Relevant bibliographies by topics / Cystic fibrosis, juvenile literature

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Academic literature on the topic 'Cystic fibrosis, juvenile literature'

Author: Grafiati
Published: 4 June 2021
Last updated: 29 July 2024

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Journal articles on the topic "Cystic fibrosis, juvenile literature"

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Orsini, Sara Immacolata, Edoardo Marrani, Ilaria Pagnini, Giovanni Taccetti, Vito Terlizzi, and Gabriele Simonini. "Concomitant Use of Elexacaftor/Tezacaftor/Ivacaftor and Etanercept in a Cystic Fibrosis Patient with Juvenile Idiopathic Arthritis." Journal of Clinical Medicine 12, no. 5 (February 21, 2023): 1730. http://dx.doi.org/10.3390/jcm12051730.

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Patients with cystic fibrosis often complain of joint manifestations. However, only a few studies have reported the association between cystic fibrosis and juvenile idiopathic arthritis and addressed the therapeutic challenges of these patients. We describe the first paediatric case of a patient affected by cystic fibrosis, Basedow’s disease and juvenile idiopathic arthritis who was contemporarily treated with elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) and anti-tumor necrosis factor α (anti-TNFα). This report seems to reassure regarding the potential side effects of these associations. Moreover, our experience suggests that anti-TNFα is an effective option in CF patients affected by juvenile idiopathic arthritis, and is even safe for children receiving a triple CFTR modulator.
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Krawczyk, Patrycja, Urszula Sioma-Markowska, Dominika Krówka, Mariola Machura, Sylwia Kubaszewska, Violetta Skrzypulec-Plinta, and Patrycja Sodowska. "Cystic fibrosis and pregnancy – literature review." Annales Academiae Medicae Silesiensis 70 (2016): 84–88. http://dx.doi.org/10.18794/aams/61771.

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Wassil, Sarah K., Kristie M. Fox, and James W. White. "Once Daily Dosing of Aminoglycosides in Pediatric Cystic Fibrosis Patients: A Review of the Literature." Journal of Pediatric Pharmacology and Therapeutics 13, no. 2 (January 1, 2008): 68–75. http://dx.doi.org/10.5863/1551-6776-13.2.68.

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Patients with cystic fibrosis receive many courses of antibiotic therapy throughout their lifetime. Dosing aminoglycosides once daily has become common practice in many of these individuals. Due to ease of home administration, decreased nursing time, and improved quality of life, this regimen is being increasingly explored in the cystic fibrosis population. Because patients with cystic fibrosis have increased aminoglycoside clearance, once daily dosing may result in a prolonged time during the dosing interval when concentrations of the drug may be undetectable. This makes the use of once daily dosing of these antibiotics in patients with cystic fibrosis controversial. Although aminoglycosides exhibit a post antibiotic effect, the duration of this effect is unknown in humans; therefore, the development of resistance to the aminoglycoside is a concern. This manuscript will review the organisms most commonly associated with a pulmonary exacerbation of cystic fibrosis, the properties of the aminoglycoside that make once daily dosing feasible, the concept of once daily dosing in those with cystic fibrosis and the current literature regarding efficacy, monitoring, toxicity and concerns of resistance with once daily dosing in this population.
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Pinzaru, Anca Daniela, Cristina Maria Mihai, Tatiana Chisnoiu, Alexandru Cosmin Pantazi, Vasile Valeriu Lupu, Mustafa Ali Kassim Kassim, Ancuta Lupu, et al. "Oxidative Stress Biomarkers in Cystic Fibrosis and Cystic Fibrosis-Related Diabetes in Children: A Literature Review." Biomedicines 11, no. 10 (September 29, 2023): 2671. http://dx.doi.org/10.3390/biomedicines11102671.

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The most common inherited condition that results in death, particularly in those of Caucasian heritage, is cystic fibrosis (CF). Of all the young adults diagnosed with cystic fibrosis, 20% will develop hyperglycemia as a complication, later classified as a disease associated with cystic fibrosis. Impaired insulin secretion and glucose intolerance represent the primary mechanisms associated with diabetes (type 1 or type 2) and cystic fibrosis. Oxidative stress represents the imbalance between oxygen-reactive species and antioxidant defense mechanisms. This pathogenic mechanism is vital in triggering other chronic diseases, including cystic fibrosis-related diabetes. It is essential to understand oxidative stress and the significant impact it has on CFRD. This way, therapies can be individually adjusted and tailored to each patient’s needs. This review aims to understand the connection between CFRD and oxidative stress. As a subsidiary element, we analyzed the effects of glycemic balance on complications and their evolution over time, providing insights into their potential benefits in mitigating oxidative stress-associated complications.
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Kutsev, S. I., V. L. Izhevskaya, and E. I. Kondratyeva. "Targeted therapy for cystic fibrosis." PULMONOLOGIYA 31, no. 2 (April 11, 2021): 226–36. http://dx.doi.org/10.18093/0869-0189-2021-31-2-226-236.

