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Step 1 notes > Pathology - part 2 Respiratory, GI, etc > Flashcards

Pathology - part 2 Respiratory, GI, etc Flashcards

1
Q

PH (pulmonary hypertension)

cause, mechanism, patho,

A

mean PA pressue > 25/30 mm Hg at rest/exercise

mechanism 2° PH: respiratory acidosis/hypoxemia → vasoconstriction PAs
MCC 2° PH: left-sided valvular disease

Patho: atherosclerosis in main PAs
MC sym: exertional dyspnea
tapering PAs/RVH (right ventricular hypertrophy) on chest X-ray: cor pulmonale (= PH + RVH, 肺心病）

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2
Q

Goodpasture syn

cause? presentation?

A

Ab attack basement membrane in pulmonary cap and glomerular cap (type II hypersensitivity)

hemoptysis (咯血）followed by renal failure

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3
Q

RLD (restrictive lung disease)

defination? cause? 表现？

A

defination: ↓ TLC (total lung capacity)
cause: chest wall disorders (ie: kyphoscoliosis, mesothelioma, obesity, acute/chronic ILDs - interstitial lung diseases. ILD的一种，idiopathic pulmonary fibrosis: most MCC of RLD; sarcoidosis)

dry cough, inspiratory crackles (湿罗音）in lower lung fields, exertional dyspea

favor shallow, fast breath to ↓ work of breathing

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4
Q

Pathogenesis of interstitial fibrosis? Dx?

A

earlist manifestation: alveolitis [leukocytes release cytokines → fibrosis] → interstitial fibrosis

↓ compliance (↓ expansion during inspiration)
↑ elasticity (↑ recoil during expiration

Pulmonary function test: all volumes and capacities are equally ↓; normal to ↑ FEV1/FVC ratio (normal is 80% = 4L/5L), respiratory alkalosis (PCO2 < 33 mmHg）， ↓PaO2

X-ray: diffuse bilateral reticulonodular infiltrates

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5
Q

silicosis: X-ray特征改变？

A

胸片见egg-shell calcification in hilar nodes

MC occupational disease
opacities contain collagen and quartz (highly fibrogenic)

↑ risk for lung cancer/TB [differentiate: CWP (coal worker’s pneumoconiosis): no risk for lung cancer/TB]

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6
Q

if lung is encased, likely Rx?

A

mesothelioma (arise from pleura serosa)

no relationship with smoking

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7
Q

sarcoidosis:

etio? patho? Dx?

A

immune regulation disorder: CD4 Th cells interact with unknown Ag

noncaseating granulomas: Dx is exclusion (rule out other granulomas diseases)

lung is the target organ: dyspnea is the MC sym [patho: granulomas contain multinucleated giant cells]

UW case: 35-yr black woman, arthralgias+tender, deep nodules on legs, hepatomegaly (scattered granulomas), chest X-ray: enlarged hilar lymph nodes, serum ACE ↑[note: important lab for sarcoidosis]

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8
Q

Caplan syn?

A

cavitating rheumatoid nodules in the lung + pneumoconiosis

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9
Q

emphysema 肺气肿

patho?
how can smoking produce emphysema?

A

MCC: smoking; α1-AAT (α1 antitrypsin: antielastase) deficiency

patho: ↑ compliance, ↓ elasticity (能张开，但是无法recoil)
inbalance between elastase vs. antielastases; free radicals vs. GSH

cigarette smoke: chemotactic to neutrophils and macrophages ➞ release free radicals ➞ inactivate AAT/GSH

分为centriacinar vs. panacinar:
centriacinar: destruction of distal terminal bronchioles /upper lobe; panacinar: entire respiratory unit, lower lobe, no α1-globulin peak on serum protein electrophoresis

early dyspnea + late hypoxia (take time to destruct respiratory unit)
X-ray: ↑ hyperlucency, ↑ anterior-posterior diameter, vertically oriented heart, depressed diaphraghms
↑ TLC, RV; ↓ FEV1/FVC
↓ PaO2, normal to ↓ arterial PCO2 (pink buffer)

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10
Q

bronchial asthma

A

extrinsic (most, autoimmune) vs intrinsic (nonautoimmune)

extrinsic: type I HSR: allergen stimulates CD4 TH2 cells ➞ release IL4, IL-5 ➞ IL-4 stimulates isotype switching to IgE (release histamine, etc from mast cells）, IL-5 stimulates production and activation of eosinophils （major basic protein and cationic proteins to damage epithelial cells)

other mediators: leukotrienes (LTC-D-4， acetylcholine)

intrinsic (ie, viral infection, etc): normal IgE, negative skin tests to allergens

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11
Q

Bronchial asthma: alkalosis or acidosis?

A

initially: respiratory alkalosis (work hard to expell air, 过度通气）
If bronchospasm is not relieved, progress into respiratory acidosis

[normal pH or acidosis: indicates need for intubation !!!!]

FEV1 is the best measure of severity

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12
Q

MCC of bronchiectasis?

A

In US: CF

worldwide: TB

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13
Q

CF: cause? symptoms?

A

AR, MC fatal heretidary disorders in whites in US

3-nucleotide deletion in chr#7， coding for Phenylalanine: mutation causes defective protein folding in CFTR (CF transmembrane conductance regulator)➞ degraded in Golgi

CFTR is regulated by cAMP/ATP

CF: loss of NaCL in sweat (for Dx) /luminal secretion (dehydrated, thickened secretion)

nasal polyps in children, respiratory infections (MCC death in CF), chronic pancreatitis producing malabsorption, type I DM), infertility in males,

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14
Q

3 (patho/histological) types of pneumomia?

A
  1. bronchopneumonia: locally, involve lower or right middle lobe; pathy areas of consolidation
  2. interstitial pneumonia: inflammatory infiltration is confined to the alveolar walls
  3. lobar pneumonia: involve an entire lung lobe
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15
Q

stages and patho characteristics of lobar pneumonia?

A
  1. congestion (充血）：< 24 hrs [vascular dilation, alveolar exudate contains mostly bacteria], affected lobe is red, heavy and boggy
  2. red hepatization: 2-3 days [alveolar exudate contains RBCs, neutrophils and fibrin]; red, firm lobe, liver-lie consistency
  3. grey hepatization: 4-6 days [RBCs distegrate, alveolar exudate contains neutrophils and fibrin]
  4. resolution: enzymatic digestion of the exudate, restore the normal architecture
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16
Q

work of breathing change in obstructive vs. restrictive pulmonary disease?

A

obstructive (asthema, COPD): compliance ↓, airflow resistance ↑: favor lower rate/higher TV (tidal volumes): show, deep breath

restrictive (fibrosis, asbestosis): elasticity ↓, elastic resistance ↑: favor faster rate/low TV: fast, shallow breath

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17
Q

most sensitive test to rule out asthma?

A

Bronchial challenge test: test bronchial hypersensitivity. Give an aerosolized bronchoconstrictive substance required to produce a 20% ↓ in FEV1

usually use Methacholine

this test is sensitive but not specific. Used to exclude asthma

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18
Q

complication of give premature newborns concentrated O2?

A

retinopathy of prematurity: concentrated O2 ⇒ VEGF ↑ ⇒ abnormal retinal neovascularization

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19
Q

asbestosis: sym? light microscope? cancer related?

A

formation of fibrocalcific plaques on the parietal pleura, Subsequent diffuse pleural thickening and fibrosis of the lower lung lobes,

clinic: dyspnea, ↓ lung volumes (restrictive lung disease)

light microscope: interstitial fibrosis, asbestos bodies (ferruginous bodies， 铁锈色体): asbestos fibers coated with protein-iron matrix

risk for bronchogenic carcinoma ↑↑↑, MC cause of death; the 2nd leading cause of death is mesothelioma

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20
Q

TB: 病原体隐藏在what kind of cells? virulence factor? 肺部病理表现（分为primary vs, reactivation TB)?

A

organisms reside in phagasomes of alveolar macrophage! Produce ‘cord factor” (a protein to prevent fusion of lysosomes with phagosomes)
[note: humoral immunity plays no role in TB, because TB is an intracellular parasite in macrophages！】

TB特征：strict aerobe (like to be in upper /apex lobe), acid-fast 抗酸

primary TB: upper part of lower lobes, or lower parts of upper lobes; Ghon focus (= casecous necrosis: yellow-tan subpleural granuloma); Ghon complex (冈氏病灶）：caseous necrosis in hilar nodes

secondary (reactivation) TB: in immunosuppressed patients (HIV, corticoids, etc): upper lobe cavitary lesions (due to release of cytokines from memory T cells)

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21
Q

cough, hemoptysis, weight loss;
acid-fast bacillion a sputum culture, cavitary upper love pulmonary lesion on X-ray:

Dx？

A

reactivation TB

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22
Q

lung cancer histology: ovoid cells smaller than resting lymphocytes, immunostaining positive for chromogranin: Dx?

