Inherited neuromuscular disorders (NMD) inevitably result in severe lung volume restriction and c... more Inherited neuromuscular disorders (NMD) inevitably result in severe lung volume restriction and cough insufficiency associated with high morbidity and mortality. The aim of this retrospective study was to evaluate whether the airways of patients with NMD are colonized with potential pathogenic microorganisms that might contribute to the pulmonary morbidity in NMD. In this cross-sectional study, cough swabs were obtained and cultured for microbiological analyses from 77 patients with NMD and different degrees of lung volume restriction and cough insufficiency. Upper airway microbial colonization was compared to lung function parameters and cough peak flow values. Upper airway microbial colonization with potential pathogenic pathogens was identified in 39/77 (51%) of the subjects. Upper airway microbial colonization was associated with lower forced vital capacity (% pred.) 26.6 ± 19.7 versus 41.8 ± 20.4, P < 0.001 and cough peak flow (l/min) 125 ± 55 versus 207 ± 100, P < 0.001....
Annals of the American Thoracic Society, Nov 10, 2014
For patients with neuromuscular disorders, lung insufflation with positive pressure is an accepte... more For patients with neuromuscular disorders, lung insufflation with positive pressure is an accepted technique to increase inspiratory volume over VC to improve peak cough flow (PCF). The aim of the study was to determine the pressure or volume required to achieve the highest individual PCF. In 40 patients dependent on noninvasive ventilation (VC, 16 ± 11% predicted; age, 20 ± 4 yr) and in 20 healthy control subjects, insufflation capacity (IC) was measured during titration from 10 to maximum 40 mbar using intermittent positive pressure breathing (IPPB) or the lung insufflation assist maneuver (LIAM) of the VENTIlogic LS ventilator. IPPB or LIAM titration resulted in a pressure-volume curve with an estimated total compliance of 0.23 ± 0.11 L/kPa in the patients and 1.0 ± 0.3 L/kPa in the controls and a plateau for IC at pressures between 30 and 40 mbar. IPPB or LIAM improved VC from 451 ± 229 ml to a maximum IC (ICmax) of 1,027 ± 329 ml, and PCF improved from 109 ± 45 to 202 ± 62 L/min (P &amp;amp;amp;amp;lt; 0.01 for all). The highest individual PCF was achieved with 27 ± 6 mbar and an IC of 924 ± 379 ml, which was significantly below ICmax (P &amp;amp;amp;amp;lt; 0.01). A submaximal insufflation is ideal for generating the best individual PCF even in patients with severely reduced compliance of the respiratory system. Optimum insufflation capacity can be achieved using IPPB or LIAM with moderate pressures. Both techniques are equally effective and considered safe.
For patients with neuromuscular disorders, lung insufflation with positive pressure is an accepte... more For patients with neuromuscular disorders, lung insufflation with positive pressure is an accepted technique to increase inspiratory volume over VC to improve peak cough flow (PCF). The aim of the study was to determine the pressure or volume required to achieve the highest individual PCF. In 40 patients dependent on noninvasive ventilation (VC, 16 ± 11% predicted; age, 20 ± 4 yr) and in 20 healthy control subjects, insufflation capacity (IC) was measured during titration from 10 to maximum 40 mbar using intermittent positive pressure breathing (IPPB) or the lung insufflation assist maneuver (LIAM) of the VENTIlogic LS ventilator. IPPB or LIAM titration resulted in a pressure-volume curve with an estimated total compliance of 0.23 ± 0.11 L/kPa in the patients and 1.0 ± 0.3 L/kPa in the controls and a plateau for IC at pressures between 30 and 40 mbar. IPPB or LIAM improved VC from 451 ± 229 ml to a maximum IC (ICmax) of 1,027 ± 329 ml, and PCF improved from 109 ± 45 to 202 ± 62 L/mi...
Total body irradiation (TBI) treatment eradicates malignant cells and suppresses the immune syste... more Total body irradiation (TBI) treatment eradicates malignant cells and suppresses the immune system before hematopoietic stem cell transplantation (HSCT). The radiation dose is limited by its toxicity to healthy organs. Many reports describe long-term sequelae from TBI in adults, but comparable data for pediatric patients are scarce. We evaluated late effects of a cohort of survivors after at least 2 years from 106 children treated with TBI and HSCT between 1985 and 2008. Follow-up was available from 39 patients with a mean duration of 8.3 (range 2.0-21.9) years. We examined cardiac, pulmonary and renal function, longitudinal growth, weight development, endocrinological parameters, and gastrointestinal problems. Initial remission status and overall survival were significantly correlated. None of the 39 patients experienced cardiac dysfunction or changes in pulmonal function, but 5 exhibited renal impairment. Gastrointestinal problems were reported by 4 patients, and 10 patients had severe growth impairment. Altogether, our follow-up of pediatric patients who survived TBI-containing conditioning regimens for more than 2 years showed no cardiac morbidity or pulmonary aggravation, but mild renal sequelae and growth impairment. The adverse long-term effects of TBI in our cohort of children surviving at least 2 years after TBI and HSCT seem to be within a tolerable range. Future studies are required to investigate whether conditioning regimens lacking TBI result in a better ratio of benefits to overall side effects.
