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GI II Exam II > DSA Jaundice > Flashcards

DSA Jaundice Flashcards

1
Q

CMP measures what?

A
  1. AST/ALT
  2. Albumin
  3. BR
  4. ALP
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2
Q

Jaundice is yellow skin pigmentation caused by ________.

A

↑ serum bilirubin

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3
Q

Causes of hyperbilirubinemia (3)

A
    1. Overproduction
    1. Impaired uptake, conjugation or excretion of BR
    1. Regurgitation of UCB or CB from damaged hepatocytes or bile ducts
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4
Q

Bilirubin is the major breakdown product of _________, released from ___________.

A
  • Hemoglobin
  • Released from senscent erythrocytes
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5
Q

What are the first signs of jaundice?

A

Yellowing of the eyes, oral mucosa and palms

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6
Q

What changes will we see in:

  • Hepatocellular damage => damage to hepatocytes.
  • Which is MORE specific?
A

↑ AST/ALT

*ALT = more specific

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7
Q

What changes will we see in:

  • Cholestatic damage => damage to bile ducts, causing ______.
A
  • Damage to the bile ducts =>
    • cholestasis, thus, the bile cannot reach the duodenum.
  • ↑ in
    • ↑ alkaline phoshatase
    • ↑ bilirubin
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8
Q

Cholestatic damage (↑ ALP and bilirubin) can cause what symptoms?

A
  1. Jaundice
  2. Pruritis
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9
Q

1st thing to do when we have jaundice is what?

A

Determine if:

  • 1. Unconjugated/indirect or Conjugated/direct hyperbilirubinemia
    1. Other biochemical liver tests are abnormal
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10
Q

DDX

Unconjugated Hyperbilirubinemia => Jaundice

A
  1. Hemolytic syndrome (anemia or reaction)
  2. Gilbert Syndrome
  3. Crigler-Najar Syndrome
  4. Viral Hepattitis (can be both))
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11
Q

DDX

Conjugated Hyperbilirubinemia => Jaundice

A
  1. Hepatitis
  2. Cirrhosis
  3. Obstruction
    1. Choledocolithiasis, Cholangitis,
    2. PBC, PSC,
    3. Budd-Chiari
    4. Pancreatic cancer
  4. Dubin-Johnson Syndrome
  5. Rotor syndrome
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12
Q

1st diagnostic studies to get in a patient with jaundice

A
    1. CBC: to look for hemolysis => anemia and thrombocytopenia (prehepatic sources that cause unconjugated hyperbilrubinemia).
    1. Chemistry labs:
      * ​AST/ALT, ALP, Total BR (+ fractionated BR to tell if indirect vs direct)
      * Fractionate ALP by ordering GGT
    1. US to see if obstructive jaundice (conjugated)
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13
Q
  • Jaundice due to unconjugated/indirect hyperbilirubinemia
    • ↑ bilrubin production

DX???

A
  1. Hemolysis
  2. Hematoma
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14
Q

Jaundice due to unconjugated/indirect hyperbilirubinemia

  • Due to impaired bilirubin uptake and storage

DX???

A
  1. Post-hep
  2. Gilbert
  3. CN Syndrome
  4. Drug reaction
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15
Q

Jaundice due to conjugated/direct hyperbilirubinemia

  • Due to impaired excretion

DX???

A
  1. Dubin Johnson Syndrome
  2. Rotor Syndrome
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16
Q

Jaundice due to conjugated/direct hyperbilirubinemia

  • Due to Hepatocellular dysfunction

DX???

A
  1. Hepatitis/ Cirrhosis
  2. Drugs/ biliary cirrhosis
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17
Q

Hemolyis causes ______ hyperbilirubinema.

What diagnostic test do we run and what are we looking for?

A
  • Hemolysis => unconjugated hyperbilirubinemia
  • CBC
    • Anemia and thrombocytopenia by looking for (↓) haptoglobin and (↑) LDH
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18
Q

What is Gilbert Syndrome?

  • Pathophys
  • Labs
  • Treatment
A
  • Benign, asymptomatic AR jaundice seen after fasting, post-exercise
  • ↓ activity of UDGT => isolated ↑ bilirubin (unconjugated hyperbilirubinemia); however NL when not in those conditions
  • No treatment needed
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19
Q

Gilbert syndrome is associated with reduced mortality from __________.

