Association between the severity of chronic spontaneous urticaria and sleep-disordered breathing.

BACKGROUND: Chronic spontaneous urticaria (CSU) is a common mast cell-driven disease, presenting with wheals, angioedema, or both. Sleep-disordered breathing (SDB) is also a common condition and contributes to various diseases by causing chronic inflammation. Recent studies have suggested an association between CSU and SDB. METHODS: To determine the association between the severity of SDB and that of CSU, we studied consecutive patients with CSU who visited the Sagamihara National Hospital allergy department or dermatology department between April 1 and October 31, 2018. The severity of CSU and SDB was evaluated based on the urticaria activity score 7 (UAS7) and peripheral arterial tone apnea-hypopnea index (pAHI) derived from out-of-center sleep testing (OCST) findings, respectively; their correlation was examined. RESULTS: Of the 37 patients studied, 19 had symptom-free-to-mild CSU (UAS7 ≤15) and 18 had moderate-to-severe CSU (UAS7 ≥16). The pAHI in the latter group was significantly higher than that in the former group (18 vs. 4.2, p = 0.001). In multivariate logistic analysis, moderate-to-severe SDB (pAHI ≥15) was significantly associated with moderate-to-severe CSU even after adjusting for the BMI (adjusted odds ratio 22 [95% confidence interval, 1.7-285]). CONCLUSIONS: The severity of SDB is correlated with that of CSU independently of the BMI. Physicians should consider comorbid SDB when treating patients with CSU.

Ictus isquémico en niño como complicación de adenoamigdalectomía / Ischemic stroke in childhood. A complication of tonsillectomy

Dentro de la edad pediátrica, la amigdalectomía es una de las cirugías otorrinolaringológicas más realizadas. Las complicaciones postoperatorias se clasifican en: primarias o inmediatas, de aparición generalmente en las primeras 24 h; y secundarias o tardías, a partir de las 48 h. Presentamos el caso de un infarto cerebral en un niño de 3 años, tras la realización de una amigdalectomía, que fue diagnosticado en el postoperatorio inmediato. En la eco-doppler y la angio-TC cerebral se visualizó un trombo intraluminal en la arteria carótida interna izquierda de etiología traumática directa, probablemente secundario a la ligadura arterial durante los procedimientos de hemostasia (AU)

Tonsillectomy is one of the most frecuently performed otorhinolaryngological procedures on children. The postoperative complications are classified into primary or intermediate, which generally appear within 24 h, and as secondary or delayed, after 48 h. We present the case of an ischemic stroke after performing a tonsillectomy on a 3 year-old boy, which was diagnosed in the immediate postoperative period. Using brain echo-doppler and angio-CT, an intraluminal clot was observed in the left internal carotid artery, probably as a result of direct vessel injury during arterial ligature for hemostasis (AU)

Impact of sleep and breathing in infancy on outcomes at three years of age for children with cleft lip and/or palate.

STUDY OBJECTIVES: To evaluate the relationship between sleep disordered breathing (SDB) in early infancy and outcomes at 3 years of age in children with cleft lip and/or palate (CL/P). DESIGN: Observational follow-up study. SETTING: Multidisciplinary CL/P clinic, tertiary centre. PARTICIPANTS: Children with CL/P who participated in a study of sleep and breathing in infancy. MEASUREMENTS AND RESULTS: The families of 52 children were approached for this follow-up study. The children underwent neurocognitive (Bayley Scales of Infant and Toddler Development, Third Edition; BSID-III), quality of life (Infant/Toddler Quality of Life Questionnaire; ITQOL), and growth assessments at 3 years. The families of 33 children (66%) completed follow-up at 36.7 ± 1.4 months. The apnea-hypopnea index (AHI) in infancy was 23.9 ± 18.0 events/h. Mean group BSID-III scores fell within the standardized normal range (10 ± 3) for all domains; however, language scores were lower than control children. Quality of life scores and growth parameter z-scores were similar to published control data. PSG variables in infancy showed significant relationships with outcomes at 3 years of age; lower percentage of AS/REM sleep was associated with lower cognition score; more obstructive events were associated with lower global behavior ITQOL score; and higher number of respiratory events in infancy was associated with lower weight z-score. CONCLUSION: Neurocognition, quality of life, and growth measures from children with CL/P fall within a normal range; however, scores in the language domain are lower than controls. Sleep and respiratory elements of SDB in infancy appear to modify these outcomes at 3 years of age.

Beneficial effects of adaptive servo ventilation in patients with chronic heart failure.