A

small cell carcinoma

strongly associated with smoking, centrally located;
round or oval cells with scant cytoplasm and large hyperchromatic nuclei; resemble lymphocytes, but smaller

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23
Q

Goodpasture syn?

A

IgG directed against basement mem. in pulmonary cap and glomerular ca. (type II HSR)

clinic: hemoptysis followed by renal failure

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24
Q

Churg-strauss syn?

A

autoimmune medium and small vessel vasculitis.

It usually (but not always) manifests in three stages. The prodromal stage is marked by airway inflammation: almost all patients experience asthma and/or allergic rhinitis; the second stage is characterized by hypereosinophilia: abnormally high numbers of eosinophils, which causes tissue damage — most commonly to the lungs and the digestive tract. The third and final stage consists of vasculitis, which can eventually lead to necrosis and is potentially life-threatening.

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25
Q

Dx: insidious-onset progressive exertional dyspnea, restrictive pulmonary function test, extensive interstitial fibrosis, “honeycomb lung”

A

Dx: idiopathic pulmonary fibrosis

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26
Q

Kaposi sarcoma口腔累及部分？caused by which virus?

A

hard palate; HHV-8

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27
Q

Behcet syndrome: cause? clinic? patho?

A

cause: enviromental + genetic factor (HLA-B51, HLA-B27 associated) 多见于土耳其人，地中海地区
clinic: triad (recurrent aphthous ulcers 口疮, uveitis 眼色素层炎，genital unclers)

patho: immune complex systemic small vessel vasculitis
can be fatal due to aneurysms or severe neurological complications

treatment: antiinflammatory medications, corticosteroids

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28
Q

Wickham striae: associated with?

A

丘疹表面形成的网状灰色线

squamous dysplasia/SCC

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29
Q

dentigerous cyst: associated with?

A

3rd molar, ameloblastoma (成釉细胞瘤）

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30
Q

benign tumors of the oral cavity?

A

squamous papillomas 鳞状细胞乳头状瘤：MC benign tumor in oral cavity

ameloblastoma 成釉细胞瘤）

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31
Q

oral SCC metastasis site?

A

superior jugular node (tonsillar node)

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32
Q

TE (tracheoesophageal) fistula 气管食管瘘

A

proximal esophagus ends blindly, and the distal esophagus arises from the trachea.

polyhydraminos (羊水过多；swallowed amniotic fluid can’t be reabsorbed in the small intestine); NB with stomach distention/feeding problem

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33
Q

VATER syn

A

verterbral abnomalities
A: anorectal
TE: tracheoesophageal fistula
R: renal disease + absent radius

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34
Q

Plummer- Vinson syn

A

chronic iron deficiency
intermittent dysphagia for solids

due to esophageal web (食管蹼）or stricture （狭窄）

↑ risk for SCC: pre-malignant condition

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35
Q

Zenker diverticulum (憩室）

A

MC pulsion diverticulum (食管压入性憩室）， mucosa/submucosa, located in upper esophagus, area of weakness is the cricopharyngeus muscle (环咽肌）

painful swallowing, halitosis （口臭）

Dx: surgery

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36
Q

define “Barrett esophagus”? complication?

A

seen in GERD (Gastro-esophageal reflux disease)

glandular (intestinal) metapliasia in distal esophagus due to acid injury

↑ risk for distal adenocarcinoma, stricture

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37
Q

Boerhaave syn: defination? cause? Dx?

A

rupture of distal esophagus

caused by retching/bulimia (贪食)/endoscopy (~ 75%)

air in the subcutaneous tissue in anterior mediastinum, crunching sound in physical exam; pleural effusion contains food, acid, amylase

Hematemesis; ↓ BP

Diagnosis: Often can be seen on X-Ray

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38
Q

Achalasia: cause? presentation?

A

MC neuromuscular disorder of esophagus, ↑ risk for esophageal cancer;

failure to relax LES (low esophageal spinkler): ↓ NO, VIP (vasoactive intestinal peptide)
Autoimmune destruction of myenteric (肠肌层）nerve fibers/inhibitory neurons in myenteric plexus

症状：1)nocturnal regurgitation undigested food; 2)dysphagia for solids + liquids (from High LES Opening pressure and uncoordinated Peristalsis leading to Aperistalsis)

3) chest pain, heartburn
4) nocturnal cough from aspiration

Dx: barium swallow shows “birds-beak”：narrowed esophagogastric junction

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39
Q

MC esophagus cancer in US?

A

distal adenocarcinoma

Barret esophagus is precursor lesion (not involve smoke)

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40
Q

MC primary esophageal cancer in developing countries?

A

SCC

MCC: smoke cigarettes
Alcohol abuse, lye strictures

dysphagia for solids + weight loss
tracheal invasion: dry cough + hemoptysis
hoarseness (invasion of recurrent laryngeal nerve)
hypercalcemia (↑PTH)

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41
Q

MCC for hematemesis (呕血，与咯血区别！！）

A

PUD (peptic ulcer disease)
esophageal varices (静脉曲张）
hemorrhagic gastritis

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42
Q

melena (黑粪症）: cause? indicator of?

A

Hb converted into hematin (black pigment) by acid，
signify bleed proximal to duodenojejunal junction (controlled by ligament of Treitz)

sign for upper GI bleed

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43
Q

Gastric analysis:分析什么指标？

A

BAO/MAO ratio (normal is 0.2 ： 1）

BAO： basal acid output (acid output of gastric juice collected via nasogastric tube over 1 hr on an empty stomach

MAO: maximal acid output (acid output collected over 1hr after pentagastrin stimulation)

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44
Q

gastroparesis: cause?

A

胃轻瘫

↓ stomach motility: autonomic neuropathy (eg: DM)

early satiety (饱满感),vomit undigested food

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45
Q

acute hemorrhagic gastritis: MC cause? clinic findings? treatment?

A

MCC: NSAIDs; alcohol second

H. pylori
smoking; burns (“Curling ulcers); CNS injury (“Cushing ulcers”)

Clinics: hematemesis, melena, iron deficiency

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46
Q

H. pylori

mechanisms of injury

A

Type B chronic atrophic gastritis

gram-negative rod; live in the mucus overlying the epithelium of the gastric antrum and fandus; produce urease (convert amino groups in protein to ammonia) - rapid test, turn urea solution (with pH indicator) into pink (pH ↑, due to more alkaline produced: urea converted into CO2 and ammonia)
colonizeds mucous layer, noninvasive

tests:

1) urea breath test: does NOT distinguish active vs. old infection
2) stool Ag test: positive = active infection; negative: no infection
3) gastric biopsy to detect urease: gold standard

H.pylori-associated antral gastritis ➞ somatostatin (inhibit gastrin) -producing antral cells ↓ ⇒ gastrin ↑ ⟹ histamine ↑ ⟹ gastric parietal cells secret more acid

NOTE: acid ⟹ duodenal ulceration, NOT gastric ulceration! H.pylori-associated gastric ulceration results from its destruction of the local mucous layer and the local inflammatory response; normal acid level is sufficient to cause ulcer if the mucus is degraded.

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47
Q

PUD (peptic ulcer disease): MCC?

A

MCC: H.pylori
duodenal > gastric
duodenal ulcers never malignant (do not need to biopsy)
gastric ulcers: small percentage malignant ➞ biopsy

4 layers in sequence are noted in histologic section of ulcers: necrotic debris ➞ inflammation (predominance of neutrophils) ➞ granulation tissue ➞ fibrosis

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48
Q

ZE syn (Zollinger-Ellison syn)

A

malignant tumor: ↑ gastrin, ↑ acid (loss of negative feedback)

duodenum MC site (NOTE: in distal, not proximal duodenum, different from most duodenum ulcer), pancreatic islets 2nd; single or multiple ulcers

PUD+diarrhea, family history of MEN type I syn (parathyroid or pituitary tumors), or PUD without H.pylori or history of NSAIDs

epigastric pain + weight loss, heartburn from GERD, peptic ulceration, acid hypersecretion with diarrhea, maldigestion of food (acid interferes with pancreatic E activities)

Lab: ↑BAO/MAO, ↑ serum gastrin

Dx: ulcer in distal duodenum or other atypical location, multiple ulcers, refractory to therapy, recurrence of ulcers after acid-reducing surgery

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49
Q

1° stomach adenocarcinoma: types? association with H.pylori?

A

2 types:

1)intestinal type of gastric adenocarcinoma (MC, due to H.pylori: most important; nitrosamines, smoked foods in Japan)
lesser curvature pylorus /antrum MC sites

2） diffuse type of gastric adenocarcinoma: NOT associated with H.pylori; “linitis plastica” 皮革胃，
signet ring cells infiltrate the stomach wall ➞ Krukenberg tumor of ovaries

metastasis to left supraclavicular (Virchow) node;
acanthosis nigricans (黑色棘皮症）; seborrheic keratoses (脂溢性角化症; Leser-Trelat sign)
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50
Q

signet ring cells

A

diffuse type of gastric adenocarcinoma: mucin produced by the cancer cells pushes the nucleus to the periphery

spreads to both ovaries hematogenously

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51
Q

MCC for extranodal malignant lymphoma?