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The basic therapy of cystic fibrosis is currently aimed at slowing down the pathological processes associated with a decrease in the CFTR protein activity (cystic fibrosis transmembrane conductance regulator) in the gastrointestinal tract and the respiratory system. The pancreatic insufficiency is well compensated by replacement therapy with microsphere enzyme preparations and a high-calorie diet rich in proteins and fat. Chronic treatment of cystic fibrosis-related lung disease aims to improve the clearance of the bronchial tree, suppressing chronic bacterial infection and local chronic inflammation. However, no therapy was available to correct the defect in the gene or its product until 2012.The aim was to analyze literature on CFTR modulators, including their efficacy and safety, and assess the potential for developing new modulators to treat cystic fibrosis.Materials. The review included literature data (45 publications) on the use of CFTR modulators and international websites’ data.Results. Since the discovery of the CFTR gene in 1989, more than 2000 mutations or variants of the CFTR gene (hereinafter referred to as genetic variants) have been described. They interfere with the synthesis of the CFTR protein, its transport to the apical membrane of the cell, or disrupt its function as a channel for chloride anions. Although it is currently not possible to completely replace the mutant gene with a normal copy, small molecules have been identified that can modify the mutant CFTR protein and amend its function. The potential therapeutic measures are determined by class of the mutation. In clinical practice, pharmacological modeling of ion transport is currently possible only with the use of CFTR modulators: correctors and potentiators. The review defines these groups of drugs and describes 4 licensed CFTR modulators, including molecules of ivacaftor, lumacaftor, tezacaftor, elexacaftor. The data on the promising emerging next generation modulators and the prospects for the personalized selection of drugs using the assays on intestinal organoids are presented.
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Eid, Nemr S., Lawrence R. Shoemaker, and Tamisha D. Samiec. "Vitamin A in Cystic Fibrosis." Journal of Pediatric Gastroenterology and Nutrition 10, no. 2 (February 1990): 265–69. http://dx.doi.org/10.1002/j.1536-4801.1990.tb09983.x.

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Much has been learned about vitamin A physiology in the last 50 years, yet few changes have been made in therapy. Unfamiliarity with vitamin A bioavailability and distribution may inadvertently result in toxicity. A literature search demonstrates that hypovitaminosis A has rarely been reported in patients with cystic fibrosis, and may manifest very differently in children of different ages. Furthermore, hypervitaminosis A may present with similar features, and can result from correction of deficiency. We report such a case in a 4.5‐month‐old infant, newly diagnosed with cystic fibrosis, who suffered first from vitamin A deficiency and then vitamin A toxicity. A brief review of vitamin A physiology, deficiency, and toxicity is presented.
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Burlutskaya, A. V., V. Yu Brisin, A. V. Statova, O. Yu Zenkina, and J. V. Pisotskaya. "Targeted Therapy for Cystic Fibrosis in Children." Innovative Medicine of Kuban, no. 4 (November 17, 2023): 142–47. http://dx.doi.org/10.35401/2541-9897-2023-8-4-142-147.

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A mutation in the CFTR gene causes the malfunctioning of the CFTR protein that is located on the apical membrane of epithelial cells and functions as a chloride channel. The discovery of CFTR modulators is an advance in cystic fibrosis treatment.Objective: To analyze modern Russian and foreign literature on targeted therapy for cystic fibrosis in children. We also describe the history of cystic fibrosis, evolution in its diagnosis and treatment, and mechanisms behind targeted therapy for cystic fibrosis.
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Rodrigues, Roberta, Carmen S. Gabetta, Karla P. Pedro, Fabio Valdetaro, Maria I. M. Fernandes, Patrícia K. R. Magalhães, José N. Januário, and Léa M. Z. Maciel. "Cystic fibrosis and neonatal screening." Cadernos de Saúde Pública 24, suppl 4 (2008): s475—s484. http://dx.doi.org/10.1590/s0102-311x2008001600002.

