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

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
by

1 Answer

Answer :

Answer : D
by
selected

Related questions

Branched chain amino acids are (A) Cysteine and cystine (B) Tyrosine and Tryptophan (C) Glycine and Serine (D) Valine, Leucine and Isoleucine

Description : Branched chain amino acids are (A) Cysteine and cystine (B) Tyrosine and Tryptophan (C) Glycine and Serine (D) Valine, Leucine and Isoleucine

Last Answer : Answer : D

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

Description : 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

Last Answer : Answer : A

Increased urinary indole acetic acid is diagnostic of (A) Maple syrup urine disease (B) Hartnup disease (C) Homocystinuia (D) Phenylketonuria

Description : Increased urinary indole acetic acid is diagnostic of (A) Maple syrup urine disease (B) Hartnup disease (C) Homocystinuia (D) Phenylketonuria

Last Answer : Answer : B

Maple syrup urine disease results from absence or serve deficiency of (A) Homogentisate oxidase (B) Phenylalanine hydroxylase (C) Branched chain amino acid transaminase (D) None of these

Description : Maple syrup urine disease results from absence or serve deficiency of (A) Homogentisate oxidase (B) Phenylalanine hydroxylase (C) Branched chain amino acid transaminase (D) None of these

Last Answer : Answer : D

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

Description : 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

Last Answer : Answer : D

In which of the following is mental retardation an expected finding? 1) Alkaptonuria 2) Cystinuria 3) Glycogen storage disease 4) Lactose intolerance 5) Maple syrup urine disease

Description : In which of the following is mental retardation an expected finding? 1) Alkaptonuria 2) Cystinuria 3) Glycogen storage disease 4) Lactose intolerance 5) Maple syrup urine disease

Last Answer : Answers-5 MENTAL RETARDATION. Fragile X syndrome-commonest male cause. Hypoxia at birth, intaventricular haemorrhage, rhesus disease, Congenital infections - toxoplasmosis, CMV, rubella ... with diet. -homocystinuria, phenylketonuria -maple syrup urine disease, tryptophanuria -galactosaemia

Methylmalonyl CoA is a metabolite of (A) Valine (B) Leucine (C) Isoleucine (D) All of these

Description : Methylmalonyl CoA is a metabolite of (A) Valine (B) Leucine (C) Isoleucine (D) All of these

Last Answer : Answer : A

In the synthesis of Angiotensin I, rennin acts on Angiotensinogen and cleaves the (A) Leucine – leucine at 10 and 11 position (B) Valine – tyrosine at 3 and 4 position (C) Isoleucine – histidine at 5 and 6 position (D) Proline – histidine at 7 and 8 position

Description : In the synthesis of Angiotensin I, rennin acts on Angiotensinogen and cleaves the (A) Leucine – leucine at 10 and 11 position (B) Valine – tyrosine at 3 and 4 position (C) Isoleucine – histidine at 5 and 6 position (D) Proline – histidine at 7 and 8 position

Last Answer : Answer : A

Coenzyme A is required for catabolism of (A) Leucine (B) Isoleucine (C) Valine (D) All of these

Description : Coenzyme A is required for catabolism of (A) Leucine (B) Isoleucine (C) Valine (D) All of these

Last Answer : Answer : D

HMG CoA is formed in the metabolism of (A) Cholesterol, ketones and leucine (B) Cholesterol, fatty acid and Leucine (C) Lysine, Lecuine and Isoleucine (D) Ketones, Leucine and Lysine

Description : HMG CoA is formed in the metabolism of (A) Cholesterol, ketones and leucine (B) Cholesterol, fatty acid and Leucine (C) Lysine, Lecuine and Isoleucine (D) Ketones, Leucine and Lysine

Last Answer : Answer : A

Carboxy peptidase B in the small intestine hydrolyzes peptides containing (A) Leucine (B) Isoleucine (C) Arginine (D) Cysteine

