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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The contribution of observations in congenital deficiency disorders to our understanding of normal mechanisms can hardly be overestimated, as may in particular be seen in the complex field of mechanisms aiming at maintaining the individual integrity. After a brief summary of the natural lines of defense we demonstrate a few cases with typical deficiencies, i.e. of the integuments (ciliary dyskinesia syndrome), of the phagocytes (chronic granulomatous disease = CGD), of specific immune reactions (antibody deficiency syndrome, severe combined immunodeficiency = SCID and syndromes with associated immunodeficiencies), and finally of the complement properdin system. Classification of these Primary Immunodeficiency Syndromes (= PIDS) with exceptional research potential as proposed by an expert panel of the WHO turned out to be quite useful also for the understanding of by far more frequent secondary disorders, in particular of the recently observed acquired immunodeficiency syndrome = AIDS due to an infection with the human immunodeficiency virus (= HIV). For pediatricians in industrialized countries, however, children with frequently recurring, but trivial infections are of considerable practical importance. A clear diagnostic concept is the necessary base for the proposed treatment, counselling and help for the inflicted parents.
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PMID:[Resistance to infection. Physiology and pathology of the immune system]. 265 37

Controversy exists regarding the management of the middle meatus after pediatric functional endoscopic sinus surgery (FESS). To prevent adhesions following pediatric FESS, gelatin film stenting of the middle meatus has been recommended. The effects of stenting, however, have not been established. Fifty-one children with similar degrees of bilateral sinus disease had a gelatin film stent placed in one middle meatus on completion of FESS, while the opposite meatus was not stented. Two to three weeks later at the time of a second, staged procedure, the sides were compared for the presence of the stent, adhesions, granulaion tissue, and patency of the maxillary sinus ostia. In 11 children the postoperative findings were more severe in the side without the stent, whereas in 29 children they were more severe in the stented side. There was no difference between the sides in 11 children. Although gelatin film stenting benefits some children, it should not be used routinely following pediatric FESS but should be reserved for children who are predisposed to develop adhesions or have poor prognostic factors, such as immunodeficiency and ciliary dyskinesia.
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PMID:The effects of gelatin film stents in the middle meatus. 920 96

The authors studied expectoration, cough clearance and immunity in 48 patients with exacerbation of chronic obstructive pulmonary affections (COPA) associated with lymphoproliferative diseases (chronic lymphoid leukemia, lymphocytic lymphoma, myeloma) versus 16 control patients suffering from COPA alone. patients of the test group received cytostatics and steroids according to standard schemes. They are shown to develop aggravation of immune defects with emergence of persistent secondary immunodeficiency, more marked disturbance of tracheobronchial clearance with more frequent occurrence of persistent ciliary dyskinesia. This resulted in slower relief of clinical symptoms, more severe course dictating the necessity of longer treatment, the addition of immunocorrectors, antibacterial drugs without immunosuppressive action.
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PMID:[The characteristics of the clinical course and treatment of chronic obstructive lung diseases against a background of lymphoproliferative diseases]. 922 20

The aim of our study was to evaluate the success, complications, and morbidity following a modified Thal fundoplication in children with reflux-associated respiratory disease (RARD). We used a procedure consisting of retroesophageal hiatal plasty, wrapping the gastric fundus around the gastroesophageal junction 180 degrees, and fixation of the lesser curvature at the abdominal wall. Follow-up by questionnaire of 128 (77 male, 51 females) out of 196 antireflux procedures between 1992 and 1995 was achieved. Surgical therapy was considered justified whenever there was gastroesophageal reflux resulting in severe recurrent respiratory symptoms. Eleven percent of the children suffered from bronchiectasis. The diagnosis of RARD was based on a high index of suspicion, barium swallow with fluoroscopy, 24-hr two-level pH-monitoring, bronchoscopy, bronchoalveolar lavage and detection of lipid-laden alveolar macrophages, esophago-gastroscopy, and esophageal biopsy. Patients with bronchopulmonary diseases such as allergy, immunodeficiency, cystic fibrosis, primary ciliary dyskinesia, and malformation of the bronchial tree or vessels had been excluded. "Evident improvement" as a result of surgery was reported in 88%, "no change" in 10%, and a "change for the worse" in 2% of patients. Persistent mild difficulties in swallowing were observed in 11%. Paraesophageal hernia, gas-bloat syndrome, and dumping syndrome were not observed. Two children needed a second operation because of relapse. The use of emergency steroidal medication for acute respiratory distress decreased impressively (219 single doses/year before surgery vs. 30 single doses/year after surgery). The need for more than 4 times/year of antibiotic therapy before surgery was reduced from 52. 3% before to 14% after surgery. Most (90.6%) of the parents stated they would agree to have surgery done again if medically indicated. In conclusion, Thal fundoplication is sufficient, safe, and effective in the management of RARD. Complications of the procedure were minor and of little consequence to the patient.
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PMID:Antireflux surgery in children suffering from reflux-associated respiratory diseases. 1128 21

The aim of our study is to evaluate the temperature distributions around the acupuncture points and channels by visualizing the infrared emissions emanated from the human skin. Our thermal imaging system that is the most sensitive to 10um long infrared wavelength allows us to recognize the actual location of acupuncture point and analyze its functional condition by examining the temperature gradient between the point and its surrounding skin area. The temperature perception of acupuncture point on extremities is especially important since its location and functional condition have a tendency to be altered if one contract disease such as immunodeficiency, arterial hypertension, and dyskinesia of biliary ducts. Also, thermovisual examinations over the acupuncture points allow us to perform the objective medical treatments by observing the transition of temperature gradients.
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PMID:Thermovisual evaluation of acupuncture points. 1139 90

