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  1. Article ; Online: Management of Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis with Intracapsular Tonsillectomy.

    Ezeh, Uche C / Kahn, Philip J / April, Max M

    The Laryngoscope

    2023  Volume 134, Issue 4, Page(s) 1967–1969

    Abstract: Objective: This study aimed to present 2 children clinically diagnosed with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome and treated with intracapsular tonsillectomy with adenoidectomy (ITA).: Methods: We conducted ... ...

    Abstract Objective: This study aimed to present 2 children clinically diagnosed with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome and treated with intracapsular tonsillectomy with adenoidectomy (ITA).
    Methods: We conducted a retrospective analysis of 2 children who were referred for an otolaryngology consultation between 2019 and 2022 for surgical treatment of PFAPA syndrome. Both patients had symptoms strongly suggestive of PFAPA and were at risk for total tonsillectomy (TT) complications. ITA was performed using a microdebrider. Both patients were followed up postoperatively to assess for symptomatic resolution and complications.
    Results: Two children exhibited recurrent febrile episodes prior to ITA. The procedure was efficacious in both patients, with neither experiencing postoperative complications or recurring PFAPA symptoms for over 1 year after surgery.
    Conclusion: Our study reported on the use of ITA as a surgical treatment option for PFAPA. We showed that ITA eliminated febrile attacks and was safely performed without postoperative complications in 2 pediatric patients after 1-year follow-up. Future studies involving larger cohorts of PFAPA patients and lengthier follow-ups will need to be conducted to further evaluate ITA as a surgical option. Laryngoscope, 134:1967-1969, 2024.
    MeSH term(s) Child ; Humans ; Tonsillectomy/methods ; Stomatitis, Aphthous/surgery ; Retrospective Studies ; Pharyngitis/surgery ; Lymphadenitis/diagnosis ; Lymphadenitis/surgery ; Lymphadenopathy ; Amyloidosis/surgery ; Fever/surgery ; Fever/complications ; Syndrome ; Postoperative Complications/surgery
    Language English
    Publishing date 2023-08-19
    Publishing country United States
    Document type Case Reports
    ZDB-ID 80180-x
    ISSN 1531-4995 ; 0023-852X
    ISSN (online) 1531-4995
    ISSN 0023-852X
    DOI 10.1002/lary.30969
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Lupus anti-coagulant hypoprothrombinemia syndrome across different ages: a case report and review of the literature.

    Chumsky, Jessica / Kahn, Philip J / Carroll, William L / Pierce, Kristyn A / Hillier, Kirsty

    Clinical rheumatology

    2023  Volume 42, Issue 8, Page(s) 2231–2236

    Abstract: Lupus anti-coagulant hypoprothrombinemia syndrome (LAHPS) is a rare condition that can be difficult to treat. It increases the risk of thrombosis and bleeding due to the presence of lupus anti-coagulant and factor II deficiency, respectively. There are a ...

    Abstract Lupus anti-coagulant hypoprothrombinemia syndrome (LAHPS) is a rare condition that can be difficult to treat. It increases the risk of thrombosis and bleeding due to the presence of lupus anti-coagulant and factor II deficiency, respectively. There are a limited number of cases described in the literature. Herein we describe a case of LAHPS with bleeding symptoms as a first clinical manifestation of systemic lupus erythematosus (SLE) in an 8-year-old female. She has had multiple recurrences of her bleeding symptoms, requiring treatment with steroids, cyclophosphamide, mycophenolate mofetil, and rituximab. Her course was later complicated by development of arthritis and lupus nephritis. Her complicated course provides a new perspective on the clinical course and treatment of LAHPS. We also present a comprehensive literature review which demonstrates the difficulty in treating patients with LAHPS with underlying SLE and the variability of the clinical course and management of LAHPS depending on the age at presentation.
    MeSH term(s) Humans ; Female ; Child ; Hypoprothrombinemias/complications ; Hypoprothrombinemias/diagnosis ; Hypoprothrombinemias/drug therapy ; Lupus Coagulation Inhibitor ; Lupus Erythematosus, Systemic/complications ; Lupus Erythematosus, Systemic/diagnosis ; Lupus Erythematosus, Systemic/drug therapy ; Cyclophosphamide/therapeutic use ; Hemorrhage/complications ; Disease Progression
    Chemical Substances Lupus Coagulation Inhibitor ; Cyclophosphamide (8N3DW7272P)
    Language English
    Publishing date 2023-05-08
    Publishing country Germany
    Document type Case Reports ; Journal Article ; Review
    ZDB-ID 604755-5
    ISSN 1434-9949 ; 0770-3198
    ISSN (online) 1434-9949
    ISSN 0770-3198
    DOI 10.1007/s10067-023-06619-3
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Synchronous disease onset and flares in siblings with PFAPA.

