Biomedical subjects
Publications and source records attributed to D J Matthew.
Brain stem gliomas may present as gastro-oesophageal reflux. In such cases gastro-oesophageal reflux presents atypically late, and computed tomography may not detect the presence of the tumour until late in the course of the disease.
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Pulmonary embolism in childhood is a rare but under-diagnosed condition. We report four children aged 5 to 13 years presenting with pulmonary emboli, their primary diagnoses being craniopharyngioma, asthma, Crohn's Disease and Reye's syndrome. The diagnosis was supported by technetium micro aggregate lung perfusion scans in three of the children and in the fourth, the only child to die, by autopsy. Three of the children had markedly elevated plasma osmolalities, one as a result of his diabetes insipidus and two following hyperosmolar treatment for cerebral oedema. The child who died was found to have a femoral vein thrombosis but the sites of origin of emboli in the other children were not defined.
We report the case of 5-week-old male infant who presented as a 'near miss cot death'. He had the immunodeficient syndrome of defective neutrophil mobility and delayed umbilical cord separation. He was shown to have staphylococcal endocarditis with a large vegetation on the mitral valve, and acute obstruction of the mitral valve flow may have accounted for the suddenness of his presentation. Death resulted from overwhelming sepsis with widely disseminated micro-abscesses.
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.
We review a family in which a mother and two of her three children suffered a distinct syndrome of thoracic dystrophy with small chest volume, laryngeal stenosis, normal stature with variable asymmetry, asthenic build, and a small pelvis. In addition to the different inheritance pattern, this syndrome is distinguished from the better known Jeune syndrome by the differing thoracic and pelvic configuration.
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Allergy and chronic Pseudomonas aeruginosa (PA) infection are two factors that possibly affect the clinical severity of cystic fibrosis pulmonary disease, although the role of allergy is controversial. We have examined the effects of these factors on actuarial survival rates in 117 children with cystic fibrosis who were skin tested in 1974 and classified as allergic (A+) or nonallergic (A-) by their reactions to 12 prick tests with common environmental allergens. Patients were also classified according to whether or not they had chronic pulmonary infection with PA in 1974 (PA-positive or PA-negative). Survival rates in A+ patients were not significantly different from those in the A- group (percent survival to age 16 years, 67% vs 80%), whereas the PA+ group had significantly worse survival rates than the PA- group (percent survival to age 16 years, 53% vs 84%). There was no significant interaction between allergic skin reactions and either age at onset of chronic PA infection or subsequent duration of survival.
Presenting features and clinical manifestations of six patients with toxic shock syndrome are reported. In four of the six cutaneous injury, sometimes trivial, occurred before the onset of symptoms and may have been a causal factor. All six children recovered. The need for early recognition and intensive management in this life threatening condition is discussed.
Twenty-seven children aged between 4 and 13 years were given two injections of a split virus influenza vaccine (MFV-Ject, Institut Merieux) at a dose of 0.5 ml and an interval of 1 month. Each vaccination contained A/Philippines/2/82 (H3 N2) 10 micrograms HA, A/Brazil/11/78 (H1 N1) 10 micrograms HA, B/Singapore/222/79 15 micrograms HA. The overall serological response was good although the B group virus produced slightly less response; however, no children were seropositive to B prior to vaccination, reflecting a low previous exposure. Children aged over 10 years showed a generally higher serological response. Side-effects were minimal. A favourable reactogenicity/efficacy ratio was found.
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 preliminary studies suggest that the pathophysiology of the disease may involve release of proteolytic enzymes (such as trypsin) into the circulation, with destruction of the microcirculation.
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The minimal inhibitory concentration of azlocillin for Pseudomonas aeruginosa is appreciably reduced when combined with the mucolytic agent mesna (Mistabron) because of an independent bacteriostatic effect of mesna. Bactericidal activity of azlocillin is unaltered by mesna. Mesna inhalations alone or combined with azlocillin may benefit cystic fibrosis patients with pseudomonas lung infections.
Life tables were calculated for 273 British children with cystic fibrosis for the period 1974-9. There was a marked improvement in survival rates in the meconium ileus group compared with the 1969-73 data, but there was little improvement in patients presenting later with other symptoms.
There were 131 deaths among children aged between birth and 14 years during a period of 18 months in one inner London area health authority. One hundred children died in hospital, 23 at home, and 8 elsewhere. Thirty three died of congenital, 46 of perinatal, and 34 of other medical causes. There were 18 violent deaths--16 among children over 1 year of age. Medical and social information, collected from analysis of records and interview with those involved, was considered by a review panel and the following conclusions were drawn: there was scope for prevention of congenital disease or malformation in only one of 33 cases; with routine pathological investigation the cause of death in postperinatal infancy was usually unclear--more detailed pathological investigation should be routinely available for the investigation of deaths in the first year of life; there were possible management failures in four of 8 non-malignant medical deaths in older children; malignant disease was promptly diagnosed; fatal accidents were almost entirely restricted to the children of families under marked psychosocial stress; and there was some evidence of faulty communication between health agencies and of the inappropriate routing of emergency admissions.
Desquamative interstitial pneumonia was observed in two infants with the late-onset congenital rubella syndrome. In both infants this unusual lung disease was associated with circulating immunoglobulin M complexes and interstitial pulmonary deposits of IgM by immunofluorescence. Both infants had IgG deficiency. The first child recovered with a reduction in IgM complex levels and synthesis of rubella-specific IgG. The second infant died during the acute phase of his illness at which time there were high serum concentrations of IgM complexes and slightly increased levels of IgG complexes. Delayed maturation of the immune response in congenital rubella may predispose to persistent antigenemia and pulmonary deposition of rubella antigen-containing IgM complexes resulting in an acute form of interstitial pneumonia.
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 and confirmed the observed improvements in cyanotic episodes, heart failure, electrocardiograms, and arterial gas tensions. In a retrospective survey of 40 infants with PRS, failure to thrive was found to be significantly correlated with the severity of the airflow obstruction. This failure to thrive was reversed in the infants managed with NP tubes in comparison with an age-matched groups nursed while prone. The lack of significant complications with the NP airway and its acceptability to nursing staff, patients, and their parents suggest that this method deserves more widespread use in PRS and perhaps in other situations in which high upper respiratory tract obstruction is predominant.
Various bedding materials used in infancy, including duvets (or continental quilts), were tested for airflow using the British Standards Institution tests for pillows or fabrics. Resistance was also measured when the items were placed on a dummy infant face. Measurements were made on washed and unwashed garments, which were tested both dry and wet. Results suggest that all the bedding materials tested are safe for use even in the newborn period. The duvets produced slightly lower resistance to breathing than conventional blankets and sheets. In view of the wide variety of infant bedding fabrics it seems desirable for standard airflow performance requirements to be introduced.