BACKGROUND: Sleep-disordered breathing (SDB) is thought to be a state of inflammation caused by hypoxic stress. Whether adaptive servo ventilation (ASV) attenuates the inflammatory response and improves the cardiac function of patients with congestive heart failure (CHF) accompanied by SDB was not been investigated. METHODS AND RESULTS: Seventeen inpatients with New York Heart Association (NYHA) II or III underwent polysomnography. There was a positive correlation between the apnea hypopnea index and high-sensitivity C-reactive protein (hs-CRP) level (r=0.753, P=0.016). The patients were divided into ASV (n=10) and non-ASV groups (n=7), and CHF-parameters were measured before and after ASV treatment. Improvement was noted for the NYHA class in the ASV group but not in the non-ASV group. In contrast to the non-ASV group, the level of brain natriuretic peptide (BNP), ejection fraction, and hs-CRP levels in the ASV group significantly improved (BNP, 212.1 ± 181.2 to 77.3 ± 54.0 pg/ml [P<0.05]; ejection fraction, 43.5 ± 6.4 to 53.3 ± 6.1% [P=0.002]; hs-CRP, 0.85 ± 0.58 to 0.21 ± 0.19 mg/dl, [P=0.008]). The increase in ejection fraction was correlated with a decrease in the hs-CRP level (r=-0.753, P=0.001). CONCLUSIONS: Anti-inflammatory effects of ASV are important contributors for improving cardiac function in patients with CHF accompanied by SDB.

A 2-year old with no ventilator requirement but who cannot be extubated.

A 2-year-old boy was intubated during treatment for pneumonia. After resolution of the infection, he had no pulmonary requirement for ventilation and could function without it while awake. When he slept, however, he would have decreasing respiratory effort, increasing hypercapnia, and episodic apnea. This report provides an example of late-onset congenital central hypoventilation syndrome.

Anesthetic care for the child with congenital central alveolar hypoventilation syndrome (Ondine's curse).

Idiopathic congenital central alveolar hypoventilation syndrome, otherwise known as Ondine's curse, is a rare neuropathologic syndrome characterized by an inadequate respiratory drive with hypoventilation and periods of prolonged apnea resulting in hypercarbia and hypoxemia. Although no definite pathologic abnormality has been identified to account for the disorder, it is thought to represent a primary defect related to altered function of central chemoreceptors resulting in defective control of minute ventilation. Associated problems related to neural crest cell migration, including neuroblastoma formation and Hirschsprung's disease, suggest that the primary defect is defective neural crest cell migration and function. Problems that may impact on perioperative care include the defective central control of ventilation and defective control of upper respiratory musculature, which may lead to upper airway obstruction. Although many patients will have previously undergone tracheostomy and chronic mechanical ventilation, problems in other organ systems can impact on perioperative care. Cardiovascular issues include the possible presence of cor pulmonale and autonomic nervous system dysfunction. Central nervous system issues include the frequent occurrence of seizures and mental retardation. The preoperative work-up, premedication, and the intraoperative/postoperative care and monitoring of these patients is reviewed.

[Sleep-phase-related home therapy in congenital central hypoventilation syndrome (CCHS)]. / Schlafphasenbezogene Atemtherapie bei Kindern mit angeborener Unempfindlichkeit für CO2.

PATIENTS AND METHOD: Eight children with congenital central hypoventilation syndrome (CCHS) (aged 3 to 16 years) underwent repeated polysomnographic recordings (sleep-EEG, induction plethysmography, PtcO2, PtcCO2, PACO2, FO2, SaO2, ECG) during spontaneous breathing and during therapy. The result led to individual therapeutic plans. RESULT: During NREM sleep a close relationship between increasing EEG-delta-activity and increasing PCO2 could be observed (PCO2 max. 107 mm Hg in NREM IV). A similar effect was seen during mechanical ventilation with decreasing spontaneous respiratory activity during increasing sleep depth (PCO2 max. 89 mm Hg in NREM IV). Associated with NREM I/II and REM sleep strong variations in spontaneous breathing with consecutive variations of blood gases were observed. Hyperventilation during REM sleep (PCO2 min. 20 mm Hg) could occur with continuous mechanical ventilation. A continuous blood gas monitoring improved home therapy since blood gas adapted control of mechanical ventilation was possible now. This caused a stabilization of blood gases in sleep. CONCLUSION: Patients with CCHS show a vigilance-dependent, enlarged variability of blood gases which should be considered in the management of home therapy. Continuous monitoring and blood gas adapted mechanical ventilation obtain a stabilization of acid-base balance during sleep. Preliminary data suggest a positive effect on sleep-wake quality and mental performance.

