Primary hyperparathyroidism (PHPT) is the leading cause of hypercalcemia. It is secondary to hypersecretion of parathyroid hormone (PTH) by the parathyroid glands. Today, PHTP is asymptomatic in 80-90% of cases. Its repercussions are mainly renal (nephrolithiasis, nephrocalcinosis, decline in renal function) and skeletal (osteoporosis, fractures), and should be systematically investigated. Diagnosis is only biological, and in its classic form relies on the association of hypercalcemia, inappropriate PTH (normal or elevated) and hypercalciuria. Diagnosis of normocalcemic forms, where only PTH is elevated, requires elimination of secondary hyperparathyroidism and confirmation of elevated PTH on two consecutive samples, over a 3 to 6 months period. Imaging evaluation, which combines neck ultrasound with scintigraphy or 18F-choline PET/CT, is of interest only if surgery is indicated. Surgical management of the hyperfunctioning parathyroid gland(s) is the only curative treatment for HPTP. Medical management concerns patients for whom surgery is not indicated, who present a surgical contraindication or who refuse surgery. The diagnosis of HPTP warrants contact with an endocrinologist to ensure its management.

OBJECTIVE: There is no specific recommendation for management in pregnant women: the aim of this review, based on a clinical case study, is to clarify its development, complications, risk factor and treatment.
METHODS: A review of the literature was performed by consulting the Pubmed, Cochrane Library, and Science Direct databases.
RESULTS: Primary hyperparathyroidism is defined as excessive production of parathyroid hormone resulting in hypercalcemia. The prevalence of primary hyperparathyroidism during pregnancy is not known. Indeed, the symptomatology, related to hypercalcemia, is not very specific and easily confused with the clinical manifestations of pregnancy. The physiological changes specific to the pregnant state frequently lead to a slight hypocalcemia which may complicate the diagnosis of primary hyperparathyroidism. Primary hyperparathyroidism results from a parathyroid adenoma in the majority of cases and is detected by ultrasound during pregnancy. Primary hyperparathyroidism in pregnancy causes significant risks to both mother and fetus. The maternal complication rate is 14-67%, however, the most serious complication is hypercalcemic crisis, which requires increased surveillance in the postpartum period. Obstetrical complications are also induced by primary hyperparathyroidism, such as acute polyhydramnios, or intrauterine growth retardation. The fetal complication rate can reach 45-80% of cases with neonatal hypocalcemia as the main complication. If medical treatment is based on hyperhydration, only surgical treatment is curative.
CONCLUSIONS: Surgery should be proposed to symptomatic patients or those with high blood calcium levels, discussed in interdisciplinary committee and should be organized ideally in the second trimester to avoid maternal and fetal complications.

Hypercalcemia - Diagnosis and Management Abstract. The diagnostic workup of hypercalcemia requires a thorough patient history, a focused clinical examination as well as a step-by-step laboratory diagnostic approach. In order to detect the exact aetiology of hypercalcemia an accurate measurement of serum calcium in correlation with the parathyroid hormone level is therefore essential. Primary hyperparathyroidism and malignancy-related hypercalcemia are responsible for about 90% of all hypercalcemia cases. Therefore, these two pathologies should always be considered in the diagnostic approach. The therapeutic procedure is based on the aetiology and severity of the hypercalcemia.
Zusammenfassung. Die Abklärung einer Hyperkalzämie bedarf einer genauen Anamnese, einer fokussierten klinischen Untersuchung, ergänzt durch eine stufenweise durchgeführte, primär laborchemische Diagnostik, um zielgerichtet die ätiologische Zuordnung zu ermöglichen. Zentral ist hierbei die korrekte Bestimmung der Kalzämie und der Korrelation mit der Konzentration von Parathormon (PTH). Der primäre Hyperparathyreoidismus oder Malignom-bedingte Hyperkalzämien stellen mit rund 90% die mit Abstand häufigsten Ursachen dar und sollten daher stets berücksichtigt werden. Die Therapie der Hyperkalzämie richtet sich grundlegend nach der Ätiologie sowie nach dem Schweregrad der Elektrolytstörung.