    Dammeyer, Kristen L / Schneider, Amanda / April, Max M / Kahn, Philip J

    Pediatric rheumatology online journal

    2022  Volume 20, Issue 1, Page(s) 87

    Abstract: Background: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is a clinical syndrome of unclear etiology. PFAPA has generally been considered a non-hereditary fever syndrome; however, this has been called into question with ...

    Abstract Background: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is a clinical syndrome of unclear etiology. PFAPA has generally been considered a non-hereditary fever syndrome; however, this has been called into question with recent reports of family clustering. Few reports have been published describing siblings with PFAPA. To our knowledge, this is the first report of siblings with near simultaneous onset of disease followed by synchronous disease flares.
    Case presentation: We describe the case of near simultaneous onset of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis in siblings followed by synchronous disease flares of clear frequency and nearly identical character. Flares were characterized predominantly by fever, aphthous ulceration, cervical lymphadenitis, and the absence of infection. The fever episodes demonstrated a robust response to glucocorticoids and recurred in the same staggered manner every four weeks, with complete absence of symptoms and normal growth and development between episodes. Nine months after onset, the older sibling, a 5-year-old female, underwent tonsillectomy resulting in dramatic resolution of episodes. At the same time, her 2-year-old sister experienced resolution of her fever episodes, though she did not undergo tonsillectomy herself.
    Conclusion: This is an unusual case of simultaneous onset PFAPA followed by synchronous disease flares. PFAPA is an uncommon clinical syndrome, and it is rarely diagnosed in siblings. The etiology of PFAPA remains unclear. Though the disease is classically considered sporadic, there is a growing body of evidence to suggest that PFAPA may be heritable.
    MeSH term(s) Child, Preschool ; Female ; Fever/diagnosis ; Fever/etiology ; Humans ; Lymphadenitis/diagnosis ; Pharyngitis/diagnosis ; Siblings ; Stomatitis, Aphthous/diagnosis ; Syndrome ; Tonsillectomy/methods
    Language English
    Publishing date 2022-10-05
    Publishing country England
    Document type Case Reports ; Journal Article
    ZDB-ID 2279468-2
    ISSN 1546-0096 ; 1546-0096
    ISSN (online) 1546-0096
    ISSN 1546-0096
    DOI 10.1186/s12969-022-00744-0
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: Multisystem Inflammatory Syndrome in Children.

    Shust, Gail F / Soma, Vijaya L / Kahn, Philip / Ratner, Adam J

    Pediatrics in review

    2021  Volume 42, Issue 7, Page(s) 399–401

    MeSH term(s) COVID-19/complications ; Child ; Humans ; SARS-CoV-2 ; Syndrome ; Systemic Inflammatory Response Syndrome
    Language English
    Publishing date 2021-05-20
    Publishing country United States
    Document type Editorial
    ZDB-ID 774515-1
    ISSN 1526-3347 ; 0191-9601
    ISSN (online) 1526-3347
    ISSN 0191-9601
    DOI 10.1542/pir.2020-004770
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Anifrolumab for Adolescent Discoid Lupus Erythematosus.

    Shaw, Katharina S / Rajeh, Ahmad / Le, Todd / Kahn, Philip J / Oza, Vikash S / Arkin, Lisa M / Vleugels, Ruth Ann

    JAMA network open

    2023  Volume 6, Issue 10, Page(s) e2338200

    MeSH term(s) Humans ; Adolescent ; Antibodies, Monoclonal, Humanized/therapeutic use ; Lupus Erythematosus, Discoid/drug therapy
    Chemical Substances anifrolumab (38RL9AE51Q) ; Antibodies, Monoclonal, Humanized
    Language English
    Publishing date 2023-10-02
    Publishing country United States
    Document type Letter
    ISSN 2574-3805
    ISSN (online) 2574-3805
    DOI 10.1001/jamanetworkopen.2023.38200
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Juvenile idiopathic arthritis: an update for the clinician.

