This book comprises a collection of categorized case-based questions, directed and meticulously selected to cover the most common and most important aspects of immunodeficiency diseases. Immunodeficiency disorders of infancy and childhood such as antibody deficiencies, phagocyte defects and defects in innate immunity are addressed among others. Each chapters starts with a brief of the initial presentation and lab data of the patient, followed by a series of 5-6 multiple choice questions (MCQs), leading the reader to the diagnosis and best of practice in a step-wise manner. This MCQ format along with precise, yet detailed answer ensures a quick, case-based, reality learning to the reader. This comprehensive MCQ series, is an essential reading material that a pediatric clinician, hematologist, immunologist, transplant specialist, or pulmonologist, can not afford to miss.

Presents cases based on real patients from referral centers all over the world Covers main areas of immunodeficiency diseases presenting over 160 cases Guides the reader through the different stages of clinical practice

Autorentext

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, Paediatric Immunology and Infectious Diseases in Northern Supra Regional Bone Marrow Transplant Unit for SCID and Related Disorders, Newcastle General Hospital. He has since authored more than 500 publications, including the Springer, "Primary Immunodeficiency Diseases", Immunology of Aging" and "Cancer Immunology" series, to which he was awarded the 10th, 16thand 17th 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''. Iranian Primary Immunodeficiency Diseases Registry (IPIDR) since 1999, having more than thousands of PIDand Paediatric Allergology and Immunology registered, is indeed one of his great scientific legacies.

