Congenital rubella affecting an infant whose mother had rubella antibodies before conception.

A woman who had had high titres of rubella antibodies some months before she became pregnant gave birth to an infant in whom congenital rubella was confirmed at 4 months. Rubella haemagglutination inhibition tests, complement fixation tests, and immunofluorescence tests with anti-human IgG were carried out on sera from the mother. Rubella antibody titres in sera obtained in March 1971, seven and a half months before conception, were equivalent to 400 units, which is usually taken as indicating good immunity. Rubella virus was isolated from the baby''s nose and throat in July 1973 but was not isolated from a cervical swab taken from the mother in December 1973; tests of her immunological competence did not show any definite abnormality. The presence of high levels of rubella haemagglutination inhibition antibodies does not invariably confer immunity or exclude the possibility of congenital rubella in a subsequent pregnancy.     

Predicting child abuse: signs of bonding failure in the maternity hospital.

Fifty children referred to the Park Hospital because of actual or threatened abuse were compared with 50 controls born at the same maternity hospital. Five factors were significantly more common in the abused group than among their controls: (a) mother aged under 20 at birth of first child, (b) evidence of emotional disturbance, (c) referral of family to hospital social worker, (d) baby''s admission to special care baby unit, (e) recorded concern over the mother''s ability to care for child. Thirty-five of the abused group had two or more of these factors compared with only five of the control group. As these data were collected from information recorded routinely at the maternity hospital, it is possible to identify most abusing families when the child is born. Such identification must lead to a comprehensive assessment of each case followed by constructive preventive action.     

Babies with spina bifida treated without surgery: parents' views on home versus hospital care.

From 1971 to 1981, 98 babies born with meningomyelocoele at the North Staffordshire Hospital Centre''s district maternity hospital, were thought not suitable for surgery. Sixty three survived for more than one week. Over the period the hospital''s policy changed: initially all such babies were kept in hospital, but later parents were given the choice of taking their baby home for palliative and terminal care. In an attempt to determine parents'' views on the care of their baby the parents of 44 of the babies who survived to one week were traced in 1985-6, five to 14 years later; 80 of them were asked how they felt about the lives and deaths of their babies. Eighteen babies had been taken home, and they had lived longer than the 26 who had been cared for in hospital. Parents whose baby had remained in hospital were sadder than those who had taken their baby home when they looked back at their experiences, and they also considered that their baby''s life had been of poor quality. Most of those who had taken their baby home had a more positive view of their child''s life. The figures suggest that the bereavement process after a baby''s death is longer than has been thought, but despite residual sadness just over half of the parents interviewed thought that something positive had come out of their experience.     

Familial Creutzfeldt-Jakob Disease: Case report and role of genetic counseling in post mortem testing

Here we present a case of an asymptomatic 53-year-old woman who sought genetic testing for Familial Creutzfeldt-Jakob Disease (fCJD) after learning that her mother had fCJD. The patient's mother had a sudden onset of memory problems and rapidly deteriorating mental faculties in her late 70s, which led to difficulties ambulating, progressive non-fluent aphasia, dysphagia and death within ～1 y of symptom onset. The cause of death was reported as “rapid onset dementia.” The patient's family, unhappy with the vague diagnosis, researched prion disorders online and aggressively pursued causation and submitted frozen brain tissue from the mother to the National Prion Disease Surveillance Center, where testing revealed a previously described 5-octapeptide repeat insertion (5-OPRI) in the prion protein gene (PRNP) that is known to cause fCJD. The family had additional questions about the implications of this result and thus independently sought out genetic counseling.

?While rare, fCJD is likely underdiagnosed due to clinical heterogeneity, rapid onset, early non-specific symptomatology, and overlap in the differential diagnosis of Alzheimer disease and Lewy body dementias. When fCJD is identified, a multidisciplinary approach to return of results that includes the affected patient's provider, genetics professionals, and mental health professionals is key to the care of the family. We present an example case which discusses the psychosocial issues encountered and the role of genetic counseling in presymptomatic testing for incurable neurodegenerative conditions. Ordering physicians should be aware of the basic issues surrounding presymptomatic genetic testing and identify local genetic counseling resources for their patients.     

