Subject(s)
Drosophila Proteins , Germ-Line Mutation , Proto-Oncogene Proteins/genetics , Receptor Protein-Tyrosine Kinases/genetics , Sleep Apnea, Central/genetics , DNA/chemistry , DNA/genetics , DNA Mutational Analysis , Gene Frequency , Humans , Polymorphism, Genetic , Proto-Oncogene Mas , Proto-Oncogene Proteins c-ret , Sleep Apnea, Central/pathologyABSTRACT
Patients suffering from neuromuscular diseases and thoracic deformities may develop global respiratory failure during their illness. We wanted to judge clinical parameters and information from the patients' medical history to reliably, quickly and noninvasively diagnose a ventilatory failure. Therefore we evaluated 105 situations with and without mechanical ventilation from 29 patients with indication for noninvasive nocturnal mask ventilation. 6 clinical parameters (e.g. heart rate, oxygen saturation, relative vital capacity), 2 test results (pH and partial pressure of carbon dioxide (pCO2)) and 6 parameters from the patients' medical history (e.g. nycturia, frontal headache in the morning, breathlessness) were investigated. After statistical evaluation we could show a relation between heart rate and pCO2 (Spearman's correlation: r = 0.331, p = 0.001, n = 105; one-tailed significance: r = 0.335, p = 0.038, n = 29). Significant differences between the groups of nycturia incidence indicate a tight relation between the incidence of nycturia and the height of hypercapnia levels (ANOVA--analysis of variance: p = 0.001). Using logistic regression we could show that information regarding medical history, especially nycturia, frontal headache and indrawings, gives important indications for global respiratory failure (sensitivity 97.62-100%, specificity 57.14-76.19%). Pathogenesis needs to be elaborated further.
Subject(s)
Medical History Taking , Neuromuscular Diseases/complications , Respiratory Insufficiency/diagnosis , Respiratory Insufficiency/etiology , Thoracic Diseases/complications , Thorax/abnormalities , Diagnosis, Differential , Dyspnea/etiology , Female , Humans , Hypercapnia/etiology , Laryngeal Masks , Male , Prospective Studies , Respiratory Insufficiency/therapy , Urination Disorders/etiologyABSTRACT
BACKGROUND: In the literature we found only five reports about noninvasive ventilation in cases with central hypoventilation syndrome. PATIENT AND METHOD: We report about a 4-year-old boy with severe late onset hypoventilation syndrome. During an interval of 3 months with nasal mask ventilation during sleep he showed an excellent cognitive and statomotoric development. After this time, he needed a noninvasive ventilation with a negative pressure system. RESULTS AND DISCUSSION: In our opinion, noninvasive nasal mask ventilation is a modern method in the treatment of patients with central hypoventilation syndrome. Tracheotomy is only necessary during the first year of life.
Subject(s)
Intermittent Positive-Pressure Ventilation , Sleep Apnea Syndromes/therapy , Child, Preschool , Follow-Up Studies , Humans , Male , PolysomnographyABSTRACT
BACKGROUND: There are only small experiences with mechanical ventilation via nasal mask in childhood. PATIENTS AND METHODS: Eleven patients using NIPPV (9 patients aged 4 to 18 years and 2 patients with cystic fibrosis aged 20 and 25 years). RESULTS: NIPPV was effective in all 11 patients. Seven patients needed supplemental oxygen. Theophyllin, Almitrin and Salbutamol could support the nasal ventilation in special conditions. CONCLUSION: Intermittent ventilation via nasal mask is a noninvasive and effective treatment of chronic respiratory failure in childhood. Monitoring with continuous pulse-oximetry is necessary.
Subject(s)
Cystic Fibrosis/rehabilitation , Intermittent Positive-Pressure Breathing/instrumentation , Masks , Neuromuscular Diseases/rehabilitation , Respiratory Insufficiency/rehabilitation , Scoliosis/rehabilitation , Sleep Wake Disorders/rehabilitation , Adolescent , Child , Child, Preschool , Female , Home Care Services , Humans , Male , Patient Acceptance of Health Care , PolysomnographyABSTRACT
Mental capacity, assessed by several anamnestic questions, was improved by adenoidectomy in 46 children with adenoid vegetations and was influenced by hypoxic episodes during sleep. Hypoxic desaturations during sleep can be predicted by 5 anamnestic and clinical parameters (specificity 70%, sensitivity 85%).
