Movement disorders in childhood: classification and genetic update]

Abnormal movements are not unusual in childhood. Recent genetic progresses provide a new approach of childhood movement disorders. Several loci have been identified in paroxysmal dyskinesia, or in Gilles de la Tourette syndrome. A gene has been cloned in Hallervorden-Spatz syndrome, and a gene has recently been implicated in benign hereditary chorea. Considerable advances concern the genetic of dystonic syndromes: several chromosomal localizations have been identified, and several genes have been cloned. Genetic advances allow nosographic reclassification of some entities and offer new molecular tools for a more appropriate diagnosis. The increasing wealth of genetic knowledge will provide further insight in the understanding of abnormal movement disorders in childhood.     

Depressive disorders in children: diagnosis and therapeutic approaches]

After recalling recent main diagnostic criteria for depressive disorders in childhood, the authors review the various available therapies: chemotherapies (antidepressants, lithium), psychotherapies and medico-social interventions. Each application is examined, as is the practical management of childhood depressive disorders.     

Allergic airway diseases in childhood: An update

Complex multifactorial diseases such as allergic rhinitis and asthma are not only becoming an increasing burden to healthcare systems, but especially affect the life quality of children and families suffering from their allergic symptoms. Also physicians are challenged by the multifaceted diseases as their work involves not only the often difficult decisions on case‐adapted diagnostics, treatment, and monitoring, but also possible preventive measures. This review gives an outline of the latest scientific developments related to the etiology, diagnosis, and management of allergic airway diseases in childhood, as well as prenatal and early life risk factors and strategies for prevention.     

Urinary tract infections in childhood: an update

Although controversies remain regarding the definition, diagnosis, and management of urinary tract infections, such infections can pose a major risk to a child's well-being. Bacteriuria or recurrent urinary tract infections often pose difficult management problems. Symptomatic and asymptomatic bacteriuria during infancy are generally characterized by a benign outcome. In some children repeated episodes and, possibly, renal scarring result. The prognosis in young boys may be guarded if neonatal bacteriuria, with or without symptoms, occurs in the presence of anatomic defects. Although a variety of pathogens have been identified as causing urinary tract infections, Enterobacteriaceae are usually the cause of initial uncomplicated lower tract infections. Accepted therapy for such infections is reviewed, as are the combination therapies used for hospitalized patients with upper tract infections. An investigation of piperacillin, a new, extended-spectrum acylaminopenicillin, raises the hope that it may provide effective monotherapy for upper tract infections. The criteria for selecting patients who require radiologic evaluation in the management of urinary tract infections are reviewed.     

Movement disorder emergencies in childhood

The literature on paediatric acute-onset movement disorders is scattered. In a prospective cohort of 52 children (21 male; age range 2mo-15y), the commonest were chorea, dystonia, tremor, myoclonus, and Parkinsonism in descending order of frequency. In this series of mainly previously well children with cryptogenic acute movement disorders, three groups were recognised: (1) Psychogenic disorders (n = 12), typically >10 years of age, more likely to be female and to have tremor and myoclonus (2) Inflammatory or autoimmune disorders (n = 22), including N-methyl-d-aspartate receptor encephalitis, opsoclonus-myoclonus, Sydenham chorea, systemic lupus erythematosus, acute necrotizing encephalopathy (which may be autosomal dominant), and other encephalitides and (3) Non-inflammatory disorders (n = 18), including drug-induced movement disorder, post-pump chorea, metabolic, e.g. glutaric aciduria, and vascular disease, e.g. moyamoya. Other important non-inflammatory movement disorders, typically seen in symptomatic children with underlying aetiologies such as trauma, severe cerebral palsy, epileptic encephalopathy, Down syndrome and Rett syndrome, include dystonic posturing secondary to gastro-oesophageal reflux (Sandifer syndrome) and Paroxysmal Autonomic Instability with Dystonia (PAID) or autonomic ‘storming’. Status dystonicus may present in children with known extrapyramidal disorders, such as cerebral palsy or during changes in management e.g. introduction or withdrawal of neuroleptic drugs or failure of intrathecal baclofen infusion; the main risk in terms of mortality is renal failure from rhabdomyolysis. Although the evidence base is weak, as many of the inflammatory/autoimmune conditions are treatable with steroids, immunoglobulin, plasmapheresis, or cyclophosphamide, it is important to make an early diagnosis where possible. Outcome in survivors is variable. Using illustrative case histories, this review draws attention to the practical difficulties in diagnosis and management of this important group of patients.     

Pediatric audiological diagnosis and therapy of hearing disorders in childhood]

Practical suggestions concerning screening hearing tests, clinical paedoaudiological diagnosis, adjustment of hearing aids and the use of cochlear implants during infancy are briefly reviewed.     

Biliary lithiasis in childhood: therapeutic approaches

Until recently, biliary lithiasis was considered infrequent in childhood. According to their composition, gallstones can be classified into cholesterol stones and pigment stones. The latter are mainly composed of calcium salts of unconjugated bilirubin and are divided into hard black and soft brown stones. In children, up to 75 % of gallstones are pigment stones. Their etiology is often unknown. Biliary lithiasis in children differs from that in adults and there is very little scientific evidence on the most suitable therapeutic procedures. Symptom-free stones usually have a benign course and do not require medical or surgical treatment. Symptoms are often nonspecific and include dyspepsia and chronic abdominal pain. These symptoms are an indication for ultrasonographic scan to rule out the presence of gallstones. Cholecystectomy is the definitive treatment for gallstones but is not always indicated. Medical treatment with ursodeoxycholic acid is indicated in oligosymptomatic and asymptomatic lithiasis with transparent, soft, cholesterol-rich stones and a functional bladder and in patients with a high surgical risk.     

Causes of childhood deaths in Bangladesh: an update

Knowledge of the causes of child death is important for health-sector planning since they relate to available interventions. Little is known about causes of child death in Bangladesh from the conventional sources since there is no vital registration system and very few deaths are attended by a qualified physician. To determine the cause structure of child deaths, verbal autopsy interviews were conducted in the Bangladesh Demographic and Health Survey (BDHS) 1993/94 national sample. Verbal autopsy is a method of finding out the causes of death based on an interview with the next of kin or other caregivers. Between BDHS 1993/94 and BDHS 1996/97, 1-4-y-old child mortality in Bangladesh declined by about 27.0%. This impressive decline prompted a verbal autopsy study using the BDHS 1996/97 national sample to determine whether the cause structure had changed. The same verbal autopsy instrument and methods to collect the data and the same computer algorithm to assign causes of death were used in both surveys. Comparison of BDHS 1993/94 and 1996/97 cause-specific mortality rates revealed that deaths due to almost all causes had declined, although significantly so only for acute respiratory infections (ARI), persistent diarrhoea and drowning. Deaths due to neonatal tetanus, acute watery diarrhoea and undernutrition had not decreased at all. Conclusion: Despite an impressive decline in deaths due to ARI, this condition remains the most important known cause of death in Bangladeshi children. Neonatal tetanus and measles together account for about 10% of deaths in children under 5 y. Further improvements in child survival are possible by improving access to and quality of available child survival interventions.