22 September 2022: Textbook Updates

We have posted updates of the following topics:

Bone & joints > Arthritis > Gout and gouty arthritis
by Sahar Suleman, M.B.B.S., Madiha Bilal Qureshi, M.B.B.S., Nasir Ud Din, M.B.B.S.
Topic summary: Gout is a metabolic disorder that affects joints, bones, skin and soft tissues; associated with hyperuricemia, which causes saturation of monosodium urate and eventual formation of crystals within tissue. Crystal induced inflammation leads to acute, severe, self limiting joint pain. More common in men than women (4:1). Presents in 3 ways: acute gout, tophaceous gout and chronic arthropathy. Maori and Pacific ethnicity is recognized as a prognostic factor for more severe outcomes in gout. Long term treatment: urate lowering therapies include xanthine oxidase inhibitors (e.g., allopurinol or febuxostat), uricosuric drugs (e.g., probenecid) or uricase agents (e.g., pegloticase).

Colon > Noninfectious colitis > Mastocytic enterocolitis
by M.J. Fernández-Aceñero, M.D., Ph.D.
Topic summary: Rare condition, described in 2006, occurring in cases of chronic intractable diarrhea; not intended as a specific diagnosis. Chronic diarrhea is a frequent health problem, affecting 5 – 10% of the adult population. Normal endoscopy; increase in the number of mast cells (immunological cells derived from a CD34+ progenitor in the bone marrow) in the gastrointestinal biopsies with sheet and nest formation. Can also be associated with abdominal pain or bloating. Treatment: antihistamines (H1 and H2); mast cell stabilizers, including cromolyn; steroids; inhibitors of mast cell mediators, inhibitors of tryptase and chymase; leukotriene antagonists; kinase inhibitor.

Kidney tumor > Adult renal cell carcinoma – rare > Tubulocystic
by Daniel Anderson, M.D., M.B.A., Maria Tretiakova, M.D., Ph.D.
Topic summary: Renal epithelial neoplasm composed exclusively of tubules and cysts lined by a single layer of cells with prominent nucleoli (equivalent to ISUP / WHO grade 3) and abundant eosinophilic cytoplasm. Well circumscribed tumor with spongy bubble wrap appearance. Rare (< 1% of renal tumors); mean age: 58.4 years (range: 30 – 74 years). Often an incidental finding. Diagnosis is made on gross and H&E with attention paid to strict criteria of tubular and cystic growth. Relatively indolent with < 10% cases showing disease progression, local recurrence or metastasis to bone, liver and lymph nodes. Treatment: radical or partial nephrectomy depending on tumor size and location in the kidney.

Testis & paratestis > Collecting duct and rete testis tumors > Adenocarcinoma of collecting ducts and rete testis
by Burak Tekin, M.D., Rafael E. Jimenez, M.D.
Topic summary: Rare malignant neoplasm originating from the epithelium of rete testis and associated with an aggressive clinical behavior. Diagnosis of exclusion, with the differential diagnosis including mesothelioma, metastatic adenocarcinoma and germ cell tumors, among other entities. Extremely rare, with fewer than 100 cases reported in the literature. Predictors associated with a favorable outcome: Tumor size < 5 cm, tumors confined to the testis.Treatment: options for primary rete testis adenocarcinoma are not standardized; consists of radical orchiectomy with or without retroperitoneal lymph node dissection, in combination with adjuvant chemotherapy or radiotherapy.

Transfusion medicine > Transfusion reactions & complications > Red blood cell alloimmunization
by Sara Bakhtary, M.D.
Topic summary: Red blood cell (RBC) alloimmunization is the formation of antibodies to non-self RBC antigens (excluding naturally occurring anti-A and anti-B); only occurs after exposure through transfusion, pregnancy or transplantation. RBC alloimmunization can result in acute hemolytic or, more commonly, delayed hemolytic transfusion reactions, which can be life threatening. Higher rates of RBC alloimmunization occurs in patients with sickle cell disease (19 – 43%), thalassemia major (5 – 45%) and myelodysplastic syndromes (15%). There is no specific treatment for RBC alloimmunization; if a patient has an identified RBC alloantibody or multiple alloantibodies, an RBC unit negative for the implicated antigen(s) should be selected for transfusion.

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