To review our experience of children with meningococcal septicaemia, and to validate, in our grou... more To review our experience of children with meningococcal septicaemia, and to validate, in our group, severity scores used in different populations to predict outcome. Retrospective review of case notes and charts. 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. Ages ranged from 4 months to 16 years, with a median age of 20 months. The median meningococcal score was 4 and the median PRISM score was 20, with scores above these being significantly associated with death (P < 0.0001). Thirty-two children (91%) received infusions of colloid for hypovolaemia and twenty-nine (83%) received one or more inotropic drugs. Twenty-one children (60%) required mechanical ventilation for a median of 16.5 h (range 7-574). Seven children (20%) underwent plasmapheresis. Six children (17%) underwent haemofiltration and two (6%), peritoneal dialysis. One patient received extracorporeal membrane oxygenation (ECMO) because of circulatory failure. Twenty-one children (60%) developed disseminated intravascular coagulation, renal failure and/or skin or limb necrosis. The overall survival was 66%, and all survivors are functionally normal. The mortality from the disease remains at 34% despite the technological advances in intensive care. The PRISM and meningococcal scores are useful in predicting outcome. Novel methods of treatment (e.g., plasmapheresis or ECMO) may be valuable.
To review the outcome of bone marrow transplant (BMT) recipients admitted to a pediatric intensiv... more To review the outcome of bone marrow transplant (BMT) recipients admitted to a pediatric intensive care unit (ICU) and attempt to identify admission characteristics that might accurately predict a poor outcome. Retrospective case-note review. Pediatric ICU of a tertiary teaching hospital. A total of 40 BMT recipients, accounting for 57 admissions to the ICU, in the 5 yrs between 1994 and 1998 were identified. Median time to ICU admission after BMT was 42 days. Of the 40 patients admitted to ICU, 11 (22.5%) are still alive, with a median time of follow-up since their most recent ICU admission of 587 days (absolute range, 308-1803 days). A total of 32 of 57 admissions (56.1%) resulted in the patient's discharge from the ICU, and 21 admissions (36.8%) resulted in survival to at least 30 days after discharge. There was no difference between the survivors and nonsurvivors in terms of underlying diagnoses, age at BMT, or time to ICU admission after BMT. Type of BMT, conditioning regimen, and presence of significant graft vs. host disease was not found to influence outcome. Although patients who died in the ICU had a significantly longer length of stay compared with the survivors (median, 7.9 days, vs. 2.1 days, p =.02), 11 of 21 admissions (52.4%) associated with survival to 30 days post-ICU discharge were of >or=2 days of duration, the longest being 22.8 days. Thirty-one of 40 patients (77.5%) required intubation and mechanical ventilation during 36 of the 57 admissions, and 15 of these episodes (41.6%) ended with the patient's discharge from the ICU. Of ten patients with respiratory failure associated with pulmonary infection, there were no survivors among those who remained ventilated at 48 hrs (n = 8). Four patients who required mechanical ventilation (12.9%) were alive at the 6-month follow-up. The majority of patients who died in the ICU did so after either withdrawal (65%) or limitation (22%) of treatment. Despite the generally poor prognosis for pediatric patients admitted to the ICU after BMT, intensive care continues to play an important role in the care of these patients. Although it is clear that patients who require mechanical ventilation have a worse prognosis, we were unable to identify factors that accurately predict with 100% sensitivity which patients will not survive. Those patients requiring mechanical ventilation due to pneumonitis have a particularly poor outcome, and our findings support the limitation of intensive care in certain circumstances. Decisions regarding treatment options and limitation of care in this group of patients should be based on ongoing outcome research in this field.
To present the UK experience in genetic diagnoses of surfactant protein dysfunction disorders and... more To present the UK experience in genetic diagnoses of surfactant protein dysfunction disorders and develop a referral algorithm for neonates and children with persistent respiratory problems. Between 2006 and 2011, 427 cases were referred for surfactant mutation analyses to the North East Thames Regional Molecular Genetics Laboratory at Great Ormond Street Hospital, London. The results were reviewed and referring physicians of mutation positive cases contacted to complete a questionnaire providing clinical, radiological, histological and outcome information. 25 new cases were found to have genetic mutations for surfactant dysfunction disorders (7.5%), with six resulting in surfactant protein B dysfunction, seven surfactant protein C dysfunction and 12 ATP-binding cassette subfamily A member 3 (ABCA3) dysfunction. The referrals were from 15 different paediatric centres. In addition, three affected surfactant protein B (SFTPB) cases were prenatal diagnoses, following the birth of previously affected children. The majority of the confirmed cases (23 of 25) were born after 37 weeks gestation. All children with SFTPB dysfunction and the majority of ABCA3 patients presented with respiratory distress at birth. All SFTPB cases died from intractable respiratory failure. The outcome for ABCA3 mutations was variable with seven survivors. The clinical and radiological presentation of surfactant protein C (SFTPC) patients suggested mainly interstitial lung process with the majority surviving on medication. Surfactant mutation analysis is now well established in the UK and allows better genetic diagnosis and counselling. The rarity of the condition makes it difficult to develop a validated algorithm for genetic evaluation with a need for international networking. Referrals need to be rationalised for the service to be time and cost effective.