    Kahn, Philip

    Bulletin of the NYU hospital for joint diseases

    2012  Volume 70, Issue 3, Page(s) 152–166

    Abstract: Juvenile idiopathic arthritis (JIA) comprises a collection of all forms of chronic arthritis in childhood with no apparent cause. JIA is the most common rheumatic disease in children, and may result in significant pain, joint deformity, and growth ... ...

    Abstract Juvenile idiopathic arthritis (JIA) comprises a collection of all forms of chronic arthritis in childhood with no apparent cause. JIA is the most common rheumatic disease in children, and may result in significant pain, joint deformity, and growth impairment, with persistence of active arthritis into adulthood. Prior to the mid 1990s, the therapeutic armamentarium for JIA was more limited, utilizing non- specific agents, many with significant adverse effects. With the relatively recent use of biologics, one can provide more target-specific therapy, which may be better tolerated. Through continued translational research and clinical trials, one better understands the biology mediating disease, with the hope of offering safer, more effective medicine, and potential cure. This review will outline the clinical features of JIA, as well as provide the latest updates in current and future pharmacotherapy.
    MeSH term(s) Adolescent ; Animals ; Antirheumatic Agents/adverse effects ; Antirheumatic Agents/therapeutic use ; Arthritis, Juvenile/diagnosis ; Arthritis, Juvenile/drug therapy ; Arthritis, Juvenile/epidemiology ; Arthritis, Juvenile/immunology ; Biological Products/adverse effects ; Biological Products/therapeutic use ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Predictive Value of Tests ; Remission Induction ; Treatment Outcome
    Chemical Substances Antirheumatic Agents ; Biological Products
    Language English
    Publishing date 2012
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 390411-8
    ISSN 1936-9727 ; 2328-5273 ; 1936-9719 ; 0018-5647 ; 0883-9344 ; 2328-4633
    ISSN (online) 1936-9727 ; 2328-5273
    ISSN 1936-9719 ; 0018-5647 ; 0883-9344 ; 2328-4633
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article ; Online: Neutrophilic dermatosis in a patient with an IKZF1 variant and a review of monogenic autoinflammatory disorders presenting with neutrophilic dermatoses.

    Guirguis, Justina / Iosim, Sonia / Jones, Derek / Likhite, Maryel / Chen, Fei / Kesserwan, Chimene / Gindin, Tatyana / Kahn, Philip J / Beck, David / Oza, Vikash S / Hillier, Kirsty

    Pediatric dermatology

    2024  

    Abstract: Monogenic diseases of immune dysregulation should be considered in the evaluation of children presenting with recurrent neutrophilic dermatoses in association with systemic signs of inflammation, autoimmune disease, hematologic abnormalities, and ... ...

    Abstract Monogenic diseases of immune dysregulation should be considered in the evaluation of children presenting with recurrent neutrophilic dermatoses in association with systemic signs of inflammation, autoimmune disease, hematologic abnormalities, and opportunistic or recurrent infections. We report the case of a 2-year-old boy presenting with a neutrophilic dermatosis, found to have a novel likely pathogenic germline variant of the IKAROS Family Zinc Finger 1 (IKZF1) gene; the mutation likely results in a loss of function dimerization defective protein based on reports and studies of similar variants. IKZF1 variants could potentially lead to aberrant neutrophil chemotaxis and development of neutrophilic dermatoses. Long-term surveillance is required to monitor the development of hematologic malignancy, autoimmunity, immunodeficiency, and infection in patients with pathogenic IKZF1 germline variants.
    Language English
    Publishing date 2024-02-27
    Publishing country United States
    Document type Case Reports
    ZDB-ID 605539-4
    ISSN 1525-1470 ; 0736-8046
    ISSN (online) 1525-1470
    ISSN 0736-8046
    DOI 10.1111/pde.15566
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Online: A letter to the editor: The effects of alcohol use on brain glutamate in first episode psychosis.

    King, Bridget / Kempton, Matthew J / Broberg, Brian V / Merritt, Kate / Barker, Gareth J / Lythgoe, David J / Perez-Iglesias, Rocio / Baandrup, Lone / Düring, Signe W / Stone, James M / Rostrup, Egill / Sommer, Iris E / Glenthøj, Birte / Kahn, René S / Dazzan, Paola / McGuire, Philip K / Egerton, Alice

    Schizophrenia research

    2024  Volume 266, Page(s) 234–236

    MeSH term(s) Humans ; Glutamic Acid ; Psychotic Disorders ; Brain/diagnostic imaging ; Proton Magnetic Resonance Spectroscopy
    Chemical Substances Glutamic Acid (3KX376GY7L)
    Language English
    Publishing date 2024-03-01
    Publishing country Netherlands
    Document type Letter
    ZDB-ID 639422-x
    ISSN 1573-2509 ; 0920-9964
    ISSN (online) 1573-2509
    ISSN 0920-9964
    DOI 10.1016/j.schres.2024.02.013
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  9. Article ; Online: Symptom resolution following a repeat concussion within the same athlete.