Young Children's Probability of Dying Before and After Their Mother's Death: A Rural South African Population-Based Surveillance Study

Background

There is evidence that a young child''s risk of dying increases following the mother''s death, but little is known about the risk when the mother becomes very ill prior to her death. We hypothesized that children would be more likely to die during the period several months before their mother''s death, as well as for several months after her death. Therefore we investigated the relationship between young children''s likelihood of dying and the timing of their mother''s death and, in particular, the existence of a critical period of increased risk.

Methods and Findings

Data from a health and socio-demographic surveillance system in rural South Africa were collected on children 0–5 y of age from 1 January 1994 to 31 December 2008. Discrete time survival analysis was used to estimate children''s probability of dying before and after their mother''s death, accounting for moderators. 1,244 children (3% of sample) died from 1994 to 2008. The probability of child death began to rise 6–11 mo prior to the mother''s death and increased markedly during the 2 mo immediately before the month of her death (odds ratio [OR] 7.1 [95% CI 3.9–12.7]), in the month of her death (OR 12.6 [6.2–25.3]), and during the 2 mo following her death (OR 7.0 [3.2–15.6]). This increase in the probability of dying was more pronounced for children whose mothers died of AIDS or tuberculosis compared to other causes of death, but the pattern remained for causes unrelated to AIDS/tuberculosis. Infants aged 0–6 mo at the time of their mother''s death were nine times more likely to die than children aged 2–5 y. The limitations of the study included the lack of knowledge about precisely when a very ill mother will die, a lack of information about child nutrition and care, and the diagnosis of AIDS deaths by verbal autopsy rather than serostatus.

Conclusions

Young children in lower income settings are more likely to die not only after their mother''s death but also in the months before, when she is seriously ill. Interventions are urgently needed to support families both when the mother becomes very ill and after her death. Please see later in the article for the Editors'' Summary     

Mothers' birth weight and survival of their offspring: population based study.

OBJECTIVE: To test the hypothesis that a baby''s survival is related to the mother''s birth weight. DESIGN: Population based dataset for two generations. SETTING: Population registry in Norway. SUBJECTS: All birth records for women born in Norway since 1967 were linked to births during 1981-94, thereby forming 105104 mother-offspring units. MAIN OUTCOME MEASURES: Perinatal mortality specific for weight for offspring in groups of maternal birth weight (with 500 g categories in both). RESULTS: A mother''s birth weight was strongly associated with the weight of her baby. Maternal birth weight was associated with perinatal survival of her baby only for mothers with birth weights under 2000 g. These mothers were more likely to lose a baby in the perinatal period (odds ratio 2.3, 95% confidence interval 1.4 to 3.7). Among mothers with a birth weight over 2000 g there was no overall association between mother''s weight and infant survival. There was, however, a strong interaction between mother''s birth weight, infant birth weight, and infant survival. Mortality among small babies was much higher for those whose mothers had been large at birth. For example, babies weighing 2500-2999 g had a threefold higher mortality if their mother''s birth weight had been high (> or = 4000 g) than if the mother had been small (2500-2999 g). CONCLUSION: Mothers who weighed less than 2000 g at birth have a higher risk of losing their own babies. For mothers who weighed > or = 2000 g their birth weight provides a benchmark for judging the growth of their offspring. Babies who are small relative to their mother''s birth weight are at increased risk of mortality.     

My baby doesn't smell as bad as yours: The plasticity of disgust

Disgust is a powerful behavioral adaptation, which confers the advantage of reducing the risk of pathogen infection. However, there are situations in which disgust at core elicitors (e.g., feces) must be modulated in the service of other goals (e.g., caring for a close kin). In Study 1, mothers of infants completed a self-report questionnaire about their reactions to changing their baby's feces-soiled diaper compared with the diaper of someone else's baby. In Study 2, mothers of infants were presented with a series of trials in which they smelled concealed samples of their own baby's feces-soiled diaper and those of someone else's baby. In addition, labels were used to identify the source of the sample (correctly labeled, mislabeled, or no label). Both studies provide evidence suggesting that mothers regard their own baby's fecal smell as less disgusting than that from someone else's baby. Furthermore, labeling had relatively little influence on this effect, and the effect persisted when social desirability was controlled.     

