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  1. Book ; Online ; E-Book: Handbook of cystic fibrosis

    Filbrun, Amy G. / Lahiri, Thomas / Ren, Clement L.

    2016  

    Author's details Amy G. Filbrun, Thomas Lahiri, Clement L. Ren
    Keywords CFTR mutations ; Cystic Fibrosis ; Cystic Fibrosis manifestations ; Pulmonary ; Respiratory medicine
    Language English
    Size 1 Online-Ressource (xii, 99 Seiten)
    Publisher Adis
    Publishing place Cham
    Publishing country Germany
    Document type Book ; Online ; E-Book
    Note Lizenzpflichtig
    Remark Zugriff für angemeldete ZB MED-Nutzerinnen und -Nutzer
    HBZ-ID HT019116461
    ISBN 978-3-319-32504-0 ; 9783319325026 ; 3-319-32504-3 ; 3319325027
    DOI 10.1007/978-3-319-32504-0
    Database ZB MED Catalogue: Medicine, Health, Nutrition, Environment, Agriculture

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  2. Article ; Online: Profound electrolyte and acid-base disturbances in an infant with cystic fibrosis.

    Lahiri, Thomas / VanNostrand, Jessica / Faricy, Lauren / Twichell, Sarah

    Pediatric pulmonology

    2024  Volume 59, Issue 5, Page(s) 1466–1468

    MeSH term(s) Humans ; Cystic Fibrosis/complications ; Infant ; Acid-Base Imbalance/etiology ; Water-Electrolyte Imbalance/etiology ; Male ; Female
    Language English
    Publishing date 2024-02-15
    Publishing country United States
    Document type Case Reports ; Letter ; Journal Article
    ZDB-ID 632784-9
    ISSN 1099-0496 ; 8755-6863
    ISSN (online) 1099-0496
    ISSN 8755-6863
    DOI 10.1002/ppul.26924
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Recent advances in the early treatment of cystic fibrosis: Bridging the gap to highly effective modulator therapy.

    Lahiri, Thomas / Sullivan, Jillian S

    Pediatric pulmonology

    2021  Volume 57 Suppl 1, Page(s) S60–S74

    Abstract: Highly effective modulator therapy (HEMT) for cystic fibrosis (CF) has been touted as one of the greatest advances to date in CF care. As these therapies are now available for many older children and adults with CF, marked improvement of their ... ...

    Abstract Highly effective modulator therapy (HEMT) for cystic fibrosis (CF) has been touted as one of the greatest advances to date in CF care. As these therapies are now available for many older children and adults with CF, marked improvement of their nutritional status, pulmonary and gastrointestinal symptoms has been observed. However, most infants and younger children are not current candidates for HEMT due to age and/or cystic fibrosis transmembrane conductance regulator (CFTR) mutation. For these young children, it is essential to provide rigorous monitoring and care to avoid potential disease sequelae while awaiting HEMT availability. The following article highlights recent advances in the care of infants and young children with CF with regard to surveillance and treatment of nutritional, pulmonary, and gastrointestinal disorders. Recent clinical trials in this population are also reviewed.
    MeSH term(s) Adolescent ; Adult ; Aminophenols/therapeutic use ; Child ; Child, Preschool ; Cystic Fibrosis/complications ; Cystic Fibrosis/drug therapy ; Cystic Fibrosis/genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/therapeutic use ; Gastrointestinal Diseases ; Humans ; Infant ; Mutation ; Nutritional Status ; Quinolones/therapeutic use
    Chemical Substances Aminophenols ; Quinolones ; Cystic Fibrosis Transmembrane Conductance Regulator (126880-72-6)
    Language English
    Publishing date 2021-09-09
    Publishing country United States
    Document type Journal Article
    ZDB-ID 632784-9
    ISSN 1099-0496 ; 8755-6863
    ISSN (online) 1099-0496
    ISSN 8755-6863
    DOI 10.1002/ppul.25660
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: The utility of the lipid-laden macrophage index for the evaluation of aspiration in children.

