previous year question on hepatomegaly based on neet pg, usmle, plab and fmge or mci screening exams

A 66-year-old woman dies of severe congestive heart failure. Her past medical history is remarkable for rheumatoid arthritis that first manifested at the age of 35. The autopsy revealed splenomegaly, hepatomegaly, and glomerulopathy. The spleen, liver, and kidneys showed a similar waxy texture. Which of the following mechanisms explains these clinical and pathologic findings?

A: Accumulation of amyloidB: Atherosclerosis of coronary arteriesC: Coxsackievirus myocarditisD: Mutation of myosin chain genesCorrect Ans:AExplanation

Chronic inflammatory conditions, such as collagen vascular diseases, rheumatoid arthritis, and bronchiectasis, may lead in time to accumulation of a specific form of amyloid, known asAA (amyloid-associated protein). Systemic amyloidosis affects many organs, especially the heart, kidneys, spleen, GI tract, liver, and tongue. The ventricular walls become thickened and inelastic, and the resulting pathophysiologic picture is that of restrictive cardiomyopathy. Congestive heart failure ensues because of decreased ventricular compliance and secondary reduced stroke volume. Note how the ventricular lumen are narrowed and the walls are extremely thickened. A waxy texture is highly suggestive of amyloid deposition, which can be confirmed histologically by using Congo red staining.

Note:

Atherosclerosis of coronary arteries may give three different types of clinical manifestations: arrhythmias (with sudden death), angina pectoris, and myocardial infarction. Loss of myocardial tissue due to ischemic necrosis may lead to congestive heart failure. The heart will show evidence of old infarcts in the form of scars, and the ventricular cavities will become dilated.

Coxsackievirus myocarditis is the most common etiologic form of viral myocarditis, which may resolve without clinical consequences or lead to dilated cardiomyopathy and heart failure.

Mutations of myosin chain genes are the most common causes of hypertrophic cardiomyopathy. This manifests with thickening of the interventricular septum (so-called asymmetric cardiac hypertrophy), with resultant impediment to both systolic outflow and diastolic filling. Here, the ventricular walls are uniformly thickened, and the remaining postmortem findings (liver, kidney, and spleen changes) cannot be explained based on a diagnosis of hypertrophic cardiomyopathy.

Ref: Kim H.W., Farzaneh-Far A., Klem I., Rehwald W., Kim R.J. (2011). Chapter 23. Magnetic Resonance Imaging of the Heart. In V. Fuster, R.A. Walsh, R.A. Harrington (Eds), Hurst's The Heart, 13e.

Sample Previous Year Question on Hepatomegaly based on previous Year Questions of NEET PG, USMLE,PLAB,FMGE (MCI Screening). Please visit www.medicoapps.org for more such Quizzes

A farmer rearing sheep, presented with complaints of fever and weakness for the last one month. There is generalised lymphadenopathy. There was also associated hepatomegaly. Biopsy of liver showed non caseating granuloma. These are most likely due to infection with:

A: Yersinia pestisB: Brucella CanisC: Francisella tularensisD: Brucella melitensis

Correct Ans:DExplanationB. melitensis, which is the most common cause of symptomatic disease in humans and for which the main sources are sheep, goats, and camels. Overall, the presentation of brucellosis often fits one of three patterns: febrile illness that resembles typhoid but is less severe; fever and acute monoarthritis, typically of the hip or knee, in a young child; and long-lasting fever, misery, and low-back or hip pain in an older man. Dual therapy with streptomycin and tetracyclines is the most effective treatment.

Ref: Corbel M.J., Beeching N.J. (2012). Chapter 157. Brucellosis. In D.L. Longo, A.S. Fauci, D.L. Kasper, S.L. Hauser, J.L. Jameson, J. Loscalzo (Eds), Harrison's Principles of Internal Medicine, 18e.


Excess intake of retinol causes:

A: NauseaB: HepatomegalyC: AnorexiaD: All of the above

Correct Ans:DExplanationAn excess intake of retinol causes nausea, vomiting, anorexia and sleep disorders followed by skin desquamation and then an enlarged liver and papillar edema.

Ref: Park 22nd edition, page 571


Which of the following is the MOST unlikely feature of hepatocellular carcinoma?

A: HepatomegalyB: Raised a- fetoprotein levelsC: Raised alkaline phosphataseD: Jaundice

Correct Ans:DExplanation

Jaundice is rare, unless significant deterioration of the liver function or obstruction of the bile duct occurs.

Serum elevation of alkaline phosphatase and alpha fetoprotein are common.

