ARTERIALIZATION OF CAPILLARY BLOOD: HISTAMINE IONTOPHORESIS IN ENDOTOXIN SHOCK

Values of pO₂ obtained from capillary blood by histamine iontophoresis were compared with arterial values both before and after the induction of endotoxin shock in the experimental animal. Capillary blood pO₂ levels were found to compare favourably with arterial blood levels in both circumstances. It is suggested that arterialisation of capillary blood by histamine iontophoresis could be used to determine pO₂ levels in the acutely ill child when arterial blood is not readily available.     

UNUSUAL CLINICAL AND ULTRASTRUCTURAL FEATURES IN A BOY WITH BIOCHEMICALLY TYPICAL MANNOSIDOSIS

Abstract. Gordon, B. A., Carson, R. and Haust, M. Daria (Departments of Biochemistry, Paediatrics and Pathology, the Children's Psychiatric Research Institute, and the University of Western Ontario, London, Ontario, Canada). Unusual clinical and ultrastructural features in a boy with biochemically typical mannosidosis. Acta Paediatr Scand, 69: 787, 1980.—A 4½-year-old boy with a history of recurring respiratory tract infections and seizures, and evidence of severe retardation of psychomotor development and growth, lacked the coarse facial features, skeletal changes and other clinical stigmata generally associated with mannosidosis, but the total α-mannosidase activity in his leukocytes, cultured fibroblasts and liver were no more than 10% of the control mean. Studies of the residual α-mannosidase enzyme suggest a specific deficiency of the thermostable isoenzyme with an acidic pH optimum. The α-mannosidase in the fibroblasts of our and another (control) patient with mannosidosis had a reduced affinity for the substrate 4-methylumbelliferyl-α-D-mannoside. Light micros-copy of the liver biopsy showed an increase in connective tissue often distorting the hepatic architecture; numerous tiny vacuoles, small dense and lipid bodies in most hepatocytes, and similar but more extensive changes in sinusoidal cells; and sinusoidal pools of hepatocytic debris. Electron microscopy of hepatocytes revealed vacuoles similar but not identical to those described in reported mannosidosis patients, and in addition several forms of secondary lysosomes; prominent peroxisomes (microbodies); increased numbers of profiles of smooth endoplasmic reticulum; dilated rough endoplasmic reticulum containing traces of fine granulo-fibrillar material; increased numbers of rosettes of α particles of glycogen and reduced numbers of mitochondria with alterations in their distribution, size and configuration. It is believed that the usual clinical and hepatic ultrastructural features in our patient reflect another variant of mannosidosis.     

ADRENOCORTICOTROPHIN AND GLUCOCORTICOID RESPONSE TO EXCHANGE TRANSFUSION

Abstract. Plasma adrenocorticotrophin (ACTH) and glucocorticoid concentrations were measured during and for 3 hours after exchange transfusion in four groups of infants. Transfusions with ACD blood via the umbilical artery were performed on 10 infants of 35–40 weeks gestation and 11 infants of 27 to 33 weeks gestation. Ten transfusions via the umbilical vein were performed with ACD blood and 9 with heparin blood on infants of 32 to 41 weeks gestation. In all four types of transfusion there was a significant washing out of ACTH and glucocorticoids from the baby. An associated rise of plasma ACTH and glucocorticoid levels indicated increased secretion in the larger infants transfused with ACD blood via the umbilical artery. Equivocal results were obtained in the other groups. After all types of transfusion plasma glucocorticoid levels remained relatively constant for one hour and then doubled in the second and third hour. The results suggest that ( a ) exchange transfusion via the umbilical artery is more stressful than that via the umbilical vein, ( b ) the glucocorticoid response of premature infants to exchange transfusion is similar to that of mature infants, ( c ) an unspecified stimulus, which is not solely hypoglycaemia nor the metabolic reaction to a citrate load, results in a rise in plasma glucocorticoid levels 2–3 hours after all types of transfusion.     

Potentially Life-Threatening Cardiac Dysrhythmia in a Child with Selenium Deficiency and Phenylketonuria

ABSTRACT. Selenium deficiency is a frequent finding in children with phenylketonuria (PKU) receiving dietary treatment. We report the occurrence of episodic ventricular tachycardia in a 9-month-old infant with PKU. Serum selenium (Se), 0.13 μmol/1 (normal range 0.28–1.12 μmol/1, mean ± 2 SD), and whole blood glutathione peroxidase (GSHPX), 16 U/g Hb (normal range 19.5–34.3 U/g Hb, mean ± 2 SD), were low. Auscultation of cardiac rhythm is recommended during the routine follow-up of young children with PKU. Se deficiency should be considered in the aetiology of a dysrhythmia and corrected with Se supplementation.     

