The events in the hemolytic crisis of hereditary spherocytosis,with particular reference to the reticulocytopenia,pancytopenia and an abnormal splenic mechanism

The course of hereditary spherocytosis (congenital or familial hemolytic anemia) is subject to major or minor exacerbations or crises. Pancytopenia, reticulocytopenia, and extreme spherocytosis characterize the major crises, during whichhypersplenic effects appear to play a major role. These are characterized by thecombination of (1) an unusual degree of hemolysis with (2) inhibitory effects uponmaturation and delivery of bone marrow cells. At the height of the crisis, an extreme degree of maturation arrest in erythropoiesis is present. Splenectomy, whichis often urgently necessary, results in a very rapid increase in all the cellular elements of the blood, confirming the phagocytic and inhibitory effects of the abnormal spleen. Following splenectomy, no further crises occur. The presence of successive cases in the same family suggest the possible role of infection as aprecipitating or trigger agent in initiating an abnormal splenic mechanism leadingto crisis. The cause of the hereditary spherocytosis is commented upon and evidencebearing upon an autopsecific mechanism is discussed.

     

Planning health sector expenditure in support of PHC

This paper describes an approach to financial control whichhas been common to the public sector health services in a numberof countries and it identifies some problems it presents tothe effective implementation of PHC development policies. Amongthe functions of existing systems are the control of expenditure,monitoring for theft and annual budgeting. As the health sectoris transformed from a relatively simple, urban-based serviceto one undertaking activities throughout the country, adaptationswill be required. Furthermore, in order to oversee a significantchange in the pattern of resource allocation, it will be necessaryto integrate the planning system more closely with the preparationof the budget and with the approaches to financial control.While the focus is on the need to strengthen the instrumentsfor resource management within the public services, the importanceof taking a broader view of the health sector is recognized.Those responsible for assuring the effective use of scarce resourcesmust take cognisance of the impact of private health activitieson PHC development. Some implications which this has for plannersare explored.     

GROWTH HORMONE RESPONSE TO FEEDING IN TERM AND PRETERM NEONATES

ABSTRACT. Plasma growth hormone concentrations were measured in 248 healthy term and preterm infants. At birth growth hormone concentrations in cord blood from both term and preterm babies were approximately 100-fold higher than those in blood drawn from healthy adults. By the sixth postnatal day basal pre-feed levels had fallen in term neonates by 65% and a marked postprandial rise was apparent; preterm infants did not show this initial fall in preprandial hormone levels nor was any response to feeding seen. However a fall in preprandial concentrations accompanied by the development of postprandial surges in growth hormone occurred during the next 2 weeks so that by 24 days the postprandial rise was similar to that of term neonates on the sixth day. We conclude that although the initial postnatal changes in plasma growth hormone concentrations are different in preterm and term infants, feeding is a major stimulus to growth hormone secretion in both groups of neonates. Further work is needed to define the precise role of this hormone in neonatal metabolic adaptation.     

METABOLIC AND ENDOCRINE RESPONSES TO A MILK FEED IN SIX-DAY-OLD TERM INFANTS: DIFFERENCES BETWEEN BREAST AND COW'S MILK FORMULA FEEDING

ABSTRACT. Lucas, A., Boyes, S., Aynsley-Green, A. (Department of Paediatrics, John Radcliffe Hospital, Oxford) and Bloom, S. R. (Hammersmith Hospital, London, England). Metabolic and endocrine responses to a milk feed in six-day-old term infants: JMfferences between breast and cow's milk formula feeding. Acta Paediatr Scand, 70:195, 1981. – There is little information on the metabolic and endocrine responses to milk feeding in the neonatal period particularly in relation to the mode of nutrition and composition of the milk. Plasma concentrations of insulin, glucagon and gastric inhibitory polypeptide (GIP) together with blood levels of glucose, ketone bodies, pyruvate, lactate and glycerol were measured pre- and post-prandially in 79 healthy six-day-old term infants who had been either breast fed or fed on a modified cow's milk formula (Cow and Gate Premium) from birth. Formula fed infants had a greater insulin and GIP response to feeding and their basal and postprandial blood ketones were considerably lower than in breast fed infants. In addition a significantly greater post feed rise in both lactate and pyruvate concentrations was observed with formula feeding. These results may have significant implications regarding infant feeding and postnatal metabolism.     

PLASMA NEUROTENSIN IN TERM AND PRETERM NEONATES

Abstract. Lucas A.¹, Aynsley-Green, A.¹, Blackburn, A. M.², Adrian, T. E.² and Bloom, S. R.² (¹University Department of Paediatrics, John Radcliffe Hospital, Oxford, and ²Hammersmith Hospital, London, England). Plasma neurotensin in term and preterm neonates. Acta Paediatr Scand, 70:201, 1981. –The new ileal peptide neurotensin has not been studied hitherto in the human neonate. Plasma concentrations of neurotensin were measured by radioimmunoassay in 276 healthy term or preterm infants either at birth or pre- or post-prandially during the neonatal period. In addition, a group of 10 6-day-old preterm infants were studied who had never been enterally fed on account of hyaline membrane disease. Plasma neurotensin values were obtained also in 12 healthy fasting adults. Term infants had higher plasma neurotensin concentrations than preterm infants at birth. Both groups showed a significant postnatal surge in basal (pre-prandial) neurotensin concentrations exceeding adult values, but no postnatal neurotensin elevation was found in the unfed group. In preterm infants, who were studied further into the neonatal period than term infants, there was a progressive increase in the rise of neurotensin following a milk feed, with a massive neurotensin response by 24 days of age. In 6-day-old term infants, the neurotensin response to bottle feeding was significantly greater than to breast feeding. These findings add further weight to the concept that neurotensin may be of physiological importance as a gut hormone. The high neurotensin levels and large feed responses seen in neonates may indicate a unique role for neurotensin in early life.     

PLASMA SECRETIN IN NEONATES

Abstract. Lucas, A., Adrian, T. E., Bloom, S. R. and Aynsley-Green, A. (University Department of Paediatrics, John Radcliffe Hospital, Oxford and Hammersmith Hospital, London). Plasma secretin in neonates. Acta Paediatr Scand, 69: 205, 1980.—Plasma secretin has been measured in 96 normal 6-day-old term infants and in 158 healthy preterm infants whose mean post-partum ages were 2½, 6, 13 or 24-days. At birth, plasma secretin levels in both term and preterm infants were high compared with those seen in healthy fasting adults ( p > 0.001), but subsequently declined towards adult values. In contrast, preterm infants who had not been fed for the first 6 days of life, had presistently high basal plasma secretin values. In term infants at 6 days of age and in preterm infants up to 13 days, there was no se-cretin response to a feed. However, by 24 days, preterm infants showed a marked post-prandial secretin elevation ( p > 0.02). No correlations were found between plasma secretin concentrations and either blood glucose or plasma insulin concentrations following a feed. Significant adjustments in plasma secretin levels occur in the early weeks of life which may be influenced by enteral feeding.