Description : Carboxy peptidase B in the small intestine hydrolyzes peptides containing (A) Leucine (B) Isoleucine (C) Arginine (D) Cysteine

Last Answer : Answer : C

All followings are ketogenic aminoacids except (A) Leucine (B) Isoleucine (C) Phenyl alanine (D) Glycine

Description : All followings are ketogenic aminoacids except (A) Leucine (B) Isoleucine (C) Phenyl alanine (D) Glycine

Last Answer : Answer : D

All the following are branched chain amino acids except (A) Isoleucine (B) Alanine (C) Leucine (D) Valine

Description : All the following are branched chain amino acids except (A) Isoleucine (B) Alanine (C) Leucine (D) Valine

Last Answer : Answer : B

Answer: b Metabolic response to injury results in increased energy expenditure. If energy intake is less than expenditure, oxidation of body fat stores and erosion of lean body mass will occur with resultant loss of weight. When weight loss exceeds 10–15% of body weight, the complications of malnutrition interact with disease processes, with increased morbidity and mortality rates. The goal of nutritional support is maintenance of body cell mass and limitation of weight loss to less than 10% preinjury. The major impact of nutritional support in the trauma patient is to aid host defense. Under-nutrition may compromise the available host defense mechanism and may thus increase the likelihood of invasive sepsis, multiple organ system failure, and death. Resuscitation, oxygenation and arrest

Description : Answer: b Metabolic response to injury results in increased energy expenditure. If energy intake is less than expenditure, oxidation of body fat stores and erosion of lean body mass will occur ... requirements, caloric requirements should be distributed at a ratio of 70% as glucose and 30% as fat

Last Answer : Which of the following statement(s) is/are true concerning body fuel reserves? a. The largest fuel reserve in the body is skeletal muscle b. Fat provides about 9 calories/gram c. Free glucose and glycogen stores are a trivial fuel reserve d. Body protein is a valuable storage form of energy

Maple syrup urine disease?

Description : Maple syrup urine disease?

Last Answer : DefinitionMaple syrup urine disease (MSUD) is a metabolism disorder passed down through families in which the body cannot break down certain parts of proteins. Urine in persons with this condition ... . Even in the mildest form, repeated periods of physical stress can cause mental retardationand h

-child , urine odor like burned sugar: - Phenylketonuria - Maple syrup urine disease

Description : -child , urine odor like burned sugar: - Phenylketonuria - Maple syrup urine disease

Last Answer : Maple syrup urine disease _______________________________

Maple syrup urine diseases is an inborn error of metabolism of (A) Sulphur-containing amino acids (B) Aromatic amino acids (C) Branched chain amino acids (D) Dicarboxylic amino acids

Description : Maple syrup urine diseases is an inborn error of metabolism of (A) Sulphur-containing amino acids (B) Aromatic amino acids (C) Branched chain amino acids (D) Dicarboxylic amino acids

Last Answer : Answer : C

A ketogenic amino acid among the following is (A) Leucine (B) Serine (C) Threonine (D) Proline

Description : A ketogenic amino acid among the following is (A) Leucine (B) Serine (C) Threonine (D) Proline

Last Answer : Answer : A

In glutathione (a tripeptide) is present apart from Glutamic acid and cysteine: (A) Serine (B) Glycine (C) Leucine (D) Phenyl alanine

Description : In glutathione (a tripeptide) is present apart from Glutamic acid and cysteine: (A) Serine (B) Glycine (C) Leucine (D) Phenyl alanine

Last Answer : Answer : B

One of the given example is an amino acid: (A) Oh-Lysine (B) Protein (C) Leucine (D) Serine

Description : One of the given example is an amino acid: (A) Oh-Lysine (B) Protein (C) Leucine (D) Serine

Last Answer : Answer : B

After being counseled about lifestyle and dietary changes, the patient was started on atorvastatin. During his treatment with atorvastatin, it is important to routinely monitor serum concentrations of (a) Blood urea nitrogen (BUN) (b) Alanine and aspartate aminotransferase (c) Platelets (d) Red blood cells (e) Uric acid

Description : After being counseled about lifestyle and dietary changes, the patient was started on atorvastatin. During his treatment with atorvastatin, it is important to routinely monitor serum concentrations of (a) ... Alanine and aspartate aminotransferase (c) Platelets (d) Red blood cells (e) Uric acid

Last Answer : Ans: D

.Khorana first deciphered the triplet codons of (a) serine and isoleucine (b) cysteine and valine (c) tyrosine and tryptophan (d) phenylalanine and methinonine.

