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Introduction: Despite a decreasing incidence, sudden infant death syndrome (SIDS) is still the most frequent cause of death in industrial nations during the first year of life. Hypoxia plays a major role in the pathogenesis, but the exact mechanism is not fully understood., Methods: This study was based on personal considerations and a selective online literature search., Hypothesis: SIDS is the result of a frequently protracted ATP-depletion., Discussion: Especially in combination, all risk factors for SIDS favour an ATP-deficiency by increasing ATP-catabolism and/or by diminishing ATP-synthesis. Prenatal chronic hypoxaemia and an insufficient supply with nutrients lead to low birth-weight, reduced adipose tissue, elevated haemoglobin F, increased sympathetic activity, hypermetabolism, and diminished hypoxia tolerance in the neonates. Because of reduced adipose tissue, more energy for thermogenesis is needed after birth. In reaction to hypoxaemia, infants with risk factors show hyperventilation instead of hypoxic hypometabolism and respiratory depression. Enhanced breathing, however, requires additional ATP and causes increasing oxygen affinity, which is elevated physiologically during the first months of life. Thereby, tissue-hypoxia and diminished ATP-synthesis may arise. Besides, enhanced sympathetic activity leads to hypermetabolism and increased ATP-catabolism. While innate risk factors may reduce ATP-production in burdening situations, like food deprivation, postnatal hyperthermia and stress augment ATP-catabolism by hyperventilation and hypermetabolism and empty energy stores. For term newborns, the peak incidence of SIDS might be explained by the haemoglobin nadir of physiological anaemia and by the therefore reduced capacity for oxygen transport. Thereby, the risk of tissue-hypoxia, which follows increased oxygen affinity and vanishing ability to hypoxic hypometabolism, is further enhanced. The almost identical symptoms of SIDS and ATP-deficiency diseases like hypophosphataemia, heat stroke, and carbon monoxide poisoning support the presented hypothesis., (Georg Thieme Verlag KG Stuttgart.New York.)

In industrial countries, sudden infant death syndrome (SIDS) poses the most common mode of deaths occurring in the postneonatal period. During the last years the incidence has decreased and is currently less than 0.5 per 1000 live births. The cause of SIDS is still unknown. One theory claims that SIDS is due to suffocation caused by failure of central respiratory control, CO(2) rebreathing or airways obstruction. Further pathological factors including hyperthermia as well as impairment of the sleeping- and waking centre are in discussion. The most important risk factors include lying prone, heat exposure of the child, premature delivery, maternal age less than 18 years, smoking and drug abuse during pregnancy and the absence of breast feeding. SIDS is not an entity but a descriptive term for sudden and unexpected deaths in infancy without adequate cause of death being established by methods currently used. SIDS in forensic medicine is not only important because of its relative frequency, but also because of the differential diagnoses which have to be considered in each case. Therefore other natural and unnatural causes of death have to be explored by autopsy and careful postmortal examination (histological, toxicological, microbiological and virological analysis), taking into account circumstances at the death scene and medical history of the infant.

Sleep-related disturbed breathing and parasomnia in very young children are in the focus of epidemiological interest. The cardinal symptom, i.e. snoring, in connection with nocturnal perspiration, mouth breathing, susceptibility to infection of the upper respiratory tract and tiredness during the day or hypermotility, can be an indication of obstructive sleep apnea (OSA). The common treatment is adenotomy unless there is indication of allergic swelling of the nasal mucous membrane. Other anatomic predispositions for OSA must be considered (tonsillar hypertrophy, midfacial hypoplasia, micro- and retrognathia, e.g. in patients with Down's syndrome or patients with preoperated cleft lip face palate). Inhalative nasal corticoids are a possible alternative to adenotomy in light to medium grade cases of OSA. Tonsillotomy is indicated only in serious OSA cases, tonsillectomy is only justified in cases of chronic tonsillitis or more than 4-6 cases of angina in the last 12 months. Treatment with nasal CPAP is tolerated well also in childhood. Patients with central hypoventilation syndromes, insufficiency of the respiratory musculature or obesitas hypoventilation syndrome can usually be ventilated by non-invasive approach using a nasal mask. Patients suffering from parasomnia should always be asked if they snore at night because if OSA is diagnosed and treated, there are very good prospects of curing somnambulism as well. Like with narcolepsy and REM sleep, a close HLA association has also been identified for family somnambulism. In cases of parasomnia which becomes manifest only after very young age frontal lobe epilepsy should be suspected and searched by polysomnographic and simultaneous continuous nocturnal video surveillance. If reversive development or unclear motoric and utterance phenomena are observed, sleep-bound convulsive disorder should be looked for. Syncopal events can require comprehensive cardiological diagnosis, including exclusion of nightly disorders of the cardiac rhythm.

