Biomedical subjects
J Lorber
Publications and source records attributed to J Lorber.
Ventricular size and intelligence in achondroplasia.
Of 10 children with macrocephaly in association with achondroplasia only two children had progressive hydrocephalus. They were treated with a shunt procedure. One of these is intellectually and neurologically normal, and one is wheelchair bound and mentally retarded, with a spastic quadraplegia. Three children showed mild dilatation of the ventricles and one of these died from severe constriction at the foramen magnum. The other two are neurologically normal; one is intellectually normal but below average (WISC 88) and the other is slow normal at 18 months of age. Four children with demonstrated megalencephaly and a fifth with probable megalencephaly are all intellectually and neurologically normal. Intelligence in achondroplasia appears to be mainly in the normal range.
When is a shunt no longer necessary? An investigation of 300 patients with hydrocephalus and myelomeningocele: 11-22 year follow up.
Three hundred consecutive patients treated with a shunt for hydrocephalus associated with open spina bifida were followed for a period ranging from 11 to 22 years with a view to answering the question: How long is a shunt necessary? At most, in 51 patients (17%) the shunt was no longer functioning and was not causing any trouble. It is possible that many of these will still develop problems of raised intracranial pressure and their shunt would have to be revised, or reinserted. In fact, this occurred in 14 patients who had a shunt without revision for 10 to 15 years before trouble started and the shunt had to be revised. It is concluded that it is unsafe to advise parents and patients that a shunt is no longer necessary, simply because a long time has elapsed without revision or because the shunt may be very short on X-ray or had been disconnected, or even removed. Nevertheless, there is a large proportion of individuals who, after a lapse of 10 years without revision, may well never require further procedures.
Children with large heads: a practical approach to diagnosis in 557 children, with special reference to 109 children with megalencephaly.
Among 557 children who presented a diagnostic problem of a large head, 109 had megalencephaly as the primary diagnosis. A clinical approach to the differentiation of this numerically important group from the various other causes of large head is outlined. The group is characterised by a familial incidence of large head in at least 50 per cent of cases; a male to female preponderance of four to one; an above-normal rate of head growth in 80 per cent of the children in the first four months after birth, and in a further 12 per cent in late infancy. The vast majority of these children were normal. Only seven children were retarded, and they also had a variety of neurological and other somatic abnormalities.
Antibiotic treatment of Haemophilus influenzae type B meningitis: the problem of bacterial resistance.
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Results of selective treatment of spina bifida cystica.
The results of selective treatment in 120 infants with open spina bifida, admitted between May 1971 and December 1976, were prospectively studied. Seventy-one infants had adverse criteria at birth and were not treated. They all died, more than 90% of them within 6 months of birth. Seven had meningocele. All were treated and survived without handicap. Forty-two infants with myelomeningocele were actively treated. Thirty-six survive at follow-up after 3 to 9 years. The quality of survival is much better than when selection was not used but 8 children have moderate or severe handicaps. The parents were fully informed and consulted at every decision-making step; they fully supported the principle of selection and the action taken on behalf of their own child.
The limits of sponsorship for women physicians.
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Can recurrent febrile convulsions be prevented?
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Perinatal practice and compensation for handicap.
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Lumbar puncture and febrile convulsions.
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[Reasons for and results of selective treatment of myelomeningocele].
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Lumbar puncture in children with convulsions associated with fever.
452 previously healthy children aged over six months and under six years were admitted to the Sheffield Children's Hospital between January, 1972, and December, 1976, after their first convulsion associated with fever. 304 of the children had a lumbar puncture. 25 punctures were necessary according to defined clinical criteria--including those in 15 children who were subsequently shown to have meningitis. All 148 children who did not have a lumbar puncture made a prompt and uneventful recovery. Children with convulsions associated with fever should be referred to hospital. A lumbar puncture should be performed when meningitis is suspected, after examination by a senior and experienced member of the staff. It should not be carried out as a routine procedure.
Nonaccidental poisoning: the elusive diagnosis.
Although nonaccidental poisoning in childhood is now more often recognised, it is still difficult to establish a diagnosis despite correct investigative procedures. In 1978 we were unable, initially, to establish the cause of intermittent episodes of loss of consciousness in a boy admitted to Sheffield Children's Hospital. Subsequently it was conclusively shown that his mother systematically poisoned him with Tuinal (amylobarbitone and quinalbarbitone) both before admission and while he was being treated in the hospital.
Acute infantile thrombocytosis and vitamin K deficiency associated with intracranial haemorrhage.
A 5-week-old breast-fed girl presented unconscious and convulsing with heavily blood-stained CSF under increased pressure. This was associated with oozing from venepuncture sites, a grossly prolonged prothrombin time, and a raised platelet count. The prothrombin time became normal 18 hours after she had been given parenteral vitamin K and a transfusion of fresh plasma and red cells, but the thrombocytosis persisted. A moderate communicating hydrocephalus also remained and was treated, and one year later health and development appeared normal. The thrombocytosis resolved after a few weeks and has not recurred.
The prognosis of occipital encephalocele.
147 cases of occipital cranium bifidum have been followed: 32 had meningoceles and 115 encephaloceles. Females predominated (69%). 90% of the meningocele and 43% of the encephalocele patients survived. Of the former 48% are normal and 16% are both physically and mentally handicapped. Of the latter only 4% are normal and 26% are physically and mentally handicapped. Cerebral tissue in the sac and associated microcephaly are bad prognostic features.
Spina bifida: to treat or not to treat?
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Spina bifida: to treat or not to treat? Selection - the best policy available.
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