3. A 15-year-old boy presented with a history of episodic vomiting associated with subacute encephalopathy from the age of one year. There were 2-3 months of asymptomatic periods betwcen the episodes. He was born of non- consanguincous parents at full term had a normal perinatal period with no evidence of birth asphyxia. During the hospitalstay he had 2 episodesofconvulsive seizures. Investigations revealed high blood ammonia (288 ug/dL, normal level is 40-80 ug/dL), markedly elevated levels of citrulline (2200 umoles/L. normal is 1–55 umoles/L), and low blood urea (5 mg/dL, normal is around 7 to 20 mg/dL). He was treated with anticonvulsives and sodium benzoate, advised a restricted protein diet. What is the reason for the presented symptoms? Why blood urea concentration is reduced? For the answer: a) specify the compound 90% of body nitrogen is excreted in; b) write down the process damaged in this patient: c) point out the enzyme deficient in this clinical case; d) explain the mcchanisms of ammonia toxieitty; c) explain the expedicney of preseribed diet and sodium benzoate.
3. A 15-year-old boy presented with a history of episodic vomiting associated with subacute encephalopathy from the age of one year. There were 2-3 months of asymptomatic periods betwcen the episodes. He was born of non- consanguincous parents at full term had a normal perinatal period with no evidence of birth asphyxia. During the hospitalstay he had 2 episodesofconvulsive seizures. Investigations revealed high blood ammonia (288 ug/dL, normal level is 40-80 ug/dL), markedly elevated levels of citrulline (2200 umoles/L. normal is 1–55 umoles/L), and low blood urea (5 mg/dL, normal is around 7 to 20 mg/dL). He was treated with anticonvulsives and sodium benzoate, advised a restricted protein diet. What is the reason for the presented symptoms? Why blood urea concentration is reduced? For the answer: a) specify the compound 90% of body nitrogen is excreted in; b) write down the process damaged in this patient: c) point out the enzyme deficient in this clinical case; d) explain the mcchanisms of ammonia toxieitty; c) explain the expedicney of preseribed diet and sodium benzoate.
Essentials of Pharmacology for Health Professions
7th Edition
ISBN:9781305441620
Author:WOODROW
Publisher:WOODROW
Chapter16: Gastrointestinal Drugs
Section: Chapter Questions
Problem 6CRQ
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