A

stomach: low-grade B-cell lymphoma, related to H.pylori

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52
Q

types of diarrhea? differentiate?

A

invasive (pathogens invade enterocytes); secretory loss of isotonic fluid - laxative), osmotic (hypotonic: lactase deficiency: disaccharidase deficiency in brush border)

to diff: 1) fecal smear for leukocytes (+ for invasive)
2) stool osmotic gap: normal = 300 mOsm/kg

< 50 mOsm/kg from normal: isotonic loss: secretory
> 100 mOsm/kg from normal: hypotonic - osmotic

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53
Q

MCC for pancreatic insufficiency

A

in adults: alcohol
in children: CF

malabsorption of fat + proteins, but NOT carbohydrates （amylase in salivary glands, disaccharidases in brush border epi cells in intestine)

pancreatic lipase: hydrolyzed TG to MGs (monoglycerides) + FAs

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54
Q

tests to differentiate sites of malabsorption

A

D-Xylose [zailouse] screening test: ↓ indicates small bowel disease (does NOT involve pancreatic E for absorption)

serum immunoreactive trypsin: specific for pancrease
↓ in chronic pancreatitis (note: ↑ in early CF)

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55
Q

Celiac disease

A

autoimmune to gluten in wheat
greatest association with dematitis herpetiforms (疱疹样皮炎）

Patho: multiorgan autoimmune disease; T-cell and IgA-mediated response against gluten in genetically predisposed people

tTG (tissue transglutaminase) plays pivotal role ⟹ atrophy/flattened intestinal villi, lengthening of crypts, inflammation infiltration

Dx: ↑ anti-tTG IgA, EMA-IgA (antiendomysial IgA， antigliadin IgA, IgG

clinics: steatorrhea, weight loss, fail to thrive (in children)

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56
Q

Whipple disease

A

caused by Tropheryma Whipplei
Whipple病是一种罕见的传染性疾病，Tropheryma whipplei是一种革兰氏染色阴性的细菌，也是基因组最小的细菌。它所引起的Whipple病主要症状包括体重减轻、腹泻、关节疼痛等。此外，Whipple病无孔不入，心、肺、神经系统均有累及。归类在malabsorption中。
Dr. Whipple因为发现肝脏在恶性贫血中的作用而获得1934年的诺贝尔生理学奖。

microscope: blunting of villi, foamy macrophages in lamina propria (特征性形态，和Neiman picks鉴别）

Clinic: steatorrhea, fever, polyarthritis, generalized lymphadenopathy, ↑ skin pigmentation

Rx: antibiotics

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57
Q

direct hernia： location?

A

triangle of Hesselbach (黑塞尔三角）

area of the anterior abdominal wall bounded by the inferior epigastric vessels （腹壁下AV), inguinal ligament （腹股沟韧带）and the lateral border of the rectus abdominis （腹直肌）. Direct inguinal hernias leave the abdomen through this triangle.

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58
Q

area blood supplied by SMA (superior mesenteric artery)? IMA (inferior mesenteric A)?

A

SMA: most of small bowel + ascending and transverse colon up to the splenic flexture

IMA: descending and sigmoid colons + proximal rectum + upper half of the anal canal

SMA and IMA junction: watershed area (splenic flexture)

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59
Q

causes for acute ischemia of small bowel?

A
  1. Acute mesenteric ischemia:1) embolism from left side of heart to the SMA (AF: MC predisposing arrhythmia); 2) thrombosis of the SMA
  2. nonocclusive ischemia:
    1) hypotension (2° to HF)
    2) hypovolemic shock
    3) taking digitalis
  3. mesenteric vein thrombosis
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60
Q

SB (small bowel) infarction: clinic? radiographic?

A

sudden onset of diffuse abdominal pain (全腹痛）， bloody diarrhea, absent bowel sounds, distention, NO rebound tenderness （反跳痛，腹膜炎体征）early in infarction

on CT: 1） “thumbprint sign” -edema in bowel wall
2) bowel distention with air-fluid levels similar to bowel obstruction

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61
Q

ischemic colitis: location? clinic?

A

splenic flexture pain (SMA ends and LMA begins: watershed of blood supply)-mesenteric angina after eating ➞ fear of eating and weight loss; bloody diarrhea

Barium study shows thumbprinting of the colonic mucosa

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62
Q

angiodysplasia: location? clinics?

A

dilation of cecal (盲肠的；cecum) submucosal venules; hematochezia (便血）， 2nd MMC hematochezia (1st MCC is sigmoid colon diverticula)

more likely to bleed if genetic/required vWD present

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63
Q

Meckel diverticulum:

A

vitelline （卵黄的) (omphalomesenteric 脐肠系膜的) duct remnant

Most common congenital anomaly of the GI tract

5 2’s: 2 inches long, 2 feet from ileocecal (回盲肠）valve, 2% of the population, Presents in first 2 years of life, Can have 2 types of epithelium (gastric and pancreatic - maybe even endometrial)

BUZZWORDS: Contains Ectopic Gastric, Pancreatic or Endometrial Tissue

Kid with massive anal bleeding with no obvious upper GI, colon or hemorrhoid cause; NB with fecal material in umbilical area

clinically can’t distinguish from acute appendicitis

Dx: 99mTc (technecium, 鍀）nuclear scan identifies parietal cells in ectopic gastric mucosa

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64
Q

Sigmoid colon diverticular disease

A

herniation mucosa/submucosa thru muscularis (sigmoid colon is the most common site for diverticula in the entire GI): mesenteric border; area of weakness where vasa recta penetrate.

MCC; constipation
MC complication: diverticulitis

clinic: fever, initial diarrhea ➞ constipation, left lower quadrant pain (“left-sided appendicitis”)

best Dx: CT-scan
MC for hematochezia and fistulas

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65
Q

IBS (irritable bowel syn)

A

intrinsic colonic motility disorder, responsible for > 50% referral to gastroenterologists

alternating constipation/diarrhea; ↑ mucus in stool

Rx: adequate fiber intake

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66
Q

carcinoid tumor

A

MC SB tumor, malignant neuroendocrine tumors

depending on the location and size, they either invade or metastasize

MC location: vermiform （蠕虫样）appendix （bright yellow)-rarely metastasize
If in terminal ileum: metastasis to liver➞ cause carcinoid syn.

症状全是由liver代谢serotonin产生的5’-HIAA引起：
5’-HIAA is excreted in the urine ➞ for Dx

clinic: 1) flushing (serotonin ➞ vasodilation in skin)
2) diarrhea (serotonin ➞ bowel motility ↑)
3) wheezing, dyspnea (serotonin ➞ bronchospasm)
4) tricuspid regurgitation, pulmonary stenosis (serotonin ➞ collagen production in the valves)
5) facial telangiectasia

Dx: ↑ urine 5’-HIAA, CT scan of liver, Scanning for 1° location and metastasis

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67
Q

acute appendicitis

A

patho:

1) children: lymphoid hyperplasia 2° of a viral infection
2) in adults: fecalith obstruction (similar to acute diverticulitis)

pathogens: E coli, Bacteroides fragilis

特征性疼痛（转移）：initially colicky periumbilical pain (afferent C fibers on viseral pertitoneal surface: refer pain to midline) ➞ then shifts to RLQ in 12-18 hrs (Aδ fibers localize pain)

pain PRECEDES nausea and vomiting (important to Dx children！！）

MC complication: periappendiceal abscess
pyelophlebitis (肾盂静脉炎）：infection of the portal vein, danger of portal vein thrombosis
-subphernic abscess: persistent postoperative fever

Dx: spiral CT

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68
Q

congenital pyloric stenosis (CPS)

A

may associated with genetic factors (if parents affected, ↑ risk for child); male > famale baby

patho: caused by progressive hypertrophy of the muscles in the pyloric sphincter (↓ NO synthase)

NOT present at birth: occurs at 3-5 wks
projectile vomiting of non-bile-stained fluid; hypertrophied pylorus can be palpated in the epigastrium, “olive-sized” mass if felt in the right upper abdomen

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69
Q

chronic mesenteric ischemia: cause? characteristics? Dx?

A

atherosclerosis, ↓ blood flow (patho similar to angina pectoris)

特征性症状：
1） epigastric (上腹部）or periumbilical pain, 30-60 min after meals: very severe pain, patients fear to eat, but no findings on physical exams
2) weight loss

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70
Q

Abetalipoproteinemia: cause? clinics? microscope?