Also know

Most common symptom of hepatocellular carcinoma is abdominal pain. Most common sign of hepatocellular carcinoma is hepatomegaly.

Ref: CSDT 13/e, Page 518; Harrison 17/e, Page 581


Ramu, a 8 yr old boy presents with upper GI bleeding. On examination, he is found to have splenomegaly; there are no signs of ascites, or hepatomegaly; esophageal varices are found on upper GI endoscopy. Most likely diagnosis is:

A: Budd chiari syndromeB: Cirrhosis

C: Veno-occlusive diseaseD: Non cirrhotic portal fibrosisCorrect Ans:DExplanation

The diagnostic criteria of NCPF includes splenomegaly, normal liver function test, esophageal varices (90-95% cases), patent hepatic and portal veins and no evidence of cirrhosis on biopsy.

Based on this criteria, the most probable diagnosis in this patient is Non cirrhotic portal fibrosis.

Ref: Arun J. Sanyal, Vijay H. Shah (2005), Chapter 25, “Noncirrhotic Portal Hypertension and Portal Vein Thrombosis”, In the book, “Portal Hypertension: Pathobiology, Evaluation and Treatment”, USA, Pages 411-12 ; Harrison’s Internal Medicine, 15th Edition, Page 1759


A child presents with complaints of oliguria, abdominal distension and diarrhoea. On examination there is deep jaundice. Hepatomegaly was present measuring 4 era below the costal margin. Conjugated bilirubin in blood was 38 mg/dL, and there was urine urobilinogen and bile pigments. Hemoglobin was 4 mg/dL and serum creatinine was 3 mg/dL. Which of the following tests is of least importance to diagnose this case?

A: G6PD level in bloodB: Serum ceruloplasminC: Reticulocyte countD: Leptospiral antibody levelCorrect Ans:BExplanation

Severe anemia, jaundice, hepatomegaly are strongly suggestive of this child is suffering with severe hemolysis. Oliguria and elevated creatinine levels are pointing out to acute renal failure due to hemolysis. Hence the investigations should be towards the etiology of

hemolysis. Serum ceruloplasmin determination is for finding out Wilson’s disease. The above clinical picture does not correlate with Wilson’s disease.

Findings of Wilson’s disease may include jaundice, hepatomegaly early in childhood, splenomegaly, and Kayser-Fleischer rings. Hepatic involvement may present as ALF, acute hepatitis, chronic liver disease, cholelithiasis, or as cirrhosis with portal hypertension. Serum ceruloplasmin levels (measured by the oxidase method) are usually less than 20 mg/dL. (Normal values are 23–43 mg/dL.)

Ref: Sokol R.J., Narkewicz M.R. (2012). Chapter 22. Liver & Pancreas. In W.W. Hay, Jr., M.J. Levin, R.R. Deterding, J.J. Ross, J.M. Sondheimer (Eds), CURRENT Diagnosis & Treatment: Pediatrics, 21e.


A Child presents with hepatomegaly and hypoglycemia. There is no improvement in blood sugar even after administration of epinephrine. What is the likely diagnosis?

A: Von Gierke's DiseaseB: Anderson's DiseaseC: Mc Ardle's DiseaseD: Pompe's Disease

Correct Ans:AExplanationPatient presenting with hepatomegaly and hypoglycemia with absence of an increase in blood glucose upon administration of glucagon or epinephrine is suggestive of Type I Glycogen storage Disease or Von Girke's disease.

Ref: Harrison’s Principles of Internal Medicine, 16th Edition, Pages 2320-2323


What will be the likely diagnosis in a child with massive hepatomegaly and hypoglycemia, whose blood glucose shows no improvement on administration of glucagon?

A: Von Gierke diseaseB: McArdle disease

C: Cori's diseaseD: Forbe's diseaseCorrect Ans:AExplanation

Presence of hepatomegaly and hypoglycemia along with absence of an increase in blood glucose upon administration of glucagon suggests the diagnosis of Von Gierke disease.

Von Gierke disease:

Children with Von gierke disease present with hypoglycemia and lactic acidosis and hepatomegaly.

? Type I glycogen storage disease? Autosomal recessive disorder? Caused by glucose- 6- phosphatase deficiency in liver. kidney and intestinal mucosa? Two subtypes: Type Ia; in which the glucose-6-phosphatase enzyme is defective? Type IIb: in which the translocase that transports glucose-6-phosphatase across the microsomal membrane is defective.

