Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0221002 (primary hyperparathyroidism)
 4,921 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

About 25% of patients with ZES have MEN 1. Except diarrhoea, less frequent in patients with ZES-MEN 1 than in sporadic ZES, and specific MEN 1-related signs, clinical characteristics are similar in both ZES types. Acid output and gastrin levels are also similar whether in the basal state or after secretin stimulation. Primary hyperparathyroidism (PHPT) exists in the majority of ZES-MEN 1 patients, 30% have pituitary adenoma (prolactinomas for half), 30% adrenal involvement, 25 to 30% have EC-Lomas; bronchial and thymic carcinoids have probably been underevaluated. Gastrinomas are multiple predominantly located in the duodenal wall, but also in the pancreas in association with clinically silent endocrine tumors. The spread of the disease: metastases to the liver (LM), mediastinum, bones, is evaluated at best by Octreoscan. Endoscopic ultrasonography evaluates the number, size and anatomical characteristics of gastrinomas. Patients without LM have an excellent prognosis. Surgery never cures ZES, but is necessary in case of associated life-threatening condition such as insulinoma and has been advocated to prevent LM development in patients with large pancreatic tumor(s). However although, indeed, the size of the tumor, when located in the pancreas > 3 cm, favours metachronous LM occurrence, surgery, in our experience, has not been able to prevent LM development. Hepatic malignancies remain however the most pejorative prognostic determinant for survival and raise the most difficult therapeutic challenge. Surgery is the best option whenever feasible; specific chemotherapy and chemo-embolisation have not conclusively achieved definite successes. Long-term octreotide treatment, however, has been shown recently to obtain tumour stabilisation. Internal irradiation with 90 Ytrium-labelled octreotide is a new promising option, presently under evaluation (Novartis European trial). Preliminary results are promising.
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PMID:[Diagnostic and therapeutic strategies in Zollinger-Ellison syndrome associated with multiple endocrine neoplasia type I (MEN-I): experience of the Zollinger-Ellison Syndrome Research Group: Bichat 1958-1999]. 1514 2

We report a 13 year-old mate with a history of multiple fractures and kidney stones. The laboratory showed a hypercalcemia of 11.5 mg/dl, a PTH of 112.6 pg/ml and 24 hour urinary calcium of 571 mg. Bone densitometry showed spine and femur Z scores of -2.9 and -1.6, respectively, kidney ultrasound showed nephrocalcinosis and a MIBI-SPECT scintigram showed a higher uptake in the right lower parathyroid gland. The diagnosis of primary hyperparathyroidism was made and the patient was operated, excising the right lower parathyroid gland. After surgery, serum calcium and PTH levels returned to normal values. In children, the proportion of cases with parathyroid hyperplasia is higher than in adults. Therefore, during surgery all four parathyroid glands must be explored. There is also a higher frequency of ectopic adenomas. Family history must be explored to discard the presence of a multiple endocrine neoplasia (MEN I or II), a familial hyperparathyroidism or a syndrome of primary hyperparathyroidism associated to mandibular tumor.
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PMID:[Primary hyperparathyroidism in an adolescent. Report of one case]. 1919 27

In summary, ZES is a syndrome caused by gastrinoma, usually located within the gastrinoma triangle and associated with symptoms of peptic ulcer disease, GERD, and diarrhea. The diagnosis of ZES is made by measuring fasting levels of serum gastrin, BAO, and the secretin stimulation test. Because of the high association of ZES and MEN1, HPT must be excluded by obtaining a serum calcium and parathyroid hormone level. Treatment of ZES consists of medical control of symptoms with PPIs and evaluation for potentially curative surgical intervention. Noninvasive imaging studies including SRS, CT, and MRI should be performed initially to evaluate for metastases and identify resectable disease. Invasive imaging modalities such as EUS may be performed to further evaluate primary tumors. IOUS, palpation, and duodenotomy are used for intraoperative localization of gastrinomas. In patients with MEN1, surgical resection should be pursued only if there is an identifiable tumor larger than 2 cm and after surgery for the primary hyperparathyroidism (3 1/2-gland parathyroidectomy). All patients with resectable localized sporadic gastrinoma should undergo surgical exploration, even those with biochemical evidence but negative imaging studies. Tumor is most commonly found in the duodenum, and the cure rate is high. In patients with liver metastases, surgery should be considered if all identifiable tumor can be safely removed. A multidisciplinary approach including surgical and nonsurgical therapies should be taken in patients with advanced disease.
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PMID:Current management of the Zollinger-Ellison syndrome. 2429 44


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