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Immunobiology of paediatric intracranial tumours. A preliminary report.

Preliminary findings in the evaluation of the immune response of children with primary neoplasms of the CNS, mainly medulloblastomas, are reported and discussed. A broad scheme for the monitoring of B- and T-cell-dependent immunity and of delayed hypersensitivity reactions in this type of patient is presented. The most important immunobiological findings are discussed. Special attention is given to the striking failure of the T-cell-dependent pool (currently identified by "active" RFC and blastigenesis tests) and to the remarkable decrease of hypersensitivity reactions (depressed skin-test response), both of which seem to be related to the degree of malignancy of the tumour. A very peculiar feature, i.e., the appearance of cells with natural cytotoxic activity, is dealt with in some detail. Our present knowledge concerning the immunobiology of primary CNS neoplasms is still very incomplete, but seems to suggest a possible role for immunotherapy in paediatric neurosurgery.

B-Lymphocytes

Aspects of tropical paediatrics.

Malnutrition interacting with infectious and parasitic diseases are the main causes of the appalling mortality in childhood in the tropics. The most important single safeguard against these in infancy is breast feeding and the trend now evident to abandon this is a disaster which demands urgent attention. Reasons for this trend are discussed. Efforts to control infectious diseases, other than smallpox, have had little success and the emergence and spread of dengue haemorrhagic fever in S.E. Asia have added new dimensions to the problem. Malaria is still widely prevalent in the tropics and falciparum malaria, holoendemic in much of Africa, remains a major cause of death with its most serious impact on pregnant women and children. The emergence and spread of drug resistant strains of this parasite in parts of the world is a cause for serious concern. Quartan malaria is also an insidious corruptor of health in childhood and commonly causes the nephrotic syndrome. Neonatal jaundice, often associated with G6PD deficiency, is increasing in frequency in urban areas of Africa and now constitutes a significant hazard to the newborn and requires urgent investigation. These problems in tropical paediatrics indicate the need for urgent reappraisal of our role as a profession in the affairs of the tropical developing world.

Adolescent

The paediatric syndrome of traumatic myelopathy without demonstrable vertebral injury.

Three detailed case histories are added to those previously published in the literature and from the evidence now available a proposal is made that a paediatric syndrome of traumatic myelopathy without demonstrable vertebral injury be recognised as a definite clinical entity. The mechanisms of injury, evolution, patterns of paralysis and pathological evidence are discussed. The conclusion is drawn that no form of treatment yet known can influence the prognosis in this syndrome.

Child

A paediatric ventilator with a fluidic control system.

A paediatric ventilator has been designed basically as a fluidic-logic controlled T-piece occluder. Inspiratory and expiratory time, inflation pressure limit, positive end-expiratory pressure (PEEP), continuous positive airway pressure (CPAP) and inspiratory flow-rate are controlled independently and intermittent mandatory ventilation (IMV) is available. Warning systems are provided for failure of the driving as, low airway pressure and inspiratory pressure limit. The breathing circuit is isolated from the control and warning systems for ease of sterilization.

Air Pressure

Whole exome sequencing of paediatric patients with Cogan's syndrome to identify monogenic mimics.