A

CV disease

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20
Q

CN Syndrome 1 and 2 and Gilbert => unconjugated hyperbilrubinemia

Differentiate them based on

  • Inheritance
  • Defect
  • Liver histology

Clinical course

A
  • CN Syndrome Type 1
    • AR
    • ABSENT UGT1A1 activity
    • NL
    • Kills bb in neonatal
  • CN Syndrome Type 2
    • AD with variable penetrance
    • Decrease UGT1A1 activity
    • NL
    • Mild; occasional kernicterus
  • Gilbert
    • AR
    • Decrease UGT1A1 Acticity
    • NL
    • Innocous
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21
Q

Which indicated acute and chronic infection: IgM and IgG

A
  • IgM = acute
  • IgG = chronic
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22
Q

How long is chronic hepatitis?

A

> 3- 6 months

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23
Q

How can we tell is fibrosis/cirrhosis is occuring in chronic liver disease?

A
  1. Serum FibroSure
  2. US elastography
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24
Q

It is important to ask about _________, because they can cause transminitis/LF or hepatitis

A

MEDICATIONSS1

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25
**Acute Heptitis** can be caused by: \_\_\_\_\_\_\_\_\_\_\_\_ What may the patient report? PE?
* Viral, drugs or ischemia * Acolic stools, suddenly grossed out by smoking * Tender hepatomegaly, jaundice
26
When a patient comes in with **acute hepatisis,** what diagnostic test is **IMPORTANT** to run? Generally, how it is treated?
* **Acetominophen levels via Rumack Matthew Nomogram** * Generally, **self-limited** and **supportive**
27
Complication of **acute hepatitis? (3)**
1. **Cirrhosis** 2. **HCC** 3. **Fulminant liver failure =\> death**
28
What is the course of **HepA virus?**
* Never chronic; self-limited; pt recovers
29
\_\_\_\_\_\_\_\_\_ can cause an **aversion to smoking.**
Hep A
30
**HepA** * RF: * Symptoms * What pattern of injury is seen and how does this affect labs:
* RF: International travel! * Symptoms: Jaundice + tender hepatomegaly + acholic stools * Labs: Hepatocellular AND cholestatic pattern * ↑ AST/ALT * ↑ BR/ALP
31
* ________ is seen in the serum in **HepA** soon after onset. * **Diagnosis** = Detection of \_\_\_\_\_\_\_ * What indicates that the persion was **PREVIOUSLY** exposed to HAV, is **non-infected** and **immune**?
* **IgM** and **IgG Anti-HAV** * Diagnosis = I**gM anti- HAV** * Previously exposed (not infected/ immune) = **IgG anti-HAV w/o IgM anti-HAV.**
32
Is there a vaccine for **Hep A?**
Yes
33
**_Hep B_** * Acute/chronic or both? * Symptoms? * Transmission? * What pattern of injury is seen and how does this affect labs? * Vaccine?
* Mainly acute, but chronic 5-10% of cases * Jaundice + tender hepatomegaly, low-grade fever + POLYARTERITIS NODOSA * Parenteral, sexual or perinatal (mom =\> bb) * **Hepatocellular pattern**; NO cholestasis pattern * **MARKEDLY ↑ AST/ALT** early in the course (higher than HepA) * If severe enough = prlong PT and INR =\> bleeding * Yes: protects against B and D
34
Where is **HepB endemic** and how is is passed down?
**Africa** and **SE ASia** =\> HBsAg (+) mom passes Hep B to BB =\> causing the risk of chronic infection to be 90%
35
How can we prevent Hep B before exposure and AFTER exposure in an unvaccinated person??
* **Before**: 3 dose vaccine * **After exposure in an unvaccinated person:** *_Vaccine_* + _HepB immune globulin_ (HBIG) immediately- 14 days after sex or birth (maternal transmission)
36
The window period is **between HBsAg disappearing** and **HBsAb appearing,** which may be several weeks, but the patient is still considered to have \_\_\_\_\_\_. To detect, we must measure \_\_\_\_\_\_. This is very important when screening blood donations.
