Nasal and intrapulmonary haemorrhage in sudden infant death syndrome.

BACKGROUND: Fresh intrapulmonary and oronasal haemorrhages in cases of sudden infant death syndrome (SIDS) might be markers for accidental or intentional smothering inappropriately diagnosed as SIDS. AIM: To compare the incidence, epidemiological association, and inter-relation of nasal haemorrhage, intrapulmonary haemorrhage, and intrathoracic petechiae in infant deaths certified as SIDS. METHODS: In SIDS cases from a large nationwide case-control study, a wide range of variables were compared in cases with and without reported nasal haemorrhage and, in a subgroup of cases, in those with and without pathologically significant intrapulmonary haemorrhage. RESULTS: Nasal haemorrhage was reported in 60 of 385 cases (15%) whose parents were interviewed. Pathologically significant intra-alveolar pulmonary haemorrhage was found in 47% of 115 cases studied, but was severe in only 7%. Infants with nasal haemorrhage had more haemorrhage into alveoli and air passages than age matched cases without nasal haemorrhage. In multivariate analysis, nasal haemorrhage was associated with younger infant age, bed sharing, and the infant being placed non-prone to sleep. Intrapulmonary haemorrhage was associated with the same three factors in univariate analysis, but in multivariate analysis only younger infant age remained statistically significant. There was no significant association between nasal or intra-alveolar haemorrhages and intrathoracic petechiae. CONCLUSIONS: Nasal and intrapulmonary haemorrhages have common associations not shared with intrathoracic petechiae. Smothering is a possible common factor, although is unlikely to be the cause in most cases presenting as SIDS.     

Laryngeal chemosensitivity: a possible mechanism for sudden infant death.

In 32 anaesthetized piglets 1 to 42 days of age the distal trachea was cannulated and pressure changes were recorded. The proximal trachea was cannulated for introduction of test fluids into the laryngeal area. Arterial pressure, heart rate, and central venous pressure were continuously recorded. Arterial blood samples were obtained at intervals and analyzed for PO2, PCO2, pH, and hematocrit. Normal saline produced no, or brief, transitory alterations of the respiratory pattern and arterial pressure. In contrast, instillation of distilled water produced apnea in 29 of 30 piglets. In 20 the apnea was sustained. Eleven died within approximately 50 minutes of asphyxia. An additional nine were expected to die with continuing apnea (PO2, 10 to 15 mm Hg; PCO2 greater than 100 mm Hg; pH smaller than 6.8) but the sequence was interrupted by replacement of water with saline. Twenty-three of 29 piglets showed an apneic response to cow's milk similar to that seen with distilled water. Seven died of asphyxia and an additional three showed sustained respiratory inhibition until milk was replaced with saline. The responses were completely abolished by superior laryngeal nerve (SLN) sectioning. Electrical stimulation of the SLN produced sustained apnea in seven of eight piglets tested. Studies in two 3-day-old lambs showed similar discrimination but only transitory apnea with water or cow's milk. Both died during SLN stimulation. Two ewes showed insignificant responses. These findings suggest a lethal reflex mechanism with implications for the SIDS problem.     

Potential role of intrapulmonary shunting in the genesis of hypoxemic episodes in infants and young children.

A number of physiologic phenomena during some apneic/cyanotic episodes in infants and young children cannot be explained sufficiently on the basis of a cessation or reduction in alveolar ventilation alone. These include (1) the extremely rapid development of hypoxemia during some episodes, (2) the occurrence of hypoxemic episodes despite continuous ventilation, (3) differences in the speed of desaturation between different forms of apneic episodes, (4) the presence of continued breathing efforts and yet absent airflow despite bypass of the upper airway, and (5) evidence that apnea and hypoxemia may begin simultaneously. Potential mechanisms to explain these phenomena include the sudden development of an unevenness in the matching between ventilation and lung perfusion, the flow of blood through unventilated areas of lung, and a sudden impairment in gas diffusion. Evidence from physiological and morphological studies suggests that all of these mechanisms, although to a different extent, may be involved in apneic/cyanotic episodes. The recognition that nonapneic mechanisms can cause sudden profound hypoxemia may alter our approach to the identification, treatment, and prevention of these episodes.     