    Lahiri, Thomas

    Cancer cytopathology

    2014  Volume 122, Issue 3, Page(s) 161–162

    MeSH term(s) Bronchoalveolar Lavage Fluid ; Bronchoscopy ; Cell Count/methods ; Cell Count/utilization ; Child ; Child, Preschool ; Gastroesophageal Reflux/diagnosis ; Gastroesophageal Reflux/pathology ; Histocytological Preparation Techniques ; Humans ; Infant ; Infant, Newborn ; Lipids/analysis ; Macrophages, Alveolar/chemistry ; Macrophages, Alveolar/pathology ; Recurrence ; Respiratory Aspiration/diagnosis ; Respiratory Aspiration/pathology
    Chemical Substances Lipids
    Language English
    Publishing date 2014-03
    Publishing country United States
    Document type Evaluation Studies ; Journal Article
    ZDB-ID 2594979-2
    ISSN 1934-6638 ; 1934-662X
    ISSN (online) 1934-6638
    ISSN 1934-662X
    DOI 10.1002/cncy.21365
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Delayed Presentation of Meconium Ileus in an Infant With Cystic Fibrosis.

    Lahiri, Thomas / Sullivan, Jillian S / Sartorelli, Kennith H / Murphy, James J

    Pediatrics

    2020  Volume 146, Issue 4

    Abstract: Although infants with meconium ileus usually present with apparent symptoms shortly after birth, the diagnosis of meconium ileus and cystic fibrosis (CF) may be delayed, awaiting newborn screening (NBS) results. We present the case of an 11-day-old term ... ...

    Abstract Although infants with meconium ileus usually present with apparent symptoms shortly after birth, the diagnosis of meconium ileus and cystic fibrosis (CF) may be delayed, awaiting newborn screening (NBS) results. We present the case of an 11-day-old term girl with delayed passage of meconium at 48 hours who had 2 subsequent small meconium stools over the following week. There was a normal feeding history and no signs of abdominal distension or distress. She then presented with an acute abdomen, decompensated shock, bowel perforation, and peritonitis, requiring multiple intestinal surgeries. Her NBS for CF was positive, and CF was ultimately confirmed with mutation analysis. Her course was complicated by prolonged parenteral feedings and mechanical ventilation via tracheostomy. The infant was managed with soy oil, medium chain triglycerides, olive oil, fish oil lipids and experienced only transaminitis without cholestasis and no chronic liver sequelae, with subsequent normalization of her transaminases without treatment. Because her only symptom was decreased stool output and NBS results were unavailable, the CF diagnosis was delayed until she presented in extremis. Delayed meconium passage and decreased stool output during the first week of life should lead to suspicion and additional evaluation for CF while awaiting NBS results. Careful monitoring is indicated to prevent serious, life-threatening complications. The use of soy oil, medium chain triglycerides, olive oil, fish oil lipids for infants requiring prolonged parenteral nutrition may also be considered proactively to prevent cholestasis, particularly for high risk groups.
    MeSH term(s) Cholestasis/prevention & control ; Cystic Fibrosis/diagnosis ; Delayed Diagnosis ; Female ; Fish Oils/therapeutic use ; Humans ; Infant, Newborn ; Lipids/administration & dosage ; Meconium Ileus/diagnosis ; Meconium Ileus/therapy ; Olive Oil/therapeutic use ; Parenteral Nutrition ; Soybean Oil/therapeutic use ; Triglycerides/administration & dosage
    Chemical Substances Fish Oils ; Lipids ; Olive Oil ; Triglycerides ; Soybean Oil (8001-22-7)
    Language English
    Publishing date 2020-09-22
    Publishing country United States
    Document type Case Reports ; Journal Article
    ZDB-ID 207677-9
    ISSN 1098-4275 ; 0031-4005
    ISSN (online) 1098-4275
    ISSN 0031-4005
    DOI 10.1542/peds.2019-3717
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: The history of workforce concerns in pediatric pulmonary Medicine.

    Oermann, Christopher M / Lahiri, Thomas / Peterson-Carmichael, Stacey L / Weiss, Pnina

    Pediatric pulmonology

    2020  Volume 58, Issue 3, Page(s) 683–689

    Abstract: Children are affected by a broad spectrum of acute and chronic respiratory disorders. The number of children with respiratory disease is increasing, as are the complexity of disease pathophysiology and the management demands on pediatric pulmonologists. ... ...