[From tracheostomy to non-invasive mask ventilation: a study in children with congenital central hypoventilation syndrome]. / Vom Tracheostoma zur nasalen Maskenbeatmung--Erfahrungen bei Kindern mit zentralem Hypoventilationssyndrom.

BACKGROUND: Children with congenital central hypoventilation syndrome (CCHS) have to be ventilated during sleep due to respiratory insensitivity to CO2. This long-term mechanical ventilation sometimes requires a tracheostomy during infancy, leading to increased risk of infections and of tracheal problems, and later on to stigmatization and restrictions in social life. PATIENTS AND METHOD: We therefore evaluated non-invasive mask ventilation in 4 children between 6 and 15 years of age, who had been ventilated via tracheal canula since early infancy under polysomnographic control. RESULTS: Best results were obtained with standard face masks in connection with pressure controlled timed ventilation. In 1 child we used a volume-controlled ventilator. The lack of dyspnea in these patients can worsen the acceptance of a face mask, which is more uncomfortable than a tracheal cannula. In 2 children we waited with the definite closure of the tracheostomy due to pavor-like symptoms and laryngeal closure during sleep and problems in acceptance of the mask, respectively. In the other 2 children we could demonstrate effective non-invasive mask ventilation during temporary tracheal closure for several nights. Therefore the tracheostomy was definitely closed. Long-term follow-up with home monitoring showed effectiveness of non-invasive ventilation in these cases.

Association of misoprostol, Moebius syndrome and congenital central alveolar hypoventilation. Case report.

We report a case showing the association of Moebius syndrome, the use of misoprostol during pregnancy and the development of central congenital alveolar hypoventilation. Pathophysiological aspects of these three diseases are discussed and also the unfavorable prognosis of this association.

Resolution of obstructive sleep apnea syndrome after adenoidectomy in congenital central hypoventilation syndrome.

We report on a 2 1/2-year-old boy who is currently ventilated at home by positive pressure ventilation through a nasal mask during the night because of congenital central hypoventilation syndrome (CCHS). Up to age 2 he had developed normally. A reevaluation was performed because of symptoms suggestive of obstructive sleep apnea syndrome (OSAS), including snoring, nocturnal sweating, frequent nighttime awakenings, speech impairment, daytime fatigue, and failure to thrive. A sleep study indicated obstructive apnea episodes lasting up to 40 s and arterial desaturations below 50% during spontaneous sleep. During mechanical ventilation snoring persisted, and capillary PCO2 rose to 60 mm Hg. Partial upper airway obstruction, leaking around the mask, and arousal movements developed on passive flexion of the neck to 20 degrees. After adenoidectomy, symptoms of OSAS resolved. There were no more obstructive apneas during spontaneous sleep, but obstructive apneas could be provoked by neck flexion to 20 degrees. During ventilation, neck flexion of 20 degrees was tolerated, but a 40 degrees flexion led to partial obstruction. In CCHS patients, the problem of upper airway obstruction is rarely noted because most patients are ventilated through a permanent tracheostomy. Today, noninvasive ventilation strategies are becoming more common. Reduced activity of upper airway muscles and impaired reflex mechanisms could lead to upper airway obstruction during face mask positive pressure ventilation in children with CCHS. Enlarged adenoids worsened this problem in our patient, leading to insufficient ventilation and OSAS. Adenoidectomy resolved symptoms of OSAS and enabled successful nasal mask ventilation. Close follow-up of the patient avoided hypoxia and sequelae from OSAS such as pulmonary hypertension.

Association of misoprostol, moebius syndrome and congenital central alveolar hypoventilation: case report

We report a case showing the association of Moebius syndrome, the use of misoprostol during pregnancy and the development of central congenital alveolar hypoventilation. Pathophysiological aspects of these three diseases are discussed and also the unfavorable prognosis of this association.

Respiratory sensations during heavy exercise in subjects without respiratory chemosensitivity.