A

microscope: normal intestinal mucosal architecture, but the enterocytes contain clear or foamy cytoplasma prominent at the villi tips: accumulation of lipids (此病特征）；
cause: impaired formation of ApoB-containing lipoproteins

AR, loss-of-funciton of MTP gene (microsomal triglyceride transfer protein， chaperon for ApoB folding and tranfer lipids to chylomicrons and VLDL). Lipids absorbed by the SB can’t be transported to blood, so accumulate in the intestinal epithelium ⟹ enterocytes with clear or foamy cytoplasm

lab: very low serum TG and cholesterol; absent chylomicrons, VLDLs, apoB
clinic: < 1yr发病，malabsorption （abdominal distention, foul-smelling greasy stool); ↓ fat-soluble vit ⟹ acanthocytes, neurological abnormalities

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71
Q

adenoma-to-carcinoma sequence

A

adenomatous polyps: premaligant
> 50 yr patients, mutation of APC gene

stepwise progression with gene mutation: PAC inactivation ⟹ COX2 overexpression ⟹ K-ras acivation, DCC, p53 inactivation

↑ COX2 activity in some colon adenocarcinoma, and in inherited polyposes syn; Aspirin (COX inhibitor) can ↓ adenomas

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72
Q

Mallory-Weiss syn

A

longitudinal mucosal tear in esophagus-gastric junction
due to severe retching, often associated with alcoholism or bulimia (暴食）. tear occur 2° to rapid ↑ of intraabdominal /intraluminal gastric pressure

hematemesis

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73
Q

differentiate ulcerative colitis vs Crohn’s diease

A

both can have bloody diarrhea (both are inflammatory bowel disease). They are inherited as a complex, multigenic disorder (non-Mendelian manner). Candidate genes: HLA association (HLA-DR1/DQW5 in Crohn’s, HLA-DR2 in ulcerative colitis) and NOD2 (triggers NF-kB pathway ⟹ cytokines)

ulcerative colitis:

1) rectum always involved
2) inflammation: limited to mucosa and submocosa ONLY
3) mucosal damage is continous
4) . bloody diarrhea, with or without pain, is HALLMARK

collection of neutrophils within crypt lumina

Crohn’s disease:
transmural inflammation, perianal fistulae, non-caseating granuloma

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74
Q

serological findings for HAV: IgM? IgG?

A

anti-HAV IgM: infeciton

anti-HAV IgG: recovery/vaccination

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75
Q

Reye Syn

A

associated with Aspirin+ infection (chickenpox, influenza, etc): reason not to give Aspirin to children!!! fatal, damage liver and brain; usually in children < 4 yr

Postinfectious triad: encephalopathy, microvesicular fatty change of liver, ↑ transaminases, bilirubin, PT, ammonia, ↓ glucose (hypoglycemia)

76
Q

cause for hepatic artery infarction?

A

liver transplantation

PAN (polyarteritis nodosa)

77
Q

portal vein thrombosis: cause? clinic?

A

cause: 1) pylephlebitis (门静脉炎）： MC due to acute appendicits, air in the portal v from bacterial gas;
2) polycythemia vera 真性红细胞增多: hyperviscosity of blood
3) hepatocellular carcinoma

clinic: ascites, PH, air in PV, splenomegaly, NO HEPATOMEGALY!!

78
Q

Budd-Chiari syn

A

Hepatic vein thrombosis

MCC: 1)polycythemia vera
2) hypercoagulable state; 3) hepatocellular carcinoma

painful hepatomegaly, splenomegaly, fever, PH, ascites

Dx: Pulse Doppler Ultrasound, MRI

79
Q

Alcoholic hepatitis病理？

A

acetaldehyde damages hepatocytes

fatty change, neutrophil infiltration, mallory bodies (ubiquntinaited cytokeratin intermediate filaments in inclusions in hepatocytes)

80
Q

Dx for acute cholecystitis?

A

Nuclear medicine hepatobilitary scans (HIDA scan): fail to visualize gallbladder

If Dx with US: distended gallbladder, thickened wall, pericholecystic fluid, positive sonographic Murphy sign

81
Q

mechanism for E2 to affect gallstone?

A

E2 ↑ hepatic HMG-CoA reductase: this enzyme ↑ synthesis of cholesterol ⟹ cholesteral gallstone formation

82
Q

effect of 7α-hydroxylase on gallstone formation?

A

7α-hydroxylase converts cholesterol into bile acids ⟹ ↓ cholesterol within the bile

suppress this enzyme by fibrate medications ↑ concentrations of cholesterol in the bile

83
Q

function of β-glucuronidase in gallstone formation

A

β-glucuronidase de-conjugates bilirubin. The resulting free bilirubin complexes with calcium ⟹ precipitates in the bile

↓ activity of β-glucuronidase ⟹ ↓ pigmented gallstones

84
Q

types of gallstone?

A
  1. cholesterol gallstones: yellow, radiolucent
  2. pigmented gallstones:
    1) black: sign of chronic extravascular hemolytic anemia (sickle cell anemia, hereditary spherocytosis)

2) brown gallstones: (Asians, infection in CBD): infection de-conjugates CB, ⟹ ↑UCB in bile, cause the brown pigment stones

85
Q

MC benign tumor of liver?

A

cavernous hemangioma 海绵状血管瘤

先天性，和hyperplasia, hypertention无关。不能活检，引起大出血

86
Q

which liver tumor can regress with the discontinuation of OCP (oral contraceptives)?

A

hepatic ademonas (note: 与cavernous hemangioma区分！后者不受避孕药影响）

87
Q

PBC (primary biliary cirrhosis)

A 
autoimmune destruction (CD8+ T cell) of bile ducts in triads
female dominant, associated with other autoimmune disease (ie, Sjogren Syn)

↑ anti-mitochondrial Ab (AMA), ANA, IgM

precirrhotic stage: interlobular bile ducts are destroyed by granulomatous inflammation; heavy portal tract infiltrate of macrophages, lymphocytes, plasma cells, and eosinophis - similar to GVHD

late stage: necrosis, generalized cholestasis; gross: liver stains green + uniformly micronodular

Cx: early pruritus, late jaundice

88
Q

Budd-Chiari syn

A

Hepatic vein thrombosis

MCC: polycythemia vera

hypercoagulable state: ie OCP, protein C and S deficiency, hepatocellular carcinoma

Cx: enlarged, painful liver; PH, ascites, splenomegaly

BUZZWORDS: Seen with Hepatic (Posthepatic) Venous Thrombus that occludes Hepatic Vein or IVC

89
Q

Wilson disease

A

AR, mutation in ATP7B gene in chr#13: copper metabolism disorder: ↓incorporation copper into ceruloplasmin (血浆铜蓝蛋白， protein synthesized in the liver, contains copper) , ↓ excretion copper into bile ⟹ precipitate in cornea (Kayser-Fleischer ring， detected by slit lamp) and basal ganglia (PD-like syn)

young patients (3 -40), liver and brain damaged
acute, fulminate, chronic hepatitis, PH, cirrhosis;
PD-like tremor, slurry speech, depression,

hemolytic anemia, renal disease (damage proximal tubule ⟹ Fonconi syn)

Dx: liver biopsy finding quatitative liver copper > 250 mcg/g, ↓ serum ceruloplasmin

Rx: Penicillamine (Cu chelator)

90
Q

Acute hepatitis B 症状？

A

“serum sickness-like” (malaise， fever, skin rash, pruritis, joint pain) first; after these sym are gone, surge of ALT and AST (ALT > AST), ⟹ then ↑ bilirubin, Alkaline phosphatase

91
Q

hereditary hemochromatosis

A

AR, male dominant
missense mutations in HFE gene on chr 6, ⟹ mutation in sensor for circulaiting iron in basallateral epi in intestine ⟹ unrestricted iron reabsorption by duodenum ⟹ iron stimulates production of hydroxyl free radicals ⟹ liver damage

“bronze diabetes” (skin hyperpigmentation + DM + cirrhosis with hepatomegaly) , malabsorption, restricted cardiomyopathy, degenerative arthritis

↑ serum iron, % saturation, ferrintin, ↓ TIBC (total iron binding capacity)

Rx: % saturation is the best screen
serum ferritin used to follow Rx
gene testing best for screening relatives

liver cirrhosis and hepatocellular carcinoma are the 2 most ominous potentail complications

women tend to present later due to the blood loss during menstruation and pregnancy

92
Q

How does HBV cause hepatocyte damage?

A

HBV itself has NO cytotoxic effect itself. The presence of viral HBsAg and HBcAg on cell surface, with MHC-I, stimulates the host’s cytotoxic CD8+ T cells to destroy infected hepatocytes.

93
Q

stages of alcohol-associated hepatic injury? Px?

