Abnormalities of breathing control and airway maintenance in infants and children as a cause of cor pulmonale

Summary Respiratory control abnormalities may result in cor pulmonale. This report summarizes the clinical history, diagnostic evaluation, treatment, and outcome of 16 infants and children presenting with cor pulmonale subsequently found to be due to sleep-dependent hypoventilation. Eleven patients had cardiomegaly and electrocardiographic evidence of right ventricular hypertrophy (RVH) while 5 had only severe RVH or biventricular hypertrophy (BVH). Four infants with central hypoventilation syndrome (CHS)—absence of sleep-related ventilatory drive—had severe sleep-dependent asphyxia and resultant acute respiratory failure; all were ultimately treated with phrenic nerve pacing. One patient with alveolar hypoventilation syndrome (AHS)—a partial deficit in ventilatory drive during sleep—presented with severe pulmonary hypertension and ultimately died despite symptomatic relief with respiratory stimulants. Eleven patients presented with obstructive sleep apnea (OSA) and sleep-dependent asphyxia secondary to intermittent complete or to prolonged partial upper airway obstruction. Localized airway obstruction due to an anomalous innominate artery in 1 child was corrected by arteriopexy. Four children underwent adenotonsillectomy (T&A) with disappearance of symptoms in 1, clinical improvement in 2 and no clinical improvement in another. This unimproved patient and the 6 remaining OSA children improved dramatically after tracheostomy to bypass the sleep-dependent airway obstruction; none presently has evidence of cor pulmonale. In summary, early recognition and appropriate treatment of respiratory control disorders will improve sleep ventilation, eliminate asphyxia during sleep, and prevent the development of cor pulmonale.Supported in part by Children's Research Guild, Otho S. Sprague Foundation and NIH Grant RR-05475-14This paper was part of the Ray C. Anderson Symposium     

儿童急性肺源性心脏病的发病机制

急性肺源性心脏病通常继发于严重肺部疾病或肺循环障碍所导致的肺动脉高压,以急性右心功能障碍、右心衰竭为主要特征。其中,低氧血症、高碳酸血症及正压通气均可导致肺动脉高压的发生。在危重患儿救治过程中,特别是呼吸支持过程中,应早期识别急性肺源性心脏病,考虑实施以右心功能监护和保护为核心的"肺保护"及"循环保护"。     

Impact of underlying cause of bronchiectasis on clinical outcome: A comparative study on CF and Non-CF bronchiectasis in Egyptian children

Background

Bronchiectasis represents an important cause of chronic lung disease in children in developing countries and continues to be one of the leading causes of morbidity and mortality with worsening quality of life in these children.

Induced sputum nitrites correlate with FEV1 in children with cystic fibrosis

Aim: To determine the difference in the levels of nitrites in induced sputum of children with cystic fibrosis (CF) and controls. Furthermore, to evaluate the association between induced sputum nitrites and lung function in children with CF. Methods: Nitrites, cell differentials, white blood cell count, were estimated in induced sputum of 20 children with CF and 10 age‐matched healthy controls. Nitrites in induced sputum samples were measured using the Greiss assay. Lung function was ascertained by spirometry. Results: We observed high levels of nitrites in CF (184.8 ± 11.07 μM/L) versus controls (56.4 ± 5.7 μM/L) (p < 0.01). A positive correlation between neturophil percent and nitrites, white blood cell count and nitrites (p < 0.05) in children with CF was observed. Sputum nitrites correlated negatively with FEV₁ (p < 0.05) in children with CF. Conclusion: Induced sputum nitrite could serve as a useful non invasive marker for assessing the degree of inflammation in the airways of children with CF.     

Association of vitamin A status with lung function in children and adolescents with cystic fibrosis

Importance:Vitamin A(or retinol)has considerable antioxidative and anti-inflammatory attributes and it may have protective effects on the respiratory health of ...     

三房心的诊断和外科治疗

目的总结14例小儿三房心合并其他心脏畸形的外科治疗经验。方法全组均在中度低温体外循环下进行手术,平均年龄(5.6±3.5)岁,单纯型1例,复杂型13例(完全型11例,非完全型3例)。采用右房-房间隔径路显露左心房纤维隔膜,仔细辨认其解剖关系,充分剪除纤维隔膜疏通左心房通道,保护好二尖瓣膜。采用自体心包补片尽量扩大左心房容积,同时彻底矫正合并的其他心脏畸形。结果无一例手术死亡,14例全部存活,随访5个月~5年,心功能正常。结论三房心手术治疗取得早期和晚期良好疗效,手术关键在于仔细辨认,彻底矫正合并畸形。     

Azithromycin is the answer in paediatric respiratory medicine,but what was the question?

