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Biomedical subjects

N J Shaw

Publications and source records attributed to N J Shaw.

At least 109 records · Page 6Linked to original sources
PubMed1995

Changes in pulmonary artery pressure in infants with respiratory distress syndrome following treatment with Exosurf.

The pulmonary artery pressure (PAP) changes were studied using Doppler echocardiography in preterm infants treated with an artificial surfactant (Exosurf) during the acute phase of respiratory distress syndrome (RDS). The ratio of pulmonary artery acceleration time to the right ventricular ejection time (AT:RVET), measured from the Doppler wave form, was determined in 38 infants before the first dose of Exosurf, at one and six hours after the first dose, immediately before the second dose of Exosurf, and at one, six, 12, 36, and 60 hours subsequently. The median AT:RVET ratio corrected for heart rate (AT:RVET(c)) increased significantly an hour after administration of the first dose of Exosurf from 0.330 (0.273-0.410) to 0.380 (0.303-0.445) and similarly an hour after the second dose from 0.426 (0.252-0.495) to 0.440 (0.373-0.500). These changes occurred against a steady increase in the median AT:RVET(c) over the first 72 hours. It is concluded that the reduction in PAP correlates well with clinical parameters of disease severity and raises questions regarding the mode of action of Exosurf.

Acute Disease↗
PubMed1995

Gall bladder contractility in neonates: effects of parenteral and enteral feeding.

The gall bladder size was measured in 30 newborn infants: 18 had been fed parenterally and 12 enterally. The two groups were comparable for gestational age, birthweight, postnatal age and study weight. Exclusion criteria were haemodynamic instability, septicaemia, abdominal disease and opioid treatment. Gall bladder size was measured at 15 minute intervals for 90 minutes using real-time ultrasonography and the volume calculated using the ellipsoid method. Parenterally fed infants had further measurements at 120, 150, and 360 minutes. The gall bladder was significantly larger in parenterally fed infants than in enterally fed infants (p = 0.0001). In enterally fed infants a 50% reduction in gall bladder volume was observed 15 minutes after starting the feed with a return to baseline volume by 90 minutes. In parenterally fed infants there was no gall bladder contraction. Such information may give insight into the pathophysiology of hepato-biliary complications during parenteral nutrition in infants.

Enteral Nutrition↗
PubMed1995

Adrenocorticotropin receptor gene mutations in familial glucocorticoid deficiency: relationships with clinical features in four families.

Familial glucocorticoid deficiency is an autosomal recessive syndrome of adrenal unresponsiveness to ACTH characterized by glucocorticoid deficiency, high plasma ACTH levels, and a normal renin-aldosterone axis. Defects of the ACTH receptor have been suggested as a possible cause, and we have previously reported a number of novel mutations of the ACTH receptor gene in some, but not all, cases, suggesting that familial glucocorticoid deficiency may have a heterogeneous molecular etiology. Here we report the clinical features and ACTH receptor gene analysis in four patients from different families. We found that two patients were compound heterozygotes for the S74I and R128C mutations (patient A) and I44M and L192fs frame shift mutations (patient B). The other two patients (C and D) were of different ethnic ancestry, but were both homozygous for a R146H mutation. Segregation studies within families revealed heterozygosity in the parents and several other family members. Human CRH tests in the parents of patients A and B showed normal cortisol and ACTH responses in the S74I, R128C, and I44M heterozygotes and exaggerated cortisol and ACTH responses in the L192fs heterozygote, suggesting that the physiological ACTH increment induced in this test did not reveal evidence of subclinical ACTH resistance, and that this test may not be of value in ascertaining heterozygosity.

Adrenocorticotropic Hormone↗
PubMed1994

Is a low cerebrospinal fluid blood glucose ratio indicative of infection in patients with post haemorrhagic hydrocephalus?

