Pemphigus

Pemphigus is an autoimmune disorder of the skin and mucous membranes characterized by blisters associated with the binding of IgG autoantibodies to epithelial cell surface. The common form is called Pemphigus vulgaris. Antigens in pemphigus are believed to be desmoglein 1 and desmoglein 3. Antibodies belong to IgG1 and IgG4 type.

Mean age of onset is approximately 50-60 years. Histologic changes consist of intercellular edema with loss of intercellular attachments in the basal layer. Suprabasal epidermal cells separate from the basal cells to form clefts and blisters. These findings can help differentiate phemphigus vulgaris from pemphigus foliaceous, which demonstrates a more superficial epidermal cleavage.

Primary Amyloidosis- Types, proteins and clinical associations


Type

Deposited Protein

Clinical Associations

Systemic

Immunoglobulin lambda chains

Multiple myeloma

Transthyretin

Familial amyloidosis, senile cardiac amyloidosis

Amyloidosis – A protein

Inflammation-associated amyloidosis

Beta2 -microglobulin

Dialysis-related amyloidosis

Immunoglobulin heavy chains

Systemic amyloidosis

Hereditary

Fibrinogen alpha chain, Apo A1 and Apo A2

Familial systemic amyloidosis

Lysozyme

Familial systemic amyloidosis

Central nervous system

Beta protein precursor

Alzheimer's

Prion protein

Creutzfeldt-Jakob disease, Gerstmann-Strãussler-Scheinker disease, fatal familial insomnia

Cystatin C

hereditary cerebral hemorrhage with amyloidosis (Icelandic)

Ocular

Gelsolin

Familial amyloidosis (Finnish)

Lactoferrin

Familial corneal amyloidosis

Keratoepithelin

Familial corneal dystrophies

Localized

Calcitonin

Thyroid Carcinoma (Medullary)

Amylin

Insulinoma, type 2 diabetes

Atrial natriuretic factor amyloid

Isolated atrial amyloidosis

Prolactin

Pituitary amyloid

Keratin

Cutaneous amyloidosis

Medin

Aortic amyloidosis in elderly people


NYHA Classification for Congestive Cardiac Failure

The New York Heart Association (NYHA) Functional Classification is used to grade the severity of congestive cardiac failure. Symptoms it refers to include dyspnea and chest pain.


NYHA Class Symptoms
I No symptoms and no limitation in ordinary physical activity, e.g. shortness of breath when walking, climbing stairs etc.
II Mild symptoms (mild shortness of breath and/or angina) and slight limitation during ordinary activity.
III Marked limitation in activity due to symptoms, even during less-than-ordinary activity, e.g. walking short distances (3-15 feet).
Comfortable only at rest.
IV Severe limitations. Experiences symptoms even while at rest. Mostly bedbound patients.

Complications of obesity

CVS - Hypertension, coronary artery disease, cor pulmonale, pulmonary hypertension of obesity, obesity related cardiomyopathy, atherosclerosis

CNS - Stroke, Benign intracranial hypertension

Neoplastic - Increased endometrial, prostate, gall bladder, breast and colon cancer

Endocrine - Non Insulin Dependent Diabetes Mellitus(NIDDM), abnormal lipid profile

GIT - cholecystitis, cholelithiasis, nonalcoholic steatohepatitis (NASH), fatty liver, gastro esophageal reflux disease(GERD)

Pulmonary - Obstructive sleep apnea, Pickwickian syndrome(hypoventilation)

Psychosocial -Depression, stigma,
Orthopedic - Osteoarthritis, slipped capital femoral epiphyses, Blount and Legg-Calvé-Perthes disease, backache

Gynecologic - Anovulation and infertility, hyperandrogenism and polycystic ovarian disease

Obstetric - Pregnancy induced hypertension, large baby

Surgical - Deep venous thrombosis, pulmonary embolism

Aspirin for MI/angina

162-325 mg of chewable aspirin should be promptly administered to patients who do not have bleeding tendency. Administration should not be delayed as the peak effect of aspirin can be seen as early as half hour after administration and it is important to prevent thrombus formation and propagation.

If the patient undergoes stenting 162-325 mg aspirin should be continued for 1 month after bare metal stent implantation, 3 months after sirolimus-eluting stent implantation, or 6 months after paclitaxel-eluting stent implantation.

Most subjects will get low dose (75-162 mg) all their life.
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