Hyperekplexia (OMIM 138491) is primarily an autosomal dominant disease characterized by exaggerated startle reflex and neonatal hypertonia. If untreated it can be associated with sudden infant death from apnea or aspiration pneumonia and serious injuries. Different mutations of the alpha1-subunit of inhbitory glyzine receptor (GLRA1) could be found. Clonazepame, a gammaaminobutyric acid (GABA) receptor agonist is the therapy of choice. An early diagnose will lead to appropriate treatment and genetic counseling.

Infant botulism represents a distinct entity of botulism. Ingestion of the ubiquitously present spores of Clostridium botulinum leads to germination of the organism and neurotoxin production in the infant intestine. Symptoms typically develop gradually in contrast to classical food botulism in which an acute onset of symptoms shortly after the ingestion of preformed toxin in a food is characteristic. Microbiologically, the diagnosis is established by identification of Clostridium botulinum organism and toxin in stool specimen. However, positive results in these tests provide only indirect evidence for the clinical relevance of the neurotoxin since asymptomatic carriers have been found. The toxin irreversibly blocks the release of acetylcholin from the motoric end plate which results in muscle weakness and paralysis. Depending on the amount of toxin produced, infant botulism exhibits a broad clinical spectrum ranging from oligosymptomatic forms to a fulminant course with acute respiratory failure within hours leading to sudden death. Unrecognized mild forms or beginning muscle weakness can be a co-factor for other risk factors of sudden infant death (SIDS). In studies analyzing infants who died from SIDS, botulism bacteria or toxin were found in up to 20 % of cases. Infant botulism therefore represents an important differential diagnosis of unexplained and inconclusive muscular hypotonia in the first year of life.

Botulism is caused by the blockage of the neural transmission in the cholinergic synapses by botulinum neurotoxin (BoNT) which is produced by Clostridium botulinum or other Clostridia. The classic form of botulism occurs after the ingestion of food contaminated by BoNT. The course of the infection can be asymptomatic, mild with subtle paralysis ("failure to thrive") oder severe with generalized paralysis ("floppy infant"). Infected infants can also die sudden and unexpectedly. These deaths often are attributed to Sudden Infant Death Syndrome (SIDS), unless a thorough postmortem examination reveals Botulism. The rate of fatal Botulism falsely attributed to SIDS is not known, because it is difficult in most cases to show the causal relationship between contamination, disease and death. We report the sudden and unexpected simultaneous death of twins of 22 months which could be attributed to Botulism. Contamination of food, colonization of the gut by Clostridia and infection with specific pathomorphological changes could be proven. The initial suspicion of infanticide could be excluded. lt could be shown, that Botulism is a potential cause of simultaneous unexpected deaths in twins.

An increasing amount of polysomnographic studies are performed in early infancy. Complete pediatric sleep laboratory systems are commercially available and provide fast and exhaustive results if operated by trained personnel. Experience in evaluation is necessary, because a broad range of possible diagnostic findings exists, and artifacts are common. Based on longitudinal studies in 31 healthy infants, we present reference values for several polysomnographic parameters. Aberrant results must be interpreted in the context of patient's history, clinical state, and additional laboratory findings. In presence of a clearly defmed indication for polysomnographic testing, clear therapeutic and interventional strategies can be derived. Possible consequences include the stimulation of the respiratory drive by drugs with subsequent polysomnographic control of therapeutic effect, a transfusion, the prescription of a home monitor, and the recommendation of further diagnostics or of control polysomnographies. In conclusion, infant polysomnography is an important diagnostic tool in pediatrics.

