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Results for “Ciliary Motility Disorders”

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At least 433 records · Page 24Linked to original sources
PubMed2000

Pregnancies established through intracytoplasmic sperm injection (ICSI) using spermatozoa with dysplasia of fibrous sheath.

AIM: Dysplasia of the fibrous sheath (DFS) is an anomaly found in asthenozoospermic patients with extremely low or absent motility. In order to determine the efficacy of ICSI in these patients, a retrospective analysis of ICSI results in DFS patients has been done. METHODS: Ten ICSI attempts were performed in 6 patients with diagnosis of Dysplasia of the Fibrous Sheath studied by transmission and scanning electron microscopy. RESULTS: In the cases studied, sperm concentration was (29.62 +/- 18.05) x 10(6)/mL, total motility was 1.14 +/- 1.31%. Progressive motility was 0% except for one case with 0.1% . One hundred and three preovulatory oocytes were obtained and 94 metaphase II oocytes were injected. Sixty-nine of them showed two pronuclei (fertilization rate: 73.4%). Forty-nine embryos were obtained and 34 were transferred (mean: 3.4 embryos per transfer). Five pregnancies were diagnosed by beta-hCG plasma level determinations that resulted to be one preclinical abortion, one clinical abortion and three deliveries. Another pregnancy (ongoing) was achieved from a cryopreserved embryo transfer. CONCLUSION: These results showed that ICSI provides a suitable solution for patients suffering from irreversible sperm defects such as DFS. Nevertheless, it is mandatory to inform couples of possible transmission risks to offspring, which are unknown at present. Only when the etiology of this problem is disclosed, it will be possible to assess the real genetic risk.

Adult↗
PubMed2001

[Association between heroin consumption in pregnancy and structural abnormalities of the respiratory cilia in newborn infants].

OBJECTIVE: To describe structural abnormalities of the respiratory cilia in newborn infants whose mothers consumed heroin during pregnancy. PATIENTS AND METHODS: The medical records of 295 newborn infants whose mothers consumed heroin either throughout or at some time during pregnancy and who were cared for in Hospital del Mar in Barcelona (Spain) between January 1982 and December 1997 were reviewed. Seven infants with neonatal respiratory distress after the withdrawal syndrome period were selected. Diagnoses were established by electron microscopy of nasal mucosa samples. RESULTS: All seven newborns with prolonged neonatal respiratory distress had ultrastructural abnormalities of the ciliary axoneme similar to those of primary ciliary dyskinesia or immotile cilia syndrome. The incidence of this alteration in this series was higher than that in the general population. CONCLUSIONS: These data suggest a possible association between ultrastructural abnormalities of the ciliary axoneme and prolonged neonatal respiratory distress in the infants of heroin-consuming mothers.

Ciliary Motility Disorders↗
PubMedDate not supplied

[Report of a case of primary ciliary aplasia].

We report a case of one boy with ciliary aplasia characterized by diagnostic peculiarity, consisting in the initial non-diagnosis, a period of relative well-being and a relapse with good recovery after adequate therapy.

Adolescent↗
PubMed2001

[A case of Mycobacterium intracellulare infection complicated by immotile cilia syndrome].

A 25-year-old woman with a history of immotile cilia syndrome (ICS) was admitted to our hospital with dyspnea. Chest roentgenography revealed dense infiltrates in both lower lung fields in addition to bronchiectasis and small nodular opacities, which had been observed previously. Transbronchial lung biopsy demonstrated evidence of non-caseating epithelioid cell granuloma. Sputum specimens were examined, and isolates were identified as Mycobacterium intracellulare. The patient was given antituberculous therapy and clarithromycin, which induced clinical improvement. It is well known that bronchial mucociliary transport is severely impaired in patients with ICS. However, to our knowledge, cases of M. intracellulare infection complicated by ICS have not been reported in Japan. We must pay close attention to the concurrence of these diseases.

Adult↗
PubMed2001

[Pediatric pulmonology].

Paediatric pulmonology (paediatric respiratory medicine) concerns such lung diseases in children as asthma, pneumonia, cystic fibrosis, primary ciliary dyskinesia, chronic interstitial pneumonia, bronchopulmonary dysplasia and congenital abnormalities. This specialty has been approved as an official subsection of the European Confederation of Specialists in Paediatrics (CESP) and acknowledged by the European Union of Medical Specialists (UEMS). A training syllabus has been defined and training centres in all EU countries, including Denmark, have been identified and approved by the local paediatric organisations. The training syllabus emphasises routine in the clinical diagnosis and treatment of the diseases, as well as methods such as lung function in all age groups, bronchoscopy, biopsy, and others. This article summarises the status of this specialty, and the training syllabus, and highlights key research questions.

