Figure - available via license: Creative Commons Attribution-NonCommercial-ShareAlike 3.0 Unported
Content may be subject to copyright.
(a) Plain MRI Brain (T1W image) revealing sessile, well-defined hypothalamic mass is intense to gray matter (b) MRI brain, post contrast, revealing a hyper intense mass lesion suggestive of hypothalamic hamartoma, measuring 1.44 × 1.38 cm
Source publication
Precocious puberty defined as an onset of puberty below eight years in girls and nine years in boys, has an incidence of approximately 1 / 5,000 - 1 / 10,000 subjects with a female / male ratio of 20: 1. It is etiologically classified broadly as central and peripheral. We present to you a case of isosexual (central), precocious puberty in a 16-mont...
Similar publications
Context:
Epidemiological evidence on maternal and paternal heritability of the wide normal variation within pubertal timing is sparse.
Objective:
We aimed to estimate the impact of parental pubertal timing on onset of puberty in boys and girls.
Design:
Annual pubertal examinations of healthy children in a longitudinal cohort study. Information...
Citations
... Pathological conditions in which puberty occurs early are divided into PP, premature adrenarche, McCune-Albright syndrome, and premature thelarche. 10 Menstruation is an important phase which is influenced by the psychological (signs of adulthood, female identity) and physiological factors (normal hypothalamus-pituitary-ovarian axis, basis for fertility and reproduction). 11 Early menarche is menarche which occurs at the age before 10-year old and part of PP. ...
... 17 The incidence of HH has increased with the use of MRI. 10 Kumar et al., mentioned that the HH prevalence on CPP was around 21% -49%. 18 HH without neurological symptoms does not require surgery, thus long-term medical therapy is a must. ...
HIGHLIGHTS 1. Presents a longitudinal case of a child with Organic Central Precocious Puberty (CPP).2. Focused on psychological aspect during the COVID-19 pandemic. ABSTRACT Objective: To present a longitudinal case of a child with organic Central Precocious Puberty (CPP) that focused on medical, growth and development, and parent’s psychological aspect during the COVID-19 pandemic. Case Report: A 14-month old girl attended with major complaints of breasts enlargement and menstruation. The Tanner's stage was at A1M3P1 and the vagina showed reddish-brown spots. The patient’s bone age was advanced (3 years and 6 months). USG examination showed a corpus uterine: cervix ratio of 2:1. GnRH stimulation test showed an elevated of FSH/LH and estradiol. MRI showed an extra-axial dense mass that leads to Hypothalamic Hamartoma (HH). The definitive diagnosis of this patient was organic CPP with HH. The patient was managed with GnRH analog. Precocious puberty (PP) becomes a financial and psychosocial burden for parents. The COVID-19 pandemic adds a double burden for the parents. Conclusion: During the COVID-19 pandemic, parents with PP children had a good psychological aspect if the child was comprehensively handled with adequate motivation and psychoeducation.
... Any girl with precocious physical signs of puberty, significantly advanced BA, decreased predicted height, and a pubertal response to gonadotropin testing should be treated with GnRH agonists to suppress pubertal progression and improve adult height. In case of progressive CPP, treatment with a depot GnRH agonist is suggested and is generally continued for 11 years, even though the best duration of therapy is undecided [3]. ...
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.
... The main manifestation of PP is testicular enlargement before 9 years of age in boys, and breast budding and the onset of menstruation in girls before the age of 8 years. The pathophysiological basis for PP is the early activation of pulsatile gonadotropin-releasing hormone (GnRH) secretion, resulting from hypothalamic or neoplastic lesions (CPP or gonadotropin-dependent PP) [2]. The cause of PPP in most boys is attributed to chorionic gonadotropinrelated tumors and is unknown in girls. ...
Precocious puberty (PP) is defined as the onset of puberty in early childhood, which presents with testicular enlargement before 9 years in boys, and breast budding and the onset of menstruation before 8 years in girls. Hypothalamic hamartoma (HH) is among the most common cause of central PP (CPP). As the pathogenesis of HH has not been elucidated to date, we explored the underlying molecular changes in three HH cases encountered in order to provide clues for clinical treatment. All specimens were reviewed from a pathological standpoint and then checked using next-generation sequencing to elucidate gene mutation profiles. In all three cases with HH, the lysine-specific methyltransferase 2C (KMT2C/MLL3) missense mutations were found. Combined with its close relationship with estrogen-related cancers, we speculate that KMT2C is involved in the pathogenesis of CPP owing to the resultant changes in estrogenic levels and release of gonadotropin-releasing hormone.
