Fats and Fatty Acid Metabolism - Mock Test 44

Question 431

Ochronosis is an important finding of?

A) Tyrosinemia B) Tyrosinosis C) Alkaptonuria D) Richner Hanhart syndrome

---

No Details

Question 432

An important feature of maple syrup urine disease is?

A) Patient can not be treated by dietary regulation B) Without treatment death, of patient may occur by the end of second year of life C) Blood levels of leucine, isoleucine and serine are increased D) Excessive brain damage

---

No Details

Question 433

Alkaptonuria occurs due to deficiency of the enzyme?

A) Maleylacetoacetate isomerase B) Homogentisate oxidase C) p-Hydroxyphenylpyruvate hydroxylase D) Fumarylacetoacetate hydrolase

---

No Details

Question 434

Maple syrup urine disease becomes evident in extra uterine life by the end of?

A) First week B) Second week C) Third week D) Fourth week

---

No Details

Question 435

An inborn error, maple syrup urine disease is due to deficiency of the enzyme?

A) Isovaleryl-CoAhydrogenase B) Phenylalnine hydroxylase C) Adenosyl transferase D) α-Ketoacid decarboxylase

---

No Details

Question 436

In glycinuria daily urinary excretion of glycine ranges from?

A) 100–200 mg B) 300–500 mg C) 600–1000 mg D) 1100–1400 mg

---

No Details

Question 437

Increased urinary indole acetic acid is diagnostic of?

A) Maple syrup urine disease B) Hartnup disease C) Homocystinuia D) Phenylketonuria

---

No Details

Question 438

An important finding in glycinuria is?

A) Excess excretion of oxalate in the urine B) Deficiency of enzyme glycinase C) Significantly increased serum glycine level D) Defect in renal tubular reabsorption of glycine

---

No Details

Question 439

An important finding in Histidinemia is?

A) Impairment of conversion of α-Glutamate to α-ketoglutarate B) Speech defect C) Decreased urinary histidine level D) Patients can not be treated by diet

---

No Details

Question 440

Tyrosinosis is due to defect in the enzyme?

A) Fumarylacetoacetate hydrolase B) p-Hydroxyphenylpyruvate hydroxylase C) Tyrosine transaminase D) Tyrosine hydroxylase

---

No Details