Stroke Volume and the Three Phase Cardiac Output Rate Relationship with Ventricular Pacing

Knowledge of how stroke volume (SV), and hence cardiac output (CO), changes with ventricular pacing rate (R) constitutes a key aspect of sensor driven, variable rate pacemakers. It has been established that the relationship between CO and pacing rate exhibits three phases for rest and constant exercise. At low rates (phase 1), CO increases with increasing R; with additional rate increase (phase 2), CO either remains constant or increases slightly; and above some critical rate, CO decreases (phase 3). However, the nature of the relationship between SV and pacing rate has not been as clearly described. Therefore, the objectives of this study were (1) to describe and document the relationship between SV and R, and (2) to demonstrate the consequence of this relationship in terms of the three phase CO versus R relationship. In six anesthetized dogs, right ventricular SV was determined from pulmonary artery blood flow measured using an electromagnetic flow meter, and the right ventricle was paced over a range of rates. In general, SV decreased with increasing R, although the exact nature of the relationship varied from animal to animal. The results demonstrate that it is the manner in which SV decreases with increasing R that determines the three phase relationship between CO and R. The relationships described in this study have important implications for choosing pacing rates for patients receiving sensor driven, variable rate pacemakers.     

EFFECTS OF SOMATOSTATIN ANALOGUE SMS 201–995 IN NORMAL MAN

Long-acting somatostatin analogues may be of benefit in certain hypersecretory endocrine and gastrointestinal disorders. The 24 h hormonal and metabolic profiles of six normal male subjects receiving a twice daily subcutaneous injection of one such analogue SMS 201-995, 50 micrograms, has been compared to that obtained following placebo injection. Spontaneous daytime peaks of GH secretion were delayed until 1400 h following SMS 201-995 but nocturnal and total 24 h GH secretion were unaffected. The nocturnal rise in thyrotrophin was abolished by SMS 201-995 but thyroid function was unaffected. Insulin levels were suppressed following SMS 201-995 and the response to meals was inhibited. Glucose intolerance followed main meals. Glucagon levels were suppressed for up to 6 h. Circulating alanine levels were raised between 1200 h and 0600 h and there were intermittent elevations in lactate, pyruvate, glycerol and 3-hydroxybutyrate. Amino acid levels, including branched chain amino acids, were also increased. All six subjects suffered gastrointestinal side-effects. SMS 201-995, 50 micrograms, given twice daily shortly before meals does not suppress 24 h GH secretion, but demonstrates significant effects on metabolism and causes side effects in normal subjects.     

Analgésie autocontrôlée chez les enfants atteints d’anémie falciforme: l’expérience d’un centre canadien

Résumé  Afin d’évaluer’ la prise en charge de la douleur chez des enfants atteints d’anémie falciforme, nous avons revu rétrospectivement les dossiers médicaux de 26 enfants hospitalisés pendant une période de 28 mois, pour crises vaso-occlusives (CVO) et qui ont bénéficié de l’analgésie contr?lée par le patient (ACP) à 54 occasions. Tous ces patients ont eu une ACP à la morphine à l’exception d’un seul qui a re?u de la mépéridine. La dose des bolus, de la perfusion de base et la dose totale de médicament ont beaucoup varié pendant les trois premiers jours du traitement. L’utilisation de morphine chez les enfants qui se sont servis du bolus plus perfusion de base a été significativement plus importante que chez ceux qui n’ont re?u que les bolus. Toutefois, les scores de douleur n’ont pas été significativement différents. Un tiers des enfants a re?u des adjuvants en plus des antalgiques. La nausée (n=6), les vomissements (n=6) et le prurit (n=6) sont survenus chez 11% des patients. II n’y a pas eu d’épisode de sédation excessive ou de dépression respiratoire. Six patients avaient un syndrome thoracique aigu à l’admission, et deux d’entre eux ont d? être admis pour une nuit aux soins intensifs. A deux occasions, les parents ont décidé d’arrêter l’ACP du fait d’une mauvaise analgésie. En nous appuyant sur notre expérience, nous proposons quelques recommandations pour l’amélioration de la qualité de l’ACP dans la prise en charge de la douleur des CVO chez les enfants et les adolescents.

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Summary  To Examine our experience of pain management in children with sickle cell disease (SCD), we retrospectively reviewed the medical charts of 26 patients hospitalized over a 28 month period, for vaso-occlusive crisis (VOC) who used patient controlled analgesia (PCA) on 54 different occasions. All of the patients used morphine PCA, except one who used meperidine. The bolus dose, use of basal infusion and total amount of drug received daily varied greatly, over the first three days of therapy. Morphine use in those admissions which used bolus plus basal PCA was significantly higher than those which used bolus only. However, pain scores were not significantly different. One-third of the admissions used adjuvant pain medication. Nausea (n=6), vomiting (n=6) and pruritus (n=6) each occured in 11% admissions. There were no episodes of excessive sedation or respiratory depression. Six patients had documented acute chest syndrome on admission, two of which necessitated overnight stays in ICU. On two occasions, parents decided to stop their child’s PCA due to dissatisfaction with pain relief. Recommendations for continuous quality improvement of PCA use in the management of VOC pain in children and teens with SCD are proposed based on this centre’s experience.