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
The objective of the study was to investigate the effects of dietary energy levels and sources on the blood metabolites,hormone secretion and the composition of follicular fluid in gilts.Fifty-four gilts with initial ...The objective of the study was to investigate the effects of dietary energy levels and sources on the blood metabolites,hormone secretion and the composition of follicular fluid in gilts.Fifty-four gilts with initial body weight of(59±4.2) kg were randomly allotted to six treatments.Treatments were low, normal,and high energy feeding levels,which were 87.5%,100%and 112.5%of recommendatory energy requirements by NRC(1998),respectively,and dietary energy sources(starch or fat).Blood samples and follicular fluids were collected on D18 and D19 of the second estrous cycle.The results showed that plasma concentrations of triglycerides and total cholesterol were higher in the fat group than that in the starch group(P【0.05),but those of glucose were similar between the two energy sources(P】0.05);dietary energy level exerted no effect on blood metabolites concentration(P】0.05).Gilts fed the high energy diet had a higher area under curve of plasma insulin(Insulin AUC),insulin-like growth factor-Ⅰ(IGF-Ⅰ) and leptin than did gilts fed the lower energy diet(P【0.05),but there was no significant difference between fat versus starch(P】0.05).Luteinizing hormone(LH) pulses were higher in gilts fed high energy rather than that in low energy diets(P】0.05),plasma concentration of estradiol(E<sub>2</sub>) was higher in the fat group than that in the starch group(P【0.05).The number of large follicles(diameter≥4 mm) and concentrations of IGF-Ⅰand E<sub>2</sub> in follicular fluid were increasing significant as the level of energy increased(P【0.05),but the numbers of large follicles and follicular fluid composition were not affected by the source of dietary energy(P】0.05).The results indicate that gilts fed high energy diets had elevated plasma concentrations of metabolic hormones,IGF-Ⅰand LH secretion,and increased follicular fluid concentrations of IGF-Ⅰ,E<sub>2</sub> and numbers of large follicles;gilts fed the dietary fat had a higher plasma concentration of cholesterol and E<sub>2</sub>.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
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
Although ectopic hormone-production is uncommon complication, certain tumors can produce symptoms due to the secretion of various bioactive substances accompanied by the aberrantly located tumors. Because of the poten...Although ectopic hormone-production is uncommon complication, certain tumors can produce symptoms due to the secretion of various bioactive substances accompanied by the aberrantly located tumors. Because of the potential for the ovary to act as a source of aberrant hormone secretion, in the literature, ectopic hormone production from ovarian tumor includes granulocyte-colony stimulating factor (G-CSF), parathyroid hormone-related protein (PTHrP), adrenocorticotropic hormone (ACTH), peptide-YY, gastrin and insulin. All patients may present with syndromes of hormone excess. Failure to localize the ovarian tumor preoperatively may be associated with a significantly higher risk of subsequent unnecessary ablative procedures. Better characterization of hormonal forms relatively specific for neoplasia may enhance the clinical value of ectopic hormones as tumor markers, especially in malignancies that are commonly associated with ectopic hormone production. These circumstances may recommend complete preoperative evaluation of the pelvis in female patients presenting with nonlocalizable endocrine tumors.展开更多
To investigate the difference of biochemical characteristics on gsp positive and gsp negative growth hormone (GH) secreting pituitary tumors, 18 GH secreting pituitary tumors were examined for their clinical char...To investigate the difference of biochemical characteristics on gsp positive and gsp negative growth hormone (GH) secreting pituitary tumors, 18 GH secreting pituitary tumors were examined for their clinical characteristics and gsp oncogenes. All patients received the pituitary function combinative stimulating test. It was found that there were no difference in the sex, age, tumor size, course of disease and plasma basal GH levels with gsp positive and gsp negative patients. The plasma levels of PRL were increased in most patients (11/18), and the plasma levels of TSH in gsp positive patients were higher than those in gsp negative patients ( P <0.05). There was no significant difference in the responses to pituitary combinative stimulating test in gsp positive and gsp negative patients. It was concluded that there was little difference in the clinical biochemical characteristics of gsp positive with gsp negative GH secreting pituitary tumors.展开更多
Background： Two recent whole-exome sequencing researches identifying somatic mutations in the ubiquitin-specific protease 8 （USP8） gene in pituitary corticotroph adenomas provide exciting advances in this field. Th...Background： Two recent whole-exome sequencing researches identifying somatic mutations in the ubiquitin-specific protease 8 （USP8） gene in pituitary corticotroph adenomas provide exciting advances in this field. These mutations drive increased epidermal growth factor receptor （EGFR） signaling and promote adrenocorticotropic hormone （ACTH） production. This study was to investigate whether the inhibition of USP8 activity could be a strategy/br the treatment of Cushing＇s disease （CD）. Methods： The anticancer effect of USP8 inhibitor was determined by testing cell viability, colony tbrmation, apoptosis, and ACTH secretion. The immunoblotting and quantitative reverse transcription polymerase chain reaction were conducted to explore the signaling pathway by USP8 inhibition. Results： Inhibition of USP8-induced degradation of receptor tyrosine kinases including EGFR, EGFR-2 （ERBB2）, and Met leading to a suppression of ArT20 cell growth and ACTH secretion. Moreover, treatment with USP8 inhibitor markedly induced AtT20 cells apoptosis. Conclusions： Inhibition of USP8 activity could be an effective strategy for CD. It might provide a novel pharmacological approach for the treatment of CD.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
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
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.