OBJECTIVES: Cogan's syndrome (CS) is a rare variable vessel vasculitis, describing sensorineural hearing loss (SNHL), inflammatory ocular disease and vestibular dysfunction. We hypothesized that within paediatric-onset (p)CS, a proportion would have monogenic disease, either autoinflammatory and/or associated with SNHL. METHODS: Whole exome sequencing (WES) was performed and analysed using an in-house pipeline incorporating virtual gene panels for inflammation and SNHL; copy number variant analysis (ExomeDepth); and phenotype-driven variant prioritization (Exomiser). Genetic variants were interpreted by a multi-disciplinary team according to American College of Medical Genetics and Genomics guidelines. RESULTS: Ten patients with a clinical diagnosis of pCS were enrolled. Three/10 (30%) had a monogenic contribution to the phenotype based on Class 4/5 variants: de novo NLRP3 p.T915R (n = 1) associated with Cryopyrin-associated periodic syndrome; MYO7A p.K542Qfs*5 (n = 1) causing SNHL; and HBB homozygous p.E7V causing sickle cell disease (associated with hearing loss and uveitis). A further two cases had possible monogenic contribution with the following rare variants of uncertain significance (class 3): ADGRV1 compound heterozygous variants (n = 1) associated with Usher syndrome; and a novel ALPK1 p.H735P (n = 1), associated with Retinal dystrophy Optic nerve oedema Splenomegaly Anhidrosis Headache (ROSAH) syndrome. CONCLUSIONS: In children presenting with features suggesting CS, genetic screening should be considered before conferring this rare diagnostic label since at least 30% had an alternative monogenic contribution to the phenotype rather than true pCS, with implications for treatment and prognosis. We thus advocate for genetic testing using next-generation sequencing for patients presenting with pCS.

Humans

The Penlon Oxford ventilator. A new ventilator for adult or paediatric use.

A pneumatically powered lung ventilator for anaesthesia and intensive care of both adult and paediatric patients is described. The design criteria were selected to produce a small machine with high power, simple to control, sterilise and service. Laboratory and clinical results are presented andprocedure for clinical use of the controls suggested.

Adult

Paediatric candidates' attitudes to computerized patient management problems in a certifying examination.

In September 1974 candidates who had taken a computerized patient managment problem examination (CPMP), as part of the certification process in paediatrics required by the Royal College of Physicians and Surgeons of Canada, completed a questionnaire designed to elicit their reactions to CPMPs. The results indicated that respondents were favourable to CPMPs, that there was little distraction caused by the equipment, logic or semantics of the problems, and that CPMPs were an acceptable examination technique. It was recommended that, in the future, CPMP examinees be allowed practice time to familiarize themselves with the equipment, that pre-examination instructions should be clarified, and that problems judged inadequate by candidates be revised.

Attitude of Health Personnel

A comparative study of halothane and enflurane in paediatric outpatient anaesthesia.

Two groups of paediatric patients, consisting of 40 patients each, were studied with regard to anaesthesia induction and recovery. Group H was given halothane and Group E received enflurane. The induction time was similar for the two groups. Enflurane had, on average, a shorter recovery time (17 +/- 4 min) than halothane (26 +/- 4min) (P less than 0.05). The mean dose of anaesthetic given, expressed as the product of inspired concentration times minute ventilation, was 2.3 +/- 0.2 1 100% halothane and 2.8 +/- 0.2 1 100% enflurane. If one assumes that the minimum alveolar concentration (MAC) value for enflurane twice that of halothane, group E received a less potent anaesthetic dose. This might explain the similar induction times, and, in part, the shorter recovery time for enflurane in this study. No difference was noted between the two agents with regard to post-operative nausea and vomiting or restlessness.

Adolescent

Child health and paediatrics in the U.S.S.R.

Observations on child health and paediatrics made during a three weeks' visit to the USSR are recorded. There are separate health services for children and their organization is outlined, as are the training and career structure of the many thousands of paediatricians employed. Provision for handicapped children is briefly discussed and a few comments are made on social aspects of child care.

Adolescent

Paediatric home nursing scheme in Gateshead.

A scheme to provide specialised nursing care for sick children in their own homes was begun in Gateshead in 1974. Selected district nurses were retrained in the paediatric unit on which the scheme was based and nursed at home 22 children referred to them by general practitioners as the alternative to hospital admission and 39 discharged to their care by the hospital. Most of the children were aged 3 years or less and came from working-class homes. Most of the mothers who were asked were in favour of the scheme.

Adolescent

An inter-laboratory survey of paediatric bilirubin analyses.

