Associated diseases with Gastric antral vascular ectasia (GAVE)
 (Pocket)

• Gastric antral vascular ectasia (GAVE):  
• “watermelon stomach” tortuous, dilated vessels;
• a/w cirrhosis, atrophic gastritis, CREST syndrome

•  ERCP + sphincterotomy:

in acute setting, reserved for severe cholangitis/sepsis and T bili >5 (ie, presumptive obstructive BD stone).

Otherwise, early ERCP does not reduce risk of local or systemic pancreatitis complications (Ann Surg 2007;245:10).

Pancreatitis complications

•  Pseudocyst (10–20%):

fluid collection, persists for 4–6 wk, encapsulated suggested by persistent pain & elevation of amylase or lipase, or mass on exam most resolve spont.;

• if >6 cm or persists >6 wk + pain → endo/perc/surg drainage

• BISAP: 5-point scoring system on admission
• BUN >25,
• GCS <15,
• SIRS,
• age >60, and
• pleural effusion

identifies Pts at risk for ↑’d mortality (Am J Gastro 2009;104:966)

•  Stages:

(1) confusion;

(2) drowsiness;

(3) stupor;

(4) coma

Criteria:

(1) cirrhosis w/ ascites;

(2) Cr >1.5 mg/dL;

(3) Ø improvement in Cr (to ≤1.5) after d/c diuretic & volume expansion (1 g/kg/d of albumin × 2 d);

(4)  Ø shock (prerenal azotemia/ATN);

(5) Ø nephrotoxic meds;

(6) Ø organic kidney dis (eg, GN) or obstruction

• Type I:
• Cr >2.5 or 1.5× baseline in <2 wk;
• usually occurs in severe liver failure, often following precipitating event (see later);
• median survival 2 wk

Liver transplantation

•  Undertake evaluation when MELD ≥15

•  Indic:

• recurrent/severe enceph,

• refractory ascites,

• SBP,
• recurrent variceal bleeding,

• HRS,

• HPS,

• HCC (if no single lesion is >5 cm or ≤3 lesions with largest ≤3 cm),
• acute liver failure

• Large-volume paracentesis (LVP);
• if >5 L, replace w/ 8–10 g/L of alb → ↓ risk of paracentesis-induced circ. dysfxn (AKI & lyte abnl) & ? ↓ mortality (Hep 2012;55:1172)

Dx ascitis cardiaca

(SAAG>1.1)

•  Ascites fluid total protein (AFTP):

useful when SAAG ≥1.1 to distinguish

• cirrhosis (AFTP <2.5 g/dL) from

• cardiac ascites (AFTP ≥ 2.5 g/dL)

Ascitis

Conteo celular: 300

cultivo: (+) 1 organismo

Ascitis

Conteo celular: ≥250

cultivo:(+) 1 org

Per bact espontanea

Ascitis

Conteo celular: 290

cultivo:neg

dx?

Ascitis

Conteo celular: ≥250

cultivo:neg

Asc neutrocitica cult negativo

paciente con datos de irritación peritoneal, posterior a DP

Conteo celular: 130 60% PMN

cultivo:+

Ascitis

Conteo celular: ≥100 leucos >50% PMN

cultivo:+

Peritonitis asociada a dialisis

Ascitis

Conteo celular: ≥250

cultivo:(+) polimicrob

Ascitis

Conteo celular: ≥250

cultivo:+ polim

secundaria

Ascitis

Conteo celular: 190

cultivo:+

Dx

Ascitis

Conteo celular: <250

cultivo:+ 1 org

asc bact no neutrocitica

Ascitis

conteo cel: 300

cultivo: + 1 moo

Tx?

