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Differentiation Syndrome in APML Epidemiology: • Incidence: common in APL (2-48% depending on the study) • Triggers: ATRA treatment Pathophysiology: • Cytokine Release from blast cells → SIRS response • Cathepsin G release → vascular permeability and endothelial damage • Induce blast cell adhesion and endothelial damage Prophylaxis: • Attempt if high risk if WBC > 5 or elevated Cr • Prednisone 5mg/kg/day vs. 2.5 mg/m2 Presentation: Subacute-Acute: • Common: fever, myalgias, HoTN, edema and effusions, weight gain. More common in severe • Rare: DAH, acute febrile neutrophilic dermatosis Diagnosis: • Labs: leukocytosis and coagulopathy common • Imaging: CXR- pulmonary opacities • Diagnosis: >3 symptoms, or> 1 with no other explanation. Moderate > 2/3 and severe >4. Timing, generally either within 6 days or 15 days of ATRA initiation • Differential Diagnosis: Infection (sepsis), PE, DAH, CHF, Anaphylaxis, Acute Renal Failure Treatment: • Steroids: IV Dexamethasone 10 mg q12h -> 10 mg q6h • Cytoreductive: hydroxycarbamide 500 mg QD until normal WBC • Continue ATRA unless severe APLS, organ dysfuxtion, ICU, refractory to steroids • Supportive Care: Diuresis for fluid overload, PCC to reverse coagulopathy, RRT/IMV as needed - Dr. Noah Rosenberg @nsrosenberg #Differentiation #Syndrome #APML #diagnosis #management #hematology #oncology
Lemierre’s Syndrome - Pathophysiology Lemierre’s is an infectious thrombophlebitis of the internal jugular vein. - It starts out as a common oropharyngeal infection, such as pharyngitis, tonsillitis, peritonsillar abscess, or dental infection. - But then if it goes untreated, it can spread to the parapharyngeal space (ie become a deep space neck infection), and get into the carotid sheath. - Once it penetrates the carotid sheath, it is only a matter of time before the patient becomes bacteremic and a clot forms in the IJ. From there, septic emboli can fly throughout the body, landing in the liver, lungs, endocardium, and joints. #Lemierres #Syndrome #Pathophysiology #Diagnosis
Clinical Manifestations of Serotonin Syndrome • Ophthalmologic: Mydriasis, Ocular clonus (slow horizontal movements) • Gastrointestinal: Diarrhea, increased bowel sounds, RUQ pain • Dermatologic: Diaphresis, flushing • CNS: Agitation, Delirium, Rigidity, Hyperreflexia • Cardiovascular and vital sign abnormalities: Tachycardia, Fever, Hypertension, Late stages (shock) Spectrum of Clinical Manifestations (Mild → Life-Threatening): Akathisia, Altered Mental Status (agitated), Tremor, Clonus (sustained), Clonus (inducible), Muscular Hypertonicity, Hyperthermia UCSD Internal Medicine @ucsdim #Serotonin #Syndrome #Symptoms #spectrum #signs #diagnosis
Guillain-Barre Syndrome - Summary Acute AIDP that presents with rapidly progressive flaccid weakness Epidemiology: • 1-2 cases/100,000 per year • Slightly greater in males than females Guillain-Barre Pathophysiology: • Immune response preceding infection - Campylobacter jejuni - HIV, CMV, EBV - Influenza like illnesses, COVID 19 - Zika virus - Vaccination (flu, meningococcal, H1N1) Clinical Manifestations: • Fairly symmetric muscle weakness • Absent or depressed DTR’s • Onset: Few days to a week • Weakness: mild difficulty with walking to nearly complete paralysis of all extremity, facial, respiratory and bulbar muscles Guillain-Barre Clinical Features: • Weakness: Starts in legs and ascends usually. Can begin in arms or face (10% of patients) • Respiratory Muscle Weakness: May require ventilator support (10-30%) • Facial Nerve Palsies / Oropharyngeal Weakness • Oculomotor Weakness • Decreased / Absent Reflexes: Arms or legs • Paresthesias: Hands or feet • Pain: Due to nerve root inflammation. Back or extremities • Dysautonomia: Life threatening labile BP and arrythmias • Other: Diarrhea/constipation, hyponatremia, bradycardia, urinary retention, tachycardia, reversible cardiomyopathy, Horner syndrome, Sudden death Miller Fisher Syndrome: • Opthaloplegia • Ataxia • Areflexia Guillain-Barre Diagnosis: • CSF: - CSF PROTEIN - NORMAL CSF WBC (Albuminocytologic dissociation) • Electrodiagnostic studies - EMG, NCV can classify the main variants: (1) acute polyneuropathy with demyelination features (2) axonal form • MRI - Thickening and enhancement of intrathecal spinal nerve roots and cauda equina • GQ1b IgG Antibody associated with Miller Fisher Variant. Treatment: • Plasmapheresis + IVIG • Mechanical ventilation • Steroids contraindicated- worse outcome #GuillainBarre #Guillain #Barre #Syndrome #diagnosis #management #treatment #neurology
Horner's Syndrome • Anhidrosis: Diminished sweating of ipsilateral face and neck • Miosis: Loss of sympathetic innervation to dilator pupillae muscle (impaired dilation) • Ptosis: Loss of sympathetic innervation to the superior tarsal muscle or Müller's muscle - Ptosis is mild in comparison to oculomotor nerve palsy (levator palpebrae superior) By Dr. Gabriela Figueiredo Pucci @gabifpucci @neudrawlogy #Horners #Syndrome #diagnosis #neurology
Multi-System Inflammatory Syndrome in Children (MIS-C) vs. Kawasaki Disease CDC Case Definition for MIS-C; • < 21 years old with fever • Lab evidence of inflammation • Illness requiring hospitalization • >2 organ involvement • No other reasons for disease • Positive COVID testin now or recentl #MISC #Kawasaki #Comparison #diagnosis #peds #pediatrics #COVID19 #Coronavirus #MultiSystem #Inflammatory #Syndrome #Children
Marfan Syndrome - Signs and Symptoms - Caused by mutations in the fibrillin-1 (FBN1) Gene (chromosome 15) - Abnormal fibrillin function - Autosomal Dominant #Marfans #Syndrome #Diagnosis #Signs #Symptoms
Causes of upper gastrointestinal bleeding - An Illustrated Differential Diagnosis #Diagnosis #Differential #UpperGI #Bleeding #Diagram #Causes ** GrepMed Recommended Text: Kumar and Clark's Clinical Medicine - https://amzn.to/2T9Y5eN
Romberg's Test for Proprioception History: First described in the 19th century in patients with tabes dorsalis (later associated with syphilis) Meaning: Positive test (Romberg's sign) → Proprioceptive loss How to perform: 1) Patient stands with eyes open 2) Observe when patient closes the eyes 3) Observe for a difference between standing balance with eyes open and closed Positive test → Increased Sway, Step, Fall Pearls: • Cerebellar disease: standing can be difficult even with the eyes open • Unilateral vestibular disease: tendency to fall toward the side of the lesion By Dr. Gabriela Figueiredo Pucci @gabifpucci @neudrawlogy #Rombergs #Test #neurology #diagnosis #proprioception #physicalexam
Water Deprivation Test - Differential Diagnosis of Diabetes Insipidus (DI) Concept of WDT: endogenous vasopressin secretion is measured upon osmotic stimulation (thirsting) indirectly as urinary concentration capacity #Diabetes #Insipidus #DI #Water #Deprivation #Test #Differential #Diagnosis #algorithm #WDT
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