Description : .Khorana first deciphered the triplet codons of (a) serine and isoleucine (b) cysteine and valine (c) tyrosine and tryptophan (d) phenylalanine and methinonine.

Last Answer : (a) serine and isoleucine

Khorana first deciphered the triplet codons of (a) serine and isoleucine (b) cysteine and valine (c) tyrosine and tryptophan (d) phenylalanine and methinonine

Description : Khorana first deciphered the triplet codons of (a) serine and isoleucine (b) cysteine and valine (c) tyrosine and tryptophan (d) phenylalanine and methinonine

Last Answer : (b) cysteine and valine

Ammonia intoxication symptoms occur when brain ammonia levels are (A) Slightly diminished (B) Highly diminished (C) Increased (D) All of these

Description : Ammonia intoxication symptoms occur when brain ammonia levels are (A) Slightly diminished (B) Highly diminished (C) Increased (D) All of these

Last Answer : Answer : C

For individuals known to be dying by virtue of age and/or diagnoses, which of the following signs indicate approaching death: a) Increased restlessness As the oxygen supply to the brain decreases, the patient may become restless. b) Increased wakefulness As the body weakens, the patient will sleep more and begin to detach from the environment. c) Increased eating For many patients, refusal of food is an indication that they are ready to die. d) Increased urinary output Based upon decreased intake, urinary output generally decreases in amount and frequency.

Description : For individuals known to be dying by virtue of age and/or diagnoses, which of the following signs indicate approaching death: a) Increased restlessness As the oxygen supply to the ... Increased urinary output Based upon decreased intake, urinary output generally decreases in amount and frequency.

Last Answer : a) Increased restlessness As the oxygen supply to the brain decreases, the patient may become restless.

Which of the following amino acids has an aliphatic R group? (a) Serine (b) Cysteine (c) Asparagine (d) Leucine

Description : Which of the following amino acids has an aliphatic R group? (a) Serine (b) Cysteine (c) Asparagine (d) Leucine

Last Answer : Leucine

A 73 year old male presented with an acute attack of gout in his left knee. What is the most likely underlying metabolic cause? 1) decreased renal excretion of uric acid 2) endogenous overproduction of uric acid 3) excessive dietary purine intake 4) lactic acidosis 5) starvation

Description : A 73 year old male presented with an acute attack of gout in his left knee. What is the most likely underlying metabolic cause? 1) decreased renal excretion of uric acid 2) endogenous overproduction of uric acid 3) excessive dietary purine intake 4) lactic acidosis 5) starvation

Last Answer : Answers-1 The aetiology of gout can broadly be divided into cases where there is underexcretion of urate via the kidney (90%) or endogenous overproduction of uric acid (10%) although in practical ... diuretic use. Excessive dietary intake of purines is unlikely to be the main cause in this case.

Which of the following statement(s) is/are true concerning the treatment of MOFS? a. Prevention and therapy of MOFS requires control of the infectious or inflammatory source b. Restoration of normal clinical parameters such as blood pressure, pulse rate, and urine output ensures optimal resuscitation in most patients c. Branch chain amino acids play and important role in the nutritional support of the patient d. Because of the nature of gut injury, total parenteral nutrition is preferred for most patients with MOFS

Description : Which of the following statement(s) is/are true concerning the treatment of MOFS? a. Prevention and therapy of MOFS requires control of the infectious or inflammatory source b. Restoration of normal ... of the nature of gut injury, total parenteral nutrition is preferred for most patients with MOFS

Last Answer : Answer: a, c The therapy of MOFS is directed towards interrupting the involving pathophysiologic process and providing an optimal physiologic environment for healing and recovery. ... Enteral absorption and processing of nutrients appears superior to TPN and lessens overall complications

Which of the following clinical characteristics is associated with Type 2 diabetes (previously referred to as non-insulin-dependent diabetes mellitus [NIDDM])? a) Can control blood glucose through diet and exercise Oral hypoglycemic agents may improve blood glucose levels if dietary modification and exercise are unsuccessful.

