DM Week 4/Review for Exam Flashcards by Jessica Lin

A 4 year old boy with acute lymphoblastic leukemia (ALL) is prescribed an enzyme transfusion as part of his treatment regimen. Which of the following enzymes was most likely given to this patient?
A. Glutaminase
B. Glutamine synthetase
C. Asparaginase
D. Glutamate dehydrogenase
E. Aspartate transaminase
F. Alanine transaminase

C. Asparaginase. This reduces the asparagine levels to starve the tumor

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Mechanism of fructose transport

Facilitated transport with the gradient and GLUT-5 in the small instestine

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Nearly all binding of cobalamin (vitamin B12) to intrinsic factor occurs in which organs?

Duodenum

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Absorption of fat-soluble vitamins requires which of the following?
A. intrinsic factor
B. chymotrypsin
C. pancreatic lipase
D. pancreatic amylase
E. secretin

C. Pancreatic lipase

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Glucagon activates glycogen degradation and gluconeogenesis in the liver. What is the action of cortisol?

Activation of PEP carboxykinase by enzyme induction.

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Poisoning with ethylene glycol is due to:

Highly toxic glycoaldehyde which leads to glycolic acid

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Poisoning with moonshine is due to:

Methanol Toxicity, Highly toxic formaldehyde inside the cells of liver, neurons and retina. Causes blindness due to formic acid.

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Mechanism of action of disulfiram

Inhibits aldehyde dehydrogenase

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What are two treatment options of acquired hyperammonemia?

-Lactulose
-Neomycin (antibiotic treatment)
-Low protein/high carb diet (less N ingested)

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What is the synthetic form of BH4?

Sapropterin

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Symptoms of Maternal PKU

Similar to fetal alcohol syndrome:
-Microcephaly
-Severe neurological problems
-Congenital heart defects
-Facial dysmorphology: long philtrum, epicanthal folds, micrognathia, short nose, microphthalmia flat midface

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Embryological origin of the faliciform ligament

Ventral Mesentery

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Embryological origin of the spleen

Dorsal Mesentery

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What can be given to prevent toxic accumulation of metabolites in Tyrosinemia Type 1?

Nitisinone: inhibits 4-OH-phenylpyruvate dioxygenase (PDO)

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Manifestations include:
-Liver failure
-Renal Failure
-Cabbage like odor of the urine

Tyrosinosis (Tyrosinemia Type I)
-Deficiency of fumarylacetoacetate hydrolase

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Therapy that can be beneficial for disorder involving increased methionine synthesis:

Homocysteinuria:
-Pyridoxine (Vitamin B6)
-Restrict dietary protein
-Supplement with methionine free medical food
-Oral Betaine-HCl

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Reabsorption defect in neutral amino acids

Hartnup Disease: low tryptophan concentrations

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How is trypsinogen activated?

It is released by the pancreas and is cleaved to trypsin only after it reaches the lumen of the duodenum by the activated enteropeptidase

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How are amino acids absorbed? Through which mechanism?

The uptake of dietary amino acids is mainly performed by secondary active transport with cotransport of sodium ions.

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The activation of pepsinogen to pepsin is dependent on what?

An acidic pH

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Substrates needed for heme synthesis:

Succinyl CoA + Glycine

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Acetaminophen degrades into which 2 products?

Acetaminophen glucuronate (urine)
Acetaminophen sulfate (urine)

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Hepatic steatosis (fatty change) is a consequence of chronic alcohol intake due to:

Acetaldehyde Toxicity–the high levels of cytosolic NADH lead to TAG synthesis and the release of VLDL is inhibited, lipid droplets accumulate inside hepatocytes. **Tubulins are damaged.

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ALT»>AST levels

Acute liver disease

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AST>>>ALT levels

Chronic liver disease due to chronic alcoholic cirrhosis (2:1 or higher)

Risk factor for pyridoxine deficiency

Isoniazid therapy (anti-tuberculosis drug)

Oral rehydration therapy with glucose targets which transporters for the treatment of osmotic diarrhea?

SGLT1 Transporter in the GIT -Secondary active transporter

Refsum disease causes an accumulation of which metabolite?

Phytanic acid

Organic Aciduria involves which amino acids?

Valine and odd chain fatty acids -Symptoms include: Hypoglycemia, metabolic keto acidosis, problems with the conversion of propionyl CoA to methylmalonyl CoA mutase

Acute phase proteins

Stimulated by the liver after inflammation: c-reactive protein, alpha-1-antitrypsin, ceruloplasmin, haptoglobin

Inherited defect in dietary copper absorption

Menkes syndrome

In iron overload, does total binding capacity (TIBC) increase or decrease?