Factors associated with physician agreement and coding choices of cause of death using verbal autopsies for 1130 maternal deaths in India

Background

The Indian Sample Registration System (SRS) with verbal autopsy methods provides estimations of cause specific mortality for maternal deaths, where the majority of deaths occur at home, unregistered. We aim to examine factors that influence physician agreement and coding choices in assigning causes of death from verbal autopsies.

Methodology/Principal Findings

Among adult deaths identified in the SRS, pregnancy-related deaths recorded in 2001–2003 were assigned ICD-10 codes by two independent physicians. Inter-rater reliability was estimated using Landis Koch Kappa classification – poor to fair agreement; > – moderate agreement; > – substantial agreement; >– high agreement. We identified factors associated with physician agreement using multivariate logistic regression. A central consensus panel reviewed cases for errors and reclassified as needed based on 2011 ICD-10 coding guidelines. Of 1130 pregnancy-related deaths, 1040 were assigned ICD-10 codes by two physicians. We found substantial agreement regardless of the woman''s residence, whether the death was registered, religion, respondent''s or deceased''s education, age, hospital admission or gestational age. Physician agreement was not influenced by the above variables, with the exception of greater agreement in cases where the respondent did not live with the deceased, or early gestational age at the time of death. A central consensus panel reviewed all cases and recoded 10% of cases due to insufficient use of information in the verbal autopsy by the coding physicians and rationale for this reclassification are discussed.

Conclusion

In the absence of complete vital registration and universal healthcare services, physician coded verbal autopsies continues to be heavily relied upon to ascertain pregnancy-related death. From this study, two independent physicians had good inter-rater reliability for assigning pregnancy-related causes of death in a nationally-represented sample, and physician coding does not appear to be heavily influenced by case characteristics or demographics.     

Parental attitudes toward pediatric organ donation: a survey.

We conducted a telephone survey of parents in the National Capital Region to assess their intention to donate their child''s organs and to provide physicians with information that could help alleviate their concerns about approaching parents for consent. Of 339 parents who agreed to answer questions after being given details of their child''s "death" 288 (85%) said that they would be willing to donate their child''s organs. The degree of willingness was associated with the certainty of death, altruism and empathy toward children in need of an organ, previous discussion of organ donation with a family member and knowledge of an adolescent or adult child''s attitude toward donation. Factors that inhibited the intention to donate included uncertainty of death, insufficient information from medical professionals and fear of multilation. The child''s age was not significantly associated with intention to donate. Concordance between the results and actual donation rates in Canada and the United States supports the generalizability of the survey findings.     

Familial occurrence of typical and severe lethal congenital contractural arachnodactyly caused by missplicing of exon 34 of fibrillin-2.

Genetic linkage studies have linked congenital contractural arachnodactyly (CCA), a usually mild heritable connective-tissue disorder, to FBN2, the fibrillin gene on chromosome 5. Recently, FBN2 mutations in two patients with CCA have been described. Here we report an A-->T transversion at the -2 position of the consensus acceptor splice site, resulting in the missplicing of exon 34, a calcium-binding epidermal growth factor-like repeat in fibrillin-2 in a mother and daughter with CCA. Significantly, the mother exhibited a classic CCA phenotype with arachnodactyly, joint contractures, and abnormal pinnae, whereas her daughter exhibited a markedly more severe CCA phenotype, which included cardiovascular and gastrointestinal anomalies that led to death in infancy. Analysis of cloned fibroblasts showed that the mother is a somatic mosaic for the exon 34 missplicing mutation, whereas all the daughter''s cells harbored the mutation.     

Severity of labour pain: influence of physical as well as psychologic variables.

