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Biomedical subjects

J Britto

Publications and source records attributed to J Britto.

At least 19 recordsLinked to original sources
PubMed2001

Inherited early onset severe axonal polyneuropathy with respiratory failure and autonomic involvement.

We report dizygotic twins who first presented at the age of 6 months with severe diaphragmatic weakness and marked abnormalities of autonomic function. A female sibling had earlier died from a disorder with similar clinical features. Both twins had a severe axonal polyneuropathy with generalized hypotonic limb weakness together with diaphragmatic paralysis resulting in respiratory failure. Associated features were tachycardia, increased sweating, elevated body temperature, and hypertension, suggesting autonomic dysfunction. Nerve conduction studies indicated an axonopathy affecting both motor and sensory nerve fibres. Sural nerve biopsy in one twin performed at the age of 7 months showed a reduced population of myelinated nerve fibres, particularly those of larger diameter, with no indication of hypomyelination, demyelination or axonal atrophy. Examples of axonal forms of hereditary motor and sensory neuropathy (HMSN) with onset in infancy are very rare and autonomic involvement associated with this condition has not so far been described.

Age of Onset↗
PubMed2000

Efficacy of chest CT in a pediatric ICU: a prospective study.

STUDY OBJECTIVES: (1) To determine whether chest CT provides additional information compared with chest radiography regarding the nature of intrathoracic disease in critically ill children, (2) to determine whether such information alters clinical management, (3) to assess the role of a low-dose high-resolution CT (HRCT) protocol in pediatric ICU (PICU) patients. DESIGN: Prospective study. SETTING: Specialized PICU in a teaching hospital serving London and the south of England. PATIENTS: Twenty children (age range, 3 weeks to 12 years; median, 11 months) underwent chest CT during a 33-month period. Inclusion criteria were (1) inconclusive diagnosis from chest radiograph (CXR) or (2) CXR appearances inconsistent with high oxygenation or ventilatory requirements (PaO(2) to fraction of inspired oxygen ratio < 30 or mean airway pressure > 15 cm H(2)O). INTERVENTIONS: Low-dose HRCT scans (50 mA, 2-mm slice thickness at intervals of 10 or 15 mm) were performed on 12 patients, and helical CT (50 to 250 mA; pitch, 1 to 1.5) performed on 8 patients. MEASUREMENTS AND RESULTS: CT provided additional information regarding the nature of intrathoracic disease in 17 of 20 patients (85%) and resulted in changes to subsequent clinical management in 12 of 20 patients (60%). CONCLUSIONS: Chest CT can add to the accuracy of intrathoracic diagnosis provided by the CXR and may directly influence the acute management of critically ill children. The CT protocol should be tailored to the clinical and radiologic question posed for each individual patient. Noncontiguous HRCT can often provide accurate assessment of pulmonary parenchymal and pleural disease at a reduced radiation dose compared with helical CT.

Child↗
PubMed1999

Stitch tricks.

Explore the source record for details and available documents.

Humans↗
PubMed1999

Distribution and pathophysiology of acute lobar collapse in the pediatric intensive care unit.

OBJECTIVE: The high incidence of lower lobe collapse in adult intensive care patients is well described. We aimed to document the incidence and distribution of acute lobar collapse in the pediatric intensive care setting. The influence of anatomical and pathophysiological differences between the adult and pediatric respiratory tract will be considered. DESIGN: Retrospective review of chest radiograph series. SETTING: Tertiary referral center for pediatric intensive care and the Department of Diagnostic Radiology in a large teaching hospital in England. PATIENTS: Cohort of 160 patients receiving intensive care during a 2-yr period (age range, 6 days-18 yrs; median, 23 months). INTERVENTIONS: None MEASUREMENTS AND MAIN RESULTS: Twenty-four of 160 children (15%) developed acute lobar collapse during their intensive care unit admission. Isolated right upper lobe collapse occurred in 14 patients, right upper lobe in association with one or more other lobes in five patients, and lobar collapse other than the right upper lobe in five patients. The development of lobar collapse and, in particular, right upper lobe collapse was associated with a lower median age (no collapse, 26 months; lobar collapse, 8 months; right upper lobe collapse, 4 months). Lobar collapse was significantly associated with the requirement for mechanical ventilation during admission (chi-square, 12.18; p = .005). It was observed in association with both high and low endotracheal tube positions. CONCLUSION: The predominance of upper lobe and, in particular, right upper lobe collapse observed in pediatric intensive care patients contrasts with the high incidence of lower lobe collapse in their adult counterparts. Multiple interrelated factors are likely to be contributory and include the following: a) anatomical and physiological differences between adults and children; b) the pathophysiology of childhood respiratory disease; c) more critical positioning of endotracheal tubes in younger patients and their movement with patient positioning.

