Repeat sudden unexpected and unexplained infant deaths: natural or unnatural?

BACKGROUND: There have been suggestions that when two or three unexpected unexplained infant deaths occur within a family they are more likely to be unnatural than natural. We aimed to estimate the probability that a second infant death is natural versus unnatural. METHODS: The Care of Next Infant programme (CONI) supports parents who have previously had an unexpected and apparently unexplained infant death and is currently available in over 90% of health districts in England, Wales, and Northern Ireland. We studied all deaths in 6373 infants who had completed the CONI programme by December, 1999. After a CONI death, we made detailed enquiries into the previous death and the CONI death, including a family interview, a review of autopsies, and case discussion. FINDINGS: 57 (8.9 per 1000) CONI infants died. Nine deaths were inevitable, and 48 were unexpected. 44 families lost one child, and two families lost two children. Of the 46 first CONI deaths, 40 were natural; the other six were probable homicides, five committed by one or both parents (two criminally convicted). The ratio of 40 natural to six unnatural deaths is 6.7 (95% CI 2.8-19.4). Enquiries identified 18 families with two SIDS(sudden infant death syndrome) deaths and two families with probable covert double homicides (ratio 9.0 [2.2 to 80.0]). There were no convictions in 13 incomplete cases. Families with three deaths are reported. INTERPRETATION: Repeat unexpected infant deaths are most probably natural.

Signs and symptoms of illness in early infancy: associations with sudden infant death.

AIMS: to describe a pattern of illness in "normal" infants, and to assess to what extent this may differ in infants who die suddenly and unexpectedly. METHODS: All infants are on the CONI (Care of the Next Infant) scheme in which mothers record symptoms and signs of illness prospectively, on a daily basis from birth to approximately age 6 months. The symptoms of infants who die suddenly and unexpectedly are compared with those of a consecutive sample of infants who survive. Twenty one babies died suddenly, of whom 11 were cot deaths and 10 had "known causes" of death. Ninety eight infants who survived were used as consecutive controls. Prospective daily records of the presence or absence of 26 signs and symptoms were kept by all of the mothers, commencing at birth and lasting an average of 176 days in survivors; 84 days in SIDS; and 93 days in infants who died of known causes. RESULTS: Standardised for age and the time of year, on any given day, the mothers of the SIDS infants were over eight times more likely to record their child as being pale, six times more likely to be sweating, and twice as likely to be irritable. Other signs and symptoms which were three times more likely to be recorded were sore gums, dry stools, and coldness. Infants who died of known causes generally had fewer symptoms. CONCLUSIONS: Cot death infants have a range of symptoms which appear to be of a general nature, and not related to any one system. Symptoms are seen throughout life and not related to the time of death.

Patterns of symptoms in siblings of sudden infant death syndrome infants.

Longitudinal day-to-day recordings of symptoms in the siblings of Sudden Infant Death Syndrome (SIDS) infant show a wide variety of symptoms varying with season and days of years. Implications are discussed.

Effect of caffeine on neonatal splanchnic blood flow.

Doppler ultrasound was used to study the effect of the first intravenous dose of caffeine on splanchnic haemodynamics in preterm neonates. Peak systolic velocity in the superior measenteric artery and coeliac axis was significantly reduced for 6 hours after caffeine infusion. The effect of this reduction in blood flow to the neonatal gut is not known.

Effect of feed interval and feed type on splanchnic haemodynamics.

AIM: To study the effect of enteral feeding on splanchnic blood flow velocity in preterm infants. METHOD: Coeliac axis and superior mesenteric artery (SMA) blood flow velocity were measured longitudinally in a cohort of 61 babies using Doppler ultrasound. RESULTS: Babies fed 1 hourly had significantly higher preprandial SMA peak systolic velocity (PSV) than those fed 3 hourly (70 vs 53 cm/s). Those fed 1 hourly showed no postprandial change whereas those fed 3 hourly showed significant postprandial hyperaemia. This hyperaemia had longer latency (42 vs 27 mins) and smaller amplitude (31 vs 25 mins) after expressed breast milk compared with preterm formula. The addition of long chain polyunsaturated fatty acids to the formulas had no effect on the postprandial response. CONCLUSION: Hourly bolus feeding leads to a persistent hyperaemic state in the SMA. The composition of feeds is an important determinant of the postprandial response of the SMA to 3 hourly feeding.

Neonatal respiratory distress in near-term infants--consider surfactant protein B deficiency.

Two infants presenting with respiratory distress in the first 24 h of life are described. Both patients underwent extensive investigation before the diagnosis of surfactant protein B-deficiency was reached. Both children died within 2 months of birth. Parental consanguinity was known to be a feature in the first case, who proved to have a previously unrecognized mutation of the surfactant protein B gene. In the second case, a history of parental consanguinity was not sought from the Caucasian family, but was later volunteered by the parents themselves. Case 2 proved to have the "common" surfactant protein B-deficient genotype. The key to diagnosis is having a high index of suspicion in any term or near-term newborn with severe respiratory distress; parental consanguinity must be excluded. Surfactant protein B-deficiency can be readily diagnosed from bronchoalveolar lavage specimens; a simple, inexpensive procedure which is well tolerated in newborns.

Comparison of cyclosporin G (Nva2-cyclosporin) concentrations measured in whole blood by monoclonal fluorescence polarization immunoassay, monoclonal radioimmunoassay, and HPLC.

