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Biomedical subjects

H Kilham

Publications and source records attributed to H Kilham.

At least 19 recordsLinked to original sources

Investigation of suspected deficient Fas-mediated apoptosis in a father and son.

BACKGROUND: A 2-year-old boy presented with symptoms consistent with a diagnosis of autoimmune lymphoproliferative syndrome (ALPS). His father had been splenectomized at age 12 with similar symptoms. ALPS is a rare hereditary syndrome that may result from a functional defect in Fas-mediated apoptosis. METHODS: Peripheral blood lymphocytes (PBL) and splenic lymphocytes from the patient and PBL from his father and a normal control were analyzed for surface Fas expression. They were then stimulated with an anti-Fas monoclonal antibody (DX2). Apoptosis was assayed by flow cytometry at 0, 20, 28, and 34 h. RESULTS: There was no significant difference in expression of Fas (CD95) in the PBL of the patient, his father, or the normal control, or the splenic lymphocytes. Compared with the normal control, the PBL of the patient and his father failed to progress to apoptosis. They also contained a markedly elevated proportion of CD3+CD4-CD8- "double-negative" cells. CONCLUSIONS: PBL from both the patient and his father expressed CD95, but failed to proceed to apoptosis after stimulation, suggesting a functional defect. These results and the clinical presentation are consistent with published descriptions of ALPS.

Adult↗

Parent-requested treatment.

The decision about EPO was referred to and made by the Drug Committee, a committee of physicians, nurses and pharmacists. This committee has perforce to make decisions about drugs and vaccines, decisions which sometimes have a significant ethical component due to concerns about cost, safety and efficacy. Our hospital is considering developing a Clinical Ethics Advisory Committee, to assist with difficult ethical decisions such as this one. Should such a committee be asked to make acute ethical judgements on patient management? Larcher describes his ideal Hospital Ethics Committee as nonprescriptive, and suggests a more appropriate role is retrospective analysis and reflective discussion of clinical ethical problems. Such discussion may help with future rather than current management issues, and can help support clinicians in their decisions and hospital staff in their management of patients.

Anemia↗

"On the spot' vaccination: does it work?

OBJECTIVE: To trial and evaluate a system of "on the spot' vaccination for children up to the age of 15 years in the Early Childhood Centres of the Central Sydney Area Health Service, at the Royal Alexandra Hospital for Children and in a number of general practices in the area. METHODOLOGY: A brief questionnaire was used to collect data from parents and health care professionals about the child's vaccination status and vaccines given "on the spot'. RESULTS: Over an 8 week period in August-September 1993, 5162 questionnaires were completed; 71% of children were up to date with their vaccination. If Haemophilus influenzae type b vaccine, which had been introduced only 2 months before commencement of the study, was excluded, 84% of the children were up to date. A total of 441 children were given 663 vaccinations "on the spot'. Very few children were too ill to be vaccinated (6%). However, only 30% of those who needed vaccination "on the spot' actually received it (441 of 1480), and only 41% (24 of 58) of a subset of those who were not vaccinated were known to have complied 1 month later. Children attending Early Childhood Centres were younger than children attending general practices or the hospital. CONCLUSIONS: A high proportion of children who attended for routine or acute health care had vaccinations overdue (30%). If this scheme could be continued and expanded it would have an important impact on vaccination coverage, and hence on the incidence of vaccine-preventable diseases.

Child↗

Review of intubation in severe laryngotracheobronchitis.

Of 208 children who required relief of severe airway obstruction due to laryngotracheobronchitis by an artificial airway (nasotracheal intubation or tracheostomy) during a 10-year-period, 181 (87%) were intubated and later extubated. Twenty-seven children (13%) had tracheostomies performed. The tracheostomies were for severe subglottic narrowing precluding the passage of an adequate size endotracheal tube in 10 children, and for severe endotracheal tube trauma in 17 children. Five children developed acquired subglottic stenosis (2.4% of 208) and 1 of these has a retained tracheostomy. One child died of cardiac disease. The remaining 202 children had no long-term complications of laryngotracheobronchitis, intubation, or tracheostomy. It is concluded that nasotracheal intubation is a satisfactory artificial airway for laryngotracheobronchitis. Endoscopic evaluation in a selected group of these children will identify those with significant intubation trauma or severe subglottic narrowing in whom continued intubation may cause permanent subglottic damage. The low incidence of acquired subglottic stenosis in this series supports the practice of selective endoscopy and tracheostomy.