Cerebrospinal fluid glucose and cerebrospinal fluid:blood glucose ratios were compared in seven patients with post haemorrhagic hydrocephalus having lumbar puncture/ventricular tap as a therapeutic measure. A control group of 10 babies was used, without intraventricular haemorrhage, and having lumbar puncture as part of a septic screen. Of 50 separate taps in the patient group, 38% had blood glucose measured and 76% had CSF glucose measured. Median cerebrospinal fluid glucose was 1.2 mmol (range, 0.4-2.5 mmol/l) in the patient group and 3.1 mmol/l (range, 1.4-10.3 mmol/l) in the control group. The median cerebrospinal fluid:blood glucose ratio in the patient group was 0.235 (range, 0.07-0.53) and in the control group was 0.709 (range, 0.6-1.4). Hypoglycorrhachia appears to be a normal finding in patients with post haemorrhagic hydrocephalus and does not indicate infection in these infants. Measurement of cerebrospinal fluid:blood glucose ratio is not warranted when cerebrospinal fluid is drained purely as a therapeutic measure in these patients.

Blood Glucose↗
PubMed1994

Bronchogenic cyst presenting in the neonatal period.

A term infant presented at birth with worsening respiratory distress which resulted in him requiring mechanical ventilation. A CT scan of the chest revealed a bronchogenic cyst, removal of which resulted in full recovery and, to date, no respiratory sequelae.

Bronchogenic Cyst↗
PubMed1994

Different subtypes of pseudohypoparathyroidism in the same family with an unusual psychiatric presentation of the index case.

A 13 year old Asian girl presenting with apparent hysterical paralysis and subsequent rapid cycling bipolar mood disorder was found to have biochemical evidence of pseudohypoparathyroidism type II. The mood disorder responded to treatment of the pseudohypoparathyroidism with a vitamin D analogue. Investigation of her parents and siblings showed phenotypes consistent with two distinct types of pseudohypoparathyroidism (type I and type II) in different family members.

Adolescent↗
PubMed1994

Osteopenia in cerebral palsy.

The bone mineral density of the lumbar spine was assessed in nine non-ambulant children with cerebral palsy combined with measurements of serum 25-hydroxyvitamin D, parathyroid hormone, and urinary calcium excretion. Three children with recurrent fractures received treatment with bisphosphonates for periods ranging from 12-18 months. All the children demonstrated a severe reduction in bone mineral density even when allowance was made for their body weight. There were no consistent abnormalities of vitamin D or parathyroid hormone status. Three children had gross hypercalciuria. Each of the children treated with bisphosphonates demonstrated an increment in bone density ranging from 20-40% with no apparent adverse effects.

25-Hydroxyvitamin D 2↗
PubMed1994

Neonatal abstinence syndrome after maternal methadone treatment.

Thirty two infants born to mothers taking methadone and 32 matched controls were studied to determine the incidence, timing, and frequency of persistence of withdrawal signs in infants born to maternal methadone users. Twelve (37%) infants received treatment for symptoms of withdrawal. The median length of time from birth to initiation of treatment was 23 hours (range 15-64 hours), the median length of treatment was six days (range 1-22 days). In the first six weeks after birth there was no significant difference in minor withdrawal symptoms and signs, general practitioner or accident and emergency visits between the group who did not receive treatment for withdrawal and the control group. If immediate treatment for withdrawal is not required major symptoms do not subsequently occur with great frequency. Late withdrawal signs were not seen in this study.

Birth Weight↗
PubMed1994

The relationship of pulmonary interstitial emphysema to subsequent type of chronic lung disease.

Chronic lung disease of prematurity (CLD) can be classified using the chest radiograph taken on day 28 into type 1 (homogeneous opacification) or type 2 (the presence of cystic change) and it has been suggested that the presence of pulmonary interstitial emphysema (PIE) leads to the development of type 2 CLD. To further study this relationship we examined the chest radiographs taken on days 1-7, 14, 21 and 28, of 202 infants treated at Liverpool Maternity Hospital between January 1980 and December 1989 who developed CLD. 39% (54/137) of infants who developed type 1 CLD had suffered from PIE compared with 78% (51/65) with type 2 CLD (p < 0.001). There was no difference in the incidence of PIE in infants who developed CLD and subsequently died compared with those who survived. In the infants who survived there was no significant difference in length of oxygen dependency between those who suffered from PIE (median 99 days oxygen dependent) and those who had no PIE (median 105 days). We conclude that the presence of PIE is associated with subsequent development of type 2 CLD but is not a prerequisite for this radiographic type of chronic lung disease. Of those infants who survive to develop CLD, the presence of PIE does not alter prognosis in terms of duration of oxygen dependency or mortality.