Unlabelled: Hypoperfusion of the brainstem during head rotation may be a risk factor for the development of SIDS. On this background we established a Doppler sonographic screening programme of the basilar cerebral arteries to evaluate the dependency of blood flow on head and body position., Patients and Method: We investigated 3840 newborns (1872 girls and 1968 boys) with a birth weight of 3399 +/- 497 g and a gestational age of 39.2 +/- 1.4 weeks. The investigations were performed in the neonatal period with an average age of 4.7 +/- 3 days. In all infants blood flow was measured in the basilar artery (BA) in supine position with the head in the midline. From the flow profile peak systolic flow velocity Vs and time average flow velocity TAV were measured. Additionally flow measurements were performed in supine and prone position with rotation of the head to the right and left side. A decrease of blood flow velocities below 50% of the value in neutral position was considered to be abnormal. Retrograde or biophasic flow profiles during rotation were considered to be pathologic. In infants with abnormal or pathologic flow during rotation of the head flow measurements in the vertebral arteries (VA) were additionally performed. Blood flow velocities in the VA were measured in supine and prone position with the head in the midline position and after rotation to the right and to the left. In neutral position unilateral vertebral hypoplasia, aplasia and normal VA were differentiated. The judgement after rotation was performed such as in the BA., Results: In 3807 infants (99.14%) blood flow velocities during head rotation did not decrease below 50% of the value measured in neutral position. In 33 infants (0.86%) a decrease of blood flow velocities below 50% could be found during rotation. In 7 infants (0.18%) a pathologic flow could be found during head rotation. 27 of the 33 infants with abnormal and pathologic blood flow in the BA during rotation showed anatomic abnormalities of the VA. 20 of these infants (61%) had unilateral vertebral hypoplasia (11 right, 9 left side), 7 (21%) had unilateral vertebral aplasia (4 right, 3 left side). 32 of the 33 infants with abnormal flow in the BA showed a decrease of blood flow in the contralateral VA during head rotation. 9 infants had an abnormal, 19 a pathologic flow within the contralateral VA. In 4 infants the corresponding VA could not be measured during head rotation. The decrease of blood flow velocities in the BA during head rotation was caused by compression of the contralateral VA at the craniocervical junction., Conclusion: Blood flow within the basilar artery of healthy infants is independent of body position and rotation of the head. A decrease of the flow velocities below 50% during rotation has to be considered as an abnormality. The incidence of pathologic blood flow during head rotation with 1.8@1000 approximates the incidence of SIDS. Hypoperfusion of the brainstem during head rotation may be a risk factor of SIDS.

With the decline of the former main causes of death in early childhood--infections and starvation--sudden infant death syndrome (SIDS) has emerged as the most important single cause of postneonatal infant mortality. It has adopted the role of a major indicator for the standard of public health care. Despite extensive input into research, its pathophysiology has remained rather obscure. The resulting helplessness of scientists and health care professionals have lead to adherence to unconfirmed pathophysiological hypotheses and to pursuit of preventive strategies of doubtful efficacy. In this overview, the medical and technical background of five major hypotheses is being presented. A lot can be learnt from the history of their development, efforts to refute them, and the reasons for unreflected adherence to them. (1) Due to its illustrative nature, the so-called 'status thymico-lymphaticus', the theory of asphixation by an enlarged thymus, could not be eradicated although well-reknowned physicians--including the Austrian pathologist Paltauf--have repeatedly attempted to do so. (2) Assumed familiarity, an aspect which attracted the attention of pediatricians to SIDS initially has been excluded, but an increased risk of SIDS for the siblings of affected babies is still common belief. (3) The sleep-apnea-hypothesis has turned out a complete error with serious consequences, but home apnea monitors are still being widely recommended. (4) The rise of SIDS in the 80ies and its subsequent decline in the 90ies has been interpreted as the advent and successful control of an epidemic although significant numbers of cot death have been reported long before the turn of the century, and the apparent increase which paralleled the introduction of the 9th edition of the ICD code is most likely due to improved registration. (5) Finally, SIDS is still being considered a random event--ignoring all evidence of an obvious role of socioeconomical factors.

