What are the characteristic clinical features of von Gierkeís disease?

What are the characteristic clinical features of von Gierkeís disease?
by

1 Answer

Answer :

Fasting hypoglycemia, which does not respond to adrenaline is very characteristic.
by

Related questions

Which is the defective enzyme in von Gierkeís disease (glycogen storage disease type I)?

Description : Which is the defective enzyme in von Gierkeís disease (glycogen storage disease type I)? 

Last Answer : Glucose-6-phosphatase.

Clinical features of hyperthyroidism include (A) Goitre, heat intolerance, weight loss and tachycardia (B) Goitre, tremors, tachycardia and cold intolerance (C) Exophthalmos, goiter, tachycardia and loss of appetite (D) Exophthalmos, goiter, tremors and obesity

Description : Clinical features of hyperthyroidism include (A) Goitre, heat intolerance, weight loss and tachycardia (B) Goitre, tremors, tachycardia and cold intolerance (C) Exophthalmos, goiter, tachycardia and loss of appetite (D) Exophthalmos, goiter, tremors and obesity

Last Answer : Answer : A

Clinical features of Kwashiorkor include all of the following except (A) Mental retardation (B) Muscle wasting (C) Oedema (D) Anaemia

Description : Clinical features of Kwashiorkor include all of the following except (A) Mental retardation (B) Muscle wasting (C) Oedema (D) Anaemia

Last Answer : Answer : A

An important feature of Von-Gierke’s disease is (A) Muscle cramps (B) Cardiac failure (C) Hypoglycemia (D) Respiratory alkalosis

Description : An important feature of Von-Gierke’s disease is (A) Muscle cramps (B) Cardiac failure (C) Hypoglycemia (D) Respiratory alkalosis

Last Answer : Answer : C

Von Gierke’s disease is characterized by the deficiency of (A) Glucose-6-phosphatase (B) α -1→ 4 Glucosidase (C) 1→ 6 Glucosidase (D) Liver phosphorylase

Description : Von Gierke’s disease is characterized by the deficiency of (A) Glucose-6-phosphatase (B) α -1→ 4 Glucosidase (C) 1→ 6 Glucosidase (D) Liver phosphorylase

Last Answer : Answer : A

Debranching enzyme is absent in (A) Cori’s disease (B) Andersen’s disease (C) Von Gierke’s disease (D) Her’s disease

Description : Debranching enzyme is absent in (A) Cori’s disease (B) Andersen’s disease (C) Von Gierke’s disease (D) Her’s disease

Last Answer : A

Glucose-6-phosphatase is absent or deficient in (A) Von Gierke’s disease (B) Pompe’s disease (C) Cori’s disease (D) McArdle’s disease

Description : Glucose-6-phosphatase is absent or deficient in (A) Von Gierke’s disease (B) Pompe’s disease (C) Cori’s disease (D) McArdle’s disease

Last Answer : A

Von Willebrand disease?

Description : Von Willebrand disease?

Last Answer : DefinitionVon Willebrand disease is the most common hereditary bleeding disorder.Causes, incidence, and risk factorsVon Willebrand disease is caused by a deficiency of von Willebrand factor. Von Willebrand ... risk factor. In women with heavy or prolonged menstrual bleeding, von Willebrand is more

There are a number of hypercoaguable states which can be associated with arterial or venous thrombosis and embolic phenomenon. These include: a. Heparin-associated thrombocytopenia b. Antithrombin III deficiency c. Von Willebrand disease d. Vitamin C deficiency

Description : There are a number of hypercoaguable states which can be associated with arterial or venous thrombosis and embolic phenomenon. These include: a. Heparin-associated thrombocytopenia b. Antithrombin III deficiency c. Von Willebrand disease d. Vitamin C deficiency

Last Answer : Answer: a, b A number of hypercoaguable states are present. These include heparin-associated thrombocytopenia in which a heparin-dependent platelet antibody causes aggregation of ... soft tissue increasing vascular permeability and fragility resulting in the potential for bleeding disorders

