Jessica Shared "Capstone" - 124 Picmonics
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Capstone
Acute Lymphoblastic Leukemia (ALL)
- Ages < 15
- May Present with Bone Marrow Involvement in Children
- Mediastinal Mass in Adolescents
- TdT Positive
- CALLA Positive
- t(12;21) Better Prognosis
- Spread to CNS and Testes
- Most Responsive to Chemotherapy
2 mins
Bruton's Agammaglobulinemia
- Pathophysiology
- X-linked Recessive
- Defective Bruton Tyrosine Kinase
- Blocks B Cell Maturation
- Signs & Symptoms
- Decreased Immunoglobulins of All Classes
- Bacterial Infections After Six Months
- Maternal IgG Protects Before Six Months
2 mins
Burkitt's Lymphoma
- Adolescents or young adults
- C-myc gene moves next to heavy chain Ig gene
- T 8;14
- Starry sky appearance
- Sheets of lymphocytes with interspersed macrophages
- Jaw lesion in endemic (African) form
- Pelvis or abdomen in non endemic form (American form)
- Associated with EBV
2 mins
Chronic Mucocutaneous Candidiasis
- Mechanism and Characteristics
- T Cell Dysfunction
- Candida Albicans
- Signs and Symptoms
- Infections of Skin
- Infections of Mucous Membrane
- Susceptible to HPV Infections like Condyloma Acuminatum
42 secs
Common Variable Immunodeficiency
- Pathophysiology
- Defect in B Cell Maturation
- Decreased Plasma Cells
- Decreased Immunoglobulins
- Signs & Symptoms
- Sinopulmonary Infections
- Increased Risk of Autoimmune Disease
- Lymphoma
1 min
Dactinomycin (Actinomycin D)
- Indications
- Wilms' Tumor
- Rhabdomyosarcoma
- Ewing's Sarcoma
- Gestational Trophoblastic Malignancy
- Mechanism
- Intercalates DNA
- Side Effect
- Myelosuppression
1 min
Epstein Barr Virus
- Characteristics
- Herpesvirus 4
- Double Stranded DNA
- Linear
- Enveloped
- Signs and Symptoms
- Infectious Mononucleosis
- Virus Attaches to CD21 in B Cells
- Nasopharyngeal Carcinoma
- Burkitt's Lymphoma
- Hodgkin's Lymphoma
1 min
Ewing's Sarcoma
- Epidemiology
- Most Common in Boys < 15 years old
- Pathophysiology
- 11;22 Translocation
- Characteristics
- Long bones, Pelvis, Ribs
- Diaphysis
- Small Blue Cells
- Malignant
- Aggressive
- Onion-skin Appearance
- TREATMENT
- Responsive to Chemotherapy
2 mins
Hyper IgE Syndrome
- Job Syndrome (Autosomal Dominant Hyper IgE Syndrome)
- Pathophysiology
- Autosomal Dominant
- STAT3 Mutation
- Increased IgE
- Abnormal Chemotaxis
- Signs and Symptoms
- Leonine Facies
- Cold (Non-Inflamed) Staph Aureus Abscesses
- Retained Primary Teeth
- Eczema
- Management
- Prophylactic Antibiotics
2 mins
Hyper IgM Syndrome
- Pathophysiology
- Deficient CD40 Ligand on Helper T-cells
- Defective CD40 Receptor on B-cells
- Inability to Class Switch
- Signs and Symptoms
- Pyogenic Abscess
- Decreased IgG, IgA, IgE
4 mins
Medulloblastoma
- Pathophysiology
- Occurs in Children
- Cerebellum
- Fourth Ventricle
- Diagnosis
- Small Blue Cells
- Lots of Mitotic Figures
- Homer Wright Rosettes
- Signs and Symptoms
- Hydrocephalus
- Ataxia
- Wide-based Gait
- Treatment
- Radiosensitive
2 mins
Neuroblastoma Characteristics and Presentation
