Philip J. Kahn, M.D.

Dysmorphic features, cognitive disability, chronic inflammation, and predisposition to vascular disease in two sisters: a new autosomal recessive disorder?
Velinov, Milen; Dolzhanskaya, Natalia; Ramaswamy, Prema; Barinstein, Laura; Stuart, R. Morgan; Kahn, Philip; Feldstein, Neil; Madrid, Ricardo E.
2012 JAN ;21(1):8-10, Clinical dysmorphology
A 20-year-old woman presented with mental retardation and a history of stroke related to moyamoya disease at the age of 8 years. She had cognitive impairment which became more pronounced after the stroke. This patient's parents were first cousins and six close family relatives had strokes in their 60s or 70s. The patient's 16-year-old sister had learning disability, chronic muscle pain, and an ECG suggestive of previous hypoxemic heart injury. The two sisters had similar dysmorphic facial appearance including a prominent philtrum, bulbous nose, and severe acne. They both had increased subcutaneous tissue in their faces, whereas their bodies were slim. Both sisters were found to have elevated levels of rheumatoid factor, C-reactive protein, and erythrocyte sedimentation rate on repeat measurements. Partial autoimmunity screening in one of the patients was negative. Chromosome analysis and array comparative genomic hybridization analyses were also normal. Nerve conduction findings in the younger sister were consistent with distal, predominantly motor, demyelinating neuropathy localized to the lower extremities. We propose that these two sisters suffer from a new autosomal recessive syndrome. Carrier status for this condition may predispose to later onset stroke. Clin Dysmorphol 21:8-10 (C) 2011 Wolters Kluwer Health vertical bar Lippincott Williams & Wilkins
— id: 149879, year: 2012, vol: 21, page: 8, stat: Journal Article,

Retiform purpura and digital gangrene secondary to antiphospholipid syndrome successfully treated with sildenafil
Gonzalez, Mercedes E; Kahn, Philip; Price, Harper N; Kamino, Hideko; Schaffer, Julie V
2011 Feb;147(2):164-167, Archives of dermatology
— id: 124105, year: 2011, vol: 147, page: 164, stat: Journal Article,

Giant Coronary Artery Aneurysm in a Patient with Behcet's Disease
Greenhouse, David G; Hackett, Katherine; Kahn, Philip; Balsam, Leora B; Galloway, Aubrey C
2011 May;26(3):268-270, Journal of cardiac surgery
Abstract Behcet's disease is a rare autoimmune vasculitis that may cause coronary artery aneurysms. We discuss the evaluation and management decisions for a 19-year-old female with a giant rapidly expanding aneurysm of the proximal left anterior descending coronary artery and Behcet's disease. (J Card Surg 2011;26:268-270)
— id: 132574, year: 2011, vol: 26, page: 268, stat: Journal Article,

Early versus later onset childhood-onset systemic lupus erythematosus: Clinical features, treatment and outcome
Hui-Yuen, J S; Imundo, L F; Avitabile, C; Kahn, P J; Eichenfield, A H; Levy, D M
2011 Aug;20(9):952-959, Lupus
The objective of the study was to compare clinical features, treatment and disease outcome in patients with early versus later onset of childhood-onset systemic lupus erythematosus (cSLE). A retrospective matched cohort study of cSLE patients diagnosed between 1988 and 2008 and followed for a minimum of one year was conducted. Thirty-four pre-pubertal cSLE patients with disease onset prior to their 12th birthday were matched by ethnicity and year of diagnosis to 34 pubertal cSLE patients. The most common criteria at diagnosis in both groups were malar rash, arthritis, hematologic manifestations, and renal disease. After a mean follow-up of more than six years, a similar proportion of patients in the two groups were still prescribed corticosteroids (47% and 41%); patients in the early onset group required a significantly higher daily dose (0.6 mg/kg prednisone-equivalent versus 0.2 mg/kg, p < 0.05). There were no significant differences in organ involvement, disease activity and disease damage between the two groups, and severe complications occurred at similar rates. There were a greater number of admissions to the pediatric intensive care unit (PICU) in the early onset group (18 versus 5, p = 0.01), with time-to-event analysis demonstrating a significantly shorter disease duration from diagnosis to first PICU admission in the early onset group (p < 0.001). While a similar proportion of patients in the early and later onset groups required treatment with cyclophosphamide, patients in the early onset group received treatment earlier in their disease course (mean 13.7 versus 19.9 months, p < 0.001). Early onset cSLE leads to earlier and more frequent PICU admission, earlier use of cyclophosphamide, and higher corticosteroid dose at long-term follow-up
— id: 138935, year: 2011, vol: 20, page: 952, stat: Journal Article,

