Block 9 Flashcards
Retroperitoneal organs
Organs are :SAD PUCKERS Suprarenal gland Aorta and IVC Duodenum 2-4th parts Pancrease Ureter and bladder Colon (ascending and desencding ) Kidneys Esophagus Rectum (mid-distal)
Ovary epithelium
Cuboidal cell epithelium
Lumacaftor
Drug used in CF , it can partially correct the folding defect –> expression of functional CFTR.(in patients who have F508del mutation)
Reoviridae
Colorado tick fever
-rocky mountain states, charactrized by fever, vomiting , mylagia and weakness
Neagleria fowleri :
Causes rapid meningoencephalitis with a high mortality rate. Its found in fresh water, and transmission occurs through nasal inhalation during recreational activities (swimming,diving).
-symptomatic encephalitis, also taste and smell abnormalities. Inflammation in temporal and facial lobes, brain stem and meninges.
C-myc is
Transcription activator , the product of c-myc is a nuclear phosphoprotein that functions as a transcription activator controlling cell proliferation, differentiation and apoptosis.
Epithelium in genital tract
Ovary-> simple cuboidal
Fallopian tube,uterus , –> simple coulmnar
Cervix -> ectocervix -> stratified squamous non-keratinized
Endocervix –> simple cpulmnar’
Vagina -> stratified squamouse non keratinized.
Injury to intraperiotneal organs can lead to
Intraperitonieal bleeding (not hematoma)
Iron deficiency anemia : blood results
- Low ferritin values
- High Iron binding capacity / high transferrin
- Low MCV
- Decreased serum iron
- Decreased hemoglobin
- Microcytic microchromic
7.
TT measure
Thrombin time, measure the rate of conversion of fibrinogen to fibrin. Low in liver diseases. (Acquired dysfibrinogenemia in liver disease MCC)
Gullain barre syndrome
- ascending paralysis
- usually preceded by respiratory infection of GI infection.
- acute demyelinating disease with an immune mediated pathogenesis.
- antibodies against infectious ag’s cross react with mylin of spinal roots and PNS–> demylination.
- demyliniation leads to flaccid paralysis, areflexia, paralysis of respiratory muscles may occur as can paralysis of CN such as CN VII (bell palsy).
- CSF have increased proteins with normal or slightly elevated cell count.
Sarcoidosis, clinical, diagnosis
Inflammatory disease of unknown etiology, non-caseating granulomas.
- consist of epithelopoid cells (activated macrophages).
- multi-nucleated giant cells consistent with chronic granulomatous inflammation.
- typical patient: african american, non specific symptoms, lung involvement present in majority of patients.
- skin involvement : erythema nodosum (painful shin nodules).
- chset X-ray is essential for diagnosis.
X-linked agammaglobulinemia
Bruton tyrosine kinase gene causes failure of bone marrow pre B cells to develop into mature, circulating B lymphocytes. Patients have low or absent B cells.
- as a result they are at increased risk of infection with pyogenic (encapsulated ) bacteria. Also increased risk to viral infections such as enterovirus and giardia lamblia (parasite)
Xeroderma pigmentosum- defect in, clinical
- AR, extreme sensitivity to sun light, skin freckling ,ulcerations, and skin cancer.
- carcinomas and melanomas occurs early in life and most patients die of them.
- the most common deficiency is in excision endonuclease
- diagnosis is done by measuring the relative enzyme excision endonuclease in white cells of blood.
Ezetimibe
Decreases intestinal absorption of cholesterol by inhibiting the niemann pick C1- like 1 transport protein (NPCL1), which transport dietary cholesterol from GI lumen into intestinal entrocytes. As a result the total amount of dietary cholesterol reaching the liver increased LDL receptor expression, which draws cholesterol out of the circulation.
Craniopharyngyoma-microscopy
Rare, slow growing (benign ) intracranial tumors found in children and typically located Suprasellar location.
Microscopy shows: cystic, or partially cystic, usually filled with brownish yellow, viscous fluid that resembles machine oil due to the presence of protein and “cholesterol crystals”.
-dystrophic calcification of cyst is highly characteristic and may be detected on neuroimaging.
- cysts are lined by cords/nests of stratified squamous epithelium with peripehral palisading and internal areas of lamellar wet keratin.
Platelet function analyzer (PFA-100)
Monitors the time needed for patients blood to form a platelet based hemostatic plug in vitro.
Abnormal results are present in vWD, NSAID use and thrombocytopenia.