Recommendations for sudden infant death syndrome prevention: a discussion document.

This article reviews the evidence for the current UK Department of Health recommendations for prevention of sudden infant death syndrome (SIDS) and suggests other factors that should be considered. The wording of the Department of Health recommendations for SIDS prevention has changed over the past 6 years, but the specific recommendations are largely consistent with the scientific evidence. The emphasis on thermal and illness factors and immunisation could be reduced. Bed sharing and sharing the parental bedroom should be given more emphasis. Two major recommendations need to be discussed in greater detail: (1) breast feeding and (2) pacifier use. Meta-analyses or reviews looking at each risk factor or a combination of risk factors are required. Further, it is recommended that a committee is established that reviews the recommendations and publishes the evidence that leads to these recommendations, as is done by the American Academy of Pediatrics Taskforce on Sudden Infant Death Syndrome.     

Bereavement support following sudden and unexpected death: guidelines for care.

Dealing with families who have suffered a sudden and unexpected death is a skill that may be needed by any paediatrician. Offering a bereavement follow up meeting to such families is part of accepted practice and is perceived to be of value in helping the family to come to terms with the loss. Unfortunately, there is very little guidance on the objectives for such a meeting, or the training required to help staff conduct such meetings. The nature of the work on a paediatric intensive care unit (PICU) means that staff have a greater experience of handling families in such a situation. We have reviewed our experience over the past five years following up the families of 51 children who have died suddenly and unexpectedly in our regional PICU. In doing this we have identified five key elements that we suggest are essential to a successful follow up meeting, and have supported this with case studies as illustration.     

Recommendations for sudden infant death syndrome prevention: a discussion document

This article reviews the evidence for the current UK Department of Health recommendations for prevention of sudden infant death syndrome (SIDS) and suggests other factors that should be considered. The wording of the Department of Health recommendations for SIDS prevention has changed over the past 6 years, but the specific recommendations are largely consistent with the scientific evidence. The emphasis on thermal and illness factors and immunisation could be reduced. Bed sharing and sharing the parental bedroom should be given more emphasis. Two major recommendations need to be discussed in greater detail: (1) breast feeding and (2) pacifier use. Meta-analyses or reviews looking at each risk factor or a combination of risk factors are required. Further, it is recommended that a committee is established that reviews the recommendations and publishes the evidence that leads to these recommendations, as is done by the American Academy of Pediatrics Taskforce on Sudden Infant Death Syndrome.     

Systemic to pulmonary arterial shunts for patients with cyanotic congenital heart disease: Current status

The various central vascular shunting operations used for palliation of patients with congenital heart disease plus decreased pulmonary blood flow are discussed. The status of each in current practice is outlined in order to provide an up-to-data reference for the pediatric radiologist. In general, the Waterston shunt is preferred for infants under six months and the Blalock-Taussig anastomosis for patients older than this.Supported by: Grant 260, Children's Bureau, Maternal and Child Health Service, Department of Health, Education and Welfare, Washington, D. C.; and grants from the National Heart and Lung Institute of the National Institutes of Health, the John A. Hartford Foundation, Inc., and the Max Kade Foundation, Inc.     

Risk factors for sudden infant death syndrome: changes associated with sleep position recommendations.