Juvenile idiopathic arthritis - an update on pharmacotherapy
Kahn, Philip
2011 ;69(3):264-276, Bulletin of the NYU Hospital for Joint Diseases
Juvenile Idiopathic Arthritis (JIA) consists of 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. The extra-articular features of JIA, such as anterior uveitis or macrophage activation syndrome, are often the greater focus of therapy. Prior to the mid 1990s, the therapeutic armamentarium for JIA was more limited, utilizing non-specific agents, many with significant adverse effects. In the current era of target-specific biologic therapy, it is possible to better tailor therapy for patients. Through continued translational research and clinical trials, the biology mediating disease is better understood, and there is the hope of 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
— id: 139926, year: 2011, vol: 69, page: 264, stat: Journal Article,

Neurocognitive impairment in childhood-onset systemic lupus erythematosus: measurement issues in diagnosis
Williams, Tricia S; Aranow, Cynthia; Ross, Gail S; Barsdorf, Alexandra; Imundo, Lisa F; Eichenfield, Andrew H; Kahn, Philip J; Diamond, Betty; Levy, Deborah M
2011 Aug;63(8):1178-1187, Arthritis care & research
OBJECTIVE: To assess the prevalence of neurocognitive impairment (NCI) in childhood-onset systemic lupus erythematosus (cSLE) by comparing published classification criteria, and to examine associations between NCI, disease characteristics, psychosocial well-being, and intelligence. METHODS: cSLE patients and ethnicity- and age-matched healthy controls completed a neuropsychological research battery, and results were categorized by 3 different NCI classification criteria with different cutoff scores (e.g., >2, 1.5, or 1 SD below the mean) and the number of required abnormal tests or domains. RESULTS: Forty-one cSLE subjects and 22 controls were included. Subjects were predominantly female (~70%) and Hispanic ( approximately 70%). Executive functioning, psychomotor speed, and fine motor speed were most commonly affected. Method 1 classified 34.1% of cSLE subjects with NCI compared to method 2 (14.6% with decline and 7.3% with NCI) and method 3 (63.4% with NCI). The prevalence of NCI was not significantly different between the controls and patients using any of the categorization methods. NCI was not associated with SLE disease activity or characteristics or with depression. Using method 3, patients in the cognitive impairment group reported significantly lower quality of life estimates (69.7 versus 79.3; P = 0.03). Below average intellectual functioning (intelligence quotient <90) differentiated the number of test scores >1 and >1.5 SDs, but not >2 SDs below the mean. CONCLUSION: NCI was prevalent in cSLE, but varied according to the chosen categorization method. A similar proportion of cSLE patients and controls had NCI, reinforcing the importance of studying an appropriate control group. Categorical classification (i.e., impaired/nonimpaired) may oversimplify the commonly observed deficits in cSLE
— id: 137982, year: 2011, vol: 63, page: 1178, stat: Journal Article,

Hepatoerythropoietic porphyria misdiagnosed as child abuse: cutaneous, arthritic, and hematologic manifestations in siblings with a novel UROD mutation
Cantatore-Francis, Julie L; Cohen-Pfeffer, Jessica; Balwani, Manisha; Kahn, Philip; Lazarus, Herbert M; Desnick, Robert J; Schaffer, Julie V
2010 May;146(5):529-533, Archives of dermatology
BACKGROUND: Hepatoerythropoietic porphyria (HEP) is a rare autosomal recessive disorder resulting from the markedly deficient, but not absent, activity of the heme biosynthetic enzyme uroporphyrinogen decarboxylase (UROD). The disorder typically manifests during infancy or early childhood with extreme photosensitivity, skin fragility in sun-exposed areas, hypertrichosis, erythrodontia, and pink urine. OBSERVATIONS: Three siblings, offspring of parents of Puerto Rican and Dominican descent, had with excessive scarring on the face and dorsal aspect of the forearms, which initially led to the erroneous suspicion of child abuse. Although these lesions were photodistributed, overt photosensitivity had not been observed, with the exception of a single episode of blistering and onycholysis after intense sun exposure in 1 affected child. Mild facial hypertrichosis, chronic anemia, polyarticular arthritis, and developmental delay represented additional findings. Biochemical studies of urine, plasma, and erythrocyte porphyrins from the affected siblings established the diagnosis of HEP. Sequencing of the UROD gene revealed compound heterozygosity for a novel missense mutation, V166A, and a complex deletion/insertion, 645del1053ins10. CONCLUSIONS: Our report expands the phenotypic and genotypic spectrum of HEP, highlighting mild cutaneous presentations that can occur without obvious photosensitivity and masquerade as child abuse
— id: 110107, year: 2010, vol: 146, page: 529, stat: Journal Article,