Inhalt

1.Introduction to Primary Immunodeficiencies.- 2. Workup of Recurrent Respiratory Infections and a Positive Family History.- 3. Pseudomonas Meningitis.- 4. Fever and Sleepiness.- 5. Recurrent Pneumonia.- 6. Developmental Regression.- 7. Recurrent Ear Infections and Pneumonia.- 8. Positive Urine Culture for Klebsiella pneumoniae.- 9. Brothers with Recurrent Sinopulmonary Infections and Chronic Lung Disease.- 10. Recurrent Infections and Arthritis.- 11. Initial Diagnosis of Neutropenia and Later Crohn's Disease.- 12. Diagnosis of "Sweat-Chloride Negative" Cystic Fibrosis.- 13. Diagnosis of Sarcoidosis.- 14. Refractory Crohn's disease.- 15. Asthma Which Was Not Asthma.- 16. Generalized Lymphadenopathy and Hypogammaglobulinemia after Abdominal Trauma.- 17. Weakness and Anemia.- 18. Normal Primary Care Workup for Recurrent Sinopulmonary Infections.- 19. Recurrent Sinusitis and Persistent Giardia Infection.- 20. Recurrent Respiratory Infections.- 21. SiblingsPresenting with Mild and Severe Lymphoproliferation.- 22. Non-Malignant Lymphoproliferation.- 23. Portal Hypertension and Progressive Lymphoproliferation.- 24. Recurrent Otitis and Upper Airway Obstruction.- 25. Recurrent Pneumonia.- 26. A Growing BCG Lesion.- 27. Respiratory Distress and hypoxemia.- 28. A Boy with Burdened Family History, Candidiasis and Diarrhea.- 29. Failure to Thrive.- 30. Recurrent Respiratory Infection and Hypereosinophilia.- 31. Neonatal Jaundice and Leukopenia, Microcephaly, Recurrent Infections and Failure to Thrive.- 33. Widespread Erythema and Skin Desquamation.- 34. Failure to Thrive, Rash and Fever.- 35. Autoimmune Cytopenia.- 36. Recurrent Respiratory Tract Infections.- 37. Protracted Diarrhea and Hypogammaglobulinemia.- 38. Gingivitis and Recurrent Tonsillitis.- 39. Respiratory Distress.- 40. Intestinal Cryptosporidiosis.- 41. Shortness of Breath.- 42. Pneumocystis jiroveciPneumonia without Failure to Thrive.- 43. Recurrent Infections and CNS Vasculitis.- 44. Recurrent Infections and Widespread Warts.- 45. Rash and Several Food Allergies.- 46. Sever Atopic Disease and Infections.- 47. Acute Mastoiditis.- 48. Chronic Papulovegetative Ulcer on Penis.- 49. Fever after DPT Vaccination.- 50. Recurrent Bronchiolitis.- 51. Oral Ulcers and Candidiasis.- 52. Neutropenia and Myelodysplasia.- 53. Loss of Appetite and Neutropenia.- 54. Delayed Umbilical Cord Separation.- 55. Recurrent Pneumonitis.- 56. Recurrent Sepsis, Leukocytosis and Bleeding Tendency.- 57. Maculopapular Rash on BCG Site.- 58. Recurrent Perianal Abscesses.- 59. Infected Hemangiomas.- 60. Recurrent Invasive Bacterial Infections.- 61. Cervical Lymphadenitis.- 62. Failure to Thrive and Nasal Obstruction.- 63. Inflammatory Bowel Disease and Frequent Skin Abscesses.- 64. Necrotizing Liver Granuloma.- 65. Constrictive Aspergillosis Pericarditis.- 66. Lung Abscess.- 67. Lethargy in a Toddler.- 68. Recurrent Cold Suppurative Granulomatous Lymphadenitis.- 69. Repetitive Fistulizing Lymphadenopathy and Hematochezia.- Chapter 70. Twins with Recurrent Candida Infections.- Chapter 71. Recurrent Stomatitis and Upper Respiratory Tract Infections.- Chapter 72. Oral Thrush and Onychomycosis.- Chapter 73. Oral Moniliasis and Failure to Thrive.- Chapter 74. Refractory Oral Thrush.- Chapter 75. Kidney Malformation and Persistent Neutropenia.- Chapter 76. Severe Pancytopenia.- Chapter 77. Silvery Hair.- Chapter 78. Fever and Blonde Skin.- Chapter 79. Probable Sepsis.- Chapter 80. Skin Abscess and Blonde Hair.- Chapter 81. Recurrent Infections and Easy Bruising.- Chapter 82. Fever and Skin Eruption.- Chapter 83. Cervical Lymphadenopathy and Nasopharyngeal Mass.- Chapter 84. Lymphadenopathy and Splenomegaly.- Chapter 85. Anemia and Neutropenia.- Chapter 86. Hemolytic Anemia and Lymphadenopathy.- Chapter 87. Recurrent Infections and Chronic Diarrhea.- Chapter 88. Recurrent Infections, Cytopenias and Lung Disease.- Chapter 89. Short Stature, Prolonged Feverand Lymphoproliferation.- Chapter 90. Siblings with Nail Dystrophy and Photophobia.- Chapter 91. Chronic Diarrhea and Failure to Thrive.- Chapter 92. Rash and Diarrhea.- Chapter 93. Recurrent Infections, Enteropathy and Granulomatous Lung Disease.- Chapter 94. Chronic Diarrhea.- Chapter 95. Early-Onset Inflammatory Bowel Disease.- Chapter 96. Recurrent Febrile Episodes and Abdominal Pain.- Chapter 97. Episodic Fever and Lymphadenopathy.- Chapter 98. Periodic Fever Syndrome and Developmental Delay.- Chapter 99. Periodic Fever and Maculopapular Rash.- Chapter 100. Long Episodes of Rash and Fever.- Chapter 101. Long-Lasting History of Urticaria.- Chapter 102. Rash and Fever since Two Weeks of Age.- Chapter 103. Aseptic Meningitis.- Chapter 104. Recurrent Fever and Sore Throat.- Chapter 105. Recurrent Fever and Stomatitis.- Chapter 106. Prolonged Fever and Swollen Joints.- Chapter 107. Skeletal Pain in Knee and Clavicle.- Chapter 108. Bone Pain in Upper Leg, Hip, Lower Back.- Chapter 109.Enterocolitis Followed by Recurrent Sepsis-Like Episodes.- Chapter 110. Recurrent Febrile Attacks, Myalgia and Livedo Reticularis.- Chapter 111. Sudden Dizziness, Somnolence and Diplopia.- Chapter 112. Recurrent Chest Pain.- Chapter 113. Irregular Recurrent Fever.- Chapter 114. Fever, Headache and Photophobia.- Chapter 115. Recurrent Meningitis.- Chapter 116. Lupus, Macrophage Activation Syndrome and Invasive Infections.- Chapter 117. Recurrent Edema.- Chapter 118. Recurrent, Non-Pitting Edema of Lips, Extremities and Genitalia.- Chapter 119. Recurrent Abdominal Pain.- Chapter 120. Laryngeal Edema.- Chapter 121. Lips Angioedema.- Chapter 122. Chronic Diarrhea.- Recurrent Edema and Respiratory Infections.- Chapter 123. Otitis Media and Gait Disturbance.- Chapter 124. Regression of Her Ability to Walk.- Chapter 125. Microceph…