麻豆传媒

The Immunodeficiency Center at SSM Health Cardinal Glennon Children's Hospital is a referral center for the Jeffrey Modell Foundation (JMF) and Immunodeficiency Foundation (IDF). The division works with the Bone Marrow Transplantation Division in transplantation of severe T-cells immunodeficiency and other Primary Immunodeficiency Disorders. The A&I Division is a referral center for evaluation of Very Early Onset Inflammatory Bowel Disorder (VEO IBD).

The A&I Division has developed special clinics to perform food and drug challenges to evaluate for these allergic diseases.

The A&I Division members are active in the Asthma and Allergy Foundation of America, St. Louis Chapter. The division helps coordinate with the AAFA St. Louis Chapter an annual asthma screening for at-risk participants in the St. Louis Cardinals' Redbird Rookies Little League.

The JMF Center actively participates in educational programs to the St. Louis Chapter of the Immune Deficiency Foundation (IDF). In addition the JMF Center participates in fund raising for the IDF.

• Primary Immune Deficiency Treatment Consortium
  Source: NIH U54 AI082973
  PI: Morton Cowan, M.D.
  Center PI: Alan P. Knutsen M.D.
• Jeffrey Modell Diagnostic & Research Center for Primary Immunodeficiencies
  Source: Jeffrey Modell Foundation
  Center PI: Alan P. Knutsen MD
• ABPA-1923: A Randomized, Double Blind, Placebo Controlled, Parallel Group Study to Evaluate the Efficacy and Safety of Dupilumab in Allergic Bronchopulmonary Aspergillosis Patients with Asthma
  Source: Regeneron Pharmaceuticals Inc Protocol R668
  Center PI: Alan P. Knutsen MD 

• Sosa AC, Kariuki B, Gan Q, Knutsen AP, Bellone CJ, Guzman MA, Tomatsu S, Barrera LA, Tomatsu S, Chauhan AK, Armbrecht E, Monta帽o AM. Induction of oral tolerance to improve enzyme replacement therapy in Morquio syndrome. J Clin Invest 130(3):1288-1300, 2020. PMID: 31743109 March

• Knutsen AP. Allergic bronchopulmonary aspergillosis in asthma. Exp Rev of Clin Immunol 13(1):11-14, 2017. PMID 27599560 January

• de la Morena MT, Leonard D, Torgerson T, Cabral-Marques O, MD, Slatter M, Aghamohammadi A, Chandra S, Murguia-Favela L, Bonilla F, Kanariou M, Damrongwatanasuk R, Kuo C, Dvorak CC, Meyts I, Chen K, Kobrynski L, Kapoor N, Richter, MD, DiGiovanni D, MD, Dhalla F, MD, Farmaki E, Speckmann DC, Espanol T, Shcherbina A, Hanson C, Litzman J, Routes J, Wong M, Fuleihan R, Seneviratne SL, Small TN, Janda A, Bezrodnik L, Seger R, Raccio AG, Edgar JDM, Chou J, Abbott J, van Montfrans J, Gonzalez-Granado LI, Bunin N, Kutukculer N, Gray P, Gisela S, Pasic S, Aquino V, Wysocki C, Abolhassani H, Gruebaum E, Dorsey M, Cunningham-Rundles C, Knutsen AP, Sleasman J, Chapel H, Ochs H, Filipovich A, Cowan M, Gennery A, Cant A, Notarangelo L, Roifman C. Long term outcomes of 176 patients with X-linked hyper IgM syndrome. J Allergy Clin Immunol 139:1282-1292, 2017. PMID 27697500 April

• Borte M, Melamed I, Pulka G, Pyringer B, Knutsen AP, Ochs HD, Kobayashi RH, Kobayashi AL, Gupta S, Strach M, Smith W, Pituch-Noworolski A, Moy J. Efficacy and safety of human intravenous immunoglobulin 10% (Panzyga) in patients with primary immunodeficiency diseases: a two-stage multicentre, prospective, open-label study. J Clin Immunol 37:603-612, 2017 PMID 28755067 August

