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The purpose of this study is to describe the prevalence and severity of respiratory symptoms in children born very preterm and to assess their association with parents' health-related quality of life (HRQoL) and family functioning. We conducted a cross-sectional study and recruited children born less than 32 weeks' gestation between January 2006 and December 2019, in the greater Zurich area, Switzerland. Between May and December 2021, parents were invited to complete an online survey for their preterm child and for a control term born (≥ 37 weeks' gestation) sibling aged 1 to 18 years. We used a validated questionnaire to assess respiratory symptoms and the Pediatrics Quality of Life Family Impact Module (PedsQL FIM) to assess parents' HRQoL and family functioning. The survey was completed for 616 very preterm children (99 with bronchopulmonary dysplasia (BPD)) and 180 controls. Girls made up 45% (46% in controls) of the sample, and 63% (60% in controls) of participants were aged 6 to 18 years (school-age). Very preterm children reported a higher risk of respiratory symptoms than controls, especially preschoolers and those with moderate-to-severe BPD. Parents of children with "mild" and "moderate-severe" respiratory symptoms had on average −3.9 (95%CI: −6.6 to −1.1) and −8.2 (−11.2 to −5.2) lower PedsQL FIM total score, respectively, than parents of children with no symptoms. The same pattern was observed after stratifying by age categories. Conclusions: Our study suggests that respiratory morbidity in very preterm children has a negative impact on parents' HRQoL and family functioning, even beyond the first years of life. What is Known: • The burden of respiratory morbidity associated with very premature birth is high and last far beyond the neonatal period. • Respiratory morbidity contributes to lower health-related quality of life (HRQoL) in parents of very preterm children in early infancy. What is New: • Respiratory morbidity in very preterm children has a negative impact on parents' HRQoL and family functioning beyond the first years of life. • Parents of very preterm children with moderate and severe respiratory symptoms are the ones who report lower scores, both for preschool and school-age children. [ABSTRACT FROM AUTHOR]

To compare the incidence of respiratory symptoms and short-term consequences between children with Down syndrome and children from the general population, we conducted a prospective parent-reported observational study. Children with Down syndrome (≤ 18 years) were included between March 2012 and June 2014. Caregivers received a baseline questionnaire with follow-up 1–2 years after inclusion. Caregivers received a weekly questionnaire about respiratory symptoms, fever, antibiotic prescriptions, doctor's visits, and consequences for school and work attendance. Children with Down syndrome were compared to a cohort of the general population ("Kind en Ziek" study) with similar weekly questionnaires. A total of 9,011 childweeks were reported for 116 participants with Down syndrome (75% response rate). The frequency of respiratory symptoms was higher in children with Down syndrome than in children from the general population (30% vs 15.2%). In addition, symptoms subsided later (around 8 vs 5 years of age). The seasonal influence was limited, both in children with Down syndrome and children from the general population. Consequences of respiratory disease were significant in children with Down syndrome compared to children from the general population, with a higher rate of doctor's visits (21.3% vs 11.8%), antibiotic prescriptions (47.8% vs 26.3%), and absenteeism from school (55.5% vs 25.4%) and work (parents, 9.4% vs 8.1%). Conclusion: Children with Down syndrome have a higher frequency of respiratory symptoms and symptoms last until a later age, confirming the impression of professionals and caregivers. Individualized treatment plans might prevent unfavorable consequences of chronic recurrent respiratory disease in children with Down syndrome. What is Known: • Children with Down syndrome have an altered immune system and are prone to a more severe course of respiratory tract infections. • The overall conception is that patients with Down syndrome suffer from respiratory tract infections more often. What is New: • Children with Down syndrome suffer from respiratory symptoms more frequently than children from the general population. • The respiratory symptoms in children with Down syndrome subside at a later age compared to children from the general population. [ABSTRACT FROM AUTHOR]