The first clinical indication of non-antibiotic benefits of macrolides was in the Far East, in adults with diffuse panbronchiolitis. This condition is characterised by chronic airway infection, often with Pseudomonas aeruginosa, airway inflammation, bronchiectasis and a high mortality. Low dose erythromycin, and subsequently other macrolides, led in many cases to complete remission of the condition, and abrogated the neutrophilic airway inflammation characteristic of the disease. This dramatic finding sparked a flurry of interest in the many hundreds of macrolides in nature, especially their anti-inflammatory and immunomodulatory effects. The biggest subsequent trials of azithromycin were in cystic fibrosis, which has obvious similarities to diffuse panbronchiolitis. There were unquestionable improvements in lung function and pulmonary exacerbations, but compared to diffuse panbronchiolitis, the results were disappointing. Case reports, case series and some randomised controlled trials followed in other conditions. Three trials of azithromycin in preschool wheeze gave contradictory results; a trial in pauci-inflammatory adult asthma, and a trial in non-cystic fibrosis bronchiectasis both showed a significant reduction in exacerbations, but none matched the dramatic results in diffuse panbronchiolitis. There is clearly a huge risk of antibacterial resistance if macrolides are used widely and uncritically in the community. In summary, Azithromycin is not the answer to anything in paediatric respiratory medicine; the paediatric respiratory community needs to refocus on the dramatic benefits of macrolides in diffuse panbronchiolitis, use modern – omics technologies to determine the endotypes of inflammatory diseases and discover in nature or synthesise designer macrolides to replicate the diffuse panbronchiolitis results. We must now find out how to do better!     

Imaging evaluation of hemoptysis in children

Hemoptysis is an uncommon but distressing symptom in children. It poses a diagnostic challenge as it is difficult to elicit a clear history and perform thorough physical examination in a child. The cause of hemoptysis in children can vary with the child’s age. It can range from infection, milk protein allergy and congenital heart disease in early childhood, to vasculitis, bronchial tumor and bronchiectasis in older children. Acute lower respiratory tract infections are the most common cause of pediatric hemoptysis. The objective of imaging is to identify the source of bleeding, underlying primary cause, and serve as a roadmap for invasive procedures. Hemoptysis originates primarily from the bronchial arteries. The imaging modalities available for the diagnostic evaluation of hemoptysis include chest radiography, multi-detector computed tomography (MDCT), magnetic resonance imaging (MRI) and catheter angiography. Chest radiography is the initial screening tool. It can help in lateralizing the bleeding with high degree of accuracy and can detect several parenchymal and pleural abnormalities. However, it may be normal in up to 30% cases. MDCT is a rapid, non-invasive multiplanar imaging modality. It aids in evaluation of hemoptysis by depiction of underlying disease, assessment of consequences of hemorrhage and provides panoramic view of the thoracic vasculature. The various structures which need to be assessed carefully include the pulmonary parenchyma, tracheobronchial tree, pulmonary arteries, bronchial arteries and non-bronchial systemic arteries. Since the use of MDCT entails radiation exposure, optimal low dose protocols should be used so as to keep radiation dose as low as reasonably achievable. MRI and catheter angiography have limited application.     

Applications of lung clearance index in monitoring children with cystic fibrosis

A sensitive, reproducible and feasible measure of lung function for monitoring the respiratory health is a prerequisite for the optimization of management of the patients with cystic fibrosis (CF). Spirometry has been considered the method of choice, although it is applicable only in children older than 6 years of age, as good cooperation is necessary for its proper performance. However, over the last 15 years, scientific interest in gas dilution techniques and particularly in multiple breath wash out (MBW) method has been revived. The most commonly reported index of MBW is lung clearance index (LCI). The aim of this review is to present the most recent developments in the application of LCI as a monitoring index of respiratory status of CF patients. LCI is a sensitive and reproducible marker of ventilation inhomogeneity. It is more sensitive than spirometry and, unlike spirometry; it can be performed across the whole pediatric age range. Since it is dependent on body size, until at least the age of 6 years, the relative and not the absolute changes are more appropriate for providing clinically meaningful conclusion on ventilation inhomogeneity. Until now, MBW has been mainly used as a research tool. Based on the currently available data LCI cannot safely predict high-resolution computed tomography findings in children with CF, especially in infants. It can be used as an end-point measure for the assessment of beneficial effect of interventions. However, its utility as an outcome measure for the efficacy of therapeutic interventions seems to be dependent on the pathophysiologic mechanisms that underlie each intervention. It seems that more studies, especially longitudinal ones, are required in order to fully clarify the clinical usefulness of LCI, not only in the research setting, but also in every day practice of CF clinic.     

Childhood sarcoidosis in Denmark 1979-1994: incidence, clinical features and laboratory results at presentation in 48 children

