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Biomedical subjects

P R Stutchfield

Publications and source records attributed to P R Stutchfield.

11 recordsLinked to original sources
PubMed2001

Severe congenital myotonic dystrophy and severe anaemia of prematurity in an infant of Jehovah's Witness parents.

Severe congenital myotonic dystrophy (CMD) is an autosomal dominant condition characterized by hypotonia and respiratory insufficiency at birth. Terminal outcome has been reported in infants requiring ventilation for longer than 30 days. The case is reported of an infant born at 34 weeks' gestation with severe CMD. Infant survived following ventilatory support from birth until day 67 of life. Subcutaneous erythropoietin (600 units, three times weekly) was commenced on day 6 as the Jehovah's Witness parents were strongly opposed to blood transfusions. Haemoglobin fell to 5.8 g/dL without adverse effects and then progressively rose to 15.4 g/dL. No blood transfusions were necessary. This case illustrates that infants with severe CMD requiring ventilation for more than 30 days do not have a universally fatal outcome. Low haemoglobin was well tolerated which calls for re-examination of the indications for blood transfusions in ventilated neonates.

Adult↗
PubMed1989

Ciprofloxacin in neonatal Enterobacter cloacae septicaemia.

Multiresistant Enterobacter cloacae infection in six premature infants was eradicated with intravenous ciprofloxacin (10 mg/kg/day). Bacterial resistance did not develop. Adequate plasma ciprofloxacin concentrations were achieved in all treated patients. No clinical evidence of side effects was observed.

Ciprofloxacin↗
PubMed1989

Electrolytes and glucose in cerebrospinal fluid of premature infants with intraventricular haemorrhage: role of potassium in cerebral infarction.

Potassium, sodium, and glucose concentrations in cerebrospinal fluid and plasma were determined in 73 infants whose gestational ages ranged from 25 to 40 weeks. Six of 29 (21%) neonates with intraventricular haemorrhages had raised potassium concentrations in the cerebrospinal fluid (3.7-30 mmol/l); five developed cerebral infarctions. There was a significant correlation between sodium and glucose concentrations in plasma and cerebrospinal fluid with prolonged hypoglycorrhachia accompanying posthaemorrhagic hydrocephalus. Raised concentrations of potassium in cerebrospinal fluid occur with intraventricular haemorrhage and may contribute to the development of cerebral infarction.

Blood Glucose↗
PubMed1988

HLA type, islet cell antibodies, and glucose intolerance in cystic fibrosis.

Impaired glucose tolerance, assessed by a raised glycated haemoglobin (HbA1) concentration, was found in 24 (39%) out of 61 patients with cystic fibrosis with an age range of 1-23 years. No correlation between age and HbA1 concentration was found indicating that factors other than progressive pancreatic fibrosis may be important in the aetiology. HLA typing, islet cell antibodies, and autoantibody screen were completed. Eighteen (75%) out of 24 patients with cystic fibrosis who had an impaired glucose tolerance had HLA-DR3 or HLA-DR4 antigens compared with 23 (62%) out of 37 patients with normal glucose tolerance. Islet cell antibodies were present in seven (15%) out of 46 patients with cystic fibrosis; the prevalence in a normal population is 0.5%. Five (25%) of the 20 patients with a raised HbA1 concentration were positive for islet cell antibodies compared with two (8%) out of the 26 with normal glucose tolerance. Six (86%) out of seven patients who were positive for islet cell antibodies had HLA-DR3 or HLA-DR4 antigens. There was no general autoantibody production. Islet cell antibodies may play a part in the development of glucose intolerance in some patients with cystic fibrosis by being produced in those who are genetically predisposed as part of an immune response to damaged pancreatic tissue.

Adolescent↗
PubMed1988

Phototherapy and the use of heat shields in very low birthweight infants.

The irradiance produced by phototherapy units in use on a neonatal unit was measured. The phototherapy tubes were found to decline at a variable rate. Heat shields used to decrease heat and evaporative losses in the very low birthweight infant reduce irradiance and may appreciably limit the effectiveness of phototherapy.

Critical Care↗
PubMed1987

Glycosylated haemoglobin and glucose intolerance in cystic fibrosis.

Sixty four patients, age range 1-20 years, with cystic fibrosis had their tolerance to glucose assessed according to their glycosylated haemoglobin (HbA1) concentrations. Raised concentrations were found in 24 (37.5%). Oral glucose tolerance tests were performed on 21 patients with raised HbA1 and 13 patients with normal HbA1 concentrations. C peptide responses were also measured to assess islet cell function. Patients with normal HbA1 had normal glucose tolerance and C peptide response. Seven of 21 patients with raised HbA1 concentrations were glucose intolerant. The remaining 14 patients with raised HbA1 concentrations had normal glucose tolerance but a reduced C peptide response, suggesting impaired islet cell function. There were no appreciable differences in the incidence of chest infections, respiratory function, and chest x-ray scores among patients with normal HbA1 concentrations, raised HbA1 concentrations, and normal oral glucose tolerant tests, and patients who were glucose intolerant. No correlation was found between HbA1 concentration and age or Shwachman score. Measuring HbA1 concentrations periodically is useful in detecting and monitoring glucose intolerance in patients with cystic fibrosis.

Adolescent↗
PubMed1986

X-linked mesangiocapillary glomerulonephritis.

Two related male patients with mesangiocapillary glomerulonephritis (MCGN) are described demonstrated by renal biopsy, inherited as an X-linked disorder. Family investigations failed to reveal any underlying immunological defects or a marker for the female carrier state. The age at diagnosis, the result of discovery of proteinuria on routine urine testing during infancy, is earlier than in any other reported cases of MCGN. This raises the possibility that this variety of MCGN may develop in utero and be detectable by alpha-fetoprotein maternal screening.

Genes, Recessive↗