Children aged <2 years with chronic lung disease (CLD) have a 10-fold higher risk for respiratory syncytial virus-positive hospitalization (RSVH) compared to healthy term infants. Based on the updated position statements, we compared respiratory-related illness hospitalization (RIH) and RSVH risks in CLD children who received palivizumab during the first year (FY) versus second year (SY) of life in the Canadian Registry of Palivizumab (CARESS). Demographic data were collected at enrolment and RIH events recorded monthly from 2005 to 2015. Eight hundred forty-seven FY and 450 SY children with CLD were identified. SY children had a lower gestational age (27 versus 29 weeks) and required more days of respiratory support (64 versus 43), oxygen therapy (108 versus 55), and length of stay (118 versus 73) during the neonatal course compared to FY children; all p < 0.0005. RIH rates were 12.2 (FY) and 18.2 (SY), and RSVH rates were 2.3 (FY) and 3.9 (SY). Cox regression showed similar hazards for both RIH (hazard ratio 0.9, 95% CI 0.6-1.6, p = 0.812) and RSVH (hazard ratio 1.1, 95% CI 0.4-2.9, p = 0.920).Conclusions: SY and FY children had similar risks for RIH and RSVH. The findings imply that SY children with CLD are correctly selected for palivizumab based on neonatal illness severity and merit prophylaxis. What is Known: • Children with chronic lung disease have a 10-fold higher risk for RSV-positive hospitalization in comparison to healthy term infants and commonly receive palivizumab prophylaxis as a preventative measure against serious RSV-related lower respiratory tract infections. • The American Academy of Pediatrics [ 2 ] and the Canadian Paediatric Society [ 30 ] have recently modified their recommendations for RSV prophylaxis in children with chronic lung disease, limiting palivizumab to either those <32 weeks gestation or those in the first year of life who are oxygen dependent or require medical therapy for the treatment of their condition. What is New: • Children with chronic lung disease receiving an additional course of palivizumab in their second year of life were determined to be at similar risk for both respiratory illness-related hospitalization and RSV-positive hospitalization as palivizumab-naïve children enrolled in the first year of life in the Canadian Registry for palivizumab (CARESS). • CARESS physicians are correctly identifying high-risk children with chronic lung disease in their second year of life, whom they believe will benefit from an additional year of palivizumab prophylaxis, based on neonatal illness severity. [ABSTRACT FROM AUTHOR]

Unlabelled: This study investigated the association between obesity and obstructive sleep apnea (OSA) in preschool and school-age children. Parents of obese and randomly chosen normal weight children completed a questionnaire on sleep-related symptoms, demography, family, and medical history. All subjects were invited to undergo polysomnography (PSG). OSA cases were defined as obstructive apnea hypopnea index (OAHI) ≥1. A total of 5930 children were studied with 9.5% obese (11.9% boys/6.1% girls), 205/2680 preschool and 360/3250 school children. There were 1030 children (535 obese/495 normal weight) who underwent PSG. OSA was higher in obese children and obese school children had higher OAHI, arousal index, and shorter total sleep time. However, there was no positive correlation between OSA and body mass index (BMI). The main risk factors for OSA in preschool children were adenotonsillar hypertrophy and recurrent respiratory tract infection. The main cause for OSA in school children was a history of parental snoring and obesity. Mallampati scores and sleep-related symptoms were found to be associated with OSA in both preschool and school children.Conclusion: We demonstrated differential risk factors for OSA in obese children, which suggest that a different mechanism may be involved in OSA development in preschool and school-age children.What Is Known: Various risk factors have been reported in obese children with OSA owing to the different age and different study design. Obese children have a higher prevalence and severity of obstructive sleep apnea (OSA). OSA risk factors in obese children are affected by different ages and study designs.What Is New: A differential prevalence and risk factors for obese preschool and school-age children with OSA has been demonstrated. [ABSTRACT FROM AUTHOR]

