate to severe bacterial infections. Ten patients had excessive epistaxis or bleeding from surgical sites, and eight suffered oral and anal mucosal ulceration. Sixteen of 21 patients exhibited chronic neutropenia associated with abnormalities in myeloid maturation and decreases in the bone marrow storage and peripheral marginating pools. Diminished neutrophil motility was documented in 14 of 15 patients tested, and adherence was decreased in three patients studied. Neutrophil microbicidal activity, reduction of nitroblue tetrazolium, and ingestion were normal in all patients tested. Bleeding times were prolonged in five of eight patients, and results of platelet function studies were abnormal in five individuals. Excessive bleeding in patients with glycogenoses Ia and Ib are similar and may be secondary to the functional deficiency of glucose-6-phosphatase. However, neutropenia, neutrophil dysfunction, and the resulting infectious complications are specific for Ib disease and may be related to abnormal glucose-6-phosphate transport.

Adolescent↗
PubMed1985

Lack of thirst, osmoreceptor dysfunction, early puberty and abnormally aggressive behaviour in two boys.

Two unrelated boys (C.C. 13 years; J.W. 18 years) presenting with early puberty and episodes of aggressive behaviour were found to have hypernatraemia and hypodipsia. Plasma vasopressin (AVP) levels were inappropriately low in relation to plasma osmolality, but the patients did not have diabetes insipidus since 24 h urinary volumes were less than 1 litre and the maximal urinary osmolality was 1232 in C.C. and 950 in J.W. Plasma renin activity was elevated (greater than 2000 mg AI/1/h) although aldosterone concentrations were normal. Excretion of a water load (20 ml/kg) was delayed, but plasma renin and aldosterone fell with increased naturesis. An infusion of 0.85 mol/l saline produced a rise in AVP in C.C. but not in J.W. Insulin and hypotension resulted in the release of AVP in both boys suggesting a selective defect of osmoreceptor function. Hyperprolactinaemia and an exaggerated PRL response to TRH were also noted but no intracranial lesion was demonstrable on CT scan. These boys appear to have a hypothalamic syndrome with early puberty, hyperprolactinaemia, hypodipsia and osmoreceptor dysfunction which may be associated with aggressive behaviour.

Adolescent↗
PubMed1985

Raynaud's disease.

We report five children who presented with Raynaud's disease in whom we could find no clinical, haematological, or immunological evidence of a collagen disorder. Oral phenoxybenzamine proved useful for maintenance treatment in most, with infusions of prostacyclin, nitroprusside, and ketanserin during acute attacks.

Antihypertensive Agents↗