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The clinical and diagnostic aspects of cystic fibrosis have been extensively reviewed, with an emphasis on neonatal screening. This systematic literature review involved a search for relevant contributions in the PubMed and SciELO databases. The first references to cystic fibrosis date to the Middle Ages. Cystic fibrosis is the most frequent autosomal recessive hereditary disease among Caucasians (1:2,000 to 3,500). More than 1,000 mutations lead to the disease, the most common being "F508, with 70% prevalence among Canadian, Northern European, and American Caucasians and 23 to 55% prevalence among Brazilians. The basic defect is in chloride ion secretion. Cystic fibrosis screening has long been controversial, and after almost three decades, there are few nationwide programs (most are regional or local). However, the U.S. Centers for Disease Control and Prevention (CDC) has concluded that screening for cystic fibrosis is justified. The lack of a specific screening test and the ethnic heterogeneity of the Brazilian population pose challenges for neonatal screening.
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Loyd, India, Nicole Papac, Jason Hirshburg, Jarad Levin, Jennifer Dannelley, Janislynn Dorris, Jason Stratton, and Nighat Mehdi. "If At First You Don't Succeed, Trikafta Again." Journal of Pediatric Pharmacology and Therapeutics 27, no. 5 (May 1, 2022): 467–69. http://dx.doi.org/10.5863/1551-6776-27.5.467.

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Adverse reactions, including severe cutaneous reactions, to cystic fibrosis transmembrane conductance regulator (CFTR) modulators have been described in the literature. Herein we present a drug eruption in response to elexacaftor/tezcaftor/ivacaftor (brand name, Trikafta) in a 7-year-old male with cystic fibrosis, followed by desensitization and successful continuation. A review of the literature outlining similar cases is provided. Attempting to mitigate and manage drug reactions to CFTR modulators is essential because they represent vital and irreplaceable therapies for individuals with cystic fibrosis (CF).
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Buzzetti, R., D. Salvatore, E. Baldo, M. P. Forneris, V. Lucidi, D. Manunza, I. Marinelli, et al. "An overview of international literature from cystic fibrosis registries: 1. Mortality and survival studies in cystic fibrosis." Journal of Cystic Fibrosis 8, no. 4 (July 2009): 229–37. http://dx.doi.org/10.1016/j.jcf.2009.04.001.

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Dissertations / Theses on the topic "Cystic fibrosis, juvenile literature"

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Hoppe, Margarete. "Perceived Social Support of Children with Severe Chronic Physical Health Conditions : A Systematic Literature Review." Thesis, Hälsohögskolan, Högskolan i Jönköping, HHJ. CHILD, 2016. http://urn.kb.se/resolve?urn=urn:nbn:se:hj:diva-30707.

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Kyriakidou, Despina. "Play integrated in physiotherapyy for children with chronic health conditions : A systematic literature review." Thesis, Högskolan för lärande och kommunikation, Högskolan i Jönköping, HLK, CHILD, 2016. http://urn.kb.se/resolve?urn=urn:nbn:se:hj:diva-30454.

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Background: Play is the child’s natural world. According to psychoanalytical studies, play has an important role in children’s development, and the absence of play during a child’s life could lead to severe pathological implications. Based on this theory and being aware that physiotherapy treatment programs could be long lasting, tiresome and lacking motivation for children, this literature review presents a perspective regarding the integration of play within physiotherapy programs and examines the physical and emotional outcomes during this integration. Aim: To investigate the outcomes of integrating play in physiotherapy for children with chronic health conditions. Method: The research strategy for this review was a thorough search of peer-reviewed articles in the databases CINAHL and AMED which include articles from the fields of allied and complementary medicine, as well as the database Scope Med. Participants were children with chronic health conditions, ranging from 2-18 years old. In the term ‘play’ virtual reality and video game activities were included due to the lack of research. In addition, articles from a previous literature review conducted by the author were also included in the present paper. Results: The focus of researchers on children with CP and the lack of evidence for children with other health conditions, the persistence of physiotherapists to assess mainly physical outcomes and not emotional needs of children, and the measurement tools used for this purpose are presented. Conclusions: For children with chronic health conditions who attend physiotherapy sessions, play could serve as a mediate and an appropriate developmental approach in order to achieve physical and emotional changes. There is a need for physiotherapists to balance physical and emotional needs, and have a more ‘human’ relationship, rather than a ‘bodily’ - strict professional relationship with children. Although the information presented in this review is not considered as sufficient to draw conclusions, it could serve as a first step for researchers to study this integration in greater depth, and to focus on children with conditions other than CP.
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Books on the topic "Cystic fibrosis, juvenile literature"

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Bjorklund, Ruth. Cystic fibrosis. New York: Marshall Cavendish Benchmark, 2009.

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Rosaler, Maxine. Cystic fibrosis. New York: The Rosen Pub. Group, 2007.

