Pathology Notes

Meigs syndrome Updated: 05/20/2020 © Jun Wang, MD, PhD General features Triad Benign ovarian tumor, ovarian fibromas most common, may be seen with thecoma , granulosa cell tumor as well Ascites Pleural effusion Ascites and pleural effusion disappear after tumor removal Unclear pathophysiology, probably lymphatic drainage of irritation/secretion associated ascite Diagnosis of exclusion after ovarian cancer ruled out Clinical presentations Associated with ovarian tumor: mass, etc Associated with pleural effusion: Dullness of percussion, tachypnea, etc Associated with ascites: shifting dullness, etc Management Rule out malignancy Symptomatic support Surgical removal of ovarian tumor Back to syndromes Back to contents

Nevoid basal cell carcinoma syndrome Updated: 05/19/2020 © Jun Wang, MD, PhD General features AKA Gorlin syndrome, basal cell nevus syndrome, etc Autosomal dominant  Germline mutation of PTCH1 Multiple developmental abnormalities Increased risk for childhood medulloblastoma Pathogenesis Inactivation of PTCH1, receptor for hedgehog protein Loss of suppression of Smoothened (SMO) Probably downstream over activation of transcription factors Gli1 and/or Gli2 Clinical presentations Major manifestations Early development of basal cell carcinoma Odontogenic keratocyst Palmar and plantar pits Lamellar calcification of the falx cerebri Family history of nevoid basal cell carcinoma syndrome   Minor manifestations Childhood medulloblastomas Craniofacial anomalies: Cleft lip/palate, macrocephaly, frontal bossing , hypertelorism Vertebral/rib anomalies, such as bifid/splayed/extra ribs or bifid vertebrae Polydactyly Cardiac or ovarian fibromas , often b

Septic shock Updated: 06/16/2022 © Jun Wang, MD, PhD General features A subset of sepsis with profound circulatory and cellular metabolism abnormalities with marked increased mortality A type of distributive shock Most common type of shock Multiorgan failure due to dysregulated inflammatory response Despite adequate fluid resuscitation Persisting hypotension Requiring vasopressors to maintain a mean arterial pressure of 65 mm Hg or higher Serum lactate level greater than 2 mmol/L (18 mg/dL) Increased risk of mortality than sepsis alone Pathophysiology Mediators Microorganism factors Bacterial components: Endotoxin, peptidoglycan, muramyl dipeptide, lipoteichoic acid, superantigens, etc Bacterial products: Staphylococcal enterotoxin B, toxic shock syndrome toxin 1, Pseudomonas exotoxin A, M protein, etc Proinflammatory mediators: TNF-alpha, Interleukin-1, granulocyte colony stimulating factor, platelet activating factor, etc Complements Immune cell