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

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

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
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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
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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
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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
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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
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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
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Article: Higher-dose Anakinra is effective in a case of medically refractory macrophage activation syndrome.

Kahn, Philip J / Cron, Randy Q

The Journal of rheumatology

2013 Volume 40, Issue 5, Page(s) 743–744

MeSH term(s)	Antirheumatic Agents/therapeutic use ; Child ; Dose-Response Relationship, Drug ; Drug Substitution ; Drug Therapy, Combination ; Female ; Humans ; Immunosuppressive Agents/therapeutic use ; Interleukin 1 Receptor Antagonist Protein/therapeutic use ; Macrophage Activation Syndrome/drug therapy ; Macrophage Activation Syndrome/pathology ; Remission Induction ; Treatment Failure ; Treatment Outcome
Chemical Substances	Antirheumatic Agents ; Immunosuppressive Agents ; Interleukin 1 Receptor Antagonist Protein
Language	English
Publishing date	2013-05
Publishing country	Canada
Document type	Case Reports ; Letter
ZDB-ID	194928-7
ISSN	1499-2752 ; 0315-162X
ISSN (online)	1499-2752
ISSN	0315-162X
DOI	10.3899/jrheum.121098
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Article ; Online: Hypertension during intravenous immune globulin infusion for Kawasaki's disease: an underreported phenomenon?

Kissel, Margaret / Phoon, Colin K L / Kahn, Philip J

Clinical pediatrics

2015 Volume 54, Issue 5, Page(s) 491–493

MeSH term(s)	Antihypertensive Agents/therapeutic use ; Child, Preschool ; Female ; Humans ; Hypertension/complications ; Hypertension/drug therapy ; Immunoglobulins, Intravenous/therapeutic use ; Labetalol/therapeutic use ; Mucocutaneous Lymph Node Syndrome/complications ; Mucocutaneous Lymph Node Syndrome/drug therapy
Chemical Substances	Antihypertensive Agents ; Immunoglobulins, Intravenous ; Labetalol (R5H8897N95)
Language	English
Publishing date	2015-05
Publishing country	United States
Document type	Case Reports ; Journal Article
ZDB-ID	207678-0
ISSN	1938-2707 ; 0009-9228
ISSN (online)	1938-2707
ISSN	0009-9228
DOI	10.1177/0009922814536776
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Article ; Online: Mucocutaneous Manifestations of Multisystem Inflammatory Syndrome in Children During the COVID-19 Pandemic.

Young, Trevor K / Shaw, Katharina S / Shah, Jinal K / Noor, Asif / Alperin, Risa A / Ratner, Adam J / Orlow, Seth J / Betensky, Rebecca A / Shust, Gail F / Kahn, Philip J / Oza, Vikash S

JAMA dermatology

2020 Volume 157, Issue 2, Page(s) 207–212

Abstract: Importance: To date, no study has characterized the mucocutaneous features seen in hospitalized children with multisystem inflammatory syndrome in children (MIS-C) or the temporal association of these findings with the onset of systemic symptoms.: ... ...

