Sleep Disordered Breathing in Pediatric Patients with Tetralogy of Fallot

Adverse effects on the pulmonary circulation in obstructive sleep disordered breathing (SDB) may place children with heart lesions affecting the right ventricle at increased risk for morbidity and mortality. We examined the distribution and effects of SDB in pediatric patients with tetralogy of Fallot (TOF). Families of 37 pediatric patients with TOF completed a survey of cardiac symptoms and school performance as well as a Pediatric Sleep Questionnaire (PSQ), a validated questionnaire for the screening of SDB in children 2–18 years of age. Medical records were reviewed for growth parameters, medical history, and most recent electrocardiogram (ECG) findings. Data from patients with SDB (PSQ score ≥8, n = 14) were compared to data from patients without SDB (PSQ score <8; n = 23). The prevalence of SDB in this population (38%) was significantly higher than the published prevalence of 5% in a healthy general pediatric population (p < 0.001). No significant difference was found in age, gender, or age and sex standardized body mass index between patients with or without SDB. No difference was seen in medication use or timing of surgical repair, whether primary or palliative. Patients with SDB had a significantly higher cardiac symptom score (p = 0.01) and increasing PSQ scores correlated with worsening cardiac symptom scores (p = 0.006). Increasing PSQ scores also correlated with worsening school performance (p = 0.001). No differences were seen in ECG data. The screened prevalence of SDB in the pediatric population with TOF is higher than in the general population; patients with TOF and SDB are more likely to have worse cardiac symptoms and poor school performance.     

Sleep apnea

Objective To study clinical presentation of sleep disordered breathing (SDB) in children, their causative factors and response to treatment. Methods A retrospective study of clinical data nd results of overnight polysomnography done at baseline and after therapy were reviewed in 56 patients under 18 years of age. Results Of the 56 patients included in the study 23(41%) cases were positive for SDB. 12 (52.1%) patients had craniofacial abnormalities, 4 (17.3%) had neuromuscular and skeletal disorders, 2 (8.6%) had adenotonsillar hypertrophy, 1 (4.3%) had bilateral vocal cord palsy and 3 (13%) had sleep apnoea associated with multisystemic disorders. Post-operative data showed improvement in all 6 cases of craniofacial abnormalities and both cases of adenotonsillar hypertrophy. Positive airway pressure treatment was useful in cases with obstructive sleep apnea (OSA) due to vocal cord palsy, theracic scoliosis, systemic disorders and central hypoventilation. Conclusion 41% of suspected cases were detected to have SDB. Craniofacial abnormality was the leading cause of OSA in the present study. Surgical correction improved symptoms apnea-hypopnea index (AHI) and desaturation in cases of craniofacial disorders and adenotonsillar hypertrophy. Vocal cord palsy thoracic scoliosis, hypoventilation and systemic disorders associated OSA responded to positive airway pressure ventilation.     

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??Objective To investigate the effect of mild obstructive sleep apnea syndrome??OSAS?? on the neurocognitive function of the children and evaluate the potential value of sleep pressure score??SPS?? in the diagnosis of the neurocognitive deficits in these children. Methods Consecutive OSAS children??aged 5??7 years?? were recruited from Shanghai Children’s Medical Center as the case group?? and non-snoring healthy children as the control group. Both of the 2 groups underwent the overnight polysomnography??PSG?? and a comprehensive neuropsychological battery including attention and memory test. The case group was divided into 2 subgroups according to the apnea hypopnea index??AHI????mild OSAS??AHI 1??5 times per hour?? and moderate to severe OSAS ??AHI≥5 times per hour??. The sleep architectures and sleep arousal parameters were analyzed and the neurocognitive tests were performed in the three groups. Analyze the SPS as a diagnosis tool of neurocognitive impairment in mild OSAS in children by ROC curve. Results There were 30 cases recruited into the control group. Mild OSAS group included 33 cases and moderate to severe group 28 cases. In the 3 groups?? there was no difference with regard to the age?? gender?? BMI z score or parents’ education level ??P??0.05??. There was significant difference in AHI?? obstructive apnea index ??OAI???? nadir SpO2 and respiratory arousal index ??RAI???? spontaneous arousal index ??SAI???? sleep pressure score ??SPS?? in the 3 groups. The AHI?? OAI?? nadir SpO2?? RAI and SPS of mild and moderate to severe OSAS group were significantly higher than those in control group. There was no significant difference in retention and the percentage of recall of the immediate memory ??P??0.05??. There was significant difference in cognition-attention index in the 3 groups even in the mild OSAS group?? and the moderate to severe OSAS group was the worst. In the ROC analysis?? the area under the curve was 0.88 ??95% confidence interval ??CI?? = 0.82 to 0.94?? for SPS. The cutoff point of 0.11 for SPS was optimal for diagnosing cognitive-attention deficit in mild OSAS group ??sensitivity 71.4%?? specificity 74%??. Conclusion Mild OSAS may be associated with the deficit of attention and SPS could be an effective index to evaluate it in mild OSAS children.     

