focal nodular hyperplasia(NFH)
common in female, age 20-50
regenerative reaction to hyperperfusion from anomalous arteries at center of nodule
☆lighter than normal liver tissue and lobulated with stellate scar from which ☆fibrous septate radiates to the periphery
helical CT scan (which evaluates the mass during different phases of vascular contrast) generally reveals a hyperdense lesion (ie, filled with contrast during hepatic arterial phase) and a ★central scar.
the first symptoms/ associated conditions of primary biliary cholangitis(PBC)fatigue and ★pruritus (very severe especially at night)
★cutaneous xanthomas and xanthelasmas
(ascending cholangitis (★narrowing and dilatations of bile ducts that may result in “beading”, both intrahepatic and extrahepatic bile ducts) is associated with PSC and not with PBC)
epidermolysis bullosaEB simplex, is caused by mutations in keratin genes that ☆impair the assembly of keratin into filaments.
It presents in early in life with friction-induced blisters at the ☆palms and soles and other exposed areas.
Lesions typically heal with no residual scarring, although patients may have chronic thickening of the skin of the feet. Infants with EB simplex may develop ☆★oral blisters with bottle-feeding. dermatologyUWorldUWorld step2CK Jun 13, 2020, 5:12 AM HirotoShishido
parasomniasREM(beta waves)→ nightmare disorder, REM sleep behavior(patients remember the dream)(more common in adults)
☆↑Ach
NREM(N3, ☆delta waves)→ ☆sleepwalking, ★night terrors(no memory of the dream)(more common in children)
★Reassurance
Administer low-dose benzodiazepine at bedtime if episodes are frequent, persistent & distressing
placental aromatase deficiencyInability to synthesize estrogens from androgens.
Masculinization of female (46,XX DSD) infants (ambiguous genitalia),serum testosterone and androstenedione.
☆★Can present with maternal virilization during pregnancy (fetal androgens cross the placenta). reproductiveUWorldUWorld step2CK Apr 8, 2020, 5:50 AM HirotoShishido
alternative treatment for patients with gastroparesis who cannot tolerate metoclopramideerythromycin (macrolide)
the prokinetic effects of erythromycin stem from the activation of the motilin receptor in the smooth muscle of the upper digestive tract microbiologyUWorldUWorld step2CK Mar 28, 2020, 1:13 AM HirotoShishido