Guidelines for diagnosis and management of congenital central hypoventilation syndrome.

Background: Congenital Central Hypoventilation Syndrome (CCHS) is a rare condition characterized by an alveolar hypoventilation due to a deficient autonomic central control of ventilation and a global autonomic dysfunction. Paired-like homeobox 2B (PHOX2B) mutations are found in most of the patients with CCHS. In recent years, the condition has evolved from a life-threatening neonatal onset disorder to include broader and milder clinical presentations, affecting children, adults and families.

Updated Swedish advice on reducing the risk of sudden infant death syndrome.

Unlabelled: This article reviews updated advice and factual material from the Swedish National Board of Health and Welfare on reducing the risk of sudden infant death syndrome. Issues covered by the guidance for parents and healthcare professionals include sleeping positions, smoking, breastfeeding, bed sharing and using pacifiers.

Conclusion: The guidelines conclude that infants under three months of age are safest sleeping in their own cot and that a pacifier can be used when they are going to sleep.

Decreased heart rate variability in children born with low birth weight.

Background: Low birth weight (LBW) is associated with cardiovascular morbidity in adulthood. Imbalance in the autonomic nervous system (ANS) has been implicated as a mechanism behind the developmental programming of cardiovascular function. We hypothesized that deviations in the ANS function are seen in children born with LBW.

Obstructive pulmonary disease in old age among individuals born preterm.

There are only few studies of the association between preterm birth and risk of chronic lung disease in old age. The aim of this study was to assess the association between poor fetal growth, preterm birth, sex and risk of asthma and Chronic Obstructive Pulmonary Disease (COPD) in adulthood. We have followed up a cohort of all infants born preterm (<35 weeks) or with low birth weight (<2,000 and <2,100 g for girls and boys, respectively) and an equal number of controls in a source population of 250,000 individuals born from 1925 through 1949 in Sweden (6,425 subjects in total).

A key circulatory defence against asphyxia in infancy--the heart of the matter!

A resumption of, and escalation in, breathing efforts (hyperpnoea) reflexively accelerates heart rate (HR) and may facilitate cardiac and circulatory recovery from apnoea. We analysed whether this mechanism can produce a sustained rise in HR (tachycardia) when a sleeping infant is confronted by mild, rapidly worsening asphyxia, simulating apnoea. Twenty-seven healthy term-born infants aged 1-8 days rebreathed the expired gas for 90 s during quiet sleep to stimulate breathing and heart rate.

Positional circulatory control in the sleeping infant and toddler: role of the inner ear and arterial pulse pressure.

Heart rate (HR) and arterial blood pressure (BP) are rapidly and reflexively adjusted as body position and the force/direction of gravity alters. Anomalies in these mechanisms may predispose to circulatory failure during sleep. We analysed the development of two key reflexes involved by undertaking a longitudinal (birth–1 year) comparison of instantaneous HR and BP changes evoked by abrupt upright, sideways or horizontal repositioning.

Long-term reprogramming of cardiovascular function in infants of active smokers.

Newborn infants of smokers show symptoms of cardiovascular stress hyperreactivity. Persistent hyperreactivity could increase the risk of short- and/or long-term complications, such as hypertension. Here we determined whether incipient dysfunction in a smoker's infant persists or worsens with age, by comparing cardiovascular reflex function of control and tobacco-exposed infants longitudinally from birth to 1 year.

The effect of long term ventilatory support on hemodynamics in children with spinal muscle atrophy (SMA) type II.

Background: Bi-level Positive Airway Pressure (Bi-PAP) treatment improves breathing efficiency and ventilation in children with SMA type II, but the effects of positive airway pressure swings on heart rate (HR) and blood pressure are not known. Here we studied children with SMA to determine whether Bi-PAP administered during sleep is associated with changes in hemodynamics.

Methods: Ten children aged 8-12 years on long term Bi-PAP therapy were evaluated during a routine overnight sleep study.

Obstructive lung disease in children with mild to severe BPD.

Background: Bronchopulmonary dysplasia (BPD) is a common cause of respiratory insufficiency in children born very premature.

Objectives: The purpose of this study was to examine the impact of the severity of BPD on pulmonary morbidity at school age, as measured by conventional spirometry and impulse oscillometry. We also studied the association between changes in lung function and structural changes in the lungs of children with BPD via High-Resolution Computed Tomography (HRCT).

Cardiovascular stress hyperreactivity in babies of smokers and in babies born preterm.

Background: Being born preterm, small, and to a mother who smokes are common perinatal complications with major public health implications. Evidence suggests that each affects the body's structure and function in ways that could increase susceptibility to cardiovascular dysfunction later in life. Here, we used 2 routine stress reactivity tests to identify incipient "silent" programming of cardiovascular dysfunction associated with adverse perinatal events.

Long-term non-invasive positive airway pressure ventilation in infants.

Aim: To evaluate the clinical application of long-term non-invasive ventilation (NIV) in infants with life-threatening ventilatory failure with regard to: diagnosis, age at initiation, indication for and duration of treatment, clinical outcome and mortality and adverse effects.

Patients And Methods: The medical records of 18 infants treated in a home setting during a 7-year period were reviewed. The criteria for ventilatory support were: (a) transcutaneous partial pressures of carbon dioxide (TcPCO(2)) >6.

Disturbances in cardiorespiratory function during day and night in Rett syndrome.

Rett syndrome causes severe autonomic dysregulation, probably due to brainstem dysfunction. Because the brainstem plays a decisive role in cardiorespiratory regulation during sleep, we investigated cardiorespiratory function in 12 girls with Rett syndrome, day and night, for 1 week in their home environment. Heart rate and breathing were recorded via standard three-lead electrocardiogram.

Abnormal circulatory stress responses of preterm graduates.

Preterm birth and chronic lung disease may increase the risk of hypertension and cardiovascular disease in infancy and adolescence. Here we looked for evidence of early circulatory dysfunction associated with these perinatal complications. We compared infants born at term (n = 12) with those born preterm with an uncomplicated neonatal course (n = 12) or diagnosed with bronchopulmonary dysplasia (BPD) (n = 10).

Resuscitation and ventilation strategies for extremely preterm infants: a comparison study between two neonatal centers in Boston and Stockholm.

Aim: To evaluate if different resuscitation and ventilatory styles exist between two neonatal units, and if the less aggressive approach has a beneficiary effect on BPD outcome.

Method: Inborn infants delivered at a gestational age <28 weeks were retrospectively studied (Boston = 70 and Stockholm = 102). Data were collected from birth to discharge or to 40 weeks.

Development of chemoreceptor responses in infants.

This paper is devoted to the field of chemoreception and its role in the control of breathing in infants. We use "chemoreception" to refer to the capacity to sense and process changes in P(O2) and P(CO2), and also to react to these changes by adjusting ventilation in order to maintain homeostasis. Functional chemoreceptors are not essential to commence or even to sustain breathing efforts immediately at or after birth; the intense brain activation, which occurs at birth, is sufficient.

Developmental risks and protective factors for influencing cognitive outcome at 5 1/2 years of age in very-low-birthweight children.

In a population-based follow-up study (the Stockholm Neonatal Project), 182 children with a birthweight of 1500 g or less (very-low birthweight: VLBW) and a control group of 125 children born healthy at term were examined with the Wechsler Preschool and Primary Scale of Intelligence-Revised (WPPSI-R) and a neuropsychological test battery (Nepsy) at 5 1/2 years of age. The WPPSI-R results of the VLBW children fell well within the normal range: WPPSI-R full-scale IQ 95.7, verbal subscale IQ 99.