A regional quality control trial of paediatric bilirubin analyses is described. The overall performance of the group was unsatisfactory with an unacceptably high inter-laboratory variation. The use of a common standard produced improvement in performance but it is concluded that the poor performance was also due to methodological problems. This lack of agreement between laboratories is a major problem when patients are transferred between hospitals.

Adult

Three years of bacteriological monitoring in the Department of Paediatric Surgery.

The results of three years of bacteriological monitoring in the Department of Paediatric Surgery at the Ospedale Maggiore Ca'Granda in Milan are reported. The tests carried out involved the patients (faeces, urine, and pus cultures), the environment (operating theatre, incubators), and healthy carriers (upper respiratory tract of nursing and paramedical staff). The aetiopathogenesis of the post-operative infectious complications that occurred in 1974-1975 was investigated with the aid of surveys of the causative pathogens, the type of the surgical interventions, the atmospheric pollution in the operating theatre (mean indicative values), the number of operations per month, and the frequency of positive findings among the carriers. As a parallel investigation, between 1971 and the time of writing of this report the authors monitored the resistance to gentamicin of bacteria belonging to the family Pseudomonadaceae isolated from patients, in view of the constant threat posed by the vicinity of the burns department.

Bacterial Infections

Adsorption of halothane from a paediatric T-piece circuit by activated charcoal.

Experiments were conducted to compare the efficacy of six commonly available charcoals in adsorbing halothane in a paediatric T-piece system. Tests were also done to compare the effect of mass of charcoal and canister shape and size on adsorption and airways resistance. It was found that charcoals vary in their adsorptive capacity and that the size and shape of cannisters is important.

Adsorption

Paediatric assessment centres in Great Britain: implications for Australia.

Assessment centres have become a prominent feature of paediatrics in the United Kingdom. Staffed by a developmental paediatrician and a team of medical and paramedical workers they are valuable for the diagnosis and coordination of management of handicapping conditions in childhood. Both local and regional assessment centres should be established. In the first instance these should be in conjunction with teaching children's hospitals in major cities in Australia.

Australia

Developmental and paediatric care of the pre-school child.

Through an Upjohn Travelling Fellowship I visited 27 experts in childcare and sought their opinions on the privileges, possibilities, and problems in organising developmental and paediatric care for pre-school children in the United Kingdom.The role of the general practitioner was seen by many of the experts clearly. How he is to play it is shrouded in uncertainty. Research is urgently needed both on the tools of surveillance and on the different methods of arranging care.

Age Factors

[Halothane concentrations in the atmosphere of a paediatric operating theatre (author's transl)].

Determinations were made of the halothane concentrations in the air of a paediatric operating theatre during operations. They averaged 5-30 p.p.m. with peaks of 50-70 p.p.m. during induction. The inhalation of halothane vapour was confirmed by demonstrating bromine metabolites in the urine of the operating room personnel. The risk attendant on chronic exposure to halothane vapour should be prevented by the installation of suitable systems for the removal of excess anaesthetic vapours.

Adolescent

Nephroblastoma in the Black child: a review of treatment in the Johannesburg Paediatric Tumour Clinic.

Twenty-five Black children with nephroblastoma who were referred to the Paediatric Tumour Clinic during the preceding 4 years, are reviewed. Results indicate that in the case of a locally advanced, fixed, primary tumour, initial surgery, even if it is combined with chemotherapy, may be a factor in the rapid postoperative development of multiple pulmonary metastases. Pre-operative irradiation appeared to be advantageous for these patients. Long-term chemotherapy holds no advantage over short-term chemotherapy.

Black or African American

Report of an incident during the use of the Servo-ventilator 900 in paediatric anesthesia.

The authors report the occurrence of an incident during the use of the Servo-ventilator 900 for paediatric anesthesia; it consisted in self-oscillation of the gas column contained in the patient respiratory circuit. After having analysed the incident and outlined the various ways of avoiding it, attention is drawn to the importance of the total circuit volume of the respirator. The respiratory circuit chosen for ventilating children, has a compliance of 1.3 ml/cm H2O.

Anesthesia, General