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Central Precocious Puberty Type 2

CPP


Presentation

  • The BMI of the untreated group was normal at presentation (percentile 44.7), remained so throughout adolescence and was equivalent to that of controls in adulthood.[news-medical.net]
  • Keywords Septo-optic dysplasia (SOD); Pituitary; Growth hormone deficiency; Precocious puberty Case Presentation The patient is a Japanese girl, currently 10.6 years old.[omicsonline.org]
  • No major signs of Prader Willi syndrome were detected in any patient with CPP in the present study.[academic.oup.com]
  • Although most girls with CAH present with ambiguous genitalia in infancy, occasionally this is overlooked, and these children present with virilizing changes in early childhood.[glowm.com]
  • None of these patients had neurological abnormalities at initial presentation.[adc.bmj.com]
Noncompliance
  • The two other patients were noncompliant with treatment ( Supplemental Data ).[academic.oup.com]
Nausea
  • GnRHa are generally well tolerated, and their side effects are infrequent and include local allergic reactions (5%-10% of the cases), headache, abdominal pain, vaginal bleeding after the first GnRHa dose, nausea, vasomotor symptoms due to hypoestrogenism[scielo.br]
High Arched Palate
  • Patient 6 had mild nonspecific syndromic features, such as high-arched palate, dental abnormalities, clinodactyly, and hyperlordosis. The age of pubertal onset in these girls ranged from 3.0 to 6.4 years (mean 5.3 y and median 6.0 y).[academic.oup.com]
Advanced Bone Age
  • You need HPA activation, which includes elevated LH/FSH from regular pulses of GnRH to enter true puberty, resulting in testicular enlargement and advanced bone age.[hippoed.com]
  • The youngest case (patient 4) was a 3-year-old girl with Tanner stage 3 breast development and advanced bone age.[academic.oup.com]
  • Precocious puberty associated with hypothalamic hamartomas usually presents before 4 years of age and is associated with accelerated linear growth for age, advanced bone age, and pubertal levels of FSH, LH, and estradiol (in girls) and testosterone (in[hopeforhh.org]
  • Girls with premature thelarche typically display breast development during the first 2 years of life, but this change is not accompanied by pubertal hormone levels, menarche, advanced bone age on x-ray, androgen effects, or growth acceleration.[merckmanuals.com]
  • GDPP is idiopathic in 80% of cases, occurs 10 to 20 times more often in girls, and demonstrates isosexual characteristics.3 In addition, you will see accelerated linear growth for age, advanced bone ages, and pubertal levels of follicle stimulating hormone[ndnr.com]
Long Arm
  • Interestingly, genes located on the long arm of chromosome 15 have been involved in human pubertal development based on clinical and genetic studies ( 32 – 34 ).[academic.oup.com]
Vaginal Discharge
  • Other signs suggestive of pubertal onset include acne, growth acceleration, voice changes, vaginal discharge or bleeding, and advanced skeletal maturation.[rarediseases.org]

Workup

  • When should you start a workup? What should you order and when do you refer to endocrinology? We discuss these things and more with our pediatric endocrine consultant.[hippoed.com]
  • The current study demonstrates the power of a detailed family history in informing the diagnostic workup of male CPP patients.[journals.plos.org]

Treatment

  • Without treatment, your child may end up shorter than they could have been. With treatment, they're more likely to reach their full adult height. Within a year, their growth should slow to a normal rate.[webmd.com]
  • The sooner you know for sure, the sooner your child can get the treatment he or she needs. Thank you![pubertytoosoon.com]
  • But they suggest that, for these women, their increased BMI “might largely reflect the persistence of childhood obesity into adulthood”, rather than being a direct consequence of CPP or its treatment.[news-medical.net]
  • Elzonris is the first approved treatment for blastic plasmacytoid dendritic cell neoplasm and first approved CD123-targeted therapy.[pharmpro.com]

