Biomedical subjects
Publications and source records attributed to D J Matthew.
Four patients with generalized lymphangiomatosis presenting with chylothoraces are described. All four had bone involvement, two had involvement of the spleen, and one of the pericardium. The diagnosis was confirmed by typical radiology, histology, and in three patients by immunohistochemistry. Treatment was mainly palliative. Three patients died within 1/2 to three years of presentation.
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Initial reporting and validation of the Camino miniaturised fibreoptic cerebral intraparenchymal pressure monitoring device has indicated that this tip transducing system (a) allows direct measurement of brain tissue pressure, (b) has a rapid response rate to intracranial changes and (c) correlates well with intraventricular pressure. However, there are no specific reports of this form of monitoring during non-traumatic coma in children, or any evaluation of change in clinical practice when compared with experience of other forms of invasive intracranial pressure monitoring. Over a 5-year-period (1985-1989) on the General Paediatric Intensive Care Unit, 74 children with presumed raised intracranial pressure complicating non-traumatic coma have had invasive intracranial pressure monitoring with a variety of devices. An intraventricular catheter was used in 16 patients, a subdural catheter in 6 patients, a subarachnoid screw in 35 patients and a fibreoptic intraparenchymal catheter in 17 patients. In 1985 to 1986 our preferred technique was the subarachnoid screw (33/49 patients monitored). Between 1987 and 1989 we have mainly used the Camino fibreoptic intraparenchymal monitoring system (17/25 patients monitored). In the whole series there were no cases of acute haemorrhage related to monitoring and only one patient developed infection and in this child an intraventricular catheter was used. The experience with the fibreoptic system has been favourable and the technique for insertion does not require additional expertise in comparison with standard subarachnoid screw pressure monitoring. Therefore in young children with raised intracranial pressure complicating non-traumatic coma, cerebral fibreoptic intraparenchymal pressure monitoring should be used in preference to standard subarachnoid screw pressure monitoring.(ABSTRACT TRUNCATED AT 250 WORDS)
The aetiology, severity of systemic and biochemical abnormalities, seizure duration, EEGs and CT scans have been reviewed in previously normal young infants with an acute critical illness occurring after the first week of life; none of whom had birth asphyxia. Findings were related to outcome in an attempt to evaluate the significance of seizures during the acute phase of severe illness. In three years seizures occurred in 54/251 (22%) young infants requiring ventilatory support. In these patients the acute illness was most commonly infection and encephalitis/encephalopathy. Twenty-one died, 24 had good or moderate outcome and 9 poor outcome (follow-up 6-27 months). The outcome was not directly related to diagnosis, but to systemic and biochemical changes, the most important being severe hypotension (40/54). After correction of these factors, in survivors, increasing number of days over which seizures occurred during the acute phase of illness was related to worsening outcome (tau(c) = 0.66, p less than 0.0001). In many of these patients one of three abnormal low density changes (generalized, boundary zone and focal) were seen on CT scan and were not uncommonly associated with focal/multifocal clonic seizures and characteristic type, distribution and evolution of EEG discharge. In the 45 patients with EEGs from presentation, severity of encephalopathy assessed by predominant background EEG activity was most closely related to outcome, irrespective of aetiology and seizures. There was a significant relationship between graded severity of background EEG activities and outcome both in the initial and serial recordings (tauB = 0.70, p less than 0.0001 and 0.75, p less than 0.0001 respectively). Seizures are a common occurrence in the previously well young infant with an acute critical illness necessitating intensive care. Prompt recognition and treatment may influence outcome in patients with a potentially reversible encephalopathy. However, in many patients seizures reflect severe, often multifactorial cerebral insult with variable morphological changes, EEG patterns and clinical outcome.
In a retrospective study, the authors evaluated the initial and follow-up radiographs and ventilation perfusion (V/Q) scans of 12 boys and girls with lobar emphysema who were treated conservatively and were followed up for a median of 3 years (range, 6 months to 12 years). All 12 boys and girls underwent anteroposterior and lateral chest radiography at admission and at varying intervals thereafter. Ten patients underwent V/Q lung scanning; six of these procedures were performed within 4 weeks of initial radiography. Six patients underwent follow-up V/Q scanning. Thoracic computed tomography was performed in four patients at admission. In all 12 cases gradual improvement in symptoms was paralleled by improvements in serial chest radiographs and, in six cases, by improvements in V/Q scans. The affected lobe appeared to be less hyperinflated on follow-up radiographs. For those children who showed functional improvement, ventilation improved more than perfusion, as demonstrated on the V/Q scans. At initial radiography the adjacent "collapsed" lobe was seen to function well, suggesting that it was only compressed.
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Practical guidelines for continuous single channel EEG monitoring using the Cerebral Function Analysing Monitor (CFAM) have been outlined based on experience of 54 critically ill comatose and/or paralysed sedated children monitored for up to 9 days during the acute phase of illness. Fall in amplitude and slowing of frequency following either a cerebral insult or barbiturate administration as well as paroxysmal events were readily recognisable in the CFAM traces. Such changes could be used to (1) identify effects of cerebral insults (acute or cumulative), (2) recognise unstable patients exquisitely sensitive to aspects of standard care and (3) evaluate seizure control. Despite these useful contributions to clinical care, significant limitations were apparent. It is recommended that CFAM monitoring should be combined with serial conventional EEG recording in order to check the appropriateness of the cortical areas being monitored, the quality and type of signal being processed as well as the significance of the 1 or 2 channel CFAM findings in relation to global cerebral function.