- Characteristics
- Malignant Neuroendocrine Tumor
- Neural Crest Cells Origin
- Sympathetic Ganglia
- Adrenal Glands
- Amplification of N-myc Oncogene
- Most Common Cancer In Infancy
- Clinical Presentation
- Abdominal Mass That Can Cross Midline
- Gastrointestinal Distress
- Metastasis
- Raccoon Eyes
- Opsoclonus-Myoclonus Syndrome
4 mins
Neuroblastoma Diagnosis and Management
- Diagnosis
- 24-hour Urine Test
- Increased Catecholamines
- Increased Homovanillic and Vanillylmandelic Acid
- Ultrasound
- Magnetic Resonance Imaging (MRI)
- Biopsy
- Pathology
- Small Round Blue Cells
- Homer-Wright Rosettes
- Bombesin Positive
- Neuron Specific Enolase Positive
- Interventions
- Local Excision
- Chemotherapy
- Radiation
3 mins
Osteosarcoma
- Pathophysiology
- Osteogenic Sarcoma
- Peak incidence men 10-20 years old
- Metaphysis of Long Bones
- Knee Region
- Radiation
- Familial Retinoblastoma
- Paget's Disease
- Diagnosis
- Codman Triangle
- Sunburst Pattern from Elevated Periosteum
- Grave Prognosis
2 mins
Pilocytic Astrocytoma
- Occurs Most Commonly in Kids
- Posterior Fossa
- Cerebellum
- Well Circumscribed
- GFAP
- Rosenthal Fibers
- Eosinophilic Corkscrew Fibers
1 min
Selective IgA Deficiency
- Pathophysiology
- Defect in Class Switching
- Decreased IgA
- Signs and Symptoms
- Often Asymptomatic
- Increase in Atopic & Autoimmune Diseases
- Sinopulmonary Infections
- Increased Risk Giardia Lamblia Infections
- Inflammatory Bowel Disease
- Celiac Disease
- Considerations
- Anaphylaxis From IgA Containing Blood Products
2 mins
Severe Combined Immunodeficiency (SCID)
- Both B and T Cell Deficiency
- Recurrent Viral, Bacterial, Fungal, Protozoal Infections
- Defective Interleukin (IL) 2 Receptor
- X-Linked
- Adenosine Deaminase Deficiency
- Failure to Synthesize MHC II Antigens
- Treat with Bone Marrow Transplant
2 mins
Thymic Aplasia (DiGeorge Syndrome)
- Pathophysiology
- DiGeorge Syndrome
- 22q11 Deletion
- Failure to Develop Third and Fourth Pharyngeal Pouches
- Signs and Symptoms
- Undeveloped Thymus
- T Cell Deficiency
- Recurrent Viral and Fungal Infections
- Undeveloped Parathyroids
- Hypocalcemia
- Tetany
- Aortic Defects
- Congenital Heart Defects
- Diagnosis
- Absent Thymic Shadow on CXR
2 mins
Wilm's Tumor Syndromes
- WAGR Syndrome
- Aniridia
- Genital abnormalities
- Mental/Motor Retardation
- Due to Deletion of WT1 tumor suppressor gene
- Denys-Drash Syndrome
- Progressive renal (glomerular) disease
- Male pseudohermaphroditism
- Mutations of WT1
- Beckwith-Wiedemann Syndrome
- Neonatal Hypoglycemia
- Muscular hemihypertrophy
- Organomegaly
- Mutations in WT2 gene cluster
- Especially IGF-2
- Hepatoblastoma
- Abdominal wall defects (omphalocele, hypospadias)
Wilms' Tumor (Nephroblastoma)
- Cause
- Malignant Renal Tumor
- Assessment
- Abdominal Mass
- Hematuria
- Fever
- Hypertension
- Interventions
- Nephrectomy
- Chemotherapy
- Radiation
- Considerations
- Do Not Palpate Abdomen
- Children Under 5 Years Old
2 mins
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