Chronic Disease↗
PubMed1993

Parathyroid hormone gene analysis in autosomal hypoparathyroidism using an intragenic tetranucleotide (AAAT)n polymorphism.

We have identified a polymorphic tetranucleotide consisting of (AAAT)n within the first intron of the parathyroid hormone (PTH) gene, and have used this to investigate the segregation of the PTH gene and idiopathic hypoparathyroidism in 7 affected and 21 unaffected members from three families. An association between the PTH locus and autosomal dominant idiopathic hypoparathyroidism in one family was excluded by observing recombination between the two loci. In the remaining two families with autosomal recessive idiopathic hypoparathyroidism, the PTH locus was not similarly excluded. We had previously demonstrated a donor splice site mutation of the PTH gene in one of these families, and PTH gene abnormalities were therefore sought in the second of these families. DNA sequence analysis of the three exons, together with 4 exon-intron boundaries and the promoter region of the PTH gene revealed no abnormalities, thereby indicating molecular pathology at another locus. Thus, our analysis of idiopathic hypoparathyroidism reveals genetic heterogeneity for this disorder. In addition, our identification of a microsatellite polymorphism of the PTH gene should help further segregation studies of this locus in families with parathyroid disorders.

Base Sequence↗
PubMed1993

Ureaplasma urealyticum and chronic lung disease.

Neonatal lower respiratory tract colonisation with mycoplasma organisms was examined for an association with chronic lung disease. Ureaplasma urealyticum colonised 9/70 (13%) infants less than 1500 g. Seven (78%) colonised and 33 (54%) non-colonised infants developed chronic lung disease. Logistic regression analyses revealed early gestation, but not mycoplasma colonisation, was independently associated with chronic lung disease.

Chronic Disease↗
PubMed1993

Smoking in diabetic teenagers.

The prevalence of smoking amongst a group of 77 diabetic teenagers aged 11-18 years attending two paediatric clinics was determined using a questionnaire and urine cotinine assay. Five individuals were identified as definite smokers with two further individuals being probable smokers, all of whom were aged 15 years or more. This low prevalence (9%) is in contrast to a 48% prevalence in a young adult diabetic clinic. Many diabetic teenagers appear to acquire a regular smoking habit after leaving the paediatric clinic so it is important that health education is targeted at this group.

Adolescent↗
PubMed1993

Randomised trial of routine versus selective paralysis during ventilation for neonatal respiratory distress syndrome.

The strategy of non-selective neuromuscular paralysis was compared with that of synchronised (fast rate) ventilation and selective paralysis in infants receiving mechanical ventilation for respiratory distress syndrome with chronic lung disease as the primary outcome measure. One hundred and ninety three infants weighing under 2000 g were randomly allocated to receive either pancuronium during mechanical ventilation in the acute phase of respiratory distress syndrome (non-selective group) or synchronised ventilation (initial ventilatory rate at or above that of the infant's) (selective group). Infants in the selective group received pancuronium if they were consistently expiring during the inspiratory phase of the ventilator cycle. There was no significant difference between the groups with respect to birth weight, gestation, and sex distribution. There was no significant difference between the group with respect to death (selective 19%, non-selective 16%), pneumothorax (selective 14%, non-selective 14%), chronic lung disease (selective 49%), non-selective 47%), and oxygen dependency at 36 weeks' postmenstrual age (selective 32%, non-selective 39%). Routine paralysis of ventilated infants has potential complications that may be avoided by using synchronised ventilation. As the latter is not associated with an increased incidence of long term respiratory complications, it is concluded that it is the optimum strategy of the two for ventilating infants with respiratory distress syndrome.

Chronic Disease↗