A comparative study of the efficacy of pH sensitive enteric coated microspheres (ECM) with an enteric coated tablet (ECT) pancreatic enzyme preparation was carried out in 20 children with cystic fibrosis in a double-blind double-placebo crossover manner. Steatorrhoea was assessed by 3 day faecal fat analysis and dosage of medication, stool frequency and consistency; abdominal pain and appetite were documented by a patient-kept diary card. ECM controlled steatorrhoea (11.8 +/- 9.2 g vs 23.2 +/- 18.9 g, P less than 0.02), stool frequency (1.7 +/- 0.6 vs 2.1 +/- 0.9, P less than 0.01) and abdominal pain (8.8 +/- 13.8 vs 23.4 +/- 24.1, P less than 0.05) significantly better than ECT. Out of 20 patients 17 preferred ECM to ECT (P less than 0.00036). ECM preparations should allow more satisfactory dietary management of patients with cystic fibrosis with longterm beneficial effect.
Cranial computed tomography (CT) scans and invasive intracranial pressure (ICP) measurements were reviewed for 34 children with non-traumatic coma from various causes. CT scan features including focal or generalised changes in density were noted and changes in cerebrospinal fluid (CSF) spaces were graded and correlated with the level of maximum ICP in the first 12 hours of treatment and monitoring. Seven patients had normal findings and 27 had abnormal scans. Nineteen of the 27 patients with abnormal scans had generalised abnormalities with varying degrees of loss of CSF space. Seventeen of these 19 patients had pressures greater than 15 mmHg. The highest pressures being found in those with the greatest degree of CSF space obliteration. In the remaining 8 patients, who had focal abnormalities either within the basal ganglia or cerebral hemisphere, there was no relationship between local CSF space obliteration and the level of ICP. In the 7 patients with normal scans three had ICPs of 20 mmHg or greater in the first 12 hours of monitoring and a further two also developed an ICP of this level 13-36 hours after the initiation of monitoring. In non-traumatic coma of various causes there is an association between loss of CSF space and increased ICP. However, there were two important exceptions to this; firstly in patients with focal abnormalities either within the basal ganglia or cerebral hemisphere, in whom the loss of adjacent CSF spaces was not a good indicator of generalised raised ICP; secondly some patients with normal scans, in whom this finding did not indicate normal ICP nor ensure that it remained at this level.
The long term results of treatment of inhalation of foreign bodies in a district children's hospital and in a tertiary referral centre were reviewed by clinical assessment, chest radiography, and standard four view 81mKr ventilation/99mTc macroaggregated albumin perfusion imaging (V/Q lung scan). The overall incidence in the population served by the district hospital was roughly one in 14,000/year. Of the 12 children reviewed there, three had abnormal chest radiographs and four had abnormal V/Q scans as a result of inhalation of the foreign bodies. Of 21 children treated and reviewed at the referral centre, eight had abnormal chest radiographs, and 14 had abnormal V/Q lung scans. Three factors were assessed for prognostic importance: site of impaction, initial radiographic appearance, and time before removal. A child who had inhaled a foreign body into the left lung and who had collapse/consolidation on the initial chest radiograph was at greatest risk of long term complications. These children merit close follow up.
Seven cases of cystic fibrosis complicated by chronic salt depletion and failure to thrive were studied. After replacement of the salt deficit, the metabolic abnormalities resolved, and weight gain was rapid. This should be considered as a differential diagnosis in children who have been diagnosed as having cystic fibrosis, but who fail to thrive despite standard treatment.
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There is no report analysing pediatric severity scoring systems in British Intensive Therapy Units (ICUs). Two previously reported pediatric severity scoring systems, the Admission Physiologic Stability Index (APSI) and the Organ System Failure (OSF) score were evaluated for 151 patients. The APSI was higher for children who died than for those who lived (p less than 0.001). This difference reflected the sharp distinction between the APSI for children who left intensive care within 24 h and those remaining in ICU longer than 24 h (p less than 0.001). For children remaining in ICU longer than 24 h, there was a large overlap of APSI scores, and the APSI did not discriminate between children in the overlap region who lived and those who died (p = 0.054). There was underscoring of neurological patients; the APSI did not differentiate neurological patients whole lived and those who died (p greater than 0.10). The OSF also underscored neurological patients. Increasing number of organ systems failed was associated with increasing mortality. In contrast to previous reports, however, the mortality rate was unaffected by whether the total number of systems failed simultaneously or non-simultaneously. There is still a need for a comprehensive yet simple pediatric scoring system for comparing the efficacy and outcome of pediatric intensive care in different ICUs in different countries.