OBJECTIVE: To quantify the decreased incidence of sudden infant death syndrome (SIDS) and investigate risk profile changes associated with changes in infant sleep position. DESIGN: A population-based case-control study that used birth and death certificates from Washington State. Cases (n = 1515) were all singletons born in Washington between 1985 and 1995 (excluding 1991) who died of SIDS. Control cases (n = 6060) were randomly selected singletons born in the same period who did not die of SIDS. Multivariate logistic regression compared changes in the magnitude of associations between risk factors and SIDS before and after 1991. RESULTS: The incidence of SIDS in Washington declined from 2.6 (1985) to 1.0 (1998) per 1000 births. Over time, the risk of SIDS increased for low birth weight infants (odds ratio [OR] 2.1 rose to 3.6), and infants born to mothers who were smokers (OR 2.7 rose to 3.7), unmarried (OR 1.4 rose to 2.0), black (OR 1.4 rose to 2.5), or received limited prenatal care (OR 1.5 rose to 2.5). CONCLUSION: Five exposures were associated with increased risks of SIDS. These may not require a prone sleep position for their deleterious impact being associated with distinct causal pathways, or possibly indicating subpopulations yet to recognize the importance of the supine sleep position.     

Obstructive sleep apnea and near miss for SIDS: I. Report of an infant with sudden death.

An infant girl followed up from birth to death at the University Medical Center had "congenital stridor' and a "near miss for SIDS' event at 3 months of age. As part of an ongoing SIDS research project, she underwent 24-hour polygraphic monitoring at 21 weeks of age. Sudden infant death occurred within 30 hours after the polygraphic study. Polygraphic data obtained from this infant are compared with those from control infants and other infants with near miss for SIDS who were of similar ages. The number of mixed and obstructive respiratory events during sleep was abnormally high on the infant's recording. Histologic findings, involving particularly the midline structures of the brain stem, are discussed.     

Successful living donor liver transplant in a child with Abernethy malformation with biliary atresia,ventricular septal defect and intrapulmonary shunting

Abstract: Congenital portosystemic shunts are the anomalies in which the mesenteric venous drainage bypasses the liver and drains directly into the systemic circulation. This is a report of a rare case of LDLT in a four‐yr old male child suffering with biliary atresia (post‐failed Kasai procedure) associated with (i) a large congenital CEPSh from the spleno‐mesentric confluence to the LHV, (ii) intrapulmonary shunts, (iii) perimembranous VSD. The left lobe graft was procured from the mother of the child. Recipient IVC and the shunt vessel were preserved during the hepatectomy, and the caval and shunt clamping were remarkably short while performing the HV and portal anastomosis. Post‐operative course was uneventful; intrapulmonary shunts regressed within three months after transplantation and currently after 18 months following transplant child is doing well with normal liver functions. CEPSh has been extensively discussed and all the published cases of liver transplantation for CEPSh were reviewed.     

A study of bereavement care after a sudden and unexpected death.

Bereaved parents'' perceptions of care after the sudden, unexpected death of their child (from 1 week to 12 years), and the care that was or could be offered by statutory and voluntary agencies, was assessed in 11 health districts in seven regions of England and Wales. In these 11 districts, 185 families were identified who met the criteria of the study. Permission to contact these families was given by only 72 general practitioners. Of these, 42 families responded (58%). Sudden infant death syndrome accounted for 43% of the deaths. The results from postal questionnaires sent to both parents showed that hospital care was perceived as good on the whole, although parents would like more choices. Most parents felt that community care was inadequate, leaving many feeling isolated. In contrast, questionnaires from health visitors and general practitioners in the same health districts showed that they believed that they were the most appropriate professionals to give follow up care, but as there were few policies to guide them and little training provided, felt unable to offer support.     

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.     

Bereavement support following sudden and unexpected death: guidelines for care

Dealing with families who have suffered a sudden and unexpected death is a skill that may be needed by any paediatrician. Offering a bereavement follow up meeting to such families is part of accepted practice and is perceived to be of value in helping the family to come to terms with the loss. Unfortunately, there is very little guidance on the objectives for such a meeting, or the training required to help staff conduct such meetings. The nature of the work on a paediatric intensive care unit (PICU) means that staff have a greater experience of handling families in such a situation. We have reviewed our experience over the past five years following up the families of 51 children who have died suddenly and unexpectedly in our regional PICU. In doing this we have identified five key elements that we suggest are essential to a successful follow up meeting, and have supported this with case studies as illustration.