Two commercial monoclonal immunoassays for monitoring cyclosporin A were used to measure whole-blood concentrations of the immunosuppressant cyclosporin G (CsG) in renal transplant patients. We performed a three-way comparison of these two immunoassays and HPLC. Although the two immunoassays agreed favorably, both the monoclonal fluorescence polarization immunoassay and the monoclonal RIA yielded higher CsG results for patients' specimens than did the liquid-chromatographic assay. The experimental data indicate that the observed differences are most likely due to the cross-reactivity of CsG metabolites in the immunoassays.

Poland anomaly with contralateral ulnar ray defect.

We report an atypical case of the Poland anomaly. Unreported features are that the hand abnormality is on the contralateral side to the chest wall defect, there is an ulnar ray predominance, and lack of syndactyly.

Abnormal gut blood flow velocities in neonates at risk of necrotising enterocolitis.

Duplex pulsed Doppler ultrasound was used to determine blood flow velocities in the coeliac axis and superior mesenteric artery in three groups of neonates: a group at high risk of developing necrotising enterocolitis (n = 27) and two control groups, i.e., a nonasphyxiated, appropriately grown group of preterm infants (n = 18) and a group of nonasphyxiated term infants (n = 14). Subjects were studied on the first, second, and fourth days of life. The median peak systolic velocities in the superior mesenteric artery were between 20 and 51% lower in the at-risk group than in the term control group on all 3 days of measurement (p less than 0.05-p less than 0.002). The ratio of peak systolic velocity in the coeliac axis to that in superior mesenteric artery, an index of relative downstream vascular resistance in the superior mesenteric artery, was 42-65% greater in the at-risk group compared with the other two groups on days 1 and 2 (p less than 0.05-p less than 0.001) and significantly greater than the term group on day 4 (73%, p less than 0.002). These data demonstrate that neonates at risk of developing necrotising enterocolitis have abnormal gut blood flow velocities. Furthermore, they provide evidence that an alteration in the splanchnic circulation may be an important factor in the final common pathway that links diverse risk factors for necrotising enterocolitis with clinical disease.

Doppler assessment of human neonatal gut blood flow velocities: postnatal adaptation and response to feeds.

Duplex pulsed Doppler ultrasound was used to study changes in gut blood flow velocities during the first week of life in a group of 14 term babies. There was a significant increase in fasting peak systolic velocity in the superior mesenteric artery between days 1 and 2 with a further upward trend until day 5; no such changes were seen in the coeliac axis. Fasting velocities were 20% lower in breast-fed babies than bottle-fed babies. Following feeds, there was a significant increase in velocity in the superior mesenteric artery that was 35% greater in the bottle-fed than breast-fed babies. Changes in the coeliac axis were qualitatively similar but substantially smaller. The peak velocity in both vessels occurred 50 min after a feed. We conclude that Doppler ultrasound can be readily used to measure gut blood flow velocities in the human newborn. It provides a noninvasive technique for investigating adaptive postnatal changes in the splanchnic circulation, and, in particular, the response to feeds.

Intestinal malrotation: the role of small intestinal dysmotility in the cause of persistent symptoms.

To investigate the possibility that small intestinal dysmotility is a cause of long-standing and persistent symptoms in patients with malrotation, we retrospectively reviewed 94 patients operated on for intestinal malrotation. In 50 patients operated on during the neonatal period, associated abnormalities were common (24%) and all presented with obstructive symptoms; only three (6%) had continuing mild symptoms the postneonatal period (aged 1 month to 1 year), five of whom (22%) had other abnormalities. Recurrent vomiting was the most common presentation (11/23); only 5 patients (21%) had acute obstruction. Response to operation was good in 18 (78%) and two had persistant symptoms; both died. Twenty-one patients presented beyond infancy (aged greater than 1 year), only 19 of whom had symptoms of less than 2 months' duration. Eight (47%) of those with long-standing symptoms had no relief from operation. Small bowel motility was recorded manometrically in 4 patients with long-standing symptoms. The findings suggest that small intestinal dysmotility may be common in patients with malrotation and persistant symptoms.

Gut blood flow velocities in the newborn: effects of patent ductus arteriosus and parenteral indomethacin.

The effects on gut blood flow velocities of parenteral indomethacin (0.2 mg/kg) given either quickly as a bolus or slowly as an infusion were compared in consecutive studies of two groups of infants with symptomatic patent ductus arteriosus. In the presence of patent ductus arteriosus the range of velocities in the superior mesenteric artery before indomethacin was given was characterised by pronounced abnormalities including absent--or in some cases even retrograde--diastolic flow. In eight subjects the first rapidly given bolus dose of indomethacin (duration 20 seconds or less) caused a pronounced and sustained fall in the velocity of the superior mesenteric artery blood flow (mean peak systolic velocity (cm/second): before 74; after 38; median time to maximum fall 7.4 minutes; median time to recovery 50 minutes). A further 10 subjects received their first dose of indomethacin by slow infusion (duration 30-35 min) and the percentage fall in peak systolic velocity was both substantially less (22% compared with 47%) and later (median time to maximum fall 37.3 minutes) than after rapid infusion. Qualitatively similar but smaller changes were seen in the coeliac axis. Return of antegrade end diastolic flow in the superior mesenteric artery within one hour of the first dose of indomethacin was a good predictor of subsequent closure of the ductus. These data suggest that there is a profound disturbance in mid gut perfusion in infants with patent ductus, which is exacerbated by indomethacin given rapidly by intravenous bolus. They may also provide a rational explanation for the well recognised association between necrotising enterocolitis and both patent ductus arteriosus and indomethacin administration. The unwanted effects of the indomethacin are abrogated by slow infusion, without loss of efficacy in closure of the ductus.