Anatomic area supplying stomach - prone to bleeding
- Proximal lesser curvature: left gastric
- Distal lesser curvature: right gastric
- Proximal greater curvature : left gastroepiploic (from splenic)
- Distal greater curvature : right gastroepiploic (from gastrodudenal)
- Proximal greater curvature above splenic artery : short gastric artery – > splenic
Ranibizumab ,bevacizumab
Anti VEGF – used to treat wet AMD and inhibit angio-genesis in cancer.
Asthma and COPD inflammatory cells
COPD: neutrophils, MAC, and CD8 T cell
Asthma : CD4+, eosinophils
Plantar wart
Warts, usually on the foot, caused by HPV 1,4 and have no malignant potential and doesn’t increase risk for CIN or CIS
Thoracic outlet syndrome
Compression of the lower trunk of the brachial plexus as it passes through the thoracic outlet causes (TOS) and present with upper extremity numbness, tingling and weakness. In addition, compression of the subclavian vein can cause upper extremity swelling whereas compression of the subclavian artery can cause exrertional arm pain .
-TOS most commonly occurs within scalene triangle, the brachial plexus and subclavian trunk passes between the anterior and middle scalenes , it can be due to anomalous cervical rib, scalene muscular anomalies or injury
Leydig cells
(Similar to theca cells )
-produces Testosterone in response to LH.
GnRH–> LH–> Leydig cells–> testosterone –> (-) feedback —> inhibit the axis
Milrinone indication
Inotropic agent in patients with refractory HF due to LV systolic dysfunction.
Dilated CM
Galactosemia vs hereditary fructose intolerance
.galactose is present in breast milk so as soon as breast feeding initiated –> symptoms , while fructose intolerance is manifested after 6 months ( after other foods are introduced in diet)
Deficiency in IFN-¥
-same mechanism as in IL-12. Receptor deficiency.
Autosomal recessive deficiencies of the IFN-¥ receptor result in disseminated myobacerial disease in infancy or early childhood, including disseminated infection by the BCG vaccine strain if administered. Once identified these require lifelong treatment with antimycobacterium antibiotics
Lynch syndrome is a consequence of
AD syndrome.
Nucleotide mismatch that escapes repair .
- the mismatch repair system involves several genese including MSH2 and MLH1–> codes for MutS and MutL homologs. –> mutation in these –> 90% of cases of lynch syndrome.
Sertoli cells
(Analogous to granulosa cells of females)
- produces inhibin hormone in response to FSH. Inhibin suppresses FSH production by anterior pituitary.
- sertoli cells also facillitates spermatogenesis within seminferous tubles.
Cocidiosis immitis on microscopy
- thick walled spherule
- packed with endospores
- some spherules may be empty
- others may be rupturing
- culture on sabouraud agar and serology are important for diagnosis.
Risk factors for developing cervical cancer
Sexual contact
Multiple partners
Lack of barrier contraceptive (condoms)
Net filtration pressure equation
Net filtration pressure = (Pc-Pi) -(πc-πi)
- positive values indicates filtration occurred, negative means absorption occurred
- hydrostatic pressure gradient ( Pc-Pi) is the difference in hydrostatic pressure in the capillaries(Pc) and interstitium (Pi). Its the main driving force of fluid efflux from the capillary.
-oncotic pressure gradient (πc-πi): its primarily dependent on the concentration of large proteins such as albumin, as large proteins usually cant cross capillary walls the oncotic pressure is generally high in capillaries and negligible in interstitum. The oncotic pressure counteracts the capillary hydrostatic pressure and decreased fluid efflux.
Vitamin A deficiency
Causes:
1. Insufficient dietary intake
2. Pancreatic insufficiency (CF, pancreatitis )
3. Cholestatic liver disease/ biliary obstruction
4. Intestinal malabsorption.
Clinical:
1. Nyctalopia.
2. Severe eye dryness and corneal ulceration.
3. Hyperkeratosis. (Xerosis cutis)
4. Growth retardation.
5. Bitot spots
Mutation of DNA repair enzyme
BRCA1 and BRCA2
Associated with breast, ovarian caners, lynch syndrome, xeroderma pigmentosum, fanconi anemia and others
CF is a result of (mutation)
Abnormal post-translational processing of transmembrane protein.
HPV strains
HPV 6,11 :–> condyloma acuminate (genital warts) and have low malignancy potential.