Despite numerous investigations the pathophysiologic mechanisms of SIDS have not been fully elucidated. In large epidemiologic studies highly variable SIDS mortality rates were noted between different countries and cultures. This presumably is due not only to differences in diagnostics and classification of SIDS but also in lifestyle and newborn care. The common denominator is the identification and prevention of the main risk factors: smoking, sleeping in the prone position, over-heating, wrong "bedding". SIDS prevention campaigns that have focussed upon these risk factors have led to a dramatic reduction in the incidence of SIDS. In preparation for the SIDS prevention campaign of Vienna ("Safe Sleep") the content, strategy and procedure of the Austrian prevention campaigns were analysed. The current focus is to convey a clear and uniform message in personal conversations before and after birth of the child. These conversations with parents are the most important tool to detect SIDS related anxiety and a possibly increased risk of SIDS. In the last 30 years various polysomnographic parameters were published that were associated with an increased risk of SIDS. Today there is international consent that polysomnography is not an efficient screening method to demonstrate increased risk of SIDS. Therefore the use of polysomnography, besides research purposes, has been limited to investigating clinical symptoms of infants and children. Concerning monitoring it is important to note that--in contrast to the undisputed importance of monitoring breathing disorders--the effectiveness in SIDS prevention is unproven. State of the art are instruments that monitor heart and breathing rate and have adequate storage functions. The duration of monitoring should encompass the symptomatic period as well as a safety period of three months. The monitor should not be routinely prescribed for a year. The guiding principle is "As short as possible with stringent indication". Prerequisite for the monitoring is good instruction of the parents and a continuous consultation by competent outpatient clinics.

The description of an association between central apneas and the sudden infant death syndrome (SIDS) by Steinschneider et al. in 1972 was followed by a sharp rise of polygraphic studies. The hope to use these as a screening method for an increased risk of SIDS could subsequently not be confirmed by prospective studies. Pathological alterations, such as obstructive apneas and hypoxemias, however, seem to have prognostical relevance. They thus represent a focus of current research even though being unable as well to reliably predict a predisposition to SIDS for an individual subject. Instead of being used as a diagnostic tool for SIDS, polygraphic studies have nowadays acquired an accepted role in the investigation of certain physical symptoms and signs such as unexplained 'apparent life threatening events' (ALTE) or cyanotic attacks. Basic technical requirements for a polygraphic laboratory include facilities to record thoracic/abdominal respiration, nasal air flow, ECG/heart rate, oxygen saturation, and to classify different stages of sleep. Registration of additional parameters (EEG, breath sounds, video etc.) is optional, but may prove helpful to arrive at an appropriate diagnosis. Recently, Austrian and German centres have been trying in cooperation to standardize performance conditions and evaluation criteria for polygraphic measurements in pediatric patients under the guidance of the German Association for Sleep Medicine, Pediatric Workgroup. Their aim is to establish common guidelines for the diagnosis and treatment of sleep and arousal disorders in infancy and childhood.

Background: During the last years immunohistochemical techniques have improved the diagnosis of myocarditis using specific markers for quantification of leucocytes and characterization of T-lymphocytes together with the definition of MHC class II Antigens (HLA-DP, DQ, DR), known to be enhanced in cases of myocarditis. These techniques allow the diagnosis of myocarditis in an early stage of the inflammatory process in contrast to the conventional diagnostic with hematoxylin-eosin., Materials and Methods: 20 autopsy cases were examined in a retrospective study with conventional histological and immunohistological methods. After routine investigations including toxicological and histological examination of internal organs all cases were considered as cases of Sudden Infant Death Syndrome (SIDS)., Results: Increased number of LCA-positive leucocytes and CD-3-positive T-lymphocytes together with enhanced expression of inflammatory marker leads to the diagnosis of a lymphomonocytic myocarditis in three cases, in one case myocarditis could be diagnosed by conventional hematoxylin-eosin staining. In one of these four cases of myocarditis a positive immunohistochemical reaction was found using a new antibody against enteroviruses., Conclusion: Immunohistochemical techniques improve diagnosis of myocarditis in cases of suspected sudden infant death syndrome. Further studies using immunohistochemical inflammatory markers to control the incidence of acute myocarditis are necessary.

