Case report: Molecular characterisation of adipose-tissue derived cells from a patient with ROHHAD syndrome.

There have been over 100 cases of Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome reported, but there is currently no curative treatment for children with this condition. We aimed to better characterise adipose cells from a child with ROHHAD syndrome. We isolated pre-adipocytes from a 4 year-old female patient with ROHHAD syndrome and assessed proliferation rate of these cells.

The impact of HPV cervical screening on negative large loop excision of the transformation zone (LLETZ): A comparative cohort study.

Objective: To determine the incidence and predictors of negative large loop excision of the transformation zone (LLETZ) following the introduction of Human Papillomavirus (HPV) cervical screening.

Method: A retrospective cohort study. Two independent cohorts, who attended for a LLETZ procedure, before and after the introduction of HPV cervical screening were compared.

Treatment of febrile seizures: historical perspective, current opinions, and potential future directions.

Although most febrile seizures do no harm and two-thirds of initial cases have no witnessed recurrence, the seizures cause much family anxiety, and are sometimes prolonged. In rare cases they are the first evidence of important epilepsy syndromes or are implicated in the development of epilepsy with mesial temporal sclerosis in later life. There have been trials of prophylactic treatment with antiepileptic drugs including carbamazepine, diazepam, phenobarbital, phenytoin, and sodium valproate.

Research governance delays for a multicentre non-interventional study.

Objectives: To evaluate the delay in research governance approval for a non-interventional, multicentre study in the United Kingdom.

Design: The times taken from application to the granting of research governance approval for an observational study of childhood stroke with ethical approval were prospectively recorded.

Setting: Ninety-two acute NHS Trusts in the United Kingdom.

Cluster headache in children--experience from a specialist headache clinic.

Background: Cluster headache is a primary headache disorder with the distinct clinical features: unilaterality, extreme pain, cranial autonomic features and periodicity. The prevalence of the disorder is 0.1% in adults and with a male predominance.

Syndrome of fixed dystonia in adolescents--short term outcome in 4 cases.

We describe the clinical features, investigations and outcome of 4 adolescents aged 13, 16, 17 and 19 years, with fixed dystonia. The diagnosis was made within 6 months of the onset of symptoms. One patient had an identifiable traumatic precipitant.

Is hypsarrhythmia a form of non-convulsive status epilepticus in infants?

Background: Hypsarrhythmia is generally associated with infantile spasms, a combination referred to as West syndrome. It is debatable whether hypsarrhythmia is usefully regarded as a form of non-convulsive status epilepticus (NCSE).

Summary Points: The earliest English language description of hypsarrhythmia reported an almost continuous EEG pattern, although later studies showed a degree of state dependence.

New knowledge, new insights, and new recommendations.

Scientific controversy and media hype in unexpected infant deaths

Advances in pneumococcal vaccines: advantages for infants and children.

The introduction of Haemophilus influenzae type b (Hib) vaccine into the universal immunisation schedules of many industrialised countries and the subsequent remarkable decline in the incidence of invasive Hib disease has further highlighted the impact of invasive pneumococcal diseases. Streptococcus pneumoniae is now the leading cause of bacterial meningitis in children in many settings and a leading cause of vaccine-preventable bacterial disease in children worldwide. The currently marketed 23-valent pneumococcal polysaccharide vaccine provides large serotype coverage at a relatively low cost.

Mucosal immunology of vaccines against pathogenic nasopharyngeal bacteria.

The introduction of Haemophilus influenzae type b conjugate vaccines during the 1990s was followed by dramatic decreases both in the incidence of Haemophilus influenzae type b related invasive disease and in nasopharyngeal carriage of the organism. The extent of this effect has been influenced by the fact that Haemophilus influenzae type b conjugate vaccines reduce nasopharyngeal carriage and induce herd immunity. Based on the success of Haemophilus influenzae type b conjugate vaccines, chemical conjugation has been applied to the development of pneumococcal and meningococcal polysaccharide conjugate vaccines.

Bacterial polysaccharide-protein conjugate vaccines.

Following demonstration that chemical conjugation of polysaccharide antigens to proteins could enhance their immunogenicity in the 1920s, interest in this approach to primary prevention of bacterial infections waned with the development and widespread use of antibiotics. Emergence of resistant bacteria rekindled interest in the late 20th century, which saw extremely rapid development and implementation of several vaccines which are already rapidly changing the epidemiology of childhood infections with Haemophilus influenzae type b, Streptococcus pneumoniae and Neisseria meningitidis. Others such as Group B streptococcus and Salmonella typhi infections may soon follow.

Mucosal immune responses to capsular pneumococcal polysaccharides in immunized preschool children and controls with similar nasal pneumococcal colonization rates.

Background: Immunization with conjugate pneumococcal vaccines induces significant primary and memory IgG anti-polysaccharide (PS) responses in serum. It can also induce mucosal responses in infants especially after a polysaccharide booster. However, it is unclear whether it can prime for mucosal memory responses on nasal exposure to pneumococcus, which may be important in protection against pneumococcal invasion and/or carriage.

Sudden unexpected deaths after discharge from the neonatal intensive care unit.

The published evidence on the risk of sudden unexpected death in infants after discharge from the neonatal intensive care unit (NICU) is reviewed, together with the relevance of the various identified potentially modifiable post-natal risk factors, particularly sleeping position. Infants of low birthweight, short gestation, and those with adverse perinatal histories are at substantially increased risk of sudden infant death syndrome (SIDS), but the potential benefits from following the measures designed to reduce the risk of SIDS are proportionally greater than for term infants. The use of home apnoea monitors has not been shown to be of value in preventing SIDS, but the importance of maintaining adequate oxygenation in infants with bronchopulmonary dysplasia is emphasised.

Lifestyle behaviours of young adult survivors of childhood cancer.

This cross-sectional study collected baseline data on the health behaviours of a large population of survivors of childhood cancer in the UK, aged 18-30 years, compared with those of sex- and age-matched controls. Data from 178 young adult survivors of childhood cancer, diagnosed and treated at Bristol Children's Hospital, 184 peers from the survivors' GP practices and 67 siblings were collected by postal questionnaire. Conditional logistic regression analysis showed that, for matched sets of survivors and controls, survivors of a variety of childhood cancers reported lower levels of alcohol consumption (P=0.

Immune responses to novel pneumococcal proteins pneumolysin, PspA, PsaA, and CbpA in adenoidal B cells from children.

Studies of mice suggest that pneumococcal proteins, including PspA, pneumolysin, PsaA, and CbpA, are promising vaccine candidates. To determine whether these proteins are good mucosal immunogens in humans, adenoidal lymphocytes from 20 children who had adenoidectomies were isolated and tested by ELISpot for antigen-specific antibody-secreting cells (ASCs). Cells were also cultured for 7 days in the presence of a concentrated culture supernatant (CCS) from a type 14 strain of pneumococcus which contained secreted pneumococcal proteins, including PspA, pneumolysin, PsaA, and CbpA, and then tested by ELISpot.

Meningococcal serogroup C conjugate vaccine.

Meningococcal meningitis and septicaemia are important causes of morbidity and mortality in many parts of the world. More than 90% of the cases are caused by serogroups A, B and C; the remaining 10% are largely caused by the W-135 and Y strains. During the mid-to-late 1990s there was an increase in meningococcal serogroup (MS) C disease in the UK and some parts of Europe.