The Castang Foundation, Bath Unit for Research in Paediatrics, National Institute of Health Research, the Royal United Hospitals Bath NHS Foundation Trust, BRONNER-BENDER Stiftung/Gernsbach, University Children's Hospital Zurich.
The growth in public and professional awareness of the sexual abuse of children has led to an increase in the number of such cases being presented to paediatricians.' 2 About a third of sexually abused children present with evidence of physical trauma,3 and the possibility of sexual abuse should be considered in all children who present with unexplained genital injuries. Erroneous investigation of sexual abuse is disturbing, however, for the child and family, particularly close male relatives, who are the most likely perpetrators,24 and medical staff should learn to recognise conditions that may mimic sexual abuse and thus avoid false accusations.We report on two girls suffering from lichen sclerosus et atrophicus who were thought to have been sexually abused.Case reports CASE 1 A 4½12 year old girl was referred to a paediatrician with vulval purpura. Haemorrhagic blistering, sometimes with pain, had been noted over the preceding six months. The only medical history was of vulvovaginal warts two years previously. The possibility of sexual interference was suggested to the parents, but this aroused hostility and a solicitor's demand for an apology.At initial presentation the predominant feature was purpura, which affected much of the vulva (fig 1). The patient was admitted to hospital and was seen by a dermatologist, who noted that the vulval skin and labial mucosa were white and wrinkled as well as being purpuric. During an examination under anaesthesia, which showed an intact hymen, a biopsy specimen was taken that confirmed the clinical diagnosis of lichen sclerosus et atrophicus. CASE 2A 51/2 year old girl was referred to a paediatrician because of a bruise over her pubic symphysis, an old bruise on her thigh, and excoriated, swollen labia minora with a purulent, bloodstained discharge but an intact hymen (fig 2). Medical attention had been sought about six months previously for a vulval injury that she had apparently incurred by falling off a bicycle. Neither parent was able to suggest how these lesions had originated, although she had been playing with some boys in a nearby wood. The patient herself volunteered no explanation.Vaginal swabs taken on admission grew group B haemolytic streptococci Bristol Royal Infirmary, Bristol BS2 8HW SUSAN E HANDFIELD-JONES, MRCP, registrar in dermatology
SUMMARY A 4 month old boy presented with respiratory difficulty and hypotonia. Clostridium botulinum and its toxin were isolated from his faeces and he had electromyographic changes typical of infantile botulism. This is only the second case in the United Kingdom: unfamiliarity with the presentation could result in misdiagnosis.Infantile botulism results from the production of Clostridium botulinum toxin in the infant gut. It was first recognised in 19761 and more than 600 cases have been reported since, most of them from North America. Only one previous case has been reported in the United Kingdom, which was caused by C botulinum type A.2 We describe a second case, and only the third ever reported caused by C botulinum producing toxins B and F. Case reportThe patient was a boy aged 4 months. He was born at full term after a normal pregnancy, was breast fed from birth, and growth and development were normal. He was given his first doses of diphtheria, tetanus, pertussis, and oral poliomyelitis vaccines three weeks before admission to hospital. Baby rice and rusks were added to his diet about one week later because of 'constipation' attributed by his parents to underfeeding, and for the week before admission he had had profuse rhinorrhoea.Twenty four hours before admission he had difficulty feeding, and was less active than normal. By the next day he was 'floppy' and in respiratory distress, and on admission there was profound generalised hypotonia with bilateral ptosis, impassive facies, reduced tendon reflexes, a poor gag reflex, and pharyngeal pooling. Spontaneous movements were few but he seemed visually alert and showed definite withdrawal from pain. Physical examination otherwise yielded normal results.Haematological measurements were unremarkable, and normal biochemical results included blood urea, electrolytes, creatinine, glucose, and lead concentrations, and normal aspartate transaminase activity. Urinary An electroencephalogram on admission showed generalised excess high amplitude slow wave activity. This non-specific abnormality was rather more pronounced a week later, but thereafter returned to normal.The patient was initially treated with netilmicin and ampicillin; subsequent management was supportive. Two days after admission (day four of the illness) intermittent positive pressure ventilation was required. Although he retained some respiratory movements throughout his illness he showed profound hypotonia, losing pupillary reflexes for several days.The child began to show definite improvement by day 18 and was extubated on day 24. Improvement was then rapid, his air of alertness progressing in advance of his muscular strength. He was discharged on day 45, able to feed from a bottle. He still had considerable hypotonia but was responsive, babbled, and was able to grasp toys. This improvement has continued.Faecal specimens were sent to
To investigate the association between the family environment and metabolic control of childhood diabetes 20 diabetic children aged between 4 and 12 years with same-sex healthy siblings and two cohabiting parents were selected. The parents were interviewed separately and completed questionnaires about their own and their spouse's involvement and shared activities with both children, diabetic management, and knowledge of and attitudes towards diabetes. They also completed the Moos Family Environment Scale (FES) which yields ten sub-scales relating to different aspects of family functioning as did 20 sets of parents with age- and sex-matched healthy children, who also completed questionnaires concerning their involvement and shared activities with their children. There were no differences between the families of diabetics and of controls in the extent to which either parent was involved with their children or in the attention each child received. Fathers of diabetic children differed from controls in their use of punishment and diabetic families were less achievement oriented. The degree of metabolic control (HbA1 assessment at three-monthly intervals) was related to parental involvement with the non-diabetic child and to some aspects of the FES including parental incongruence. These differences in family functioning suggest useful directions for possible intervention programmes.
Vitamin D-dependent rickets type II (VDDR II) is a rare syndrome resulting in severe rickets and is resistant to treatment with vitamin D and its derivatives. Patient with this disease, who are frequently the children of consanguinous marriages, present with elevated circulating concentrations of 1,25-dihydroxy vitamin D, the active metabolite of vitamin D, and in vitro studies have indicated a failure of intracellular binding of the hormone. Alopecia has been noted in many of these patients and it has been suggested that this feature may indicate a more marked resistance to treatment. However we describe a 3-year-old boy with this disease who, although having normal hair growth, displayed extreme resistance to treatment with active vitamin D metabolites. In vitro studies of skin fibroblasts disclosed not only an absence of hormone binding or 1,25(OH)2D3-induced 24-hydroxylase activity but reduced metabolism of 1,25(OH)2D3 itself. In this child, treatment with exogenous 1,25-dihydroxy vitamin D3 at doses of up to 24 micrograms/day, which increased the circulating concentration of the metabolite to greater than 100 times the normal adult mean, failed to alleviate his condition and he died at the age of 39 months. This would therefore suggest that absence of alopecia, in this condition, cannot be regarded as a constant predictive sign of a lesser resistance and of responsiveness to Vitamin D treatment.
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