Davies Va

A Comparison of High versus Low Dose Recombinant Human Erythropoietin versus Blood Transfusion in the Management of Anaemia of Prematurity in a Developing Country

The purpose of this study was to evaluate the effectiveness of early treatment with erythropoietin (EPO) in two different treatment regimes (high vs. low dose) in comparison to the conventional treatment of packed red blood cell (PRBC) transfusions in the management of anaemia of prematurity in a country with limited resources. An open controlled trial was conducted on 93 preterm infants (7 days postnatal age, 900-1500 g birthweight). Patients were randomly assigned either to a low dose (250 IU/kg), a high dose (400 IU/kg), or a control group. EPO was administered subcutaneously three times a week and all infants received 6 mg/kg iron orally from study entry to endpoint of therapy. Haematological parameters were measured and compared. The success was defined as an absence of transfusions and a haematocrit that did not fall below 30 per cent during the time period that the infants were in the study. The three groups were statistically comparable at study entry with respect to gestational age, birthweight, Apgar scores, and haematological values. Over the period that the infants were in the study, 75 per cent of the low dose group and 71 per cent of the high dose group met the criteria for success compared with 40 per cent in the control group (p < 0.001). However, there was no significant difference in the number of transfusions when the low and high EPO dose groups (9.5 per cent) were combined and compared with the control group (26.7 per cent) p = 0.0587. It was concluded that in stable infants, 900-1500 g, where phlebotomy losses are minimized and stringent transfusion guidelines are adhered to, EPO does not significantly decrease the number of transfusions. A conservative approach in the management of anaemia of prematurity, is a viable alternative in areas with limited resources.

Comparative growth and biochemical response of very low birthweight infants fed own mother's milk, a premature infant formula, or one of two standard formulas.

Summary: Very low birthweight (VLBW) infants weighing < 1,600 g at birth were fed their own mothers milk (OMM) or randomly assigned to receive one of three formulas: a “humanized” formula (SF), a partially modified casein-predominant cows milk formula (CF), or a premature formula (PF). All infants were fed at 120 kcal/kg/day where possible, PF infants had significantly greater weight increments (28.0 g/day) than those on OMM (19.4 g/day), SF (18.9 g/day), and CF (18.2 g/day). Those on PF also had greater increments of length, head circumference, and skinfold thickness than those on the other two formulas and greater length increments than those on OMM. Dynamic skinfold measurements suggested that no infants accumulated excessive amounts of interstitial fluid. Infants on the two standard formulas had significantly greater base deficits, whereas those on CF also had higher urea values. Those on OMM had lower phosphate and higher alkaline phosphatase values than the other groups. Thus VLBW infants fed a premature formula had better growth and fewer biochemical problems than those on standard formulas, whereas supplementation of OMM may be necessary to ensure optimal growth and bone mineralization.

Does hypoxemia prevent brain damage in birth asphyxia

The clinical syndrome of hypoxic ischemic encephalopathy (HIE) which occurs in association with birth asphyxia, is thought to represent a reperfusion injury consequent upon the generation of cytotoxic oxygen derived free radicals. It has recently been suggested that resuscitation of asphyxiated infants with unrestricted oxygen may aggravate the brain damage by causing hyperoxia and increased free radical production. To determine whether sustained hypoxemia may be protective in birth asphyxiated infants, we investigated the relationship between HIE and persistent pulmonary hypertension of the neonate (PPHN). The latter condition is also related to intrauterine and intrapartum birth asphyxia but is associated with persistent hypoxemia in the infant. In a retrospective analysis of 39 asphyxiated neonates admitted to the neonatal intensive care unit, we found that 28 had HIE, 10 had PPHN and only 1 had both HIE and PPHN. We therefore suggest that the hypoxemia due to PPHN may limit the production of oxygen derived free radicals in asphyxiated neonates and hence protect against the development of HIE. These findings lend support to current research into air vs. oxygen resuscitation for infants with birth asphyxia.