Description : Which of the following clinical characteristics is associated with Type 2 diabetes (previously referred to as non-insulin-dependent diabetes mellitus [NIDDM])? a) Can control blood glucose ... islet cell antibodies Individuals with Type 2 diabetes do not demonstrate islet cell antibodies.

Last Answer : a) Can control blood glucose through diet and exercise Oral hypoglycemic agents may improve blood glucose levels if dietary modification and exercise are unsuccessful.

The following is true of selegiline: A. It does not exert antiparkinsonian action unless combined with levodopa B. It overcomes the ‘on-off ’ effect in levodopa treated advanced parkinsonian patients C. It retards the progression of Parkinson's disease D. At doses used in parkinsonism it does not interfere with peripheral metabolism of dietary amines

Description : The following is true of selegiline: A. It does not exert antiparkinsonian action unless combined with levodopa B. It overcomes the on-off ' effect in levodopa treated advanced parkinsonian ... D. At doses used in parkinsonism it does not interfere with peripheral metabolism of dietary amines

Last Answer : . At doses used in parkinsonism it does not interfere with peripheral metabolism of dietary amines

Which of the following statements about head injury and concomitant hyponatremia are true? A. There are no primary alterations in cardiovascular signs. B. Signs of increased intracranial pressure may be masked by the hyponatremia. C. Oliguric renal failure is an unlikely complication. D. Rapid correction of the hyponatremia may prevent central pontine injury. E. This patient is best treated by restriction of water intake.

Description : Which of the following statements about head injury and concomitant hyponatremia are true? A. There are no primary alterations in cardiovascular signs. B. Signs of increased intracranial pressure may be ... central pontine injury. E. This patient is best treated by restriction of water intake.

Last Answer : Answer: A DISCUSSION: Acute symptomatic hyponatremia is characterized by central nervous system signs of increased intracranial pressure. Changes in blood pressure and pulse are secondary to ... lead to central pontine myelinolysis. Oliguric renal failure may rapidly develop in severe hyponatremia

When the nurse notes that the post cardiac surgery patient demonstrates low urine output (< 25 ml/hr) with high specific gravity (> 1.025), the nurse suspects: a) Inadequate fluid volume Urine output of less than 25 ml/hr may indicate a decrease in cardiac output. A high specific gravity indicates increased concentration of solutes in the urine which occurs with inadequate fluid volume. b) Normal glomerular filtration Indices of normal glomerular filtration are output of 25 ml or greater per hour and specific gravity between 1.010 and 1.025. c) Overhydration Overhydration is manifested by high urine output with low specific gravity. d) Anuria The anuric patient does not produce urine.

Description : When the nurse notes that the post cardiac surgery patient demonstrates low urine output (< 25 ml/hr) with high specific gravity (> 1.025), the nurse suspects: a) Inadequate fluid volume Urine ... by high urine output with low specific gravity. d) Anuria The anuric patient does not produce urine.

Last Answer : a) Inadequate fluid volume Urine output of less than 25 ml/hr may indicate a decrease in cardiac output. A high specific gravity indicates increased concentration of solutes in the urine which occurs with inadequate fluid volume.