Bronchiectasis is associated with heterogeneous predisposing conditions that cause abnormal dilatation and persistent inflammation in the bronchial tree and lung parenchyma. The disease remains a common cause of significant morbidity and mortality, especially when associated with hereditary disorders such as cystic fibrosis, ciliary dyskinesia, and immunodeficiency states. Recent investigations have focused on the inflammatory mediators involved in the pathogenesis of bronchiectasis. High-resolution computed tomography is now the diagnostic modality of choice and may also contribute to clinical management. Computed tomography and high-resolution computed tomography have identified bronchiectasis in individuals with HIV and alpha-1-antitrypsin deficiency. Early identification of predisposing disorders and aggressive management of symptoms has already been demonstrated by prolonged survival and decreased morbidity in cystic fibrosis patients, and similar management may benefit other populations with bronchiectasis. New treatments such as recombinant human DNase have been shown to improve pulmonary function and quality of life in cystic fibrosis patients and may prove useful in other chronic inflammatory lung disease.
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PMID:An update on bronchiectasis. 1578 1

The aim of the current study was to review the aetiology of non-cystic fibrosis (CF) bronchiectasis from two tertiary paediatric respiratory units in order to determine how often making a specific aetiological diagnosis leads to a change in management, and to assess the contribution of computed tomography (CT) in determining the underlying diagnosis. The case records of all patients who were diagnosed as having bronchiectasis by CT, currently being seen at the Royal Brompton Hospital and Great Ormond Street Hospital for Children (London, UK), were reviewed. All patients had undergone extensive investigations, and the underlying aetiology and the area of pulmonary involvement (as seen on CT) were recorded. A total of 136 patients were identified; there were 65 young males and the group median (range) age was 12.1 yrs (3.1-18.1). Immunodeficiency, aspiration and primary ciliary dyskinesia accounted for 67% of the cases. In 77 (56%) children, the identification of a cause led to a specific change in management. There was no association between aetiology and the distribution of CT abnormalities. In conclusion, immunodeficiency and other intrinsic abnormalities account for the majority of cases of non-cystic fibrosis bronchiectasis seen in the current authors' units. Computed tomography scans do not contribute towards identifying the aetiology and, most importantly, a specific aetiological diagnosis frequently leads to a change in management.
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PMID:Non-CF bronchiectasis: does knowing the aetiology lead to changes in management? 1620 14

Clinical practice guidelines for the management of acute sinusitis in children have been published by the American Academy of Pediatrics. Of note is that in this document, a brief discussion of chronic disease concluded that the pathogenesis and management are essentially unknown. Although there are insufficient data in the literature to develop evidence-based clinical guidelines, a careful review of the literature and clinical experience of experts who manage pediatric chronic sinusitis is presented in an effort to develop specific recommendations and to offer practical treatment options. Factors associated with chronic sinusitis should be addressed individually and include recurrent viral upper respiratory infections, allergic and nonallergic rhinitis, ciliary dyskinesia, cystic fibrosis, immunodeficiency, and anatomic abnormalities. Bacteriology includes the 3 pathogens associated with acute disease i.e., Streptococcus pneumoniae, Haemophilus influenzae, and Moraxella catarrhalis but with chronic sinusitis also includes Staphylococcus aureus, anaerobic bacteria, and fungi. Medical interventions discussed include endoscopic sinus surgery, saline nasal irrigation, intranasal decongestant therapy, intranasal steroids, and oral antibiotics. Clinical ranking without regard to side effects and cost suggests that endoscopic sinus surgery and antral irrigation have the highest probability of substantial symptom improvement. Other issues discussed include identification and management of gastroesophageal reflux disease (GERD), allergy, and immune deficiency.
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PMID:Chronic sinusitis in children. 1601 92

Bronchiectasis is becoming less common as the treatment for acute lower respiratory tract infections improves and immunization programmes decrease the frequency of pertussis and measles. However bronchiectasis is still a challenge to the paediatric chest physicians in many developing parts of the world and it remains a frequent problem being the final common pathway of several different lower respiratory tract insults such as cystic fibrosis, immunodeficiency, ciliary dyskinesia. Although the treatment of patients with bronchiectasis is primarily medical, surgical treatment is required in a small group of patients with recurrent episodes of pneumonia and atelectasis localized to one area, severe or recurrent hemoptysis and in those unresponsive to aggressive medical treatment with abnormal growth and development. There are unanswered questions about childhood bronchiectasis, mainly on aetiology and treatment which require more research.
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PMID:Non cystic fibrosis bronchiectasis. 1626 48

Clinical practice guidelines for the management of acute bacterial rhinosinusitis in children were published by the American Academy of Pediatrics in 2001. Changes in the antibiotic susceptibility patterns for the common pathogens causing both acute and chronic rhinosinusitis warrant a reevaluation and update of these recommendations. In addition, there was only a very brief discussion of chronic disease in this publication, with the conclusion that the pathogenesis and management of recurrent or prolonged infection were essentially unknown. Although there are still insufficient data in the literature to develop evidence-based clinical guidelines, a careful review of recent literature and the clinical experience of experts who manage pediatric chronic sinusitis are presented in an effort to provide some specific recommendations and to offer practical treatment options. Factors associated with chronic rhinosinusitis should be addressed individually and include environmental pollution, recurrent viral upper respiratory infections, allergic and nonallergic rhinitis, ciliary dyskinesia, cystic fibrosis, immunodeficiency, gastroesophageal reflux, and anatomic abnormalities.
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PMID:Rhinosinusitis in children. 1702 77


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