Clinical features:

? Develop hypoglycemia and lactic acidosis during neonatal period.? Present at 3-4 months of age with hepatomegaly.? Children have doll like faces with fat cheeks, relatively thin extremities,short stature and a protuberant abdomen which is due to massive hepatomegaly.? Easy bruising and epistaxis.

Ref: Harrison 17th Edition, Page 2459; Nelson 18th Edition, 03, 604, 605


A 2-month-old boy is evaluated for failure to thrive. As the pediatrician examined the child, she witnesses a seizure. Physical examination is remarkable for hepatomegaly, a finding later confirmed by CT scan, which also reveals renomegaly. Serum chemistries

demonstrate severe hypoglycemia, hyperlipidemia, lactic acidosis, and ketosis. Which of the following diseases best accounts for this presentation?

A: Gaucher's diseaseB: McArdle's diseaseC: Niemann-Pick diseaseD: Von Gierke's disease

Correct Ans:DExplanationVon Gierke's disease is a glycogen storage disease caused by a deficiency of glucose-6-phosphatase. It typically presents with neonatal hypoglycemia, hyperlipidemia, lactic acidosis, and ketosis. Failure to thrive is common in early life; convulsions may occur due to profound hypoglycemia. The glycogen accumulation in von Gierke's disease occurs primarily in the liver and kidneys, accounting for the enlargement of these organs. Gout may develop later because of the derangement of glucose metabolism.

Even if you do not remember all of the details of the presentation of these genetic diseases, you should be able to narrow the choices:

Gaucher's disease and Niemann-Pick disease are lipid storage diseases, and would not be expected to produce hypoglycemia.The other diseases are glycogen storage diseases, but McArdle's and Pompe's disease affect muscle rather than liver and would not be expected to produce profound hypoglycemia, since the liver is the major source for blood glucose.


A 5 year old child with hepatomegaly, hypoglycemia, and growth failure is admitted in the hospital for further evaluations. These signs are pathognomonic of:

A: DMB: Glycogen storage diseaseC: Lipid disorderD: All of the aboveCorrect Ans:BExplanationDifferent enzyme defects in glycogen storage diseases affect its biosynthesis and degradation. The hepatic forms of the glycogenoses cause growth failure, hepatomegaly, and severe fasting hypoglycemia.

They include,

Glucose-6-phosphatase deficiency (type I; von Gierke disease) Debrancher enzyme deficiency (type III) Hepatic phosphorylase deficiency (type VI) Phosphorylase kinase deficiency (type IX)

Ref: Thomas J.A., Van Hove J.L. (2012). Chapter 36. Inborn Errors of Metabolism. In W.W. Hay, Jr., M.J. Levin, R.R. Deterding, J.J. Ross, J.M. Sondheimer (Eds), CURRENT Diagnosis & Treatment: Pediatrics, 21e.


Child of Vasanthi was weaned from breast milk on the 5th day and was given sugarcane juice the child developed hypoglycemia and hepatomegaly. Biochemical examination showed hypophosphatemia and reducing substances in urine. The child is probably suffering from the following enzyme deficiency:

A: FructokinaseB: Fructose-1-phosphate aldolaseC: Glucose 6 PhosphataseD: Beta galactosidase

Correct Ans:BExplanation

Hereditary fructose intolerance is an autosomal recessive disorder in which deficient activity of fructose-1-phosphate aldolase causes hypoglycemia and tissue accumulation of fructose-1-phosphate on fructose ingestion. Other abnormalities include,

Failure to thrive Vomiting

Jaundice Hepatomegaly Proteinuria Renal Fanconi syndrome Liver failure

The diagnosis is suggested by finding fructosuria or an abnormal transferrin glycoform in the untreated patient. Diagnosis is made by sequencing the HFI gene. An alternative method for diagnosis is enzyme assay of fructose-1-phosphate aldolase in liver biopsy.

Ref: Thomas J.A., Van Hove J.L. (2012). Chapter 36. Inborn Errors of Metabolism. In W.W. Hay, Jr., M.J. Levin, R.R. Deterding, J.J. Ross, J.M. Sondheimer (Eds), CURRENT Diagnosis & Treatment: Pediatrics, 21e.


An 8 year old boy presented with fever and bilateral cervical lymphadenopathy with prior history of sore throat. There was no hepatomegaly. The peripheral blood smear shows > 20% lympho-plasmacytoid cells. The most likely diagnosis is:

A: InfluenzaB: TuberculosisC: Infectious mononucleosisD: Acute lymphoblastic leukemiaCorrect Ans:CExplanation

Most infections in children are asymptomatic. In symptomatic cases, there is fever, malaise, sore-throat and loss of appetite to begin with. There may be ulceration in oral cavity with enanthem at junction of hard palate and soft palate. Spleen is enlarged in more than half of the patients. Periorbital edema is reported in 33% of cases.

