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* small tests
  medupch - 11/18/06 13:10
  15 yo pt, with small testes, and no male secondary characteristcs, he is tall, and also he has some troubble with smelling ...dx is?  
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* Re:small tests
  progesterone - 11/18/06 13:16
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* Re:small tests
  progesterone - 11/18/06 13:17
  Sorry, is Kallman's Syndrome  
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* Re:small tests
  cd45 - 11/18/06 13:31
  wowwww progesterone..that was excellent.. i would have overlooked the smelling bit
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* Re:small tests
  progesterone - 11/18/06 16:09
  Thanks CD45

Kallmann syndrome is characterized by:

Hypogonadotropic hypogonadism (a lack of the pituitary hormones LH and FSH)
Congenital (present from birth) anosmia (complete inability to smell) or hyposmia (decreased ability to smell) this is present in 80% of the patients and establishes the diagnosis of the syndrome in patients with isolated gonadotropin deficiency.
It can occasionally be associated with optic problems, such as colour blindness or optic atrophy, nerve deafness, cleft palate, cryptorchidism, renal agenesis, and mirror movement disorder.
Males present with delayed puberty and may have micropenis (although congenital micropenis is not present in the majority of male KS cases).

Females present with delayed puberty (i.e. primary amenorrhea) and lack of secondary sex characteristics, such as breast development.

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* Re:small tests
  progesterone - 11/18/06 16:19
Other names for this syndrome and; Why is it called Kallmann’s syndrome?

Franz Kallmann was an American scientist who published a paper in 1944 about the cases of 3 families who all had members who failed to enter puberty and had no sense of smell. He was the first person to propose that this was a genetic condition and it was named after him.

The first mention of the condition came in 1856 when a paper was published by Aureliano Maestre de San Juan highlighted an autopsy report on a patient with undeveloped sex organs and absent olfactory lobes (the part of the brain concerned with sense of smell). In Spanish speaking parts of the world the condition is still called after him.

In 1954 de Morsier published a paper on 14 cases of anosmia (lack of sense of smell) and absent puberty and proved the neuro-pathological basis of the condition.

This has led to a number of different names being given to the same syndrome, depending on what part of the world you are in:

de Morsier’s Syndrome II;

Morsier-Gauthier Syndrome;

Kallmann-de Morsier Syndrome;

Maestre-Kallmann-de Morsier Syndrome;

Maestre de San Juan-Kallmann Syndrome;

Maestre de San Juan-Kallmann-de Morsier Syndrome;
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* Re:small tests
  medupch - 11/19/06 11:06
  good job guys.
take care
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