scholarly journals Cystic Fibrosis with Severe Anaemia and Hypoproteinaemia

2019 ◽  
Vol 38 (2) ◽  
pp. 118-121
Author(s):  
Md Abu Bakkar Siddique ◽  
Kalimuddin Khan ◽  
Aparajita Bera ◽  
Sudipta Ghorai ◽  
Jasmine Mallik ◽  
...  

The presentation of cystic fibrosis (CF) is dependent upon which organs are affected. Severe anaemia is reported to occur rarely in patients with CF. We are presenting a case of 2 months old infant admitted with anaemia, malnutrition, hypoproteinaemia and hypoalbuminaemia, without any pulmonary symptoms at the initial presentation. Abnormal sweat chlorides and low faecal elastase level confirmed the diagnosis of CF. Respiratory symptoms and signs developed later on further follow up.

2020 ◽  
Author(s):  
Mariestefany Romero ◽  
Elizabeth Leiba ◽  
Fhabián Stevens Carrión-Nessi ◽  
Diana Carolina Freitas-DeNobrega ◽  
Ángel Gamardo ◽  
...  

Abstract BackgroundPregnant women are particularly vulnerable to malaria infections, increasing the risk of maternal-foetal complications, mainly in areas of high endemicity. However, few studies of malaria in pregnancy (MiP) have been carried out in Latin America, a region with low endemicity and transmission of both, Plasmodium falciparum and P. vivax. Despite the high malaria burden in Venezuela in the last years, no recent studies of MiP have been conducted. Hence, epidemiological and clinical characteristics of pregnant women with malaria in Venezuela are described herein.MethodsA retrospective study in pregnant women attending to the “Ruíz y Páez” University Hospital Complex, Bolívar state, Venezuela between February and October, 2019 was carried out. Epidemiological, clinical, and laboratory information was analysed.ResultsThirty-seven out of 52 pregnant women analysed, were infected with P. vivax. Age ranged between 15 and 39 years, and adolescent pregnancies were common. Malaria infection was diagnosed mainly during the third trimester of pregnancy (63.4%). The distribution of symptoms and signs as well as clinical laboratory values was similar among Plasmodium spp. Although uncomplicated malaria was most frequent, 30% (13/52) had severe anaemia. A high proportion of studied women (44%) presented at least one complication during the pregnancy or delivery. Spontaneous abortion was recorded in four women, and three foetal deaths were observed. Six women had preterm delivery without any further complication.ConclusionsA high prevalence of maternal and foetal complications was found in the studied population, highlighting the requirement for a careful medical follow up during the prenatal check-ups, aimed to reduce the negative impact of malaria in the new-born and mother.


2020 ◽  
Vol 13 (1) ◽  
pp. 413-414 ◽  
Author(s):  
Mohamed Farouk Allam

Due to the international spread of COVID-19, the difficulty of collecting nasopharyngeal swab specimen from all suspected patients, the costs of RT-PCR and CT, and the false negative results of RT-PCR assay in 41% of COVID-19 patients, a scoring system is needed to classify the suspected patients in order to determine the need for follow-up, home isolation, quarantine or the conduction of further investigations. A scoring system is proposed as a diagnostic tool for suspected patients. It includes Epidemiological Evidence of Exposure, Clinical Symptoms and Signs, and Investigations (if available). This scoring system is simple, could be calculated in a few minutes, and incorporates the main possible data/findings of any patient.


2021 ◽  
Vol 10 (7) ◽  
pp. 1527
Author(s):  
Jamie Duckers ◽  
Beth Lesher ◽  
Teja Thorat ◽  
Eleanor Lucas ◽  
Lisa J. McGarry ◽  
...  