Ref: Kliegman, Behrman, Jenson, Stanton, (2007), Chapter 251, “Epstein Barr Virus”, In the book, “Nelson’s Textbook of Pediatrics”, Volume 1, 18th Edition, New Delhi, Pages 1372-1376


A female infant appeared normal at birth but developed signs of liver disease and muscular weakness at 3 months. She had periods of hypoglycemia, particularly on awakening. Examination revealed hepatomegaly. Laboratory analyses following fasting revealed ketoacidosis, blood pH 7.25, and elevations in both alanine transaminase (ALT) and aspartate transaminase (AST). Administration of glucagon following a carbohydrate meal elicited a normal rise in blood glucose, but glucose levels did not rise when glucagon was administered following an overnight fast. Liver biopsy revealed an increase in the glycogen content. To prevent the frequent episodes of hypoglycemia, which of the following dietary supplements would be most appropriate for this patient?

A: Casein (milk protein)B: Uncooked cornstarchC: FructoseD: Lactose


Because fasting hypoglycemia results from an inability to break down glycogen past the limit dextrin of phosphorylase, a patient with type III glycogen storage disease should be given frequent meals high in carbohydrates.

Uncooked cornstarch is an effective supplement because it is slowly digested, and therefore the glucose is released slowly into the bloodstream, helping to maintain blood glucose concentrations.

Ref: Powers A.C., D'Alessio D. (2011). Chapter 43. Endocrine Pancreas and Pharmacotherapy of Diabetes Mellitus and Hypoglycemia. In L.L. Brunton, B.A. Chabner, B.C. Knollmann (Eds), Goodman & Gilman's The Pharmacological Basis of Therapeutics, 12e.


A child presents with hepatomegaly and bilateral lenticular opacities. Which of the following enzyme deficiency will NOT cause such features?

A: Galactose-1 -phosphate uridyl transferaseB: UDP-galactose-4-epimeraseC: Galactokinase

D: Lactase

Correct Ans:DExplanationInability to metabolize galactose occurs in the galactosemias, which may be caused by inherited defects of galactokinase, uridyl transferase, or 4-epimerase, though deficiency ofuridyl transferase is best known. Galactose is a substrate for aldose reductase, forming galactitol, which accumulates in the lens of the eye, causing cataract. Ultimately, liver failure and mental deterioration result.

Ref: Bender D.A., Mayes P.A. (2011). Chapter 21. The Pentose Phosphate Pathway & Other Pathways of Hexose Metabolism. In D.A. Bender, K.M. Botham, P.A. Weil, P.J. Kennelly, R.K. Murray, V.W. Rodwell (Eds), Harper's Illustrated Biochemistry, 29e.


Hepatomegaly is a feature of all of the following, EXCEPT:

A: Hurler's DiseaseB: Hepatic porphyriasC: Von Gierke's DiseaseD: Nieman Pick DiseaseCorrect Ans:BExplanation

Frank Hepatomegaly is rare in hepatic porphyrias. Although hepatomegaly may occasionally be seen in hepatic porphyrias, frank hepatomegaly is rare and not a prominent manifestation of hepatic porphyria. Hepatic porphyria is therefore the single best answer by exclusion.

Hepatomegaly is a prominent manifestation of Hurler's syndrome, Nieman Pick disease and Von Girke's Disease.

Ref: Harrison’s Internal Medicine, 16th Edition, Pages 2316, 2320; Oxford Textbook of Medicine, 4th Edition, Page 69; Gastrointestinal and Liver Disease By Feldman, 8th Edition, Page 1623; Harrison’s Principles of Internal Medicine, 18th Edition, Chapters 361-362


Which of the following is not commonly seen in Hepatocellular carcinoma?

A: Raised AFPB: Raised ALPC: HepatomegalyD: Jaundice

Correct Ans:DExplanation

Among the common presenting symptoms of patients with HCC, jaundice is not commonly seen. It is seen only when there is mechanical obstruction of the bile duct. Most of the patients presents with abdominal pain or are completely asymptomatic and are detected on routine laboratory investigation. Hepatomegaly is the most common physical sign. AFP levels are elevated in more than half of the cases.

Reference:

Harrison's Principles of Internal Medicine 18e chapter 92.


