2015.04.14 Flashcards
(35 cards)
Administration of human chorionic gonadotropin (hCG)
Stimulates LH surge which causes ovulation.
Menotropin (human menopausal gonadotrophin) acts like FSH and leads to formation of a dominant ovarian follicle.
Potter Syndrome
Marked by pulmonary hypoplasia, limb deformities, and characteristic facies (suborbital creases, depressed nasal tip, low-set ears, and retrognathia) as a result of oligohydraminos.
Classically occurs in fetuses with bilateral renal agenesis, where lack of fetal urine causes oligohydraminos. (Amniotic fluid is required for proper lung development).
Lesch-Nyhan Syndrome
X-linked recessive disorder caused by a defect in hypoxanthine-guanine phosphoribosyltransferase (HGPRT). Elevated uric acid levels. Developmental delays and hypotonia.
This results in failure of the purine salvage pathway. Because they are not recycled, increased amounts of purine bases hypoxanthine and guanine are degraded to uric acid.
De novo purine synthesis must increase to replace the lost bases. (PRPP to form phosphoribosylamine in first committed step of de novo synthesis by Phosphoribosyl phosphate amidotransferase)
Pre-eclampsia
Triad of hypertension, proteinuria and edema in pregnancy.
May progress to HELLP syndrome (Hemolytic anemia, elevated liver enzymes, and low platelets)
Factor V Leiden
Modifed to resist activated protein C.
Resulting hypercoaguable state predisposes to DVTs, which are the source of most pulmonary emboli.
Glucose 6-phosphate dehydrogenase deficiency
Defect in the HMP shunt that impairs glutathione reduction due to failure to produce NADPH.
Glutathione reductase deficiency causes a similar clinical picture and is pathophysiologically similar to G6PD.
Testicular Hydrocele
Serous fluid accumulates within the tunica vaginallis
Tunica vaginalis is embryologically derived from the peritoneum; when it remains in communication with the peritoneum (patent processus vaginalis), a communicating hydrocele results.
Tunica albuginea
Fibrous tissue that overlies the corpus spongiosum and corpora cavernosa of the penis
Spermatic fascia layers are derived from what?
External spermatic fascia - aponeurosis of external oblique abdominal muscle.
Cremasteric - from internal oblique abdominal muscle and aponeurosis
Internal - from transversalis fascia. Immediately overlies the spermatic cord and tunica vaginalis
Psoriasis
Chronic inflammatory skin disorder characterized by sharply demarcated, salmon-colored, rounded plaques covered with a loosely adherent, silvery-white scale.
Cytokines lead to keratinocyte proliferation, inflammation, and angiogenesis.
Treatment: topical vitamin D analogs (calcipotriene, calcitriol, tacalcitol) which bind to vitamin D receptor (nuclear TF) and causes inhibition of keratinocyte proliferation/differentiation. Cyclosporine, Etanercept, Methotrexate, Ustekinumab
Cyclosporine
Inhibits NFAT (nuclear factor of activated T cells) from entering the nucleus and modulating transcription activity, impairing the production and release of IL-2 (and inhibiting IL-2 activation of resting T-lymphocytes)
Etanercept
Recombinat form of human TNF receptor that binds to TNF-a.
Methotrexate
Folate antimetabolite that reversibly binds to dihydrofolate reductase, resulting in inhibition of purine and thymidylic acid synthesis.
In psoriasis, thought to target rapidly proliferating epithelial cells in the skin.
Ustekinumab
Huamn monoclonal antibody that targets IL-12 and IL-23. Inhibits differentiation and activation of CD4 Th1 and Th17 cells.
Lambert-Eaton Myasthenic Syndrome
Disorder of NMJ manifesting with proximal muscle weakness (difficulty rising from chair, climbing stairs), cranial nerve involvement, autonomic symptoms (dry mouth or impotence).
Antibodies directed against voltage-gated calcium channels that participate in acetylcholine release.
50% patients have a malignancy (usually small cell lung cancer).
Differentiate from myasthenia gravis: hyporeflexia/areflexia, autonomic symptoms and classic incremental response to reptietive stimulation
Myasthenia Gravis
Autoantibodies against postsynaptic nicotinic acetylcholine receptors.
Leads to decreased numbers of functional acetylcholine receptors at the NMJ, which reduces the end-plate potential following acetylcholine release. Because threshold potential is not reached, muscle cells do not depolarize.
Improvement with cholinesterase inhibitors and with rest.
Treatment of Myasthenia Gravis
Cholinesterase inhibitor (pyridostigmine, etc) which causes excessive cholinergic stimulation (of gut, causing nausea, cramping, sweating, diarrhea)
Scopolamine is a selective muscarinic ACh receptor antagonist that will reduce the effects of cholinesterase inhibitors in sites where ACh action is mediated by muscarinic receptors. (Won’t affect nicotinic receptors - skeletal muscle). Hyoscyamine works as well.
Fever, vesiculoulcerative gingivostomatitis, and cervical lymphadenopathy
common clinical manifestations of primary infection with herpes simplex virus (HSV-1).
Reactivation of a latent HSV infection in the trigeminal ganglia generally results in more limited perioral blisters or “cold sores”
CFTR transmembrane protein
In respiratory and gastric glands, imapired functioning of CFTR reduces luminal chloride secretion and increases sodium and net water absorption, resulting in dehydrated mucus and a more negative transpeithelial potential difference.
In sweat glands, abnormal CFTR function causes reduced luminal salt absorption, leading to production of hypertonic sweat and high chloride content.
Nasal transepithelial difference is more negative than normal due to chloride ions retained at mucosal surface
Congenital Hypothyroidism
Presents soon after birth with hypotonia, poor feeding, jaundice, macroglossia, constipation, and umbilical hernia.
It should be diagnosed as early as possible to prevent the development of mental retardation.
First dose hypertension
adverse effect when initiating ACE inhibitors
predisposing risk factors: hyponatremia, hypovolemia secondary to diuretics, low baseline blood pressure, high renin or aldosterone levels, renal impairment, and heart failure.
High specificity antibody for Rheumatoid arthritis
Anti-cyclic citrullinated peptide antibodies
Rheumatoid factors
autoantibodies targeting the Fc portion of human IgG
present in RA, SLE, mixed cryoglobulinemia, and other inflammatory conditions
Acyclovir nephrotoxicity
excreted principally in the urine via glomerular filtration and tubular secretion.
when the acyclovir concentration in the collecting duct exceeds its solubility, crystallization, crystalluria, and renal tubular damage may result.
treatment: hydration