The role of physical factors in the severity of labour pain has been neglected. The amount of cervical dilation, the frequency of the contractions, the woman''s height and usual weight before the pregnancy and other physical factors were therefore examined in relation to the intensity of labour pain in 141 primiparous and 99 multiparous women. In general, pain increased gradually during labour in both groups of women, though the severity of the pain was lower in the women who had received prepared childbirth training than in those who had not. Although the average pain scores in this study were high, there were striking individual differences, some women having extremely severe pain and others having almost none. The pain scores in both groups of women were significantly correlated with the ratios of the women''s usual weight to height. In the multiparous women the scores were also correlated with the woman''s usual weight and the baby''s weight but not with the woman''s weight gain during pregnancy. Thus, the results show that physical as well as psychologic factors contribute to the severity of labour pain.     

Blunt abdominal trauma in pregnancy.

Two cases are presented in which the effects of blunt trauma to a pregnant woman''s abdomen were apparently minor but resulted in fetal death. Blunt trauma may result in serious injury to the fetus or the placenta. Three-point restraint systems should be worn by pregnant women travelling in automobiles to minimize the risks to mother and fetus. Awareness of the potential for injury in these circumstances is essential to reduce the risks to the fetus.     

Another Look at the Medical “Generation Gap”—Second Triennial Survey of Attitudes of Medical Students and Recent Graduates

A 1969 survey of attitudes held by medical students and recent graduates was repeated in 1972, using the same samples of respondents and adding a new freshman group. Findings from both surveys showed that a “generation gap” existed on many issues.Furthermore, a comparison of the two studies suggested that two kinds of attitude change are occurring simultaneously. On some issues, the next generation of physicians will probably continue to differ from their predecessors. Perhaps the primary thread woven through this cluster of attitudes is that today''s medical students and young physicians perceive themselves as members of a larger structure for providing health care.However, many of the students'' attitudes held early in their medical careers appear to have been modified in the process of becoming physicians. It appears that medical education continues to convey many traditional professional values to students, and it seems likely that tomorrow''s physicians will retain a core of those values.     

Maternal fatness and viability of preterm infants

To investigate the effect of maternal fatness on the mortality of infants born preterm up to the corrected age of 18 months 795 mother-infant pairs were studied. Maternal fatness was defined by Quetelet''s index (weight/(height²)) and all infants weighed less than 1850 g at birth. In 771 mother-infant pairs maternal age, complications of pregnancy, mode of delivery, parity, social class, and the baby''s sex and gestation were analysed by a logistic regression model for associations with infant mortality (but deaths from severe congenital abnormalities and those occurring during the first 48 hours after birth were excluded). In a subgroup of 284 mother-infant pairs all infant deaths except those from severe congenital abnormalities were analysed in association with the infant''s birth weight and gestation and the mother''s height and weight; this second analysis included another 24 infants who had died within 48 hours after birth. In the first analysis mortality overall was 7% (55/771), rising from 4% (71/173) in thin mothers (Quetelet''s index <20) to 15% (6/40) in mothers with grades II and III obesity (Quetelet''s index >30). After adjusting for major demographic and antenatal factors, including serious complications of pregnancy, maternal fatness was second in importance only to length of gestation in predicting death of infants born preterm. In the second analysis mortality overall was 15% (44/284), rising from 9% (5/53) in thin mothers to 47% (8/17) in mothers with grades II and III obesity. In both analyses the relative risk of death by 18 months post-term was nearly four times greater in infants born to obese mothers than in those born to thin mothers. In addition, maternal fatness was associated with reduced birth weight, whereas it is associated with macrosomia in term infants.These data differ fundamentally from those reported in full term babies of obese mothers. It is speculated that the altered metabolic milieu in obesity may reduce the ability of the fetus to adapt to extrauterine life if it is born preterm.     

Deaths under 50. Medical Services Study Group of the Royal College of Physicians of London.

The Medical Services Study Group has started a collaborative study in the Mersey, West Midlands, and Grampian regions to examine the causes of death among medical inpatients aged 1 to 50. The cause of death is determined from the case notes and the consultant''s opinion. The rate of ascertainment of cases was initially low, though it is increasing; despite this limitation an analysis of the first 250 cases showed one important finding. No fewer than 98 patients contributed to their own deaths through overeating, drinking, smoking, or not complying with treatment. Despite the present statistical limitations of the study, it has been valuable in enlisting the good will of the participating physicians, which will help to ensure the success of other projects, and it has avoided the problems inherent in determining the cause of death from death certificates alone.     