Asthma↗
PubMed2002

[A case of immotile-dyskinetic cilia syndrome responding to clenbuterol hydrochloride and azithromycin].

In this case, a 30-year-old man had been treated for chronic sinusitis and bronchiectasis since 2000, and presented at our outpatient clinic in May 2001 with chief complaints of massive yellow sputum expectoration and dyspnea. After he was admitted by our hospital, Pseudomonas aeruginosa bacteria were isolated at the rate of 10(8)/ml from his sputum culture. In electron-microscopic observation, the cilia of the bronchial epithelium were found to lack dynein arms. Semen examination revealed decreased sperm motility. Thus, the following diagnosis was made: diffuse bronchiectasis associated with the immotile-dyskinetic cilia syndrome, complicated with a P. aeruginosa infection. After the airway infection was ameliorated, 40 mg/day of clenbuterol hydrochloride was administered in combination with 250 mg of azithromycin, which was given twice a week, and which led to a markedly decreased frequency of exacerbation of airway infection. Moreover, chest CT scanning and respiratory function testing also indicated improvements. It was hypothesized that the decreased cilia motility due to P. aeruginosa-produced pyocyanin would be ameliorated with a b2 stimulant, and the inhibitory effect of a macrolide on the P. aeruginosa biofilm and production of pyocyanin would also be involved in the improvement of this case.

Adult↗
PubMed2003

Nasal ciliary studies in children with chronic respiratory tract symptoms.

Ciliary abnormalities include a range of morphological dysfunctions resulting in dysmotility. These typically manifest with upper and lower respiratory symptoms later in the infancy and early childhood. The diagnosis is based on ciliary studies i.e. measurement of ciliary beat frequency (CBF). We present a series of 67 children who had nasal biopsy and ciliary studies done between 1993-2002. There were 44 boys and 23 girls of age between 1 to 17 years. In 49 (73%) cases indication of ciliary studies was chest symptoms. There were six diagnostic categories: asthma, recurrent chest infections, bronchiectasis, rhinosinusitis, dextrocardia and prematurity. Fourteen patients (20%) had dextrocardia and of these 9 had no measurable beating cilia. In the rest 5 CBF ranged 8-12.7 Hz. If the patients with dextrocardia are excluded, 5 of the 53 (10%) did not have any ciliary activity and in remaining CBF ranged 5.3-19.7 Hz. Our results showed a significant number of children with immotile cilia had associated dextrocardia. In the absence of situs inversus index of suspicion should be very high to detect these cases early. Otolaryngologists can play a key role in diagnosis because of an easy access for nasal biopsy, which is much simple than bronchial biopsies.

Adolescent↗
PubMed2003

Changes in the bronchial epithelia in patients with immotile cilia syndromes.

Immotile cilia syndromes is a cause of recurrent infection of the airways and recurrent bronchopneumonias. Among the ciliary abnormalities are found changes in the structure of the microtubules, unco-ordinated ciliary movements caused by the absence of inner or outer or both dynein arms, and abnormalities of the kinetosomes and/or rete ridges. In patients with ciliary dyskinaesia bronchitis occurs early in life (during infancy) and usually has a recurrent tendency, so that bronchial biopsy is frequently undergone for diagnostic purposes. In this study we include 127 bronchial biopsies from patients (from 2 months to 49 years) unsuccessfully treated for recurrent respiratory tract infections. When performing regular diagnostic procedures on the light and electron microscopic level, we have looked for cilia abnormalities and also focused on changes within the mucosa and submucosa. The most common abnormality recorded was absence of the inner dynein arms, but in 40 cases neither of the dynein arms were present. Only a few patients had classical Kartagener's syndrome. Special attention is drawn to biopsies from elderly patients, in whom long-standing infections were followed by extensive damage to the bronchial epithelium, including even a total absence of ciliated cells. In some cases enhanced regenerative processes and some foci of squamous metaplasia were found. In two cases even foci of low-grade dysplasia were diagnosed.

Adolescent↗