Pediatric Autoimmunity and Transplantation A Case-Based Collection with MCQs, Volume 3

This book is a collection of case-based questions, directed towards and meticulously selected to cover the most common and important aspects of pediatric autoimmune disorders. Autoimmune disorders of infancy and childhood, rheumatological disorders, dermatologic autoimmune disorders, autoinflammatory disorders, and clinical immunology in solid organ and hematopoietic stem cell transplantation are among the topics covered.

 Each chapter starts with a case description followed by a series of 5-6 multiple choice questions (MCQ), presenting the flow from diagnosis to treatment in a step-wise, logical manner. The text guides the reader through the stream of real clinical practice from initial presentation, differential diagnosis, next best step, lab data, and definitive diagnosis to management and adverse effects of therapy. This MCQ format along with precise, yet detailed answer ensure a quick, reality learning to the reader.

 The book provides a quick guide and hands-on learning experience for pediatricians, hematologists, immunologists, transplant specialists, pulmonologists, as well as PhD and post-graduate researchers around the world.

Introduction to Autoimmunity, Secondary Immunodeficiency, and Transplantation-Fever and Cervical Lymphadenopathy-Prolonged Refractory High Fever-Pain in Both Knees-Challenging Pain in Knee and Ankle-Fever and Urticarial Rash-Knee Swelling and Rash-Malaise, Weight Loss and Intermittent Fever-Anemia, Microhematuria and Proteinuria-Fever, Anasarca and Arthralgia-Bradycardia in a Neonate-Annular Rash in a Neonate- Itchy Facial Rash- Muscle Weakness and Fever-Pruritic Erythematous Rashes on Face and Eyelids-Edema of Hands and Hypopigmented Lesions on Her Neck and Cheeks-Asymptomatic Atrophic Plaque on the Face-Morbiliform Rash and Fever-Recurrent Interstitial Keratitis and Audiovestibular Dysfunction-Breathlessness and Weight Loss- Neck Swelling and Fever- Puffy Face and Abdominal Distension- Hematuria and Abdominal Mass-Pitting Edema andDesquamation-Fever and Cutaneous Nodules-  Neck Stiffness and Fever-Rash and Generalized Body Pains-Positive HIV and Violet Macular Rash-Weight Loss and Tuberculin Skin Test Anergy-Edema on Lower Limbs-Asthenia and Fatigue-Infections, Whitish Skin Papules and Subcutaneous Nodules-Recurrent Pneumonia and History of Transplantation-Painful Skin Nodules, Prolonged Fever and Cervical Lymphadenopathy-Malaise, Laryngitis and Fever-Acute Myeloid Leukemia Requiring Hematopoietic Stem Cell Transplantation- Acute Myeloid Leukemia Requiring Hematopoietic Stem Cell Transplantation-Suspected Immunodeficiency- Acute Myeloid Leukemia Requiring Hematopoietic Stem Cell Transplantation- T-Lymphoblastic Lymphoma- No Lymphocytes in an Infant- DOCK8 Deficiency and No Matched Donor- Diarrhea Post-HSCT- Follow-Up of a Severe Combined Immunodeficiency, Who Received Bone-Marrow-Transplantation Four Decades Ago- Nonalcoholic Steatohepatitis Referred for Liver Transplantation- Acute Respiratory Distress during Stem Cell Infusion- Acute Dyspnea after Platelet Transfusion-Requiring Immunization after Rescue Autologous HSCT-Fever and Abdominal Pain-Prolonged Fever, Rash and Mucosal Bleeding-Recurrent Infections, Diarrhea and Hypereosinophilia-Liver Transplantation Who Developed Pancytopenia Post-Transplantation-Kidney Transplant Candidate Showing Presence of Pre-Formed Donor-Specific-Antibodies Against a Prospective Living Donor- Heart Transplant Candidate Who Required Extra-Corporal Membrane-Oxygenation (ECMO)-Acute Antibody-Mediated Rejection (AMR) Transplantation with Negative-Prospective Crossmatch Results-Kidney Transplantation in a Patient with End Stage Renal Disease (ESRD)-Secondary to Focal Segmental Glomerulosclerosis-Itchy Violaceous Plaques- Photosensitivity and Bullous Lesions-Recurrent Bullous Lesions-Refractory Blisters and Erosions-Annular Blisters and Erosions-Vesicular Pruriginous Lesions- Widespread Vesiculobullous Rash-Itchy Skin Rashes- Blisters on Back and Upper Extremities-Itchy Blisters-Sudden Onset Grouped Blisters-Pruritic Blisters- Widespread Depigmented and Hypopigmented Patches- Whitish Patches- Hyperpigmented Patches-Outbreaks of Erythematous and Necrotic Papules with Hemorrhagic Crusts- Persistant, Annular Urticarial Plaque- Yellow, Reddish Brown Skin Lesions- Erythroderma- Erythematous Scaly Plaques- Multiple Circinate, Sterile, Flaccid, Relapsing Pustules- Purpuric Macules and Oral Erosions- Diffuse Skin Rash and Mucosal Lesions- Painful Facial Ulceration- Patchy Hair Loss- Concerning Weight Loss Over Three Weeks- Toddler with Bruising on Knees.

Farzaneh Rahmani is a MD, MPH graduate from Tehran University of Medical Sciences. A three times winner of the Avicenna Student Award as outstanding young scientist in 2015, 2017, and 2019, and the National Outstanding Student of the Ministry of Health in 2019, she has achieved academic excellence way ahead of her peers. More than 40 publications and 2 book titles published within only 4 years of research activity report of a scientific future that promises to be bright for her.

Nima Rezaei is a MD, PhD in clinical immunology and human genetics. Having finished his PhD, he was awarded a fellowship in Clinical Immunology and Bone Marrow Transplantation, Pediatric Immunology and Infectious Diseases in Northern Supra Regional Bone Marrow Transplant Unit for SCID and Related Disorders in the Newcastle General Hospital.

He has since authored more than 700 publications, including the Springer, "Primary Immunodeficiency Diseases", Immunology of Aging" and "Cancer Immunology" series, for which he was awarded the 10^th, 16^th and 17^th Avicenna Award for the book of the year. He was recognized as the distinguished young researcher to the prestigious Rhazi Award in in Medical Science Research on 2006 and 2012. In 2017 he was recognized by the National Academy of Medical Sciences, as “Iran’s Best Medical Researcher of the Past Decade’’. The Iranian Primary Immunodeficiency Diseases Registry (IPIDR) is indeed one of his great scientific legacies, enrolling thousands of patients since 1999.