Ectopic Cushing syndrome associated with thymic carcinoid tumor as the first presentation of MEN1 syndrome-report of a family with MEN1 gene mutation

Multiple endocrine neoplasia type 1(MEN1) is an autosomal dominant syndrome. Although thymic carcinoid tumor is recognized as a part of MEN1 syndrome but functioning thymic carcinoid tumor as the first presentation of the MEN1 seems to be very rare. In this report, we present a 29-year-old male who developed ectopic Cushing syndrome secondary to thymic carcinoid tumor and was diagnosed as MEN1 syndrome 2 years later. Further evaluation revealed the presence of carcinoid tumor and other MEN 1 manifestations in several other member of family. Genetic evaluation showed presence of a previously reported mutation in exon 10(R527X) of MEN1 gene in these patients. This presentation showed that thymic neuroendocrine tumor could be the first manifestation of the MEN1 syndrome and it might be diagnosed as a dominant manifestation of this syndrome in a family. We suggest biochemical or genetic screening for MEN-1 syndrome for patients with thymic carcinoid.     

Dietary intake of fish,n-3 polyunsaturated fatty acids,and risk of inflammatory bowel disease: a systematic review and meta-analysis of observational studies

Malnutrition in an obese world was the fitting title of the 13th Federation of European Nutrition Societies (FENS) conference held in October 2019. Many in     

Dapsone as an Alternative Therapy in Children with Familial Mediterranean Fever

Objective

Familial Mediterranan Fever is an hereditary autoinflammatory disease that presents with recurrent febrile attacks and poly serositis. Colchicine is the only known treatment in this diease. However, nearly 5-10% of patients are resistant to colchicines. There are many different modalities in colchicine resistant patients, biologic and immunosupressive drugs being the known ones. We studied the efficacy of Dapsone as an anti inflammatory drug in children with FMF who did not tolerate colchicine well.

Methods

This is a case series study in 10 patients who had FMF on the base of Tel-Hashomer criteria and did not tolerate colchicine or did not respond to it well. Patients took 2mg/kg dapsone in single dose, during 6 months.

Findings

In four patients episodic attacks returned after 27 days, so the drug was discontinued. One patient refused to continue the study; in five patients dapsone was taken in average for 8 months and 6 days, at least for 6 months. These five patients had no episodes of attack during the following observation.

Conclusion

Dapsone could control episodic attacks of FMF in 50% of cases. It might be considered as an alternative therapy in FMF cases not responding to colchicine.     

Characteristics of anatomical landmarks in the mandibular interforaminal region: a cone-beam computed tomography study

Objectives: This study was conducted to assess appearance, visibility, location and course of anatomical landmarks in mandibular interforaminal region using cone-beam computed tomography (CBCT). Study design: A total of 96 CBCT examinations was re-evaluated to exploit anatomical landmarks. The examinations used the Promax 3D CBCT unit. A sole examiner carried out all the measurements. Visibilities of the anatomical landmarks were scored using a four-point rating scale. Results: The mandibular foramen, anterior loop, incisive canal and lingual foramen were observed in 100,84,83,49 % of the images, respectively. The mean size, diameter and width of anterior loop, incisive canal and lingual foramen were obtained 3.54± 1.41, 1.47±0.50 and 0.8 ± 0.09mm, respectively. Conclusion: It is not safe to recommend any definite distance mesially from the mental foramen. The diameter of the canals and foramens should be determined on a case-by-case basis to exploit the appropriate location for each individual. Key words:Anatomical landmarks, cone-beam computed tomography, implant surgery, radiographic evaluation, surgical complications.