Viral infections can cause apnoea, bradycardia, and desaturation events in preterm and new born infants. These symptoms do not always occur in older infants. A link between virus infection, apnoea, apparent life threatening events (ALTE) and sudden infant death (SID) is speculated. We report a 6-week-old infant with long central apnoea as the first and main symptom of meningoencephalitis caused by enterovirus.

Aim: Hypoperfusion of the brain stem in dependence on head and body position followed by central bradycardia and apnea may be an important cause of sudden infant death syndrome (SIDS)., Methods: 39 infants with a mean age of 10.6 +/- 10.2 weeks (6 days to 11 months) with apparent life threatening events (ALTE) were investigated by cranial Doppler sonography. Additionally 68 healthy infants aged 6 days to 5 months (m 3.7 +/- 4.1 weeks) were investigated. In all patients flow measurements were performed in one anterior cerebral artery (ACA), both internal carotid arteries (ICA), the basilar artery (BA), and both vertebral arteries (VA) in dependence on head (right/left/neutral) and body position (prone/supine)., Results: In healthy infants flow velocities within all intracranial arteries were independent of head and body position. In none of our infants with ALTE significant flow alterations in the ACA and both ICA in dependence on head and body position could be found. In 23 patients with ALTE no dependence of flow in the BA and both VA of head and body position could be shown. In 16 infants however pathologic flow profiles with low flow velocities could be found in the contralateral VA if the head was rotated to the other side. In 9 infants additionally pathologic flow profiles with a dramatic decrease of the flow velocities in the BA could be found. The reduction of the blood flow is caused by compression of the contralateral vertebral artery at the craniocervical junction., Conclusion: The reduction of blood flow in the VA and especially the BA may cause hypoperfusion of the brainstem followed by central bradycardia and apnea. Hypoperfusion of the brainstem in dependence on head and body position may be a significant cause of SIDS. By means of cerebral Dopplersonography infants at risk for SIDS may be detected. Body and head positions which should be avoided can be evaluated noninvasively.

Sudden infant death [SID] is defined as the "sudden death of an infant under one year of age that remains unexplained after a thorough case investigation, including performance of a complete autopsy, examination of the death scene and the review of the clinical history". This definition, given by the National Institute of Child Health and Human Development in the USA. recognized SID as a diagnostic entity, however, SID remains a pathological diagnosis of exclusion. SID-cases are divided into three categories: group 1 in which autopsy does not reveal any cause of death, group 2 in which postmortem findings are not sufficient to be the cause of death; group 3 in which the death is adequately explained, is classified as "non-SID". The purpose of the study was to analyse the situation about SID over a 25-year period, from 1969 through 1993, looking for changes in frequency and developmental trends. The study includes 364 SID-infants which were examined according to a standard method. Histological, microbiological, virological and immunological studies were performed and data of the death scene investigation and of the clinical history were collected, 284 infants who had died due to clinical diseases served as one control group, a second group consisted of 6397 newborns of the city of Zürich. Of the SID-infants, almost every third infant [29%] was grouped in group 1, more than half of the infants [52%] with signs of mild infections in group 2, whereas almost every fifth child [19%] was grouped in group 3. With regard to epidemiological parameters the peak of mortality was found at about three months of age; the percentage of boys and twins, of third and later born infants was higher compared to the general population. SID probands were born twice as often in October as in March. The percentage of mothers of first, second and third born SID-infants at an age younger than 20, 22 and 24 years was higher compared to the general population. All these parameters were also found in group 3. The risk of familial recurrence in group 1 and group 2 was 0.8%. Between 1969 and 1993 the rate of SID deaths dropped from 1.1 to 0.9 per thousand live births, whereas SID as a percentage of postneonatal mortality increased to 34 per cent.