Unlabelled: To analyse the usefulness of bedside lung ultrasound (LUS) in detecting lung consolidation in a paediatric emergency room (ER) setting, febrile children seen at our ER from 2008 to 2012 with a moderate to severe respiratory distress underwent LUS, chest X-ray (CXR) and laboratory investigations. At first ER assessment, LUS identified a lung consolidation in 207 patients of 222 children enrolled, with a liver-like appearance in 75 (36.2%) and an associated pleural effusion in 36.7% of cases. CXR proved positive in 197 cases, showing a parenchymal consolidation (68.5%) or a focal ground-glass opacity (31.4%). LUS liver-like consolidation was significantly associated with longer duration of fever (p = 0.002), higher neutrophil counts and C-reactive protein (CRP) values (p = 0.015 and p < 0.0001, respectively), and with the discovery of a homogeneous and dense parenchymal consolidation on CXR (p < 0.0001).Conclusion: LUS can be adopted by the clinician as a non-invasive bedside tool to expand the physical evaluation of febrile children with respiratory distress. In our study, LUS results appeared not only as reliable as CXR in detecting lung consolidations but also consistent with clinical and laboratory data.What Is Known: The diagnosis of pneumonia is mainly based on physical examination plus radiologic and laboratory evaluation when needed. Although lung ultrasound (LUS) has shown high sensitivity in detecting several pleuropulmonary diseases in adults, its role in the work-up of pneumonia in children is not yet widely recognized.What Is New: LUS is confirmed to be a reliable imaging technique for the diagnostic work-up of febrile children with respiratory distress, consistent not only with CXR results as previously reported by others but also with clinical and laboratory data. In the hands of trained clinicians, it may represent a valuable supplemental bedside tool for a rapid evaluation in such circumstances. [ABSTRACT FROM AUTHOR]

Influenza surveillance is usually based on nationally organized sentinel networks of physicians and on hospital reports. This study aimed to test a different report system, based on parents' phone contact to the research team and in home collection of samples by a dedicated team. The identification of influenza and other respiratory viruses in children who attended a Hospital Emergency Department was also recorded. Real-time PCR and reverse transcription PCR were performed for influenza A and B, parainfluenza 1-4, adenovirus, human metapneumovirus, respiratory syncytial virus A and B, rhinovirus, enterovirus, group 1 coronaviruses, group 2 coronaviruses, and human bocavirus. One hundred children were included, 64 from the day care centers and 36 from the Hospital. Overall, 79 samples were positive for at least one respiratory virus. Influenza A (H3) was the virus most frequently detected: 25 cases, 20 of these in children under 5 years of age (ten from day care centers and ten who went to the hospital) which was higher than those reported by the National Influenza Surveillance Programme for this age. Conclusion: The results obtained in this study suggest that a surveillance system based on parents' reports could complement the implanted system of the National Influenza Surveillance Programme. [ABSTRACT FROM AUTHOR]

Congenital nasal obstruction can result in neonatal respiratory distress because neonates are obligate nasal breathers. Therefore, all physicians who deal with infants should be familiar with the structural abnormalities, masses, and syndromes that cause nasal obstruction, so that appropriate work-up and treatment can be promptly initiated. This paper reviews the embryology of the nasal passage and then continues with the different causes of nasal obstruction. Special attention is paid to the presentation, physical exam findings, and imaging modality of choice. [ABSTRACT FROM AUTHOR]

Unlabelled: A 4-year-old girl presented with sleep-disordered breathing. Her parents described breathing pauses of up to 20 s and progressive tiredness during the day. Obstructive apneas from an enlarged adenoid were thought to be the most probable cause. However, an adenotomy did not resolve the problem. Polysomnography demonstrated central apneas, and cerebral magnetic resonance imaging revealed a Chiari type I malformation. We describe the differential diagnosis of apnea in children and the role of polysomnography in the distinction between obstructive and central apneas.Conclusion: This case illustrates that, in children with apnea, it is important to consider central causes as well as the more common obstructive causes, even in the absence of additional neurological signs or symptoms. [ABSTRACT FROM AUTHOR]

Apnea of prematurity (AOP) is a common problem affecting premature infants, likely secondary to a "physiologic" immaturity of respiratory control that may be exacerbated by neonatal disease. These include altered ventilatory responses to hypoxia, hypercapnia, and altered sleep states, while the roles of gastroesophageal reflux and anemia remain controversial. Standard clinical management of the obstructive subtype of AOP includes prone positioning and continuous positive or nasal intermittent positive pressure ventilation to prevent pharyngeal collapse and alveolar atelectasis, while methylxanthine therapy is a mainstay of treatment of central apnea by stimulating the central nervous system and respiratory muscle function. Other therapies, including kangaroo care, red blood cell transfusions, and CO(2) inhalation, require further study. The physiology and pathophysiology behind AOP are discussed, including the laryngeal chemoreflex and sensitivity to inhibitory neurotransmitters, as are the mechanisms by which different therapies may work and the potential long-term neurodevelopmental consequences of AOP and its treatment. [ABSTRACT FROM AUTHOR]

