[Survival, Medical Care and Quality of Life in Children with Trisomy 13 and 18]. / Überleben, medizinische Betreuung und Lebensqualität von Kindern mit Trisomie 13 und 18.

BACKGROUND: While infants with trisomy 13 (T13) and trisomy 18 (T18) are known to die early, parents want to know more about life expectancy and quality of life. METHODS: 30-year single-center retrospective chart analysis (1980-2010) of cytogenetically confirmed T13 and T18 cases. Mothers of infants who had lived 3 months or longer were approached to judge their infant's quality of life and talk about their experiences with medical staff. RESULTS: Data of 18/20 T13 infants and 18/21 T18 infants could be retrieved. Median survival times were 5 d for T13 and 19 d for T18. One T13 and 2T18 children survived past 1 year. Out of 5 mothers whose infants had survived at least 3 months, 4 described their infant as friendly, happy and peaceful. They observed some degree of psychomotor development and were in favour of the numerous medical and surgical interventions performed. They wished to have had a doctor coordinating these interventions and missed an active offer for psychological help. CONCLUSION: While most infants with T13 or T18 die as neonates, mothers of infants surviving longer periods of time have positive memories about their infants' quality of life.

[Palliative Care in the Delivery Room]. / Palliativersorgung im Kreißsaal.

Palliative care in the delivery room is a multiprofessional challenge directed to the dying newborn and the parents as well as to the care-givers. Clinical experience shows that many aspects regarding this unique palliative care situation are not well known and cause relevant emotional distress to the health care providers. In this article basic background information of palliative care of newborns in the delivery room are presented. Furthermore, recommendations are given focusing on 2 main aspects: 1. non-pharmacological and pharmacological ways of symptom control in palliative care of dying newborns and 2. meeting the individual psychological, emotional and spiritual needs of the parents.

[Individual neonatal end-of-life care and family-centred bereavement support]. / Familienzentrierte Sterbe- und individuelle Trauerbegleitung an einem Perinatalzentrum.

Neonatal end-of-life care and family-centred bereavement support in perinatal medicine are a multiprofessional challenge directed to the dying newborn and the parents as well as to the care-givers. Clinical experience shows that many aspects of individual neonatal end-of-life care and family-centred bereavement support are not well known to the health-care providers. This is especially true for a standardised quality management and the components of bereavement support offered to parents. An interdisciplinary concept for an individual neonatal end-of-life care and famlily-centred bereavement support has been developed at the Center of Perinatal Medicine at the Charité, Berlin. The concept aims for two main aspects: (1) meeting the individual medical, psychological, emotional and spiritual needs of the dying newborn, the parents and family, and (2) facilitating standardised and process-orientated preparation, evaluation and reflexion of every case of end-of-life care. In this article some recommendations for implementing a basic care concept for families and their dying newborns are presented.

Primary palliative care in the delivery room: patients' and medical personnel's perspectives.

OBJECTIVE: To investigate circumstances of primary palliative care (PPC) in the delivery room (DR), medical personnel's experience with neonates who died under PPC in the DR and perceived sources of care-related distress in DR staff. STUDY DESIGN: Retrospective chart review of all neonates who were cared for under PPC in the DR during the years 2000-2010 at Charité University Medical Center Berlin, and structured face-to-face interviews with DR nursing staff and physicians. RESULT: Neonates undergoing PPC could be grouped as preterm infants at the limits of viability with a gestational age between 22 (0)/7 and 23 (6)/7 weeks (n=86, 76%) and newborn infants with complex chronic conditions (n=27, 24%). The median age of neonates at death was 59 min (interquartile range [IQR] 28-105 min). Most of DR staff did not report relevant signs of distress in dying neonates, and providing palliative care was not named as a relevant care-related source of distress by medical personnel. However, half of the participants reported on high degrees of caregiver's emotional distress in PPC situations, identifying insecurity of how to communicate with parents and to provide emotional support as the most common source of distress. CONCLUSION: Caregiver's emotional distress primarily originates from providing support to parents and not from providing medical care to the dying newborn. Implications for future practice include the need for structured education to improve DR staff's communication and counselling skills related to parents in PPC situations.

A direct catecholaminergic projection from the brainstem to the neurohypophysis of the rat.

The presence of the catecholamines dopamine and noradrenaline in the posterior pituitary is well documented. Dopaminergic terminals are thought to derive from cells in the periventricular hypothalamus including the rostral arcuate nucleus, but the origin of the noradrenergic terminals is uncertain. The majority of central noradrenaline-containing neurons are in the brainstem and lesions of the ventral noradrenergic tract significantly reduce the noradrenaline content of the neural lobe. We have explored the possibility of a direct noradrenergic projection from the brainstem to the neural lobe using as a retrograde tract tracer horseradish peroxidase alone or in combination with immunohistochemical detection of tyrosine hydroxylase. Horseradish peroxidase was injected into the neural lobes of 20 rats using a ventral approach, and the animals were perfusion fixed 12 h later. In all 20 cases, cells retrogradely labelled with horseradish peroxidase were found not only in the periventricular zone and magnocellular nuclei of the hypothalamus but also in the A2 region of the brainstem. In all six cases simultaneously processed for horseradish peroxidase and tyrosine hydroxylase immunohistochemistry, retrogradely labelled cells in both the arcuate nucleus and A2 region were found to be tyrosine hydroxylase-positive. These findings demonstrate the presence of a direct projection from catecholaminergic neurons in the A2 region of the brainstem to the neurohypophysis. Since catecholaminergic neurons in this region are known to be noradrenergic and lesions of the ventral noradrenergic tract deplete the neurohypophysis of noradrenaline, these neurons may represent an important source of noradrenaline in the neurohypophysis.