* **Acute HepB** * **IgM HBcAg**
37
What is very important when screening blood donations?
Screen for **IgM HBcAg t**o see if patient has acute HepB
38
**HepB** 1. Window period 2. Acute infection 3. Chronic infeciton 4. Prior infection 5. Immunization
1. Window period: **IgM HBcAg** 2. Acute infection: HBsAg, IgM HBcAg, HBe Ag and HBV DNA (if replicating) 3. Chronic infeciton: **same + [IgG anti-HBcAg]** 4. Prior infection (2): **Anti-HBsAg** and **IgG anti-HBcAg** 5. Immunization: **ONLY AntiHBsAg**
39
\_\_\_\_\_\_\_\_ typically parellaels prescee o**f HBeAg.**
**HBV DNA**
40
If ______ persists **_\>6 months_** after the acute illness =\> **chronic hepatitis B.**
**HBsAg**
41
When is Anti-HBsAg increased?
**1. After clearance of HBsAg** **2. Vaccination**
42
\_\_\_\_ indicates **ACUTE** hep B infection
**_IgM anti-HBcAg_** ## Footnote **\*\*\*IgG also appears but persists FOREVER**
43
\_\_\_\_\_\_\_\_ may reappear during **flares** of **previously inactive chronic hepatitis B**
IgM anti-HBcAg
44
\_\_\_\_\_\_ is the **secretory form** of HBcAg and if it persists longer than _____ =\> increase liklihood of **chronic HepB**
**HBeAg** ## Footnote **\> 3months =\> chronic HepB**
45
**_Hep D_** * **Endemic areas** in HepB ( \_\_\_\_\_\_\_\_\_), spreads via \_\_\_\_\_\_\_\_\_ * In **non-endemic areas** (\_\_\_\_\_\_\_\_\_\_), spreads via \_\_\_\_\_\_\_\_\_\_.
* **Endemic** = Mediterranean Basin = spread non-percutaneously * **Nonendemic areas** = N europe and US = percutaneously in HBsAg+ IV drug users or transfusion.
46
What is a ssRNA genome with **7 genotypes** and is most commonly **CHRONIC?**
**HepC**
47
* **30%** of people with _____ are co-infected with **HepC** * **\>90%** of patients got hepatitis from \_\_\_\_\_\_\_\_, have **HepC** * **\>50%** of people with **HepC** get it from via \_\_\_\_\_\_\_, with ________ being a **risk factor.**
* 30% of people with **HIV** are co-infected with HepC * \>90% of patients got hepatitis from **transfusion**, have HepC * \>50% of people with HepC get it from via **IV drug use**, with **intranasal cocaine** being a risk factor.
48
* Hep C aquired in the **hostpital** and **outpatient clinics** have received it via \_\_\_\_\_\_\_\_\_ * In the developing world, ____________ =\> leads to **↑ HepC cases** * **Covert transmission** of HepC has occured via **\_\_\_\_\_\_.**
* multidose vials of saline used to flush catheters * unsafe medical practies * Bloody fisticuffs
49
**HepC** * What pattern of injury is seen and how does this affect labs? * Complications Prevention?
* Hepatcellular: marked FLUCTUATING ↑ of AST/ALT * Cirrhosis, HCC, HIV co-infection, mixed cryoglobinemia, membranoproliferative Gnephritis, lichin platus * If infected =\>SAFE SEX
50
What is the most **SENSITIVE** indicator of HepC infection? How do we properly dx?
**HCV RNA** 1. Screen =\> check for **Anti HCV** in the serum 2. If (+) =\> **HCV RNA** =\> most sensitive
51
When has a person **recovered** from prior HepC infection?
* + anti-HCV **w/o** HCV RNA
52
Which viral hepatisis is associated with **↓ serum cholesterol?**
**Chronic HepC infection**
53
CDC and USPSTF recommend screening persons born between _______ for **HepC**
**1945-65 (Baby Boomers)**
54
New recommendation say that who should be screened for **HepC?**
1. **Once in all adults over 18YO,** except in areas where prev is infectoin is less than 0.1% 2. **All pregnant W in each pregnancy**, except in areas where prev of infection is less than 0.1%
55
**_HepE_** * RF * Where is it epidemic? * Transmission * Vaccine? * **Acute with no carrier state**, however, what group of people have seen Chronic HepE infection that progresses to Cirrhosis?
* **Immunocompromised ppl** * Asia, Middle East, N. Africa, C. America, India * Enterically fecal-oral from water; spread by **SWINE** * **Clinical tria**ls are testing one that is promissing * **Transplant patients treated with tacrolimus**
