Pre- and post-natal treatment of hemophagocytic lymphohistiocytosis

Hemophagocytic lymphohistiocytosis (HLH) is a rare autosomal recessive disorder of infancy and childhood that is invariably fatal if not treated. We report on the first patient to receive post-natal HSCT for HLH after receiving in utero chemotherapy for disease stabilization.     

Vascular retinal abnormalities in neonates of mothers who smoked during pregnancy

OBJECTIVE: To investigate whether maternal smoking during pregnancy causes retinal abnormalities in the newborn. STUDY DESIGN: One hundred sixty-two neonates of smoking mothers and 162 matched neonates of nonsmoking mothers (112 appropriate for gestational age [AGA], 30 small for gestational age [SGA], 20 large for gestational age [LGA] in each group) were studied. RESULTS: Retinal arterial narrowing and straightening (RANS) was observed in 52 and 10 eyes of the newborns of smoking and nonsmoking mothers, respectively (P <. 000001) in association with elevated blood pressure in the neonates. The frequency of RANS was more than 3-fold greater in the SGA neonates than in the AGA and LGA neonates of the smoking mothers. Retinal venous dilatation and tortuosity (RVDT) was found in 100 and 36 eyes of neonates of smoking and nonsmoking mothers, respectively (P <.000001). The frequency of RVDT in the SGA neonates of the smoking mothers was 2.5-fold and 4.2-fold greater than in the AGA infants and the LGA infants, respectively. Also, intraretinal hemorrhages were found in 61 and 31 eyes of neonates of smoking and nonsmoking mothers, respectively (P =.0007) in association with elevated hematocrit and RVDT, whereas no intraretinal hemorrhages were found when RANS was present. All retinal abnormalities resolved by 6 months in infants of smoking mothers and by 2 months in infants of nonsmoking mothers. CONCLUSIONS: Maternal smoking during pregnancy causes increased frequency of RANS, RVDT, and intraretinal hemorrhages; but these retinal abnormalities resolve by 6 months of age.     

11- to 15-Year-old children of women who danced during their pregnancy

AIM: To determine whether high levels of maternal physical activity (dancing during pregnancy), influenced the offspring. MATERIALS AND METHODS: Sixty-four women (32 of whom had continued to dance for at least 2 months after the beginning of pregnancy) filled in a questionnaire to assess the health and behavior of their offspring, now 11-15 years of age. RESULTS: More children born from women who had continued to dance during pregnancy needed to be rocked to sleep during their first year (p < 0.05). More children in the study group played musical instruments (p < 0.05). There were no significant differences between the groups regarding other parameters. CONCLUSIONS: The greater need to be rocked to fall asleep and the attitude to play musical instruments recorded in the children of dancers may be related to prenatal maternal movement. But they may also be due to other causes as discussed.     

Management of women who use drugs during pregnancy

Substance abuse in pregnancy is associated with significant maternal and fetal morbidity. The complication for care is that the mother is the cause of the problem that potentially harms not only herself but also her unborn child. The abuse is further complicated by the associated legal, social and environmental problems. The aim for the obstetrician is to provide a non-judgemental, supportive environment to minimise the risk, not only during pregnancy and the neonatal period, but also in the long term. To achieve this, caregivers need to be multidisciplinary and tolerant of the mother's problem. The aim of antenatal care is to reduce risk, which does not mean that the mother must abstain from drug use. The aim is to keep her within the care system and encourage her to take responsibility for her situation. No-one should be turned away or denied help as, ultimately, this is harmful to the mother and her baby. To provide this care, the obstetrician needs support from midwives, addiction counsellors, social workers, neonatologists, health visitors and general practitioners. Whereas the healthcare structure in the UK lends itself to this approach, this is not the case in other parts of the world. However, programmes of comprehensive antenatal care do not universally improve the health of the mother and the outcome of her pregnancy. The specifics of the care provided are probably less important than the quality of the care given and the degree of engagement of the individual. This chapter outlines the problems and potential solutions with reference to the service in Leeds in the UK.     