HPV 16,18,31,33: –> cervical, vaginal ,vulvular and anal neoplasia–> high oncogenic risk–>over expression of E6- E7 viral oncogenes –> E6 bind P53 (increases its degradation) while E7 bind to RB1 gene(displace transcription factors normally bound by pRB, the tumor supressor protein product of RB)
Nitrates effect
- Decrease TPR(they have modest effect on arteroilar dilation )
- Decrease EDLV pressure
- Increase venous capicatance
Why acei/arbs cant be combined with diuretics
ACEI/ARBS blocks RAAS pathway–> feedback increase in renin levels.
Diuretic induced hypovolemia –> decreased RBF –> increase renin and activates RAAS .
Lipid lowering agents :
- HMG-CoA reductase: statins
- Bile acid resins: chylomicrons, colesevelam
- Ezetimibe: prevent cholesterol absorption in intestinal brush border.
- Fibrates: gemifibrozil, fenofibrate: –> upregulate LPL= increased TG clearance, activates PPAR-å to induce HDL synthesis,
- Niacin(nicotinic acid) : inhibit lipolysis in adipose tissue, reduce hepatic VLDL synthesis.
- PCSK9 inhibitors: inactivation of LDL receptor degradation –> increasing amounts of LDL removed from blood stream (Evolocumab, and alirocumab)
Toxin penetration through blood nerve barrier
Cornybacterium diphteriae
Rubella vs rubeola rash
The rash of rubella typically spread faster, and doesnt darks or coalesce.
COPD inflammatory cells
- Cigarette smoke and other irritants –>
macrophage stimulation –> cytokine release – > CD 8+ cell–> alveoli destruction.
Cigarette smoke and other irritants –> macrophages –> neutrophils –> proteases secretion (neutrophil elastase, MMP9) ->mucus hyper-secretion in bronchi
Flecainide
Class 1c anti arrhythmic that typically used for supraventicular tachycardia such as those caused by atrial fibrillation.
- class 1c binds to fast Na+ channels responsible for phase 0 depolarization of cardiac myocytes, blocking inward sodium current -> prolongation of QT interval.
- class 1c are the slowest drugs to dissociate from the sodium channels and do so primarily during diastole. This result in a phenomenon known as use dependence, in which sodium blocking effects intensify as heart rate increase due to less time of drug to dissociate from the receptor.
TE fistula with Esophageal atresia
- normally trachea develops from diverticulum that form the primitive foregut–> tracheobronchial divericulum –> primitive foregut–> tracheobronchial lengthens and seperate to form the respiratory tract–> foregut becomes esophagus.
- failure to separate most commonly leads to esophageal atresia with a remaining fistula between trachea and esophagus.
- polyhydroamnios is present
- infant present with drooling, coughing, choking and cyanosis with feeds.
- diagnosis is made with X-ray showing position of nasogastric tube in atretic esophagus.
- X-ray shows stomach bubble,(result from air delivered from trachea to distal esophagus)
Tricohonoma is diagnosed using
Saline Microscopy(wet mount), motile flagellated protozoan (tumbling motility).
- clinically patients have erythmatous vagina, blue-green discharge and vaginal ph may be elevated
- strawberry cervix
Duchenne muscular dystophy
X-lined recessive disorder, caused by deletion resulting in frameshift mutation, non-sense mutation may also occur leading to formation of truncated dystrophin protein.
-clinically present with thigh atrophy, calf pseudoatrophy (gowers sign) elevated serum creatine kinase.
Exercise changes - cardiovascular
- HR and CO increased
- PaO2 and PaCO2 normal due to adaptations such as – > improved V/Q matching, and hyperventilation.
Viral infection –> mycoarditis
Most common cause of myocarditis in healthy, (adenovirus, coxacie B virus and parvovirus)
Hemophilia A and B
Clinical
Lab
Treatment
-X-linked recessive disease
- clinically : prolonged or delayed bleeding after mild trauma or procedure. –> hemarthrosis, intramuscular hematoma, GI or genitourinary tract bleeding.
LAB: prolonged Acitvated PTT
- normal platelet count, bleeding time, PT
-decreased or absent factor VIII(in Hemophilia A) or factor IX in hemophilia B.
Treatment: administration of factors VIII or IX, desmopressin for mild hemophilia A
Mantle cell lymphoma translocation
T11:14
Cyclin D1 on locus of chrom 11 and Ig heavy chain on chrom 14 –> mantle cell
Cyclin D = promoter of G1 to S phase transition during cell cycle.
Acute rheumatic fever on microscopy
Interstitial fibrosis with central lymphocytes and macrophages as well as scattered multi-nucleated giant cells. This interstitial myocardial granuloma or Aschoff body, is pathogenic for ARF.