56
What are **dose-dependent direct hepatotoxins** that can cause **hepatitis**?
**1. Mushroom poisoining** **2. Acetominophein**
57
What are **idiosyncratic drugs/toxins (sporadic and not related to dose)** that can cause hepatitis?
1. **Isoniazid** 2. **Sulfonamides**
58
How do we **treat** toxic/drug- induced hepatitis?
1. **Supportive**: 1. stop taking drug/toxin 2. Gastric lavage and give oral charcoal & cholestyramine
59
**_Acute Liver Failure_** * MCC * Patients will have ________ and \_\_\_\_\_\_\_\_\_. * Diagnostics: \_\_\_\_\_\_\_\_\_\_\_\_\_ * Complication?
* Acetominophin OD * Hepatic encephalopthy + coagulopathies * Acetominophen level via Rumak Matthew Nomogram * Not reco/treated =\> multiorgan failure and death.
60
Tyelonal/ acetominophen OD is detected by the R**umack- Matthew Nomogram** (measures the acetominophen plasma concetration after (x) hours of ingestion) to determine in patient has hepatoxicity). * It is important to obtain \_\_\_\_\_\_\_. * How do we treat
* **4 hour acetominophen level** * **NAC (N-acetylcystine)** within **8 hours** of ingestion (but can be effective if 24-36 hours later) provides sulfhydrl groups to bind toxins
61
* What is **fulminant hepatic failure?** * **Subfulimant**?
* **Fulminant hepatic failure =\>** pt gets hepatic encephalopthy within 8 weeks of onset of acute liver disease * **Subfulminant** =\> pt gets hepatic encephalopathy between 8 weeks - 6 months.
62
**Fulminant Hepatitis** * What is this? Assx symptoms? * Diagnosis and
* **LF + encephalopathy:** Massive hepatic necrosis + hepatic encepholapthy (impaired consciousness) that occurs within 8 weeks of the onset of an illness. * Rapidly shrinking liver + ascites + edema * [Rapidly ↑ bilirubin] & [marked prolongation of PT] ***EVEN*** as AST/ALT ↓
63
What type of edema is most common in **Fulminant Hepatitis?**
**Cerebral** edema
64
How do we treat **Fulminant Hepatitis?**
1. Support patient 1. Maintain fluids, circulation and respiration 2. Control bleeding, fix hypoglycemia, tx other complications 2. No proteins 3. Consider liver transplant
65
How can we treat **encephalopthy** seen in fulminant hepatitis?
1. **Oral lactulose** 2. **Neomycin**
66
What is the KEY to improving survival in patients with **Fulminant Hepatitis?**
1. **Meticulous intensive care +** 2. **Prophylatic ABX**
67
**Mortality rate** in Fulmanant Hep is \_\_\_\_\_\_\_.
**VERY HIGH!**
68
\_\_\_\_\_\_ is the most common cause of chronic liver disaese in the **US**.
**NAFLD**
69
What is chronic hepatitis?
Chronic inflammtion of the liver for at **LEAST 6 months.**
70
Which 3 hepaittis can cause **chronic Hep,** key signs and how are they mediated?
* Chronic HepB, C, Autoimmune =\> IMMUNE COMPLEX MEDIATED * HBV: polyarteritis nodsa * HCV: mixed cryoglobenimia
71
In **Chronic Hepatitis,** ______ is used to **histologically** classify by _________ and \_\_\_\_\_\_\_.
* **BIOPSY** * **Grade** & **Stage**
72
What are the causes of **Chronic Hepatitis?**
1. **HBV +/- HDV** 2. **HCV** 3. **AI Hep** 4. **Wilsons** 5. **Hemochromatosis** 6. **A1-antitrypsin deficiency**
73
What is the most common type of **AIH**, which can lead to chronic Hep? Who does it occur in Serology? What do we see on exam?
* Type 1: * 30-50YO W * **(+) ASMA ab**\*\*\* and **(+) ANA ab** and **_hypergammaglobulinemia_** * Healthy young W with **stigmata of cirrhosis**
74
_In **AIH**,_ 1. **Serum AST/ALT** levels will be \_\_\_\_\_\_\_\_\_\_\_ 2. _________ is usually increased
1. AST/ALT: **\> 1000 units/L** 2. **Total bilirubin**
75
What is **Stigmata of cirrhosis?**
