Kallmann"s syndrome - significado y definición. Qué es Kallmann"s syndrome
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Qué (quién) es Kallmann"s syndrome - definición

A FORM OF HYPOGONADOTROPIC HYPOGONADISM WHICH IS ALSO ACCOMPANIED BY A TOTAL LACK OR PARTIAL LOSS IN THE SENSE OF SMELL. THE CONDITION AFFECTS BOTH MALES AND FEMALES AND CAUSES A FAILURE IN PUBERTY AND INFERTILITY.
Kallmann's syndrome; Kallman's Syndrome; Morsier-Gauthier Syndrome; Maestre-Kallmann-Morsier syndrome; Kallmann's Syndrome; Kallman syndrome; Kallmann Syndrome
  • 19 year old with Kallmann syndrome before diagnosis and treatment
  • Shows the effect of the interruption of GnRH hormone release from the hypothalamus and the subsequent inability of the testes and ovaries to function correctly at puberty as seen in cases of KS/HH. In most cases of KS/HH the testes and ovaries are able to function correctly, but fail to do so because they have not had the correct hormonal signals.
  • Shows the normal hormonal control of puberty from the hypothalamus down to the testes or ovaries and their negative feedback mechanisms. The negative feedback control allows just the right amount of hormone to be released according to the needs of the body at that time.
  • Franz J. Kallmann]], circa 1950
  • 1YY1}})
  • Testosterone gel sachets, Testosterone undecanoate injection (Nebido), Human chorionic gonadotropin (hCG) injection, Menotropin injection (hMG).
  • Tanner scale-female
  • The genetic and molecular basis of idiopathic hypogonadotropic hypogonadism

Kallmann syndrome         
Kallmann syndrome (KS) is a genetic disorder that prevents a person from starting or fully completing puberty. Kallmann syndrome is a form of a group of conditions termed hypogonadotropic hypogonadism.
Reye's syndrome         
SYNDROME CHARACTERIZED BY ACUTE BRAIN DAMAGE AND LIVER FUNCTION PROBLEMS
Reyes Syndrome; Reye Syndrome; Reye's Syndrome; Reyes syndrome; Reye’s Syndrome; Reye hepatocerebral syndrome; Rye syndrome; Reye s syndrome; Reye's s syndrome; Reye's syndrome; Reye’s syndrome
['re?z, 'r??z]
¦ noun a life-threatening metabolic disorder in young children, of uncertain cause.
Origin
1960s: named after the Australian paediatrician Ralph D. K. Reye.
Stewart–Treves syndrome         
HUMAN DISEASE
Stewart-treves syndrome; Stewart–Treves Syndrome; Postmastectomy lymphangiosarcoma; Stewart Treves syndrome; Cutaneous angiosarcoma; Stewart-Treves syndrome; Stewart-Treves Syndrome
Stewart–Treves syndrome refers to a lymphangiosarcoma, a rare disorder marked by the presence of an angiosarcoma (a malignant tumor of blood or lymph vessels) in a person with chronic (long-term) lymphedema. Although it most commonly refers to malignancies associated with chronic lymphedema resulting from mastectomy and/or radiotherapy for breast cancer, it may also describe lymphangiosarcomas that result from congenital and other causes of chronic secondary lymphedema.

Wikipedia

Kallmann syndrome

Kallmann syndrome (KS) is a genetic disorder that prevents a person from starting or fully completing puberty. Kallmann syndrome is a form of a group of conditions termed hypogonadotropic hypogonadism. To distinguish it from other forms of hypogonadotropic hypogonadism, Kallmann syndrome has the additional symptom of a total lack of sense of smell (anosmia) or a reduced sense of smell. If left untreated, people will have poorly defined secondary sexual characteristics, show signs of hypogonadism, almost invariably are infertile and are at increased risk of developing osteoporosis. A range of other physical symptoms affecting the face, hands and skeletal system can also occur.

The underlying cause is a failure in the correct production or activity of gonadotropin-releasing hormone by the hypothalamus. This results in low levels of the sex hormones testosterone in males or oestrogen and progesterone in females. Diagnosis normally occurs during teenage years when puberty fails to start.

Lifelong treatment for both sexes is normally required. Hormone replacement therapy (HRT) is the major form of treatment with the aim to replace the missing testosterone or oestrogen and progesterone. Specialised fertility treatments are also available.

The condition is more commonly diagnosed in males than in females. A 2011 study of the Finnish population produced an estimated incidence of 1 in 48,000 people overall, with 1 in 30,000 for males and 1 in 125,000 for females. Kallmann syndrome was first described by name in a paper published in 1944 by Franz Josef Kallmann, a German-American geneticist. The link between anosmia and hypogonadism had already been noted by Spanish doctor Aureliano Maestre de San Juan in 1856.