Stimulus-related changes in the dendrites of magnocellular neurones.

A Golgi-Cox study was undertaken to determine whether enhanced electrical activity was associated with any morphological changes in the dendrites of the magnocellular neurones in the hypothalamic supraoptic nucleus. Brattleboro rats, animals dehydrated by administration of 2% sodium chloride solution instead of drinking water and animals given 1% sodium chloride solution and deoxycortone to induce vasopressin-dependent hypertension were compared with controls. In each of the stimulated groups, the cell bodies were hypertrophied implying that the stimuli were effective. Dendritic span (the area of a triangle drawn round, and containing the entire Golgi-stained dendritic tree) was significantly increased (p less than 0.01) in Brattleboro rats but was decreased by sodium chloride-induced dehydration (p less than 0.01). Deoxycortone treatment reversed the reduction induced by dehydration. Hippocampal cells showed no significant differences. Thus, the cells of the magnocellular system rapidly alter their morphology when stimulated but the changes are more complex than a simple hypertrophy associated with enhanced activity.

Pulmonary arteriovenous fistulas in association with hereditary hemorrhagic telangiectasia and carotid aneurysm.

A case is presented of a patient with multiple bilateral pulmonary arteriovenous fistulas, hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber Disease), and an intracranial carotid aneurysm. Over a twenty-five year period, the patient has survived several life-threatening complications, and has successfully undergone staged bilateral pulmonary lobectomies and clipping of the carotid aneurysm. Preoperative evaluation included cardiac catheterization studies both with and without temporary balloon occlusion of the pulmonary artery. The pathogenesis, clinical features, pathophysiologic changes, and treatment of pulmonary arteriovenous fistulas are discussed.

Pilot study of experiences and needs of 111 fathers of very low birth weight infants in a neonatal intensive care unit.

OBJECTIVE: To study the experiences and needs of the fathers of very low birth weight (VLBW, <1500 g) infants in a neonatal intensive care unit (NICU). STUDY DESIGN: An anonymous self-report questionnaire was administered to fathers of VLBW infants born between Jan 1, 2008 and December 31, 2009 at two tertiary NICUs. RESULT: A total of 111 of 273 fathers responded to the questionnaire. Responses to a variety of items, including self-perception, parenting attitudes, confidence, emotional pressure to satisfaction and self-efficacy as a parent were similar for the fathers of this survey and previous results for mothers of VLBW infants. Fathers judged direct bedside support by the NICU team as sufficient. However, 54.4% of fathers reported missing nonbedside interventions such as VLBW father-specific baby care courses, seminars or workshops, and platforms or chatrooms on the internet. CONCLUSION: Bedside support of fathers, accomplished by the NICU team, could be complemented by additional father-specific non-bedside support, such as peer-education measures or interactive mass media.

Reversal of morphine-induced urinary retention after methylnaltrexone.

Methylnaltrexone, a peripherally acting µ-opioid receptor antagonist, has been studied in adults for the treatment of opioid-induced constipation in advanced illness. Here, the authors document the first neonate to receive methylnaltrexone in an attempt to resolve morphine-induced urinary retention. An asphyxiated term newborn infant underwent induced hypothermia and received morphine by continuous intravenous infusion. After 36 h, the patient developed progressive urinary retention (calculated bladder volume 63 ml), followed by venous congestion of the lower extremities. Attempted bladder catheterisation was unsuccessful. Voiding occurred within 20 min after intravenous administration of methylnaltrexone (0.15 mg/kg body weight). A relapse of urinary retention 24 h later responded well to a second dose of methylnaltrexone. There were no adverse effects and no opioid withdrawal symptoms. The neonate had normal findings in cranial MRI that was performed after elective cessation of induced hypothermia.

The morphological effects of lead on the developing central nervous system.

The pathological changes found in the central nervous system of lead-exposed humans and laboratory animals are reviewed. Data in man relate to relatively high exposure levels. In human childhood lead encephalopathy, which occurs with blood lead levels in the range 100-800 micrograms Pb/100 ml, oedema, vacuolation, haemorrhage and reactive glial changes appear to be secondary to microvascular lesions. No primary neuronal lesions have yet been clearly identified. Neurological signs and a pathological picture closely resembling that seen in human lead encephalopathy are obtained in young lead-exposed rats with blood lead levels above 500 micrograms Pb/100 ml. Oedema and haemorrhage, cyst formation, reactive glial changes and nerve cell alterations are observed consequent to changes in capillary endothelial cells and basement membranes. High-level lead exposure in rats also produces disturbances in myelinated axons and may affect neural network formation in the central nervous system. With intermediate lead levels (200-500 microgram Pb/100 ml blood), vascular changes and their sequelae are not seen, but nutritional effects occur which may produce neuropathological changes. Data from recent studies on developing rats with low blood levels (up to 100 microgram Pb/100 ml) appear to show effects of lead on maturing and differentiated nerve cell populations. The relevance of these changes to human subclinical lead intoxication remains to be seen. However, the overall correspondence of findings in lead-poisoned man and rat would make further investigation in this area appear necessary.