1. Multiple spider telangiectasias 2. Cutaneous striae 3. Acne 4. Hirsutism 5. Hepatomegaly
76
What is a complication of **Autoimmune Hepatitis?** ## Footnote **Treatment?**
1. **Cirrhosis** 2. **HCC** * Treat: **glucocorticoids**
77
\_\_\_\_\_\_\_ if the most common precursor to **cirrhosis**.
**Alcohol liver disease**
78
How much alcohol must be drinken to classify as **Alcohol Liver Disease?** When doing an H&P, what is ONE thing we need tp be catious be?
* **M: \> 80g/day** * **W: \> 30-40 g/day** **bitchez lie and wont admit too much alcohol use**
79
* **Alcoholic Fatty Liver Disease (Steatosis)** is often \_\_\_\_\_\_\_\_\_\_\_\_\_. * In **Alcoholic hepatitis,** you will see what 2 key characterstics .
* Steatosis: **Asymptomic hepatomegaly** * AH: * **2:1 AST: ALT ratio (**not above 300 u/L) * **Mallory Denk Bodies: alcoholic hyaline**
80
Think **alcohol** when you see \_\_\_\_\_\_\_\_\_\_\_\_.
**2:1 AST: ALT ratio** (not \>300 u/L)
81
What increases our risk of developing **alcoholic cirrhosis?**
**\>50grams/day for \>10 years**
82
In **alcoholic liver disease,** what will you see on **CMP**?
1. 2:1 ratio of AST/ALT (not above 300/L) 2. ↓ albumin; ↑ gamma-globulin level 3. ↑ bilirubin 4. ↑ ALP and GGT
83
In alcoholic liver disease, what will you see on **CBC**?
1. Macrocytic/megaloblastic anemia 2. Leukocytosis 3. Lerkopenia 4. Thrombobocytopenia
84
PT/PTT/INR will be ________ in **alcohol liver disease.**
increase
85
\_\_\_\_\_\_ deficiency can be seen in **alcohol liver disease**
**Folic acid deficiency**
86
**Alchol Liver Diseease** * Imaging * Biopsy of Liver * Treatment
* US Fibrosure to see if cirrhosis * Biopsy findings are SAME as NASH: mallor bodies (alcoholic hyaline) * Tx 1. STOP alcochol 2. Daily mutlivitamin + 100 mg of thiamine + 1 mg folic acid + zinc 3. **Liver transplant** if end-stage, but must abstain alcohol for 6 months
87
When treatming **Alcohol Liver Disease,** what must be done to prevent **Wernicke-Korsakoff** Syndrome**?**
* Before giving glucose to a patient, r**eplace thiamine (vitamins) 1st**. Otherwise, patient can get Wernickes
88
**Wernickes encepalopthy** Triad * Diagnosis? * Treat:
* **1. Confusion** * **2. Ataxia-staggering/ no coordination** * **3. Involuntary eye movements (horizontal nystagmus** **Dx:** clinical suspicion **Treat:** thiamine
89
**Korsakoff Syndrome** sx
1. REALLY bad Wernickes **=\>** that causes **permenant** and **severe memory issues,** so will make up shit to fill in gaps
90
**50%** of **critically ill pts** with **\_\_\_\_\_\_\_\_\_** will die in **30 days**
**alcoholic hepatitis**
91
**_Alcohol Liver Disease_** 1. **Maddrey’s discriminant function: ?** 1. _____ = poor prognosis and tx with \_\_\_\_\_\_ 2. A Model for End-Stage Liver Disease (**MELD/MELD-Na**) score 1. _______ = significant mortality in alcoholic hepatitis. 3. **Glasgow Alcoholic Hepatitis Score**: predicts mortality based on a multivariable model 1. \_\_\_\_\_= who got glucocorticoids had higher survial rates than those who did not
* **Maddrey’s discriminant function:** severity and mortality in pts w alcoholic hepatis by measuring **PT** and **serum bilirubin** * **​\> 32 =** high short term death and treat w glucocorticoids * (**MELD/MELD-Na**) * \> 21 = significant chance of death from alcoholic heaptitis * **Glasgow Alcholic HEpatitis Score** = predicts death based on many variables * **\> than or equal to 9**
92
**Fatty liver** aka Hepatic steatosis can be caused by what 2 things?
1. Acoholic fatty liver diasease 2. NAFLD
93
How much alcohol must someone drink to be dx with **NAFLD**?
* **M: \< 30 mg/day** * **W: \< 20 mg/ day**
94