Von Willebrand’s disease is a common, congenital bleeding disorder. Which of the following statement(s) is/are true concerning Von Willebrand’s disease? a. As in hemophilia, it is much more common in men b. A history of spontaneous bleeding is common c. Screening laboratory tests will include a prolonged aPTT with a normal prothrombin time d. Pre-treatment for elective surgery require administration of cryoprecipitate to achieve levels of 23–50% of normal

Description : Von Willebrand's disease is a common, congenital bleeding disorder. Which of the following statement(s) is/are true concerning Von Willebrand's disease? a. As in hemophilia, it is ... -treatment for elective surgery require administration of cryoprecipitate to achieve levels of 23-50% of normal

Last Answer : Answer: c, d Von Willebrand's factor is an adhesive protein that mediates platelet adhesion to collagen. In addition, it protects and prevents the rapid removal of factor VIII ... platelet aggregation responses to ristocetin. The most reliable source of Von Willebrand's factor is cryoprecipitate

Which of the statements listed below about bleeding disorders is/are correct? A. Acquired bleeding disorders are more common than congenital defects. B. Deficiencies of vitamin K decrease production of factors II, VII, IX, and X, protein C, and protein S. C. Hypothermia below 32؛C rarely causes a bleeding disorder. D. Von Willebrand's disease is a very uncommon congenital bleeding disorder.

Description : Which of the statements listed below about bleeding disorders is/are correct? A. Acquired bleeding disorders are more common than congenital defects. B. Deficiencies of vitamin K decrease production ... disorder. D. Von Willebrand's disease is a very uncommon congenital bleeding disorder.

Last Answer : Answer: AB DISCUSSION: Acquired bleeding disorders are significantly more common than congenital bleeding defects. Vitamin K deficiency may be related to malnutrition or competitive inhibition ... a relatively common disorder of bleeding and is generally undetectable by routine screening methods

Bleeding time is increased in: a. massive blood transfusion b. vitamin K deficiency c. von Willebrand's disease d. disseminated intravascular coagulation (DIC)

Description : Bleeding time is increased in: a. massive blood transfusion b. vitamin K deficiency c. von Willebrand's disease d. disseminated intravascular coagulation (DIC)

Last Answer : disseminated intravascular coagulation (DIC)

hematological disease occurs in children treated with heparin and fresh frozen plasma what is the disease : A)hemophilia A B)hemophilia B C )VON WILL brand disease d)DIC thrombosis

Description : hematological disease occurs in children treated with heparin and fresh frozen plasma what is the disease : A)hemophilia A B)hemophilia B C )VON WILL brand disease d)DIC thrombosis

Last Answer : d)DIC thrombosis

How do you treat a child with severe Von Willebrand’s disease:** A. Like a normal child B. Like a diabetic child C. Like a haemophilic child

Description : How do you treat a child with severe Von Willebrand’s disease:** A. Like a normal child B. Like a diabetic child C. Like a haemophilic child

Last Answer : C. Like a haemophilic child

Brown skin pigmentation does not occur in: A. Hyperparathyroidism B. Von Willebrand’s disease C. Addison’s desease

Description : Brown skin pigmentation does not occur in: A. Hyperparathyroidism B. Von Willebrand’s disease C. Addison’s desease

Last Answer : B. Von Willebrand’s disease

Von Willebrand disease is, A. Haemophilic disease B. Bacterial Endocarditis C. Congenital cardiac disease D. Rheumatic fever Decreased PTT, bleeding time prolonged, deficiency factor VIII

Description : Von Willebrand disease is, A. Haemophilic disease B. Bacterial Endocarditis C. Congenital cardiac disease D. Rheumatic fever Decreased PTT, bleeding time prolonged, deficiency factor VIII

Last Answer : A. Haemophilic disease

Café au lait spots are seen in: A. Von Willebrand’s disease B. Recklinghausen C. Neurofibromatosis

Description : Café au lait spots are seen in: A. Von Willebrand’s disease B. Recklinghausen C. Neurofibromatosis

Last Answer : . Recklinghausen C. Neurofibromatosis

The patient whom you are about to treat states that he has Von Willebrand’s disease. Which one of the following preoperative haematological analyses may reflect this disease: A. Bleeding time and factor VIII level ptt also prolonged decreased platelet count. B. Bleeding time and factor IX level C. Bleeding time and factor X level D. Platelet count E. Thromboplastin generation time

