Recurrent cyanotic attacks due to unilateral upper airway obstruction in infants.

Two infants presented with recurrent brief cyanotic attacks and had unilateral nasal discharge. Both were poor feeders. The triad--transient cyanotic attacks, poor feeding and unilateral nasal discharge--points to unilateral upper airway obstruction. When the obstruction was relieved, the symptoms disappeared.

BCG, tuberculin skin-test results and asthma prevalence in school children in North London.

OBJECTIVE: To test whether there is any relationship between asthma prevalence and BCG immunization or tuberculin skin text reaction. DESIGN: Cross-sectional survey. SETTING: Secondary school in Haringey, North London, U.K. SUBJECTS: 780 children aged 11-18 years (median 13.35 years). INTERVENTIONS: Administration of tuberculin skin text and questionnaire. MAIN OUTCOME MEASURES: Diagnosis of asthma, presence of nocturnal cough, exercise-induced wheeze or wheeze with viral respiratory infections; diameter of induration with tuberculin skin text; history of BCG immunization. RESULTS: 57 of 629 children (8.5%) had a significantly positive Mantoux reaction (>or=15 millimeters of induration). Children with and without a history of BCG immunization did not differ significantly in prevalence of asthma diagnosis (11.8% vs 14.1%, p > 0.6), exercise-induced wheeze (16.9% vs 21.2%, p>0.4), viral induced wheeze (15.4% vs 7%, p>0.6) or nocturnal cough (32.3% vs 32.7%, p> 0.6). We also found no significant correlation of the prevalence of asthma diagnosis or symptoms with diameter of Mantoux test reaction. CONCLUSION: There is no evidence of an effect of BCG immunization or tuberculin reactivity on the incidence of asthma in secondary school children in Haringey, North London and the exposure to tuberculosis is high in these children.

Severe nutritional iron deficiency and behaviour disorder in an infant.

The case report describes a 9-month-old Indian child with severe iron deficiency and a haemoglobin of 3.1 g/dl (3.1 g per cent). The case illustrates certain behavioural problems (Pollitt & Leibel, 1976)- irritability, anorexia and vomiting - of infants with iron deficiency, which make them more iron-deficient and anaemic. Blood transfusion was used to break this vicious circle. The child was given oral iron supplements, her behaviour improved and the anaemia disappeared.

Measles induced remission of psoriasis.

A 6-year-old girl suffering from severe psoriasis had been treated unsuccessfully by various conventional methods. She developed measles and, on recovery from measles, the psoriasis soon cleared up and now, 6 months later, she still has had no further recurrence. The basic defect in psoriasis, basal cell hyperplasia and defective keratinization, may well be immunologically mediated. Measles virus, by its immunosuppressive effect can lead to remission of psoriasis.

Neurological abnormalities in Wilson's disease are reversible.

The therapeutic responses of seven children with Wilson's disease who presented with neurological disease were evaluated. Neurological abnormalities comprised intellectual deterioration in 7, conduct disorder in five, dystonia in three, choreoathetosis in three, seizures in one and hemiparesis in one. Lethargy and weight loss were present for several months in 6 children. Four children had clinically demonstrable liver disease which was fatal in two. Electroencephalography performed in two children was normal. Computed tomography (CT) of the brain in three children showed cerebral atrophy in all and areas of low attenuation in the basal ganglia which resolved on treatment in one. All patients were treated with penicillamine but, in four, triethylene tetramine (TETA) was substituted because of adverse effects. Neurological abnormalities in these patients were reversible.

Giving assessment reports to parents.

Reactions of 25 parents to receiving copies of written reports concerning developmental assessment of their children were assessed. All parents wanted to have a written report.

Hereditary stiff-baby syndrome.

Clinical and electrophysiologic features of an infant with stiff-baby syndrome were studied; his father, uncle, and paternal grandmother suffered from a similar disorder. They all had a similar facial appearance during infancy (an alert, frightened, tense look) and a tendency to vomit because of hiatal hernias. The condition is benign but causes anxiety and, later, embarrassment as affected adults suddenly fall when stumbling or startled. We suggest that the condition be called hereditary stiff-baby syndrome. The family history is suggestive of autosomal dominant inheritance.

Role of an immunisation advisory clinic.

An immunisation advisory clinic was set up in Redbridge in 1984 to try to allay the anxieties of parents and doctors about vaccination against whooping cough and measles. The parents agreed to vaccination for 54 out of 67 children against whooping cough and 54 out of 57 against measles. Most of the 117 children who were referred to the clinic probably would not have been vaccinated, although only two had valid contraindications.

Antibody response and clinical reactions in children given measles vaccine with immunoglobulin.

Antibody responses and clinical reactions to three measles vaccines (Attenuvax, Mevilin, and Rimevax) injected into the opposite arm to immunoglobulin were assessed in 45 children with brain disorders making them susceptible to fits if given measles vaccine alone. In this small study no unacceptable reactions occurred and in only three cases was the antibody response minimal or absent. More children in this special category should be considered for vaccination against measles in this way.

Neurological features and computed tomography of the brain in children with ornithine carbamoyl transferase deficiency.

The clinical features and the computed tomographic appearances of the brain in seven children with ornithine carbamoyl transferase deficiency are described. Episodic vomiting and drowsiness, acute encephalopathy, failure to thrive and developmental retardation were common, but focal neurological symptoms and signs were also observed. The CT appearances were non-specific with generalised or focal changes. They were related to the severity, the duration and the age of onset of the hyperammonaemia. Since the CT changes may suggest conditions other than metabolic disease, the emergency investigation of a child with an encephalopathy should include the estimation of plasma ammonium and, if elevated, the appropriate investigations to establish the cause.