Forehead Skin Blood Flow in Normal Neonates during Active and Quiet Sleep, Measured with a Diode Laser Doppler Instrument

ABSTRACT. Changes in forehead skin blood flow during active and quiet sleep were determined in 16 healthy neonates using a recently developed semi-conductor laser Doppler flow meter without light conducting fibres. Measurements were carried out at a postnatal age varying from 5 hours to 7 days. The two sleep states could be distinguished in 17 recordings. The mean skin blood flow values during active sleep were significantly higher ( p <0.01) than those during quiet sleep, the mean increase being 28.1%. The variability of the flow signal, expressed as the coefficient of variation, changed significantly from 23.1% during active sleep to 18.2% during quiet sleep.     

Sleep disordered breathing in children with achondroplasia

Background

Children with achondroplasia often have breathing problems, especially during sleep. The most important treatments are adenotonsillectomy (for treating upper obstruction) and/or neurosurgery (for resolving cervicomedullar junction stenosis).

Data sources

We reviewed the scientific literature on polysomnographic investigations which assessed the severity of respiratory disorders during sleep.

Results

Recent findings have highlighted the importance of clinical investigations in patients with achondroplasia, differentiating between those that look for neurological patterns and those that look for respiratory problems during sleep. In particular, magnetic resonance imaging (MRI) and somatosensory evoked potentials are the main tools to evaluate necessary neurosurgery and over myelopathy, respectively.

Conclusions

The use of polysomnography enables clinicians to identify children with upper airway obstruction and to quantify disease severity; it is not suitable for MRI and/or neurosurgery considerations.

     

A CASE OF NEAR-MISS SIDS DEVELOPING AN ABNORMAL RESPIRATORY REACTION TO HYPOXIA

ABSTRACT. A SIDS sibling is described who showed a normal respiratory regulation at 1.5 months but who was equipped with an apnea monitor at home on psychological indications. At 3 months he had a near-miss SIDS episode. He was then found to have developed a reaction to hypoxia with appearance of periodic breathing. With theophylline this response pattern was normalized but despite theophylline medication episodes of prolonged apnea occurred.     

Arterial Blood Pressure in the Very Low Birthweight Neonate

ABSTRACT. The noninvasive oscillometric technique of arterial blood pressure determination was evaluated in fifteen very low birth weight neonates (<1400 g). Measurement of invasive umbilical artery catheter pressure was used as a reference standard. By using a cuff width to arm circumference ratio of 0.33–0.42, mean blood pressure was overestimated by 6.2 (7.2) mmHg (mean and 1 SD), p <0.001. More accurate mean blood pressure measurements were recorded with a cuff width to arm circumference ratio of 0.44–0.55. Measurements of systolic and diastolic blood pressure showed similar results.     

Sleep disordered breathing (SDB) in neonates and implications for its long-term impact

Sleep-disordered breathing (SDB) is a significant cause of morbidity in neonates and young infants. SDB occurs more commonly in preterm infants and in neonates with underlying syndromes. Recent evidence shows that infants with obstructive sleep apnoea (OSA) or SDB have greater health care resource utilization, including longer hospital stay. Management of SDB includes non-invasive ventilation or surgical interventions tailored to the patient. Screening high risk newborns should allow for early diagnosis and timely therapeutic intervention for this population. However, the thresholds for diagnosing SDB and for guiding and implementing treatment in neonates remain unclear. A collective effort is required to standardize the practice worldwide. This article will discuss neonatal sleep physiology and characteristics of neonatal sleep, with an emphasis on the epidemiology and diagnosis of SDB in neonates and its implications for long term outcomes.     

Neonatal Hemochromatosis, Renal Tubular Dysgenesis, and Hypocalvaria in a Neonate

We report a neonate with neonatal hemochromatosis (NH), renal tubular dysgenesis (RTD), and hypocalvaria. NH is a fatal condition of the newborn, characterized by severe idiopathic liver failure of intrauterine onset and siderosis, intra- and extrahepatic, with sparing of the reticuloendothelial system. RTD is characterized by short, abnormally developed cortical tubules that lack proximal tubule differentiation. Although both NH and RTD have been reported as entities with a genetic component, similar findings can be secondary to in utero insults. Hypocalvaria has been reported in association with fetal hypoxia including that secondary to angiotensin converting enzyme inhibitors. This 38-week-old infant died at 8.5 h. The small nodular liver weighed 44 g. Grossly, the kidneys were normal. Hypocalvaria was present. Microscopically, the hepatic parenchyma was distorted by fibrous tracts, proliferation of bile ducts, and abundant iron deposition in hepatocytes. Extrahepatic siderosis in the pancreas, myocardium, and other organs was consistent with NH. Proximal convoluted tubules were not seen on routine stains and markers for proximal tubules were negative. Previous reports have linked NH with RTD and RTD with hypocalvaria. This infant had all three of these rare conditions, which have been hypothesized or shown to be due to genetic factors, hypoxia, or drugs. The etiology in this case is unknown. Received May 20, 1997; accepted August 15, 1997.     

