Comparative outcome study between triplet and singleton preterm newborns.

Aim: To evaluate the outcome of triplet versus singleton preterm newborns.

Methods: The study population included 64 sets of preterm triplet (gestational age 25-34 wk) and 64 singleton controls. Data on prenatal and perinatal findings, neonatal complications, duration of hospitalization, and neonatal mortality were collected by chart review.

Acquired trisomy 21 and distinct clonal evolution in acute megakaryoblastic leukaemia in young monozygotic twins.

An intrauterine origin of childhood acute lymphoblastic leukaemia (ALL) was proven by the identical clonotypic gene rearrangement in the concordant leukaemias of monozygotic twins, arising from a single clonogenic progeny. The monozygotic twins, presented at the age of 22 months with acute megakaryoblastic leukaemia (AML-M7) in one and myelodysplasia transformed to AML-M7 in the other. Leukaemic cells in both twins carried trisomy 21 and additional different clonal evolution changes of del(20q) in the first twin and trisomy 8 in the second.

Whole blood platelet deposition on extracellular matrix under flow conditions in preterm neonatal sepsis.

Unlabelled: Platelet function in preterm infants with sepsis was evaluated by measuring their adhesion and aggregation properties using the Cone and Plate(let) Analyser. This may lead to earlier detection of bleeding tendency in septic infants. Platelet function was investigated in 54 preterm infants, of whom 32 had proven neonatal sepsis and 22 were healthy matched controls.

Recurrent facial nerve palsy in paediatric patients.

Unlabelled: The aim of the study was to investigate the clinical presentation and prognosis of recurrent facial nerve palsy (RFNP) in children. The files of 182 patients referred to the Schneider Children's Medical Centre of Israel for neurological evaluation of isolated peripheral facial nerve palsy between October 1992 and December 1998 were reviewed. RFNP was found in 11 patients (9 females, 2 males), with an incidence of 6%.

Lactic dehydrogenase isoenzymes in cerebrospinal fluid associated with hydrocephalus.

Unlabelled: Various neurological disorders are associated with specific changes in the level of total lactic dehydrogenase and concentrations of its isoenzymes in the cerebrospinal fluid. We describe the lactic dehydrogenase isoenzyme values in children with hydrocephalus. Cerebrospinal fluid samples collected from 10 patients (2 to 16 mo) with hydrocephalus were analysed for total lactic dehydrogenase activity and lactic dehydrogenase isoenzymes.

Fanconi anaemia group A (FANCA) mutations in Israeli non-Ashkenazi Jewish patients.

Fanconi anaemia (FA) is a genetically heterogeneous disease with at least eight complementation groups (A-H). In the present study, we investigated the molecular basis of the disease in 13 unrelated Israeli Jewish (non-Ashkenazi) patients with FA. All 43 exons of the Fanconi anaemia A (FANCA) gene were amplified from genomic DNA and screened for mutations by single-strand conformation polymorphism and DNA sequencing.

Faecal occult blood in children with coeliac disease.

Unlabelled: It has recently been suggested that in adults with coeliac disease, faecal blood loss may play a role in the development of iron deficiency. A group of 45 children diagnosed with coeliac disease during 1996 and 1997 were therefore prospectively evaluated for the presence of gluten in their diet, iron deficiency anaemia, and faecal occult blood. Sixty children admitted for elective surgery or asthma served as controls.

Serum transferrin receptor in children and adolescents with inflammatory bowel disease.

Unlabelled: Iron studies are difficult to interpret in patients with chronic inflammatory states such as inflammatory bowel disease (IBD). Serum transferrin receptor (TfR) has been reported to be a reliable tool for the diagnosis of iron deficiency in adults. Our aim was to evaluate the role of serum TfR in diagnosing iron deficiency in children and adolescents with IBD.

Increased prevalence of urinary tract infections and anomalies in infants with pyloric stenosis.

We performed a retrospective analysis of all patients admitted to our institution with a diagnosis of infantile hypertrophic pyloric stenosis (IHPS) during a 10-year period from 1985-95 in order to assess the possible association between IHPS and urinary tract infections (UTIs). All 285 patients with IHPS had radiological or ultrasonographic confirmation of that diagnosis and underwent the Ramstedt procedure. Those who continued to be symptomatic were evaluated for UTI by urine analysis and culture.

