ABSTRACT
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.
Subject(s)
Mucopolysaccharidosis III/complications , Mucopolysaccharidosis III/diagnosis , Mutation , Puberty, Precocious/complications , Adolescent , Body Height/drug effects , Brain/pathology , Child , Child Behavior Disorders/complications , Child Behavior Disorders/drug therapy , DNA Mutational Analysis , Gonadotropin-Releasing Hormone/agonists , Humans , Luteolytic Agents/therapeutic use , Magnetic Resonance Imaging/methods , Male , Mucopolysaccharidosis III/pathology , Puberty, Precocious/diagnosis , Puberty, Precocious/drug therapy , Triptorelin Pamoate/therapeutic useABSTRACT
The impact of treatment of central precocious puberty (CPP) with gonadotropin-releasing hormone agonists (GnRHa) on final height remains controversial. We analyzed the long term results of 23 girls with CPP treated with triptorelin or leuprolide. Their "near final height" (NFH) assessed at a bone age of at least 14 years and expressed as SDS, was compared either with predicted height before treatment (PAH) or with parental height (TH). We also compared NFH of 12 girls treated before 8 years of age (7.0 +/- 0.5 yr) with NFH of 11 girls treated after 8 years old (8.5 +/- 0.3 yr). The NFH of the 23 girls (-0.9 +/- 1.0 SDS) was not different either from PAH (-0.85 +/- 1.5 SDS) or from TH (-0.5 +/0.6 SDS). Earlier treated girls reached a NFH (-0.97 +/- 1.0 SDS) not different from later treated girls (-0.91 +/- 1.0 SDS; p = ns) and both groups reached parental height (NFH - TH = -0.44 +/- 1 and -0.09 +/- 0.83 SDS, respectively). In conclusion, our patients, treated either earlier or later, reached a near final height comparable to predicted height and familial target; however, these results might still improve further because the girls have not yet reached their final adult height.
Subject(s)
Body Height/drug effects , Brain Diseases/complications , Gonadotropin-Releasing Hormone/agonists , Puberty, Precocious/drug therapy , Puberty, Precocious/etiology , Bone Development , Child , Female , Humans , Leuprolide/therapeutic use , Puberty, Precocious/physiopathology , Triptorelin Pamoate/therapeutic useABSTRACT
It has been reported that girls at onset of idiopathic central precocious puberty (ICPP) and during treatment have symbiotic character traits. The aim of this study was to investigate the development of character in a group of adolescents. Ten adolescent girls aged 14 years treated for ICPP were evaluated. All the adolescents in the study had a negative body image compared with age-matched controls and expressed a strong inhibition of their femininity. Their poor body image is reflected by their limited self-esteem. These adolescents have not been able to operate a reorganization of their affective life and therefore go through the necessarily slow and painful separation from their family. Symbiotic traits are "hard-wired" into their lives. These results suggest that at ICPP onset, in addition to setting up an educational program for the parents, it is equally important to supply psychological support for the patients in order to gain a better interaction between biological, psychological and cultural influences.
Subject(s)
Brain Diseases/complications , Character , Puberty, Precocious/etiology , Puberty, Precocious/psychology , Child , Female , Follow-Up Studies , Gonadotropin-Releasing Hormone/analogs & derivatives , Humans , Puberty, Precocious/drug therapyABSTRACT
BACKGROUND: Head circumference of children with multiple pituitary-hormone deficiency (MPHD) may be subnormal for age. Moreover it is known that linear growth in infancy is growth-hormone (GH) dependent. Therefore, aim of the study has been to compare head circumference measurements in children with hypopituitarism after L-thyroxine (L-T4) therapy alone, before therapy with GH, and after GH was added to the therapy. METHODS: Five infants (2 girls, 3 boys) with MPHD, diagnosed and treated before the age of 2 years and whose auxological parameters records during L-T4 therapy alone were available, were retrospectively studied. Head circumference and length measurements were expressed as standard deviation score (SDS). Weight measurements were expressed as weight for length ratio percentage. RESULTS: Initially treated with L-T4 alone for a mean period of 4, 5 months, there were neither positive effects on head circumference nor on linear growth. A significant catch-up growth was observed only employing GH therapy in addition to L-T4: mean head circumference SDS and length SDS increased respectively from -2.20 to -0.89 SDS and from -4.16 to -0.87 SDS after a mean period of 18 months of combined GH and L-T4 therapy. CONCLUSIONS: Therefore, head circumference growth, in infancy, is growth hormone dependent as well as linear growth, and during GH treatment, monitoring head circumference growth is important as much as monitoring linear growth.
Subject(s)
Head/anatomy & histology , Human Growth Hormone/therapeutic use , Hypopituitarism/drug therapy , Thyroxine/therapeutic use , Adolescent , Anthropometry , Child , Female , Humans , Male , Retrospective StudiesABSTRACT
BACKGROUND: Head circumference of children with multiple pituitary-hormone deficiency (MPHD) may be subnormal for age. Moreover it is known that linear growth in infancy is growth-hormone (GH) dependent. Therefore, aim of the study has been to compare head circumference measurements in children with hypopituitarism after L-thyroxine (L-T4) therapy alone, before therapy with GH, and after GH was added to the therapy. METHODS: Five infants (2 girls, 3 boys) with MPHD, diagnosed and treated before the age of 2 years and whose auxological parameters records during L-T4 therapy alone were available, were retrospectively studied. Head circumference and length measurements were expressed as standard deviation score (SDS). Weight measurements were expressed as weight for length ratio percentage. RESULTS: Initially treated with L-T4 alone for a mean period of 4, 5 months, there were neither positive effects on head circumference nor on linear growth. A significant catch-up growth was observed only employing GH therapy in addition to L-T4: mean head circumference SDS and length SDS increased respectively from -2.20 to -0.89 SDS and from -4.16 to -0.87 SDS after a mean period of 18 months of combined GH and L-T4 therapy. CONCLUSIONS: Therefore, head circumference growth, in infancy, is growth hormone dependent as well as linear growth, and during GH treatment, monitoring head circumference growth is important as much as monitoring linear growth.