Normal Insulin-Like Peptide-3 Levels Despite Low Testosterone in Adult Males with Prader-Willi Syndrome: Variations in Leydig Cell Function from Infancy through Adulthood
Background: Cryptorchidism, incomplete pubertal development, and low testosterone are manifestations of hypogonadism in Prader-Willi syndrome (PWS). Insulin-like peptide-3 (INSL3) facilitates testicular descent in the fetus and reflects Leydig cell number in adults. INSL3 levels in PWS have not been...
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Published in | The journal of clinical endocrinology and metabolism Vol. 98; no. 1; pp. E135 - E143 |
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Main Authors | , , , , , |
Format | Journal Article |
Language | English |
Published |
United States
Endocrine Society
01.01.2013
Copyright by The Endocrine Society |
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Abstract | Background:
Cryptorchidism, incomplete pubertal development, and low testosterone are manifestations of hypogonadism in Prader-Willi syndrome (PWS). Insulin-like peptide-3 (INSL3) facilitates testicular descent in the fetus and reflects Leydig cell number in adults. INSL3 levels in PWS have not been previously reported.
Objectives:
The objectives of the study were to characterize the age-related changes in INSL3 in PWS males and correlate INSL3 with unilateral vs. bilateral cryptorchidism, body mass index, gonadotropins, testosterone, anti-Mullerian hormone (AMH), and inhibin B.
Study Design and Participants:
We measured INSL3, LH, FSH, testosterone, AMH, and inhibin B in 40 PWS males (23 deletion, 17 uniparental disomy) aged 2 months to 36 yr. Control samples for INSL3 were obtained from 365 normal males, aged 1 d to 36 yr.
Results:
INSL3 levels (mean and range) for PWS age groups younger than 6 months, 0.5–10.0 yr, 10.1–19.0 yr, and older than 19.0 yr were 217 (68–380), 42 (16–112), 390 (16–1028), and 642 (290–964) pg/ml, respectively, and did not differ significantly from values for normal males. In seven of 14 boys aged 10.1–19 yr, INSL3, testosterone, and LH were low (37.4 ± 19.4 pg/ml, 1.44 ± 0.46 nmol/liter, 0.3 ± 0.6 IU/liter). The other seven with higher INSL3, testosterone, and LH (693.1 ± 305.8 pg/ml, 5.91 ± 2.77 nmol/liter, 2.7 ±1.9 IU/liter) had more advanced pubertal development. INSL3 was normal in seven of nine males aged older than 19 yr, despite low testosterone in six. After controlling for age, INSL3 correlated with LH (P = 0.005) and testosterone (P < 0.001) but not with FSH, AMH, or inhibin B.
Conclusions:
Most PWS males have normal INSL3 levels. By contrast, testosterone levels after infancy are low. These findings suggest a specific defect in Leydig cell function. |
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AbstractList | BACKGROUND:Cryptorchidism, incomplete pubertal development, and low testosterone are manifestations of hypogonadism in Prader-Willi syndrome (PWS). Insulin-like peptide-3 (INSL3) facilitates testicular descent in the fetus and reflects Leydig cell number in adults. INSL3 levels in PWS have not been previously reported.
OBJECTIVES:The objectives of the study were to characterize the age-related changes in INSL3 in PWS males and correlate INSL3 with unilateral vs. bilateral cryptorchidism, body mass index, gonadotropins, testosterone, anti-Mullerian hormone (AMH), and inhibin B.
STUDY DESIGN AND PARTICIPANTS:We measured INSL3, LH, FSH, testosterone, AMH, and inhibin B in 40 PWS males (23 deletion, 17 uniparental disomy) aged 2 months to 36 yr. Control samples for INSL3 were obtained from 365 normal males, aged 1 d to 36 yr.
RESULTS:INSL3 levels (mean and range) for PWS age groups younger than 6 months, 0.5–10.0 yr, 10.1–19.0 yr, and older than 19.0 yr were 217 (68–380), 42 (16–112), 390 (16–1028), and 642 (290–964) pg/ml, respectively, and did not differ significantly from values for normal males. In seven of 14 boys aged 10.1–19 yr, INSL3, testosterone, and LH were low (37.4 ± 19.4 pg/ml, 1.44 ± 0.46 nmol/liter, 0.3 ± 0.6 IU/liter). The other seven with higher INSL3, testosterone, and LH (693.1 ± 305.8 pg/ml, 5.91 ± 2.77 nmol/liter, 2.7 ±1.9 IU/liter) had more advanced pubertal development. INSL3 was normal in seven of nine males aged older than 19 yr, despite low testosterone in six. After controlling for age, INSL3 correlated with LH (P = 0.005) and testosterone (P < 0.001) but not with FSH, AMH, or inhibin B.