A

3 stages: steatosis (reversible), alcoholic hepatitis (resersible), cirrosis (irreversible)

hepatocyte damage (ALT, AST, etc） ⟹ loss of hepatic function (↑ PT, ↓hypoalbuminesia: indicator of liver failure and poor Px)

94
Q

acute acalculous cholecystitis

A

无结石胆囊炎

common in hospitalized patients; arise secondary (after sepsis, immunosuppression, major trauma, burns, infection, etc) to gallbladder stasis and ischemia

与胆结石引起的胆囊炎区别：无结石胆囊炎有jaundice and palpable right upper quandrant mass (胆囊突出肋骨缘）

结石胆囊炎：Murphy sign

95
Q

porcelain gallbladder indicate?

A

a rim of calcium deposits outline the gallbladder; associated with gallbladder carcinoma;

96
Q

markers for?

AFP
CEA (carcinoembryonic antigen)
CA-125
prostate-specific Ag
acid phosphatase
human chorionic gonadotrophin
A

AFP - hepatocellular carcinoma
CEA (carcinoembryonic antigen) - colon cancer
CA-125: ovarian cancer
prostate-specific Ag: screen for prostate cancer
acid phosphatase: 2° prostate cancer Ag, also in active osteoclast-initiated bone resorption
human chorionic gonadotrophin: gestational trophoblastic disase

97
Q

viral hepatitis on liver biopsy?

A

panlobular lymphocytic infiltrations, ballooning hepatocytes, necrosis, apoptosis

Apoptotic hepatocytes form round acidophilic bodies (Councilman bodies, or apoptotic bodies)

98
Q

pathogenesis of alcohol-induced hepatic steatosis

A

alcohol DH (dehydrogenase), aldehyde DH ⟹ ↑ NADH production ⟹ ↓ FA oxidation ⟹ accumulation of TG in hepatocellular cytoplasm

99
Q

black gallstone is associated with?

A

intravascular hemolysis (sickle cell anemia, β-Thalassemia, hereditary spherocytosis) - when circulating unconjugated bilirubin ↑; often seen in rural Asian populations

gallstone types: cholesterol, black, brown (infection)

100
Q

patho for brown gallstone

A

infection damages hepatocytes ⟹ release of β-glucoronidase, ⟹ hydrolysis of bilirubin glucuroninides ⟹ ↑ unconjugated bilirubin in bile

101
Q

azotemia: types? cauases?

A

azotemia: ↑ serun BUN + creatinine (Cr)

urea: filtered; partly reabsorbed in proximal tubules
creatinine: filtered

serum BUN/Cr ratio: normal = 15

1) prerenal azotemia: ↓ CO (hypoperfusion of kidney), ↓ GFR, ↑ BUN: ratio > 15
2) renal azotemia: intrinsic renal disease, extra loss of urea: ratio ≤ 15
3) postrenal azotemia: obstruction past kidneys (stone, cancer, etc); initially ratio > 15; ≤ 15 if obstruction persists to damage parenchymal

102
Q

Linear immunofluoresence in kindey: indicate what disease?

Granular IF?

fusion of podocytes?

A

linear IF: anti-GBM disease (e.g: Goodpasture Syn)

granular IF: immunocomplex deposition in the glomerulus (if do this under EM: dense deposits: the normal BM has a light grey apprearance)

fusion of podocytes: nephrotic syn

103
Q

Nephritic syn vs. nephrotic syn

A

Nephr(i)tic syn (肾病综合症）：neutroph(i)l-related injury to glomerul(i)

Cx: HT due to salt retention; periorbital puffiness/pitting edema, ↑ plasma hyrdostatic pressure, oliguria (~ 400 mL/day, due to ↓ GFR in inflamed glomeruli), moderate proteinuria (150 mg ~ 3.5 g/24 hr), hematuria, dysmorphic RBC, neutrophils are present in the urine sediment. Tubular function is intact!

Key to Rx: RBC casts

Nephr(o)tic Syn: cyt(o)kine injury p(o)docytes; negative charge on GBM is lost, etc ⟹ ↑ permeability of glomerular cap, MASSIVE protein loss (特征）, ↑ liver synthesis of albumin can’t compensate ⟹ ↓ colloid osmotic pressure ⟹edema ⟹ decreased intravascular volume ↓ renal perfusion pressure ⟹ 1) activation of renin-AngII- aldoesterone, 2) secrete antidiuretic hormone, both cause water and sodium retention

Key Rx: proteinuria > 3.5 g/24 hr, fatty casts (特征：蛋白尿和脂肪尿显著）

Generalized pitting edema and ascites due to hypoalbuminemia ⟹ ↓ plasma oncotic pressure
hypercoagulable (lose antithrombin III), hypercholesterolemia, hypogammaglobulinemia

104
Q

Diabetic glomerulopathy

A

MCC for CRF in US, type I DM > type II

non-enzymatic glycosylation of the GBM and tubular BM: ↑ permeability to protein

hyaline arteriolosclerosis, involve efferent arterioles before afferent arterioles;
glucose is converted by aldose reductase to sorbitol ⟹ ↑ osmotic damage

microangiopathy: ↑ deposition of type IV collagen

1st lab sign: microalbuminuria (Rx: ACE inhibitor)

105
Q

patho of BNS (benign nephrosclerosis)

A

hyaline arterioslcerosis

kidney of essential HT

Malignant HT: preexisting BNS MCC
hyperplasic arteriolosclerosis (onion skin lesion); > 210/120 mmHg, encephalopathy, RF
Rx: IV nitroprusside

106
Q

renal cell carcinoma: MCC?

A

smoking MCC
Von Hippel-Lindau disease： AD, chr3; translocation with loss of VHL suppressor gene (if see upper pole mass with cysts and hemorrhage: reminder of VHL)

bright yellow tumor with renal vein invasion; cancer derives from proximal tubular cells

microscope: clear cells contain lipid

invade renal vein, inferior vena cava （extend to right heart) ⟹ poor Px
metasize to lung, bone, regional nodes

RCC triad: hemauria, flank pain, abdominal mass

ectopic secretion of EPO (⟹ 2° polycythemia) and PTH-related peptide (hypercalcemia)

107
Q

mechanism of Enalapril on GFR?

A
  1. Enalapril (依那普利）：ACE inhibitor: inhibit Ang II
  2. Ang II: 1) systemic vasocontriction; 2) preferentially constrict efferent arteriole; 3) ↑ adrenal cortical aldosterone secretion

Enalapril acutely ↓ systemic vascular and efferent arteriolar resistance, both ⟹ ↓ GFR

108
Q

tumor lysis syn: happen in which segment of kidney?

A

distal tubules and collecting ducts

in chemo for lymphomas, leukemia, etc （rapid cell turnover and high sensitivity to chem）： rapid destruction of cells during chemo ⟹ release intracellular ions and uric acids are released into blood

the lowest pH along the nephron is in distal tubules and collecting ducts ⟹ uric acid precipitation

risk of tumor lysis syn can be ↓ by urine alkalinization and hydration

109
Q

IgA nephropathy can be caused?

A

1) HSP (Henoch-Schonlein purpura) 2) Berger disease
both: mesangial proliferation and crescent formation, 看肾脏无法区分

HSP: triad (abdominal pain + joint pain + palpable purpura on buttocks and lower extremities); hemauria

Berger disease: children + young adults; painless hematuria 2-3 days after upper RT infection; hematuria lasts for several days and subsides, but return every few months.

110
Q

RBC casts: Dx?

fatty casts?

A

RBC casts: nephritic syn (IHC: “starry sky”)

fatty casts: nephrotic syn

111
Q

PSGN (poststrepococcal glomerulonephritis

A

BUZZWORDS: Kids after Pharyngeal or Skin Infection (Mosquito Bite);

usually group A beta-hemolytic streptococci (impetigo, 脓疱病）
Immune Complex Mediated; - nephritic syn一种（RBC cast)

Cx: periorbital edema, cola-colored urine, oligouria, HT; ↑ anti-streptococcal Ab, ↓ serum C3

IHC: “starry sky”: granular deposits of IgG, IgM, C3 in GBM and mesangium
EM: subepithelial humps (electron-dense deposit of Ag-Ab complexes at the epithelial surface)
光镜：hypercellular glomerulus

Important factor for Px: age (if in adults: poor Px, 40% will become chronic GN)

112
Q

linear deposits of Ig： Dx?

A

anti-GBM = Goodpasture Syn

deposit: IgG (anti-Type IV col), C3 [important: GBM is composed of type IV collagen]

Differential: IgM and C3 deposits are found in focal segmental glomerulosclerosis, but no linear deposit of Ig

113
Q

特征Dx for Goodpasture Syn?

A

crescents in the glomeruli on light microscopy +
under fluorescence: see linear deposits

Cx: pulmonary (hemoptysis) + kidney (hematuria)

属于type I RPGN (rapid progressive glomerulonephritis),

114
Q

Dx: crescent formation under light microscopy?

A

RPGN (rapid proliferative/crescentic glomerulonephritis)

fibrin deposits是crescent formation关键一步

115
Q

types and characteristics of RPGN (rapid progressive GN)?