• Dhandha MM, Siegfried EC, Knutsen AP. Treatment of selective antibody deficiency with IVIG resulting in decreased frequency of Streptococcal infection and improvement of guttate-psoriasis. Dermatol Online J 23(9):1-6, 2017. August

• Caruthers C, Fernandes H, Shams A, Rodrigues J, Bhatla D, Knutsen AP. Outcomes for umbilical cord blood transplantation in severe combined immunodeficiency disorders: Ten-year experience. Pediatr Allergy Pulmol Immunol 30(3):171-180, 2017. September

• Heimall J, Logan BR, Cowan MJ, Notarangelo L, Griffith LM, Puck JM, Kohn D, Pulsipher MA, Parikh S, Martinez C, Kapoor N, O鈥橰eilly R, Boyer M, Pai SY, Goldman F, Burroughs L, Chandra S, Kletzel M, Thakar M, Connelly J, Cuvellier G, Davila B, S hereck E, Knutsen A, Sullivan K, DeSantes K, Gillio A, Haddad E, Petrovic A, Quigg T, Smith A, Stenger E, Yin Z, Shearer WT, Fleisher T, Buckley RH, Dvorak CC. Immune reconstitution and survival of 100 SCID patients post hematopoietic cell transplant: A Primary Immune Deficiency Treatment Consortium Natural History Study. Blood 130(25):2718-2727, 2017 PMID 29021228. December

• Kelly BT, Knutsen AP, Routes JM. Difficult cases: bronchiectasis. J Allergy Clin Immunol In Pract 6(1):315-316, 2018. PMID 29310762 January-February

• Melamed I, Borte M, Trawnicek L, Kobayashi A-L, Kobayashi RH, Knutsen A, Gupta S, Smits W, Pituch-Noworolska A, Strach M, Pulka G, Ochs H D, Moy JN. Pharmacokinetics of a novel high-yield human intravenous immunoglobulin 10% in patients with primary immunodeficiency diseases: analysis of a phase III, multicentre, prospective, open-label study. Eur J Pharm Sci 118:80-86, 2018. PMID 29522908 March

• Lee J, Becker B, Kirby A, Knutsen AP. Chromosome 1q23.3q31.1 deletion associated with decreased newborn screening of T cell receptor rearrangement circles (TRECs). Ann Allergy Asthma Immunol 121(1): 125-126, 2018. PMID 29653236 July

• Foster KJ, Rodrigues JM, Lee J, Powell S, Azmeh R, Knutsen AP. Periodic fever, aphthous stomatitis, pharyngitis, adenitis syndrome at a single children's hospital. Inter J Allergy Medications 4(2):1-6, August 18, 2018. doi./org 10.23937/2572-3308.1510034

• Rodrigues J, Fernandes HD, Caruther C, Braddock SR, Knutsen AP. Cohen syndrome: review of literature. Cureus 10(9): e3330. doi:10.7759/cureus.3330 September 18, 2018

• Dvorak CC, Hadad E, Buckley RH, Cowan MJ, Logan B, Griffith LM, Kohn DB, Pai S-Y, Notarangelo L, Shearer W, O鈥橰eilly R, Kapoor N, Heimall J, Chaudhury S, Shyr S, Chandra S, Cuvellier G, Moore T, Shenoy S, Goldman F, Smith A, Sunkersett G, Vander Lugt M, Caywood E, Quigg T, Burroughs L, Chandrakasan S, Craddock J, Saldana B, Gillio A, Shereck E, Aquino V, DeSantes K, Knutsen A, Thakar M, Yu L, Puck JM. The genetic landscape of SCID in the Current era. J Allergy Clin Immunol ePub September 5, 2018 PMID 30193840

• Ochs HD Melamed I, Borte M, Moy JN, Pyringer B, Kobayashi AL, Knutsen AP, Smits W, Pituch-Noworolska A,. Kobayashi RH. Intravenous immunoglobulin 10% in children with primary immunodeficiency diseases. Immunotherapy 10(14):1193-1202, 2018. PMID 30088423 October