Spontaneous bacterial peritonitis

•  Rx:

• 3rd gen. ceph (eg, cefotaxime 4g/d total dose) or
• amox/clav × 5 d,

• if  [+] enceph/AKI can use FQ (cipro/oflox) but avoid if already on for ppx or in ↑ FQ resist. areas IV

•  Ppx:

if h/o SBP or AFTP <1.5 + Na <130, Cr >1.2 or

Child-Pugh B (Am J Gastro 2009;4:993)

norfloxacin 400 mg PO qd or Bactrim DS qd

•  2° prevention:

 for all Pts after 1st bleed b/c ~50% rebleed & ~30% mortality

bB + EVL > either alone (Annals 2008;149:109);

• TIPS if refractory or consider in Child-Pugh B or C w/in 72 h of admission for esoph variceal bleed (↑ 1-y survival; NEJM 2010;362:2370)

Type 1:

• octreotide (100–200 mcg SC tid) +

midodrine (7.5–12.5 mg PO tid, titrate to ↑ MAP 15 mmHg) +

20–40 g/d albumin or

• terlipressin + albumin (Hep 2010;51:576);

• definitive Rx = liver tx.

paciente con CH child 8

score?

Child C: 10-15

supervivencia a un año de 45%

•  Epi: 

• Female 40–60 y/o;

• a/w Sjögren’s, Raynaud’s, scleroderma, celiac & thyroid disease
• 
  

  •  Dx:

  ↑ A Φ , ↑ bili, ↑ IgM, ↑ chol, 

• [+] antimitochondrial Ab (AMA) in 95%.

pac con cBP que ha recibido colestiramina para tx del prurito sin obtener respuesta

Tx?

• Pruritus:
• cholestyramine (give 2–4 h after UDCA);
• if refractory sx: naltrexone, rifampin

    Mutant HFE gene identified on chromosome 6  (85% cases)

      Disease may be present in homozygotes with the C282Y or H63D mutation, variable penetrance in

compound heterozygotes

•  typically C282Y homozygotes (~0.5% of N. European Caucasians), rarely C282Y/H63D compound heterozygotes;

Wilson’s disease (J Hep 2012;56:671)

•  Recessive disorder of copper transport (mutation in ATP7B)

• ↑ 24-h urine Cu,

• ↓ serum ceruloplasmin (Se 90%),
• rarely penicillamine challenge w/ ↑urine Cu excretion,

• liver bx w/ hepatic Cu content.

In acute liver failure,  A Φ /bili <4 +AST/ALT >2.2 better Se & Sp than urine Cu or ceruloplasmin (Hepatology2008;4:1167).

Paciente con CH

Lab: ceruloplasmina baja, cobre ur 24H elevado

Ha recibido tx con  penicillamine + pyridoxine

sin obtener respuesta

tx?

Enf wilson

•  Treatment:

• chelation w/ penicillamine + pyridoxine; (±Zn)

•  2nd line trientine (↓ toxicity w/similar efficacy).
• Zinc:
• ↓ intestinal Cu transport and can help delay disease;
• best used if asx or in conjunction w/ chelation (must give 4–5 h apart from chelators).

•  CCY in asx Pts w/:

• GB calcification (~7% risk of ca) (Surgery 2001;129:699),
• GB polyps >10 mm,
• Native American,
• stones >3 cm or bariatric surgery or cardiac transplant candidates

•  Type 1:

antismooth muscle Ab (ASMA), ANA;

• antisoluble liver antigen (anti-SLA), a/w more severe disease and relapsing disease

•  Type 2:

• anti–liver/kidney microsome 1 (anti-LKM1) or liver/cytosol (LC1);

•  kids (2–14 y)

Que medicamentos pueden generar asocicion de hepatitis autoinmune con PBC / PSC

•  Overlap syndrome:

AIH + PBC or PSC;

Rx-induced: minocycline, nitrofurantoin, infliximab

      Treatment indicated in patients with

• ALT ≥10 times normal;

• ALT ≥5 times normal, and
• gamma globulin ≥2 times normal; or
• significant inflammation and/or (bridging/multiacinar)necrosis on liver biopsy

•       Prednisone with or withoutazathioprine for at least 18 to 36 months (many patients require lifelong therapy)
• 65% remission within 3 years
• 50% relapse on withdrawal of meds at 6mo

     The discriminant function (DF), = 4.6 × [PT − control (in seconds)] + serum bilirubin (in mg/dL),

determines the need for corticosteroid therapy

•       Score of greater than 32 predicts a (2nd indication for Tx: encephalopathy)
• greater than 50% 30-day mortality rate and is
• used to indicate the need for corticosteroid therapy