Manitoba municipalities want foreign-trained physicians to fill medical-care gap

For the third year in a row, Manitoba municipalities have passed a resolution asking that physicians trained outside the Commonwealth be permitted to work as medical residents in rural communities. The resolution noted that most graduates of the University of Manitoba''s Class of ''96 had already accepted positions outside the province. Although the provincial government has sweetened the pot for Canadian-trained physicians, a long-term solution to Manitoba''s critical shortage of rural physicians seems elusive.     

Death of phone service indicates Canadians not willing to pay for medical advice

The two physicians who started Canada''s first national pay-per-use medical-advice line closed the service after determining that Canadians are not willing to pay directly for their medical services. The issue of public versus private payments will likely be raised during the CMA''s annual meeting later this month.     

Physician communication skills and malpractice claims. A complex relationship.

We assessed the relationship between patients'' opinions about their physicians'' communication skills and the physician''s history of medical malpractice claims. The sample consisted of 107 physicians and 2,030 of their patients who had had an operation or a delivery. Although patients tended to give their physicians favorable ratings, they were least satisfied with the amount of explanations they received. Patients gave higher ratings to general surgeons and obstetrician-gynecologists and poorer ratings to orthopedists and anesthesiologists. Women and better-educated patients gave higher ratings on explanations and communication to physicians with fewer claims. Men and patients with less education, however, gave higher ratings on these dimensions to physicians with more claims. These findings suggest the need for physicians to tailor their communications to a patient''s individual needs. Improved communication between physicians and patients may result in fewer nonmeritorious malpractice claims while leading to less costly resolution of meritorious claims.     

Can the fall in Avon's sudden infant death rate be explained by changes in sleeping position?

OBJECTIVE--To examine the impact of changing practice with regard to infant sleeping position on mortality from the sudden infant death syndrome. DESIGN--A population based study of all infants dying suddenly and unexpectedly during February 1990 to July 1991, and two groups of controls; one comprising every 125th baby born to Avon residents and the other comprising pairs of infants matched to each index case for age, neighbourhood, and date of study. Information about sleeping position was collected at home visits soon after the index baby''s death or, for the population based controls, on several occasions in the first six months of life. The design was comparable to that of an earlier study of the same population. SETTING--County of Avon. SUBJECTS--35 infants who died suddenly and unexpectedly (32 of the sudden infant death syndrome), 70 matched controls, and 152 population based controls. RESULTS--The prevalence of prone sleeping in the matched controls was much lower than that found in an earlier study in Avon (28% (18/64) 1990-1 v 58% (76/131) 1987-9; p less than 0.001) and was comparable with the prevalence in population based controls (29%). This would be expected to lead to a reduction in the incidence of the sudden infant death syndrome to 2.0/1000 live births (95% confidence interval 1.8/1000 to 2.5/1000). The actual mortality fell from 3.5/1000 in 1987-9 to 1.7/1000. CONCLUSION--The fall in mortality can be almost entirely accounted for by the reduction in prone sleeping, suggesting a causal relation exists between them. Side and supine positions confer protection but the side position is unstable and the infant may roll prone. We therefore recommend supine as the safest sleeping position for babies.     

Support for parents experiencing perinatal loss.

Grief following perinatal loss is just as debilitating as that following the death of an older person and may not be completely resolved for years. The physician''s role in assisting parents following perinatal loss is one of a sympathetic listener and compassionate informant, but each category of perinatal loss--miscarriage, stillbirth, neonatal death and sudden infant death syndrome--requires a somewhat different approach. To be of assistance, physicians must understand the normal process of grief and the differences between the reactions of mothers, fathers and siblings. The advent of liberal attitudes to family visiting in perinatal units has helped parents better understand perinatal illness, and appropriate management in the event of perinatal death can greatly benefit the family.