To review our experience of children with meningococcal septicaemia, and to validate, in our grou... more To review our experience of children with meningococcal septicaemia, and to validate, in our group, severity scores used in different populations to predict outcome. Retrospective review of case notes and charts. 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. Ages ranged from 4 months to 16 years, with a median age of 20 months. The median meningococcal score was 4 and the median PRISM score was 20, with scores above these being significantly associated with death (P < 0.0001). Thirty-two children (91%) received infusions of colloid for hypovolaemia and twenty-nine (83%) received one or more inotropic drugs. Twenty-one children (60%) required mechanical ventilation for a median of 16.5 h (range 7-574). Seven children (20%) underwent plasmapheresis. Six children (17%) underwent haemofiltration and two (6%), peritoneal dialysis. One patient received extracorporeal membrane oxygenation (ECMO) because of circulatory failure. Twenty-one children (60%) developed disseminated intravascular coagulation, renal failure and/or skin or limb necrosis. The overall survival was 66%, and all survivors are functionally normal. The mortality from the disease remains at 34% despite the technological advances in intensive care. The PRISM and meningococcal scores are useful in predicting outcome. Novel methods of treatment (e.g., plasmapheresis or ECMO) may be valuable.
To review the outcome of bone marrow transplant (BMT) recipients admitted to a pediatric intensiv... more To review the outcome of bone marrow transplant (BMT) recipients admitted to a pediatric intensive care unit (ICU) and attempt to identify admission characteristics that might accurately predict a poor outcome. Retrospective case-note review. Pediatric ICU of a tertiary teaching hospital. A total of 40 BMT recipients, accounting for 57 admissions to the ICU, in the 5 yrs between 1994 and 1998 were identified. Median time to ICU admission after BMT was 42 days. Of the 40 patients admitted to ICU, 11 (22.5%) are still alive, with a median time of follow-up since their most recent ICU admission of 587 days (absolute range, 308-1803 days). A total of 32 of 57 admissions (56.1%) resulted in the patient's discharge from the ICU, and 21 admissions (36.8%) resulted in survival to at least 30 days after discharge. There was no difference between the survivors and nonsurvivors in terms of underlying diagnoses, age at BMT, or time to ICU admission after BMT. Type of BMT, conditioning regimen, and presence of significant graft vs. host disease was not found to influence outcome. Although patients who died in the ICU had a significantly longer length of stay compared with the survivors (median, 7.9 days, vs. 2.1 days, p =.02), 11 of 21 admissions (52.4%) associated with survival to 30 days post-ICU discharge were of >or=2 days of duration, the longest being 22.8 days. Thirty-one of 40 patients (77.5%) required intubation and mechanical ventilation during 36 of the 57 admissions, and 15 of these episodes (41.6%) ended with the patient's discharge from the ICU. Of ten patients with respiratory failure associated with pulmonary infection, there were no survivors among those who remained ventilated at 48 hrs (n = 8). Four patients who required mechanical ventilation (12.9%) were alive at the 6-month follow-up. The majority of patients who died in the ICU did so after either withdrawal (65%) or limitation (22%) of treatment. Despite the generally poor prognosis for pediatric patients admitted to the ICU after BMT, intensive care continues to play an important role in the care of these patients. Although it is clear that patients who require mechanical ventilation have a worse prognosis, we were unable to identify factors that accurately predict with 100% sensitivity which patients will not survive. Those patients requiring mechanical ventilation due to pneumonitis have a particularly poor outcome, and our findings support the limitation of intensive care in certain circumstances. Decisions regarding treatment options and limitation of care in this group of patients should be based on ongoing outcome research in this field.
To present the UK experience in genetic diagnoses of surfactant protein dysfunction disorders and... more To present the UK experience in genetic diagnoses of surfactant protein dysfunction disorders and develop a referral algorithm for neonates and children with persistent respiratory problems. Between 2006 and 2011, 427 cases were referred for surfactant mutation analyses to the North East Thames Regional Molecular Genetics Laboratory at Great Ormond Street Hospital, London. The results were reviewed and referring physicians of mutation positive cases contacted to complete a questionnaire providing clinical, radiological, histological and outcome information. 25 new cases were found to have genetic mutations for surfactant dysfunction disorders (7.5%), with six resulting in surfactant protein B dysfunction, seven surfactant protein C dysfunction and 12 ATP-binding cassette subfamily A member 3 (ABCA3) dysfunction. The referrals were from 15 different paediatric centres. In addition, three affected surfactant protein B (SFTPB) cases were prenatal diagnoses, following the birth of previously affected children. The majority of the confirmed cases (23 of 25) were born after 37 weeks gestation. All children with SFTPB dysfunction and the majority of ABCA3 patients presented with respiratory distress at birth. All SFTPB cases died from intractable respiratory failure. The outcome for ABCA3 mutations was variable with seven survivors. The clinical and radiological presentation of surfactant protein C (SFTPC) patients suggested mainly interstitial lung process with the majority surviving on medication. Surfactant mutation analysis is now well established in the UK and allows better genetic diagnosis and counselling. The rarity of the condition makes it difficult to develop a validated algorithm for genetic evaluation with a need for international networking. Referrals need to be rationalised for the service to be time and cost effective.
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