... Precocious puberty is estimated to occur in 1/5000 to 1/10,000 children and it is ten times less common in boys than in girls. Reports on precocious puberty amongst Nigerians are scanty [1][2][3][4][5][6]. ...
... The main pathophysiology for central precocious puberty (gonadotrophin-dependent precocious puberty) is early activation of the pulsatile gonadotrophin-releasing hormone secretion from the hypothalamus leading to activation of the hypothalamo-pituitary-gonadal axis and consequent development of secondary sexual characteristics in the affected children [2,6]. The effect of excessive sex hormones, secreted from abnormal gonads or adrenal gland, on target organs are responsible for the physical changes seen in peripheral precocious puberty, also known as gonadotrophin-independent precocious puberty. ...
Precocious puberty is an uncommon cosmopolitan disease, characterized by attainment of secondary sexual characteristics before the age norm for sex. Reports of precocious puberty with attendant hypertension are rare. Detection of the cause of hypertension and precocious puberty which can be central or peripheral is a prerequisite for institution of appropriate management. The outcome of the time, resources and efforts expended in obtaining care for this condition is dependent on the knowledge and expertise of the health care provider. Other determinants of outcome include the understanding of the minor concerned, the parental skills and parental resources. We present an 8 year old Nigerian boy with features of precocious puberty and hypertension. We highlight the not so desirable outcome and challenges associated with previous and current management of this patient. Hopefully, this report will increase awareness on this condition and improve management practices amongst health practitioners in similar settings.
... GnRH analog tedavisi ile sürekli pituiter uyarı, yüksek GnRH seviyeleri, persistan resetör sensitizasyonu, hipotalamo pitüiter gonadal aksı baskılayarak pubertal bulguları durdurmakta, büyüme hızını normale çekip iskelet olgunlaşmasını yavaşlatarak erişkin final boyun korunmasını sağlamaktadır (7). Literatürde HH vaka seri-lerinde GnRH ile etkin medikal tedaviyi bildirilmiştir (4,10). Kotwal ve ark. ...
Hipotalamik hamartom konjenital neoplastik olmayan tümör benzeri bir lezyondur. Santral puberte prekoksun santral sinir sistemi ile ilgili yaygın görülen organik sebeplerindendir. Bu olgu hipotalamik hamartoma bağlı küçük yaşta santral puberte prekoks görülmesi ve beraberinde faktör yedi eksikliğinin eşlik etmesi nedeniyle sunulmuştur.
... The overall incidence of precocious puberty is estimated to range from 1/5000 to 1/10,000 children and it occurs ten times more in girls than in boys. 1,2 The incidence of precocious puberty in Nigeria is not known as there are few reported cases from this part of the world. 3,4 The cause of precocious puberty in girls is unknown in 90% of cases while 8-10% of girls and 25-75% of boys with precocious pu-berty may have structural central nervous system abnormality. ...
... The main pathophysiology for central precocious puberty is early activation of the pulsatile gonadotrophin-releasing hormone secretion from the hypothalamus leading to activation of the hypothalamopituitary-gonadal axis and consequent development of secondary sexual characteristics in the affected children. 2,5 Peripheral (gonadotrophin-independent) precocious puberty can result from disorders of the gonads (tumors), or the adrenals (tumors or congenital adrenal hyperplasia). Another form of precocious puberty, gonadotrophin releasing hormone (GnRH) independent precocious puberty, is caused by disorders like McCune-Albright syndrome and autonomous ovarian cysts. ...
... There was no radiological feature of CNS abnormality or intracranial tumor suggestive of hypothalamic harmatoma which is common in her age group and has been reported by previous authors. 2,4 There was also no periodic fluctuations in the size of the breasts as commonly seen in premature thelache. Premature thelache is often diagnosed in female infants (infantile mammoplasia) or a girl up to the age of three years, who has breast enlargement without other signs of puberty and is growing at a normal pre-pubertal growth velocity. ...
Precocious puberty refers to the appearance of signs of puberty at an earlier age than is considered normal. It occurs ten times more commonly in girls than in boys. The overall incidence ranged from 1/5000 to 1/10,000 children. The cause is idiopathic in 90% of cases of female precocious puberty.