The diagnosis "innocent murmur" is made when there is no evidence for a heart disease e... more The diagnosis "innocent murmur" is made when there is no evidence for a heart disease either by history nor by examination, auscultation, electrocardiogram or echocardiography. The purpose of the present study was to determine the usefulness of echocardiography for diagnosing of an innocent heart murmur and further to compare the findings of ultrasound with those of the clinical examination. Children were accepted for the study after clinical examination and evaluation of the electrocardiogram (but before echocardiography) only when two independent pediatricians did not realize any symptoms of a heart disease. A total number of 200 children were examined; 121 were male and 79 female. 194 children (97%) had an innocent murmur, six children (3%) a heart disease [atrial septum defect (3), bicuspid aortic valve (1), aortic stenosis (1) or pulmonary stenosis]. The sensitivity and positive predictive value of clinical examination and auscultation ranged from 92% to 99%, the spec...
The field of mechanical ventilation is highly important in pulmonary medicine. The German Medical... more The field of mechanical ventilation is highly important in pulmonary medicine. The German Medical Association of Pneumology and Ventilatory Support [&quot;Deutsche Gesellschaft für Pneumologie und Beatmungsmedizin e. V. (DGP)&quot;] therefore has formulated these guidelines for home mechanical non-invasive and invasive ventilation. Non-invasive home mechanical ventilation can be administered using various facial masks; invasive home mechanical ventilation is performed via a tracheostomy. Home mechanical ventilation is widely and increasingly accepted as a treatment option for chronic ventilatory failure which most often occurs in COPD, restrictive lung diseases, obesity-hypoventilation syndrome and neuromuscular disorders. Essential for the initiation of home mechanical ventilation are the presence of symptoms of ventilatory failure and the detection of hypoventilation, most importantly hypercapnia. These guidelines comprise general indication criteria along with disease-specific criteria summarised by treatment algorithms. In addition, the management of bronchial secretions and care of paediatric patients are addressed. Home mechanical ventilation must be organised around a specialised respiratory care centre with expertise in patient selection, the initiation and the control of home mechanical ventilation. In this regard, the guidelines provide detailed information about technical requirements (equipment), control and settings of mechanical ventilation as well as organisation of patient care. A key requirement for home mechanical ventilation is the qualification of specialised home-care services, which is addressed in detail. Independent living and the quality of respiratory care are of highest priority in patients receiving home mechanical ventilation, since home mechanical ventilation can interfere with the integrity of a patient and often marks a life-sustaining therapy. Home mechanical ventilation has been shown to improve health-related quality of life of patients with chronic ventilatory failure. Long-term survival is improved in most patient groups, even though the long-term prognosis is often severely limited. For this reason, ethical issues regarding patient education, communication with ventilated patients at the end of life, living will, testament and medical care during the dying process are discussed.
Sleep disordered breathing (SDB) is an inevitable complication of progressive neuromuscular disor... more Sleep disordered breathing (SDB) is an inevitable complication of progressive neuromuscular disorders (NMD). Aim of the study was to prospectively investigate the impact of three months of non-invasive ventilation (NIV) on sleep and SDB-associated symptoms in children and adolescents with advanced NMD. Twenty-two patients (11.1 +/- 4.3 years) with symptomatic SDB were enrolled in the study and evaluated with polysomnography and a symptom questionnaire. Twenty patients were treated with pressure assisted ventilation during sleep. The impact of NIV on SDB, sleep and symptoms was re-evaluated after 3 months of NIV. In 2/22 patients, after adenotomy, SDB was no longer demonstrable. In the remaining 20 patients NIV improved SDB (Respiratory Disturbance Index 1.8 +/- 1.6 vs. 9.2 +/- 4.5/h; minimal SpO2 91 +/- 3 vs. 79 +/- 12%). Three months of treatment resulted in shortened sleep latency (17 +/- 13 vs. 42 +/- 38 minutes), improved sleep efficiency (95 +/- 3 vs. 89 +/- 9%) and less EEG-arousals (11 +/- 5 vs. 22 +/- 9/h, p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.001 for all). Sleep-stage proportion did not change. Patients reported significant improvements of sleep quality, morning headaches, mood, concentration and daytime sleepiness (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.001 for all). In children with NMD intermittent nocturnal NIV results in objective and subjective improvements of sleep quality and symptoms associated with SDB.