• Hadad E, Logan BR, Griffith LM, Buckley RH, Parrott R, Prockop SE, Dvorak CC, Puck JM, Murnane M, Kapoor N, Pulsipher MA, Abdel-Azim H, Hanson IC, Martinez C, Bleesing JJ, Chandra S, Smith AR, Pai S-Y, Jyonouchi S, Sullivan KE, Burroughs L, Skoda-Smith A, Haight AE, Tumlin AG, Quigg TC, Taylor TC, Taylor C, Davila Saldana BJ, Keller MD, Seroogy CM, Desantes KB, Petrovic A, Leiding JW, Shyr DC, Decaluwe H, Teira P, Gillio AP, Knutsen A, Moore TB, Kohn DB, Kletzel M, Craddock JA, Aquino V, Davis JH, Yu LC, Cuvelier GDE, Bednarski JJ, Goldman FD, Kang EM, Shereck E, Porteus MH, Connelly JA, Fleisher TA, Maleck HL, Shearer WT, Szabolcs P, Thakar MS, Vander Lught MT, Heimall J, Yin Z, Cowan MJ, O鈥橰eilly RJ, Notarangelo LD. Genotype and 6-month posttransplant CD4 count predict survival and immune recovery. Blood 132(17):1737-1749, 2018. PMID 30154114 October

• Lam JK, Braddock SR, Huddleston CB, Knutsen AP. Co-existent TBX1 mutation and chromosomal 20q13.13-q13.2 duplication in an infant with abnormal T cell receptor rearrangement excision circle (TREC) newborn screening. Ann Allergy Asthma Immunol 122(2):222-223, 2019. PMID 30712578 February

• Knutsen AP, Kariuki B. Allergic bronchopulmonary aspergillosis and fungal allergy in severe asthma. Archives Applied Medicine 1(1):1-19, 2019. May

• Marsh R, Leiding JW, Logan BR, Griffith LM, Arnold D, Haddad E, Falcone EL, Yin Z, Patel K, Arbuckle E, Bleesing JJ, Sullivan KE, Heimall J, Burroughs LM, Skoda-Smith S, Chandrakasan S, Yu L, Oshrine BR, Cuvelier G, Thakar M, Chen K, Teira P, Shenoy S, Rhelen R, Forbes L, Chellapandian D, Salda帽a BD, Shah A, Weinacht K, Joshi A, Boulad F, Quigg T, Dvorak CC, Grossman D, Torgerson T, Graham P, Prasad V, Knutsen A, Chong H, Miller H, de la Morena MT, DeSantes K, Cowan MJ, Notarangelo LD, Kohn DB, Stenger E, Pai S-Y, Routes JM, Puck JM, Kapoor N, Pulsipher MA, Malech HL, Parikh S, Kang EM. Chronic granulomatous disease-associated IBD resolves and does not adversely impact survival following allogeneic HCT. J Clin Immunol 39(7):653-667, 2019. PMID 31376032 October

• Sosa AC, Kariuki B, Gan Q, Knutsen AP, Bellone CJ, Guzman MA, Tomatsu S, Barrera LA, Tomatsu S, Chauhan AK, Armbrecht E, Monta帽o AM. Induction of oral tolerance to improve enzyme replacement therapy in Morquio syndrome. J Clin Invest 130(3):1288-1300, 2020. PMID: 31743109 March

• Yakhmi N, Knutsen AP. Tumor necrosis factor associated periodic syndrome (TRAPS) in a pediatric patient. Ann Allergy Asthma Immunol 125(4):369-371, 2020. PMID 32376455 May