Selected trace elements and proteins in serum of apparently healthy newborn infants of mothers who smoked during pregnancy

The serum concentrations of selected trace elements and proteins in cord blood from 17 newborn infants whose mothers were habitual smokers were compared with values from 22 infants of non-smoking mothers. All the mothers were healthy with normal pregnancies and deliveries. Cigarette smoke exposure was verified by determinations of nicotine, cotinine and thiocyanate concentrations in cord blood. Infants of smoking mothers had a slightly lower mean birth weight (3490 +/- 430 g) than control infants (3780 +/- 460 g). Infants of smokers had lower serum iron (p = 0.05) and prealbumin (p less than 0.05), but higher serum copper (p less than 0.05) and ceruloplasmin (p less than 0.01) levels than the controls. Infants of smoking mothers tended to have higher levels of the acute-phase reactants alpha-2-macroglobulin and orosomucoid, but lower levels of albumin, transferrin and retinol-binding-protein, although differences were not statistically significant.     

A follow-up study up to the age of 14 of children whose mothers smoked during pregnancy

The children of mothers who smoked during pregnancy (n = 1819) and their controls, all born in northern Finland in 1966, were followed up to the age of 14. The children of the smokers were more prone to respiratory diseases than the others. They were also shorter and their mean ability at school poorer than among the controls. The differences remained significant after adjusting for the mother's height and age, social class as determined by the father's occupation, number of older and younger children in the family and the sex of the child. The smoking mothers were found to differ from their controls in each social class, in contrast to the situation at the time of pregnancy, now having on average poorer health, being more often unemployed and having more often left their families. Maternal smoking was still seen to have an effect on the children's physical and mental development, even when these factors were also taken into consideration in the regression analyses, although it was less important than many other socio-biological factors, and it was no more important than paternal smoking.     

Improvement of growth after growth hormone treatment in children who undergo liver transplantation

BACKGROUND: Chronic liver insufficiency in children is frequently associated with growth retardation. Growth resumes after successful orthotopic liver transplantation in the majority of children with previous chronic liver failure. However, a subgroup of children demonstrates stunted growth even after orthotopic liver transplantation. The current study was conducted to determine whether administration of recombinant human growth hormone might benefit these patients. METHODS: Ten children were identified who met the criteria of growth failure despite normal transplant function in a cohort of 60 transplantation patients: height standard deviation score (hSDS) for chronological age less than -2, and growth velocity SDS (gvSDS) for chronological age equaling 0. Seven of these patients were treated with subcutaneous injections of recombinant human growth hormone at 4.0 U/m2 body surface area per day for at least 1 year. Two patients in this group showed insufficient growth hormone response to stimulation (arginine, clonidine) before therapy. Treatment was begun after a median time of 4.6 years after liver transplantation (2.55-8.4 years). Five children were treated with cyclosporin A and prednisolone and two with tacrolimus and prednisolone for maintenance immunosuppression. RESULTS: Within 3 months of treatment, median serum levels of insulin-like growth factor (IGF)-I increased from 0.05 to 0.71 (P < 0.02). Within 1 year, median hSDS improved from -2.7 (range, -5.6 to -2.3) to -2.1 (-4.5 to -1.4; P < 0.03). Median annual growth rate increased from 3.9 cm/year (range, 3-6) in the year before treatment to 8.2 cm/year (range, 6.1-10.4; P < 0.02) after the beginning of recombinant human growth hormone therapy. All patients tolerated treatment without side effects. During the cumulative treatment time of 14 years no rejection episode was observed. CONCLUSIONS: Short-statured prepubertal liver transplant recipients who do not show sufficient compensatory growth after transplantation benefit from treatment with recombinant human growth hormone. Treatment with the hormone was safe without any side effects.     

Pre- and postoperative growth in persistent ductus arteriosus

Children with a persistent ductus arteriosus requiring ligation under 6 months of age show evidence of intrauterine growth retardation. By the time of operation the weight centile of 17 (71%) of 24 such infants had fallen, but 20(84%) of the 24 gained weight postoperatively. Those who did not so were either large-for-dates infants growing towards the normal or small-for-dates infants who remained small postoperatively.     

Squamous cell carcinoma of the lung in an autistic child who has never smoked

Primary bronchogenic carcinoma of the lung is extremely rare in childhood, particularly the squamous cell type. Only 13 cases have been reported in the literature. We report a case of squamous cell carcinoma in an autistic, 16-year-old boy who presented with a productive cough. Interestingly, he was a never-smoker, but had been exposed to environmental tobacco smoking by his father for 13 years. The diagnosis was delayed by approximately 1 month due to his young age. He was diagnosed with squamous cell carcinoma of the lung by video-assisted thoracoscopic surgery, and chemotherapy was arranged. Considering his age, autism, and good performance status, a combined chemotherapeutic regimen with gemcitabine plus carboplatin was planned. After the second cycle of chemotherapy, the cough resolved and a computed tomography scan showed a partial response of the central conglomerated mass with the absence of the malignant pleural effusion.     