A

All have crescent formation, fibrin deposition

Type I: anti-GBM Ab (Goodpasture); linear IgG and C3

Type II: immune complex-mediated: “lumpy bumpy” granular pattern; (SLE, HSP, IgA nephropathy/Berger’s, PSGN)

Type III: pauci-immune (no Ig or complement deposits on BM): anti-ANCA (anti-neutrophil cytoplasmic Ab) [Wegener’s or idiopathic]

116
Q

Dx: circulating IgG4 Ab to the PLA2R (phospholipase A2 receptor)?

A

idiopathic membranous nephropathy

117
Q

Dx: ARF sym (oligouria) + ballooning, vacuolar (空泡样）degeneration of proximal renal tubules + oxalate crystals in tubular lumen

A

ethylene glycol (乙二醇）中毒

118
Q

earliest detection for diabetic nephropathy?

patho change?

A

albumin (microalbuminuria)

光镜特征：nodular glomerulosclerosis (Kimmelstiel-Wilson disease): round/oval shape, laminated and eosinophilic on HE stain, PAS+

119
Q

Dx: elder man, gross hematuria, history of smoking or occupational exposure to rubber, plastics, dyes, textiles, or leather?

A

transitional cell carcinoma of the bladder

120
Q

Which cells in the kidney will go hypertrophy and hyperplasia in kidney hypoperfusion?

A

SMCs in JG (juxtaglomerular) apparatus of the afferent or efferent arterioles

hypoperfusion ⟹ JG SMCs secrete renin ⟹ convert Ang I to Ang II ⟹ ↑ aldosterone release and vasocontriction ⟹ retension of sodium and water

121
Q

Why is anti-CD3 Ab used in treating acute rejection in kidney, liver, heart transplatation?

A

acute rejection: mediated by CD4+ Th mononuclear cells (MHC class II receptor +/IL-2 receptor +)

CD3 is required for proper function of CD4+ Th cells

122
Q

Cx for Wegener’s disease?

A

polyangiitis + granulomatosis

累及upper respiratory tract (sinusitis, nasal ulcer),
pulmonary (hemoptysis) , kidney (RPGN: oligouria)

Diff with Goodpasture (累及肺和肾脏）: Wegener’s associated with c-ANCA (anti-neutrophil cytoplasmic Ab)

123
Q

major pathophysiologic abnormality caused by pain killers (NSAID, Cox-2 inhibitor, etc)?

A

chronic interstitial nephritis

124
Q

kids with recent upper RI infection ⟹ nephronpathy

如何鉴别是PSGN vs. Berger disease?

A

timing of the renal syn/ C3 level helps to differentiate:

PSGN: a few weeks to develop renal syn / ↓ C3 level
Berger： a few days / normal C3

Berger’s Dx is made by the detection of IgA deposits in the mesangium of glomeruli on IFM

125
Q

Dx: cystic dilatations of the medullary collecting ducts?

A

medullary sponge kidney (MSK)

MC complication: kidney stone

126
Q

Dx: renal biopsy shows eosinophilic casts?

A

multiple myeloma - Bence Jones protein (light chain)

127
Q

why use mesna when giving cyclophosphamide?

A

cyclophosphamide: chemotherapy for cancer or autoimmune diseases

美司钠在临床上用于降低环磷酰胺和异环磷酰胺化疗引起病人出现血尿及出血性膀胱炎的可能性。环磷酰胺和异环磷酰胺在膀胱中会被转化成丙烯醛，而后者具有对膀胱上皮细胞的高度性，能够引起出血性膀胱炎，甚至膀胱癌。[1][2] 美司钠的巯基部分能够与丙烯醛的碳碳双键反应，生成无毒无反应活性的硫醚复合物并排泄出人体，从而在不影响疗效的情况下起到了泌尿系统保护剂的作用。[3][4] 同时它还有抗氧化剂的作用，而这进一步保护了泌尿系统，使其免受化疗药物代谢产物的伤害

128
Q

zones of prostate? BPH happens where?

A 
peripheral (MC site for prostate cancer)
transitional zone (MC for the glandular component of BPH)
periurethral zone (MC for the fibromuscular/stromal component of BPH)

patho: ↑ sensitivity to DHT

stromal cells contain 5a-reductase; are the site of DHT synthesis

BPH is NOT a risk factor for prostate cancer!

obstructive uropathy is an early sign for BPH, but not for prostate cancer （发现常是晚期，locate in peripheral zone)

129
Q

prostate cancer“ patho? Cx? metastasis? Lab?

A

patho: DHT-dependent
generally silent until advanced; obstructive uropathy indicates extension into the bladder base;

放射痛：lower back, pelvic, inguinal

metastases：血行转移最常见为骨转移： lumbar spine, pelvic bones (via Batson vertebral venous plexus) ⟹ compression of the spine cord

Lab: ↑ serum ALP, ↑ PSA (prostate specific Ag, sensitive but not specific for cancer - could be ↑ in BPH: if > 10 ng/mL: highly indicate cancer);

Diff between BPH: ↑ free PSA: consider BPH
↑ bound PSA: consider cancer

Dx: transrectal needle core biopsy, imaging (radionuclide bone scan

130
Q

function in male: FSH? LH? T? SHBG (sex hormone-bindng protein)?

A

FSH: + spermatogenesis, negative feedback with inhibin (synthesized by Sertoli cells 睾丸支持细胞）

LH: + T synthesis in the Leydig cells in the testes; T negative feedback to LH

SHBG: bind to both T and E2;
major synthesis site: liver; in male T also + synthesis in Sertoli cells; E2 ↑ liver synthesis

↑ SHBG causes ↓ free T; ↓ SHBG causes ↑ free T

131
Q

primary hypogonadism: types? Cx?

A

1) due to Leydig cell dysfunction (Leydig cell: 睾丸里生成T的细胞）： ↓ T, sperm count; ↑ LH (没有了T的negative feedback; normal FSH - Sertoli cells not affected) = hypergonadotropic hypogonadism
2) due to Leydig cell dysfunction + seminiferous tubule (细精管）dysfunction: 和1）一致： ↓ T, sperm count, ↑ LH; 不一致：↑ FSH (loss of inhibin feedback to FSH)

132
Q

Kallmann Syn?

A

AD, maldevelopment of olfactory bulb and GnRH-producing cells in the anterior pituitary (GnRH stimulates FSH and LH) ⇒ ↓ GnRH, FSH, LH, T, sperm count, delayed puberty + anosmia (嗅觉缺失）

133
Q

MCC impotence in men > 50 yr?

A

vascular insufficiency

Leriche syn: aortoiliac atherosclerosis ⟹ ↓ penal blood flow, calf claudication and atrophy, ↓ femoral artery pulse

134
Q

控制erection vs ejaculation的神经节段？

A

Parasympathetic for erection: S2 - S4

sympathetic for ejaculation: T12 - L1

135
Q

mechanism for Sildenafil?

A

Viagra: inhibit the breakdown of cGMP by type 5 phosphodiesterase ⟹ ↑ cGMP, cause vasodilation in corpus cavernosum (海绵体）

136
Q

pap smear: location? findings?

A

have to sample TZ (transformation zone for the potential SCC): presence of metaplastic squamous cells or mucus-secreting columnar cells indicating proper sampling; absence of these cells means the Pap smear must be repeated

Superficial cells: indicate adequate E2
intermediate cells: indicate adequate P4
parabasal cells (atrophoic): lack of E2 or P4

normal nonpregnant adult: 70% superficial + 30% intermediate
normal pregnant: 100% intermediate
elderly: 100% parabasal, inflammatory cells

137
Q

patho characteristics for HPV?

A

associated with CIN (cervical intraepithelial neoplasia) - precursor for SCC

HPV type-6, 11: low risk
HPV type-16, 18: high risk

病理特征：koilocytosis in squamaous cells (clear halo/pyknotic nucleus)

138
Q

CIN classification

A

cervical intraepithelial neoplasia/dysplasia

CIN I: mild, lower 1/3 of the epithelium (can reverse to normal)
CIN II: moderate, lower 2/3 of the epithelium
CIN III: severe, involve the full thickness

10 yrs for CIN I to CInN III
10 yrs for CIN III to cervical cancer

139
Q

Krukenberg tumor

A

signet ring cells, implicate diffuse cancer of the stomach or breast as the primary site

bilateral; multinodular outer appearance

140
Q

Lab findings in Klinefelter Syn?

A

47 XXY; MCC for gynecomastia (男子女性型乳房）

histology: some or all of the testicular tubules atrophied/ replaced by pink, hyalinized tissues

Lab: ↑ FSH, E2, ↓ T; E2: T ratio determines the extenf of feminization

141
Q

function:

aromatase?
21- or 17-hydroxylase?
5a-reductase?
HMG CoA reductase?