There are several aerosolized drugs which have been used in the treatment of neonatal respiratory illnesses, such as bronchodilators, diuretics, and surfactants. Preclinical in vitro and in vivo studies identified a number of variables that affect aerosol efficiency, including particle size, aerosol flows, nebulizer choice, and placement. Nevertheless, an optimized aerosol drug delivery system for mechanically ventilated infants still does not exist. Increasing interest in this form of drug delivery requires more controlled and focused research of drug/device combinations appropriate for the neonatal population. In the present article, we review the research that has been conducted thus far and discuss the next steps in developing the optimal aerosol delivery system for use in mechanically ventilated neonates. [ABSTRACT FROM AUTHOR]

Conventional microbiology (CM) and real-time polymerase chain reaction (PCR) were used to determine rate and serotype of pneumococcal nasopharyngeal colonization in healthy children and children with upper respiratory illnesses (URI). One hundred and thirty-six healthy children and 79 children with URI were evaluated. Pneumococcal colonization was detected more often by real-time PCR than CM in healthy children (50% vs. 24%, p

Jeune syndrome (asphyxiating thoracic dystrophy, ATD) is a rare autosomal recessive skeletal dysplasia characterized by a small, narrow chest and variable limb shortness with a considerable neonatal mortality as a result of respiratory distress. Renal, hepatic, pancreatic and ocular complications may occur later in life. We describe 13 cases with ages ranging from 9 months to 22 years. Most patients experienced respiratory problems in the first years of their life, three died, one experienced renal complications, and one had hepatic problems. With age, the thoracic malformation tends to become less pronounced and the respiratory problems decrease. The prognosis of ATD seems better than described in literature and in our opinion this justifies long term intensive treatment in the first years. We also propose a follow-up protocol for patients with ATD. [ABSTRACT FROM AUTHOR]

Introduction: Acute respiratory tract infections (ARI) in children are often treated with antibiotics even without evidence of bacterial infection. Serum procalcitonin (PCT) is elevated in bacterial but not in viral infections.Patients and Methods: We performed a retrospective analysis of children in the PID-ARI.net study on respiratory infections to address the question of whether plasma PCT could potentially distinguish between bacterial infections requiring antibiotic therapy and viral ARI. We analysed data on 327 children who had been included in the German PID-ARI.net study and in whom nasopharyngeal aspirates had been analysed with a 19-valent multiplex reverse transcription-polymerase chain reaction-enzyme-linked immunosorbent assay for viral and atypical bacterial pathogens. Serum PCT was determined using a quantitative immunoassay (BRAHMS Kryptor PCTsensitive, Henningsdorf, Germany). We then focussed specifically on those children who were treated with antibiotics and therefore had been suspected of having bacterial infection but who had a serum PCT level lower than 0.1 ng/ml.Results: Out of 327 children, 132 had serum PCT levels below 0.1 ng/ml. Of these 132, 38 children had been treated with antibiotics. After exclusion of 26 patients (with critical illnesses, antibiotics on admission or for reasons other than ARI), 12 children remained for further evaluation. Of these 12 children, four had atypical pneumonia; four others had positive virus testing, and, in the last four, the aetiology of ARI remained unknown; evidence of bacterial infection could not be detected in any.Conclusions: Taken the results of this retrospective analysis, serum PCT values below 0.1 ng/ml might be a marker to identify children with acute respiratory tract infection in whom antibiotic treatment could be withheld. However, only a prospective intervention trial will prove the general safety of this limit. [ABSTRACT FROM AUTHOR]

Neuroglial heterotopias are rare congenital masses that are thought to represent encephaloceles that become sequestered on the extracranial side of the skull base. Although most often adjacent to bony skull base defects, they lack communication to the subarachnoid space. They contain mature neuroglial tissue and specialized central nervous system elements, such as a functioning choroid plexus. A case is presented of neonatal airway obstruction due to neuroglial heterotopia in the nasopharynx. The patient's clinical course and treatment are discussed, along with their radiology and histology. The relevant scientific literature is reviewed. [ABSTRACT FROM AUTHOR]