Cystic fibrosis (CF) is a rare, progressive, multi-organ genetic disease. Ivacaftor, a small-molecule CF transmembrane conductance regulator modulator, was the first medication to treat the underlying cause of CF. Since its approval, real-world clinical experience on the use of ivacaftor has been documented in large registries and smaller studies. Here, we systematically review data from real-world observational studies of ivacaftor treatment in people with CF (pwCF). Searches of MEDLINE and Embase identified 368 publications reporting real-world studies that enrolled six or more pwCF treated with ivacaftor published between January 2012 and September 2019. Overall, 75 publications providing data from 57 unique studies met inclusion criteria and were reviewed. Studies reporting within-group change for pwCF treated with ivacaftor consistently showed improvements in lung function, nutritional parameters, and patient-reported respiratory and sino-nasal symptoms. Benefits were evident as early as 1 month following ivacaftor initiation and were sustained over long-term follow-up. Decreases in pulmonary exacerbations, Pseudomonas aeruginosa prevalence, and healthcare resource utilization also were reported for up to 66 months following ivacaftor initiation. In studies comparing ivacaftor treatment to modulator untreated comparator groups, clinical benefits similarly were reported as were decreases in mortality, organ-transplantation, and CF-related complications. The safety profile of ivacaftor observed in these real-world studies was consistent with the well-established safety profile based on clinical trial data. Our systematic review of real-world studies shows ivacaftor treatment in pwCF results in highly consistent and sustained clinical benefit in both pulmonary and non-pulmonary outcomes across various geographies, study designs, patient characteristics, and follow-up durations, confirming and expanding upon evidence from clinical trials.


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.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 846.3-846
Author(s):  
N. Madeira ◽  
M. Alvarenga Santos ◽  
L. Cunha Miranda ◽  
S. Clemente ◽  
S. Furtado

Background:Interstitial Lung Diseases (ILD) may present features suggesting an underlying autoimmune process, which seem to differentiate them from idiopathic interstitial pneumonias, although without fully meeting the classification criteria (CC) for a specific connective tissue disease. Different terms had been used to describe these conditions and, to reach a consensus, the European Respiratory Society/American Thoracic Society proposed the CC for an entity named Interstitial Pneumonia with Autoimmune Features (IPAF). Clinical evolution and prognosis of this entity are still poorly understood.Objectives:To evaluate clinical evolution and prognosis of a population of patients with IPAF.Methods:Retrospective analysis of clinical files of patients followed by the Pulmonology Department since 02/2012 until 06/2019, who met the CC for IPAF, regarding clinical, functional and radiological evolution. Patients were considered to have a progressive phenotype in 24±3 months from their 1stevaluation if they fulfil 1 of the 4 criteria: relative decline in FVC ≥10% predicted; relative decline in FVC ≥5–<10% predicted and worsened respiratory symptoms; relative decline in FVC ≥5–<10% predicted and increased extent of fibrosis on High-resolution Computed Tomography (HRCT); worsened respiratory symptoms and increased extent of fibrosis on HRCT.Results:22 (7.4%) of 296 ILD patients met IPAF CC. 59.0% were female with an age at the 1stevaluation of 66.7±12.4 years. They were all non-smokers (63.6%) or ex-smokers (36.4%). Serologic and morphologic criteria were both present in 21 (95.4%) and clinical criteria in 5 patients (22.7%). Antinuclear antibodies (ANA) were identified in 19, rheumatoid factor in 4, SSA in 3 and anti-Jo-1 in 1 patient. HRCT patterns were identified in 21 patients: 15 nonspecific interstitial pneumonia (NSIP), 5 organizing pneumonia (OP) and 2 lymphocytic interstitial pneumonia (LIP). One NSIP and 1 LIP identified on HRCT were confirmed by histopathology. Three patients had inflammatory arthritis and 2 had Raynaud’s phenomenon. Immunosuppressive therapy was introduced in most cases (18 patients, including systemic corticotherapy in 17, azathioprine in 4, mycophenolate mofetil in 1), azithromycin was prescribed in 2 patients and 3 remained without therapy. Regarding the follow up at 24±3 months from the 1stevaluation (3 patients were excluded due to too recent follow-up), 4 patients (18.2%) had progressive phenotype, 7 (31.8%) had a favourable evolution and 3 (13.6%) patients had died. During a follow-up of 31.1±19.8 months, this number rose to 6 patients (27.3%), all of them died by respiratory cause and had NSIP pattern. No differences were found in age, last FVC, therapy and time of disease evolution between those who died and the others.Conclusion:Our study showed that a small proportion of IPAF patients had a progressive phenotype and the NSIP pattern seemed to be a poor prognosis factor for survival.References:[1]Ito Y, Arita M, Kumagai S, et al. Serological and morphological prognostic factors in patients with interstitial pneumonia with autoimmune features. BMC Pulm Med 2017; 17:111 10.1186/s12890-017-0453-zDisclosure of Interests:None declared


2021 ◽  
Vol 9 (5) ◽  
pp. 232596712110040
Author(s):  
Ji Hye Choi ◽  
Kug Jin Choi ◽  
Chin Youb Chung ◽  
Moon Seok Park ◽  
Ki Hyuk Sung ◽  
...  