A patient suffers a blow to his maxillary central incisor without resulting in fracture. The pulp may: A. Show immediate necrosis B. Become non-vital but only if treatment is delayed too long C. Become non vital irrespective of treatment D. No changes are seen later if fracture does not occur

Description : A patient suffers a blow to his maxillary central incisor without resulting in fracture. The pulp may: A. Show immediate necrosis B. Become non-vital but only if treatment is delayed too long C. Become non vital irrespective of treatment D. No changes are seen later if fracture does not occur

Last Answer : C. Become non vital irrespective of treatment

The most suitable laxative for a patient of irritable bowel disease with spastic constipation is: A. Dietary fibre B. Liquid paraffin C. Bisacodyl D. Senna

Description : The most suitable laxative for a patient of irritable bowel disease with spastic constipation is: A. Dietary fibre B. Liquid paraffin C. Bisacodyl D. Senna

Last Answer : A. Dietary fibre

In the genetic disorder of cystinuria, the patient excretes large quantities of cystine in their urine and its low solubility causes crystalline cystine to precipitate as stones in kidneys. The remedy involves ingesting Na HCO3. Reaction of this treatment is (A) NaHCO2 combines with cystine (B) NaHCO3 raises the pH above the isoelectric point of cystine (C) NaHCO3 prevents stone formation by hydrolysis of cystine to cysteine (D) None of these

Description : In the genetic disorder of cystinuria, the patient excretes large quantities of cystine in their urine and its low solubility causes crystalline cystine to precipitate as stones in kidneys. The remedy ... C) NaHCO3 prevents stone formation by hydrolysis of cystine to cysteine (D) None of these

Last Answer : Answer : C

The nurse recognizes which of the following signs as typical of the patient in shock? a) Rapid, weak, thready pulse Pulse increases as the body tries to compensate. b) Flushed face Pallor is an indicator of shock. c) Warm, dry skin Skin is generally cool and moist in shock. d) Increased urine output Usually, a low blood pressure and concentrated urine are observed in the patient in shock.

Description : The nurse recognizes which of the following signs as typical of the patient in shock? a) Rapid, weak, thready pulse Pulse increases as the body tries to compensate. b) Flushed face Pallor is ... urine output Usually, a low blood pressure and concentrated urine are observed in the patient in shock.

Last Answer : a) Rapid, weak, thready pulse Pulse increases as the body tries to compensate.

During compensation of respiratory alkalosis, all the following changes occur except (A) Decreased secretion of hydrogen ions by renal tubules (B) Increased excretion of sodium in urine (C) Increased excretion of bicarbonate in urine (D) Increased excretion of ammonia in urine

Description : During compensation of respiratory alkalosis, all the following changes occur except (A) Decreased secretion of hydrogen ions by renal tubules (B) Increased excretion of sodium in urine (C) Increased excretion of bicarbonate in urine (D) Increased excretion of ammonia in urine

Last Answer : Answer : D

All of the following occur in orotic aciduria except (A) Increased synthesis of pyrimidine nucleotides (B) Increased excretion of orotic acid in urine (C) Decreased synthesis of cytidine triphosphate (D) Retardation of growth

Description : All of the following occur in orotic aciduria except (A) Increased synthesis of pyrimidine nucleotides (B) Increased excretion of orotic acid in urine (C) Decreased synthesis of cytidine triphosphate (D) Retardation of growth

Last Answer : Answer : A

A 35-year-old male presented with an attack of acute gout. He was treated with a 10 day course of naproxen. His blood uric acid level is high. What future line of treatment is most appropriate: A. No regular medication. Treat attacks of acute gout when they occur with naproxen. B. Regular long-term treatment with naproxen C. Regular long-term treatment with allopurinol D. Start with allopurinol + naproxen for 2 months followed by long-term allopurinol treatment

Description : A 35-year-old male presented with an attack of acute gout. He was treated with a 10 day course of naproxen. His blood uric acid level is high. What future line of treatment is ... treatment with allopurinol D. Start with allopurinol + naproxen for 2 months followed by long-term allopurinol treatment

Last Answer : D. Start with allopurinol + naproxen for 2 months followed by long-term allopurinol treatment

Which of the following statements are true of a patient with hyperglycemia and hyponatremia? A. The sodium concentration must be corrected by 5 mEq. per 100 mg. per 100 ml. elevation in blood glucose. B. With normal renal function, this patient is likely to be volume overloaded. C. Proper fluid therapy would be unlikely to include potassium administration. D. Insulin administration will increase the potassium content of cells. E. Early in treatment adequate urine output is a reliable measure of adequate volume resuscitation.