Paciente con DM descontrolada y hepatitis alcoholica

Tx:-

•       Steroids are not recommended for patients with
• concomitant infection (sever infection such as sepsis)
• poorly controlled diabetes
• GIB
• Chronic HBV

      Pentoxifylline 400 mg three times daily

• improves shortterm survival by

• decreasing the risk of hepatorenal syndrome and should be considered as a surrogate for prednisone

• IV NAC:
• 150 mg/kg over 1 h → 50 mg/kg over 4 h → 100 mg/kg over 16 h;
• risk ofanaphylaxis;
• use if unable to tolerate POs, GIB, preg, fulminant hepatic failure

•  Neurologic

• encephalopathy:
• stage I = DMS;
• stage II = lethargy, confusion;
• stage III = stupor;
• stage IV = coma

• asterixis in stage I/II/III encephalopathy;
• hyperreflexia, clonus, rigidity in stage III/IV
• cerebral edema → ↑ ICP, ↓ CPP →
• cerebral hypoxia,
• uncal herniation,
• Cushing’s reflex (hypertension + bradycardia),
• pupillary dilatation, decerebrate posturing, apnea

Anti-HbC: IgM [+]

HBV DNA (PCR)

•  Extrahepatic syndromes:

cryoglobulinemia,

porphyria cutanea tarda (blistering rash in sun-exposed areas),

MPGN,

MGUS,

IPF,

NHL and

DM

Natalizumab

(blocks leucocyte trafficking) approved if refractory to anti-TNF-alpha tx

aumenta riesgo de Leucocencefalopatia multifocal progresiva en pacientes con ab JC[+], con el uso cronico

(pocket)

Ustekinumab(anti-IL12/23)

Vedolizumab (Anti alpha4beta7 integrin)

    Indications for surgery:

      Fulminant disease (toxic megacolon)

      Colitis refractory to medical therapy

      Dysplasia or concern for colorectal cancer

      Stricture

      Massive bleeding

  Indications for surgery include

• obstructive symptoms,

• refractory severe inflammation, and

• repair of fistulas

      More than 40% of patients require surgery within

first 10 years of diagnosis

      Up to 80% of patients will have evidence of recur-

rence endoscopically, and 10% to 15% will have

clinical recurrence

Peutz-Jeghers
syndrome

Multiple gastrointestinal polyps:
Stomach (24%)
Small bowel (96%)
Colon (27%)
Rectum (24%)

A que sx poliposico de colon se asocia la mut

SmaD 4
BMPR1A

Juvenile polyposis
syndrome

Multiple juvenile polyps in:
Colon/rectum (98%)
Stomach (14%)—most commonly with SMAD4 mutations
Duodenum/jejunum/ileum (7%)

 Genetic profile in sporadic CRC:

• APC (~80%),

• KRAS (~40%),
• TP53 (50–70%),

• DCC or SMAD4, or BRAF (~15%);

•  Mutations in adenoca.:

KRAS (>90%), p16 (80–95%), p53 (50–75%), SMAD4 (~55%)

Hereditary risk factors:

genetic susceptibility may play a role in 5–10% of cases

• Hereditary chronic pancreatitis:
• mutation in cationic trypsinogen gene (PRSS1)

• Familial cancer syndromes and gene mutations with ↑ risk:
• familial atypical multiple mole melanoma (CDKN2A/p16),
• familial breast and ovarian cancer (BRCA2),
• Peutz-Jeghers (LKB1),
• ataxia-telangiectasia (ATM),
• ? hereditary colorectal cancer (HNPCC and FAP)

•  Metastatic:

• FOLFIRINOX (5-FU + leucovorin, irinotecan, oxaliplatin) if good perform. status (NEJM 2011;364:1817);

• gemcitabine combination (eg, w/ nab-paclitaxel; JCO 2011;29:4548) or monotherapy if poor performance status (JCO 1997;15:2403). Offer clinical trials.