    Tang, Alan R / Williams, Kristen L / Davis, Philip J / Grusky, Alan Z / Hou, Brian Q / Hajdu, Katherine S / Yengo-Kahn, Aaron M / Zuckerman, Scott L / Terry, Douglas P

    Brain injury

    2024  Volume 38, Issue 4, Page(s) 295–303

    Abstract: Introduction: Repeat sport-related concussion (SRC) is anecdotally associated with prolonged recovery. Few studies have examined repeat concussion within the same athlete. We sought to explore differences in symptom burden and recovery outcomes in an ... ...

    Abstract Introduction: Repeat sport-related concussion (SRC) is anecdotally associated with prolonged recovery. Few studies have examined repeat concussion within the same athlete. We sought to explore differences in symptom burden and recovery outcomes in an individual athlete's initial and repeat SRC.
    Methods: A retrospective within-subject cohort study of athletes aged 12-23 years diagnosed with two separate SRCs from 11/2017-10/2020 was conducted. Primary outcomes were initial symptom severity and time-to-symptom-resolution. Secondary outcomes included return-to-learn (RTL) and return-to-play (RTP) duration.
    Results: Of 868 athletes seen, 47 athletes presented with repeat concussions. Median time between concussions was 244 days (IQR 136-395). Comparing initial to repeat concussion, no differences were observed in time-to-clinic (4.3 ± 7.3vs.3.7 ± 4.6 days,
    Conclusion: No significant differences in symptom duration and RTP/RTL were seen between initial/repeat concussion.
    MeSH term(s) Humans ; Athletic Injuries/complications ; Athletic Injuries/diagnosis ; Cohort Studies ; Retrospective Studies ; Brain Concussion/diagnosis ; Brain Concussion/complications ; Athletes
    Language English
    Publishing date 2024-02-09
    Publishing country England
    Document type Journal Article
    ZDB-ID 639115-1
    ISSN 1362-301X ; 0269-9052
    ISSN (online) 1362-301X
    ISSN 0269-9052
    DOI 10.1080/02699052.2024.2311341
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article ; Online: Juvenile idiopathic arthritis - what the clinician needs to know.

    Kahn, Philip J

    Bulletin of the Hospital for Joint Disease (2013)

    2013  Volume 71, Issue 3, Page(s) 194–199

    Abstract: Juvenile idiopathic arthritis (JIA) includes several forms of chronic arthritis in childhood with no apparent cause. JIA is the most common rheumatic disease in children, and may result in pain, joint deformity, and growth im- pairment, with possible ... ...

    Abstract Juvenile idiopathic arthritis (JIA) includes several forms of chronic arthritis in childhood with no apparent cause. JIA is the most common rheumatic disease in children, and may result in pain, joint deformity, and growth im- pairment, with possible persistent active arthritis into adulthood. Prior treatment involved non-specific agents, several with significant adverse effects. The recent use of biologics now provides target-specific therapy, which may be better tolerated. Through continued translational research and clinical trials, one better understands the biology mediating disease, with the hope of offering safer, more effective medicine, and potential cure. This review will outline the clinical features of JIA, as well as provide the latest updates in treatment.
    MeSH term(s) Age Factors ; Animals ; Antirheumatic Agents/therapeutic use ; Arthritis, Juvenile/diagnosis ; Arthritis, Juvenile/drug therapy ; Arthritis, Juvenile/immunology ; Child ; Disease Progression ; Humans ; Remission Induction ; Treatment Outcome
    Chemical Substances Antirheumatic Agents
    Language English
    Publishing date 2013
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 390411-8
    ISSN 2328-5273 ; 1936-9727 ; 1936-9719 ; 0018-5647 ; 0883-9344 ; 2328-4633
    ISSN (online) 2328-5273 ; 1936-9727
    ISSN 1936-9719 ; 0018-5647 ; 0883-9344 ; 2328-4633
    Database MEDical Literature Analysis and Retrieval System OnLINE

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