Familial asplenia.
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Biomedical subjects
Publications and source records attributed to J Gillis.
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We have recently reported abnormal insulin-like growth factor-II (IGF-II) mRNA levels in a number of human colorectal adenocarcinomas. Using an IGF-II radioimmunoassay, we have now detected high levels of both 10-kDa and 7.5-kDa IGF-II species (2,370 ng/g) in a right colon tumor showing a 800-fold IGF-II gene over-expression in comparison to the normal adjacent tissue. The higher-molecular-mass form represents 74% of the total immunoreactive IGF-II detected in the tumor. This form appears to be less reactive in the radioreceptor assay than in the radioimmunoassay. The insulin-like growth factor-I (IGF-I) concentration in the tumor is low. The patient's pre-operative serum IGF-II level is not increased and the proportion of the 10-kDa species is normal. In addition, the IGF-II/IGF-I ratio is 3 in the serum and 308 in the tumor. Our results show that the very high IGF-II level produced by the tumor does not influence the seric concentration of the growth factor.
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.
The issue of the ventilator-dependent child is a relatively-new one in Australia. Ventilator-dependent children pose complex and unique ethical, medical, economic and psychological problems. The experience of two Australian centres that are involved with the care of ventilator-dependent children is reported. Most of these children now are being cared for at home. Aspects of home care are outlined. After the initial period, the technical aspects are not a problem for most parents for whom the major issues are the provision and funding of nursing support. The complex ethical issues that are involved are discussed. It is concluded that undergoing ventilation at home rather than in a hospital appears to make the best of an otherwise almost-intolerable situation for ventilator-dependent children, but that much more information is required about the outcome for these children and the long-term psychosocial impact of this treatment.
Between 1982 and 1985, 14 infants aged 3-26 weeks presented with severe hypoxic episodes as a result of the 'near miss' sudden infant death syndrome (SIDS). They all had metabolic acidosis, cardiovascular instability, acute renal failure, ischaemic colitis, or acute neurological dysfunction. Investigation of the cause excluded infection and trauma, or a primary metabolic, pulmonary, cardiac, or seizure disorder. Seven infants were deeply comatose on admission, never regained consciousness, and died within 60 hours. A characteristic evolution of hypoxic-ischaemic encephalopathy not previously clearly described after near miss SIDS was seen in the seven who lived. Five of the seven were conscious within one hour of resuscitation and showed a striking interval of near normality before neurological deterioration that was characterised by status epilepticus, deep coma, and brain stem dysfunction from 36-96 hours after the event. A biphasic course was not apparent in the remaining two, each of whom was comatose on admission, though refractory seizures did develop. Computed tomograms of the brain more than a week after the event showed cortical infarction or cerebral atrophy. Six of the survivors, followed up from 16-55 months, have serious residual deficits including spastic quadriplegia, delayed development, cortical blindness, or infantile spasms.
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.
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.
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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.
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.
A 21 month old girl who presented with what seemed to be hypertensive encephalopathy is described. Although her encephalopathy resolved with antihypertensive treatment, subsequent investigations revealed haemophilus meningitis.
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Twenty-six cases of Reye syndrome from The Children's Hospital, Camperdown, Australia, occurring between 1973 and 1982 were reviewed. Of these, 20 cases met the US Public Health Service Centers for Disease Control criteria for the diagnosis of Reye syndrome. Aspirin or salicylate ingestion had occurred in only one of the 20 cases (5%), and paracetamol (acetaminophen) had been administered in only six of the cases (30%). Pathologic confirmation of the diagnosis of Reye syndrome was accomplished in 90% of the cases. The incidence of Reye syndrome in New South Wales, Australia, is estimated from this study to be approximately nine cases per 1 million children compared with recent US data of ten to 20 cases per 1 million children and three to seven cases per 1 million children in Great Britain. The mortality for these Reye syndrome cases in Australia was 45% as compared with a 32% case-fatality rate in the United States. In Australia, the pediatric usage of aspirin has been extremely low for the past 25 years (less than 1% of total dosage units sold), with paracetamol (acetaminophen) dominating the pediatric analgesic and antipyretic market. Reye syndrome may be disappearing from Australia despite a total lack of association with salicylates or aspirin ingestion, since there were no cases found at The Children's Hospital in 1983, 1984, or 1985.
We retrospectively reviewed the results of 42 cardiopulmonary arrests occurring over 1 yr in the general ward of a pediatric hospital. These data were compared to those of a similar study done 10 yr previously in the same institution. Patients were divided into those having pure respiratory arrest and those who also had cardiac arrest. In the most recent series, overall 6-month survival was 17%; however, only 9% of the cardiac arrest patients survived. Ten years previously, the survival rate from cardiac arrest was 11%. In both series, pure respiratory arrest had a significantly better outcome than cardiopulmonary arrest, and predictors of nonsurvival were a duration of arrest greater than 15 min and the administration of more than one iv bolus of epinephrine. During the more recent series, sepsis and upper airway problems produced fewer arrests. There was one neurologically damaged survivor in each study period. Our study confirms that the outcome of pediatric cardiac arrest is poor when arrest occurs in the hospital.