GI Re-Up #6 Flashcards
Primary Biliary Cirrhosis/Cholangitis (PBC) is idiopathic autoimmune disorder of intrahepatic small bile ducts that leads to decreased bile salt excretion, cirrhosis, and end-stage liver disease.
This is MC in ____________.
What are some symptoms?
-MC in middle-aged women (30-60 years old)
-Symptoms: Fatigue (first symptom), pruritus, RUQ discomfort, hepatomegaly, jaundice, xanthelasma, osteoporosis. Cirrhosis symptoms late in disease.
What is the initial diagnostic study done?
What gives a definitive diagnosis?
What do labs show (there is a specific lab)
-Initial: US
-Definitive: Liver biopsy
Labs (cholestatic pattern): increased Alkaline phosphatase and GGT
–Antimitochondrial antibody HALLMARK
First line treatment for PBC
-Ursodeoxycholic acid (like cholelithiasis)
-Liver transplant is definitive
On the other hand, Primary Sclerosing Cholangitis (PSC), is autoimmune progressive cholestasis leading to diffuse fibrosis of intra AND extra hepatic biliary ducts.
This is MC associated with what other condition?
Name some symptoms
-Associated with IBD (Ulcerative Colitis)
Symptoms: jaundice, pruritus, fatigue, RUQ pain, hepatomegaly, splenomegaly (similar to PBC)
What do labs show for PSC (there is a specific one)
-Cholestatic pattern: Increased Alkaline phosphatase and GGT
-Positive P-ANCA HALLMARK
What is the most accurate imaging study for PSC and what is seen?
-MRCP and ERCP - beaded appearance of biliary ducts (narrowing, strictures)
What is Wilson’s Disease?
What is the pathophysiology of this condition?
rare autosomal recessive disorder leading to copper accumulation in the body (liver, brain, kidney, joints, cornea)
Defect in chromosome 13 leads to decreased biliary copper excretion due to decreased ceruloplasmin
Symptoms of Wilson’s Disease
What is seen on a slit-lamp examination?
-Cirrhosis, liver failure, hepatosplenomegaly
-Dysarthria, bradykinesia, tremor, hallucinations, dementia, seizures, ataxia
-Psychosis, Delusions
-Arthralgias from copper deposition
Slit Lamp: Kayser-Fleischer Rings (brown or green rings due to copper deposition in cornea)
What is seen on labs for Wilson’s Disease?
-Decreased serum ceruloplasmin
-Molecular genetic testing for ATP7B
-Elevated transaminases
-Hemolytic Anemia
-Increased 24 hour urinary copper excretion**
What gives a definitive diagnosis of Wilson’s Disease?
Treatment options
-Liver biopsy
-Treatment
–Trientine or D-Penicillamine (Copper chelating agents)
–Give pyridoxine (B6) with Penicillamine to prevent B6 deficiency
–Zinc supplementation
What is the pathophysiology of acute pancreatitis?
2 MCC of this condition, along with others?
-Acinar cell injury –> intracellular activation of pancreatic enzymes –> auto digestion of pancreas
-2 MCC are gallstones and alcohol abuse.
-Others: Thiazides, Protease Inhibitors, Exenatide, Valproic Acid, Scorpion Sting, ERCP, CF, Infection
Symptoms of acute pancreatitis (remember there are two named physical exam findings)
-Epigastric pain: constant, boring pain that radiates to the back or other quadrant. Pain worse if supine, relieved with leaning forward, sitting, or fetal position.
-Nausea, vomiting, fever common
-Decreased bowel sounds
-Cullen Sign: periumbilical ecchymosis
-Grey Turner Sign: flank ecchymosis
Best initial test for acute pancreatitis?
Increased amylase and lipase (lipase more specific)
What else is seen on labs for pancreatitis?
Hypocalcemia (necrotic fat binds to calcium)
Diagnostic imaging of choice for pancreatitis?
What is seen on abdominal XR?
Abdominal CT (DOC)
XR: sentinel loop: localized ileus of segment of small bowel in LUQ. Colon Cutoff Sign: abrupt collapse of colon near pancreas.
Management for acute pancreatitis
-90% recover in 3-7 days with just supportive measures (rest the pancreas)
-NPO, high volume IVF (Lactated Ringers), Analgesia
-ABX not routinely used
MCC of chronic pancreatitis?
There is a triad of clinical manifestations with this condition…what is it?
-ETOH abuse most common
-Others: idiopathic
Calcifications + steatorrhea + Diabetes Mellitus
-Others: weight loss, back pain, epigastric pain
With diagnostics with chronic pancreatitis, amylase and lipase are usually normal or mildly elevated. What is seen on CT scan?
What is seen on abdominal radiographs?
