BACKGROUND: Late familial hyperinsulinemic @entity4053 is characterized by recurrent episodes of @entity4053 and an inappropriate insulinemic response. Treatment with @entity2390 ( @entity1943 analogue) reduces the prevalence of clinical significant @entity4053 and might be beneficial during pregnancy. To our knowledge this is the first report of a @entity1 with late familial hyperinsulinemic @entity4053 experiencing pregnancies with and without @entity2390 treatment. CASE PRESENTATION: A 35-year-old Caucasian @entity1 known to suffer from late familial hyperinsulinemic @entity4053 due to a well-known mutation in the @entity8277 gene has been pregnant 6 times. The @entity1 was treated with injections of @entity2390 LAR   ( @entity2390 ) during the first four pregnancies. Her first pregnancy in 1999 was unknown until approximately 25th gestational weeks with fatal @entity1592 . The following two pregnancies were terminated on parental request after a chorion villus biopsy revealed the mutation causing late familial hyperinsulinemic @entity4053 . During the fourth pregnancy, in which the fetus also had the mutation, serial ultrasound examinations showed a small fetus with appropriate growth. At birth the @entity1 was small for gestational age. She was admitted to the neonatal special care unit due to low blood @entity413 and intravenous @entity413 and early feeding was initiated. One day old, her condition deteriorated with signs of an abdominal catastrophe indicating @entity3166 . After two laparotomies - both confirming @entity3166 - the @entity1 died 8 days after birth.In the following two pregnancies Sandostatin LAR   was stopped before pregnancy and the @entity1 was treated only with diet restriction and intensive @entity413 monitoring. Both pregnancies ended successfully. One @entity1 carried the mutation and was small for gestational age at birth while the other @entity1 did not carry the mutation and had normal birth weight. CONCLUSION: In a @entity1 with late familial hyperinsulinemic @entity4053 @entity2390 was given during the first four pregnancies resulting in 2 cases of early termination of pregnancy on parental request and 2 cases of inappropriate fetal growth and unviable outcome. The following two pregnancies treated with diet only had a successful outcome.

Poor pregnancy outcome after @entity2390 treatment during pregnancy for familial hyperinsulinemic XXXX : a case report.

@entity1 :: ('9606', 'Species') :: ['girl', 'woman', 'patient', 'child'] @entity3166 :: ('MESH:D020345', 'Disease') :: ['necrotizing enterocolitis'] @entity8277 :: ('3643', 'Gene') :: ['insulin receptor'] @entity4053 :: ('MESH:D007003', 'Disease') :: ['hypoglycemia'] @entity1592 :: ('MESH:D005317', 'Disease') :: ['intrauterine growth retardation'] @entity2390 :: ('MESH:D015282', 'Chemical') :: ['octreotide', 'Sandostatin'] @entity1943 :: ('MESH:D013004', 'Chemical') :: ['somatostatin'] @entity413 :: ('MESH:D005947', 'Chemical') :: ['glucose']
@entity4053 :: (MESH:D007003,Disease) :: ['hypoglycemia']