Inherited neuromuscular disorders (NMD) inevitably result in severe lung volume restriction and c... more Inherited neuromuscular disorders (NMD) inevitably result in severe lung volume restriction and cough insufficiency associated with high morbidity and mortality. The aim of this retrospective study was to evaluate whether the airways of patients with NMD are colonized with potential pathogenic microorganisms that might contribute to the pulmonary morbidity in NMD. In this cross-sectional study, cough swabs were obtained and cultured for microbiological analyses from 77 patients with NMD and different degrees of lung volume restriction and cough insufficiency. Upper airway microbial colonization was compared to lung function parameters and cough peak flow values. Upper airway microbial colonization with potential pathogenic pathogens was identified in 39/77 (51%) of the subjects. Upper airway microbial colonization was associated with lower forced vital capacity (% pred.) 26.6 ± 19.7 versus 41.8 ± 20.4, P < 0.001 and cough peak flow (l/min) 125 ± 55 versus 207 ± 100, P < 0.001....
Annals of the American Thoracic Society, Nov 10, 2014
For patients with neuromuscular disorders, lung insufflation with positive pressure is an accepte... more For patients with neuromuscular disorders, lung insufflation with positive pressure is an accepted technique to increase inspiratory volume over VC to improve peak cough flow (PCF). The aim of the study was to determine the pressure or volume required to achieve the highest individual PCF. In 40 patients dependent on noninvasive ventilation (VC, 16 ± 11% predicted; age, 20 ± 4 yr) and in 20 healthy control subjects, insufflation capacity (IC) was measured during titration from 10 to maximum 40 mbar using intermittent positive pressure breathing (IPPB) or the lung insufflation assist maneuver (LIAM) of the VENTIlogic LS ventilator. IPPB or LIAM titration resulted in a pressure-volume curve with an estimated total compliance of 0.23 ± 0.11 L/kPa in the patients and 1.0 ± 0.3 L/kPa in the controls and a plateau for IC at pressures between 30 and 40 mbar. IPPB or LIAM improved VC from 451 ± 229 ml to a maximum IC (ICmax) of 1,027 ± 329 ml, and PCF improved from 109 ± 45 to 202 ± 62 L/min (P &amp;amp;amp;amp;lt; 0.01 for all). The highest individual PCF was achieved with 27 ± 6 mbar and an IC of 924 ± 379 ml, which was significantly below ICmax (P &amp;amp;amp;amp;lt; 0.01). A submaximal insufflation is ideal for generating the best individual PCF even in patients with severely reduced compliance of the respiratory system. Optimum insufflation capacity can be achieved using IPPB or LIAM with moderate pressures. Both techniques are equally effective and considered safe.
For patients with neuromuscular disorders, lung insufflation with positive pressure is an accepte... more For patients with neuromuscular disorders, lung insufflation with positive pressure is an accepted technique to increase inspiratory volume over VC to improve peak cough flow (PCF). The aim of the study was to determine the pressure or volume required to achieve the highest individual PCF. In 40 patients dependent on noninvasive ventilation (VC, 16 ± 11% predicted; age, 20 ± 4 yr) and in 20 healthy control subjects, insufflation capacity (IC) was measured during titration from 10 to maximum 40 mbar using intermittent positive pressure breathing (IPPB) or the lung insufflation assist maneuver (LIAM) of the VENTIlogic LS ventilator. IPPB or LIAM titration resulted in a pressure-volume curve with an estimated total compliance of 0.23 ± 0.11 L/kPa in the patients and 1.0 ± 0.3 L/kPa in the controls and a plateau for IC at pressures between 30 and 40 mbar. IPPB or LIAM improved VC from 451 ± 229 ml to a maximum IC (ICmax) of 1,027 ± 329 ml, and PCF improved from 109 ± 45 to 202 ± 62 L/mi...
Total body irradiation (TBI) treatment eradicates malignant cells and suppresses the immune syste... more Total body irradiation (TBI) treatment eradicates malignant cells and suppresses the immune system before hematopoietic stem cell transplantation (HSCT). The radiation dose is limited by its toxicity to healthy organs. Many reports describe long-term sequelae from TBI in adults, but comparable data for pediatric patients are scarce. We evaluated late effects of a cohort of survivors after at least 2 years from 106 children treated with TBI and HSCT between 1985 and 2008. Follow-up was available from 39 patients with a mean duration of 8.3 (range 2.0-21.9) years. We examined cardiac, pulmonary and renal function, longitudinal growth, weight development, endocrinological parameters, and gastrointestinal problems. Initial remission status and overall survival were significantly correlated. None of the 39 patients experienced cardiac dysfunction or changes in pulmonal function, but 5 exhibited renal impairment. Gastrointestinal problems were reported by 4 patients, and 10 patients had severe growth impairment. Altogether, our follow-up of pediatric patients who survived TBI-containing conditioning regimens for more than 2 years showed no cardiac morbidity or pulmonary aggravation, but mild renal sequelae and growth impairment. The adverse long-term effects of TBI in our cohort of children surviving at least 2 years after TBI and HSCT seem to be within a tolerable range. Future studies are required to investigate whether conditioning regimens lacking TBI result in a better ratio of benefits to overall side effects.