Background: Ankle stress radiographs are important tools for evaluating chronic lateral ankle instability. The consistency of a patient’s ankle condition as it affects the reliability of ankle stress radiographs has never been evaluated. Purpose: To investigate the consistency and reliability of ankle stress radiographs in patients with chronic lateral ankle instability without an ankle injury during the study period. Study Design: Cohort study (diagnosis); Level of evidence, 3. Methods: Included were patients with chronic lateral ankle instability who underwent 2 repeated ankle stress radiographs between January 2014 and July 2019; those with an ankle injury during the study period were excluded. The tibiotalar tilt angle on varus stress radiographs and anterior translation of the talus on anterior drawer stress radiographs were measured at initial presentation and final follow-up examination. Interobserver reliability and consistency of ankle stress radiographs were analyzed using the intraclass correlation coefficient (ICC). Results: A total of 45 patients (mean ± standard deviation age, 36.4 ± 13.4 years; 18 men and 27 women; follow-up duration, 9.1 ± 3.2 months) were included. The mean ± standard deviation tibiotalar tilt angle and anterior talar translation at initial presentation were 10.8° ± 5.2° and 6.9 ± 2.7 mm, respectively. The interobserver reliabilities of the tibiotalar tilt angle and anterior talar translation were excellent (ICC = 0.926 [95% CI, 0.874-0.959] and 0.911 [95% CI, 0.766-0.961], respectively). The consistency between the initial and final radiographs was good for tibiotalar tilt angle (ICC = 0.763 [95% CI, 0.607-0.862]) and poor for anterior talar translation (ICC = 0.456 [95% CI, 0.187-0.660]). Conclusion: Although the interobserver reliability of the radiographic measurements was excellent, the consistency of the ankle stress radiographs was not as acceptable. Surgeons need to be cautious when deciding whether to operate on a patient with chronic lateral ankle instability based on a single ankle stress radiograph.


2021 ◽  
Vol 8 (1) ◽  
pp. e000538
Author(s):  
Alejandra Tepox-Padrón ◽  
Rafael Ambrosio Bernal-Mendez ◽  
Gilberto Duarte-Medrano ◽  
Adriana Fabiola Romano-Munive ◽  
Milton Mairena-Valle ◽  
...  

Idiopathic acute recurrent pancreatitis (IARP) is defined as at least two episodes of acute pancreatitis with the complete or near-complete resolution of symptoms and signs of pancreatitis between episodes, without an identified cause. There is a paucity of information about the usefulness of endoscopic ultrasound (EUS) in IARP.ObjectivesTo determine the diagnostic yield of EUS in IARP.DesignA retrospective study was performed in patients with IARP evaluated by EUS between January 2009 and December 2016. Follow-up assessments of acute pancreatitis recurrence were carried out.ResultsSeventy-three patients with 102 EUS procedures were included. EUS was able to identify the cause of IARP in 55 patients (75.3%). The most common findings were chronic pancreatitis in 27 patients (49.1%), followed by lithiasic pathology in 24 patients (43.6%), and intraductal papillary mucinous neoplasm in four patients (7.3%). A directed treatment against EUS findings had a protective tendency associated with the final resolution of recurrence. There were no complications reported.ConclusionEUS performed in patients with IARP helped to identify a possible cause in 2/3 of the cases. The majority of patients have a treatable disease.


Author(s):  
Akın Çinkooğlu ◽  
Selen Bayraktaroğlu ◽  
Naim Ceylan ◽  
Recep Savaş

Abstract Background There is no consensus on the imaging modality to be used in the diagnosis and management of Coronavirus disease 2019 (COVID-19) pneumonia. The purpose of this study was to make a comparison between computed tomography (CT) and chest X-ray (CXR) through a scoring system that can be beneficial to the clinicians in making the triage of patients diagnosed with COVID-19 pneumonia at their initial presentation to the hospital. Results Patients with a negative CXR (30.1%) had significantly lower computed tomography score (CTS) (p < 0.001). Among the lung zones where the only infiltration pattern was ground glass opacity (GGO) on CT images, the ratio of abnormality seen on CXRs was 21.6%. The cut-off value of X-ray score (XRS) to distinguish the patients who needed intensive care at follow-up (n = 12) was 6 (AUC = 0.933, 95% CI = 0.886–0.979, 100% sensitivity, 81% specificity). Conclusions Computed tomography is more effective in the diagnosis of COVID-19 pneumonia at the initial presentation due to the ease detection of GGOs. However, a baseline CXR taken after admission to the hospital can be valuable in predicting patients to be monitored in the intensive care units.