Decreases Clinical Correlate: Hemochromatosis

In iron deficiency anemia, does total binding capacity (TIBC) increase or decrease?

Increases

Microcytic anemia is seen in which conditions?

Iron Deficiency Pyridoxine deficiency Copper Deficiency **Due to reduced heme synthesis

Macrocytic anemia is seen in which conditions?

Vitamin B12 Folic acid deficiency **Due to reduced cell division

Mucopolysaccharidoses

Hunter and Hurler Syndromes

Iduronidase deficiency

Hurler Syndrome

Accumulation of dermatan sulfate and heparan sulfate

Hurler syndrome

Iduronate sulfatase deficiency

Hunter Syndrome

beta-hexosaminidase A deficiency

Tay-Sachs

Accumulation of gangliosides

Tay-Sachs

Onion shell inclusions in lysosomes, cherry-red spot on macula

Tay-Sachs

beta-glucosidase deficiency

Gaucher disease

Bone crisis, distal femur in the shape of an erlenmeyer flask, crumpled tissue paper appearance of foam cells

Gaucher disease

deficiency of alpha-galactosidase

Fabry disease

Accumulation of globoside (ceramide trihexoside)

Fabry disease

Skin rash with "bathing trunk distribution" and peripheral neuropathy

Fabry disease

Deficiency of sphingomyelinase

Niemann-Pick disease

Accumulation of sphingomyelin

Niemann-Pick disease

Hepatomegaly and cherry-red spot on macula, foamy cell appearance

Niemann-Pick disease

Deficiency of Aryl sulfatase A

Metachromatic leukodystrophy

Accumulation of sulfatide

Metachromatic leukodystrophy

Progressive paralysis and demyelination

Metachromatic leukodystrophy

Deficiency of lysosomal acid maltase (alpha 1-4 glucosidase)

Pompe disease

Deficiency of N-acetylglucosamine 1-phosphate transferase

I-cell disease

A man has a history of peptic ulcer disease. A gastroscopy shows a perforating ulcer in the posterior wall of the stomach. Which of the following locations will peritonitis most likely develop initially?

Omental bursa (lesser sac)

A CT scan shows part of the bowel is being compressed between the abdominal aorta and superior mesenteric artery. Diagnosis of "nutcracker syndrome" is made. Which of the following intestinal structures is most likely being compressed?

Third part of the duodenum

Which artery would most likely provide collateral supply to the descending colon?

Middle colic

Which of the following anatomic features are the most useful to distinguish the jejunum from the ileum?

Jejunum has less mesenteric fat compared with the ileum

Massive bleeding from the descending colon. What is the most likely source of the hemorrhage?

Left colic artery (branch of the inferior mesenteric artery)

A 4 month old baby girl is brought to the ED. X ray of abdomen shows a posterolateral defect of the diaphragm and abdominal contents in the left pleural cavity. Which of the following is the most likely cause of this defect?

Failure of pleuroperitoneal fold to close

Which vessels will most likely provide collateral circulation between the celiac trunk and superior mesenteric artery?

Superior and inferior pancreaticoduodenal

In which of the following locations will blood be detected with an ultrasound exam if the patient stands up (male)?

Rectovesical space

Which lymph node initially drains the pyloric antrum?

Celiac nodes

A diagnosis of esophageal varices is made. Which surgical anastomosis is most commonly used to relieve these symptoms?

Splenic to left renal vein (portal to caval system)

Which of the following structures would most likely be identified and ligated within the triangle of Calot?

Cystic Artery

Ethylene glycol poisoning can cause the accumulation of which toxic metabolite?

Oxalate

Clinical features of Anderson disease

Hepatomegaly and cirrhosis

Glycogen storage disease that can lead to muscular dystrophy later in life

Cori disease

Central role of glucose-6-phosphate in glycogen metabolism and glycolysis

Used to form glucose 1 phosphate during glycogen synthesis

During strenuous activity, how is glycogen phosphorylase kinase activated?

By calcium ions

What is the regulatory enzyme of ketogenesis?

Mitochondrial HMG CoA synthase

Are weak acids and reduce serum bicarbonate levels during fasting

3-hydroxybutyrate and acetoacetate

Which of the following substances has a paracrine action in producing smooth muscle relaxation? A. Secretin B. Gastrin C. CCK D. VIP E. Gastric Inhibitory peptide

D. VIP

What are the boundaries of the triangle of Calot?

Inferior border of the left ventricle (LV), cystic duct, common hepatic duct

Absorption of fat soluble vitamins requires which of the following?

Pancreatic lipase

DM Week 4/Review for Exam Flashcards

(77 cards)