Open lung biopsy--successful diagnostic tool with therapeutic implication in the critically ill paediatric population.

Open lung biopsy (OLB) is an important diagnostic tool in children with immune deficiency and/or chronic lung disease with diffuse pulmonary compromise. These patients require a tailored therapeutic approach owing to their fragile status and the side effects of unnecessary or inadequate treatment. Twenty-six patients of mean age 5.

Prolonged croup due to herpes simplex virus infection.

Unlabelled: Herpes simplex virus (HSV) is an uncommon cause of acute laryngitis in immunocompetent patients since it mostly occurs in immunocompromised subjects. We present two previously healthy children with prolonged gingivostomatitis and stridor (lasting 3 and 4 weeks) in whom HSV-1 was isolated from subglottal ulcers.

Conclusion: HSV should be considered a possible pathogen in cases of prolonged or atypical croup not only in immunocompromised or elderly patients but also in otherwise healthy children.

Intramuscular ceftriaxone compared with oral amoxicillin-clavulanate for treatment of acute otitis media in children.

Unlabelled: Two hundred and fifteen children aged 4 months 6 years with acute otitis media (AOM) were randomized to be treated either by a single i.m. injection of ceftriaxone, 50 mg/kg, with a second dose in the event of unsatisfactory response after 48 h or a history of recurrent AOM (109 patients) or amoxicillin clavulanate 12.

A new classification for pericarditis associated with meningococcal infection.

Unlabelled: Acute meningococcal pericarditis is a rare clinical disorder. Our review of the literature disclosed that current classifications are confusing since they fail to differentiate between two distinct criteria: time and causality. We suggest a new classification of the various states of meningococcal pericarditis on the basis of the pathophysiological process: disseminated meningococcal disease with pericarditis (purulent, culture-positive, associated with meningococcal bacteraemia); isolated meningococcal pericarditis (purulent, culture-positive but without signs of meningeal or other clinical systemic involvement); and reactive meningococcal pericarditis (immunological, late-onset, culture-negative, resembling post-viral pericarditis).

International epidemiological and microbiological study of outbreak of Salmonella agona infection from a ready to eat savoury snack--II: Israel.

Objectives: To explain an increase in the incidence of salmonellosis caused by Salmonella agona in Israel between October 1994 and January 1995 in the light of an outbreak of S agona phage type 15 infection in England and Wales caused by consumption of a ready to eat savoury snack produced in Israel.

Design: Epidemiology of S agona in 1994-5 was analysed and two consecutive, case-control studies of 32 and 26 case-control pairs were performed. Phage typing and molecular methods were used to characterise strains of S agona isolated from cases and samples of the snack in Israel and England and Wales.

Familial hypothyroidism with autosomal dominant inheritance.

Three generations of a family with clinical and subclinical hypothyroidism caused by thyroid stimulating hormone (TSH) unresponsiveness are described. Findings were low to normal serum thyroxine, raised serum TSH, and low radioiodine uptake; goitre was notably absent. This family is the first evidence of an autosomal dominant mode of transmission of TSH unresponsiveness and may enable identification of the precise defect by genetic linkage study.

Effective treatment of painful bone crises in type I gaucher's disease with high dose prednisolone.

In type I Gaucher's disease, episodes of severe disabling bone pain, the so called bone crises, may be resistant to all analgesics, including narcotics. The demonstration of subperiosteal oedema on magnetic resonance imaging (MRI) led to an attempt to use steroids to relieve the oedema and thereby the pain. On eight occasions, five patients with documented bone crises received conventional dose steroids (20 mg/m2/day) with considerable shortening of the attacks.

MRI in acute poliomyelitis.

MRI can be used in the diagnosis of anterior horn infection and for assessing the extent of disease. There are no specific MRI signs to differentiate between the various possible pathogens. This is demonstrated in the present case of poliomyelitis, in which MRI of the spine played an important role in establishing the diagnosis.