A

aromatase: T → E2 in ovaries, testes, placenta and other peripheral tissues; and androstenenone → estrone

21-hydroxylase: a cytochrome P450 enzyme that is involved with the biosynthesis of the steroid hormones aldosterone and cortisol
deficiency: MC type of congenital hyperplasia (CAH); Cx: ambiguous genitalia in the female fetus, salt wasting, hypotension, hyponatremia, hyperkalemia

17-hydroxylase: rare form of CAH: sodium retention, HT (和21-hydroxylase缺乏相反）， sexual infantilism in females

5a-reductase:

142
Q

fever, maculopapular rash + acute renal failure 1-3 wks after treatment with β-lactam antibiotics (ampicillin)， NSAIDs, diuretics: Dx?

A

AIN (acute interstitial nephritis)

important Dx clue: peripheral eosinophilia and eosinophiluria (drug-induced hypersensitivity reactions are the cause)

Symptoms resolve completely after cessation of the medication

143
Q

Parinaud syndrome

A

tumors in pineal gland: pinealoma

Paralysis of Conjugate Vertical Gaze
Brain Lesion in Superior Colliculus/Pretectal Area

Presentation:
Paralysis of Upward Gaze (setting-sun sign)

Bilateral Papilledema if causing noncommunicating Hydrocephalus by blocking Cerebral Aqueduct
Accommodation, but lack of Pupillary Light Reflex

the pineal region is the most common location of brain germinomas. Histologically very similar to testicular seminomas. Clinically: precocious puberty,

144
Q

MCC of hypopituitarism in children?

A

craniopharyngioma (颅咽管瘤）

benign tumor derived from Rathke pouch remnants; Rathke pouch is derived from the oral ectoderm, and develops into the anterior lobe of the pituitary gland;

tumor extends into the sella turcica (蝶鞍）and destroys the gland; compress optic chiasm ⇒ bitemporal hemianopia (偏盲）；

may cause CDI (central diabetes insipidus, 尿崩症）

145
Q

DiGeorge Syn

A

3rd/4th pharyngeal pouches undevelpped:

3rd: superior parathyroid
4th: inferior parathyroid + thymus

Cx: hypoparathyroidism + no thymus shadow

146
Q

Waterhouse-Friderichsen syn (WFS)

A

N. meningitidis sepsis ⇒ endotoxic shock, release of tissue thromboplastin, damage the endothelial cells ⇒ DIC ⇒ bilateral adrenal hemorrhage (infarction)

cause acute adrenocortical hypofunction

147
Q

Addison disease

A

“A” - chronic “a”drenal insufficiency

  • “a”utoimmune destruction: MCC in US
  • military TB: MCC of addison disease in developing countries
  • MCC in children: adrenogenital syn

Cx: weakness and hypotension (Na+ loss from mineralocorticoid and glucocorticoid deficiency), diffuse hyperpigmentation (↑ plasma ACTH stimulates melanocytes)

Lab: ↓ Na+, cortisol, bicarbonate; ↑ K+, ACTH (醛固酮促进肾脏Na-K交换；缺乏醛固酮引起metabolic acidosis）；hypoglycemia (↓ cortisol, which is gluconeogenic), eosinophilia, lymphocytosis, neutropenia (due to ↓ cortisol)

148
Q

MCC for adrenogenital syn?

A

classic 21-hydroxylase deficiency: (P4 converted to mineralocorticoid/cortisol)
impaired mineralocorticoid/cortisol production: salt loser, ↑ androgens

149
Q

MEN type I

A

MEN like 3P:
Parathyroidism (hyper)
Peptic ulcer due to gastrin-secreting tumor in pancrease;
Pituitary adenoma

149
Q

diff. Cushing syn

A
  • pituitary Cushing: ↑ ACTH, ↑ cortisol (can be suppressed by high-dose dexamethasone, DXM)
  • adrenal Cushing: ↓ ACTH, ↑ cortisol
  • ectopic Cushing: ↑↑ ACTH, ↑ cortisol (MCC: small cell carcinoma of lung; thymus, thyroid)

Cx: truncal obesity (↑insulin), thin extremeties (↑ cortisol, muscles breakdown), purple stria

weight gain: due to hyperinsulinism from hyperglycemia: insulin ↑ fat deposit in adipose and retain Na+

150
Q

Does hypernatremia happen in mineralocorticoid excess?

A

No - although Aldoesterone ↑ Na+ /H2O retention, excrete K+ and H+, increased Na+/H2O also ↑ renal BF and GFR ⟹ ↑ Na+ excretion; ↑ intravascular volume also ↑ release of ANP ⟹ natriuresis, so 虽然醛固酮保钠排钾，却很少在病人中见到高钠血症

Cx for aldosterone ↑： HT, hypokalemia, matabolic alkalosis, without marked hypernatremia

151
Q

Cx: MEN 1, MEN 2A, MEN 2B?

A

MEN 1: 3P (AD, tumors of the parathyroid gland, pituitary, pancreas)

MEN 2A: medullary carcinoma of the thyroid, pheochromocytoma, oral/intestinal mucosal neuromas, parathyroid hyperplasia TPPGI (踢PP的胃肠道）

MEN 2B: Cx for 2A WITHOUT parathuyroid hyperplasia

152
Q

Cause of amenorrhea in anorexia nervosa?

A

Loss of pulsative secretion of GnRH from the hypothalamus (not the pituitary or ovary!)

Low E2 for long-term can cause osteoporosis

152
Q

Paget’s disease of the bone

A

Osteitis Deformans

Increased Osteoblast and Osteoclast activity (with Osteoclast predominating) causing abnormal Bone Architecture (Inc Resorption an replaced with poorly mineralized matrix - looks woven)

BUZZWORDS: Mosaic Pattern of Bone Marrow Replacement; High-Output Cardiac Failure; Bigger Hat/shoe Size

Cause: Possibly due to Paramyxovirus

Phases:

Osteolytic Phase, Osteoblastic Phase

Presentation:

Often Polyostotic (Skull, Pelvis, Femur, Vertebrae, possibly Humerus)

Bone Pain (especially affects Spine and Calvarium)

Bone Enlargement (Skull enlargement with Frontal Bossin) - Need bigger hat size, or shoe size

Hypervascular Bone - warms overlying skin

Fracture

Arthritis

Hearing Loss due to Auditory Foramen narrowing

Diagnosis:

Normal Calcium, Phosphorus and PTH levels - BUT Very Inc Alkaline Phosphatase

Mosaic Bone Pattern (Enlarged, inc bone density with cortical thickening, Radiolucent Bones) with irregular cement line and thick bony trabeculae - seen later on

Chalk-Stick Fractures in Long Bones

Complications:

Can lead to Osteogenic Sarcoma

High-Output Heart Failure from inc blood flow from increased Arteriovenous Shunt

153
Q

diffenrential between RA and OA (osteoarthritis)

A

RA: morning stiffness IMPROVEs with use, last > 30 min and may linger for hours; involves MCP (metacarpophalangeal) and PIP joints, not DIPs

OA: use-dependent joint pain in weight-bearing joints, impoves with rest, could have morning stiffness but < 30 min, involve DIP and PIP

154
Q

bullous pemphigoid 大疱性类天疱疮 特征

A

elderly patients, autoimmune (Ab against hemidesmosomes along the basement membrane of the dermal-epidermal junction)

blister (>2 cm in diameter) on inner thighs, flexor asepects of the forearms, axillae (腋下），groin, lower abdomen

155
Q

Diff: rubeola vs. rubella

A

rubeola (regular measles):

caused by RNA paramyxovirus (副粘液v), prodrome (前驱症状）3Cs (cough, coryza - 鼻炎，conjunctivitis); koplik spots appear first, then rash; confluent on face and truck, but discrete on extremitis;
complications: giant cell pneumonia, acute appendicitis in children, encephalitis
NOT TERATOGENIC

rubella (German measles) 德国鬼子比较凶残

caused by RNA togavirus (批盖病毒），3-day measles, at first: dusky red spots on palate, then maculopapular rash begins at hairline and rapidly spreads cephalocaudally: NOT confluent, 特征性painful postauricular lymphadenopathy (耳后淋巴结肿痛）

TERATOGENIC!

156
Q

roseola infantum: cause? Cx?

A

HHV-6 (human herpesvirus 6), MC viral exanthem (皮疹）in children < 2yrs old

erythematous macules develop on soft palate 48 hrs BEFORE rash;
high fever, then 3-7 days LATER: abruptly develop maculopapular rash；

may precipitate a febrile convulsion (热性惊厥）

与rubella (German measles)鉴别的重要要点：tender cervical and/or posterior occipital (枕骨）lymphadenopathy is the key!!! (rubella: 耳骨后疼痛性淋巴结肿大）

157
Q

Paralysis of upward gaze: ? syn

“locked-in” syn indicates?

