Daniel J Matthew

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21 patients (10 male, 11 female) aged between 11 months and 29 years with Shwachman's syndrome are reviewed. All patients had exocrine pancreatic insufficiency. Haematological features included neutropenia in 19 (95%), anaemia in 10 (50%), and thrombocytopenia in 14 (70%); one patient developed erythroleukaemia. Severe infections occurred in 17 (85%) from(More)
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,(More)
Recent studies of Guillain-Barré syndrome in adults have shown that the requirement for assisted ventilation correlates closely with a poor outcome, and the need for ventilation is now being used as an indication for plasmapheresis. As comparable studies in children have not been performed we reviewed our experience of patients who had Guillain-Barré(More)
In the past year, ten infants have been admitted to hospital with a new or previously unrecognised disorder, characterised by an acute onset of encephalopathy, fever, shock, watery diarrhoea, severe disseminated intravascular coagulation, and renal and hepatic dysfunction. Seven of the infants died. No specific causative agent has been identified, but(More)
In a retrospective study, the authors evaluated the initial and follow-up radiographs and ventilation perfusion (V/Q) scans of 12 boys and girls with lobar emphysema who were treated conservatively and were followed up for a median of 3 years (range, 6 months to 12 years). All 12 boys and girls underwent anteroposterior and lateral chest radiography at(More)
Chestradiographic scores and respiratory function on 80 sets of results from 50 patients with Cystic Fibrosis were analysed. Chest radiographic scores were assessed independently using the method of Chrispin and Norman. Respiratory function tests were found to correlate well with the chest radiographic score, the best correlation being with the forced(More)
Nasopharyngeal airways have been assessed in the management of infants with severe Pierre Robin syndrome. In 12 such infants the positioning and subsequent maintenance of these tubes were found to be important in ensuring adequate relief of the airway obstruction. In five infants measurements of lung mechanics demonstrated the benefits of NP tube placement(More)
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(More)
Children with growth hormone deficiency can now be treated with biosynthetic human growth hormone. Before treatment is started the pituitary function of the child should be investigated. This is usually done with a pharmacological test of growth hormone secretion,' the commonest test being insulin induced hypoglycaemia. The results of these tests may,(More)
Four patients with generalized lymphangiomatosis presenting with chylothoraces are described. All four had bone involvement, two had involvement of the spleen, and one of the pericardium. The diagnosis was confirmed by typical radiology, histology, and in three patients by immunohistochemistry. Treatment was mainly palliative. Three patients died within 1/2(More)