In this paper, a retrospective study was performed to find out whether the introduction of early nasal continuous positive airway pressure (nCPAP) as a new standard regime of very low birthweight infants will lead to a decreasing tracheal intubation and ventilation rate, as well as to a lower incidence of bronchopulmonary dysplasia in a tertiary-level perinatal centre. Ninety-three infants (study group) with early nCPAP as the first respiratory support were compared to 63 infants (historical control group) born before the use of early nCPAP. No statistically significant differences were found in the baseline characteristics. The main results of the study include reduced intubation mainly in infants with a birthweight <1,000 g (study group): 58% vs. 81% (p < 0.05). The mean duration of ventilation was 248 h (control group) vs. 128 h (study group) (p < 0.001) and 437 h vs. 198 h in infants <1,000 g (p < 0.001). There was significantly reduced incidence of bronchopulmonary dysplasia from 55% to 18% for all surviving infants (p < 0.001), and for infants <1,000 g, it was 90% vs. 30% (p < 0.001). No significant differences for other outcome criteria were noted, but a significant reduction in the use of central i.v. lines, fluids, drugs, volume expansion, sedation, catecholamines, surfactant, steroids and buffer, as well as antibiotics, was observed (p < 0.05). Therefore, we can conclude that early nCPAP is an easy-to-use and safe procedure for very low birthweight infants to treat respiratory distress. [ABSTRACT FROM AUTHOR]

Background: Recurrent acute respiratory tract infections (ARTI) are a common problem in childhood. Some evidence suggests a benefit regarding the prevention of ARTI in children treated with the immunomodulator OM-85 BV (Bronchovaxom).Methods: We summarised the evidence on the effectiveness of the immunomodulator OM-85 BV in the prevention of ARTI in children. We searched randomised comparisons of oral purified bacterial extracts against inactive controls in children with respiratory tract diseases in nine electronic databases and reference lists of included studies. We extracted salient features of each study, calculated relative risks (RR) or weighted mean differences (WMD) and performed meta-analyses using random-effects models.Results: Thirteen studies (2,721 patients) of low to moderate quality tested OM-85 BV. Patients and outcomes differed substantially, which impeded pooling results of more than two trials. Two studies (240 patients) reporting on the number of patients with less than three infections over 6 month of follow-up in children not in day care showed a trend for benefit RR 0.82 (95% CI, 0.65-1.02). One out of two studies examining the number of children not in day care without infections over 4-6 month reported a significant RR of 0.42 (95% CI, 0.21-0.82) whereas the smaller, second study did not [RR 0.92 (95% CI, 0.58-1.46)]. Two studies reporting the number of antibiotic courses indicated a benefit for the intervention arm [WMD 2.0 (95% CI, 1.7-2.3)]. Two out of the three studies showed a reduction of length of episodes of 4-6 days whereas a third study showed no difference between the two groups.Conclusion: Evidence in favour of OM-85 BV in the prevention of ARTI in children is weak. There is a trend for fewer and shorter infections and a reduction of antibiotic use. [ABSTRACT FROM AUTHOR]

Acute lower respiratory infection due to adenovirus is an important cause of chronic lung disease (CLD) in infants. The objectives of this paper is to describe growth in the height and weight of children with post-viral chronic lung disease (PVCLD) and to relate it with associated variables (invasive mechanical ventilation, chronic hypercapnia, oxygen therapy at home and steroid treatment). Ninety-nine patients with diagnosis of PVCLD with one or more years of follow-up were included in the study. The median duration of follow-up was 3.10 years. The median age at onset of the disease was 0.50 years. According to the growth in height, there were 48 children with normal growth (Type A) and 50 with slow growth (Type B) followed by catch-up growth ( N=45) or normal growth velocity ( N=5); only one patient showed persistent slow growth. Chronic hypercapnia was a risk factor significantly associated to slow growth, with odds ratio, OR: 5.03; and 95% confidence interval, CI: 1.83/13.83. Patients with higher weight for height at the end of the slow-growth period showed greater gains in height during the following period ( r=0.46). Growth in children with PVCLD is heterogeneous but, in the majority of individual cases, it can be considered satisfactory. Chronic hypercapnia was associated with slow growth. [ABSTRACT FROM AUTHOR]