Description : The patient whom you are about to treat states that he has Von Willebrand's disease. Which one of the following preoperative haematological analyses may reflect this disease: A. Bleeding time and ... C. Bleeding time and factor X level D. Platelet count E. Thromboplastin generation time

Last Answer : A. Bleeding time and factor VIII level ptt also prolonged decreased platelet count

Choose the correct statement(s) about terlipressin: A. It is a prodrug B. It is used to control bleeding from esophageal varices C. It is preferred for controlling bleeding in von Willebrand's disease D. Both 'A' and 'B' are correct

Description : Choose the correct statement(s) about terlipressin: A. It is a prodrug B. It is used to control bleeding from esophageal varices C. It is preferred for controlling bleeding in von Willebrand's disease D. Both 'A' and 'B' are correct

Last Answer : D. Both 'A' and 'B' are correct

Characteristic features of active site are (A) Flexible in nature (B) Site of binding (C) Acidic (D) Both (A) and (B)

Description : Characteristic features of active site are (A) Flexible in nature (B) Site of binding (C) Acidic (D) Both (A) and (B)

Last Answer : Answer : D

What are the characteristic features of type 1, diabetes mellitus?

Description : What are the characteristic features of type 1, diabetes mellitus?

Last Answer : Here circulating insulin level is deficient. These patients are dependent on insulin injections. Onset is during adolescence. Rapid loss of body weight is observed. They are more prone to developing ketosis.

What are the characteristic features of iso-elec- tric point?

Description : What are the characteristic features of iso-elec- tric point?

Last Answer : At the pI value, the proteins will not migrate in an electrical field; solubility, buffering capacity and viscosity will be minimum and precipitation will be maximum.

What are the characteristic features of iso-elec- tric pH.

Description : What are the characteristic features of iso-elec- tric pH.

Last Answer : At iso-electric point the amino acid will carry no net charge, there is no mobility in electrical field, solubility will be minimum, the tendency for pre- cipitation will be maximum

What is the best clinical course of therapy in HIV bone disease?

Description : What is the best clinical course of therapy in HIV bone disease?

Last Answer : Feel Free to Answer

Infectious disease transmission during blood transfusions is of clinical significance to surgeons and of major importance to patients contemplating surgery potentially associated with the need for blood administration. Which of the following statement(s) is/are true concerning the transmission of infectious disease during blood transfusions? a. Post-transfusion hepatitis is usually due to hepatitis B b. Hepatitis and HIV transmission is greatest with the administration of pooled plasma products such as serum albumin c. The most important cause of post-transfusion disease in immunosuppressed patients is CMV infection d. The risk of HIV transmission in blood transfusions is significantly less than the risk of hepatitis transmission

Description : Infectious disease transmission during blood transfusions is of clinical significance to surgeons and of major importance to patients contemplating surgery potentially associated with the need for ... transmission in blood transfusions is significantly less than the risk of hepatitis transmission

Last Answer : Answer: c, d The most common infectious diseases transmitted during blood transfusions include viral hepatitis, CMV, and HIV infection. Post-transfusion hepatitis in 90% of cases consists of ... infectious disease transmission, the risks of HIV transmission is markedly less than that of hepatitis

Rank the clinical scenarios in order of greatest likelihood of serious postoperative pulmonary complications. A. Transabdominal hysterectomy in an obese woman that requires 3 hours of anesthesia time. B. Right middle lobectomy for bronchogenic cancer in a 65-year-old smoker. C. Vagotomy and pyloroplasty for chronic duodenal ulcer disease in a 50-year-old who had chest film findings of old, healed tuberculosis. D. Right hemicolectomy in an obese 60-year-old smoker. E. Modified radical mastectomy in a 58-year-old woman who is obese.

Description : Rank the clinical scenarios in order of greatest likelihood of serious postoperative pulmonary complications. A. Transabdominal hysterectomy in an obese woman that requires 3 hours of anesthesia time. B. Right ... smoker. E. Modified radical mastectomy in a 58-year-old woman who is obese.

