Die olfacto-genitale Dysplasie

Müller, P

doi:10.1159/000303772

Cited by 4 publications

(2 citation statements)

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“…Kallman et al (2) indicated that three of their patients with hypogonadism and anosmia failed to respond clinically to HCG administration, and there is brief mention of similar observations by several other investigators (1,40,41), including a patient with Laurence-Moon-Biedl syndrome (24 Patient 2. D. F. contracted chicken pox, bilateral otitis media, and pneumonia at 6 months of age.…”

Section: Methodsmentioning

confidence: 91%

Studies of the pituitary-Leydig cell axis in young men with hypogonadotropic hypogonadism and hyposmia: comparison with normal men, prepuberal boys, and hypopituitary patients

Bardin¹,

Ross²,

Rifkind³

et al. 1969

J. Clin. Invest.

164

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A B S T R A C T Pituitary and gonadal function was studied in seven chromatin-negative men, ages 15-27 yr, with retarded sexual and somatic development, skeletal anomalies, and hyposnmia. These hyposmic patients were compared with normal men, prepuberal boys and hypogonadal patients with hypopituitarism. The urinary follicle-stimulating hormone (FSH) and luteinizing hormone (LH) levels of hyposmic subjects were the same as those of normal boys and hypopituitary patients but significantly lower than those of normal men. Clomiphene citrate did not cause an increase in plasma FSH and LH levels in either hypogonadal group as it does in normal men. In contrast to hypopituitary patients, thyroid and adrenocortical function and release of growth hormone in the hyposmic subjects were normal. The plasma testosterone levels were equally low in prepuberal, hypopituitary, and hyposmic patients but were increased to a greater extent by human chorionic gonadotropin (HCG) treatment in prepuberal and hypopituitary subjects than in the hyposmic patients. Prolonged treatment with HCG has failed to return plasma testosterone levels to normal in two hyposmic patients. These observations suggest that there are defects of both pituitary and Leydig cell function in men with the syndrome of hypogonadism, skeletal anomalies, and hyposmia. They have impaired secretion of FSH and LH and a Leydig cell insensitivity to gonadotropin.

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Section: Methodsmentioning

confidence: 91%

Studies of the pituitary-Leydig cell axis in young men with hypogonadotropic hypogonadism and hyposmia: comparison with normal men, prepuberal boys, and hypopituitary patients

Bardin¹,

Ross²,

Rifkind³

et al. 1969

J. Clin. Invest.

164

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“…In published reports many patients were diagnosed in the third decade of life or later [Lieblich et al, 1982;Merriam et al, 1977;Naftolin and Harris, 1971;Rogol et al, 1980;Stolp, 1976;Wegenke et al, 1975;Bardin et al, 1969;Boyar, 1969;Schroffner and Furth, 1970;Soules and Hammond, 1980;Turksoy, 1979;Check et al, 1979;Sokol et al, 1981;Wang et al, 1980;Zarate et al, 1973;Males et al, 1973;Sparkes et al, 1968;Tagatz et al, 1970;Mroueh and Kase, 1968;Schaupp and Neubauer, 1973;Hockaday, 1966;Johnson et al, 1983;Jones et al, 1975;Kemman et al, 1980;Muller, 1964;Turner et al, 1974;Santen and Paulsen, 1973a,b;Wortsmann et al, 1982;Yalaburgi, 1982;Swanson et al, 19711. After prolonged prepubescence the patient's compliance for therapy may be reduced [Huffer et al, 1964;Kallmann and Schonfeld, 19431.…”

Section: Introductionmentioning

confidence: 99%

Estimating frequency of Kallmann syndrome among hypogonadic and among anosmic patients

et al. 1987

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The frequency of Kallmann syndrome (hypogonadotropic hypogonadism and anosmia, HHA) was estimated in patients presenting with hypogonadism and patients with anosmia. Of 791 hypogonadal males 19 had HHA. The frequency of HHA was about 1:25 (n = 8/189) in outpatients questioned about their sense of smell, about 1:50 (n = 11/579) in patients whose blood samples were sent to us for chromosome analysis, and about 1:30 (n = 19/605) in males with hypogonadism and 46,XY chromosomes. The relation of patients with HHA to those with Klinefelter syndrome was 1:10 (n = 19/186). From 24 patients presenting with anosmia we found 1 hitherto undiagnosed case of HHA. The mean age at diagnosis was 24.8 and 24.9 years in our cases and cases from literature, respectively. These data provide evidence that Kallmann syndrome is not infrequent and that most patients remain undiagnosed until the third decade of life. Earlier diagnosis is emphasized by questioning each hypogonadal patient about his sense of smell because therapeutic success seems to be age dependent.

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Familial Hypogonadotropic Hypogonadism With Anosmia

Sparkes¹

1968

Arch Intern Med

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Die olfacto-genitale Dysplasie

Cited by 4 publications

References 0 publications

Studies of the pituitary-Leydig cell axis in young men with hypogonadotropic hypogonadism and hyposmia: comparison with normal men, prepuberal boys, and hypopituitary patients

Studies of the pituitary-Leydig cell axis in young men with hypogonadotropic hypogonadism and hyposmia: comparison with normal men, prepuberal boys, and hypopituitary patients

Estimating frequency of Kallmann syndrome among hypogonadic and among anosmic patients

Familial Hypogonadotropic Hypogonadism With Anosmia

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