What is the most sensitive and specific test for chronic pancreatitis?
-CT: calcification of pancreas.
-Abdominal XR: calcified pancreas
Pancreatic function testing (fecal elastase) most sensitive and specific
Treatment for chronic pancreatitis
-ETOH abstinence, pain control, low fat diet, vitamin supplementation
-oral pancreatic enzyme replacement
-Pancreatectomy if refractory
70% of pancreatic carcinoma is found in which part?
What is the MC type?
Name some important risk factors for this condition?
-head of pancreas
-Adenocarcinoma
-Smoking, > 55 years old, males, Obesity, chronic pancreatitis, AA
Symptoms of pancreatic carcinoma, including physical exam findings
-Painless jaundice (classic)
-Weight loss
-Abdominal pain radiating to the back
-Pruritus (increased bile salts in skin)
-Acholic stools and dark urine (CBD obstruction)
-Trousseau’s Sign: migratory phlebitis associated with malignancy.
-Courvoisier’s Sign: Palpable, NON TENDER, distended gallbladder due to CBD obstruction.
Initial diagnostic of choice for pancreatic carcinoma
-If negative, what should you do?
-What tumor marker if often used to monitor after treatment?
-CT scan
-If negative, endoscopic US with biopsy of pancreatic lesion
CA 19-9
What surgical procedure is done for pancreatic carcinoma (if confined to head or duodenal area)?
Whipple Procedure (pancreaticduodenectomy) with post-op chemo or radiotherapy
What is a Meckel’s (Ileal) Diverticulum?
This is the MC congenital anomaly of the GI tract
-persistent portion of embryonic vitelline duct (yolk sack, omphalomesenteric duct) in the small intestine
What is the pathophysiology of Meckel’s Diverticulum?
Symptoms of this condition
-Ectopic gastric or pancreatic tissue may secrete digestive hormones, leading to bleeding.
Usually asymptomatic. Painless rectal bleeding or ulceration. May cause diverticulitis in adults.
What is done to diagnose a Meckel’s Diverticulum?
Treatment?
-Meckel Scan: looks for ectopic gastric tissue in the ideal area
-Treatment: surgical excision if asymptomatic
An indirect hernia is bowel protrusion at the internal inguinal ring. The origin of the sac is ________ to the inferior epigastric artery.
This is the MC type of hernia in both sexes, young children, and young adults.
What is the pathophysiology of this type of hernia?
-Lateral to inferior epigastric artery
-Often congenital due to persistent patent process vaginalis. An increased in abdominal pressure may force intestines through internal ring into inguinal canal.
Symptoms of both indirect and direct inguinal hernia
-How about if incarcerated?
-How about if strangulated?
-Symptoms: swelling or fullness at hernia site. Enlarges with increased pressure and standing. May develop scrotal swelling.
-Incarcerated: painful, enlargement of an irreducible hernia (unable to return contents to abdominal cavity). N/V
-Strangulated: ischemic incarcerated hernias with systemic toxicity (irreducible hernia with compromised blood supply). Severe, painful bowel movement (may refrain from defecation)
On the other hand, direct inguinal hernias have an origin where the sac is ______ to the inferior epigastric artery within Hesselbach’s Triangle. What are the components of this triangle?
Why do direct inguinal hernias MC occur?
-Medial
-RIP: Rectus Abdominus (medial), Inferior epigastric vessels (lateral), Poupart’s Ligament (inferior)
occurs as a result of weakness in the floor of the inguinal canal
Initial imaging of choice for inguinal hernia, if not clinical
-Groin US
Treatment for inguinal hernias
-Often require surgical repair. Strangulated hernias are surgical emergencies.
Vitamin A functions include…
What are some sources?
Vision, immune function, embryo development, skin health, cellular health
Found in egg yolk, butter, green leafy veggies
Symptoms of Vitamin A Excess
-Blurred vision, nausea, vertigo, vomiting, increased intracranial hypertension
-Alopecia, ataxia, skin disorders, visual changes, hepatotoxicity
On the other hand, patients with liver disease, alcoholics, and those with fat malabsorption (CF, Crohn’s, Short Bowel Syndrome) are at risk for Vitamin A deficiency. What are some symptoms of this?
-Visual changes: night blindness**, xerophthalmia (dry eyes), retinopathy
-Impaired immunity (poor wound healing)
-Squamous metaplasia (conjunctiva, lungs, urinary tract)
-Bitot’s Spots: white spots on conjunctiva
Symptoms of Vitamin C (Ascorbic Acid) Deficiency
-Thinks of the H’s
-Scurvy
–Hyperkeratosis: hyperkeratotic follicular papules. Coiled hairs.
