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1.
Pediatr Surg Int ; 15(5-6): 385-6, 1999 Jul.
Article in English | MEDLINE | ID: mdl-10415293
2.
J Pediatr Surg ; 34(4): 568-71, 1999 Apr.
Article in English | MEDLINE | ID: mdl-10235324

ABSTRACT

BACKGROUND: Patients with multiple endocrine neoplasia (MEN) type 2 are at risk for early medullary thyroid carcinoma (MTC). Recently, the cloning of the ret oncogene has made it possible to identify patients at risk for MEN 2 syndrome with a high degree of reliability before presenting any symptoms. METHODS: Children of families with MEN 2 were screened genetically if one of the parents was a known gene carrier of the RET proto-oncogene. If they were carriers, thyroidectomy was performed. RESULTS: The authors report five children with MEN 2 who underwent prophylactic thyroidectomy irrespective of the results of calcitonin screening tests after genetic screening had shown that they were carrier of the RET proto-oncogene. Apart from a temporary hypocalcemia in one, the operations were uneventful. Pathology results showed MTC in three children of one family with MEN 2A at age 2, 3, and 6 years. In two families with MEN 2B the thyroidectomy specimen showed bilateral MTC in a 1-year-old and a 3-year-old child. CONCLUSIONS: These findings show that MTC occurs at very young age in children with MEN 2. The authors advocate performing prophylactic thyroidectomy in the first year of life in children with MEN 2B and at age 2 years in children with MEN 2A to obtain an optimal cure rate.


Subject(s)
Carcinoma, Medullary/prevention & control , Multiple Endocrine Neoplasia Type 2a/genetics , Multiple Endocrine Neoplasia Type 2b/genetics , Proto-Oncogene Proteins/genetics , Proto-Oncogenes/genetics , Thyroid Neoplasms/prevention & control , Thyroidectomy , Carcinoma, Medullary/genetics , Child , DNA Mutational Analysis , Female , Genetic Carrier Screening , Humans , Infant , Male , Proto-Oncogene Mas , Receptor Protein-Tyrosine Kinases/genetics , Thyroid Neoplasms/genetics
3.
Gynecol Obstet Invest ; 43(3): 206-8, 1997.
Article in English | MEDLINE | ID: mdl-9127138

ABSTRACT

Ileus during pregnancy is a rare complication. Malrotation in the adult is an even rarer finding. A case of a woman who developed an ileus in her seventeenth week of pregnancy is presented. At surgery the cause appeared to be a malrotation of the midgut. The incidence and causes of ileus during pregnancy are discussed. Also a short description of the different forms of malrotation are presented. New insights in the etiology of malrotation based on experiments with rats are mentioned.


Subject(s)
Intestinal Obstruction/etiology , Pregnancy Complications , Adult , Female , Humans , Intestinal Diseases/complications , Intestinal Obstruction/diagnosis , Intestinal Obstruction/surgery , Pregnancy , Pregnancy Outcome
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