基金supported by Program for Changjiang Scholars and Innovative Research Team in University (IRT0555)National Natural Science Foundation (30471257) of China
文摘The objective of the study was to investigate the effects of dietary energy levels and sources on the blood metabolites,hormone secretion and the composition of follicular fluid in gilts.Fifty-four gilts with initial body weight of(59±4.2) kg were randomly allotted to six treatments.Treatments were low, normal,and high energy feeding levels,which were 87.5%,100%and 112.5%of recommendatory energy requirements by NRC(1998),respectively,and dietary energy sources(starch or fat).Blood samples and follicular fluids were collected on D18 and D19 of the second estrous cycle.The results showed that plasma concentrations of triglycerides and total cholesterol were higher in the fat group than that in the starch group(P【0.05),but those of glucose were similar between the two energy sources(P】0.05);dietary energy level exerted no effect on blood metabolites concentration(P】0.05).Gilts fed the high energy diet had a higher area under curve of plasma insulin(Insulin AUC),insulin-like growth factor-Ⅰ(IGF-Ⅰ) and leptin than did gilts fed the lower energy diet(P【0.05),but there was no significant difference between fat versus starch(P】0.05).Luteinizing hormone(LH) pulses were higher in gilts fed high energy rather than that in low energy diets(P】0.05),plasma concentration of estradiol(E<sub>2</sub>) was higher in the fat group than that in the starch group(P【0.05).The number of large follicles(diameter≥4 mm) and concentrations of IGF-Ⅰand E<sub>2</sub> in follicular fluid were increasing significant as the level of energy increased(P【0.05),but the numbers of large follicles and follicular fluid composition were not affected by the source of dietary energy(P】0.05).The results indicate that gilts fed high energy diets had elevated plasma concentrations of metabolic hormones,IGF-Ⅰand LH secretion,and increased follicular fluid concentrations of IGF-Ⅰ,E<sub>2</sub> and numbers of large follicles;gilts fed the dietary fat had a higher plasma concentration of cholesterol and E<sub>2</sub>.
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Although ectopic hormone-production is uncommon complication, certain tumors can produce symptoms due to the secretion of various bioactive substances accompanied by the aberrantly located tumors. Because of the potential for the ovary to act as a source of aberrant hormone secretion, in the literature, ectopic hormone production from ovarian tumor includes granulocyte-colony stimulating factor (G-CSF), parathyroid hormone-related protein (PTHrP), adrenocorticotropic hormone (ACTH), peptide-YY, gastrin and insulin. All patients may present with syndromes of hormone excess. Failure to localize the ovarian tumor preoperatively may be associated with a significantly higher risk of subsequent unnecessary ablative procedures. Better characterization of hormonal forms relatively specific for neoplasia may enhance the clinical value of ectopic hormones as tumor markers, especially in malignancies that are commonly associated with ectopic hormone production. These circumstances may recommend complete preoperative evaluation of the pelvis in female patients presenting with nonlocalizable endocrine tumors.
基金This project was supported by a grant from the National Natural Sciences Foundation of China(No.396 70 736 )
文摘To investigate the difference of biochemical characteristics on gsp positive and gsp negative growth hormone (GH) secreting pituitary tumors, 18 GH secreting pituitary tumors were examined for their clinical characteristics and gsp oncogenes. All patients received the pituitary function combinative stimulating test. It was found that there were no difference in the sex, age, tumor size, course of disease and plasma basal GH levels with gsp positive and gsp negative patients. The plasma levels of PRL were increased in most patients (11/18), and the plasma levels of TSH in gsp positive patients were higher than those in gsp negative patients ( P <0.05). There was no significant difference in the responses to pituitary combinative stimulating test in gsp positive and gsp negative patients. It was concluded that there was little difference in the clinical biochemical characteristics of gsp positive with gsp negative GH secreting pituitary tumors.
基金grants to Qing-Fang Sun from National Natural Science Foundation of China，National High-tech R&D Program (863 program)
文摘Background： Two recent whole-exome sequencing researches identifying somatic mutations in the ubiquitin-specific protease 8 （USP8） gene in pituitary corticotroph adenomas provide exciting advances in this field. These mutations drive increased epidermal growth factor receptor （EGFR） signaling and promote adrenocorticotropic hormone （ACTH） production. This study was to investigate whether the inhibition of USP8 activity could be a strategy/br the treatment of Cushing＇s disease （CD）. Methods： The anticancer effect of USP8 inhibitor was determined by testing cell viability, colony tbrmation, apoptosis, and ACTH secretion. The immunoblotting and quantitative reverse transcription polymerase chain reaction were conducted to explore the signaling pathway by USP8 inhibition. Results： Inhibition of USP8-induced degradation of receptor tyrosine kinases including EGFR, EGFR-2 （ERBB2）, and Met leading to a suppression of ArT20 cell growth and ACTH secretion. Moreover, treatment with USP8 inhibitor markedly induced AtT20 cells apoptosis. Conclusions： Inhibition of USP8 activity could be an effective strategy for CD. It might provide a novel pharmacological approach for the treatment of CD.
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.
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.