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Silverstein, Alvin. Cystic fibrosis. New York: F. Watts, 1994.

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Gold, Susan Dudley. Cystic fibrosis. New York: Crestwood House, 2000.

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Giddings, Sharon. Cystic fibrosis. New York: Chelsea House, 2009.

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McDaniel, Lurlene. A time to die. New York: Bantam Books, 1992.

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Napoli, Donna Jo. Breath. New York: Atheneum Books for Young Readers, 2003.

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Napoli, Donna Jo. Breath. Waterville, Me: Thorndike Press, 2005.

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Copyright Paperback Collection (Library of Congress), ed. Breath. New York: Simon Pulse, 2005.

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Curtis, Vanessa. The baking life of Amelie Day. London: Curious Fox, 2014.

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Book chapters on the topic "Cystic fibrosis, juvenile literature"

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Clark, William R. "Cystic Fibrosis." In The New Healers, 26–37. Oxford University PressNew York, NY, 1997. http://dx.doi.org/10.1093/oso/9780195117301.003.0002.

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Abstract Cystic fibrosis (CF) is a terrible disease because it strikes children and because there is no cure. It is caused by a defect in one of Mendel’s “atoms of heredity,” a tiny mistake in a single gene. CF has obviously been a disease in the human population for a very long time; people suffering from symptoms associated with CF (such as excessively salty sweat preceding or accompanying a serious illness) have been described in the medical literature for at least several hundred years. But a precise clinical definition of CF did not emerge until the late 1930s. Dr. Dorothy Andersen, a pathologist at Columbia University, had become intrigued by several cases of newborns, infants, and children she had seen at autopsy that seemed to share certain symptoms. Comparing these with other cases gleaned from the medical literature, she felt she could fit them together into a common syndrome, implying the possibility of a single underlying cause.
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Wat, Dennis. "Respiratory Virus in Cystic Fibrosis — A Review of the Literature." In Cystic Fibrosis in the Light of New Research. InTech, 2015. http://dx.doi.org/10.5772/60905.

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Theocharous, Katerina, Bernadette Prentice, Charles F. Verge, Adam Jaffé, and Shihab Hameed. "Detection and Management of Early Glucose Abnormalities in Cystic Fibrosis." In Cystic Fibrosis - Facts, Management and Advances. IntechOpen, 2021. http://dx.doi.org/10.5772/intechopen.92847.

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With advances in technology, it is now possible to detect the emergence of glucose abnormalities in cystic fibrosis with improved sensitivity, and from a very early age. These abnormalities are increasingly recognized as predictors of clinical decline, raising the possibility that early intervention may slow or prevent this deterioration. In this chapter, we will review the available literature on methods of detecting glucose abnormalities in cystic fibrosis (random and fasting glucose, HbA1c, oral glucose tolerance testing, and continuous glucose monitoring), and detail their advantages and possible limitations in the interpretation of glycemic data. We will also discuss treatment outcomes of early intervention, prior to the diagnosis of diabetes as currently defined.
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Peterson, Karin S. "Musculoskeletal and autoimmune manifestations of non-rheumatic disorders." In Arthritis in Children and Adolescents, 155–78. Oxford University PressNew York, NY, 2006. http://dx.doi.org/10.1093/oso/9780192632920.003.0010.

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Abstract The aim of this chapter is to provide a comprehensive discussion of disorders that may occasionally manifest with secondary musculoskeletal complaints and mimic Juvenile Idiopathic Arthritis. Musculoskeletal pain, arthritis or autoimmune phenomena are some- times encountered in a child already diagnosed with another disorder, such as diabetes, cystic fibrosis, or an immunodeficiency. In most instances this is not the occurrence of a second disease but a symptom related to the underlying disorder or, occasionally, its treatment.
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Conference papers on the topic "Cystic fibrosis, juvenile literature"

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Gomes Vieira Carvalho, Thainá, Joyce Mothé de Souza, Elisa Haddad Pessanha Rangel, Caio Gomes Muniz, Julia Maria Maia de Azevedo, and Luciano Matos Chicayban. "Bronchial hygiene technique in patients with cystic fibrosis." In 7th International Congress on Scientific Knowledge. Biológicas & Saúde, 2021. http://dx.doi.org/10.25242/8868113820212402.

