Precocious puberty in Sanfilippo IIIA disease: diagnosis and follow-up of two new cases.
To date the association between mucopolysaccharidosis (MPS) IIIA and precocious puberty has been found in only three polish patients. We observed two children affected by MPS IIIA with central precocious puberty (CPP) both treated with GnRH agonists. The occurrence of CPP in both patients with MPS IIIA suggests that it is necessary to look for an association between the two conditions. The follow-up of our two patients leads us to believe also that GnRH agonist treatment can have a beneficial effect on final height and probably on the improvement of behavioural problems.
Concolino
D
D
Department of Pediatrics, University "Magna Graecia" of Catanzaro, c/o Ospedale Civile "A.Pugliese", Viale Pio X, 88100 Catanzaro, Italy.
Muzzi
G
G
Pisaturo
L
L
Piccirillo
A
A
Di Natale
P
P
Strisciuglio
Pietro
P
D000293:Adolescent; D001827:Body Height / Q000187:drug effects; D001921:Brain / Q000473:pathology; D002648:Child; D002653:Child Behavior Disorders / Q000150:complications / Q000188:drug therapy; D004252:DNA Mutational Analysis; D007987:Gonadotropin-Releasing Hormone / Q000819:agonists; D006801:Humans; D008186:Luteolytic Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D009084:Mucopolysaccharidosis III / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology; D009154:Mutation; D011629:Puberty, Precocious / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D017329:Triptorelin Pamoate / Q000627:therapeutic use
D008186:Luteolytic Agents; D017329:Triptorelin Pamoate; D007987:Gonadotropin-Releasing Hormone