We present BA a 24 month old female toddler who presented with one year history of progressive breast development and 6 month history of pubic hair growth. There was associated increasing weight, height and vaginal secretion. There was no similar occurrence in the family. Mother attained menarche at 14 years of age. Essential finding at presentation revealed a toddler who is heavy and tall for age with a weight of 17kg (>95th percentile for age and sex), height of 90.5cm (90th percentile for age and sex), Occipito-frontal circumference of 49cm (normal). Her sexual maturity rating was Tanner stage 3 for breasts and stage 2 for pubic hair. An assessment of precocious puberty was made. Her investigation result showed an advanced bone age of 5 years; elevated serum gonadotrophins in the pubertal range; and essentially normal cranial CT. Abdomino-pelvic USS showed an enlarged uterus for age, and a dominant right follicle with internal echo measuring 17.1mm X 15.2mm. Parents were counseled on the need for treatment to arrest the progression of precocious puberty but yet to respond because of financial constraint after 2 years of diagnosis.
Female precocious puberty is ten times more common than male precocious puberty. The aetiology is idiopathic in 90% of cases and It is amenable to treatment. Integration of the investigation and treatment of childhood endocrine disorders into the national health insurance scheme will be a great panacea to the challenge of prompt management in developing countries.
KEYWORDS: Precocious, puberty, 24 months old, female, idiopathic, poverty, Nigeria
... Kadınlarda santral puberte prekoks %80 in üzerinde sporadik ve idiopatiktir. Kadın:erkek oranı 20:1 olmasına rağmen erkeklerde altta yatan organik patoloji daha sık temeldir (8,9). Genel olarak NF-1'deki bütün endokrin bozuklukların hipotalamik ve pituiter fonksiyonu etkileyen merkezi sinir sistemi tümörleri ile ilişkili olabileceği düşünülmektedir. ...
... True (central) precocious puberty is linked to several lesions of the CNS that provoke the precocious activation of the hypothalamic-pituitary-gonadal axis (HPG-A) and leads to premature skeletal maturity. [2] About 90% of cases of central precocious puberty (CPP) are idiopathic in girls and only 10% in boys. [1,3,4] Gonadotropin-independent precocious puberty (Peripheral and precocious pseudopuberty) is mainly due to HCG-secreting tumors, congenital adrenal hyperplasia, tumors of the testis, adrenal or ovary that produce sex steroids, accidental or deliberate exogenous sex steroid administration, hypothyroidism, activating mutations of the LH receptor, and GSα subunit. ...
The development of secondary sexual characteristics before the expected age and sex is termed precocious puberty. It is usually due to excessive sex steroids production. Cases of precocious puberty are uncommon worldwide with a prevalence of 1/5000 - 1/10000 per children population and female to male ratio of 10:1. In Nigeria, the prevalence is unknown with only a few reported cases, probably due to superstitious and religious beliefs. A high proportion of reported cases globally are of the gonadotropin - dependent (central) type. A case is being reported of a 30-month old female non-identical twin in Cross River State, South-South of Nigeria, who presented with a 6-month history of bleeding per vagina, premature breast development and pubic hair distribution (Tanner stage 3). The patient had appropriate serum concentrations of pituitary gonadotropins and gonadal hormones for age. Precorcious puberty was not a finding in the family history, and twin and other siblings had normal growth pattern. Radiological and endocrine investigations were diagnostic of precocious puberty. Hence being proactive with a high index of suspicion is required of clinicians to be able to identify endocrine abnormalities that are increasing in prevalence and are going unnoticed in our environment among the paediatric age group.
... CPP constitutes the premature activation of the hypothalamic-pituitary axis (HPA), either in a familial pattern, in the setting of positive family history for precocious puberty, as a result of central nervous system (CNS) pathology (hydrocephalus, meningitis, encephalitis, hypothalamic tumours etc.), or in an idiopathic manner should the above-mentioned aetiologies be ruled out. Elevated levels of basal gonadotropins as a result of hypothalamic stimulation is indicative of the integrity of the HPA, which is why the GnRH stimulation test remains the gold standard test for diagnosing CPP [7] . It is construed by interpreting pubertal FSH and LH levels in response to the administration of a synthetic GnRH. ...
Precocious puberty (PP) is the development of pubertal changes at a pathologically earlier age than the mean age for the general population. It may either occur as a result of premature activation of the Hypothalamic-Pituitary-Axis or as a result of peripheral stimulation. PP is now recognised to occur more frequently in the preschool population, and therefore we report these cases aiming to develop a better understanding of the responsible causation and underlying factors.
... In majority (90%) of cases of precocious puberty the specific cause is not present and no active intervention is needed. [3,4]Our patient probably falls into this category. In the index case, enlarged protruding hymen was noted along-with appearance of pubic hair. ...