Airway Obstruction↗

Entrapment.

Explore the source record for details and available documents.

Child↗

Outcome of children with hematologic malignancy who are admitted to an intensive care unit.

Sixty-four (48%) of 133 children with hematologic malignancy who were admitted to three pediatric ICUs died. Children who required management because of airway obstruction or after general anesthesia had the best outlook (mortality rate of 7% or less); those children who required major circulatory support or mechanical ventilation for hypoxemia did poorly (mortality rate of 84% or greater). Certain conditions in children with hematologic malignancy that require intensive care are associated with a mortality rate of approximately 75%. These include the following: suspected sepsis, interstitial pneumonitis, encephalopathy due to sepsis or hemorrhage. In children with these life-threatening conditions, therapy must be improved because at this stage, the patients do not benefit from admission to the ICU.

Anemia, Aplastic↗

Artificial ventilation in severe pertussis.

A retrospective review was conducted of all children admitted to our intensive care unit over eight years with a diagnosis of pertussis that had been proved on culture. Altogether 789 children were seen as outpatients and inpatients. Twenty four of these children were admitted to the intensive care unit, 13 of whom required ventilatory support; two of the ventilated patients died. Intubation and ventilation were usually started for appreciable apnoea. Most patients requiring support were less than 3 months of age and required intervention within the first 16 days of cough. For these patients ventilation was neither difficult nor prolonged. Coughing spasms were not a problem and intubation and ventilation appeared to attenuate the progress of the disease. The presence of severe bacterial pneumonia associated with difficult ventilation requiring neuromuscular paralysis indicated a poor prognosis. It is suggested that intubation and ventilation can be safely used in very severe pertussis infection and, because of their greater risk of hypoxic damage and death, it should not be reserved as a last resort in critically ill infants.

Apnea↗

Quinine poisoning in children.

The accidental ingestion of quinine by children causes significant morbidity and mortality. We have reviewed quinine poisoning as presented in the medical literature and our experience of paediatric quinine poisoning during the period from January 1975 to September 1986. The clinical features that were seen in our patients were similar to those that are described in larger series. In our series, 13 children were identified, of whom 11 children were aged one to two years. Ten children either remained asymptomatic or made a complete recovery. Two children had a persisting major deficit and one died. Once ingestion of quinine has occurred, absorption should be prevented by the emptying of the stomach with ipecacuanha or gastric lavage promptly and then by the administration of activated charcoal. Once toxicity develops, supportive therapy should be given, but there is no specific therapy to reduce toxicity or to enhance elimination. Physicians who prescribe quinine should be aware of the potential dangers to small children.

Adolescent↗

Severe upper airway obstruction.

This article describes management of newborn and older children with severe upper airway obstruction, that is, those needing or likely to need airway support for their intact survival. These children have more to gain from optimal care, and more to lose from any error than most other children requiring admission to a pediatric intensive care unit.

Airway Obstruction↗

Dicarboxylic aciduria: the response to fasting.

The urine of a child who presented with an episode of a disease resembling Reye's syndrome was found to contain large quantities of the dicarboxylic acids adipic and suberic acids, as well as the glycine conjugate of suberic acid, suberyl glycine. A variety of other dicarboxylic acids, both saturated and unsaturated, were also found in the urine at the time of the attack. It was found that the excretion of these unusual metabolites could be markedly increased by fasting for periods of greater than 10 h. These results indicate that the patient may have a defect in fatty acid oxidation which becomes clinically significant during periods of prolonged fasting.

Adipates↗

Running, walking, and hyperventilation causing asthma in children.

To examine further the relation between type of exercise, workload, ventilation, and exercise-induced asthma, we compared treadmill walking with treadmill running and treadmill running with isocapnic hyperventilation in separate studies in children and adolescents. Inspired air conditions were identical during each pair of tests. Walking and running with similar minute ventilation and oxygen consumption were followed by similar falls in peak expiratory flow rate as were running and hyperventilation with similar minute ventilation and end-tidal carbon dioxide tension. This study supports the concept that hyperventilation is a central mechanism in exercise-induced asthma.

Adolescent↗