Document Detail

Precocious puberty in three boys with Sanfilippo A (mucopolysaccharidosis III A).
MedLine Citation:
PMID:  8821908     Owner:  NLM     Status:  MEDLINE    
Three boys with mucopolysaccharidosis type III A (Sanfilippo) entered puberty at the ages of 5.9, 6.6 and 9.6 years. The diagnosis of central precocious puberty (GnRH-dependent) was established on the basis of pubertal levels of basal and GnRH-stimulated gonadotropins, LH and FSH. All the patients were tall from early infancy, but there was no acceleration of growth velocity or bone maturation despite advanced pubertal stage and high testosterone levels. Computerized tomography of the brain showed changes which are characteristic for Sanfilippo. This is the first time that precocious puberty has been described in Sanfilippo patients. The question of whether the precocious puberty in our Sanfilippo patients is a coincidence or is related in some way remains open.
A Tylki-Szymańska; M Metera
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of pediatric endocrinology & metabolism : JPEM     Volume:  8     ISSN:  0334-018X     ISO Abbreviation:  J. Pediatr. Endocrinol. Metab.     Publication Date:    1995 Oct-Dec
Date Detail:
Created Date:  1996-11-12     Completed Date:  1996-11-12     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  9508900     Medline TA:  J Pediatr Endocrinol Metab     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  291-3     Citation Subset:  IM    
Department of Inherited Metabolic Diseases, Child Health Centre, Warsaw, Poland.
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MeSH Terms
Brain / pathology,  radiography
Child, Preschool
Follicle Stimulating Hormone / blood
Gonadotropin-Releasing Hormone / diagnostic use
Luteinizing Hormone / blood
Mucopolysaccharidosis III / complications*,  pathology
Puberty, Precocious / complications*
Testosterone / blood
Tomography, X-Ray Computed
Reg. No./Substance:
33515-09-2/Gonadotropin-Releasing Hormone; 58-22-0/Testosterone; 9002-67-9/Luteinizing Hormone; 9002-68-0/Follicle Stimulating Hormone

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