BMC Medicine ◽  
2021 ◽  
Vol 19 (1) ◽  
Author(s):  
K. Lokuge ◽  
E. Banks ◽  
S. Davis ◽  
L. Roberts ◽  
T. Street ◽  
...  

Abstract Background Following implementation of strong containment measures, several countries and regions have low detectable community transmission of COVID-19. We developed an efficient, rapid, and scalable surveillance strategy to detect remaining COVID-19 community cases through exhaustive identification of every active transmission chain. We identified measures to enable early detection and effective management of any reintroduction of transmission once containment measures are lifted to ensure strong containment measures do not require reinstatement. Methods We compared efficiency and sensitivity to detect community transmission chains through testing of the following: hospital cases; fever, cough and/or ARI testing at community/primary care; and asymptomatic testing; using surveillance evaluation methods and mathematical modelling, varying testing capacities, reproductive number (R) and weekly cumulative incidence of COVID-19 and non-COVID-19 respiratory symptoms using data from Australia. We assessed system requirements to identify all transmission chains and follow up all cases and primary contacts within each chain, per million population. Results Assuming 20% of cases are asymptomatic and 30% of symptomatic COVID-19 cases present for testing, with R = 2.2, a median of 14 unrecognised community cases (8 infectious) occur when a transmission chain is identified through hospital surveillance versus 7 unrecognised cases (4 infectious) through community-based surveillance. The 7 unrecognised community upstream cases are estimated to generate a further 55–77 primary contacts requiring follow-up. The unrecognised community cases rise to 10 if 50% of cases are asymptomatic. Screening asymptomatic community members cannot exhaustively identify all cases under any of the scenarios assessed. The most important determinant of testing requirements for symptomatic screening is levels of non-COVID-19 respiratory illness. If 4% of the community have respiratory symptoms, and 1% of those with symptoms have COVID-19, exhaustive symptomatic screening requires approximately 11,600 tests/million population using 1/4 pooling, with 98% of cases detected (2% missed), given 99.9% sensitivity. Even with a drop in sensitivity to 70%, pooling was more effective at detecting cases than individual testing under all scenarios examined. Conclusions Screening all acute respiratory disease in the community, in combination with exhaustive and meticulous case and contact identification and management, enables appropriate early detection and elimination of COVID-19 community transmission. An important component is identification, testing, and management of all contacts, including upstream contacts (i.e. potential sources of infection for identified cases, and their related transmission chains). Pooling allows increased case detection when testing capacity is limited, even given reduced test sensitivity. Critical to the effectiveness of all aspects of surveillance is appropriate community engagement, messaging to optimise testing uptake and compliance with other measures.


Thorax ◽  
2021 ◽  
pp. thoraxjnl-2020-216721
Author(s):  
Md Omar Faruque ◽  
H Marike Boezen ◽  
Hans Kromhout ◽  
Roel Vermeulen ◽  
Ute Bültmann ◽  
...  

ObjectivesTo date, only a few studies have investigated the associations between occupational exposures and respiratory outcomes longitudinally in the general population. We investigated the associations between occupational exposures and the development of respiratory symptoms and airway obstruction in the Lifelines Cohort Study.MethodsWe included 35 739 occupationally active subjects with data on chronic cough, chronic phlegm, chronic bronchitis or airway obstruction at baseline and approximately 4.5 years follow-up. Exposures to biological dust, mineral dust, gases/fumes, pesticides, solvents and metals in the current job at baseline were estimated with the ALOHA+job-exposure matrix (JEM). Airway obstruction was defined as FEV1/FVC below the lower limit of normal. Logistic regression analysis adjusted for baseline covariates was used to investigate the associations.ResultsAt follow-up, 1888 (6.0%), 1495 (4.7%), 710 (2.5%) and 508 (4.5%) subjects had developed chronic cough, chronic phlegm, chronic bronchitis and airway obstruction, respectively. High exposure to biological dust was associated with a higher odds to develop chronic cough and chronic bronchitis. High exposure to pesticides was associated with a higher odds for the development of all respiratory symptoms and airway obstruction. In the multiple exposures analyses, only the association between pesticides exposure and respiratory symptoms remained.ConclusionsSubjects exposed to high pesticides had a higher odds to develop respiratory symptoms on average 4.5 years later. Control measures should be taken to reduce pesticides exposure among the working population to prevent respiratory symptoms and airway obstruction.


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