Favorable outcome of juvenile dermatomyositis treated without systemic corticosteroids
Levy, Deborah M; Bingham, C April; Kahn, Philip J; Eichenfield, Andrew H; Imundo, Lisa F
2010 Feb;156(2):302-307, Journal of pediatrics
OBJECTIVE: To describe the course of patients with juvenile dermatomyositis (JDM) treated effectively without systemic corticosteroids. STUDY DESIGN: A retrospective study of 38 patients with JDM treated at a tertiary care children's hospital identified 8 patients who had never received corticosteroids. Disease presentation and course, pharmacologic, and ancillary treatments were recorded. RESULTS: Patients in the no corticosteroid group were followed for a median of 2.8 years (range, 2.1 to 9.5 years). Treatment was primarily with intravenous immunoglobulin (IVIG) (75%) and methotrexate (50%), with favorable response in all. No serious treatment complications were observed; headaches were reported by 3 patients receiving IVIG. Two patients had a myositis flare after discontinuing all medications for more than 1 year; complete resolution of symptoms was observed after either 1 or 2 further doses of IVIG. Two patients had calcinosis (at 1 and 9 years of disease); however, no patient had joint contractures, muscle atrophy, lipodystrophy, or functional limitations. CONCLUSIONS: Systemic corticosteroids can be avoided in a select group of patients with JDM. Alternative agents such as methotrexate and IVIG may be prescribed to effectively treat JDM and prevent complications
— id: 106905, year: 2010, vol: 156, page: 302, stat: Journal Article,

Juvenile idiopathic arthritis--current and future therapies
Kahn, Phillip
2009 ;67(3):291-302, Bulletin of the NYU Hospital for Joint Diseases
Juvenile idiopathic arthritis (JIA) consists of a group of heterogeneous disorders of chronic arthritis in childhoodwith no apparent cause. JIA is the most common rheumatic disease in children and may still result in signifcant morbidity, with joint deformity, growth impairment, and persistence of active arthritis into adulthood. In addition to arthritis, the extra-articular features that may be present in JIA, such as anterior uveitis or the fever of systemic-onset JIA, are often the greater focus of therapy. Prior to the mid 1990s, the therapeutic armamentarium for JIA was more limited, utilizing nonspecifc agents, many with signifcant adverse effects. In the new era of target-specifc biologic therapy, the clinician is now able to better tailor therapy for patients with JIA. Still, despite the bells and whistles of biologics, the consistent performance of methotrexate as the gold standard disease-modifying anti-rheumatic drug (DMARD), against which all other agents are compared, cannot be overemphasized. Through continued translational research, rheumatologists better understand the biology behind the clinical symptoms. This review will discuss the clinical features of JIA, as well as past, present, and future therapeutic approaches in the care of children with arthritis
— id: 104900, year: 2009, vol: 67, page: 291, stat: Journal Article,

Prevention of murine antiphospholipid syndrome by BAFF blockade
Kahn, Philip; Ramanujam, Meera; Bethunaickan, Ramalingam; Huang, Weiqing; Tao, Haiou; Madaio, Michael P; Factor, Stephen M; Davidson, Anne
2008 Sep;58(9):2824-2834, Arthritis & rheumatism
OBJECTIVE: This study was undertaken to determine whether BAFF blockade can be used to prevent or treat antiphospholipid syndrome in a mouse model. METHODS: Eight- and 12-week-old (NZW x BXSB)F(1) mice were treated with BAFF-R-Ig or TACI-Ig alone or in addition to a short course of CTLA-4Ig. Mice were monitored for thrombocytopenia and proteinuria. Sera were tested for anticardiolipin antibodies (aCL), BAFF levels, and levels of soluble vascular cell adhesion molecule and E-selectin. Mice were killed at 17, 22, or 32 weeks of age, and kidneys and hearts were subjected to histologic examination. Spleen cells were phenotyped and enzyme-linked immunospot assays for autoantibody-producing B cells were performed. RESULTS: Both BAFF-R-Ig and TACI-Ig prevented disease onset and significantly prolonged survival. Treated mice had significantly smaller spleens than controls, with fewer B cells and fewer activated and memory T cells. BAFF blockade did not prevent the development of aCL, and there was only a modest delay in the development of thrombocytopenia. However, treated mice had significantly less nephritis and myocardial infarcts than did controls. CONCLUSION: Our findings suggest that aCL are generated in the germinal center, which is relatively independent of BAFF. Effector function of antiplatelet antibodies was only modestly affected by BAFF blockade. In contrast, myocardial infarctions were prevented, suggesting that triggering of thromboses requires both autoantibodies and mediators of inflammation. Similarly, renal damage requires both immune complexes and effector cells. The dissociation between autoantibody production and inflammation that may occur with B cell-depleting therapies underscores the role of B cells as effector cells in the autoimmune response
— id: 90031, year: 2008, vol: 58, page: 2824, stat: Journal Article,