• Dorsey M, Wright N, Chiamowitz N, Davila B, Miller H, Keller M, Thakar M, Shah A, Cowan MJ, Buckley RH, Dvorak CC, Griffith L, Haddad E, Kohn DB, Logan B, Notarangelo LD, Pai SY, Puck J, Pulsipher M, Heimall J, Survey Participants: Abu-Arja R, Andolina J, Aquino V, Barnum J, Bednarski JBhatia M, Bonilla F, Butte M, Chandra S, Chandrakasan S, Chaudhury S, Chen K, Chong H, Cuvelier G, Dalal J, DeFelice M. DeSantes K, Forbes L, Gillio A, Goldman F, Joshi A, Kapoor N, Knutsen AP, Kobrynski L, Lieberman J, Oshrine B, Patel K, Prockop S, Quigg T, Quinones R, Roncarolo MG, Schultz K, Seroogy C, Shyr D, Siegel S, Smith A, Torgerson TR, Vander Lugt M, Yu L. Infections in SCID Newborns: Isolation, Infection Screening and Prophylaxis in PIDTC Centers. J Clin Immunol 2020. PMID 33006109 October

• Donegan R, Mathew S, Knutsen AP. Complement factor I deficiency in a 4 year old body with glomerulonephritis. Ann Allergy Asthma Immunol 125(5):613-614, 2020. PMID 32687990 November

• Dixit C, Thatayatikom A, Pappa H, Knutsen AP. Treatment of severe atopic dermatitis and eosinophilic esophagitis with dupilumab in a 14-year-old boy with autosomal dominant hyper-IgE syndrome. J Allergy Clin Immunol In Practice 16:S2213-2198, 2021 PMID 34280586 July

• Knutsen AP, Temprano J, Bhatla D, Slavin RG. Hypersensitivity pneumonitis and eosinophilic lung diseases. In: Wilmott R, Bush V, Deterding RR, Ratjen F, Sly P, Zar H, Li AP (editors), Kendig鈥檚 Disorders of the Respiratory tract in Children, 9th Edition, Saunders Elsevier Inc, Philadelphia, Chapter 65 2018

• Knutsen AP. IgG subclass deficiency. UpToDate (original June 2006) 14.2: 1-8, April 2021

• Haddad E, Logan BR, Griffith LM, Buckley RH, Parrott RE, Dvorak CC, Puck J, Prockop SE, Kapoor N, Abdel-Azim H, Hanson IC, Martinez C, Bleesing J, Chandra S, Smith AR, Pai S-Y, Jyonouchi S, Sullivan K, Haight AE, Tumlin AG, Burroughs L, Saldana BJD, Seroogy C, Petrovic A, Shyr DC, Quigg TC, Gillio AP, Decaluwe H, Kletzel M, Knutsen A, B Moore T, Aquino V, Davis JH, Yu LC, Kang EM, Schroeder ML, Shereck E, Craddock JA, Connelly JA, Bednarski II JJ, Goldman F, Porteus MH, Fleisher T, Kohn DB, Malech HL, Pulsipher MA, Shearer W, Szabolcs P, Thakar M, Lugt MV, Yin Z Notarangelo LD, Cowan MJ, O'Reilly RJ. Genotype, phenotype and T cell counts at one year predict survival and long term immune reconstitution after transplantation in severe combined immune deficiency (SCID) - the Primary Immune Deficiency Treatment Consortium (PIDTC). BMT Tandem Meetings February 23, 2017, Orlando, FL. Biol Blood Marrow Transplant 23(3):S133-134, 2017.

• Paris K, Haddad E, Hussain I, Darter A, Knutsen AP, Kobrnyski L, Wasserman R, Engl W, Rabbat CJ, Leibl H, McCoy B, Yel L. Efficacy, safety, and tolerability of the new human subcutaneous immunoglobulin (SCIG 20%) in pediatric patients (pts) with primary immunodeficiency diseases (PIDD) during the North American (NA) Phase 2/3 Study. CIS 2017: March 23-26, 2017; Seattle, WA

• Foster K, Lee J, Powell S, Azmah R, Knutsen AP, Rodrigues J. Periodic fever, aphthous stomatitis, pharyngitis, adenitis syndrome at a single children's hospital. Pedatr Acad Soc Meeting, May 6-9, 2017, San Francisco, CA