Description : Which of the following statements are true of a patient with hyperglycemia and hyponatremia? A. The sodium concentration must be corrected by 5 mEq. per 100 mg. per 100 ml. elevation in blood ... . E. Early in treatment adequate urine output is a reliable measure of adequate volume resuscitation.

Last Answer : Answer: D DISCUSSION: Each 100-mg. per 100 ml. elevation in blood glucose causes a fall in serum sodium concentration of approximately 2 mEq. per liter. Excess serum glucose ... correction of the patient's associated acidosis produce movement of potassium ions into the intracellular compartment

A patient has been taking digoxin for several years for chronic heart failure is about to receive atropine for another condition. A common effect of digoxin (at therapeutic blood levels) that can be almost entirely blocked by atropine is (a) Decreased appetite (b) Increased atrial contractility (c) Increased PR interval on the ECG (d) Headaches (e) Tachycardia

Description : A patient has been taking digoxin for several years for chronic heart failure is about to receive atropine for another condition. A common effect of digoxin (at therapeutic blood levels) that can ... Increased atrial contractility (c) Increased PR interval on the ECG (d) Headaches (e) Tachycardia

Last Answer : Ans: C

Which stage of shock is best described as that stage when the mechanisms that regulate blood pressure fail to sustain a systolic pressure above 90 mm Hg? a) Progressive In the progressive stage of shock, the mechanisms that regulate blood pressure can no longer compensate, and the mean arterial pressure falls below normal limits. b) Refractory The refractory or irreversible stage of shock represents the point at which organ damage is so severe that the patient does not respond to treatment and cannot survive.

Description : Which stage of shock is best described as that stage when the mechanisms that regulate blood pressure fail to sustain a systolic pressure above 90 mm Hg? a) Progressive In the progressive stage of ... which organ damage is so severe that the patient does not respond to treatment and cannot survive.

Last Answer : a) Progressive In the progressive stage of shock, the mechanisms that regulate blood pressure can no longer compensate, and the mean arterial pressure falls below normal limits.

The presence of bilirubin in the urine without urobilinogen suggests (A) Obstructive jaundice (B) Hemolytic jaundice (C) Pernicious anemia (D) Damage to the hepatic parenchyma

Description : The presence of bilirubin in the urine without urobilinogen suggests (A) Obstructive jaundice (B) Hemolytic jaundice (C) Pernicious anemia (D) Damage to the hepatic parenchyma

Last Answer : Answer : A

Down regulation is (A) Increased destruction of a hormone (B) Feed back inhibition of hormone secretion (C) Decreased concentration of a hormone in blood (D) Decrease in number of receptors for a hormone

Description : Down regulation is (A) Increased destruction of a hormone (B) Feed back inhibition of hormone secretion (C) Decreased concentration of a hormone in blood (D) Decrease in number of receptors for a hormone

Last Answer : Answer : D

Increased protein intake is accompanied by an increased dietary requirement for (A) Thiamine (B) Riboflavine (C) Folic acid (D) Nicotininic acid

Description : Increased protein intake is accompanied by an increased dietary requirement for (A) Thiamine (B) Riboflavine (C) Folic acid (D) Nicotininic acid

Last Answer : Answer : A

Increased carbohydrate consumption increases the dietary requirement for (A) Thiamine (B) Riboflavine (C) Pyridoxine (D) Folic acid

Description : Increased carbohydrate consumption increases the dietary requirement for (A) Thiamine (B) Riboflavine (C) Pyridoxine (D) Folic acid

Last Answer : Answer : A