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30 Cards in this Set

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Wegener Granulomatosis

Upper + lower resp findings


Renal insufficiency




+ C-ANCA




Tx: Prednisone + Cyclophosphamide

Goodpasture syndrome

Lung + kidney + anemia


NO upper resp, systemic vasculitis, skin, joint, GI or neuro




Best initial text = antiglomerular basement membrane antibodies


Most accurate = biopsy of lung or kidney




Tx: plasmaphoresis, steroids, +/- cyclophosphamide

Berger disease (IgA nephropathy)




Population


Presentation


Dx


Tx

Berger disease (IgA nephropathy) - Most common cause of acute GN


- Look for ASIAN patient


- Recurrent episodes of gross hematura 1-2 days post URTI




Increased IgA


Biopsy = most accurate test




Severe proteinuria can be treated w ACEi + Steroids

Postinfectious GN




- Symptoms


- Dx


- Tx

Usually after streptococcus


Follows throat or skin infxn by 1-3 weeks




Dark coca cola coloured urine


Periorbital edema


HTN


Oligouria




Dx: antistreptolysin O titre


Anti-DNAse antibody titre


Low complement levels if post streptococcal




Tx: If it does not resolve use abx + diuretics



Alport syndrome

Cause of GN




Congenital defect in collagen


Sensorineural hearing loss


Visual disturbance (loss of collagen that holds lens in place)

Polyarteritis nodosa




What is it associated with?


What does it cause in young people?


Dx tests + treatment?

Systemic vasculitis of small + med sized arteries (most commonly affects the kidneys) - spares the lungs


Associated w Hep B




Symp: fever, malaise, weight loss, myalgias, arthralgias, purpura, peritchiae, digital gangrene, livedo reticularis (mottled)




STROKE or MI in young person = PAN




Dx:


- Anemia


- Leukocytosis


- Elevated ESR + CRP




Tx:


- Prednisone


- Cyclophosphamide

Lupus nephritis

Lupus nephritis




Usually mild, asymptomatic GN




Tx:


1. Glucocorticoids


2. Cyclophosphamide


3. Mycophenolate

Heinz Bodies on peripheral smear

Small inclusions w/in an erythrocyte from hemolysis secondary to G6P deficiency or thalassemia

Howell Jolly bodies

Sickle cell anemia

Helmet cells + schistocytes

Traumatic microangiopathic hemolytic conditions




- DIC


- Hemolytic uremic syndrome


- TTP

Kallman syndrome

- Decreased sense of smell


- Hypogonadotrophic hypogonadism


- Short


- X-linked

Hemolytic uremic syndrome

- Acute renal injury


- Thrombocytopenia


- Microangiopathic


- Increased lactate dehydrogenase


- Secondary to shiga toxin from E. Coli

Sjoren syndrome

Sjoren Syndrome


- Greatest incidence in middle age




Exocrine features: Dry eyes, salivary hypertrophy, dry skin


Extraglandular features: Raynaud phenomenon, cutaneous vasculitis, athralgias/arthritis, interstitial lung disease




Dx findings: + anti-Ro (SSA) or anti-La (SSB)



Causes of normal anion gap metabolic acidosis

Normal AG = 8 - 12




Diarrhea


Fistulas


Carbonic anhydrase inhibitors


RTA


Urethral diversion


Iatrogenic

Sturge Weber Syndrome

Sturge Weber Syndrome




- Focal or generalized seizures


- Mental retardation


- Port wine stain over the trigeminal nerve


- After 2 years old will see gyriform intracranial calcifications

McCune Albright Syndrome

McCune Albright Syndrome




Triad:


1. Cafe au lait spots


2. Polystotic fibrous dysplasia


3. Autonomous endocrine hyperfunction




(early puberty)

MEN Type 1

MEN Type 1




- Primary hyperparathyroidism (high Ca)


- Pituitary tumours (prolactin, visual defects)


- Pancreatic tumours (gastrinomas)

MEN Type 2A

Men Type 2A




- Medullary thyroid CA (calcitonin)


- Pheochromocytoma


- Parathyroid hyperplasia

MEN Type 2B

MEN Type 2B




- Medullary thyroid CA


- Pheochromocytoma


- Mucosal neuromas


- Marfanoid habitus

Medullary Thyroid CA

Medillary Thyroid CA




- Increased serum calcitonin


- Calcitonin producing tumour arising from neuroendocrine parafollicular C cells of the thyroid gland

DiGeorge Syndrome

DiGeorge Syndrome




- 22q11.2 deletion


- Facial deformities


- Developmental delays


- Hypocalcemia


- Decreased T cell count and function (thymic hypoplasia)



X-linked (Bruton) Agammaglobulinemia

X-linked (Bruton) Agammaglobulinemia = a type of immunodeficiency




- Decreased B Cells


- Normal T cells




- Male children


- Increased sinopulmonary infection


- Decreased size or absence of tonsils, adenoids, lymph nodse,




Tx: IVIG



Wiskott-Aldrich Syndrome

Wiskott-Aldrich Syndrome




Immune deficiency + thrombocytopenia + eczema




Decreased T lymphocytes

Hyper-IgE Syndrome

Hyper-IgE Syndrome




Recurrent skin infection w staph




Tx = prophylactic abx


- Dicloxacillin


- Cephalexin

Chronic Granulomatous Disease

Chronic Granulomatous Disease




- Genetic disease


- Extensive inflammatory reactions


- Lymph nodes w purulent material


- Aphthous ulcers in nares




Text = Abn nitro blue tetrazolium testing



Rheumatoid Arthritis




Symptoms

Rheumatoid Arthritis


- Morning Stiffness (at least 30 minutes)


- Rheumatoid nodules


- Occular symptoms (episcleritis - red sclera)


- Lung involvement (pleural effusions, nodules)


- Vasculitis


- Cervical joint involvement (C1 + C2)


- Baker cyst


- Pericarditis, pleural disease, carpal tunnel

Rheumatoid Tx: DMARDs

Rheumatoid Tx: DMARDs


1. Methotrexate - Best first line drug. Do a CBC q3mo. S/E = liver tox, bone marrow suppression, lung toxicity


2. TNFi - First line for those not responding to Methotrexate. Can add on top. Major S/E = reactivation of TB.


3. Rituximab - Major S/E = infection


4. Hydroxycholoroquine - S/E = toxic to the retina


5. Sulfalazine - S/E = bone marrow toxicity, hemolysis w G6PD, rash


6. Leflunomide - hepatotoxix, cytopenia


7. Cyclophosphamide - Bladder CA, sterility, myelosuppression, acute hemorrhagic systitis

FELTY Syndrome

Felty syndrome




- RA


- Splenomegaly


- Neutropenia

Caplan syndrome

Caplan syndrome




- RA


- Pneumoconicosis


- Lung nodules

Antiphospholipid syndrome

Antiphospholipid syndrome


- Sometimes associated w SLE




Symp:


- VTE + arterial embolic dx


- Miscarriage