The authors are reporting here a documented case of hyperparathyroidism leading to hypercalcemia diagnosed spontaneously. A 75-year old female patient with has been treated since 3-4 years with metformin and ramipiril fortype 2 diabetes and high blood pressure, respectively. She was seen in our endocrinology outpatient visit for tingling in the arms associated with cervical pain with increasing intensity over the last six months. The patient\'s mother was diabetic and her father had high blood pressure. The diagnostic of primary hyperparathyroidism, resulting from a diffuse hyperplasia of the parathyroid gland, was retained after medical, biological, chirurgical and anatomo-pathological investigations. The blood level of PTH1-84 was 916.60pg/ml Normal value=15-68.3pg/ml.
CONCLUSIONS: Diffuse poly-arthralgia in the neck and the lower limbs may not always be due to arthrosis in elderlies. Further investigation such as the dosage of blood level of calcium and PTH are required.
Les auteurs rapportent un premier cas d\'hyperparathyroïdie primaire de découverte fortuite documentée au Mali et responsable d\'hypercalcémie. Il s\'agissait d\'une patiente âgée de 75 ans, diabétique de type 2 depuis trois ans traitée par metformine et hypertendue depuis 4ans traitée par ramipril ; venue en consultation externe en endocrinologie pour fourmillement brachial et douleur cervicale diffuse augmentant progressivement d\'intensité depuis 6 mois. Aux antécédents familiaux de diabète chez la mère et hypertension artérielle chez le père. Apres les investigations médicaux, biologiques, chirurgicaux anatomopathologiques le diagnostic retenu fut une hyperparathyroïdie primaire (PTH1-84= 916,60pg/ml (N=15-68, 3pg/ml) causée par hyperplasie diffuse de la glande parathyroïde.
CONCLUSIONS: La présence de poly arthralgie diffuse cervicale et des membres inférieurs peut ne pas être seulement due à une arthrose chez le sujet âgé. Cela mérite une investigation plus approfondie telle que le dosage de la calcémie et de la PTH.

In 1953, for the first time, Paul Wermer described a family presenting endocrine gland neoplasms over several generations. The transmission was autosomal dominant and the penetrance was high. Forty years later in 1997, the multiple endocrine neoplasia type 1 (MEN1) gene was sequenced, thus enabling diagnosis and early optimal treatment. Patients carrying the MEN1 gene present endocrine but also non-endocrine tumors. Parathyroid, pancreatic and pituitary impairment are the three main types of endocrine involvement. The present article details therapeutic management of hyperparathyroidism, neuroendocrine pancreatic tumors and pituitary adenomas in patients carrying the MEN1 gene. Significant therapeutic progress has in fact been made in the last few years. As concerns the parathyroid glands, screening of family members and regular monitoring of affected subjects now raise the question of early management of parathyroid lesions and optimal timing of parathyroid surgery. As concerns the duodenum-pancreas, proton-pump inhibitors are able to control gastrin-secreting syndrome, reducing mortality in MEN1 patients. Mortality in MEN1 patients is no longer mainly secondary to uncontrolled hormonal secretion but to metastatic (mainly pancreatic) disease progression. Tumor risk requires regular monitoring of morphological assessment, leading to iterative pancreatic surgery in a large number of patients. Finally, pituitary adenomas in MEN1 patients are traditionally described as aggressive, invasive and resistant to medical treatment. However, regular pituitary screening showed them to be in fact infra-centimetric and non-secreting in the majority of patients. Consequently, it is necessary to regularly monitor MEN1 patients, with regular clinical, biological and morphological work-up. Several studies showed that this regular monitoring impairs quality of life. Building a relationship of trust between patients and care provider is therefore essential. It enables the patient to be referred for psychological or psychiatric care in difficult times, providing long-term support and preventing any breakdown in continuity of care.