Non-oliguric hyperkalaemia is a common and serious complication of extreme prematurity, resulting from a potassium loss from the intra- into the extracellular space during a specific post-natal period. Treatment of this disorder has been adapted from the treatment of hyperkalaemia in renal failure, an entity of completely different pathophysiology. A few years ago, the administration of salbutamol, which induces cellular potassium uptake, was proposed as a new therapeutic option. In this review article we discuss the pathogenesis and current therapy of non-oliguric hyperkalaemia of the premature infant, with special emphasis on the presently available knowledge and concerns with regard to the use of salbutamol. Being aware of the paucity of studies on non-oliguric hyperkalaemia, we propose treatment recommendations which are based on best available evidence. These comprise the administration of calcium, infusion of insulin plus glucose, correction of acidosis, and exchange transfusion or peritoneal dialysis as a last resort therapy. Before controlled trials on efficacy of salbutamol treatment of non-oliguric hyperkalaemia of the premature infant can be initiated, more data on safety are needed. [ABSTRACT FROM AUTHOR]

Unlabelled: The study aimed at evaluating possible associations between snoring and/or noisy breathing in sleep and daytime behaviour in 2-4-month-old infants using the Early Infancy Temperament Questionnaire (EITQ) as a tool. It covered the period from 1997 to 1998 and comprised 200 randomly selected clinically healthy infants aged 2-4 months from the community who were singletons and born in St. Petersburg within the chosen period. The mothers were asked to complete the questionnaires addressing infant, maternal, and major demographic characteristics, some infant care practices as well as the infant's habitual breathing symptoms during sleep. As a part of interview, the mothers answered the EITQ consisting of 76 items which describe different aspects of infant behaviour. Groups of questions were added according to a scoring sheet to produce total scores describing nine different aspects of infant temperament: activity, rhythm, approach, adaptability, intensity, mood, persistence, distractibility and threshold. In 129 cases (64.5%), mothers reported no respiratory disturbances during sleep in their infants. Mothers of ten infants (5.0%) described their babies as habitual snorers; 48 babies (24.0%) were characterised as having other than snoring noisy breathing during sleep, and 13 (6.5%) habitually had both snoring and noisy breathing. Symptomatic infants were rated as being moodier when awake compared with asymptomatic ones and most moodiness was the feature of those infants who had both snoring and noisy breathing during sleep. These associations remained after adjustment had been made for major potential confounders.Conclusion: Snoring and noisy breathing during sleep, rather common symptoms in young infants, may be associated with specific behavioural disturbances, and moody infants should be investigated carefully for possible obscure respiratory problems. [ABSTRACT FROM AUTHOR]

Unlabelled: To investigate the pathophysiology of the neonatal pulmonary emphysema, we assayed platelet activating factor (PAF) in the tracheal aspirates of the low birth weight infants. A total of 29 neonates (birth weight <1750 g) who required mechanical ventilation were enrolled. Tracheal aspirates were obtained within 48 h and blood samples collected within 24 h of life. PAF was assayed on the basis of its ability to cause aggregation of washed rabbit platelets. PAF was significantly elevated in four infants who showed pulmonary emphysema within the 1st week of life (median 24 pg/g lipid phosphorus, range 9.9-200) compared with those detected in the other three groups of infants; infants with respiratory distress syndrome (RDS) in whom chronic lung disease (CLD) did not develop (median 1.8 pg/g lipid phosphorus, range 0-30; P < 0.05), infants without RDS nor CLD (median 0.64 pg/g lipid phosphorus, range 0-14; P < 0.05) and infants with other types of CLD (median 1.1 pg/g lipid phosphorus, range 0-1.8; P < 0.01). The four infants who developed pulmonary emphysema within the 1st week of life, had significantly elevated serum IgM and neutrophilia at birth. The increased amount of PAF in the tracheal aspirates shows the presence of inflammation in the lung at birth. The elevated serum IgM level and neutrophilia indicate that the inflammation begins in utero.Conclusion: Our data suggest that neonatal pulmonary emphysema is caused by intra-uterine inflammation increasing platelet activating factor in the lungs. Platelet activating factor may play a role in aggravating the process of pulmonary emphysema. [ABSTRACT FROM AUTHOR]

