The NIH experience with precocious puberty: Diagnostic subgroups and response to short-term luteinizing hormone releasing hormone analogue therapy
Ora Hirsch PescovitzFlorence ComiteKaren HenchKevin M. BarnesANN McNEMARCarol M. FosterDaniel KenigsbergD. Lynn LoriauxGordon B. Cutler
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Keywords:
Sex steroid
Hypothalamic–pituitary–gonadal axis
Central precocious puberty
Hypothalamic disease
Central precocious puberty (CPP) presenting at a very young age is likely to have an underlying pathology. One of the pathologies is hypothalamic hamartoma (HH), a non-neoplastic tumour-like lesion located at the floor of the third ventricle, near the tuber cinereum. Two young children with CPP due to HH without gelastic seizures or mental retardation were successfully managed and described in this report.
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We described the clinical presentation,image changes,pathologic manifestation,and hormone data before and after surgical excision in a 2 years-old boy with typical central precocious puberty (CPP) resulted from hypothalamic hamartoma(HH).With literature review,the epidemiologic and clinical features,hormone profile,diagnosis,and therapeutic options of CPP induced by HH were discussed to update the general understandings for the management of this disease.
Key words:
Hypothalamic disease; Hamartoma; Central precocious puberty
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Precocious puberty defined by the onset of secondary sexual characteristics before 8 years in girls and 9 years in boys. It is more common in females than males and is usually sporadic. Depending on the primary source of hormonal production, precocious puberty is classified as central and peripheral. Precocious puberty in infants is very rare. While investigating a case of precocious puberty, it is essential to progress systematically, with an identification of isolated or complete precocious puberty followed by bone age estimation, relevant hormonal assays, including GnRH stimulation, as well as neuroimaging when indicated. We present a case of isosexual (central) precocious puberty in a 1 year, 3-month-old girl, who was symptomatic for 1 year of age and was diagnosed to have hypothalamic hamartoma after methodical evaluation and responded to treatment with GnRH agonists.
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Hypothalamic hamartomas (HHs) are tumors generally associated with isolated central precocious puberty (CPP). To our knowledge, we report a unique case of a girl with HH associated with CPP and growth hormone deficiency. This case highlights the complex interaction between HHs and the hypothalamic-pituitary-gonadal axis. It also emphasizes the value of close follow-up of growth velocity in these patients even after treatment of the CPP.
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Central precocious puberty (CPP) is caused by premature activation of the hypothalamic-gonadal axis, and must be treated adequately. In particular, CPP that occurs at a relatively young age or in boys is likely to be caused by an organic lesion. Hypothalamic hamartoma (HH) is the most common organic cause of CPP. The present case report describes an 11-month-old female infant who presented with vaginal bleeding and rapidly progressive secondary sex characteristics from the age of 6 months. She was diagnosed with CPP following the detection of HH via magnetic resonance imaging. The infant girl was successfully treated with gonadotropin-releasing hormone agonist. After 6 months, her breast had regressed and clinical and radiological follow-up demonstrated stable findings with no evidence of tumor growth or secondary sexual characteristics until the fourth year after the initiation of treatment. This patient is the one of the youngest infants presenting with CPP and HH in Korea; treatment was successful over a relatively long follow-up period. Keywords: Hypothalamic hamartoma, Precocious puberty, Menstruation
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Article abstract Patients with hypothalamic hamartomas and precocious puberty may develop gelastic seizures that are resistant to conventional antiepileptic drug therapies. While treating precocious puberty in two such patients with long-acting GnRH analogue, the authors observed cessation of gelastic seizures. Although the mechanism is unclear, long-acting GnRH analogue should be considered as a possible therapy for gelastic seizures in patients with hypothalamic hamartomas.
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Hypothalamic hamartoma (HH) is a congenital malformation diagnosed based on magnetic resonance imaging (MRI) and histological findings; it is often associated with central precocious puberty (CPP), gelastic seizures, abnormal behavior and mental retardation. In the present paper, we report our retrospective hypothesis that there is a relationship between symptoms and therapy, as well as the treatment for HH, and describe two cases of HH associated with CPP. Both cases had sessile masses located in the interpeduncular cistern, with extension to the hypothalamus on MRI (1.2 ~ 1.5 cm and 2.0 ~ 2.5 cm, respectively). The first case had intractable seizures, while the second had no seizures with paroxysmal discharge. In both patients, the hamartomas were partially removed, by γ-knife and surgical operation in the first case and surgically in the second, and a gonadotropin releasing hormone (GnRH) analogue was prescribed. One case showed improvement of both intelligence quotient (IQ) score and seizures, and the other showed improvements in IQ and abnormal behavior. It was difficult to determine any topology/symptom relationships. Surgery and GnRH analogue treatment can alleviate seizures, abnormal behavior and mental retardation associated with HH.
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