Adolescent↗
PubMed1999

Emergency management of meningococcal disease.

Meningococcal disease remains a major cause of mortality in children in the UK. Aggressive early volume resuscitation, meticulous attention to the normalisation of all physiological and laboratory parameters, and prompt referral to specialist paediatric intensive care may lead to a sharp reduction in mortality. Application of the management algorithm described in this article may be helpful to those involved in the early part of management of critically ill patients with meningococcal disease.

Algorithms↗
PubMed1999

Pertussis is increasing in unimmunized infants: is a change in policy needed?

The proportion and trend in absolute number of pertussis notifications in young infants has increased each year in England and Wales since the accelerated immunization schedule was introduced. We report five infants all less than 3 months of age admitted with life threatening pertussis infection to two paediatric intensive care units. Despite aggressive cardiorespiratory support measures, three of the infants died. Pertussis remains a significant cause of morbidity and mortality in unimmunized infants. In this age group presentation is likely to be atypical and infection more severe. Public health measures to prevent the disease could be strengthened. Chemoprophylaxis should be offered to susceptible contacts and booster vaccinations against pertussis considered.

Age of Onset↗
PubMed1999

Emergency cranial computed tomography in the management of acute febrile encephalopathy in children.

OBJECTIVE: Evaluation of the influence of emergency cranial computed tomography on the management of acute febrile encephalopathy in children. METHODS: A retrospective study in children with acute febrile encephalopathy who underwent emergency cranial computed tomography within 12 hours of admission to the paediatric intensive care unit. All scans were evaluated by two independent radiologists. RESULTS: Thirty nine children were included. Fourteen scans were abnormal and two had clinically insignificant incidental findings. Four children with focal neurological signs had scans demonstrating extra-axial collections. None required neurosurgical intervention. Clinically, raised intracranial pressure was present in 10 patients. Only five had cerebral oedema on computed tomography; these five children died. Emergency cranial computed tomography influenced subsequent management in no child without focal neurological signs and in only one child with focal neurology. CONCLUSION: Emergency cranial computed tomography in acute febrile encephalopathy in children without focal neurological signs has little influence on subsequent management. Where cranial computed tomography is thought to be necessary, it should be carried out when the child's clinical condition has been stabilised.

Adolescent↗
PubMed1998

Avoidable deficiencies in the delivery of health care to children with meningococcal disease.

OBJECTIVES: It is apparent that delays and inadequate or inappropriate management occur frequently and may contribute to the continued high mortality seen in meningococcal disease. An attempt has been made to define the major sources of delay or inappropriate treatment. METHODS: A prospective, descriptive study of children with meningococcal disease referred to a tertiary centre paediatric intensive care and infectious disease unit. Definitions of optimal care were established at three stages: parental; general practitioner (GP)/accident and emergency (A&E) department; and hospital. Duration of symptoms and management were recorded from direct questioning of parents and carers, and from hospital records. RESULTS: 54 consecutive children with meningococcal disease were recruited to the study. Delayed parental recognition occurred in 16 children. GPs correctly diagnosed 19 of 35 children. Delay of 2.5-21 hours occurred in those who were incorrectly diagnosed. Two of 15 children who presented to the A&E department with specific features were incorrectly diagnosed. Hospital treatment was suboptimal in 71%. Shock was not recognised or treated in 50%, 20% of children had unnecessary lumbar punctures. Time from illness onset to treatment was longer in fatal disease (median 18.3, range 8-24 hours), compared with survivors (median 12, range 2-48 hours; p < 0.01, Mann-Whitney U test). CONCLUSION: Suboptimal treatment in meningococcal disease is due to failure of parents, GPs, and hospital doctors to recognise specific features of the illness. Improvement by public education and better training of clinicians in recognition, resuscitation, and stabilisation of seriously ill children.

Adolescent↗