Abstract	Importance: To date, no study has characterized the mucocutaneous features seen in hospitalized children with multisystem inflammatory syndrome in children (MIS-C) or the temporal association of these findings with the onset of systemic symptoms. Objective: To describe the mucocutaneous findings seen in children with MIS-C during the height of the coronavirus disease 2019 (COVID-19) pandemic in New York City in 2020. Design, setting, and participants: A retrospective case series was conducted of 35 children admitted to 2 hospitals in New York City between April 1 and July 14, 2020, who met Centers for Disease Control and Prevention and/or epidemiologic criteria for MIS-C. Main outcomes and measures: Laboratory and clinical characteristics, with emphasis on mucocutaneous findings, of children who met criteria for MIS-C. The characterization of mucocutaneous features was verified by 2 board-certified pediatric dermatologists. Results: Twenty-five children (11 girls [44%]; median age, 3 years [range, 0.7-17 years]) were identified who met definitional criteria for MIS-C; an additional 10 children (5 girls [50%]; median age, 1.7 years [range, 0.2-15 years]) were included as probable MIS-C cases (patients met all criteria with the exception of laboratory test evidence of severe acute respiratory syndrome coronavirus 2 [SARS-CoV-2] infection or known exposure). The results of polymerase chain reaction tests for SARS-CoV-2 were positive for 10 patients (29%), and the results of SARS-CoV-2 immunoglobulin G tests were positive for 19 patients (54%). Of the 35 patients, 29 (83%) exhibited mucocutaneous changes, with conjunctival injection (n = 21), palmoplantar erythema (n = 18), lip hyperemia (n = 17), periorbital erythema and edema (n = 7), strawberry tongue (n = 8), and malar erythema (n = 6) being the most common findings. Recognition of mucocutaneous findings occurred a mean of 2.7 days (range, 1-7 days) after the onset of fever. The duration of mucocutaneous findings varied from hours to days (median duration, 5 days [range, 0-11 days]). Neither the presence nor absence of mucocutaneous findings was significantly associated with overall disease severity. Conclusions and relevance: In this case series of hospitalized children with suspected MIS-C during the COVID-19 pandemic, a wide spectrum of mucocutaneous findings was identified. Despite their protean and transient nature, these mucocutaneous features serve as important clues in the recognition of MIS-C.
MeSH term(s)	Adolescent ; COVID-19/complications ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Mucous Membrane ; New York City ; Retrospective Studies ; Skin Diseases/etiology ; Systemic Inflammatory Response Syndrome/complications
Language	English
Publishing date	2020-12-09
Publishing country	United States
Document type	Journal Article
ZDB-ID	2701761-8
ISSN	2168-6084 ; 2168-6068
ISSN (online)	2168-6084
ISSN	2168-6068
DOI	10.1001/jamadermatol.2020.4779
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Article ; Online: Asymptomatic giant coronary aneurysm in an adolescent with Behcet's syndrome

Kahn Philip J / Yazici Yusuf / Argilla Michael / Srichai Monvadi / Levy Deborah M

Pediatric Rheumatology, Vol 10, Iss 1, p

2012 Volume 2

Abstract: Abstract Objective Behcet's is an idiopathic multi-organ syndrome, which may have onset during childhood. Vascular involvement is uncommon, with rarely reported coronary aneurysm formation. We present a case report of a teenager girl who developed ... ...

Abstract	Abstract Objective Behcet's is an idiopathic multi-organ syndrome, which may have onset during childhood. Vascular involvement is uncommon, with rarely reported coronary aneurysm formation. We present a case report of a teenager girl who developed recalcitrant life-threatening Behcet's vasculitis, involving both small and large venous and arterial systems including a giant coronary aneurysm. Case report De-identified data were collected retrospectively in case report format. Although our sixteen year old female with Behcet's vasculitis had resolution of many arterial aneurysms, she had persistent venous thrombosis of large vessels, as well as persistent, giant arterial aneurysms requiring intra-arterial coiling of a lumbar artery and coronary bypass grafting despite intensive immunosuppression including glucocorticoids, cyclophosphamide, infliximab, methotrexate, azathioprine and intravenous immunoglobulin. Conclusions Vascular manifestations may be seen in Behcet's syndrome, including asymptomatic coronary aneurysm, which may be refractory to immunosuppression and ultimately require surgical intervention. Increased awareness is essential for prompt diagnosis and management.
Keywords	Behcet's syndrome ; vasculitis ; coronary aneurysm ; Diseases of the musculoskeletal system ; RC925-935 ; Specialties of internal medicine ; RC581-951 ; Internal medicine ; RC31-1245 ; Medicine ; R ; DOAJ:Internal medicine ; DOAJ:Medicine (General) ; DOAJ:Health Sciences ; Pediatrics ; RJ1-570 ; DOAJ:Pediatrics
Subject code	610
Language	English
Publishing date	2012-01-01T00:00:00Z
Publisher	BioMed Central
Document type	Article ; Online
Database	BASE - Bielefeld Academic Search Engine (life sciences selection)

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