An inborn error of purine metabolism, deafness and neurodevelopmental abnormality.

A syndrome of hyperuricemia, sensorineural deafness, mild mental handicap and congenital disequilibrium in a four-year-old boy is probably inherited as a sex-linked condition since his mother has sensorineural deafness and similar biochemical abnormalities. There is evidence of a superactive PP-ribose-P synthetase, normal purine salvage enzymes, and severe depletion of nicotinamide adenine dinucleotide and guanine triphosphate in red cells.

Treatment of preterm cervical dilatation with glyceryl trinitrate, a nitric oxide donor.

We report an observational study on the use of transdermal glyceryl trinitrate as a treatment to delay delivery of women presenting in pregnancy with dilatation of the cervix. Ten women found to have cervical dilatation more than 2 cm (range 2 to 8 cm) at gestations between 16 and 32 weeks were studied. These women were treated with 50 mg glyceryl trinitrate patches. The mean prolongation of gestation from the time of admission was 46.2 days (range 1 to 130 days). There was 1 stillbirth in a 22.6-week fetus and 1 neonatal death secondary to prematurity. Vaginal delivery occurred in all but 3 cases. All babies were of size appropriate for gestational age at delivery. The only reported maternal side-effect was headache. No adverse fetal effects were noted. We conclude that glyceryl trinitrate may provide an effective and safe method of tocolysis, well suited for long-term use. The purpose of reporting this study is to highlight the mounting accumulation of data on glyceryl trinitrate and reinforce the need to evaluate its use in randomized controlled trials.

Congenital trypanosomiasis in a child born in London.

A female infant of 22 months was referred to the Hospital for Sick Children, London, because of delayed psychomotor development. Extensive investigations revealed no cause, but eventually trypanosomiasis was diagnosed. The infant had not been outside the UK, but her mother came from Zaire, where the disease is endemic, but had lived in Kinshasa, where there is no sleeping sickness. It is thought, that the mother may have been asymptomatically infected by a fresh-blood transfusion four years earlier, since no other source of infection was apparent.

Value of computerised tomography in children with non-specific mental subnormally.

Seventy-six children had computerized tomography scans as part of an investigation of mental subnormality; most of them are severely retarded and all those over age 5 years attend special schools. Seventy-two per cent of the children had normal scans. Twenty per cent showed cerebral atrophy and in only 8% was there a specific abnormality (agenesis of corpus callosum, arachnoid cyst, communicating hydrocephalus). None of these findings had any positive prognostic implication. Sedation or general anaesthesia was required for all except one child. Injection pethidine compound was used for children under age 5 years or less than 30 kg in weight, and trimeprazine orally was used for older children. The radiation exposure was high--5.6 rad per scan, which is 100 times greater than that from a posteroanterior and lateral chest x-ray film. For these reasons computerised tomography scans cannot be recommended as a routine part of the investigation of children with non-specific mental subnormality.

Early diagnosis of herpes simplex encephalitis in childhood. Clinical, neurophysiological and neuroradiological studies.

In the initial phase of HSE the clinical symptomatology is more variable and insidious in babies and young children than in older children and adults. Combined clinical, neurophysiological and neuroradiological studies have been carried out in 12 children with proven HSE. Ten patients had the first EEGs taken during the acute phase of the illness and all showed large amplitude irregular slow activity, sharp waves and often spikes with variable distribution; in 7 cases periodic phenomena were recognisable. At a later stage localised low amplitude EEG activities were found in children with focal neurological symptoms. Areas of low attenuation were seen in the CT scans of the 7 children who had this investigation done at an early stage of their illness. Such low density regions persisted at follow-up and eventually cerebral atrophy with irregular features became obvious. Prompt EEG investigations combined with CT scans provide an early diagnostic clue for treatment. Follow-up EEG studies (including VEP) and CT scans may help assess the severity of residual cerebral damage in the survivors.

Multicystic encephalomalacia due to fetal viral encephalitis.

Two children, aged 2 weeks and 2 months, with multicystic encephalomalacia aer described. Although computerized tomography (CT) scan was used for the diagnosis, the value of the simple method of transillumination is stressed. The mother of one child had mumps with meningitis at 26 weeks' gestation. The other child had an echovirus 11 isolated from the cerebrospinal fluid and herpesvirus hominis (HVH) from the skin at 8 weeks. We speculate that the changes in the brains of these babies may be due to the respective viruses causing a fetal encephalitis.

Antibody responses and reactions to the whole cell pertussis component of a combined diphtheria/tetanus/pertussis vaccine given at school entry.

To measure the clinical effect of adding a whole cell pertussis component to diphtheria/tetanus vaccine (DT) given as a pre-school booster, 190 children aged 4-5 years were randomised by a double-blind method to receive either diphtheria/tetanus/pertussis (DTP) or DT vaccine in a 1:1 ratio at selected clinics in England. The geometric mean antibody titres to each of the three pertussis antigens were at least sixfold higher in the DTP than the DT vaccine group and equalled or exceeded those in infants immediately after primary immunisation with DTP vaccine. There were no significant differences between DTP and DT vaccinated children in their diphtheria and tetanus antitoxin levels. The frequency of large local reactions and systemic symptoms such as crying and a disturbed night was 2-3-fold higher in the DTP vaccinees than in the DT vaccinees. Medication was given to 44% of DTP and 23% of DT vaccinees (p = 0.006). Although the change to whole cell DTP vaccine at school entry would result in good pertussis antibody titres, the 2-3-fold increase in reactogenicity that would be caused may be unacceptable at a time when whooping cough is not circulating widely. Evaluation of acellular DTP vaccines given as a pre-school booster in children vaccinated under the accelerated schedule is planned.