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1.
Eur J Pediatr ; 175(3): 305-12, 2016 Mar.
Article in English | MEDLINE | ID: mdl-26811298

ABSTRACT

UNLABELLED: Enterocutaneous fistula and its conservative management still pose a challenge for the surgeon. The use of octreotide and somatostatin in neonates and children as adjunctive therapy in the conservative management of this condition, leads to major controversy regarding its efficacy. Therefore, we conducted an extensive literature review of published articles regarding the use of somatostatin and its analogues in the treatment of enterocutaneous fistula in neonates and children. Our review is then presented together with a case vignette and discusses the different practical aspects of the treatment with these drugs. CONCLUSION: The major diversity in treatment regimens among published studies makes outcomes difficult to compare. However, given the results of the different cases reported in the literature and of our own experience, we suggest a possible beneficial effect of octreotide and somatostatin on closure of enterocutaneous fistula in these patients.


Subject(s)
Gastrointestinal Agents/therapeutic use , Intestinal Fistula/drug therapy , Octreotide/therapeutic use , Somatostatin/therapeutic use , Child , Child, Preschool , Conservative Treatment , Gastrointestinal Agents/adverse effects , Humans , Infant , Infant, Newborn , Infant, Premature , Male , Octreotide/adverse effects , Somatostatin/adverse effects
2.
Am J Med Genet A ; 132A(1): 41-8, 2005 Jan 01.
Article in English | MEDLINE | ID: mdl-15551327

ABSTRACT

Craniofacial dyssynostosis (CD) is characterized by premature fusion of the lambdoid and posterior part of the sagittal sutures, and short stature. Thus, the skull shape becomes dolichocephalic with protuberant forehead and either bulging or flat occiput. Facial changes are secondary to the skull defects, and some additional findings have also been described. We report on the first four known Spanish patients. They were unrelated and had Spanish ancestors. In the three previous reports about this syndrome, the authors hypothesized that the frequency of the gene causing CD must be rather high in the Spanish population, and relatively common in areas with Spanish ancestry. We have estimated the minimal birth prevalence of the syndrome in 0.51 per million livebirths. It has been previously suggested that the syndrome is inherited as an autosomal recessive trait, since there were two affected sisters among the nine published cases. Phenotypic variability is discussed in detail in this paper. We also underline several aspects for the anticipatory guidance of affected individuals, especially recommending a neurologic evaluation taking into account the radiologic findings in order to plan early interventions to avoid undesirable consequences of craniosynostosis. It is also recommended to perform additional studies (ophthalmologic, cardiologic, among others) to rule out the existence of associated anomalies, which are more frequent than previously considered.


Subject(s)
Craniofacial Dysostosis/pathology , Craniosynostoses/pathology , Child, Preschool , Craniofacial Dysostosis/genetics , Craniosynostoses/genetics , Humans , Karyotyping , Male , Spain , Syndrome
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