–Hemorrhage: vascular fragility with recurrent hemorrhages in gums, skin and joints. Impaired wound healing.
–Hematologic: anemia, glossitis, malaise, weakness. Increased bleeding time.
Riboflavin (Vitamin B2) Deficiency has symptoms that include…
-Oral, Ocular, Genital
–Magenta colored tongue, glossitis, angular cheilitis, stomatitis, pharyngitis.
–Photophobia, corneal lesions
–Scrotal dermatitis
Vitamin D Deficiency is low bone turnover and decreased osteoid mineralization (osteomalacia).
Osteomalacia MCC is ________. What are some symptoms of this?
-Severe Vitamin D Deficiency: leads to decreased serum calcium and phosphate with demineralization of the bone osteoid only (soft bones)
-Diffuse bone pain and tenderness, muscular weakness, bowing of lone bones
-Hypocalcemia symptoms
Vitamin D deficiency in kids ______, in adults _______
Rickets
Osteomalacia
What is seen on labs in those with Vitamin D deficiency?
How about on Radiographs in those with osteomalacia?
-Decreased calcium, phosphate, and 25-hydroxyvitamin D levels
-XR: Looser lines (zones) – transverse pseudo-fracture lines (radiolucent lines from visible osteoid)
Treatment for Vitamin D deficiency
-Vitamin D supplement (Ergocalciferol)
What are some symptoms of Rickets?
-Delayed fontanel closure, growth delays, delayed dentition, gene varum (lateral bowing of the femur and tibia)
What is expected on radiographs in Vitamin D deficiency
-Widening of epiphyseal plate, costochondral junction enlargement (rachitic rosary)
-Long bones have a fuzzy cortex
What are some important etiologies to remember for Rickets in children?
-MC seen in 3 months - 3 year olds when calcium needs are high (to grow) + decreased sunlight exposure
-Prolonged breastfeeding without Vitamin D supplementation
MCC of Thiamine (B1) Deficiency
-Chronic alcoholism
-Others: weight loss surgery
B1 Deficiency has many symptoms. Explain:
-Dry Beriberi:
-Wet Beriberi:
-Wernicke Encephalopathy:
-Korsakoff Dementia:
-Dry: nervous system changes (symmetric peripheral neuropathy, paresthesias, sensory and motor neurons, impaired reflexes, anorexia, muscle cramps)
-Wet: high output heart failure, dilated cardiomyopathy
-Wernicke: Ataxia + Global Confusion + Ophthalmoplegia (paralysis of EOM). This is MC in chronic alcoholics.
-Korsakoff: Short term memory loss and confabulation. Irreversible.
Treatment for Thiamine (B1) Deficiency
-IV thiamine followed by oral thiamine
Niacin/Nicotinic Acid (B3) comes from things such as _______________. Etiologies of deficiency of B3 are those that include….
Symptoms of this condition are (Pellagra)
-Meats, grains, legumes
-Diets high in untreated corn, lack tryptophan, alcoholism, anorexia, INH therapy, alcoholism.
3D’s: Dermatitis (photosensitive hyper pigmented dermatitis on sun exposed areas), Diarrhea, Dementia
Pyridoxine (B6) Deficiency
-Etiologies
-Symptoms
-Etiologies: Alcoholism, Isoniazid, Hydralazine, Levo/Carbidopa, OCP’s
-Symptoms: Neuro (peripheral neuropathy, seizures, headache, mood changes). Anemia, stomatitis, seborrheic dermatitis, flaky skin
B12 (Cobalamin Deficiency)
-Sources of B12
-Absorption
-Etiologies
Mainly animal sources (meats, eggs, dairy products)
B12 released by stomach and combines with intrinsic factor where it is absorbed mainly by distal ileum
Pernicious Anemia (MCC), Crohn’s, Vegan Diet, Chronic Alcohol use, Metformin, PPI/H2 blockers
Symptoms of B12 deficiency
What is seen on a CBC with peripheral smear?
-Fatigue, exercise intolerance, pallor
-Glossitis, diarrhea, malabsorption
-Neuro Symptoms: Symmetric paresthesias, lateral and posterior spinal cord degeneration (ataxia, vibratory, sensory, proprioception deficits, decreased DTR’s, seizures, psychosis
CBC with smear: increased MCV (macrocytic anemia with megaloblastic anemia) hypersegmented neutrophils and macro-ovalocytes
What other labs are drawn and what do they show for B12 deficiency?
Decreased serum b12 levels
Increased LDH
Increased homocysteine
Increased methylmalonic acid (distinguishes b12 from folate)
If symptomatic anemia or neuro findings, what treatment should you start with?
What should patients with pernicious anemia get?
IM B12
Patients with pernicious anemia need monthly, lifelong B12 injections