Symptoms for cerebellar lesions?

A

Parinaud syn (Dorsal midbrain syn) - tumor in pineal region, such as germinomas (malignant germ line tumor, cause precocious puberty in boys)

locked-in syn: pontine hemorrhage or tumor

Cerebellar lesions: gait and limb ataxia (can’t walk), intention tremor, nystagmus (眼球震颤）

158
Q

ataxia + dysarthria, degeneration of the posterior columns + spinocerebellar tracts: Dx?

A

VE deficiency or Friedreich ataxia

159
Q

ischemic stroke后按时间段的病理变化？ 24-48 hr？ 3-5 days? 2 wks?

A

first 48 hrs: neuron death (red neurons), neutrophils migrate into the area

3-5 days: microglia, phagocytosis of lipids (from breakdown of myelin)

2 wks: gliosis: astrocytes migrate and proliferation

160
Q

VDRL tests what?

A

syphilis

(venereal disease research lab test)

If VDRL + in CSF: neurosyphilis

161
Q

MC neoplasm of childhood, and microscopic presentation?

A

MC #1: pilocytic astrocytoma 毛细胞性星形细胞瘤
located in cerebellum (diff vs. medulloblastoma), see pilocytic astrocytes and Rosenthal fibers; prognosis better than medulloblastoma

MC #2: medulloblastoma, part of a group called PNETs (primitive neuroectodermal tumors); in cerebellum (⟹ ataxia, gait instability); sheets of small cells with deeply basophilic nuclei and scant cytoplasm (small, round, blue cells); abundant mitosis

MC #3: ependymomas 室管膜瘤
arise in the walls of ventriculi, can hamper CSF flow to cause hydrocephalus; form gland-like “rosettes”

162
Q

Dx between dementia: early presentation of gait disturbance + urinary incontinence?

A

NPH (normal pressure hyrdocephalus)

can progress into dementia, loss of short-term memory, emotional blunt (Diff vs. AD)

163
Q

Cranial tumor， S-100 positive, biphasic (dense/loose) pattern, elongated (spindle) cells with regular, oval nuclei: Dx?

A

Schwannoma;

melonoma is also S-100 +: both are from neural crest

164
Q

Horner’s syn

A

交感神经损伤； 副交感activity ↑

ipsilateral

1) partial ptosis (drooping of upper eye lid): denervation of the sympathetically controlled Muller muscle of the upper lid
2) miosis (缩瞳）：interruption of the sympathetic fibers to the dilator pupillae muscle
3) anhydrosis (impaired sweating)

causes: MC lung adenocarcinoma (compress the brachial plexus causes ipsilateral shoulder pain, hand muscle atrophy, loss of deep tendon reflexes)

can also be caused by lesions in the spinal cord and brain stem

165
Q

Dx: proximal muscle weakness + pre-existing tumor + ↑ muscle response on repetitive motor nerve stimulation

A

Lambert-Eaton Syn, （Ab against presynaptic Ca2+ channel); diff vs. myasthenia gravis (Ab agaisnt Ach R)

166
Q

Dx: brain watershed infarcts (bilateral wedge-shaped bands of necrosis over the cerebral convexity, just a few cm lateral to the interhemispheric fissure)

A

caused by global cerebral ischemia (= ischemic-hypoxic encephalopathy）

can be caused by extensive MI (not cardiac embolism, which leads to infarcts in different vascular territories)

167
Q

Dx: skull fracture, loss consciousness quickly and regain it, feel well for hours (lucid interval) and then loss consciousness again

A

epidural hematoma: life-threatening
(blood between skull bone and dura mater)

diff vs. subdural hematoma (bleed from bridging cortical veins, gradual onset of headache and confusion). vs. subarachnoid hemorrage (aneurysm, severe headache)

168
Q

define:

neuron cell body rounding, peripheral displacement of the nuclei, dispersion of Nissl substance to the periphery of the cells

A

“axonal reaction”, after sever of axon from the neuronal body. Severed axon is digested by Schwann cells. Neuronal cell body shows cellular edema (round, swollen, nuclei to the peripheral, Nissl substrance dispersed through the cell body - indicating increased synthesis of proteins to regenerate axon)

169
Q

MS

A

年轻人， 双视，动眼神经障碍，反复发病，中间有几个月-1年间隙，

MS: neuronal demyelination, depletion of oligodendricytes, Dx by MRI, ↑ CSF IgG (autoimmune), which shows up as “oligoclonal bands” on protein electrophoresis

170
Q

VB12-deficient neurodegeneration:

Cx?

A

affect the following 3 in spinal cord

1) dorsal column (bilateral loss of position/vibration sensation: gait abnormality - can’t walk, especially when eyes closed)
2) lateral CS (corticospinal) tracts: UMN signs (spastic paresis 痉挛性轻瘫), hyperreflexia, patholigic reflexes (Barbinski sign)
3) PNS axonal degeneration: numbness, parethesias

与ALS区别：ALS involves both UMN and LMN (lower motor neuron）

171
Q

CN III function?

A

CN III: oculomotor nerve

2 types of fibers (with different blood supply) 2 functions:
1) somatic component: 受损lead to ptosis ; “down and out” gaze

2) parasympathetic fibers: sphincter of the iris and the ciliary muscle. Paralysis of these fibers causes a fixed, dilated pupil

172
Q

pathway for pain/temperature sensation

A

1st N: DRG → 2nd N: dorsal horn , axon decussate in the ventral white commissure, ascend in the contralateral lateral funiculus → 3rd N: thalamus → 4th N: somatosensory cortex in the parietal lobe (顶叶）

173
Q

Dx: young adult, slow relaxation of muscles (can’t release hands after hand-shaking, can’t release doornob), + cataracts, frontal boldness + gonadal atrophy

A

myotonic muscle dystrophy (肌强直性肌萎缩），
除了Ducchenne之外第二MC的肌萎缩

CTG trinucleotide repeats, AD, anticipation

174
Q

Pick’s disease

A

profound atrophy of frontal /and temporal lobes,

diff vs. AD; dementia, inappropriate behavior, impaired judgement, lost problem-solving skills, dysarthria (构音障碍， damage of Broca’s speech area in frontal lobe)

175
Q

lung cancer compresses which nerve to cause:
1。hiccups + dyspnea
2. shoulder pain
3. Horner’s syn

A
  1. phrenic N (膈神经， C3-C5)
  2. lung cancer at apex can invade the rami of the branchial plexus to cause shoulder pain
  3. Horner’s： ipsilateral miosis, ptosis, anhydrosiss; oculosympathethic nerve (眼交感神经）
176
Q

opsoclonus (眼阵挛）- myoclonus (肌阵挛）syn is associated with ?

A

neuroblastoma, (N-Myc), MC extracranial neoplasma in children (~2 yrs)

177
Q

Dx: cerebellum and/or retina angiomatoma, + cystic mass in the kedney, liver or pancrease

A

Von Hippel-Lindau disease

178
Q

Sturge-Weber Syn

A

encephalotrigeminal angiomatosis (脑三叉神经血管瘤病,瑟志-韦伯综合征)颜面血管瘤综合征

congenital neurocutaneous disorder: cutaneous facial angioma, skin involvement overlies V1 and V2 area of the trigeminal N.

associated with mental retardation, seizures, hemiplegia, skull radiopacities (X光不透性）

179
Q

TS or TBC (tuberous sclerosis)

A

AD, caused by a mutation of either of two genes, TSC1 and TSC2, which code for the proteins hamartin and tuberin respectively. These proteins act as tumor growth suppressors,

特征：cutaneous facial angiofibromas (“butterfly lesion”)

visceral cysts in kidney, liver and pancrease; HAMARTOMAS (not angiomatous lesions! Dx from von Hipple-lindau). 心脏表现也为错构瘤

Seizures are a major clinic complication

180
Q

paraneoplastic syn: caused by?

A

autoimmune

181
Q

Mechanism for neuromuscular sym in CF?

A

recurrent pancreatitis → ↓ fat-soluble Vit (A, D, E, K) reobsorption → ↓ of Vit E antioxidative function → cell membrane damage, most prominent in neurons with long axons and RBC

↓ proprioception, hyporeflexia over the longer extremities, mild hemolytic anemia

182
Q

Guillian-Barre syn

A

吉利刀片划伤了外周神经

autoimmune-mediated acute demyelination of the peripheral nerves. often preceded by a febrile illness and is strongly associated with Campylobacter jejuni (空肠弯曲杆菌）infection.

Cx: ascending muscle weakness and areflexia

184
Q

Dx: pulmonary histology change

  1. Transudate in the alveolar lumen , 漏出液： Cardiogenic acute pulmonary edema (acute M
  2. scattered necrosis with alveolar Hemorrhage I)
A
  1. Pulmonary hemorrhage syndromes, Such as Goodpasture syndrome Wegener’s syndrome

Step 1 notes (22 decks)

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