**_NAFLD_** What are the main causes of **NAFLD**? what do these increase risk of? (3 C's) Most commonly occur in \_\_\_\_\_\_\_\_\_\_\_ What protects AGAINST NAFLD? Who is **unlkely** to get cirrhosis caused by NASH?
1. Metabolic syndrome 1. obesity, DM, hyperTAG + insulin resistance 2. Increase risk for: **CV disease, CKD, colorectal cancer** 3. **Hispanics** 4. Protects**: coffee** and **physical activity** 5. **AA**
95
**_NAFLD_** * Sx? * Serum levels? * **Liver Biopsy = diagnostic**
* Asx or mild RUQ pain + hepatomegaly, but all you may see is **INC AST/ALT/ALP** * **​however, NL in 80% w hepatic steatosis** * Biopsy * Focal infiltration of polymorphonuclear neutrophils * Mallory hyaline * LOOKS EXACLTY LIKE ALCOHOLIC HEPATITIS =\> NASH
96
**Vinyl Chloride** is a weird cause of \_\_\_\_\_\_\_
**Fatty LIVER**
97
**_Hemochromatosis_ = increased iron ab from duodenum** 1. What causes us to suspect hemochromatosis? 2. Rarely recognized before patient is \_\_\_\_\_\_ 3. Overall mortality is \_\_\_\_\_\_\_\_\_\_. 4. Risk factors for developing advanced fibrosis: \_\_\_\_, \_\_\_\_\_, \_\_\_\_
1. ↑ iron saturation; ↑ serum ferritin or FH 2. 50s 3. slightly incrased 4. M; DM; excess alcohol consumption
98
Describe sx of patient with **Hemochromatosis**
* Mostly asymptomatic. Early, sx are non-specific. L * Later, as iron starts to deposit, patient can get 1. **skin pigmentation** 2. **DM and complication** 3. **erectile dysfunction** 4. ARthritis
99
Patients with **Hemochromatiss** are at INCREASED RISK FOR \_\_\_\_
**Yersinia enterocolitica**
100
**_Hemochromatosis_** * Labs * Who do we screen?
Labs 1. HFE gene mutation 2. Mild abnl AST/ALT/ASP 3. ↑ plasma iron with \> 45% transferrin saturation 4. ↑ serum ferritin Screen 1. Anyone w iron overload 2. **All** first degree family members
101
Hemochromatisis Tx
1. Avoid foods with iron 2. **Weekly phlebotomies\*\*\*** 3. PPI to decrease iron absorbtion 4. if you get a liver transplant =\> nl levels and no more phleb
102
If patients have **[hemochromatosis + anemia]** or secondary iron overload due to **thalassemia who cannot tolerate plebotomies**, how do we treat?
**Chelating agent deferoxamine**
103
**Wilsons disease** is a **AR** disorder than can cause **cirrhosis** and **organ toxicity** due to mutation in _____ and dx at age \_\_\_\_\_\_, causing \_\_\_\_\_
* ATP7B mutation * Dx under 40 YO * too much absoprtion of Cu from SI, but impaired copper excretion in bile and failure to put it in ceruloplasm
104
Sx seen in **Wilsons** which is the pathogomeunomic sign
* 1. Hemolytic anemia (Cu toxic to RBC\_ * **2. Kayser Fleischer rings - brown green ring around eye \*\*\*\*\* pathomneumonic sign** * 3. Neuro defects (migrains, insomnia, seizures) * 4. Psych features (behavior and personality changes) * can occur b4 neuro
105
**Wilsons Disease** is a __________ in adolescents; but a _______ in young adults
**liver disease** = adolescents **neuropsyc**h = young adults
106
When should we consider **wilsons diasese** as a dx
**Child/young adult wiht** 1. hepatitis 2. splenomegaly with hypersplenism 3. Coombs neg hemolytic anemia 4. Portal HTN 5. NEuro/psych problems
107
Wilsons Diagnosists Labs and which imaging study should we do?
1. Low serum ceruloplasm 2. High concentation of copper in liver; increased urine excretion of copper 3. **MRI** of the brain show copper deposts
108
ALPHA 1 ANTITRYPSIN DEFICIENCY * The most commonly diagnosed **inherited** hepatic disorder in i**nfants and children.** Develop what 2 problems? * Diagnositcs?
* Pulmonary emphysema at a young age in lower lobes (if smoker = upper lobes) * Liver disease due to acc of misfolded protein * Check A1-AT phenotype (homozygous PIZZ)
109
ALPHA 1 ANTITRYPSIN DEFICIENCY Tx:
1. Smoking abstinence/cessation 2. Liver transplant 1. **MC genetic cause requiring liver transplant in children**
110

GI II Exam II (10 decks)

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