To further define the clinical, pathologic, and biochemical features of hemorrhagic shock and encephalopathy syndrome, we studied 25 affected children (aged 3 months to 14 years) admitted to a single center between 1982 and 1985. A prodromal illness comprising vomiting, diarrhea, listlessness, and fever was present in 84% of the cases. Acute onset of shock, convulsions and coma, bleeding (or laboratory evidence of disseminated intravascular coagulation), elevated plasma activity of hepatic enzymes, acidosis, and impaired renal function was present in every case. Twenty patients died, and all the survivors are neurologically damaged. At postmortem examination, intravascular microthrombi coexisting with hemorrhages and petechiae were found in most organs. Centrilobular liver necrosis and cerebral edema were prominent features. No microbiologic cause for the disorder was identified, but decreased plasma levels of the protease inhibitors alpha 1-antitrypsin and alpha 2-macroglobulin, together with increased levels of circulating proteolytic enzymes, were frequently present. An overrepresentation of the uncommon variant phenotypes of alpha 1-antitrypsin was found in first-degree relatives of affected patients (four had the MZ phenotype, and one each the MS or MC phenotype, of 19 relatives studied). Abnormal accumulation of alpha 1-antitrypsin was detected immunohistochemically in the livers of six of the patients. Defective protease inhibitor production or release may be involved in the pathogenesis of the disorder.
Thiopentone anaesthesia was used in the treatment of seizures in 19 infants and young children. Nine had a pre-existing seizure disorder without acute cerebral injury and 10 had status epilepticus secondary to an acute cerebral illness. Clinical details, drug levels and findings from serial multichannel electroencephalograms (EEG) and continuous 1 to 2 channel signal processed EEGs using a Cerebral Function Analysing Monitor (CFAM) were reviewed to evaluate the contribution of these techniques to clinical management. Initial EEGs showed discharges that were not generalised in 16/19 patients. This necessitated the use of 7 different montages for continuous monitoring. Three different CFAM patterns representing particular EEG paroxysmal findings were seen. Acute seizure control was achieved in all 19 patients. All patients with a pre-existing seizure disorder survived, although 4/10 with an acute cerebral illness died. During treatment, patterns of burst-suppression to electrocerebral silence (ECS) were eventually seen in 16/19 patients and repeat EEGs demonstrated concordant multi-channel findings in 8/8. Although increasing thiopentone levels (13 patients) correlated with deeper suppression of cortical electrical activity, there was marked inter-patient variability. Inotropic support was not required during the period of treatment even when ECS was induced. In young children a combination of multi-channel EEG and continuous 1 to 2 channel monitoring has advantages over either method used alone. In individual patients on-line monitoring can be easily incorporated in a protocol of controlled cortical suppression and drug titration for seizure control.
The arterial blood pressure, intracranial pressure, and organ system failure scores were reviewed for 49 infants and children with non-traumatic coma from various causes. The neurological outcome was good in 21 patients, moderate in five, and poor in 23. There was no significant difference in maximum intracranial pressures between patients with a good outcome and those with a poor one, but patients with a poor outcome had significantly lower minimum cerebral perfusion pressures. During the period of admission 18 patients had cardiovascular failure, none had renal failure, and two developed severe coagulopathy. Seventeen of the 19 patients in whom at least one of these systems failed died. Our findings emphasise the diversity of illnesses associated with raised intracranial pressure in children and the number who develop multiple organ failure, and the values and limitations of using minimum cerebral perfusion pressure and the organ system failure scores as guides to severity of illness and prognosis.
Forty eight comatose children had electroencephalograms (EEG) recorded during the acute phase of their illnesses. These were classified according to a simple grading system and the findings correlated with the presence of seizures, deep coma, minimum cerebral perfusion pressure, and eventual neurological outcome. Serial EEGs proved important, particularly when slow activity was seen initially. None of the 20 patients who showed low amplitude EEG activity or electrocerebral silence at any stage of the acute illness did well. Discharges were seen in only 13 of the 29 patients with seizures and their presence did not correlate with outcome except in five patients with a distinctive pattern of discharges, none of whom had a good outcome. EEG findings associated with poor outcome did not always correlate with the clinical assessment of deep coma, emphasising the difficulties of neurological evaluation in these patients. Five of the patients with cerebral perfusion pressures greater than 42 mm Hg had a poor outcome that was predicted by serial EEGs. In nine patients with a minimum cerebral perfusion pressure in the borderline range 38-42 mm Hg the EEG was useful as an indication of the outcome. The EEG reflects changes in cerebral function which may be due to multifactorial or repeated insults. An EEG is therefore important in both the initial assessment and as an indicator of the neurological outcome, particularly in those patients in whom the cerebral perfusion pressure has apparently been adequate or within the borderline range.
Recent studies of Guillain-Barré syndrome in adults have shown that the requirement for assisted ventilation correlates closely with a poor outcome, and the need for ventilation is now being used as an indication for plasmapheresis. As comparable studies in children have not been performed we reviewed our experience of patients who had Guillain-Barré syndrome severe enough to require assisted ventilation. In a group of 11 children two died in the acute stage of the illness and nine made an excellent recovery. It would seem that the need for mechanical ventilation is not necessarily a bad prognostic factor for neurological recovery in children.