Suffocation, choking, and strangulation in childhood in England and Wales: epidemiology and prevention

The causes, classification, and prevention of mechanical asphyxial death in children were examined. The Office of Population Censuses and Surveys (OPCS) identified children, under 15 years of age, who had died as a result of choking, suffocation, or strangulation in England and Wales during the years 1990 and 1991. Cases in the International Classification of Diseases (ICD) codes of E911-3, E953, E963, and E983 were selected and case details from HM coroners' records and the death certificates were extracted. The OPCS identified 136 children (99 boys, 37 girls) in the two year period, 65% were under 3 years of age. The children were classified as dying from choking (21 cases), aspirating gastric contents (39 cases), suffocation (29 cases), strangulation (11 cases), and hanging (36 cases). The strangulation cases could be further subdivided into a group of 12 younger children who were suspended from ligatures around the home and a group of 21 boys (8-14 years) who died of self initiated hanging. Overall, 11 children were deliberately killed and 31 children died in beds or cots. Children whose deaths are classified as being due to aspiration of vomit appear to be cases of the sudden infant death syndrome or background medical conditions. This study suggests the need for advice on maintaining a safe sleeping environment. Only one child choked on a toy and European Standards for Toy Safety appear to have been successful. The prevention of hanging in the group of older boys needs further exploration.     

Infantile osteopetrosis; bone marrow transplantation from a cousin donor

The successful correction of infantile osteopetrosis in an Asian child by bone marrow transplantation (BMT) from an HLA-A,B matched cousin donor is reported. Retrospective HLA molecular analysis revealed that patient and donor were incompatible for HLA-DPB1. Donor type cells detected in the patient after transplantation indicate successful engraftment. The patient is currently alive and well.     

Severe microcephaly with normal intellectual development: the Nijmegen breakage syndrome

A brother and sister are described with severe microcephaly of prenatal onset, normal intellectual and motor development, chromosomal breakage and cellular immunodeficiency, which is characteristic of the autosomal recessive condition, Nijmegen breakage syndrome. The proband was a girl who presented at 15 months, with normal developmental milestones and an extremely small head circumference of 36 cm. Twenty per cent of her lymphocytes showed spontaneous translocations involving chromosome 7p13, 7q35, 14q11, and 14q32. The lymphocytes also showed excessive x ray induced chromosome damage. She had T cell lymphopenia, but normal immunoglobulins, and a normal alpha fetoprotein. A brother was born shortly after her diagnosis was made. He also had extreme microcephaly of 28 cm, with similar spontaneous and x ray induced chromosomal breakage, and T cell lymphopenia. Neither child has clinical evidence of immunodeficiency. To test the hypothesis that Nijmegen breakage syndrome and ataxia telangiectasia are allelic disorders, haplotype analysis was carried out in the family using DNA markers spanning the AT locus on chromosome 11q22. The affected boy had a different haplotype from his affected sister. Thus in this family, the Nijmegen breakage syndrome is not allelic to the ataxia telangiectasia locus on chromosome 11q, and the two conditions are genetically distinct. The normal intellect in these children raises questions about normal brain development in the presence of severe microcephaly.     

Hypothyroidism secondary to topical iodine treatment in infants with spina bifida

Four infants with spina bifida, who had not undergone surgical closure of a lumbar myelomeningocele, were assessed and investigated for hypothyroidism. From birth, all were treated once daily with an iodine-containing ointment (Betadine) as a local antiseptic applied to the spina defect. All infants showed excess urinary iodine concentration. Two infants, without clinical evidence of hypothyroidism or goitre, showed low serum free thyroxine and high thyroid stimulating hormone concentrations at a mean age of four weeks and were started on thyroxine replacement treatment. Betadine ointment and thyroxine were stopped simultaneously at a mean age of nine months, following which all infants remained euthyroid. Thyroid function tests should be monitored routinely if iodine is applied as a topical antiseptic to infants.     

