Hypercalcemic crisis in third trimenon: evaluating the optimal treatment strategy

Hypercalcemia due to primary hyperparathyroidism during pregnancy is a rare condition and associated with increased morbidity and mortality for the mother and the unborn child. Whereas parathyroidectomy is favored during the second trimester, no clear recommendations exist for its management during the third trimenon. We here report the case of a 26-year-old woman in the 29th week of her first pregnancy, who was admitted to our clinic with hypertension, intra-uterine growth retardation and polyhydramnios. Severe hypercalcemia due to primary hyperparathyroidism was diagnosed (total calcium 3.34?mmol/l; PTH 216?pg/ml), but no enlarged parathyroid gland could be localized by ultrasound. Treatment with calcitonin and cinacalcet could not control hypercalcemia. Therefore explorative surgery was performed and a single parathyroid adenoma was resected, resulting in normalization of serum calcium levels. The surgical procedure was tolerated well by the mother and fetus. Hypercalcemia-induced hypertension and polyhydramnios ameliorated before C-section was performed two weeks later and unrelated to the intervention. This case report underlines the importance of early diagnosis and treatment of primary hyperparathyroidism during pregnancy. If diagnosed in the third trimenon, an interdisciplinary approach is crucial. If medical treatment fails to sufficiently control hypercalcemia, surgical parathyroid exploration should be considered even in cases of unsuccessful localization of adenomatous parathyroid glands.     

Familial hyperparathyroidism syndromes

Primary hyperparathyroidism is a common endocrine disorder and the most prevalent cause of hypercalcemia worldwide. While most cases are sporadic, 5–10% of cases are inherited as part of a familial syndrome: multiple endocrine neoplasia (MEN-1, MEN-2A, MEN-4), hyperparathyroidism jaw-tumor syndrome (HPT-JT), familial hypocalciuric hypercalcemia (FHH), neonatal severe hyperparathyroidism (NSHPT), autosomal dominant moderate hyperparathyroidism (ADMH), or familial isolated hyperparathyroidism (FIHPT). Recent developments in molecular pathology identified specific germline mutations (MEN1, RET, CDKIs, CDC73/HRPT2, CaSR, GNA11, AP2S1) implicated in their pathogenesis. In contrast to sporadic primary hyperparathyroidism which is usually caused by a solitary parathyroid adenoma, hereditary hyperparathyroidism tend to present with multiglandular parathyroid disease, with variable penetrance according to the genetic syndrome. As a result, the clinical severity of each familial condition varies tremendously, resulting in distinct prognosis and treatment strategies. With the advent of molecular testing, genetic subtyping has become an integral part of treatment decision making, requiring correlation with clinical and pathologic findings. This review provides an update on the current knowledge of hereditary hyperparathyroidism and its associated genetic syndromes.     

Efficacy and its predictor in microwave ablation for severe secondary hyperparathyroidism in patients undergoing haemodialysis

Background: Microwave ablation (MWA) can be used to treat severe secondary hyperparathyroidism; however, its efficacy and the predictor of its efficacy are unclear. In this retrospective study we determined the predictor of efficacy of MWA and compared the efficacy of MWA and parathyroidectomy.

Materials and methods: Patients with severe secondary hyperparathyroidism who had received MWA or parathyroidectomy were enrolled in the study. Participants with MWA were divided into response and no response groups based on efficacy. Possible predictors were analysed using logistic regression to determine efficacy predictors. The participants were divided into MWA and parathyroidectomy groups, and the efficacy (including rates of achieving recommended goals for intact parathyroid hormone (iPTH), calcium, and phosphorus levels) were compared between the two groups.

Results: Thirty-one participants were enrolled for predictor analysis. Only baseline iPTH level predicted efficacy (OR 0.997, P?=?0.018). The optimal threshold value of iPTH for predicting efficacy was 1493.5?pg/mL. To compare efficacy, 30 patients were enrolled in MWA (18/30) and parathyroidectomy (12/30) groups. The rates of achieving recommended goals for iPTH levels varied between 0 and 60%; a significant difference was found between the groups at 5 months (P?=?0.01). However, in the parathyroidectomy group, the iPTH level and rate of iPTH <124?pg/mL (lower limit of target range) were significantly lower than in the MWA group after treatment (40–75% versus 0–16.7%).

Conclusion: Baseline iPTH level is a good predictor of MWA efficacy for severe secondary hyperparathyroidism; parathyroidectomy is more effective for severe secondary hyperparathyroidism than MWA.     

^60Coγ射线放疗水平二级剂量标准的TLD国际比对

目的通过参加IAEA/WHO组织的TLD国际比对,检查二级标准剂量学实验室（SSDL）放疗水平剂量标准和国际标准的一致性。方法 SSDL对IAEA邮寄的TLD进行照射,并计算出其吸收剂量,然后将TLD及其计算结果寄往IAEA剂量学实验室,IAEA对其进行评价后给出比对的偏差。结果本次60Coγ射线吸收剂量比对的偏差为-1.8%。结论按照IAEA要求,该项比对的最大允许偏差为±3.5%,所以这次比对结果是合格的。     

SIMULTANEOUS OCCURRENCE OF ADVANCED NEUROBLASTOMA AND ACUTE MYELOID LEUKEMIA

The authors report the case of a 4-year-old boy with a diagnosis of stage IV neuroblastoma (NB), who had been treated with 6 cycles of cyclophosphamide, doxorubicin, cisplatin, and etoposide for 12 months. The patient reached partial remission and presented a diagnosis of acute myelomonocytic leukemia (M4 AML), confirmed by immunophenotyping. After 2 months of therapy for leukemia, the child died with both malignancies in activity. A necropsy histologically confirmed the simultaneity of the two diseases. The authors review the possibilities of this association. The review leads to the conclusion that AML can occur as a secondary malignancy after the onset of the neuroblastoma, or be suggested by a misdiagnosis. The simultaneous occurrence of both as described here is not, however, found in the literature, to the best of the authors' knowledge.     

Hypertension,Coronary Artery and Cerebrovascular Diseases in the Population. Has Epidemiology Changed in the Last Decades?

Life expectancy has significantly increased in the last decades in many western populations, due to the fall of total and cardiovascular death rate. However, morbidity from cardiovascular diseases has decreased to a smaller extent. The overall population risk profile has improved, but it is still unsatisfactory. This is true for blood pressure control (with only 20% of hypertensive patients achieving normotension with antihypertensive drugs), hypercholesterolemia (with bordeline-high serum cholesterol levels in 50% of the population), and smoking habits. Other potential causes of the poor cardiovascular prevention are: 1) a limited knowledge of the optimal blood