Last Answer : Answer: BDCAE DISCUSSION: If one considers the constellation of risk factors for pulmonary complications that is provided in tabular form in the accompanying chapter, one should ... is associated with relatively little postoperative pain, and provides free and unrestricted respiratory function

Which of the following statements concerning monitoring techniques in the intensive care unit are true? A. Pulmonary artery and pulmonary capillary wedge pressure readings should be made at end inspiration, to minimize ventilatory artifacts. B. Continuous SvO 2 monitoring based on the technique of reflectance spectrophotometry has been shown to be accurate and reliable. C. Direct measurement of gastric intramucosal pH can be provided by gastrointestinal tonometry. D. Hyperlactatemia may be seen in a number of clinical conditions not associated with tissue hypoxia, including liver disease and hypermetabolic states.

Description : Which of the following statements concerning monitoring techniques in the intensive care unit are true? A. Pulmonary artery and pulmonary capillary wedge pressure readings should be made ... clinical conditions not associated with tissue hypoxia, including liver disease and hypermetabolic states.

Last Answer : Answer: BD DISCUSSION: Many different monitoring techniques may be used to assess the adequacy of therapy for shock. The pulmonary artery catheter can provide important hemodynamic and oxygen ... also be seen with liver disease, toxin ingestion, and hypermetabolic states not associated with shock

Clinical Mareks disease can appear as early as B A. 1 week old birds B. 8 weeks old birds C. not before 30 weeks of age D. None of these

Description : Clinical Mareks disease can appear as early as B A. 1 week old birds B. 8 weeks old birds C. not before 30 weeks of age D. None of these

Last Answer : 8 weeks old birds

Clinical disease of Avian encephalomyelitis occurs in A A. chicks up to one week of age B. chicks of 4 weeks of age C. in old birds D. None of these

Description : Clinical disease of Avian encephalomyelitis occurs in A A. chicks up to one week of age B. chicks of 4 weeks of age C. in old birds D. None of these

Last Answer : chicks up to one week of age

Emphysema is described as: a) A disease of the airways characterized by destruction of the walls of overdistended alveoli. Emphysema is a category of COPD. b) A disease that results in a common clinical outcome of reversible airflow obstruction. Asthma is the disease described. c) The presence of cough and sputum production for at least a combined total of two or three months in each of two consecutive years. Bronchitis is the disease described. d) Chronic dilatation of a bronchus or bronchi Bronchiectasis is the condition described.

Description : Emphysema is described as: a) A disease of the airways characterized by destruction of the walls of overdistended alveoli. Emphysema is a category of COPD. b) A disease that results in ... disease described. d) Chronic dilatation of a bronchus or bronchi Bronchiectasis is the condition described.

Last Answer : a) A disease of the airways characterized by destruction of the walls of overdistended alveoli. Emphysema is a category of COPD.

Sjogren’s syndrome is a chronic disease characterized by which of the following clinical feature: Options: 1) Esophageal dysmotility 2) Keratoconjunctivitis sicca 3) Widespread damage to small blood vessels 4) Libmann Sachs Endocardititis

Description : Sjogren’s syndrome is a chronic disease characterized by which of the following clinical feature: Options: 1) Esophageal dysmotility 2) Keratoconjunctivitis sicca 3) Widespread damage to small blood vessels 4) Libmann Sachs Endocardititis

Last Answer : Correct Answer: 2) Keratoconjunctivitis sicca

What is the MOST significant clinical feature of periodontal disease:** A. Bleeding B. True pocket formation and apical migration of attached gingiva

Description : What is the MOST significant clinical feature of periodontal disease:** A. Bleeding B. True pocket formation and apical migration of attached gingiva

Last Answer : B. True pocket formation and apical migration of attached gingiva

A patient who recently had a calculus removed from the kidney presented with radiolucent area in the left maxilla with clinical evidence of swelling. The disease that you would immediately suggest is,** M07 A. Diabetes B. Thyrotoxicosis C. Hyperparathyroidism D. Osteoporosis E. Adrenal insufficiency

Description : A patient who recently had a calculus removed from the kidney presented with radiolucent area in the left maxilla with clinical evidence of swelling. The disease that you would immediately suggest ... A. Diabetes B. Thyrotoxicosis C. Hyperparathyroidism D. Osteoporosis E. Adrenal insufficiency