Fetal and postnatal growth of children born to narcotic-dependent women

We studied the effect of heroin and methadone on birth length and 3-year stature of children of untreated heroin addicts (n = 22), women receiving methadone maintenance therapy (95% were polydrug users) (n = 21), and a drug-free comparison group (n = 28), after adjustment for biologic, demographic, and health variables. The mean birth lengths of both groups of drug-exposed infants were significantly below that of a comparison group; however, group means were similar after adjustment for sex, race, prenatal care, pregnancy weight gain, obstetrical risk, maternal education, and smoking. At 3 years of age the mean height was comparable for all groups. When adjusted for birth length, parental height, and smoking, the methadone group was significantly shorter than children exposed to heroin in utero, and the comparison group assumed an intermediate position. These data indicate that the effect of heroin and methadone on intrauterine growth cannot be differentiated from that of associated factors, and that postnatal growth of children exposed to narcotics during pregnancy is no more impaired than that of a high-risk comparison group. Children of all three groups deserve continued observation and efforts to improve their environment in order that their full potential might be achieved.     

Pre- and perinatal clinical characteristics of infants who suffer sudden infant death syndrome

In a population-based study, clinical and physiological data were collected in the neonatal period on 7,496 full-term (greater than or equal to 37 weeks) infants of birthweights greater than 2.50 kg born in 3 hospitals. The sample excluded a small number of infants who were born with severe illnesses from which there was no period of recovery before death in the neonatal period. Twenty-seven of the infants had died by the time all of the population had passed their third birthday. Twenty-one of these cases died suddenly and unexpectedly, and in 13 there was no adequate explanation for death (sudden infant death syndrome-SIDS). Pre- and early postnatal clinical data on the infants who had died were compared to a randomly selected sample of 478 survivors. This analysis showed that the infants who suffered SIDS showed similar clinical characteristics to those previously reported. Unlike the SIDS group, however, the prenatal histories of the non-SIDS deaths were similar to controls, showing no evidence of a sub-optimal intra-uterine environment.     

Cross-sectional growth of children whose mothers abused amphetamines during pregnancy

Eriksson M, Jonsson B, Steneroth G, Zetterström R. Cross-sectional growth of children whose mothers abused amphetamines during pregnancy. Acta Pediatr 1994;83:612–17. Stockholm. ISSN 0803–5253
Growth of 65 children whose mothers were addicted to amphetamines during pregnancy was followed prospectively from birth to eight years of age. At birth, one and four years of age, mean weight, height and head circumference were below the mean for Swedish children and significantly so in girls. A statistically significant correlation was found in both sexes between impaired growth and social factors such as stress index, increasing number of siblings, length of maternal cohabitation and abuse in maternal relatives.     

Effects of birth size, post-natal growth and current size on insulin resistance in 9-year-old children: a prospective cohort study

The influence of pre-natal conditions on later type 2 diabetes risk factors such as insulin resistance (IR) may be mediated by post-natal growth trajectory. We aimed to investigate the association of body size at birth and 9 years with IR at 9 years. Using data from a prospective Australian cohort study, we examined the influence of body size from birth to 9 years [z-score for weight or body mass index (BMI)] on IR at 9 years (estimated by homeostasis model assessment). At age 9 years, 151 children provided a fasting blood sample. z-BMI at age 9 was positively associated with IR. Birth z-BMI was inversely associated with IR only after adjustment for z-BMI at age 9 years. This may be interpreted as an effect of accelerated growth between birth and 9 years on IR. There was a statistically significant interaction between birth and 9-year z-BMI. Results from regression models including z-BMI at all available time points (birth, 6 and 12 months, and 2, 3.5 and 9 years) indicate a possible inverse association between body size at 3.5 years and HOMA-IR at 9 years. Results were similar when the analyses were repeated with z-weight substituted for z-BMI. These results add to the body of evidence concerning the importance of growth in early life for later IR, and highlight a possible interaction between pre- and post-natal growth. The potential influence of growth at around 3.5 years for HOMA-IR at 9 years warrants further investigation.