The diagnosis "innocent murmur" is made when there is no evidence for a heart disease e... more The diagnosis "innocent murmur" is made when there is no evidence for a heart disease either by history nor by examination, auscultation, electrocardiogram or echocardiography. The purpose of the present study was to determine the usefulness of echocardiography for diagnosing of an innocent heart murmur and further to compare the findings of ultrasound with those of the clinical examination. Children were accepted for the study after clinical examination and evaluation of the electrocardiogram (but before echocardiography) only when two independent pediatricians did not realize any symptoms of a heart disease. A total number of 200 children were examined; 121 were male and 79 female. 194 children (97%) had an innocent murmur, six children (3%) a heart disease [atrial septum defect (3), bicuspid aortic valve (1), aortic stenosis (1) or pulmonary stenosis]. The sensitivity and positive predictive value of clinical examination and auscultation ranged from 92% to 99%, the spec...
The field of mechanical ventilation is highly important in pulmonary medicine. The German Medical... more The field of mechanical ventilation is highly important in pulmonary medicine. The German Medical Association of Pneumology and Ventilatory Support [&quot;Deutsche Gesellschaft für Pneumologie und Beatmungsmedizin e. V. (DGP)&quot;] therefore has formulated these guidelines for home mechanical non-invasive and invasive ventilation. Non-invasive home mechanical ventilation can be administered using various facial masks; invasive home mechanical ventilation is performed via a tracheostomy. Home mechanical ventilation is widely and increasingly accepted as a treatment option for chronic ventilatory failure which most often occurs in COPD, restrictive lung diseases, obesity-hypoventilation syndrome and neuromuscular disorders. Essential for the initiation of home mechanical ventilation are the presence of symptoms of ventilatory failure and the detection of hypoventilation, most importantly hypercapnia. These guidelines comprise general indication criteria along with disease-specific criteria summarised by treatment algorithms. In addition, the management of bronchial secretions and care of paediatric patients are addressed. Home mechanical ventilation must be organised around a specialised respiratory care centre with expertise in patient selection, the initiation and the control of home mechanical ventilation. In this regard, the guidelines provide detailed information about technical requirements (equipment), control and settings of mechanical ventilation as well as organisation of patient care. A key requirement for home mechanical ventilation is the qualification of specialised home-care services, which is addressed in detail. Independent living and the quality of respiratory care are of highest priority in patients receiving home mechanical ventilation, since home mechanical ventilation can interfere with the integrity of a patient and often marks a life-sustaining therapy. Home mechanical ventilation has been shown to improve health-related quality of life of patients with chronic ventilatory failure. Long-term survival is improved in most patient groups, even though the long-term prognosis is often severely limited. For this reason, ethical issues regarding patient education, communication with ventilated patients at the end of life, living will, testament and medical care during the dying process are discussed.
Sleep disordered breathing (SDB) is an inevitable complication of progressive neuromuscular disor... more Sleep disordered breathing (SDB) is an inevitable complication of progressive neuromuscular disorders (NMD). Aim of the study was to prospectively investigate the impact of three months of non-invasive ventilation (NIV) on sleep and SDB-associated symptoms in children and adolescents with advanced NMD. Twenty-two patients (11.1 +/- 4.3 years) with symptomatic SDB were enrolled in the study and evaluated with polysomnography and a symptom questionnaire. Twenty patients were treated with pressure assisted ventilation during sleep. The impact of NIV on SDB, sleep and symptoms was re-evaluated after 3 months of NIV. In 2/22 patients, after adenotomy, SDB was no longer demonstrable. In the remaining 20 patients NIV improved SDB (Respiratory Disturbance Index 1.8 +/- 1.6 vs. 9.2 +/- 4.5/h; minimal SpO2 91 +/- 3 vs. 79 +/- 12%). Three months of treatment resulted in shortened sleep latency (17 +/- 13 vs. 42 +/- 38 minutes), improved sleep efficiency (95 +/- 3 vs. 89 +/- 9%) and less EEG-arousals (11 +/- 5 vs. 22 +/- 9/h, p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.001 for all). Sleep-stage proportion did not change. Patients reported significant improvements of sleep quality, morning headaches, mood, concentration and daytime sleepiness (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.001 for all). In children with NMD intermittent nocturnal NIV results in objective and subjective improvements of sleep quality and symptoms associated with SDB.
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