Last Answer : C. Hyperparathyroidism

Study of individual's immune response to a certain disease is called as __________. a. Clinical pathology b. Surgical pathology c. Neuropathology d. Immunopathology

Description : Study of individual's immune response to a certain disease is called as __________. a. Clinical pathology b. Surgical pathology c. Neuropathology d. Immunopathology

Last Answer : d. Immunopathology

Choose the correct statement about nicotine: A. It selectively stimulates parasympathetic ganglia B. It has no clinical application C. It is used as an aid during smoking cessation D. It is used in Alzheimer's disease

Description : Choose the correct statement about nicotine: A. It selectively stimulates parasympathetic ganglia B. It has no clinical application C. It is used as an aid during smoking cessation D. It is used in Alzheimer's disease

Last Answer : C. It is used as an aid during smoking cessation

Clinical jaundice is present with an icteric index above (A) 4 (B) 8 (C) 10 (D) 15

Description : Clinical jaundice is present with an icteric index above (A) 4 (B) 8 (C) 10 (D) 15

Last Answer : Answer : D

Clinical symptom in urea cycle disorder is (A) Mental retardation (B) Drowsiness (C) Diarrhoea (D) Oedema

Description : Clinical symptom in urea cycle disorder is (A) Mental retardation (B) Drowsiness (C) Diarrhoea (D) Oedema

Last Answer : Answer : A

What is the clinical application of fructose estimation in semen?

Description : What is the clinical application of fructose estimation in semen?

Last Answer : Fructose is secreted by seminal vesicles. A block in seminal vessels is indicated by the absence of fructose in semen.

What is the clinical significance of polyol pathway?

Description : What is the clinical significance of polyol pathway?

Last Answer : The elevated level of sorbitol has been implicated in the development of neuropathy, cataract and retinopathy in diabetes mellitus.

Abnormal transketolase leads to what clinical condition?

Description : Abnormal transketolase leads to what clinical condition?

Last Answer : The occurrence of Wernickís encephalopathy (seen in alcoholics and in thiamine deficiency) is due to a genetic defect in the enzyme transketolase. It occurs owing to the enzyme having low binding capacity for TPP.

What is the clinical significance of transketolase enzyme?

Description : What is the clinical significance of transketolase enzyme?

Last Answer : The transketolase reaction is measured in RBCs as an index of the thiamine status of an individual.

What is the clinical significance of C peptide?

Description : What is the clinical significance of C peptide?

Last Answer : Sometimes measurement of endogenous insulin may be difficult because of the presence of antibodies against insulin in the circulation. Then measurement of C-peptide is useful.

What is the clinical importance of lactosuria?

Description : What is the clinical importance of lactosuria?

Last Answer : The condition is harmless. But it is important to distinguish lactosuria from glucosuria when gestational diabetes mellitus is suspected.

What is the clinical significance of GDM?

Description : What is the clinical significance of GDM?

Last Answer : Women with GDM are at increased risk for subsequent development of frank diabetes. GDM is associated with increased birth weight of child and increased incidence of neonatal mortality.

Give the clinical implications of these enzymes.

Description : Give the clinical implications of these enzymes. 

Last Answer : In infective hepatitis, ALT level is increased; in alcohol abuse, GGT level is increased; in obstructive jaundice, ALP level is increased.

What is their clinical significance?

Description : What is their clinical significance?

Last Answer : Their normal levels in blood are very low, but are drastically increased during cell death (necrosis) or disease. Therefore, assays of these enzymes are very useful in diagnosis of diseases.

Give examples of clinical application of competitive inhibition.

Description : Give examples of clinical application of competitive inhibition. 

Last Answer : Sulfonamide inhibits PABA incorporation in bacteria, and so acts as an antibacterial agent. Methotrexate inhibits folate reductase system, dicoumarol inhibits vitamin K.

What is the clinical significance of transami- nases?

Description : What is the clinical significance of transami- nases?

Last Answer : Transaminases in blood are elevated in liver and heart diseases.

What is its clinical significance?

Description : What is its clinical significance?

Last Answer : Digoxin increases the contractility of the cardiac muscle, by inhibiting the sodium pump