Aim: To describe the incidence, clinical presentation and paraclinical findings in childhood sarcoidosis in Denmark, 1979-1994. Methods: Patients (n = 5536) with a diagnosis of sarcoidosis were drawn from the nationwide Patient Registry; 81 patients were ≤15 y of age. The diagnosis of sarcoidosis was reconfirmed in 48/81 (59%) patients. In 35/48 (73%) patients, diagnosis was verified by histology, and in 13 it was substantiated by paraclinical/clinical findings. Results: The series comprised 26 boys and 22 girls (male/female ratio 1.18). Median age at diagnosis was 13 y (range 0.7-15). In 1979-1994 the incidence was 0.29 per 100 000 person-years ≤15 y of age. The incidence was 0.06 in children ≤4 y of age and increased gradually with age to 1.02 in children aged 14-15 y. General malaise, fever, weight loss, abdominal discomfort, respiratory symptoms, lymphadenopathy and central nervous system symptoms were common; 31% of patients had erythema nodosum, 12.5% sarcoid skin lesions, 25% uveitis/iridocyclitis and 4.2% sarcoid arthritis. Chest X-rays were normal (stage 0) in 10% of patients, and showed pulmonary infiltrates stage I in 71%, stage II in 8.3% and stage III in 8.3%. Lung function tests were examined in 13 patients: 50% had decreased FEV ₁ and vital capacity, 80% decreased D _L CO. Haemoglobin values were normal. Some patients had mild leukopenia, some moderate leukocytosis and a few had moderate eosinophilia. Erythrocyte sedimentation rate was elevated in 40% of the patients. Plasma calcium was elevated in 30% of the patients; 4 patients had severe hypercalcaemia and elevated plasma creatinine, and 1 patient had nephrocalcinosis. Serum angiotensin-converting enzyme was elevated in 55% of the patients. Liver function tests were normal with no sarcoid hepatitis. Urinary examination (glucose, albumin, haemoglobin) was normal in 96% of the patients; the patient with nephrocalcinosis had albuminuria and haematuria.

Conclusion: The incidence of sarcoidosis in Danish children is low and increases with age. Sarcoidosis in young children may present clinical features that are different from the appearance of those in older children and often constitute a diagnostic challenge. In older children, the clinical appearance has many features in common with the presentation in adults.     

Influence of the development of diabetes mellitus on clinical status in patients with cystic fibrosis

The impact of pre-diabetes on clinical status was retrospectively studied in 38 cystic fibrosis (CF) patients with diabetes mellitus (DM) and 38 non-diabetic CF patients (control patients), matched in pairs for age, sex, and chronicPseudomonas aeruginosa lung infection. Quarterly parameters of CF clinical status were collected for 6 years prior to the diagnosis of DM in the index case. Compared to the control patients, decreases in body weight, body mass index (BMI), forced expiratory volume in 1s (FEV₁), and forced vital capacity (FVC) and an increase in the daily intake of pancreatic enzyme capsules were found in the pre-diabetic patients. Statistically significant differences in body weight, BMI, FEV₁, FVC, and intake of pancreatic enzyme capsules between pre-diabetic and control patients emerged 4, 4, 1.25, 3 and 4.5 years prior to the diagnosis of DM, respectively. The number of lung infections did not differ between the two groups of patients. Thus, when DM develops in CF patients, an insidious decline in overall clinical status is observed for years prior to its diagnosis. Whether clinical deterioration in CF leads to DM, or pre-diabetes results in declining CF clinical status is presently unknown. Accumulating evidence suggests that the latter may be the case since insulin therapy seems to improve lung function in CF.     

儿童囊性纤维化伴铜绿假单胞菌感染2例

对2018年4月至2019年6月四川省人民医院儿科收治的2例感染铜绿假单胞菌囊性纤维化(CF)患儿资料进行分析。例1为11岁女童,既往无反复呼吸道感染史,首次痰培养发现铜绿假单胞菌,反复纤维支气管镜检查下可见大量黄白色分泌物附着,胸部CT示双肺支气管周围多发斑点影及树芽征。 CFTR基因检测结果示3处杂合突变...     

Healthcare inequalities in paediatric respiratory diseases

Identifying that health inequalities exist is not enough; nor does the knowledge that a patient has a high-risk genotype or comes from a higher risk socioeconomic background does not, by itself, help the patient. To thoroughly examine the origins of health disparities, a broad view of environmental and molecular influences must be included. As these factors are identified, it is important to focus on interventions that can change outcomes for patients. Tools for education, community involvement, literacy, and environmental safety need to be developed, tested and disseminated. The basic science of health disparities must move forward in a coordinated fashion by structuring research that is an integrated effort between basic sciences, clinical medicine and include all traditionally underserved communities. Only through these collaborations can we hope to eliminate health inequalities in the future.     

Evaluation of long term tobramycin therapy in patients with cystic fibrosis and advanced pulmonary disease

To nine cystic fibrosis patients with chronic bronchopulmonary infection of severely damaged lungs invaded by Pseudomonas aeruginosa, eleven courses of prolonged tobramycin treatment (5 mg/kg/day) for four to 16 weeks were administered. Pulmonary symptoms improved and a better quality of life was achieved in all but one patient. Objective parameters (chest X-ray, pulmonary function tests) changed to a lesser extent. In only one patient was Pseudomonas eradicated from the sputum but reappeared after discontinuation of therapy. In the rest of the patients Pseudomonas was significantly suppressed or replaced by other pathogens. Four patients showed rises of antibody titres to Candida and two to Aspergillus fumigatus. No nephrotoxic side effects were observed, but vestibular function was reversibly impaired in one patient without corresponding clinical symptoms. No bacterial resistance to tobramycin was observed during therapy.