Breathlessness arises from increased medullary respiratory center activity projecting to the forebrain (respiratory corollary discharge hypothesis). Subjects with congenital central hypoventilation syndrome (CCHS) lack the normal hyperpnea and breathlessness during hypercapnia. The corollary discharge hypothesis predicts that if CCHS subjects have normal hyperpnea during exercise, they will experience normal breathlessness during exercise. To test this, we studied four CCHS subjects and six matched controls during an exhausting constant-load cycling test requiring substantial anaerobiosis. CCHS subjects rated significantly less breathlessness at the end of the test than controls, but ventilation (index of respiratory corollary discharge) was also somewhat lower in CCHS (not significant). In both groups, breathlessness increased disproportionately more than ventilation towards the end of exercise. These data failed to disprove the corollary discharge hypothesis of breathlessness, but do suggest that the relationship between ventilation and breathlessness is non-linear and/or that projections of chemoreceptor afferents to the forebrain (presumed lacking in CCHS) is one source of breathlessness in normals.

Congenital central hypoventilation syndrome: an update.

Awareness of the existence of CCHS has led to increasingly frequent reports of such patients from all over the world. However, the exact pathophysiologic mechanisms underlying the clinical manifestations of this congenital disease entity remain unknown. For the respiratory physiologist, CCHS can be viewed as an experiment of nature that provides an important and unique window into central cardiorespiratory regulation. For the pediatrician, CCHS children represent an unique clinical challenge in coordinating the diagnostic and therapeutic procedures required to enhance the patients' quality of life.

[Home mechanical ventilation with nasal intermittent positive pressure ventilation for a boy with congenital central hypoventilation syndrome].

We report a 4-year-old boy with congenital central hypoventilation syndrome (CCHS) successfully treated with home mechanical ventilation with nasal intermittent positive pressure ventilation (NIPPV) during sleep hours. He had had frequent severe apneic attacks from the neonatal period. At 8 months, he was treated with positive pressure ventilation following a tracheostomy. At 4 year and 2 months, NIPPV was attempted because of recurrent respiratory tract infections and cor pulmonale. The tracheostomy was successfully abandoned 6 months later. Adequate ventilation has been maintained for more than 3 years without troubles. NIPPV is an effective and non-invasive treatment of CCHS that it significantly improves the quality of life during daytime.

[Obstructive sleep apnea in the child: an interdisciplinary treatment concept with special reference to craniofacial changes]. / Obstruktive schlafbezogene Atmungsstörungen beim Kind: Ein interdisziplinäres Behandlungskonzept unter besonderer Berücksichtigung kraniofazialer Veränderungen.

Diagnosis and therapy of obstructive sleep-related breathing disturbances SRBD in adults may not be applied without hesitation to children. SRBD in newborn and infants are often due to craniofacial disturbances (Pierre Robin syndrome, Goldenhars syndrome etc.), obesity is of minor importance. More than 30 infants with SRBD and craniofacial changes have been diagnosed and successfully treated over a 2-year period. Conservative therapy starts immediately after birth. The first step in newborn with Pierre Robin syndrome, for instance, is prone position for protrusion of tongue and mandible and mandibular growth stimulation. Intermediate nightly nasopharyngeal tubes are an alternative to nCPAP-/BiPAP treatment. Conventional orthopaedic/orthodontic treatment should not be neglected, even if it takes years to become effective. Surgical therapies are able to support, sometimes to replace or at least to shorten conservative methods. In rare cases when prone position in combination with palatal plates in cases of Robin syndrome, for instance, are not fully effective, mandibular extension is indicated. Aplasia or defects demand adequate surgical reconstruction, even if this does not necessarily mean abolishment of SRBD. In contrast to adults adenotonsillectomy is highly effective in infants and does not only reduce SRBD, but also improves nasal breathing and thus positively influences facial growth. A relatively new method is gradual mandibular distraction osteogenesis according to Ilizarov, which also enhances soft tissue growth. Maxillary and mandibular advancement osteotomies should not be considered before the termination of facial growth. Thanks to this refined treatment concept we were able to avoid tracheotomies in children during the past few years.

Pediatric hypoventilation syndromes.

Hypoventilation syndromes are an uncommon but important group of respiratory control disorders in infants and children. Congenital central hypoventilation syndrome (CCHS) is the principal and most important example. No specific anatomical or biochemical mechanism has yet been identified. This article summarizes current knowledge regarding CCHS in infants and children, and emphasizes the most recent and most important publications. The most recent advances in CCHS pertain to its genetics, pathophysiology, diagnosis, and treatment and provide state-of-the-art information regarding advances in diaphragm pacing, responses to exercise, and long-term outcome. CCHS is now being recognized more frequently, treatment is more successful, and long-term outcomes are encouraging with timely diagnosis, state-of-the-art treatment, and comprehensive follow-up at an experienced pediatric referral center.