We report a case of a 42-year-old man with a rare disorder known as primary intestinal lymphangiectasia,which is characterized by dilated intestinal lymphatics that lead to the development of protein-losing enteropath...We report a case of a 42-year-old man with a rare disorder known as primary intestinal lymphangiectasia,which is characterized by dilated intestinal lymphatics that lead to the development of protein-losing enteropathy. The patient presented with a grand mal seizure caused by malabsorption-derived electrolytes and a protein disorder. Signs of the disease, including chronic diarrhea and peripheral edema, manifested10 years ago, but a diagnosis was never made. The diagnosis was suspected because of the clinical manifestations, laboratory tests, imaging and endoscopic findings. Hyperemic and edematous mucosa of the small intestine corresponded to scattered white spots with dilated intestinal lymphatics and whitish villi in the histological specimen of the biopsied jejunal mucosa.Although numerous therapeutic strategies are available,only octreotide therapy proved to be an effective means of therapeutic resolution in this patient. Although the patient had a partial remission following the use of a slow release formula of octreotide, his prognosis, clinical course, and future treatment challenges are yet to be determined.展开更多
A 24-year-old female patient with parathyroid carcinoma,the rarest endocrine malignancy,had two pregnancies.In the first pregnancy,she had severe nausea and fatigue.Hypercalcemia and hyperparathyroidism were diagnosed...A 24-year-old female patient with parathyroid carcinoma,the rarest endocrine malignancy,had two pregnancies.In the first pregnancy,she had severe nausea and fatigue.Hypercalcemia and hyperparathyroidism were diagnosed in the postpartum period.Hyperemesis gravidarum masked a diagnosis of hypercalcemia.Neck ultrasound and Tc-99 m sestamibi found an enlarged lower right parathyroid gland.The gland was surgically removed,and an initial pathology report described atypical adenoma.Shortly afterward,she became pregnant again.During the second pregnancy,her calcium level was frequently controlled but was always in the normal range.Normocalcemia is explained by the specific physiology of pregnancy accompanied by hemodilution,hypoalbuminemia and maternal hypercalciuria(mediated by increased glomerular filtration).During lactation,calcium levels rose,and a newneck ultrasound showed a solitary mass in the area of prior surgery and an enlarged pretracheal lymph node.Fine needle aspiration of the solitary mass and node showed parathyroid carcinoma cells.The tumor mass was resected en bloc with the contiguous tissues and surrounding lymph nodes(pathology report; parathyroid carcinoma with metastases).Over the next five years,four consecutive surgeries were performed to remove malignant parathyroid tissue,lymph nodes and local metastases.Following the surgical procedures,no hypocalcemia was observed.More serious hypercalcemia recurred; the calcium level was difficult to control with a combination of pamidronate,cinacalcet and loop diuretic.No elements of multiple endocrine neoplasia were present.展开更多
文摘We report a case of a 42-year-old man with a rare disorder known as primary intestinal lymphangiectasia,which is characterized by dilated intestinal lymphatics that lead to the development of protein-losing enteropathy. The patient presented with a grand mal seizure caused by malabsorption-derived electrolytes and a protein disorder. Signs of the disease, including chronic diarrhea and peripheral edema, manifested10 years ago, but a diagnosis was never made. The diagnosis was suspected because of the clinical manifestations, laboratory tests, imaging and endoscopic findings. Hyperemic and edematous mucosa of the small intestine corresponded to scattered white spots with dilated intestinal lymphatics and whitish villi in the histological specimen of the biopsied jejunal mucosa.Although numerous therapeutic strategies are available,only octreotide therapy proved to be an effective means of therapeutic resolution in this patient. Although the patient had a partial remission following the use of a slow release formula of octreotide, his prognosis, clinical course, and future treatment challenges are yet to be determined.
文摘A 24-year-old female patient with parathyroid carcinoma,the rarest endocrine malignancy,had two pregnancies.In the first pregnancy,she had severe nausea and fatigue.Hypercalcemia and hyperparathyroidism were diagnosed in the postpartum period.Hyperemesis gravidarum masked a diagnosis of hypercalcemia.Neck ultrasound and Tc-99 m sestamibi found an enlarged lower right parathyroid gland.The gland was surgically removed,and an initial pathology report described atypical adenoma.Shortly afterward,she became pregnant again.During the second pregnancy,her calcium level was frequently controlled but was always in the normal range.Normocalcemia is explained by the specific physiology of pregnancy accompanied by hemodilution,hypoalbuminemia and maternal hypercalciuria(mediated by increased glomerular filtration).During lactation,calcium levels rose,and a newneck ultrasound showed a solitary mass in the area of prior surgery and an enlarged pretracheal lymph node.Fine needle aspiration of the solitary mass and node showed parathyroid carcinoma cells.The tumor mass was resected en bloc with the contiguous tissues and surrounding lymph nodes(pathology report; parathyroid carcinoma with metastases).Over the next five years,four consecutive surgeries were performed to remove malignant parathyroid tissue,lymph nodes and local metastases.Following the surgical procedures,no hypocalcemia was observed.More serious hypercalcemia recurred; the calcium level was difficult to control with a combination of pamidronate,cinacalcet and loop diuretic.No elements of multiple endocrine neoplasia were present.