BACKGROUND Holoprosencephaly(HPE)is a congenital malformation with various degrees of incomplete separation of the cerebral hemispheres due to differentiation disorders of the forebrain.Although HPE with diabetes insi...BACKGROUND Holoprosencephaly(HPE)is a congenital malformation with various degrees of incomplete separation of the cerebral hemispheres due to differentiation disorders of the forebrain.Although HPE with diabetes insipidus due to associated pituitary dysfunction has been reported,HPE with the syndrome of inappropriate antidiuretic hormone secretion(SIADH)is very rare.Tolvaptan,a vasopressin V2 receptor antagonist,is effective in adults with SIADH.However,there is no report of its efficacy in infants with SIADH.The purpose of this report is to demonstrate that tolvaptan is effective for SIADH in infants and that administration of tolvaptan eliminates the need for restriction of water intake and sodium administration.CASE SUMMARY A 2414-g female infant was born at 38 wk by normal vaginal delivery.Facial anomalies and head magnetic resonance imaging indicated semilobar HPE.After birth,she had hyponatremia due to SIADH and was treated using water and sodium restriction.However,she developed an exaggerated response to the fluid restrictions,resulting in large fluctuations in serum sodium levels.Subsequent administration of tolvaptan improved the fluctuations in serum sodium levels without the need for adjustment of water or sodium administration.Serum sodium was maintained within the normal range after discontinuation of tolvaptan at 80 d of life.There were no side effects,such as hypernatremia or liver dysfunction,during the administration of tolvaptan.CONCLUSION This is the first report on the safety and efficacy of tolvaptan in an infant with SIADH associated with HPE.展开更多
BACKGROUND Syndrome of inappropriate secretion of antidiuretic hormone(SIADH)is relatively common in several cancers,such as small cell lung cancer.However,nedaplatin-induced SIADH is rare.We describe a case of SIADH ...BACKGROUND Syndrome of inappropriate secretion of antidiuretic hormone(SIADH)is relatively common in several cancers,such as small cell lung cancer.However,nedaplatin-induced SIADH is rare.We describe a case of SIADH mediated by nedaplatin.CASE SUMMARY A 54-year-old female with nasopharyngeal carcinoma was treated with nedaplatin and developed severe hyponatremia due to SIADH.The side effects were successfully treated by fluid restriction and sodium supplementation.CONCLUSION This case report highlights the importance of cautiously treating life-threatening hyponatremia in patients treated with nedaplatin.展开更多
Antidiuretic hormone (ADH) is produced by an area of he brain called the hypothalamus. The hormone is stored in and released by the pituitary gland. When ADH (also called vasopressin) is produced somewhere other ...Antidiuretic hormone (ADH) is produced by an area of he brain called the hypothalamus. The hormone is stored in and released by the pituitary gland. When ADH (also called vasopressin) is produced somewhere other than the hypothalamus, the condition is called syndrome of inappropriate antidiuretic hormone (SIADH). A variety of conditions can trigger abnormal ADH production, but the main cause is cancer. It is frequently one of the first signs of lung cancer, especially small cell carcinoma, which produces ADH ectopically.l展开更多
Hyponatremia is a common disorder among cancer patients and is associated with a poor prognosis in several malignancies.It is classified by volemic status into hypovolemic,euvolemic,and hypervolemic hyponatremia.Clini...Hyponatremia is a common disorder among cancer patients and is associated with a poor prognosis in several malignancies.It is classified by volemic status into hypovolemic,euvolemic,and hypervolemic hyponatremia.Clinical history,physical examination,and blood and urine tests are important for a correct classification and diagnosis of hyponatremia,to assure correct management.Treatment of hyponatremia in cancer patients depends on the etiology of hyponatremia,as well as on the chosen therapy for the tumor.Supportive care is also a factor to be taken into account.展开更多
INTRODUCTION Acute intermittent porphyria (AIP) is an autosomal dominant inborn error of metabolism caused by deficiency of porphobilinogen (PBG) deaminase, also known as hydroxymethylbilane synthase (HMBS), the...INTRODUCTION Acute intermittent porphyria (AIP) is an autosomal dominant inborn error of metabolism caused by deficiency of porphobilinogen (PBG) deaminase, also known as hydroxymethylbilane synthase (HMBS), the third enzyme in the heine biosynthetic pathway.展开更多
文摘BACKGROUND Holoprosencephaly(HPE)is a congenital malformation with various degrees of incomplete separation of the cerebral hemispheres due to differentiation disorders of the forebrain.Although HPE with diabetes insipidus due to associated pituitary dysfunction has been reported,HPE with the syndrome of inappropriate antidiuretic hormone secretion(SIADH)is very rare.Tolvaptan,a vasopressin V2 receptor antagonist,is effective in adults with SIADH.However,there is no report of its efficacy in infants with SIADH.The purpose of this report is to demonstrate that tolvaptan is effective for SIADH in infants and that administration of tolvaptan eliminates the need for restriction of water intake and sodium administration.CASE SUMMARY A 2414-g female infant was born at 38 wk by normal vaginal delivery.Facial anomalies and head magnetic resonance imaging indicated semilobar HPE.After birth,she had hyponatremia due to SIADH and was treated using water and sodium restriction.However,she developed an exaggerated response to the fluid restrictions,resulting in large fluctuations in serum sodium levels.Subsequent administration of tolvaptan improved the fluctuations in serum sodium levels without the need for adjustment of water or sodium administration.Serum sodium was maintained within the normal range after discontinuation of tolvaptan at 80 d of life.There were no side effects,such as hypernatremia or liver dysfunction,during the administration of tolvaptan.CONCLUSION This is the first report on the safety and efficacy of tolvaptan in an infant with SIADH associated with HPE.
文摘BACKGROUND Syndrome of inappropriate secretion of antidiuretic hormone(SIADH)is relatively common in several cancers,such as small cell lung cancer.However,nedaplatin-induced SIADH is rare.We describe a case of SIADH mediated by nedaplatin.CASE SUMMARY A 54-year-old female with nasopharyngeal carcinoma was treated with nedaplatin and developed severe hyponatremia due to SIADH.The side effects were successfully treated by fluid restriction and sodium supplementation.CONCLUSION This case report highlights the importance of cautiously treating life-threatening hyponatremia in patients treated with nedaplatin.
文摘Antidiuretic hormone (ADH) is produced by an area of he brain called the hypothalamus. The hormone is stored in and released by the pituitary gland. When ADH (also called vasopressin) is produced somewhere other than the hypothalamus, the condition is called syndrome of inappropriate antidiuretic hormone (SIADH). A variety of conditions can trigger abnormal ADH production, but the main cause is cancer. It is frequently one of the first signs of lung cancer, especially small cell carcinoma, which produces ADH ectopically.l
文摘Hyponatremia is a common disorder among cancer patients and is associated with a poor prognosis in several malignancies.It is classified by volemic status into hypovolemic,euvolemic,and hypervolemic hyponatremia.Clinical history,physical examination,and blood and urine tests are important for a correct classification and diagnosis of hyponatremia,to assure correct management.Treatment of hyponatremia in cancer patients depends on the etiology of hyponatremia,as well as on the chosen therapy for the tumor.Supportive care is also a factor to be taken into account.
文摘INTRODUCTION Acute intermittent porphyria (AIP) is an autosomal dominant inborn error of metabolism caused by deficiency of porphobilinogen (PBG) deaminase, also known as hydroxymethylbilane synthase (HMBS), the third enzyme in the heine biosynthetic pathway.