Prognosis

  • What is the prognosis of central precocious puberty? Similar to the treatment of central precocious puberty, the prognosis is related to the precise cause of the child's disease.[medicinenet.com]
  • Recent studies have shown some indication for brain imaging studies if (1) onset is between 7-8 years of age, and (2) lack of pubic hair at the time of diagnosis Prognosis : Most patients undergo puberty appropriately.[pedclerk.bsd.uchicago.edu]
  • Prognosis CPP can have a psychological effect during childhood. The disease has minimal consequence during adulthood, although the association of variation of pubertal timing with adult disease or behavior has been questioned.[orpha.net]
  • There are several drugs that have been developed to do this: histrelin (Supprelin) nafarelin (Synarel) synthetic gonadotropin-releasing hormone agonist deslorelin ethylamide triptorelin leuprolide Prognosis Drug treatments can slow growth to 2–3 in (5[healthofchildren.com]

Etiology

  • […] of peripheral precocious puberty in boys - Gonadotropin-secreting tumors (hepatomas, teratomas, choriocarcinomas of mediastinum) - Autonomous androgen secretion d/t inborn errors of adrenal enzyme functions Etiology of peripheral precocious puberty in[quizlet.com]
  • CENTRAL PRECOCIOUS PUBERTY Etiology The main etiologies of CPP are listed in Table 1 ( 10 - 15 ). Idiopathic CPP represents 90% of the cases in girls, whereas organic etiologies are more frequent (60% to 70%) in boys.[scielo.br]
  • Argente Localización: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría ( AEP ) , ISSN 1695-4033, ISSN-e 1696-4608, Vol. 74, Nº. 5, 2011 , pág. 336 Idioma: español Títulos paralelos: Central precocious puberty: epidemiology, etiology[dialnet.unirioja.es]
  • Etiology CPP is due to premature activation of the hypothalamic-pituitary-gonadal (HPG) axis.[orpha.net]

Epidemiology

  • Pathogenesis and epidemiology of precocious puberty. Effects of exogenous oestrogens. Hum Reprod Update. 2001;7(3):292-302. 3. Mayo Clinic Staff. Precocious puberty: symptoms and causes.[pubertytoosoon.com]
  • , Jesús Argente Localización: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría ( AEP ) , ISSN 1695-4033, ISSN-e 1696-4608, Vol. 74, Nº. 5, 2011 , pág. 336 Idioma: español Títulos paralelos: Central precocious puberty: epidemiology[dialnet.unirioja.es]
  • Summary Epidemiology The prevalence of CPP has been evaluated at approximately 1/ 500 girls and 1/ 2,000 boys in Denmark. Girls are more affected than boys, with an overall male to female ratio of at least 1:10.[orpha.net]
  • In 1997, an epidemiologic study reported that 6.7% of American girls had clinical evidence of puberty at age 7 years, and 14.7% at age 8 years. That study described the youngest ever reported population age at puberty onset of 9.96 1.82 years.[frontiersin.org]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology : GnRH secretion is activated early, either because of tumor (central or peripheral), but more commonly due to unexplained causes.[pedclerk.bsd.uchicago.edu]
  • Pathophysiology of the neuroregulation of growth hormone secretion in experimental animals and the human. Endocr. Rev. 19, 717–797 (1998). 69. Harris, D. A. et al.[nature.com]

Prevention

  • Preventing body odor. WebMD website. . Updated July 15, 2017. Accessed March 21, 2018. 11. American Academy of Pediatrics. Physical development in boys: what to expect. HealthyChildren.org website. . Updated November 21, 2015.[pubertytoosoon.com]
  • In some cases, doctors can use a drug to block sex hormones and prevent further development. A man-made version of GnRH stops the pituitary gland from sending out gonadotropins.[webmd.com]
  • Treatments exist to help prevent the onset of puberty, as well as other medications that prevent individuals from sexually developing.[diseaseinfosearch.org]
  • According to the review, treating precocious puberty should halt or regress secondary sexual characteristics, prevent early menarche, slow skeletal maturation and improve adult height and prevent psychological problems.[healio.com]
  • Can central precocious puberty be prevented or avoided? You cannot prevent CPP. Children who are at greater risk for CPP are: genetically female overweight or obese.[familydoctor.org]

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