Monocytes in the urine of children with lupus: A potential marker of active nephritis
Kahn, PJ; Zhang, HZ; Levy, D; Imundo, L; Eichenfield, A; Winchestet, R
2008 ;58(9):S252-S252, Arthritis & rheumatism
— id: 90034, year: 2008, vol: 58, page: S252, stat: Journal Article,

Longitudinal performance on the Pediatric Automated Neuropscyhological Assessment Metrics (Ped-ANAM) Childhood-Onset Systemic Lupus Erythematosus (cSLE)
Levy DM; Batres C; Yu G; Imundo LF; Eichenfield A; Kahn PJ; Diamond B; Arnow C
2008 ;58(9 Suppl):S222-S222, Arthritis & rheumatism
— id: 90042, year: 2008, vol: 58, page: S222, stat: Journal Article,

Neurocognitive dysfunction is prevalent in childhood-onset systemic lupus erythematosus (cSLE)
Levy, DM; Barsdorf, A; Imundo, LF; Eichenfield, AH; Kahn, PJ; Batres, C; Ross, GS; Aranow, C
2008 ;58(9):S249-S249, Arthritis & rheumatism
— id: 90033, year: 2008, vol: 58, page: S249, stat: Journal Article,

Higher mortality and cyclophosphamide use in SLE with onset prior to eleventh birthday
DiSipio C; Imundo LF; Eichenfield AH; Kahn PJ; Levy DM
2007 ;55(9 Suppl):S366-S366, Arthritis & rheumatism
— id: 90045, year: 2007, vol: 55, page: S366, stat: Journal Article,

Neurocognitive function in Childhood-Onset Systemic Lupus Erythematosus (cSLE)
Levy DM; Barsdorf AI, Imundo LF; Kahn PJ; Eichenfield A; Batres C; Ross GS; Aranow C
2007 ;55(9 Suppl):S368-S368, Arthritis & rheumatism
— id: 90043, year: 2007, vol: 55, page: S368, stat: Journal Article,

Utilizing a Lupus Teen/Parent Support Group for pediatric medical education
Rose J; Horton R; Paget S; Flics S; Pfeffer B; Kahn P; Eichenfield A; Levy DM; Imundo L
2007 ;55(9 Suppl):S552-S552, Arthritis & rheumatism
— id: 90044, year: 2007, vol: 55, page: S552, stat: Journal Article,

Juvenile rheumatoid arthritis and spondyloarthropathy syndromes
Kahn P; Imundo L
2006 ;18:?-?, Current pediatric therapy
— id: 90047, year: 2006, vol: 18, page: ?, stat: Journal Article,

Prevention of anti-phospholipid syndrome in NZW/BXSB mice using BAFF blockade
Kahn, P; Ramanujam, M; Tao, H; Huang, WQ; Davidson, A
2006 ;54(9):S797-S797, Arthritis & rheumatism
— id: 90036, year: 2006, vol: 54, page: S797, stat: Journal Article,