    Abstract Children are affected by a broad spectrum of acute and chronic respiratory disorders. The number of children with respiratory disease is increasing, as are the complexity of disease pathophysiology and the management demands on pediatric pulmonologists. Despite slowly increasing numbers of board-certified pediatric pulmonologists, large areas of the country are underserved and there is a perception of an impending workforce crisis. There are multiple reasons for these concerns. A joint effort between the Pediatric Pulmonology Division Directors Association and Pediatric Pulmonary Training Directors Association was undertaken to address these issues.
    MeSH term(s) Humans ; Child ; United States ; Pulmonary Medicine/education ; Workforce ; Certification ; Fellowships and Scholarships
    Language English
    Publishing date 2020-10-12
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 632784-9
    ISSN 1099-0496 ; 8755-6863
    ISSN (online) 1099-0496
    ISSN 8755-6863
    DOI 10.1002/ppul.25094
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article: Approaches to the treatment of initial Pseudomonas aeruginosa infection in children who have cystic fibrosis.

    Lahiri, Thomas

    Clinics in chest medicine

    2007  Volume 28, Issue 2, Page(s) 307–318

    Abstract: Pseudomonas aeruginosa remains an important cause of pulmonary disease in patients who have cystic fibrosis. The development of antimicrobial therapy directed against this organism has resulted in the preservation of lung function and improved longevity. ...

    Abstract Pseudomonas aeruginosa remains an important cause of pulmonary disease in patients who have cystic fibrosis. The development of antimicrobial therapy directed against this organism has resulted in the preservation of lung function and improved longevity. Efficacy has been demonstrated with agents administered via parenteral, inhaled, and oral routes. The optimal antibiotic regimen remains unclear. There is an active effort to use randomized, controlled clinical trials to rigorously test effective antibiotic for the eradication of P aeruginosa in young children or at least to delay the establishment of chronic infection.
    MeSH term(s) Anti-Bacterial Agents/therapeutic use ; Child ; Cystic Fibrosis/complications ; Cystic Fibrosis/microbiology ; Humans ; Pseudomonas Infections/drug therapy ; Pseudomonas Infections/prevention & control ; Pseudomonas aeruginosa/isolation & purification
    Chemical Substances Anti-Bacterial Agents
    Language English
    Publishing date 2007-06
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 447455-7
    ISSN 1557-8216 ; 0272-5231
    ISSN (online) 1557-8216
    ISSN 0272-5231
    DOI 10.1016/j.ccm.2007.02.003
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Online: A statement on the current status and future needs of the pediatric pulmonology workforce: Pipeline Workgroup.

    Nelson, Benjamin A / Boyer, Debra / Lahiri, Thomas / Oermann, Christopher M / Rama, Jennifer A

    Pediatric pulmonology

    2020  Volume 58, Issue 3, Page(s) 690–696

    Abstract: There is growing concern that current trends in pediatric pulmonology will lead to a workforce shortage resulting in patients having difficulty accessing subspecialty care. As part of the Pediatric Pulmonology Division Directors Association and Pediatric ...

    Abstract There is growing concern that current trends in pediatric pulmonology will lead to a workforce shortage resulting in patients having difficulty accessing subspecialty care. As part of the Pediatric Pulmonology Division Directors Association and Pediatric Pulmonary Training Directors Association Workforce Summit, we examined factors affecting the recruitment of learners into pediatric pulmonary fellowship training (PPFT) programs. The goal of our workgroup was to describe these issues and develop a plan to increase the pipeline of learners who ultimately pursue PPFT. Specifically, we summarize factors that impact decisions to undertake PPFT, describe existing initiatives to enhance recruitment, and propose future strategies to increase early career learner interest.
    MeSH term(s) Humans ; Child ; Pulmonary Medicine/education ; Workforce ; Motivation ; Fellowships and Scholarships
    Language English
    Publishing date 2020-11-03
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 632784-9
    ISSN 1099-0496 ; 8755-6863
    ISSN (online) 1099-0496
    ISSN 8755-6863
    DOI 10.1002/ppul.25139
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  9. Book: Handbook of cystic fibrosis