• Paris K, Haddad E, Hussain I, Darter A, Knutsen AP, Kobrnyski L, Wasserman RL, Engl W, Rabbat CJ, Leibl H, McCoy B, Yel L. Efficacy, adverse events, and tolerability of the new human subcutaneous immunoglobulin 20% in pediatric patients with primary immunodeficiency diseases During the North American Pivotal Study. IgNS 2017: October 5-8, 2017, Las Vegas, NV

• Leiding JW, Logan BR, Yin Z, Arbuckle E, Bleesing JJ, Sullivan KE, Heimall J, Burroughs LM, Chandrakasan S, Yu L, Oshrine BR, Cuvelier G, Thakar M, Chen K, Shenoy S, Salda帽a BD, Weinacht K, Bram R, Boulad F, Quigg T, Dvorak CC, Knutsen A, Chong H, Miller H, de la Morena MT, DeSantes K, Cowan MJ, Notarangelo LD, Kohn DB, Stenger E, Pai S-Y, Puck JM, Kapoor N, Pulsipher MA, Haddad E, Rawling D, Griffith LM, Shearer WT, Malech HL, Parikh S, Marsh RA, Kang EM. Resolution of CGD related colitis after allogeneic hematopoietic stem cell transplantation in patients with Chronic Granulomatous Disease 鈥� early results from the 6903 study of the Primary Immune Deficiency Treatment Consortium (PIDTC). Am Soc Blood Marrow Tandem Meeting (ASBMT) February 21-25, 2018, Salt Lake City, UT.

• Sosa A, Lee A, Kariuki B, Gan Q, Guzm谩n M, Knutsen A, Bellone, C, Monta帽o AC.. How to decrease Immune Response to Enzyme Replacement Therapy? Pediatric Academic Societies (PAS) 2018 Meeting, May 5 - 8, in Toronto, Canada

• Donegan R, Knutsen AP.  7 week old male with truncas arteriosus and abnormal Missouri Newborn Screening TRECs.  Ann Allergy Asthma Immunol 121(5):5109, 2018.   November
  Jonathan M. Rodrigues JM, Fernandes HD, Caruthers C, Braddock SR, Knutsen AP.  Cohen syndrome: report of a novel VPS13B (COH 1) mutation. World Allergy Congress, Lyon, France, December 2019.

• Thakar MS, Logan BR, Buckley RH, Haddad E, Dvorak CC, O'Reilly RJ, Kapoor N, Satter LF, Martinez C, Pai S-Y, Heimall J, Jyonouchi S, Sullivan KE, Chandra S, Smith AR, Chaudhury S, Saldana BD, Sunkersett G, Shyr DC, Burroughs LM, Petrovic A, Quigg TC, Shenoy S, Bednarski JJ II, DeSantes K, Cuvelier GDE, Chandrakasan S, Gillio AP, Knutsen AP, Eissa H, Goldman F, Moore TB, Aquino V, Shereck E, Vander Lugt M, Caywood EH, Yu LC, Rozmus J, Talano J-AM, Malech HL, Shah AJ, Abu-Arja R, Miller HK, Bani-Hashemi T, Chang CK, Dunn E, Torgerson T, Pulsipher MA, Griffith LM, Cowan MJ, Kohn DB, Puck J, Notarangelo LD.  Transplantation Outcomes for Children with Severe Combined Immune Deficiency (SCID) Have Improved over Time: A 36-Year Summary Report By the Primary Immune Deficiency Treatment Consortium (PIDTC).  Transplantation & Cellular Therapy Meetings of ASTCT and CIBMTR Orlando, FL February 19-23, 2020

• Azmeh R, Leipzig JR, Rodrigues JM, McLaughlin AM, MacKrell K, Han RN, Boakye EA, Buchanan PM, Dykewicz MS, Becker BA. Twenty-one year trends in the treatment of pediatric acute severe asthma by Pediatric Emergency Medicine and Critical Care Program Directors. J Allergy Clin Immunol 2017 Feb 139:2 pAB95.