A relationship between neurovegetative symptoms and an increased risk of sudden infant death (SID) has been frequently described. Such symptoms are vomiting because of gastroesophageal reflux, breathing disorders while nutritive sucking, excessive sweating during sleep, prolonged apneas and apneas with associated symptoms, further unexplained episodes of cyanosis, pallor and loss of muscle tone. An acute decompensation of the cardiorespiratory autonomic system during sleep is currently discussed to be one of the causes of SID. Polysomnographic investigations are used to diagnose sleep related cardiorespiratory autonomic dysfunction. In this study, infants up to the age of 5 months were examined using polysomnography throughout their entire nocturnal sleep. The findings from 79 infants with neurovegetative symptoms were compared with the results from 163 healthy control children. Indications of an impaired respiratory regulation during sleep were shown to be significantly more frequent in infants with neurovegetative symptoms. In particular the occurrence of periodic breathing with a high frequency of periodic apnea plus a large number of acute decreases in oxygen saturation could be shown in infants with neurovegetative symptoms. The findings were most pronounced during the first 1.5 months after birth. The results of this investigations lead to the conclusion that, in the case of young infants with breathing abnormalities, neurovegetative symptoms can frequently be observed. The occurrence of such symptoms should therefore be a reason for a polysomnographic examination.

Apnea characteristics of infants, who were destined to die, were compared with control infants, matched for gestational age, sex, birth weight and age at sleep study. From 1989 to 1994 polygraphic recordings were performed in 4268 infants. In this population 7 cases of sudden infant death (SID) and 13 deaths from other causes (non-SID) occurred. Concerning all forms of apneas a higher frequency and maximal duration was found in the SID group. The number of infants with obstructive apneas and the number of obstructive apneas per infant were higher in future SID victims, as was the frequency, mean and maximal duration of central apneas of 3 to 10 s. The recordings of non-SID-infants were not different from their controls. As a group, future SID victims showed apnea characteristics different from control infants. Together with epidemiologic risk factors these polygraphic factors contribute to an identification of infants at risk for SID.

In a pilot study we interviewed parents of 100 formerly home monitored infants about problems during the monitoring period. The duration of home monitoring was between 1 and 48 months (median 15 months). 95% of the parents reported false alarms. 66% of the infants had real alarms, in 50% of the infants interventions were necessary. 3% of the infants had to be resuscitated. Only 47% of the parents felt safe in the practice of resuscitation even though all parents had been trained before. Perspectives of improvement of home monitoring are discussed.

Oxycardiorespirographies, recording arterial oxygen saturation (SaO2), breathing movements, heart rate and ECG with a mean recording time of 22.3 hours, were performed on 85 preterm (mean postconceptional age: 38 weeks) and 81 term infants (mean postconceptional age 42.4 weeks). 83% of the preterm infants showed periodic breathing (PB), in 97% of them this was accompanied by periodic variations of arterial oxygen saturation (PVO). Periodic breathing occurred in 61% of the term infants, 84% of them showed PVO during periodic breathing. The mean variation of oxygen saturation was between 92.8 and 96.8% (+/- 1.7) for preterm and between 92.9 and 96.0% (+/- 2.2) for term infants. In some infants the peak to peak amplitude of the SaO2 cycles was up to 22%, sometimes a further fall of SaO2 occurred. There was a strong correlation of the PVO both at the beginning and end of the episode as well as with the PB-cycle periodicity itself. The fall of the oxygen saturation occurred 3.1 to 7.8 s after the beginning of the first apnea of an episode of periodic breathing, the minimum SaO2 was reached approximately 4.2 to 8.6 s later. This periodic rapid fall of SaO2 from a high oxygenation level cannot be explained by the apneas of a rather short duration during periodic breathing. It is discussed that PVO during periodic breathing may be caused by an ideopathic right to left shunting across fetal circulation pathways which occurs intermittently and periodically. This mechanism could-via patterns of reaction exhibited during the fetal and neonatal time period-lead to acute hypoxemia, as found in apparently life threatening events (ALTE) and as postulated in sudden infant death (SID).

Parents and attending physicians of 100 SIDS victims were interviewed applying a questionnaire especially designed for this purpose. Particular attention was focussed on the courses of pregnancy and birth, medical check-ups and the infants' unusual behaviour during the last days of their lives. As far as possible we compared our data with the results of the Rhenian Perinatal Survey 1990.

