Hypothalamo-Pituitary Function in Four Cases of True Precocious Puberty

Buonaguidi, Roberto; Ferdeghini, M; Leoncini, Rossella; Tusini, G

doi:10.1159/000120057

Cited by 4 publications

(3 citation statements)

References 3 publications

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“…Various case reports published later on endocrine manifestations in NF1 [17][18][19][20] concerned primarily CPP (Table 2), not GHD. Comparison of the studies is difficult because of the lack of clinical data as well as the fact that diagnostic criteria for NF1 were only defined by NIH in 1987.…”

Section: Discussionmentioning

confidence: 99%

Endocrinologic Disorders and Optic Pathway Gliomas in Children With Neurofibromatosis Type 1

et al. 1997

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ABSTRACT. Objective. To establish the prevalence of endocrinologic disorders in children with neurofibromatosis type 1 (NF1) and the relationship between these disorders and cerebral abnormalities on magnetic resonance imaging.Design. A prospective follow-up study. Setting. A multidisciplinary neurofibromatosis clinic.Patients. A total of 122 children diagnosed with NF1 according to diagnostic criteria set by the National Institutes of Health.Results. Central precocious puberty (CPP) was diagnosed in 3 children and growth hormone deficiency (GHD) in 3 children. Optic pathway gliomas were observed in 15 children; in 9 of the 15 cases, the optic chiasm was involved. Of the 3 children with CPP, only 1 showed a chiasma glioma on magnetic resonance imaging. In 1 case with GHD, an optic chiasm glioma was detected on neuroimaging. Two of the 9 children with an optic chiasm glioma presented with CPP or GHD.Conclusions. It has been suggested that CPP in children with NF1 is found exclusively in the presence of a chiasma glioma. We conclude that chiasma glioma may not be obligatory in children with NF1 and CPP or GHD. Moreover, we report a prevalence of GHD in children with NF1 of 2.5%, which has not been established earlier. Endocrine disorders have been reported in ϳ1% to 3% of all NF1 patients. In adults, pheochromocytoma is the most common disorder, presenting in ϳ1% of NF1 patients.2 In children with NF1, central precocious puberty (CPP) is the most frequent endocrinopathy and is seen in 3% of NF1 patients compared with .06% in the general pediatric population.2-4 Although various case reports have been published, the prevalence of growth hormone deficiency (GHD) in children with NF1 is unknown and is assessed in this paper.Listernick et al 5 reported that routine magnetic resonance neuroimaging in 176 children with NF1 revealed optic pathway gliomas in 33 (19%). Almost half of these gliomas were asymptomatic. In particular, gliomas of the optic chiasm have been reported to cause endocrinologic disorders, especially CPP. 6In a prospective follow-up study in a multidisciplinary NF1 clinic, we analyzed the prevalence of endocrinologic disorders in children and the relationship between these disorders and cerebral abnormalities on neuroimaging. PATIENTS AND METHODSSince 1985, a multidisciplinary NF1 team at Sophia Children's University Hospital in Rotterdam (including a pediatrician, dermatologist, pediatric neurologist, ophthalmologist, and clinical geneticist) has evaluated children suspected of having NF1 according to NIH criteria. Besides periodic clinical evaluations, xrays of the cranium and the entire spine and a visual evoked potential (VEP) are performed at presentation. Computed tomography (CT) scanning and/or magnetic resonance imaging (MRI) is initiated when indicated.Before January 1995, 171 new cases were seen. NF1 was diagnosed in 122 children (58 girls, 64 boys). The average follow-up period in the NF1-affected group was 4.4 years (standard deviation [SD] ϭ 4.1). The mean age of these children at last e...

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Section: Discussionmentioning

confidence: 99%

Endocrinologic Disorders and Optic Pathway Gliomas in Children With Neurofibromatosis Type 1

et al. 1997

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“…Most of the chiasmal and hypothalamic gliomas occur in infants and children and are usually low-grade astrocytomas/pilocytic astrocytomas [9]. With the advent of MRI, lesions involving the retrochiasmal optic pathways and hypothalamus can be distinguished readily from the more indolent anterior optic nerve glioma [1,2,3,8,9,10,11,12]. These gliomas are less amenable to surgical resection, with frequent residual tumors left behind to avoid postoperative neurological deterioration/visual deterioration.…”

Section: Discussionmentioning

confidence: 99%

“…The association of hydrocephalus with these tumors is reported [11, 12]. The defective blood-brain barrier of tumor blood vessel may allow escape of protein into the CSF.…”

Section: Discussionmentioning

confidence: 99%

Hypothalamic Glioma Presenting with Seizures

Gupta

Satyarthee²,

Sharma³

et al. 2006

Pediatr Neurosurg

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A rare case of hypothalamic glioma in a 7-year-old boy presenting with seizures and diabetes insipidus is reported. Near total decompression of the hypothalamic glioma was done successfully using subfrontal approach. The patient had a stormy postoperative course due to status epilepticus but went on to make a complete recovery. Postoperative radiotherapy/chemotherapy was not given in view of tumor histology (low grade glioma), patient’s age and tumor location. Patient remains symptom and seizure free on antiepileptics at 3-year follow-up. The case is presented in the light of its rarity and the literature is reviewed.

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Optic chiasm glioma associated with inappropriate secretion of antidiuretic hormone, cerebral ischemia, nonobstructive hydrocephalus and chronic ascites following ventriculoperitoneal shunting

et al. 1991

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An optic chiasm glioma may cause loss of vision, endocrine disturbances, hydrocephalus and cerebral ischemia due to its proximity to the pituitary, hypothalamus, III ventricle and internal carotids. A 3-month-old infant with optic chiasm glioma developed hypopituitarism and inappropriate secretion of antidiuretic hormone with plasma hypo-osmolality. The cerebrospinal fluid (CSF) protein concentration was markedly elevated. The impairment of fluid absorption via arachnoid villi and peritoneum by the high protein content, and reversed osmotic gradient between protein-rich CSF and hypo-osmolar plasma may have contributed to both nonobstructive hydrocephalus and recurrent ascites following ventriculoperitoneal shunting. Cerebral ischemia from carotid compression may have led to cerebral atrophy.

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Hypothalamo-Pituitary Function in Four Cases of True Precocious Puberty

Cited by 4 publications

References 3 publications

Endocrinologic Disorders and Optic Pathway Gliomas in Children With Neurofibromatosis Type 1

Endocrinologic Disorders and Optic Pathway Gliomas in Children With Neurofibromatosis Type 1

Hypothalamic Glioma Presenting with Seizures

Optic chiasm glioma associated with inappropriate secretion of antidiuretic hormone, cerebral ischemia, nonobstructive hydrocephalus and chronic ascites following ventriculoperitoneal shunting

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