All are TRUE about the clinical features of polycythemia rubra vera, EXCEPT:

A: Hepatomegaly is the initial presenting signB: Aquagenic pruritus presentC: Vertigo, tinnitus, headache and visual disturbances are due to hyperviscosityD: Systolic hypertension is a feature of increased red cell mass

Correct Ans:AExplanationPolycythemia rubra vera clinical features:

Splenomegaly may be the initial presenting sign Aquagenic pruritus Hyperviscosity leads to vertigo, tinnitus, headache, visual disturbances,

and transient ischemic attacks (TIAs). Systolic hypertension is due to red cell mass elevation. Venous or arterial thrombosis may be the presenting manifestation of

PV. Erythromelalgia, is a complication of the thrombocytosis Hyperuricemia with secondary gout, uric acid stones, and symptoms due

to hypermetabolism can also complicate the disorder.

Ref: Harrison, E-18, P-899


Which of the following condition is associated with hepatomegaly with liver pulsation?

A: Mitral StenosisB: Mitral RegurgitationC: Tricuspid RegurgitationD: Pulmonary HypertensionCorrect Ans:CExplanation

Characteristic features of Tricuspid regurgitation are marked hepatomegaly and systolic pulsations of the liver. Hepatic pulsation in tricuspid regurgitation is caused by reversed systolic blood flow in the great veins.

Ref: Current Medical Diagnosis and Treatment, 2012, Chapter 10; The Cleveland Clinic Cardiology Board Review By Brian P. Griffin, Page 290


All of the following are features of Right ventricular infarction EXCEPT:

A: NocturiaB: Normal JVPC: HepatomegalyD: Ascites


Right sided MI will lead to right ventricular dysfunction with features of right ventricular failure. Features of right ventricular failure are :

Backward failure of the right ventricle leads to congestion of systemic capillaries. Tk generates excess fluid accumulation in the body. This causes swelling under the skin (termed peripheral edema or anasarca) and usual affects the dependent parts of the body first

(causing foot and ankle swelling in people w are standing up, and sacral edema in people who are predominantly lying down).

Nocturia (frequent nighttime urination) may occur when fluid from the legs is returned to the bloodstream while lying down at night.

In progressively severe cases, ascites (fluid accumulation in the abdominal cavity caus:- swelling) hepatomegaly (enlargement of the liver) may develop.

Significant liver congestion may result in impaired liver function, and jaundice even coagulopathy (problems of decreased blood clotting) may occur.

Pleural effusion, Raised JVP, positive hepatojuglar reflex, parasternal heave.


Hepatomegaly is a feature of all of the following, EXCEPT:

A: Hurler's DiseaseB: Hepatic porphyriasC: Von Gierke's DiseaseD: Nieman Pick DiseaseCorrect Ans:BExplanation

Frank Hepatomegaly is rare in hepatic porphyrias. Although hepatomegaly may occasionally be seen in hepatic porphyrias, frank hepatomegaly is rare and not a prominent manifestation of hepatic porphyria. Hepatic porphyria is therefore the single best answer by exclusion.

Hepatomegaly is a prominent manifestation of Hurler's syndrome, Nieman Pick disease and Von Girke's Disease.

Ref: Harrison’s Internal Medicine, 16th Edition, Pages 2316, 2320; Oxford Textbook of Medicine, 4th Edition, Page 69; Gastrointestinal and Liver Disease By Feldman, 8th Edition, Page 1623; Harrison’s Principles of Internal Medicine, 18th Edition, Chapters 361-362


A 2 weeks old newborn is brought to the clinic with complaints of vomiting and diarrhea. On examination she

appears to be jaundiced, shows hepatomegaly and bilateral cataract.

Assertion: Child is showing features of Galactosemia which is an autosomal recessive disorder.

Reason: There is a deficiency of the enzyme Galactose 1 phosphate uridyl transferase which result in accumulation of galactose 1 phosphate.

A: Both Assertion and Reason are true, and Reason is the correct explanation for Assertion

B:Both Assertion and Reason are true, and Reason is not the correct explanation for Assertion

C: Assertion is true, but Reason is falseD: Assertion is false, but Reason is trueCorrect Ans:AExplanation

Neonate in the question is showing features of galactosemia caused by deficiency of the enzyme galactose 1 phosphate uridyl transferase. Deficiency of this enzyme result in accumulation of galactose 1 phosphate. Neonates usually presents with vomiting, diarrhea, failure to thrive and prolongation of physiologic jaundice. Examination shows evidence of cataract and hepatomegaly.

Ref: Essentials of Paediatrics by OP Ghai, 6th Edition, Page 613.


previous year question on hepatomegaly based on neet pg, usmle, plab and fmge or mci screening exams

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