Subcapsular Hemorrhage of the Liver in the Very Low Birthweight Neonate

ABSTRACT. Between 1974 and 1989, 15 neonates were found, at autopsy, to have subcapsular hematoma of the liver in a retrospective clinicopathologic study of 644 neonates. The majority (75 %) of the neonates were < 28 weeks gestation, male gender, and were delivered by the vaginal route following a complicated pregnancy and labor including malpresentation. The clinical course included resuscitation at birth, ventilatory support and hypovolemic shock with death occurring within 24 hours in 11 neonates. Other clinical events included air leak (n=4 infants) and infection (n=4). Intracranial hemorrhage was suspected in all but was found at autopsy in 8 neonates. The subcapsular hematoma was intact in 7 and ruptured in 8 neonates. Hence, subcapsular hemorrhage of the liver should be considered in the differential diagnosis of hypovolemic shock in very low birthweight infants.     

Sleep deprivation, disorganization and fragmentation during opiate withdrawal in newborns

OBJECTIVE: To determine specific sleep characteristics in neonatal opiate withdrawal, referred to as the Neonatal Abstinence Syndrome (NAS), by measuring sleep efficiency, deprivation, disorganization and fragmentation in three groups: (i) healthy term neonates; (ii) opiate-exposed neonates who were treated for opiate withdrawal; and (iii) a group of opiate-exposed neonates who did not require treatment. METHODS: A cohort study recording sleep patterns of neonates at 2-10 days of age (after 36 or more weeks of gestation) was carried out. Twenty-one neonates were exposed to opiates during pregnancy and 15 neonates were healthy controls. Sleep characteristics were predefined, and treated newborns were divided into early and stabilized treatment groups. Polygraphic recordings of sleep, movement and breathing were made continuously after a daytime feed. RESULTS: Sleep deprivation, disorganization and fragmentation were found in newborns with NAS and were associated with the severity of the withdrawal. Neonates treated for NAS displayed increased wakefulness during early treatment (deprivation), but were similar to controls once stabilized. Both treated and non-treated groups had reduced amounts of quiet sleep (deprivation). Treated newborns showed an increase in indeterminate sleep (disorganization) and arousals-to-wakefulness (fragmentation). CONCLUSION: This study determined the exact nature and degree of sleep disturbances in newborns during acute opiate withdrawal. The findings contribute to a further understanding of the physiology underlying neonatal opiate withdrawal and suggest that some changes in sleep are due to opiate withdrawal but others may reflect opiate dependency in utero.     

睡眠障碍与儿童睡眠癫癎

睡眠障碍在儿童中较常见，呈发作性，临床表现多样。国外报道约有20％～25％的儿童存在各种类型的睡眠障碍，个别报道达33％～40％，一般不需特殊治疗，预后良好。癫痫是小儿神经科常见疾病，临床表现复杂多样。有些癫痫发作又仅见于睡眠期，尤其是额叶癫痫具有夜发性倾向，部分病例有夜间躯体自动症发作。临床上夜间癫痫发作与睡眠障碍有许多相似之处，但其发病机制又有本质不同，故对任何睡眠发作性疾病，都应该首先区分为痫性发作与非痫性发作。本研究旨在探讨此类患儿24h脑电图（AEEG）及其与临床的关系，做好睡眠中癫痫性发作的诊断与鉴别诊断，以期对睡眠癫痫患者及早治疗，避免和减少神经系统损害。     

Snoring in children

Chronic snoring (≥4 nights per week) is not benign. Otherwise healthy children with chronic snoring and evidence of adenotonsillar hypertrophy can be referred directly for adenotonsillectomy. Snoring children <30 months or with significant medical comorbidities should be referred for specialist sleep evaluation. Older children with intermittent snoring or without significant medical comorbidities can be managed with a combination of medical and surgical interventions listed herein.     

SCSB Method Compared to EEG-Based Polygraphy in Sleep State Scoring of Newborn Infants

Abstract. Knowledge of the sleep state is important in physiological studies since many physiological variables show different properties in different sleep states. The recently developed static charge sensitive bed (SCSB) method allows long-term recordings of body movements, respiration and ballistocardiogram without electrodes attached to the subject. The recordings are easy to carry out and they do not disturb the subject in any way. The recorded variables are basic characteristics of different sleep states. SCSB-recordings and electroencephalography (EEG) based polygraphy as well as blind sleep state scoring were carried out in 8 newborn infants. The positive correlation between SCSB-scoring and EEG-based polygraphy scored by two clinical neurophysiologists was 68.1 and 64.1%. Only in 2 % of all epochs was active sleep scored as quiet sleep or vice versa. The results indicate the usefulness of the SCSB method in sleep state scoring of newborn infants.