The occurrence of obstructive apneas in premature and young infants is associated with a higher risk for SIDS. In order to assess the incidence of obstructive apnoeas in infants with different risk for SIDS pneumography was performed including the registration of the nasal air flow in 312 children: 69 preterm infants, 42 children after intensive care, one infant that later died of SIDS, 14 children after ALTE, 84 children after apnoeas observed by their parents, 25 siblings of SIDS-victims and 77 controls. Obstructive apnoeas were found in 24.6% of the preterm infants, in 28.5% of the children after intensive care, in 50% of the children after ALTE and in the one infant that later died of SIDS. Obstructive apnoeas however were registered only in 16.7% in the "apnoea-group", in 12% of the SIDS-siblings and in 11.7% of the controls. We therefore conclude that obstructive apnoeas which were observed more frequently in children with a higher risk for SIDS are of predictive value for the SIDS risk. Pneumography should therefore include the measurement of the nasal air flow. Home monitoring should be performed with devices that are able to assess bradycardias as indirect signs of obstructive apnoeas.

Sudden infant death syndrome [SIDS] is the most common cause of death between the ages of one month and one year, affecting one to three infants of 1000 life births. SIDS is defined as a sudden death of an infant under one year of age, which remains unexplained even after performance of a complete postmortem examination, examination of the death scene and review of the case history. SIDS is unexpected, so parents have not had the opportunity to prepare for the death. Because SIDS remains unexplained, SIDS parents blame themselves for the death. For most families of SIDS victims, the best resource for help and support is a SIDS parent support group. Finally, risk factors and possible preventive measures are discussed.

The harmful effects of passive smoking (PSE) start early in intrauterine life and comprise direct toxic effects of components of tobaccos smoke on the fetus as well as indirect effects by impeding the normal nutrition of the growing child over effects on the placenta. Consequences are diminished birthweight, and increased perinatal mortality. The sudden infant death syndrome is associated with PSE as are increased incidence of respiratory illnesses in early childhood. Increased bronchial responsiveness, increased asthma prevalence, delayed lung growth and increased incidence of chronic respiratory symptoms later in childhood may well put these children at increased risk for developing chronic obstructive pulmonary disease in their later life.

The article reviews the history of hypoglycaemia in children, documenting its importance in the genesis of neurological damage while attacking conventional dogmas on postnatal changes and the definitions of hypoglycaemia. A great deal of further research needs to be performed on the levels of blood glucose leading to "functional" neurological changes, as well as further research on the effects of hypoglycaemia on NMDA receptors. Of greatest practical importance would be new and better monitoring techniques to obtain more precise biochemical data that are instantly available to the clinical staff at the bedside. Finally, modern technology can be applied in defining one particular cause of what may be regarded as "idiopathic hypoglycaemia". A knowledge of basic biochemical processes can be used to predict patterns of metabolite profiles which are pathognomonic of the underlying disease.

Survivors of an "apparent-life-threatening-event" subsequently more often die from sudden infant death syndrome than others. The aim of this study was to find out abnormal clinical symptoms and/or polysomnographic patterns in this group of patients. Between January 1989 and September 1990 122 infants (mean age 13.98 weeks) were examined after a life threatening event (mean age 9.3 weeks at the event). In total, 222 polysomnographic studies were performed. In 46 cases additional esophageal pH-metric measurements, and in 26 cases a Holter 24 hours monitoring were done. Seven infants were premature and had been ventilated, and 6 were siblings of sudden-infant-death-syndrome victims. Pulmonary problems were identified in 7 (6%), cardiac problems in 17 (14%), 18 (15%) had neurological problems, and 40 (33%) showed a gastroesophageal reflux. In 14 (11%) other diseases were found. Only 43 (35%) infants were without pathologic findings and were classified as having had an "idiopathic" event. The polysomnographic studies showed that obstructive apnea occurred significantly more often, the maximal duration of apnea was longer, and the number of sudden pO2 decreases was significantly higher than in a group of 188 normal infants. Thus, patients having had an "apparent-life-threatening-event" showed a broad spectrum of abnormal clinical symptoms and some respiration disturbances compared to a reference group of infants.