    Filbrun, Amy G / Lahiri, Thomas / Ren, Clement L

    2016  

    Author's details Amy G. Filbrun, Thomas Lahiri, Clement L. Ren
    MeSH term(s) Cystic Fibrosis
    Language English
    Size xii, 99 pages :, illustrations
    Document type Book
    ISBN 9783319325026 ; 9783319325040 ; 3319325027 ; 3319325043
    Database Catalogue of the US National Library of Medicine (NLM)

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  10. Article ; Online: Complications and Practice Variation in the Use of Peripherally Inserted Central Venous Catheters in People With Cystic Fibrosis: The Prospective Study of Peripherally Inserted Venous Catheters in People With Cystic Fibrosis Study.

    Gifford, Alex H / Hinton, Alexandra C / Jia, Shijing / Nasr, Samya Z / Mermis, Joel D / Lahiri, Thomas / Zemanick, Edith T / Teneback, Charlotte C / Flume, Patrick A / DiMango, Emily A / Sadeghi, Hossein / Polineni, Deepika / Dezube, Rebecca H / West, Natalie E / Dasenbrook, Elliott C / Lucas, F Lee / Zuckerman, Jonathan B

    Chest

    2023  Volume 164, Issue 3, Page(s) 614–624

    Abstract: Background: Peripherally inserted central catheters (PICCs) are used commonly to administer antibiotics to people with cystic fibrosis (CF), but their use can be complicated by venous thrombosis and catheter occlusion.: Research question: Which ... ...

    Abstract Background: Peripherally inserted central catheters (PICCs) are used commonly to administer antibiotics to people with cystic fibrosis (CF), but their use can be complicated by venous thrombosis and catheter occlusion.
    Research question: Which participant-, catheter-, and catheter management-level attributes are associated with increased risk of complications of PICCs among people with CF?
    Study design and methods: This was a prospective observational study of adults and children with CF who received PICCs at 10 CF care centers in the United States. The primary end point was defined as occlusion of the catheter resulting in unplanned removal, symptomatic venous thrombosis in the extremity containing the catheter, or both. Three categories of composite secondary outcomes were identified: difficult line placement, local soft tissue or skin reactions, and catheter malfunction. Data specific to the participant, catheter placement, and catheter management were collected in a centralized database. Risk factors for primary and secondary outcomes were analyzed by multivariate logistic regression.
    Results: Between June 2018 and July 2021, 157 adults and 103 children older than 6 years with CF had 375 PICCs placed. Patients underwent 4,828 catheter-days of observation. Of the 375 PICCs, 334 (89%) were ≤ 4.5 F, 342 (91%) were single lumen, and 366 (98%) were placed using ultrasound guidance. The primary outcome occurred in 15 PICCs for an event rate of 3.11 per 1,000 catheter-days. No cases of catheter-related bloodstream infection occurred. Other secondary outcomes developed in 147 of 375 catheters (39%). Despite evidence of practice variation, no risk factors for the primary outcome and few risk factors for secondary outcomes were identified.
    Interpretation: This study affirmed the safety of contemporary approaches to inserting and using PICCs in people with CF. Given the low rate of complications in this study, observations may reflect a widespread shift to selecting smaller-diameter PICCs and using ultrasound to guide their placement.
    MeSH term(s) Adult ; Child ; Humans ; Prospective Studies ; Catheterization, Central Venous/adverse effects ; Catheterization, Central Venous/methods ; Central Venous Catheters ; Cystic Fibrosis/complications ; Cystic Fibrosis/therapy ; Retrospective Studies ; Catheterization, Peripheral/adverse effects ; Venous Thrombosis/etiology ; Catheter-Related Infections/epidemiology ; Catheter-Related Infections/etiology ; Catheters, Indwelling
    Language English
    Publishing date 2023-04-03
    Publishing country United States
    Document type Observational Study ; Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
    ZDB-ID 1032552-9
    ISSN 1931-3543 ; 0012-3692
    ISSN (online) 1931-3543
    ISSN 0012-3692
    DOI 10.1016/j.chest.2023.03.043
    Database MEDical Literature Analysis and Retrieval System OnLINE

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