Severe illness caused by Rickettsia conorii

An 18-month-old boy presented with a 5-day history of lethargy, fever, vomiting and rash. He required intensive care for inotropic and ventilatory support. He developed a disseminated intravascular coagulopathy and gangrene of his extremities. In addition, he had severe neurological dysfunction and loss of vision, both of which recovered spontaneously with time. The potential severity of tick typhus caused by Rickettsia conorii is described as well as the importance of paired serological tests in the diagnosis of this condition.

Three-year growth and developmental follow-up of very low birth weight infants fed own mother's milk, a premature infant formula, or one of two standard formulas

A cohort of 40 very low birth weight (VLBW) infants was followed until they reached 3 years of age. These infants were originally part of a feeding trial in the early postnatal period whereby they were fed either their own mothers milk, a standard whey-predominant formula, a casein-predominant formula, or a premature formula. Those fed the latter formula grew significantly better while in the hospital and had no biochemical derangements. At 3 years of age, there were no significant intergroup differences with respect to growth or development. There was a positive correlation between head growth in hospital and weight at 3 years, but there were no other significant relationships between early postnatal growth and growth parameters at 3 years. Socioeconomic status was the only predictor of developmental scores at 3 years of age. No adverse effects from early metabolic acidosis or alterations of amino acid profiles during the neonatal period were detectable at 3 years of age. However, the small sample size of this study may have missed true differences in outcome measures at 3 years, and larger studies are required to examine these questions further.

Use of the Bayley Scales of Infant and Toddler Development, Third Edition, to Assess Developmental Outcome in Infants and Young Children in an Urban Setting in South Africa

Background The Bayley Scales of Infant and Toddler Development (III) is a tool developed in a Western setting. Aim To evaluate the development of a group of inner city children in South Africa with no neonatal risk factors using the Bayley Scales of Infant and Toddler Development (III), to determine an appropriate cut-off to define developmental delay, and to establish variation in scores done in the same children before and after one year of age. Methods Cohort follow-up study. Results 74 children had at least one Bayley III assessment at a mean age of 19.4 months (95% CI 18.4 to 20.4). The mean composite cognitive score was 92.2 (95% CI 89.4 to 95.0), the mean composite language score was 94.8 (95% CI 92.5 to 97.1), and mean composite motor score was 98.8 (95% CI 96.8 to 101.0). No child had developmental delay using a cut-off score of 70. In paired assessments above and below one year of age, the cognitive score remained unchanged, the language score decreased significantly (p = 0.001), and motor score increased significantly (p = 0.004) between the two ages. Conclusion The Bayley Scales of Infant and Toddler Development (III) is a suitable tool for assessing development in urban children in southern Africa.

Retrospective cross-sectional review of survival rates in critically ill children admitted to a combined paediatric/ neonatal intensive care unit in Johannesburg, South Africa, 2013-2015