Favorable response to high-dose infliximab for refractory childhood uveitis
Kahn, Philip; Weiss, Michael; Imundo, Lisa F; Levy, Deborah M
2006 May;113(5):860-4.e2, Ophthalmology
OBJECTIVE: Uveitis in children most commonly is associated with juvenile idiopathic arthritis. In addition to topical glucocorticoids, treatment may include systemic immunosuppressive agents. Tumor necrosis factor alpha (TNF-alpha) has been implicated in the pathogenesis of uveitis; therefore, TNF-alpha blockade seems to be a reasonable therapeutic option to investigate. We report successful treatment of children with uveitis using infliximab. STUDY DESIGN: A retrospective study of our complete experience using infliximab for the treatment of childhood uveitis was conducted. PARTICIPANTS: Seventeen children (14 females, 3 males) with chronic uveitis were administered high-dose infliximab (10-20 mg/kg/dose). MAIN OUTCOME MEASURES: Our main outcome measure was the ability to eliminate all signs of intraocular inflammation. RESULTS: All 17 patients demonstrated a dramatic, rapid response, with no observed inflammation in 13 patients after the second infusion, and 4 patients requiring 3 to 7 infusions to achieve disease quiescence. Additional immunosuppressives and topical glucocorticoids were tapered when patients achieved no intraocular inflammation. CONCLUSIONS: In this series, high-dose infliximab was a rapidly effective, well-tolerated therapeutic agent for the treatment of chronic, medically refractory, noninfectious uveitis
— id: 81141, year: 2006, vol: 113, page: 860, stat: Journal Article,

The detection of wrist synovitis on magnetic resonance imaging in clinically quiescent juvenile idiopathic arthritis
Kahn, PJ; Ruzal-Shapiro, C; Imundo, LF; Eichenfield, A; Levy, DM
2006 ;54(9):S165-S166, Arthritis & rheumatism
— id: 90035, year: 2006, vol: 54, page: S165, stat: Journal Article,

Rituximab in treatment-resistant childhood-onset systemic lupus erythematosus
Levy, DM; Adams, BS; Kahn, PJ; Imundo, LF
2005 ;52(9):S531-S531, Arthritis & rheumatism
— id: 90037, year: 2005, vol: 52, page: S531, stat: Journal Article,

A retrospective review of six patients with medically refractory chronic uveltis and a favorable response to high-dose infliximab
Kahn, PJ; Weiss, M; Imundo, L; Levy, DM
2004 ;55(4):16A-17A, Pediatric research
— id: 90039, year: 2004, vol: 55, page: 16A, stat: Journal Article,

Favorable response to higher dose infliximab in refractory uveitis: Eleven cases
Kahn, PJ; Weiss, M; Imundo, LF; Levy, DM
2004 ;50(9):S92-S92, Arthritis & rheumatism
— id: 90038, year: 2004, vol: 50, page: S92, stat: Journal Article,

Incidence of arrhythmias after thoracic surgery: Thoracotomy versus video-assisted thoracoscopy
Neustein, SM; Kahn, P; Krellenstein, DJ; Cohen, E
1998 ;12(6):659-661, Journal of cardiothoracic & vascular anesthesia
Purpose: Atrial arrhythmias, especially supraventricular tachycardia (SVT) and atrial fibrillation, are common after thoracotomy and lung surgery. There are few existing data on the incidence of postoperative arrhythmias after video-assisted thoracoscopy (VAT). The purpose of the present investigation was to retrospectively determine the incidence of postoperative arrhythmias in patients who underwent VAT compared with those who underwent thoracotomy, and which factors are associated with an increased risk for arrhythmias in both groups. Design: A retrospective investigation. Setting: A metropolitan university hospital. Participants: The medical records of 124 patients who underwent thoracotomy and 81 patients who underwent VAT over a 2-year period were reviewed. Measurements and Main Results: There was a 17% incidence of atrial arrhythmias after thoracotomy and 10% after VAT, but the difference was not statistically significant. In both groups, atrial fibrillation was the most common atrial arrhythmia. Conclusion: Patients receiving digoxin were at higher risk for postoperative arrhythmias. Patients older than 65 years were at risk for arrhythmias after thoracotomy and patients older than 80 years were at risk for arrhythmias after VAT. Patients who had postoperative arrhythmias had prolonged hospital stays compared with patients who did not have arrhythmias.
— id: 90040, year: 1998, vol: 12, page: 659, stat: Journal Article,

Incidence of shivering in patients undergoing ambulatory gynecological laparoscopy increases by fresh gas flow rate and surgical time
Panah, M; Rosenblatt, M; Kahn, P; Bronheim, D
1997 ;84:S18-S18, Anesthesia & analgesia
— id: 90032, year: 1997, vol: 84, page: S18, stat: Journal Article,

Failure of subhypntoic doses of propofol to treat pruritus induced by intrathecal morphine following cesarean section
Beilin Y; Kim J; Kahn P
1996 ;82:S22-S22, Anesthesia & analgesia
— id: 90046, year: 1996, vol: 82, page: S22, stat: Journal Article,