Current status of prenatal diagnosis, operative management and outcome of esophageal atresia/tracheo-esophageal fistula.

Ultrasonographic features suggestive of esophageal atresia with or without tracheo-esophageal fistula (EA/TEF) are only in a small minority of fetuses with EA/TEF (<10%) identifiable on prenatal scans.The prenatal diagnosis of EA/TEF relies in principle, on two nonspecific signs: polyhydramnios and absent or small stomach bubble. Polyhydramnios is associated with a wide range of fetal abnormalities, but most commonly it pursues a benign course.

The treatment of subluxation of the hip in children over the age of four years.

Subluxation of the hip presenting for the first time in a child over the age of four years is rare. We report ten cases treated over nearly 11 years by the senior author (JAF). We describe the surgical procedures and the results, at maturity, of nine of the ten patients.

Associated urologic anomalies in low imperforate anus are capable of causing significant morbidity: a 15-year experience.

Objectives: To determine the incidence and morbidity caused by urologic anomalies in patients with low imperforate anus.

Methods: The case records and imaging studies of 95 patients who underwent surgery for low imperforate anus over a 15-year period were retrospectively reviewed. Follow-up ranged from 2 to 18 years (mean 5.

The outcome of children admitted to intensive care with meningococcal septicaemia.

Objective: To review our experience of children with meningococcal septicaemia, and to validate, in our group, severity scores used in different populations to predict outcome.

Design: Retrospective review of case notes and charts.

Patients: A total of 35 children were admitted to the paediatric intensive care unit (ICU) in the Royal Children's Hospital (RCH) in the 8 years between January 1985 and December 1992 with proven meningococcal septicaemia.

Childhood soft tissue chondroma: a case report.

In this paper we draw attention to the existence of soft tissue chondromas in childhood. This entity is well recognized in adults, where over 90% of the recorded cases have occurred in the hands and feet. Our case is unusual in that the child was only 10 years of age and the lesion was on the back, not in the extremity.

Surfactant adjunctive therapy for Pneumocystis carinii pneumonitis in an infant with acute lymphoblastic leukaemia.

We report successful treatment of adult respiratory distress syndrome (ARDS) with artificial surfactant (40mg/kg, Colfosceril Palmitate, ‘Exosurf’, Wellcome) in an infant with severe pneumonitis.

Hypogammaglobulinaemia secondary to reflux oesophagitis.

A 7 year old girl referred for investigation of hypogammaglobulinaemia had hypoalbuminaemia and severe necrotising oesophagitis on oesophagogastroduodenoscopy. Nissen fundoplication resolved all clinical and laboratory abnormalities, but she remains under surveillance because of histological findings of Barrett's oesophagus. Erosive reflux oesophagitis can present with minimal localising symptoms, and hypogammaglobulinaemia and hypoalbuminaemia, presumably from protein loss.

Persistent polyuria after posterior urethral valves.

Objective: To assess urinary concentrating ability, urine production and glomerular filtration rates in a cohort of boys with previously treated posterior urethral valves.

Patients And Methods: Urinary concentrating capacity was assessed in 51 boys aged 5.4-9.

Filtration of fentanyl is not the cause of the elevation of arterial blood pressure associated with post-bypass ultrafiltration in children.

Modified ultrafiltration after cardiopulmonary bypass in children has been shown to be associated with an increase in arterial blood pressure. As part of a series of studies to investigate the possible causes of this blood pressure elevation, the hypothesis that if filtration was removing a significant amount of fentanyl, then the increase in blood pressure might be due to pain was proposed. Ten children, aged between 0.

Depth of epidural space in children.

We have studied the depth of epidural space in 586 children who had lumbar epidural block as part of their anaesthetic management. The mean depth of epidural space in neonates was 1 cm (SD 0.2, range 0.

A new technique for fibreoptic intubation in children.

We describe a technique involving the use of a laryngeal mask airway, fibreoptic bronchoscope and a guide wire to manage the intubation of a child who was known to be a difficult intubation. The technique is simple, atraumatic, permits the use of an adult bronchoscope for infants and children, and allows control of the airway and ventilation throughout the period of intubation.

Mercapto-acetyltriglycine (MAG 3) renography and indirect radionuclide cystography in posterior urethral valves.

Objective: To assess dynamic isotope renography with 99mTc-labelled mercapto-acetyltriglycine (MAG 3) in conjunction with indirect radionucleide cystography (IRC) in the follow-up of boys with posterior urethral valves.