Objective Report on survival to discharge of children in a combined paediatric/neonatal intensive care unit (PNICU). Design and setting Retrospective cross-sectional record review. Participants All children (medical and surgical patients) admitted to PNICU between 1 January 2013 and 30 June 2015. Outcome measures Primary outcome—survival to discharge. Secondary outcomes—disease profiles and predictors of mortality in different age categories. Results There were 1454 admissions, 182 missing records, leaving 1272 admissions for review. Overall mortality rate was 25.7% (327/1272). Mortality rate was 41.4% (121/292) (95% CI 35.8% to 47.1%) for very low birthweight (VLBW) babies, 26.6% (120/451) (95% CI 22.5% to 30.5%) for bigger babies and 16.2% (86/529) (95% CI 13.1% to 19.3%) for paediatric patients. Risk factors for a reduced chance of survival to discharge in paediatric patients included postcardiac arrest (OR 0.21, 95% CI 0.09 to 0.49), inotropic support (OR 0.085, 95% CI 0.04 to 0.17), hypernatraemia (OR 0.16, 95% CI 0.04 to 0.6), bacterial sepsis (OR 0.32, 95% CI 0.16 to 0.65) and lower respiratory tract infection (OR 0.54, 95% CI 0.30 to 0.97). Major birth defects (OR 0.44, 95% CI 0.26 to 0.74), persistent pulmonary hypertension of the new born (OR 0.44, 95% CI 0.21 to 0.91), metabolic acidosis (OR 0.23, 95% CI 0.12 to 0.74), inotropic support (OR 0.23, 95% CI 0.12 to 0.45) and congenital heart defects (OR 0.29, 95% CI 0.13 to 0.62) predicted decreased survival in bigger babies. Birth weight (OR 0.997, 95% CI 0.995 to 0.999), birth outside the hospital (OR 0.21, 95% CI 0.05 to 0.84), HIV exposure (OR 0.54, 95% CI 0.30 to 0.99), resuscitation at birth (OR 0.49, 95% CI 0.25 to 0.94), metabolic acidosis (OR 0.25, 95% CI 0.10 to 0.60) and necrotising enterocolitis (OR 0.23, 95% CI 0.12 to 0.46) predicted poor survival in VLBW babies. Conclusions Ongoing mortality review is essential to improve provision of paediatric critical care.

Umbilical cord ulceration and jejunal atresia

The association between umbilical cord ulceration and congenital intestinal atresia is being increasingly reported and carries a high mortality. We report on a case of jejunal atresia associated with massive fetal haemorrhage from an umbilical cord ulcer. Fetal distress noted on continuous fetal heart monitoring allowed for delivery by emergency caesarean section followed by appropriate neonatal resuscitation and intact survival. This and other reported cases highlight the need for identification of high-risk fetuses with congenital intestinal atresia. Close fetal monitoring during labour is imperative in order to improve outcomes.

Assessment of developmental outcome in very low birth weight infants in Southern Africa using the Bayley Scales of Infant Development (III)

Objectives The study aimed to compare the developmental outcome of very low birth weight infants with a group of normal-term controls in a tertiary hospital in sub-Saharan Africa. Design A group of 105 very low birth weight infants were assessed at a mean age of 17.6 months (95% CI 16.7 to 18.6) using the Bayley Scales of Infant Development, Third Edition, and compared with a group of normal-term controls at the same mean age. Results Seven of the study infants (7%) had developmental delay (a score below 70), compared with none in the control group (p=0.04). Three of the seven study infants were delayed on all three subscales, one of whom had cerebral palsy. A further 34% of the study infants were ‘at risk’ of developmental delay (a score below 85). There was no difference in the mean composite score between the study group and controls for the cognitive (p=0.56), motor (p=0.57) or language (p=0.66) subscales. There was no difference in mean composite scores on all subscales between infants who were appropriate for gestational age and those who were small for gestational age. Cognitive and motor scores remained stable in paired assessments of study infants before and after 1 year of age; language scores decreased significantly (p<0.001). Mechanical ventilation was the only risk factor significantly associated with a cognitive score below 85 in study infants. Conclusion Very low birth weight infants in sub-Saharan Africa are at risk of developmental delay and require long-term neurodevelopmental follow-up.

Congenital central hypoventilation syndrome and intestinal aganglionosis: A case report

Congenital central hypoventilation syndrome (CCHS), also called ‘Ondine’s curse’, is characterised by an abnormal ventilatory response to progressive hypercapnia and sustained hypoxaemia. Neonates with this condition experience hypoventilation or apnoea while asleep. Patients may also have congenital intestinal aganglionosis (CIA), aganglionic megacolon or Hirschsprung’s disease, suggesting an aberrant phenotype arising from a defect of migration or diff erentiation of neural crest cells. Some patients also have tumours of neural crest cell origin, including neuroblastoma, ganglioneuroma and ganglioneuroblastoma. Th e association of CCHS and CIA is called Ondine- Hirschsprung disease (Haddad syndrome). A few cases have been diagnosed in South Africa, but none has been reported. We report a case of CCHS and CIA with a PHOX2B gene mutation.