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Biomedical subjects

R Dinwiddie

Publications and source records attributed to R Dinwiddie.

At least 55 records · Page 3Linked to original sources

Oxygen saturation in cystic fibrosis.

The availability of non-invasive oxygen saturation (SaO2) measurement could prove to be a useful tool for following up the progress of patients with cystic fibrosis. The present study was undertaken to compare its use with other routine measurements in the clinic. A total of 100 patients with cystic fibrosis were compared with 50 patients with stable asthma. The children were aged between 5 and 16 years and were studied as outpatients. A positive correlation was found between forced expiratory volume in one second and SaO2 in the group with cystic fibrosis and in the comparison group. Median SaO2 in those with cystic fibrosis (94.0%) was significantly lower than in controls (97.0%). Significant correlations were also found in the patients with cystic fibrosis for the following: Shwachman-Kulczycki score, higher weight centiles, and chronic pseudomonas lung infection with or without staphylococci. An inverse correlation was found with the Chrispin-Norman chest x ray score. SaO2 measurements are useful in the outpatient assessment of patients with cystic fibrosis and compare well with other standard tests of disease severity.

Adolescent↗

Successful surgical treatment of tracheal compression following right pneumonectomy in infancy. Long-term follow-up.

Two patients (aged 10 and 16 months) underwent right pneumonectomy: one for an oesophageal bronchus, the other for a hypoplastic right lung with sequestration. Both patients subsequently developed severe stridor. The heart rotated to the right and the aortic arch compressed the trachea. This unusual vascular compression was treated by a bypass graft between the ascending and descending aorta and division of the aortic arch between the left carotid and left subclavian artery at the age of 5 years and 4 months, and 20 months respectively. The postoperative recovery was uneventful in the first patient. The second patient required 7 weeks of ventilatory support. Both patients are well (14 and 7 years after surgery). Cardiac catheterisation and angiography in the first patient and Doppler echocardiography in the second demonstrated unrestricted flow through the graft into the descending aorta. Pulmonary function studies showed a restrictive defect with some obstruction in both children. Despite this finding, both patients enjoy a normal life with some sporting activities. The long-term results suggest the effectiveness of this procedure in the treatment of this rare but severe complication of pneumonectomy in infancy.

Aorta↗

Pseudo-Bartter's syndrome in cystic fibrosis.

Seven cases of cystic fibrosis complicated by chronic salt depletion and failure to thrive were studied. After replacement of the salt deficit, the metabolic abnormalities resolved, and weight gain was rapid. This should be considered as a differential diagnosis in children who have been diagnosed as having cystic fibrosis, but who fail to thrive despite standard treatment.

Alkalosis↗

Hemorrhagic shock and encephalopathy: clinical, pathologic, and biochemical features.

To further define the clinical, pathologic, and biochemical features of hemorrhagic shock and encephalopathy syndrome, we studied 25 affected children (aged 3 months to 14 years) admitted to a single center between 1982 and 1985. A prodromal illness comprising vomiting, diarrhea, listlessness, and fever was present in 84% of the cases. Acute onset of shock, convulsions and coma, bleeding (or laboratory evidence of disseminated intravascular coagulation), elevated plasma activity of hepatic enzymes, acidosis, and impaired renal function was present in every case. Twenty patients died, and all the survivors are neurologically damaged. At postmortem examination, intravascular microthrombi coexisting with hemorrhages and petechiae were found in most organs. Centrilobular liver necrosis and cerebral edema were prominent features. No microbiologic cause for the disorder was identified, but decreased plasma levels of the protease inhibitors alpha 1-antitrypsin and alpha 2-macroglobulin, together with increased levels of circulating proteolytic enzymes, were frequently present. An overrepresentation of the uncommon variant phenotypes of alpha 1-antitrypsin was found in first-degree relatives of affected patients (four had the MZ phenotype, and one each the MS or MC phenotype, of 19 relatives studied). Abnormal accumulation of alpha 1-antitrypsin was detected immunohistochemically in the livers of six of the patients. Defective protease inhibitor production or release may be involved in the pathogenesis of the disorder.

Adolescent↗

Monitoring in non-traumatic coma. Part I: Invasive intracranial measurements.

The arterial blood pressure, intracranial pressure, and organ system failure scores were reviewed for 49 infants and children with non-traumatic coma from various causes. The neurological outcome was good in 21 patients, moderate in five, and poor in 23. There was no significant difference in maximum intracranial pressures between patients with a good outcome and those with a poor one, but patients with a poor outcome had significantly lower minimum cerebral perfusion pressures. During the period of admission 18 patients had cardiovascular failure, none had renal failure, and two developed severe coagulopathy. Seventeen of the 19 patients in whom at least one of these systems failed died. Our findings emphasise the diversity of illnesses associated with raised intracranial pressure in children and the number who develop multiple organ failure, and the values and limitations of using minimum cerebral perfusion pressure and the organ system failure scores as guides to severity of illness and prognosis.

Acute Disease↗

Recognition and early management of Reye's syndrome.

Reye's syndrome continues to be associated with a high mortality. Out of 12 cases treated on our intensive care unit over a four year period, seven died, one suffered minimal cerebral damage, and four were normal. Rapid progression through coma stages and high peak ammonia concentrations worsened prognosis. Reye's syndrome was suspected in only 50% of cases at the referring hospital and resulted in prompt referral to the intensive care unit in only one case. Late referral to the unit was associated with a poor outcome. Sudden neurological deterioration followed diagnostic lumbar puncture in six children. Papilloedema was an unreliable sign of raised intracranial pressure and was absent in all cases. While computed tomography of the brain was useful in providing additional evidence of raised intracranial pressure, this could only be confirmed by direct measurement. Lumbar puncture in the presence of rapidly progressive coma should be deferred until raised intracranial pressure has been excluded. To this end, early admission to a paediatric intensive care unit with facilities for computed tomography and monitoring of intracranial pressure is recommended.

Child↗

The role of the laboratory in the diagnosis of cystic fibrosis.

Laboratory investigations used for detecting cystic fibrosis have been critically reviewed. The sweat test is still the only investigation that can be used for detecting the disease at any age. Trypsin in blood or serum might have the same discriminatory power during the first three months of life and could be used for neonatal screening. There is no immediate prospect for prenatal diagnosis or heterozygote detection. The change of the pattern of plasma proteins in cystic fibrosis is the same as in other chronic disorders with varying clinical intensity. Albumin and alpha-1-antitrypsin may be used to monitor treatment. The plasma concentration of alpha-2-macroglobulin is considerably decreased in many younger patients.

Adolescent↗

Pulmonary artery sling in identical twins--report of two cases.

Pulmonary artery sling was successfully treated by division and reimplantation of the left pulmonary artery in identical twins. Long-term patency of the reimplanted pulmonary artery in both twins as well as in 4 other operated children was confirmed by lung perfusion scan. Early operative treatment of pulmonary artery sling is advocated.

Diseases in Twins↗