Patients And Methods: Fifty-one boys (95 renal units; mean age = 89 months, range 65-118) with previously treated posterior urethral vales underwent MAG 3 renography followed by IRC. Glomerular filtration rate (GFR) was estimated from the plasma clearance of 51Cr-ethylenediamine tetra-acetic acid following single intravenous injection.

A clinical and immunological study of Netherton's syndrome.

Netherton's syndrome is a rare genodermatosis of unknown cause, which is classified as an ichthyosiform syndrome. A clinical and immunological study of seven patients with Netherton's syndrome illustrates the clinical spectrum of this disorder, the frequent association with atopy, and the absence of consistent immunological abnormalities. Failure to thrive in infancy was a feature in six of the seven patients, and was considered to be life-threatening in three.

Conservative surgery in multimodal therapy for pelvic rhabdomyosarcoma in children.

Twenty-six previously untreated children, median age 3.4 years, with pelvic rhabdomyosarcoma (RMS) were seen between 1983 and 1988. Fourteen were girls.

Respiratory complications due to inadequate analgesia following thoracotomy in a neonate.

We present a case in which a 2-day-old infant suffered respiratory complications shortly following premature termination of a postoperative extradural infusion. Restoration of analgesia led to a marked clinical improvement.

Late effects of intensive treatment for acute myeloid leukemia and myelodysplasia in childhood.

Purpose: To perform a comprehensive assessment of the late effects of short-term intensive chemotherapy for childhood acute myeloid leukemia (AML) and myelodysplasia, and compare the sequelae of intensive chemotherapy alone with those of total-body irradiation (TBI).

Patients And Methods: Of 33 survivors studied, 26 (group A) received intensive chemotherapy including anthracyclines, one also received busulfan, cyclophosphamide (Bu/Cy), and bone marrow transplantation (BMT). Seven patients (group B) received chemotherapy, TBI, and BMT.

Identification of adenoviruses in faeces from patients with diarrhoea at the Hospitals for Sick Children, London, 1989-1992.

Faecal samples from 137 patients that had been shown to contain adenoviruses by electron microscopy were identified in a series of enzyme immunoassays (EIA) using a single monoclonal antibody (Mab) to adenovirus 40 and four different Mabs to adenovirus 41. Adenoviruses were partially characterised by restriction enzyme analysis (REA) of DNA extracts using SmaI. Samples were also run in a commercial EIA (Adenovirus IDEIA; Dako, Ltd.

Case report 834: Chronic sclerosing osteomyelitis of the mandible with long bone periostitis.

We present the case of a patient with primary chronic osteomyelitis of the mandible, the radiological appearance of which is compatible with a diagnosis of chronic sclerosing osteomyelitis. The accompanying femoral and tibial periosteal reactive new bone formation and the benign clinical course suggest that this presentation may represent a form of chronic multifocal osteomyelitis.

Investigation and management of the painful hip in childhood.

The cause of the painful hip in childhood must be identified quickly to avoid permanent damage resulting from conditions such as septic arthritis. A painful hip may be the presenting feature of a number of conditions and their investigation and management are described here.

Plain chest radiographic changes of the absent pulmonary valve syndrome.

The plain chest radiographs of 40 patients with the absent pulmonary valve syndrome in association with tetralogy of Fallot have been reviewed. The conventional frontal projection showed characteristic changes of massively dilated central pulmonary arteries with normal peripheral markings in all patients (100%). Lobar hyperinflation was present in a significant proportion of the infant group (38%) which in a smaller group can be a complication requiring surgery in addition to that for the cardiac malformation.

Injuries to the physis by extravasation. A rare cause of growth plate arrest.

We report two cases of deformity of the ankle caused by physeal damage secondary to extravasation of an intravenous infusion in infancy. The possible mechanisms of injury to the physis are discussed.

Waxy keratoses of childhood.

Three cases of waxy keratoses of childhood occurring in two families are described. The disorder seen in these three cases appears to be clinically and histopathologically distinct from previously described familial disorders of keratinization, which also feature multiple discrete papules.

Growth promotion by homocysteine thiolactone and its alpha-alkylated derivative - A role for excess growth in homocystinurias?

It is controversial whether homocysteic acid or other homocysteine derivatives show growth promoting effects. In a clonogenic assay we could show that homocysteine thiolactone and its alpha alkylated derivative increased colony formation significantly. Our work favorizes previous observations showing growth promoting activity of homocysteine derivatives and encourages further studies on that subject with implications for growth in physioogy and under pathological conditions.