Subject(s)
Adenoidectomy , Airway Obstruction/surgery , Arousal/physiology , Attention/physiology , Mental Recall/physiology , Neuropsychological Tests , Sleep Apnea Syndromes/surgery , Airway Obstruction/physiopathology , Child , Child, Preschool , Circadian Rhythm/physiology , Female , Follow-Up Studies , Humans , Male , Oxygen/blood , Sleep Apnea Syndromes/physiopathology , Sleep Stages/physiologySubject(s)
Hypercapnia/etiology , Pulmonary Heart Disease/etiology , Respiration, Artificial/instrumentation , Respiratory Insufficiency/etiology , Respiratory Insufficiency/therapy , Scoliosis/congenital , Scoliosis/complications , Blood Gas Monitoring, Transcutaneous , Child , Female , Humans , Pulmonary Heart Disease/physiopathology , Radiography , Respiration, Artificial/methods , Respiratory Insufficiency/blood , Scoliosis/diagnostic imaging , Scoliosis/physiopathology , Sleep, REM/physiology , Thoracic VertebraeABSTRACT
Nasal intermittent positive pressure ventilation in childhood is a major advance in the noninvasive treatment of chronic respiratory failure in childhood. Between 1987 and 1994 author could found reports about 58 patients aged 2 to 18 years. Diagnosis were neuromuscular diseases (n = 51), scoliosis (n = 4), cystic fibrosis (n = 2) and central hypoventilation syndrome (n = 1). Author reports about indications, physiological effects, advantages, side effects and limits, about ethical considerations, technical and psychological preparation and realization of intermittent nasal ventilation in childhood.
Subject(s)
Intermittent Positive-Pressure Ventilation , Respiratory Insufficiency/therapy , Sleep Apnea Syndromes/therapy , Adolescent , Child , Child, Preschool , Home Care Services , Humans , Hypercapnia/etiology , Hypercapnia/therapy , Hypoxia/etiology , Hypoxia/therapy , Respiratory Insufficiency/etiology , Sleep Apnea Syndromes/etiologyABSTRACT
METHODS: In 30 children suffering from cystic fibrosis, the long-term effect (2 years) of treatment with an acid-protected micro-encapsulated pancreatin preparation was investigated in comparison with prior enzyme replacement with a conventional pancreatin preparation given over a period of years. Assessment criteria was the development of body length and body weight, lung function (vital capacity, FEF1) and the Shwachman-Kulczycki score. RESULTS: The therapeutic efficacy of the acid-protected micro-encapsulated preparation is considered to be higher than that of conventional preparations, since the same effect was achieved with only one-quarter to one-third of the pancreatin dose of the conventional preparation (average: 6.5 +/- 2.9 g pancreatin/d, as compared with 1.6 +/- 0.6 g pancreatin in the case of the acid-protected micro-encapsulated preparation). However, none of the clinical parameters investigated revealed any significant changes during the course of the observation period. The results indicate that, if the general state of health and nutritional status of patients with cystic fibrosis is to be improved further, in addition to a special diet, attempts must be made to achieve further optimization of the intraluminal digestion.
Subject(s)
Cystic Fibrosis/therapy , Lipase/administration & dosage , Pancreatic Extracts/administration & dosage , Pancreatin/administration & dosage , Adolescent , Capsules , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Long-Term Care , Male , PancrelipaseABSTRACT
For the time between 1985 and 1990 we found in the literature 14 reports on 483 patients with chronic lung diseases and 140 healthy controls which were investigated by right ventricular catheter and echocardiographic methods. The critical review about ability of echocardiography to recognise the pulmonary hypertension secondary to chronic lung diseases shows following results: 1. Acceleration time (time to peak velocity) revealed correlations to mean or systolic pulmonary artery pressure (SPAP) of -0.72 to -0.92. The sample volume of pulsed Doppler should be taken near the pulmonary valve and in the middle of pulmonary artery diameter. Correction of acceleration time by heart frequency is necessary in children (Akiba). 2. Tricuspid regurgitation to SPAP: r = 0.65-0.92, but sensitivity to recognise pulmonary hypertension was only 20 (to 91)%. Accurate quantitative measurement is possible only in 24 to 66% of the patients with chronic lung diseases. 3. Isovolemic relaxation time: r = 0.70-0.89, limitations by some cardiac influences--but also the ability to reflect effects of medication (Hatle). 4. End-diastolic diameter of tricuspid valve/m2 of body surface: r = 0.84, in connection with right ventricular end-diastolic diameter r = 0.90. Diameters of pulmonary valve, of right pulmonary artery, of right ventricle and characteristics of the flow in the Vena cava inferior have a smaller diagnostic value. Thickness of right ventricular systolic time intervals are without reliable informations about pulmonary hypertension. In conclusion there are some echocardiographic parameters with high diagnostic value in the noninvasive diagnostic of pulmonary hypertension secondary to chronic pulmonary diseases. A table of practicable values and regression equations completes this review.