In a prospective study (1988-1990) the SIDS risk questionnaire SRFB was applied to 6000 infants born in the Department of Obstetrics, University Hospital Graz. In all infants at an increased statistical risk for SIDS a standardized intervention including pediatric and polysomnographic investigations was performed. As a result the SIDS incidence decreased from an average of 1.92 per thousand lifeborns (1984-1988) to 0.83 per thousand lifeborns (1989-1990) in our test group, which means a striking reduction of the SIDS incidence by 57%. In addition to this effect, the intensified information of parents and physicians about possible signs of an increased risk for SIDS lead to a general reduction of SIDS in Styria.

Based on the results from semistructured interviews with parents of fifty SIDS victims and of fifty matched controls we developed a SIDS risk questionnaire, the so-called SRFB Graz. In a retrospective study this questionnaire was applied to 65 SIDS victims and 195 comparable controls. By statistical analysis a cut point was computed, which discriminates SIDS cases from controls with a sensitivity of 86% and a specificity of 95%.

While many aspects related to the etiology and pathogenesis of sudden infant death remain unclear, the ultimate trigger event appears to be an acute regulatory disturbance of the cardiorespiratory neurons of the reticular formation of the brain stem. Retrospective studies have demonstrated that SIDS often occurs after infection of the upper respiratory tract. We present the case history of a 10-week old infant, where the respiratory pattern demonstrated highly pathological alterations induced by a mild infection of the upper airways, as confirmed by oxycardiorespirography. Oxycardiorespirography provides continuous recording of the respiration (transthoracic impedance) extending over several hours during sleep and during the critical phase just before going to sleep and waking up in relation to heart rate, partial oxygen pressure (measured transcutaneously) and nasal flow. The presented infant is a premature infant delivered in the 34th week of gestation according to Dubowitz (artificial respiration for 7 days due to hyalin membrane syndrome stage II). During infection periodic breathing was found to be 19.3% with partly severe hypoxemia. The longest single episode of apnea was 30 seconds. On the average, 5 episodes of bradycardia occurred (less than 100/min/measuring hour). An OCRG investigation performed at the age of 5 weeks and after disappearance of the infection at the age of 12 weeks yielded normal findings. This permits the conclusion that in out patient an infection of the upper airways may have induced a pathologic respiratory pattern, which could be a major trigger mechanism for sudden infant death.

We analysed the pregnancy and delivery courses in 23 children, who subsequently died of SIDS in Berlin. 83% (n = 19) of these children had died by the 6th month of their life, and more than 90% by the 8th month. The first precautionary health examination during pregnancy was performed markedly late, in 22% of the pregnant women only after the 20th week of gestation. 55% of all "SIDS mothers" had smoked more than 10 cigarettes daily during the pregnancy period (control group: 8%). Preterm delivery rate was 30%, i.e. almost three times that of the control group. Almost one-third of all the examined children had a birth weight below the 10th percentile (control group: less than 7%). 61% of all mothers of the examined group by us were unmarried or divorced (control group: 16%). Whether these anomalies--which must of course also be considered as being somehow connected with one another--are causally connected with SIDS, requires to be clarified by more extensive studies. At the present time we can only recommend to make an effort to convince families, in whom several of the above mentioned anomalies occur, to accept special counselling and care as well as meaningfully employed home monitoring.

A case of "shaken baby syndrome" (SBS) is reported. A 3 1/2 months old female baby has been found by his mother death in bed. The circumstances predicted a case of Sudden Infant Death Syndrome (SIDS), but the father, a disc-jockey, who had to look for the baby, had left a notice: "Gitti--i didn't want it, I don't know what had happened, I'm sorry!". The corpse of the baby had no external signs of violence. At the medicolegal examination we found small subarachnoidal and subdural hemorrhage from ruptured bridge veins and signs of prolonged asphyxia. Few hours later the father could be arrested. He told that he had shaken the baby, because she didn't stop crying. The infant lost conscious and he layed him down to the bed with his face below. Then he looked TV for at least a few minutes. After insufficient reanimation he had left the house. The court found the man to be guilty of fatal infant child abuse and convicted him to detention of 8 months. The main signs of SBS are discussed (subarachnoidal and subdural hemorrhage, intraretinal and periretinal hemorrhages, brain edema). Especially in the german speaking Europe many medical examiners are not familiar with this form of infant child abuse.