Unlabelled: Isolated partial 3-methylcrotonyl-CoA carboxylase (MCC) deficiency has been described to be the cause for a distinct relatively mild clinical picture in a single patient. We describe another patient with isolated partial MCC deficiency who suffered from failure to thrive, muscular hypotonia and progressive respiratory insufficiency with fatal outcome at the age of 6.5 months. MCC deficiency was suspected at 3 months of age on the basis of mildly elevated urinary excretion of 3-hydroxyisovaleric acid and 3-methylcrotonylglycine and confirmed by enzyme analysis in lymphocyte and fibroblast homogenates. Residual MCC activity in lymphocytes was 25% of the mean normal value. Residual activity in fibroblasts was lower than in lymphocytes (3.8% of mean normal) and not significantly different from that in patients with complete MCC deficiency. However, the residual incorporation of 14C-isovalerate into macromolecules in intact fibroblasts, was clearly higher (28% of mean normal) than in fibroblasts with complete MCC deficiency (<4%). In both patients with partial deficiency the residual MCC activity was higher in lymphocytes than in fibroblasts. Clinical symptoms and signs in our patient attributable to MCC deficiency include muscular hypotonia, failure to thrive (already present at birth), progressive respiratory failure due to diaphragmatic paresis and a moderate brain atrophy. The clinical presentation was more severe than in many patients with complete MCC deficiency. Dietary therapy was biochemically effective as shown by normalization of organic acid excretion, however, had no effect on the CNS symptoms.Conclusion: We speculate that the severity of the disease could be related primarily to deficiency of MCC activity in the brain. Variable MCC activity among various organs may explain the peculiar clinical picture in this patient. [ABSTRACT FROM AUTHOR]

Unlabelled: Bronchodilators are often used in the treatment of patients with bronchopulmonary dysplasia (BPD). However, few studies evaluate their efficacy in patients with stable disease beyond the newborn period. Therefore, pulmonary function was measured before and after aerosol treatment with salbutamol (0.25 ml Ventolin 0.5%) and subsequently after aerosol with ipratropium bromide (0.25 ml Atrovent 0.025%). Studies were performed at the corrected postnatal age of 52 +/- 2 weeks in 52 patients who had been ventilated after birth because of newborn lung disease. Twenty-two of these 52 patients had developed BPD. Pulmonary function was measured after sedation and using the PEDS system. Expiratory resistance (median 52.1 versus 39.1 cmH2O/l/s; P < .008) and inspiratory resistance (median 42.5 vs 27.8 cmH2O/l/s; P < .04) were significantly worse in BPD patients at the age of 1 year. Half of the BPD patients had a decrease in pulmonary resistance after salbutamol. However, there was no statistically significant decrease in pulmonary resistance after salbutamol or ipratropium in the BPD patients as a group. After salbutamol pulmonary resistance significantly worsened in the patients who did not develop BPD.Conclusion: Although individual patients may benefit, routine administration of bronchodilators seems not warranted in stable BPD patients at the age of 1 year. [ABSTRACT FROM AUTHOR]

Chilaiditi syndrome is the interposition of the colon between the diaphragma and the liver. In general, patients are asymptomatic, but some patients have been associated with gastrointestinal and respiratory symptoms. A 5-month-old infant boy was admitted to the hospital with a history of cough, cyanosis and recurrent respiratory distress that had persisted during the preceding 2 months. The chest X-ray revealed an elevation of the right hemidiaphragma caused by the presence of a dilated colonic loop below. Computed tomography showed a hepatodiaphragmatic interposition of the colon, leading to the diagnosis of Chilaiditi syndrome. The patient was conservatively treated with oxygen, fluid supplementation and stool softeners. We conclude that this rare syndrome should be kept in mind when young patients present with recurrent respiratory distress. [ABSTRACT FROM AUTHOR]

