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Many promising treatments for epidermolysis bullosa have turned out to be disappointing. In Chacago, Illinois, USA (Archives of Dermatology1999;135:997−8 [letter];Ibid: 981−2 [editorial]) a man with epidermolysis bullosa simplex noticed that his lesions improved when he was given tetracycline for concurrent acne. The response was dose dependent with an optimal dose of 500 mg three times daily. His father had the same disease and responded similarly to tetracycline. A randomised, controlled trial seems indicated.
As people with Down's syndrome live longer they are more likely to need treatment for “adult” diseases. Avascular necrosis and acetabular dysplasia, alone or combined, may cause severe and early hip disease. Surgeons in Oregon, USA (Journal of Bone and Joint Surgery (British edition) 1999;81-B:436–9) have reported nine hip replacement operations (three bilateral) on six patients with Down's syndrome aged 22–47. All did well with relief of pain and full hip function.
Mortality in severely malnourished children with diarrhoea at a centre in Dhaka, Bangladesh was running at around 15%. After the introduction of a management protocol (Lancet1999;353:1919–22) the mortality almost halved. Key features of the protocol include slow oral rehydration, immediate feeding with a defined diet of inexpensive local foods, broad spectrum antibiotics, micronutrient supplements, and management of complications. A similar, but not identical, protocol has been developed by the World Health Organisation. Management of severe malnutrition: a manual for physicians and other senior health workers. Geneva: WHO, 1999).
Two studies, one in Japan on 8–16 year olds (Thorax1999;54:196–201) and one in Brazil on 9–12 year olds (Ibid: 202–6) showed that exercise training (cycle ergometer or swimming) makes children with asthma fitter (improved aerobic capacity). The Brazilian children needed less inhaled or oral steroid after their exercise training. The children who were least fit at the start of the study benefited most from training.
The Thousand Family Study included all children born in Newcastle upon Tyne, UK in May and June 1947. A selected sample of 262 of these people were interviewed when they were 32 years old (British Journal of Psychiatry1999;174:112–20) and 27 of them were found to have had major depressive disorder in the past year. Women who at the age of 5 years had lived in families with multiple disadvantages (marital, financial, poor parental health, overcrowding, poor mothering) had a fourfold increase in risk of depression at age 32 and for men the increase in risk was ninefold. Poor mothering and poor care seemed to be risk factors particularly in girls.
In Kingston, Jamaica (Journal of Pediatrics1999;134:304–9) 130 patients with sickle cell disease underwent splenectomy over a 22 year period: 46 for recurrent splenic sequestration and 84 for chronic hypersplenism. They were no more likely than non-splenectomised controls with sickle cell disease to die or to suffer from septicaemia during the study period. The splenectomy group were more likely to have been given pneumococcal and Haemophilus influenzae vaccines and penicillin prophylaxis. Painful crises and acute chest syndrome were more common in splenectomised patients, and acute chest syndrome was more common when the splenectomy had been done for recurrent sequestration rather than for hypersplenism.
The common assumption that children who experience the death of a parent or sibling are at risk of emotional or psychiatric disorder and need professional help has been questioned (Journal of the Royal Society of Medicine1999;92:230–3). Most children are very resilient, few develop psychiatric symptoms or dysfunctional behaviour, and childhood bereavement is not strongly linked to depression as an adult. It is questionable whether children need to go through a well defined grief process, and bereavement counselling might not be completely harmless, but certain vulnerable children probably do need professional help.
At a large paediatric ophthalmology practice in California (British Journal of Ophthalmology1999;83:670–5) 2–4% of patients had cortical visual impairment. In addition to their neurological deficits 65% of these children had ophthalmic problems, which included convergent (19%) or divergent (18%) squint, optic nerve atrophy (16%), ocular motor apraxia (15%), nystagmus (11%), and retinal disease (3%). Sixty per cent of patients who returned for follow up has some visual improvement.
When she read the title “Hedgehog hives” (Archives of Dermatology1999;135:561–3) Lucina struggled with the thought of diagnosing urticaria in a hedgehog. But she need not have worried; the patients were an 11 year old boy and his mother who developed skin reactions after adopting one of the animals as a pet.
Rickets is still common in Africa and other parts of the disadvantaged world but studies in African children with rickets have shown normal serum levels of vitamin D. Now a study in Nigeria (New England Journal of Medicine1999;341:563–8) has shown the importance of including calcium in the treatment. Children treated with calcium did as well as those given both calcium and vitamin D and better than those given vitamin D alone. Healing by 24 weeks was achieved in 60% of the calcium alone and calcium plus vitamin D groups and 20% of the vitamin D alone group. Dietary calcium intake was low in children with or without rickets.
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