Search

Objective To investigate the clinical characteristics and management strategies for multiple endocrine neoplasia 1 （MEN1） during pregnancy. Methods The gender, age, detailed medical history, clinical symptoms and signs, laboratory, imaging and pathological examination data of a MEN1 proband were collected. And the demographic characteristics, clinical characteristics and auxiliary examination of 20 members from the MEN1 family were also collected. MEN1 cases during pregancy were searched from PubMed and Wanfang Data using the keywords of “Multiple Endocrine Neoplasia Type 1“and“Pregnant” in both English and Chinese. Results The main clinical manifestations of the MEN1 proband were pituitary microadenoma （galactorrhea, elevated prolactin）, pancreatic endocrine tumor （hypoglycemia） and parathyroid adenoma （asymptomatic, elevated parathyroid hormone and blood calcium）. Gene analysis confirmed that exon 9 of MEN1 gene （CGA > TGA, Arg415Term） was the pathogenic mutated gene. The father and daughter of the proband carried this pathogenic mutation and had hyperparathyroidism. After drug treatment for pituitary microadenoma and surgical treatment for pancreatic endocrine tumor and parathyroid adenoma, the proband was pregnant and delivered a female infant successfully. During this period, there was no recurrence of galactorrhea and hypoglycemia, the levels of parathyroid hormone and blood calcium were stable, and fetal growth and development were normal. A total of seven pregnant cases of MEN1 in English literature and one in Chinese literature were retrieved. It was found that all MEN1 patients developed onset of diseases at childbearing age. As for clinical symptoms, parathyroid, pancreas and pituitary were the most involved. Most patients had family history and MEN1 gene mutation. In terms of pregnancy outcome, seven patients delivered successfully under strict condition monitoring and treatment. Conclusion Clinicians’ active detection, diagnosis and treatment will help to improve the prognosis of MEN1 patients and their offspring.

In our previous study, we showed that with increasing time in culture, the growth characteristics of enteric neural crest-derived cells (ENCCs) change, and that the proliferation, migration and neural differentiation potential of these cells in vitro notably diminish. However, there are no studies on the developmental differences in these characteristics between fetal and early-postnatal stages in vitro or in vivo. In this study, we isolated fetal (embryonic day 14.5) and postnatal (postnatal day 2) ENCCs from the intestines of rats. Fetal ENCCs had greater maximum cross-sectional area of the neurospheres, stronger migration ability, and reduced apoptosis, compared with postnatal ENCCs. However, fetal and postnatal ENCCs had a similar differentiation ability. Fetal and postnatal ENCCs both survived after transplant into a rat model of Hirschsprung's disease. In these rats with Hirschsprung's disease, the number of ganglionic cells in the myenteric plexus was higher and the distal intestinal pressure change was greater in animals treated with fetal ENCCs compared with those treated with postnatal ENCCs. These findings suggest that, compared with postnatal ENCCs, fetal ENCCs exhibit higher survival and proliferation and migration abilities, and are therefore a more appropriate seed cell for the treatment of Hirschsprung's disease. This study was approved by the Animal Ethics Committee of the Second Affiliated Hospital of Xi'an Jiaotong University (approval No. 2016086) on March 3, 2016., Competing Interests: None