Variable outcome of a congenital cytomegalovirus infection in a quadruplet after primary infection of the mother during pregnancy

A congenital cytomegalovirus (CMV) infection in a quadruplet has not been reported previously in the literature. We describe a congenital CMV infection in a quadruplet after primary CMV infection of the mother during pregnancy. One infant died antenatally, while another had cholestatic jaundice at birth and died of liver failure at three months of age. Of the two surviving infants, one showed no signs or symptoms of congenital CMV infection at 18 months of age, whereas the other had hearing loss and delayed development. CMV cultures of urine were positive at six weeks of age in all three infants born alive. The diagnosis of congenital CMV infection after primary CMV infection of the mother during pregnancy was made retrospectively by detection of CMV-immediate early antigen in three placentas as well as by examination of serum obtained from the mother during pregnancy.     

The insulin-like growth factor I generation test in the investigation of short stature

Genotypic and phenotypic heterogeneity in patients with growth hormone (GH) insensitivity syndrome suggests that partial defects exist in the GH receptor. The insulin-like growth factor I (IGF-I) generation test was assessed as a means of identifying partial GH receptor defects in a heterogeneous group of 22 prepubertal children with short stature. In a subgroup of nine patients with peak GH levels of 63.7 ± 3.7 mU/l during a glucagon tolerance test, the response to the IGF-I generation test was no different from that for the group as a whole (peak GH, 43.3 ± 4.5 mU/l), despite the fact that this subgroup exhibited a negative relationship between height SDS and peak GH and a positive relationship between height SDS and IGF binding protein-3. This preliminary study therefore suggests that the IGF-I generation test in its present form will not be useful as a primary screening test for partial GH insensitivity. Despite this, the IGF-I generation test has been extremely useful in the confirmation of the diagnosis of GHIS and may therefore also prove useful in the confirmation of partial defects in the GH receptor. A subgroup of short children with peak GH levels above 40 mU/l had some characteristics of partial GH receptor deficiency. These children, to whom GH therapy would not normally be given, may respond better to recombinant human IGF-I.     

Accidents and child abuse in bathtub submersions

Non-accidental bath drowning is an infrequently reported form of child abuse. Details of 44 children who suffered from drowning or near drowning in the bath were analysed from a two year (1988-9) UK study to investigate factors that might point to abuse. Cases of near drowning were notified through the British Paediatric Surveillance Unit inquiry system and drowning cases from the Office of Population Censuses and Surveys, the Scottish Government Record Office, and the Northern Ireland Office. In 28 cases the story was of accidental submersion with a baby of modal age 9 months being left unsupervised in a bath. Two other neonates briefly slipped from the parents arms while having a bath. Four bathtub drownings were related to epilepsy. In contrast 10 cases (six drowning and four near drowning) had stories very suggestive of abuse with inconsistent histories, previous history of abuse, and late presentation for medical care. A diagnosis of abuse should be considered in the differential diagnosis of atypical bathtub immersions in the absence of epilepsy and developmental delay.     

Pure thoracolumbar facet dislocation: clinical features and CT appearance

The use of computed tomography (CT) in demonstrating pure dislocations of the thoracolumbar facets and in predicting the prognosis of this injury was evaluated and compared with radiography retrospectively. The records of 29 patients with pure thoracolumbar bilateral facet dislocation who were admitted to the trauma unit over a 4-year period were reviewed. Twenty-two patients (76%) had a complete neurologic loss that remained complete following immediate surgical stabilization; five (17%) had an incomplete neurologic loss, and two (7%) were normal neurologically. Plain radiographs of the spine, including anteroposterior and lateral views, documented the level and type of fracture but failed to depict the full extent of bony ad soft-tissue injuries. CT provided essential additional information, particularly regarding the status of the posterior elements of the vertebrae and the adequacy of the spinal canal. Pure thoracolumbar facet dislocations have a characteristic appearance on axial CT scans. Sagittal reformation through CT is essential in the evaluation of this type of spinal injury.     

Long-term assessment of children exposed in utero to carbimazole

Twenty-five children aged 3 to 13 years who had been exposed in utero to carbimazole were assessed physically and psychologically to evaluate the long-term effects of the drug on growth and development. 2 children had congenital malformations but all had normal pituitary-thyroid function and appeared to have grown and developed normally.