Multiple Endocrine Neoplasia Type 1 (NEM1) is related to mutations of the menin gene. It is an autosomal dominant disease. Its prevalence is about 1/30 000 with a hugh penetrance. There is no genotype-phenotype correlation. This hereditary syndrome is characterized by the presence of tumors of the endocrine system (parathyroid, endocrine pancreas, pituitary and adrenal gland). Other disorders have also been described (bronchial and thymic carcinoid tumor, breast cancer, skin lesions). Management must take into account the specificities of these pathologies in NEM1 compared to sporadic forms (young age at diagnosis, multiple lesions within the same gland, multi-focal disease). © 2019 Published by Elsevier Masson SAS. All rights reserved. Cet article fait partie du numéro supplément Les Must de l\'Endocrinologie 2019 réalisé avec le soutien institutionnel de Ipsen-Pharma.

BACKGROUND: Parathyromatosis is a rare cause of recurrent hyperparathyroidism. It results from hyperfunctioning parathyroid tissue scattered throughout the thyroid bed region.
METHODS: A 51-year-old man with a history of parathyroidectomy, presented 18 years later with recurrent primary hyperparathyroidism. Surgical exploration identified a single parathyroid gland. The act was completed by a central compartment dissection and ipsilateral lobectomy. The patient was free of recurrence after a one-year follow-up.
CONCLUSIONS: Parathyromatosis a rare cause of recurrent hyperparathyroidism. Its management is challenging. Extensive surgery is required with clearance of the central neck compartment and homolateral lobectomy. Medical therapy could be used to decrease parathormone level in recurrent parathyromatosis.

BACKGROUND: With the current aging of the world\'s population, diagnosis of primary hyperparathyroidism is being reported in increasingly older patients, with the associated functional symptomatology exacerbating the vicissitudes of age. This retrospective study was designed to establish functional improvements in older patients following parathyroid adenomectomy under local anesthesia as outpatient surgery.
METHODS: Data were collected from 53 patients aged 80 years or older who underwent a minimally invasive parathyroid adenomectomy. All patients underwent a preoperative ultrasound, scintigraphy, and were monitored for the effectiveness of the procedure according to intra- and postdosage of parathyroid hormone (PTH) at 5min, 2h and 4h.
RESULTS: Mean preoperative serum calcium level was 2.8mmol/L (112mg/L) and mean PTH was 180pg/ml. Thirty-eight patients were operated under local anesthesia using minimally invasive surgery and 18 patients were operated under general anesthesia. In 26 cases, the procedure was planned on an outpatient basis but could only be carried out in 21 patients. Fifty-one patients had normal serum calcium and PTH levels during the immediate postoperative period. Two patients were reoperated under general anesthesia, since immediate postoperative PTH did not return to normal. Four patients died due to reasons unrelated to hyperparathyroidism. Five patients were lost to follow-up six months to two years postsurgery. Of the 44 patients (83%) with long-term monitoring for PTH, none had recurrence of biological hyperparathyroidism. Excluding the three asymptomatic patients, 38 of the 41 symptomatic patients (93%) with long-term follow-up were considering themselves as \"improved\" or \"strongly improved\" after the intervention, notably with respect to fatigue, muscle and bone pain. Two patients (4.9%) reported no difference and one patient (2.4%) said her condition had worsened and regretted having undergone surgery.
CONCLUSIONS: In patients 80 years or older, minimally invasive surgery as an outpatient under local anesthesia offered an excellent risk/benefit ratio given its many advantages: simplicity, speed, absence of general anesthesia, ease of monitoring, direct voice control intraoperatively, very low morbidity, effectiveness in treating primary hyperparathyroidism in more than 95% of first intention patients, and the possibility of immediate or delayed recovery in the event of multiglandular disease going unnoticed.

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