-over the years Aschoff body is replaced by fibrous scar tissue –> MS and MR
Prolonged biliary obstruction can lead to
-Bile acids are important for solubilization and absorption of fats, patients with chronic billiary obstruction (due to PBC) may develop malabsorption of fat and fat soluble vitamin ( A,D,E,K) .
Hemosiderin deposits and endometrial glands or stroma outside the uterus
Endometriosis
HSV encephalitis
Pathogenesis: HSV -1 infection of the oropharynx travels through the olfactory tract or trigiminal nerves to invade temporal lobes
Presentation: acute onset of fever ,headache, seizures, aphasia, mental status/behavior changes.
MRI, CT and postmortem macroscopic brain examination reveals edema and hemorrhagic stoke of temporal lobe.
DIC –> RBC changes
Shistocyte
Measles (rubeola)
Confluent rash, beginning at head and moving down , preceded by cough coryza, conjunctivitis , and koplik spots.
CD15
Pesent on granulocytes. Its also present in nearly all reed stenberg cells –> useful marker in HD lymphoma.
Aldolase B deficiency
fructose 1 -p –> DHAP, and Glyceraldehyde
Fructose is converted from sucrose. So sucrose should be removed from diet.
Aldolase B is present in kidney and liver
-lethargy, vomiting,
-liver damage.
-hypoglycemia
-hyperuricemia
-[fanconi syndrome(renal PCT damage]
Rubella (german measles)
Pink macules and papules begins at head then move down -> remain discrete fine desqumating truncal rash –> post auricular lymphadenopathy .
Milrinone pharmacology
- phosphodiasterase 3 inhibitor –> increase cAMP levels –> Ca++ channel activation –> positive inotropy.
- increase cAMP levels –> cAMP dependent protein kinase, –> augmented Ca++ uptake by SR –> vasodilation.
Age related macular degeneration
Types
Pathogenesis
- the leading cause of blindness in indutralized countries. AMD occurs in genetically preisposed individuals as a result of advanceing age and envornoment factors (smoking) its classified to wet and dry.
- dry –> gradual vision loss in one or both eyes and can cause difficulty with driving/ reading ( due to oxidative damage to retinal pigment epithelium –> drusen with BM thickening). These are seen in retinoscopy as subretinal drusen deposits
- wet: progressive extracellular matrix accumulation can result in retinal hypoxia –> stimulates VEFG –> subretinal neovascularization with formation of leaky vessels. It present with acute vision loss with metamorphopsia ( distortion of straight lines)
Follicular lymphoma - translocation
T 14:18
BCL2 gene - > inhibit apoptosis
Retroperitoneal hematoma – injury to retroperitoneal organs
Commonly associated with abdominal and pelvic trauma. Pancreatic injury is a common cause. It can occur following a blunt or penetrating trauma.
-retroperitoneal hematoma can occur in any of the retroperitoneal organs.
Ethanol effect on glucose
Ethanol metabolism by alcohol dehydrogenease–>increases NADH/NAD ratio –> inhibition of all pathways requiring NAD including reactions required for gluconeogenesis. –> lactate can’t be converted to pyruvate.
- excess NADH –> inhibit conversion of malate to oxaloacetate. (Pyruvate and oxaloacetate are part of gluconeogenesis) > inhibition of gluconeogenesis.
Stages - sarcoidosis,
Stage I: bilateral hilar lymphadenopathy
Stage II: bilateral hilar lymphadenopathy with pulmonary infiltrates commonly in the upper lobes.
Stage III: lung infiltrates
Stage IV: lung fibrosis
Postauricular lymphadenopathy, with rash
Rubella , togavirus
Gullain barre syndrome – demylination accompanied with
demylination accompanied by an endoneural inflammatory infiltrate consisting of lymphocytes and macrophages.
Host defense against myobacterium
- depends on the interactions between macrophages and T cells .
- IFN-¥, pleiotropic TH1 cytokine is a key factor in the elimation of these infections
- MAC infected with myobacterium –> IL-12 production –>NK and Tcells production of IFN-¥.
- IFN-¥ binds to its receptos –> receptor dimerization and activation of janus kinases 1 and 2. The result is nuclear signaling via STAT1 and transcription of IFN-¥ regulated genes –> killing of myobacterium by phagocytes.
CLL special features Fa
- CD20,CD23,CD5 B-cell neoplasm
- Smudge cells
- Autoimmune hemolytic anemia
Intense itching after hot shower
polycythemia vera
