scholarly journals A review of the etiology and clinical presentation of non-cystic fibrosis bronchiectasis: A tertiary care experience

2018 ◽  
Vol 137 ◽  
pp. 35-39 ◽  
Author(s):  
Ozlem Satırer ◽  
Ayse Mete Yesil ◽  
Nagehan Emiralioglu ◽  
Gökcen Dilsa Tugcu ◽  
Ebru Yalcın ◽  
...  
Author(s):  
Abed AlLehbi ◽  
Abdullah AlMtawa ◽  
Adel Alqutub ◽  
Khalid Alsayari ◽  
Ahmed Alomair ◽  
...  

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Qionghua Chen ◽  
Yuelin Shen ◽  
Hui Xu ◽  
Xiaolei Tang ◽  
Haiming Yang ◽  
...  

Abstract Background Since public awareness of cystic fibrosis (CF) has increased, more children have been diagnosed with CF in China. This study aimed to investigate medical and other challenges faced by pediatric CF patients in China. Method Treatments and treatment outcomes were retrospectively analyzed for 46 pediatric CF patients diagnosed from August 2009 to June 2019. Pre- and post-treatment results were compared using independent samples t-test. Results Of 46 pediatric CF study patients, four died and five were lost to follow-up. Thirty-seven patients were monitored for 0.03 to 9.21 years; patients exhibited fewer attacks of respiratory tract infections after diagnosis (4.49 ± 2.13 episodes/year before diagnosis vs 1.97 ± 1.87 times/year after 1-year treatment, p < 0.05), significantly reduced sputum production and experienced 1.62 ± 1.71 exacerbations/year. Patient mean body mass index was 16.87 ± 3.53 and pancreatic malfunction persisted in 15 patients. For 17 children, no significant differences in lung function were found at follow-up as compared to lung function at diagnosis (FEV1: 82.45% ± 16.56% vs 75.26% ± 22.34%, FVC: 87.18% ± 13.64% vs 86.99% ± 19.95%, FEF75%: 46.51% ± 28.78% vs 36.63% ± 24.30%, P = 0.27, 0.97, 0.20, respectively). Pseudomonas aeruginosa (17/27) and bronchiectasis (22/22) were found during follow-up evaluation. Twenty-four patients (64.8%) maintained good adherence to therapies. Overall, azithromycin and tobramycin treatments were administered for 0.5–62 months and 0.5–48 months, respectively, and triggered no obvious adverse reactions. Conclusion No obvious declines in clinical presentation or lung function were found in Chinese pediatric CF patients after receiving standard therapeutic and active treatments, although malnutrition and low compliance were persistent challenges.


Author(s):  
Josee Paradis ◽  
Agnieszka Dzioba ◽  
Hamdy El-Hakim ◽  
Paul Hong ◽  
Frederick K. Kozak ◽  
...  

Abstract Background To evaluate the clinical presentation of choanal atresia (CA) in tertiary centers across Canada. Methods Multi-centre case series involving six tertiary care pediatric hospitals across Canada. Retrospective chart review of patients born between 1980 and 2010 diagnosed with CA at a participating center. Results The health charts of 215 patients (59.6% female) with CA were reviewed and included in this study. The mean age of patients at time of CA presentation was 0.4 months (range 0.1 to 7.2 months) for bilateral CA and 37.8 months (range 0.1 to 164.1 months) for unilateral cases. The most common presenting symptoms for bilateral CA in decreasing order were respiratory distress (96.4%), feeding difficulties (68.2%), and rhinorrhea (65.5%), and for unilateral cases in decreasing order were rhinorrhea (92.0%), feeding difficulties (24.7%), and respiratory distress (18.0%). For the majority of patients (73.2%), the obstruction comprised mixed bony and membranous tissue, with only 10.5% presenting with a purely membranous obstruction. Familial history of CA was confirmed in only 3.3% of cases. One half of patients with CA presented with one or more associated anomalies and 30.6% had a syndrome. Conclusions The present investigation is the first national multi-institutional study evaluating the clinical presentation of CA over three decades. The present cohort of CA patients presented with a breadth of co-morbidities with highly variable presentations, with bilateral cases being more severely affected than unilateral cases. Further investigation into hereditary linkages to CA development is warranted. Graphical abstract


2019 ◽  
Vol 13 (1) ◽  
pp. 39-46
Author(s):  
Jen Standen

In the UK over 10 000 people live with cystic fibrosis (CF), with 1-in-25 people being carriers of the disease. Multidisciplinary care is provided by tertiary care CF centres, with or without local secondary service shared care agreements. There are still, however, several reasons why CF sufferers or their families present to their GPs. This article aims to provide a brief overview of CF and its management. It also gives the information needed to guide patients about genetic testing and neonatal screening for the disease.


PEDIATRICS ◽  
1969 ◽  
Vol 43 (5) ◽  
pp. 905-906
Author(s):  
Aubrey Milunsky

The patient reported in the foregoing letter is of particular interest in view of the recent observations on patients with tnisomy 21 and cystic fibrosis. The multiple possibilities that could explain the clinical presentation have no doubt been considered by the authors. However, the description of "hypoplastic thrombocytopenia" and later pancytopenia in this patient, associated with pancreatic insufficiency, leads to the serious consideration of Shwachman's syndrome (pancreatic insufficiency and bone marrow dysfunction). The wide discrepancy between the sodium and chloride concentrations in the sweat reported in their letter indicates that evaporation of water may have occurred from the sweat sample, leading to an apparently higher sodium and chloride concentration.


2012 ◽  
Vol 1 (2) ◽  
pp. 85-87
Author(s):  
Ishrat Jahan ◽  
Mehedi Parvez ◽  
Laisha Yeasmin

Congenital tuberculosis is an unusual and severe clinical presentation of Mycobacterium tuberculosis (MTB) infection. It is usually difficult to diagnose and treat. We report a tenweek-old male infant who had presented with fever, difficulty in breathing, abdominal distension, convulsion, low weight gain since one month of his age. The diagnosis was made by demonstration of MTB bacilli in the gastric aspirate of baby and chest radiography. Treatment with the four drug regimen including streptomycin was initiated, but the baby died   on the third day of ATT. This case gives an account of difficulties in diagnosis and therapeutic   management of congenital tuberculosis and alerts for development of protocols that foresee   these difficulties. DOI: http://dx.doi.org/10.3329/jemc.v1i2.11469   J Enam Med Col 2011; 1(2): 85-87  


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