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Cystic Fibrosis is characterized by excess pulmonary secretions that cause recurrent respiratory infections, with consequent deterioration of gas exchange. Bronchial hygiene techniques aim to mobilize secretions from the peripheral airways so that they can be eliminated by coughing or tracheal aspiration. To identify the effects of different bronchial hygiene techniques on improving lung function in patients with Cystic Fibrosis. Through a systematic review of the literature, randomized controlled trials (RCTs) published between 2007 and 2021 were selected, according to the highest score in the PEDro score. The search involved the PEDro and PubMed databases, using the following keywords: bronchial hygiene. Six ECR`s were included. One study performedthe techniques during anesthesia and observed increased resistance and reduced compliance. Regarding FEV1, 3 RCTs with hospitalized patients showed improvement in lung function, regardless of the technique used. In outpatients, there was no improvement. Regarding secretion weight, the cough machine produced more secretion than autogenous drainage, as well as a drop in saturation after the 2-min walk test, and increased FEV1. Bronchial hygiene techniques in patients with cystic fibrosis have been shown to be effective in removing mucus, with consequent improvement in lung function and aerobic fitness.
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Vagg, Tamara, Barry Plant, Joe Eustace, and Sabin Tabirca. "A REVIEW OF EDUCATIONAL INTERVENTIONS FOR ADULTS WITH CYSTIC FIBROSIS." In eLSE 2017. Carol I National Defence University Publishing House, 2017. http://dx.doi.org/10.12753/2066-026x-17-117.

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Cystic Fibrosis is the most common life limiting genetic disease affecting Caucasians. The life expectancy of this cohort has increased and is predicted to reach retirement age. As most CF patients are diagnosed from birth, they undergo educational and management programs throughout their lives. However numerous studies have identified various knowledge gaps. This paper presents a scoping literature review of the available educational interventions for adult CF patients. This review follows the framework as outlined by Arksey et al. [9]. A search string is entered into four databases and the resulting literature is downloaded into the academic reference manager software, Mendeley version 1.16.1. The manuscripts are then subjected to title, abstract, and introduction refinement. The remaining manuscripts are then reviewed under the following headings: 1) Aims/Objective, 2) Patient Demographic, 3) Educational Focus, 4) Research Type Conducted, 5) Methodologies, 6) Results, and 7) Author Noted Limitations. Of the 3649 manuscripts, only eight remained after the refinement stages. A significant amount of the papers that were excluded focused on CF pediatric patients only. Of the remaining papers, four manuscripts focused solely on CF adults. Seven of the papers identified a range of issues with CF education, including anxiety, knowledge gaps, and a lack of educational teams. Five papers introduced an educational intervention, only one was for CF adults only. Two of the interventions were ICT based. Of the manuscripts, one intervention that targeted both children and adults was reported as being implemented into the hospital services long term. From the final papers reviewed, it is apparent that there appears to be an issue with adult CF education and knowledge gaps. With CF survival rates rising and a new aging CF population emerging, there is a need for educational programs and interventions to meet the changing needs of the CF adult population.
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Tayyeb, M., A. Abu Shanab, M. Saad Anwar, D. A. Aquino, P. Amog, F. Khalid, and D. Du. "Constrictive Pericarditis in an Adult With Cystic Fibrosis: A Case Report and the Literature Review." In American Thoracic Society 2024 International Conference, May 17-22, 2024 - San Diego, CA. American Thoracic Society, 2024. http://dx.doi.org/10.1164/ajrccm-conference.2024.209.1_meetingabstracts.a5853.

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Gangadharan, A., and V. Patel. "G415 Literature review of diagnostic tests used for the assessment of glycaemic abnormalities or CFRD in children with cystic fibrosis (CF)." In Royal College of Paediatrics and Child Health, Abstracts of the RCPCH Conference–Online, 25 September 2020–13 November 2020. BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health, 2020. http://dx.doi.org/10.1136/archdischild-2020-rcpch.357.

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Pereira, Mônica Corso, Jessica de Campos Medeiros, and Àdria Cristina Da Silva. "Exacerbations in non- cystic fibrosis bronchiectasis (NCFB): is there a difference between self-reported events and those defined by the criteria in the literature?" In ERS International Congress 2021 abstracts. European Respiratory Society, 2021. http://dx.doi.org/10.1183/13993003.congress-2021.pa2015.

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Loshkova, Elena, Elena Kondratyeva, Nuriniso Odinaeva, Leonid Klimov, Natalia Ilyenkova, Elena Zhekaite, Natalia Geppe, et al. "PP-057 The state of vitamin D supply and the influence of VDR gene polymorphisms on the course of cystic fibrosis, asthma, juvenile idiopathic arthritis in children of the Russian federation." In 11th Europaediatrics Congress, Antalya, Türkiye, 17 – 21 April 2024. BMJ Publishing Group Ltd, 2024. http://dx.doi.org/10.1136/bmjpo-2024-epac.201.

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