Subject(s)
Echocardiography , Hypertension, Pulmonary/diagnosis , Lung Diseases/complications , Adolescent , Adult , Aged , Child , Chronic Disease , Diagnosis, Differential , Echocardiography, Doppler , Humans , Hypertension, Pulmonary/etiology , Middle Aged , Models, Biological , Regression AnalysisABSTRACT
Theophylline concentrations in serum between 10 to 20 micrograms/ml cannot be obtained with usual dosage of theophylline in children with individual very short elimination half time of theophylline. Multiple corrections of dosage and repeated controls of theophylline concentrations in serum are necessary for empirical adjustment of individual optimal dosage. Pharmacokinetic investigations were made to shorten this management. 12 patients with bronchial asthma, 6 to 18 years of age, received 1.5 to 3 tablets of Aminophyllin under conditions of steady state. Blood samples were taken up to 6 hours after oral application. As a result of statistical analysis we found that the biological half time of theophylline could be determined from 2 to 3 blood samples. Estimation of optimal individual dosage is possible by simple mathematical pharmacokinetic formulas.
Subject(s)
Aminophylline/administration & dosage , Asthma/drug therapy , Adolescent , Aminophylline/pharmacokinetics , Child , Half-Life , Humans , Theophylline/bloodABSTRACT
Today Doppler echocardiography is a reliable noninvasive method in the diagnostic of pulmonary hypertension. We investigated 52 patients (age 1 to 19 years) with pulsed- or continuous-wave Doppler. Systolic pulmonary artery pressure (SPAP) was calculated about the equation of Akiba using acceleration time, corrected by heart rate. SPAP estimated in this way was less than 20 mmHg in 14% of the patients. 20-39 mmHg in 29%, 40-59 mmHg in 31% and about 60 mmHg in 26%. No correlation was found between age and SPAP and only low correlation between score of Shwachman (r = 0.37) or score of Norman-Crispin and SPAP. Between body fat, calculated from the thickness of skin folds, and SPAP was found a good correlation (r = -0.73, p = 0.01).
Subject(s)
Cystic Fibrosis/complications , Echocardiography, Doppler , Hypertension, Pulmonary/diagnostic imaging , Adolescent , Blood Pressure , Child , Child, Preschool , Cystic Fibrosis/physiopathology , Heart Rate , Humans , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/physiopathology , Infant , Pulmonary Artery/physiopathologyABSTRACT
The etiology of alcoholic embryopathy-maternal use of alcoholic beverages during pregnancy-only is known about 20 years. But already 3,000 years ago the authors of the Old Testament warned the wife of Manoah to drink alcoholic beverages during pregnancy (judges 13,2-25). Investigation of the historical, ethnological, ideological and mythological background of this law shows, that this text reflected ideas of social alternatives and reforms from the 9th century before our time-but not any knowledge about embryo- and fetotoxic effects of alcoholic beverages during pregnancy.
Subject(s)
Bible , Fetal Alcohol Spectrum Disorders/history , Medicine in Literature , Female , Fetal Alcohol Spectrum Disorders/prevention & control , History, Ancient , History, Medieval , Humans , Infant, Newborn , Jews/history , PregnancyABSTRACT
Quintuplets were born in the little town of Lommatzsch near Meissen and Dresden, Saxony, on June 25, 1688. This birth of quintuplets, though not yet considered by medical literature, is historically authentic. Evidences of this exceptional event are notations in the birth and death registers of the town parish church in Lommatzsch, a letter reporting on the "unusual event" to the elector Johann Georg III (1600-1691) and the so-called five-children gravestone. On the basis of these Lommatzsch quintuplets, the author deals with interesting aspects of social life and medicine within the living conditions in a small town around the turn from the 17th to the 18th century.- Other historical multiplets are referred.
Subject(s)
Quintuplets , Germany , Humans , Infant, Newborn , ResearchABSTRACT
We report the case of an infant with alcoholic embryopathy associated with punctate chondrodysplasia. In 1983, this syndrome was first described by Badois et al. in three children. Our report adds a fourth case and enlarges the number of symptoms occurring in alcoholic embryopathy. We are not able to answer the question whether the stippled epiphyses in our case are due to the maternal alcoholism or part of a mild form of chondrodysplasia punctata.