Unlabelled: In order to survey current management of oxygen in children with chronic lung disease (CLD) of prematurity in the United Kingdom and Ireland, 260 questionnaires asking about criteria that were considered important when removing supplemental oxygen from a child with CLD were sent to consultant paediatricians identified as being likely to be involved in the follow-up of children with CLD. A total of 120 questionnaires were returned from 114 centres. The factors that were considered important when removal of the patient's supplemental oxygen was being considered were: the oxygen saturation level in 100% responders; the respiratory rate in 62%; the heart rate in 37%; the length of time that the child had been in oxygen in 25%; changes on the chest radiograph in 22%; capillary blood gases in 18%, and arterial blood gases in 7%. The specific oxygen saturation level above which it was felt that the patient could stop oxygen therapy ranged from 85% to 98% (mean 93%). The situations which would prevent discontinuation of oxygen were: desaturation while feeding/ sleeping or exercising in 95% responders; failure to gain weight in 78%; presence of pulmonary hypertension in 62%, the parents' opinion or perception of their child's needs for supplemental oxygen in 58%; recent withdrawal of steroid therapy in 52%; a recent infection of the respiratory tract in 42%; history of cyanotic spells in 28%; and the advent of winter in 23%. Conclusion: there is an enormous variety of practice with regard to the management of supplemental oxygen for children with chronic lung disease suggesting an urgent need for research and evidence-based guidelines. [ABSTRACT FROM AUTHOR]

Unlabelled: We report a 4-week-old boy with acute respiratory distress, due to massive haemorrhages in multiple thymic cysts. A right hemithymectomy was performed because of mechanical obstruction of the trachea by the cysts. The origin of the multilocular thymic cysts remained unclear. Most likely, these haemorrhages were caused by vitamin K deficiency, although the infant received vitamin K prophylaxis. In addition, he developed transient cholestasis, but the aetiology remained unclear. It is postulated that massive haemorrhages in thymic cysts produce large amounts of bilirubin, causing sludging of bile excretions in the liver. Four weeks after the operation, all laboratory findings were normal and 6 months after the operation the boy is still healthy.Conclusion: This case report shows that respiratory distress in an infant can be caused by multiple haemorrhages in multilocular thymic cysts. [ABSTRACT FROM AUTHOR]

Presents a hypothesis on the relationship between chorioamnionitis (CA) and pulmonary hemorrhage (PH) in extremely low birth weight infants (ELBW). Percentage of PH occurrence among low birth weight infants with respiratory disease syndrome; Histological CA proportions in ELBW newborns with PH; Role of the perinatal inflammatory process in PH in ELBW infants.

Provides information on congenital chylothorax and respiratory distress in newborns with trisomy 21. Discussion on respiratory diseases; Observations on chromosome aberration to detect congenital problems; Treatment of thrombocytopenia.

Unlabelled: Thymic hyperplasia, although not a rare condition in infancy, is usually asymptomatic. We describe an infant presenting in the perinatal period with marked tachypnoea. An enlarged thymus, demonstrated on chest radiograph and CT, was associated with small-volume, non-compliant lungs. Other causes of pulmonary malfunction and maldevelopment were excluded.Conclusion: Thymic enlargement is unusually associated with neonatal respiratory distress but should be considered in the differential diagnosis. [ABSTRACT FROM AUTHOR]

Unlabelled: The aim of this pilot study was to determine the effect of early inhaled budesonide on clinical and inflammatory parameters in preterm infants ventilated for respiratory distress syndrome.Conclusion: Neither the inflammatory process associated with respiratory distress syndrome nor the progression to bronchopulmonary dysplasia appeared to be altered by treatment with inhaled budesonide. [ABSTRACT FROM AUTHOR]

Presents a case of a male newborn with respiratory distress and multiple congenital anomalies involving the thorax and left upper extremity noted since birth. Diagnosis of congenital diaphragmatic hernia (CDH); Subclavian artery supply disruption sequence theory for the pathogenesis of Poland anomaly; Description of CDH.

Diphtheria has become a rare disease in Germany. We report on an unimmunized 3.5-year-old German girl with a 7-day history of respiratory distress and fever, presenting a clinical picture mimicking typical bacterial tracheitis without pharyngeal and laryngeal manifestation. Diagnosis of diphtheria was not made until culture of tracheal secretions yielded growth of a toxigenic strain of Corynebacterium diphtheriae. The patient died from toxic cardiac failure despite treatment with diphtheria antitoxin. This is the second reported case of isolated bacterial tracheitis caused by Corynebacterium diphtheriae.Conclusion The observation of a lethal course of diphtheric tracheitis emphasizes the paramount im-portance of immunization against diseases like diph-theria. [ABSTRACT FROM AUTHOR]

The article presents an obituary for pediatric pneumologist and infectious disease specialist Klaus Magdorf.