CONCLUSIONS:Most PWS males have normal INSL3 levels. By contrast, testosterone levels after infancy are low. These findings suggest a specific defect in Leydig cell function. Cryptorchidism, incomplete pubertal development, and low testosterone are manifestations of hypogonadism in Prader-Willi syndrome (PWS). Insulin-like peptide-3 (INSL3) facilitates testicular descent in the fetus and reflects Leydig cell number in adults. INSL3 levels in PWS have not been previously reported. The objectives of the study were to characterize the age-related changes in INSL3 in PWS males and correlate INSL3 with unilateral vs. bilateral cryptorchidism, body mass index, gonadotropins, testosterone, anti-mullerian hormone (AMH), and inhibin B. We measured INSL3, LH, FSH, testosterone, AMH, and inhibin B in 40 PWS males (23 deletion, 17 uniparental disomy) aged 2 months to 36 yr. Control samples for INSL3 were obtained from 365 normal males, aged 1 d to 36 yr. INSL3 levels (mean and range) for PWS age groups younger than 6 months, 0.5-10.0 yr, 10.1-19.0 yr, and older than 19.0 yr were 217 (68-380), 42 (16-112), 390 (16-1028), and 642 (290-964) pg/ml, respectively, and did not differ significantly from values for normal males. In seven of 14 boys aged 10.1-19 yr, INSL3, testosterone, and LH were low (37.4 ± 19.4 pg/ml, 1.44 ± 0.46 nmol/liter, 0.3 ± 0.6 IU/liter). The other seven with higher INSL3, testosterone, and LH (693.1 ± 305.8 pg/ml, 5.91 ± 2.77 nmol/liter, 2.7 ±1.9 IU/liter) had more advanced pubertal development. INSL3 was normal in seven of nine males aged older than 19 yr, despite low testosterone in six. After controlling for age, INSL3 correlated with LH (P = 0.005) and testosterone (P < 0.001) but not with FSH, AMH, or inhibin B. Most PWS males have normal INSL3 levels. By contrast, testosterone levels after infancy are low. These findings suggest a specific defect in Leydig cell function. BACKGROUNDCryptorchidism, incomplete pubertal development, and low testosterone are manifestations of hypogonadism in Prader-Willi syndrome (PWS). Insulin-like peptide-3 (INSL3) facilitates testicular descent in the fetus and reflects Leydig cell number in adults. INSL3 levels in PWS have not been previously reported.OBJECTIVESThe objectives of the study were to characterize the age-related changes in INSL3 in PWS males and correlate INSL3 with unilateral vs. bilateral cryptorchidism, body mass index, gonadotropins, testosterone, anti-mullerian hormone (AMH), and inhibin B.STUDY DESIGN AND PARTICIPANTSWe measured INSL3, LH, FSH, testosterone, AMH, and inhibin B in 40 PWS males (23 deletion, 17 uniparental disomy) aged 2 months to 36 yr. Control samples for INSL3 were obtained from 365 normal males, aged 1 d to 36 yr.RESULTSINSL3 levels (mean and range) for PWS age groups younger than 6 months, 0.5-10.0 yr, 10.1-19.0 yr, and older than 19.0 yr were 217 (68-380), 42 (16-112), 390 (16-1028), and 642 (290-964) pg/ml, respectively, and did not differ significantly from values for normal males. In seven of 14 boys aged 10.1-19 yr, INSL3, testosterone, and LH were low (37.4 ± 19.4 pg/ml, 1.44 ± 0.46 nmol/liter, 0.3 ± 0.6 IU/liter). The other seven with higher INSL3, testosterone, and LH (693.1 ± 305.8 pg/ml, 5.91 ± 2.77 nmol/liter, 2.7 ±1.9 IU/liter) had more advanced pubertal development. INSL3 was normal in seven of nine males aged older than 19 yr, despite low testosterone in six. After controlling for age, INSL3 correlated with LH (P = 0.005) and testosterone (P < 0.001) but not with FSH, AMH, or inhibin B.CONCLUSIONSMost PWS males have normal INSL3 levels. By contrast, testosterone levels after infancy are low. These findings suggest a specific defect in Leydig cell function. Background: Cryptorchidism, incomplete pubertal development, and low testosterone are manifestations of hypogonadism in Prader-Willi syndrome (PWS). Insulin-like peptide-3 (INSL3) facilitates testicular descent in the fetus and reflects Leydig cell number in adults. INSL3 levels in PWS have not been previously reported. Objectives: The objectives of the study were to characterize the age-related changes in INSL3 in PWS males and correlate INSL3 with unilateral vs. bilateral cryptorchidism, body mass index, gonadotropins, testosterone, anti-Mullerian hormone (AMH), and inhibin B. Study Design and Participants: We measured INSL3, LH, FSH, testosterone, AMH, and inhibin B in 40 PWS males (23 deletion, 17 uniparental disomy) aged 2 months to 36 yr. Control samples for INSL3 were obtained from 365 normal males, aged 1 d to 36 yr. Results: INSL3 levels (mean and range) for PWS age groups younger than 6 months, 0.5–10.0 yr, 10.1–19.0 yr, and older than 19.0 yr were 217 (68–380), 42 (16–112), 390 (16–1028), and 642 (290–964) pg/ml, respectively, and did not differ significantly from values for normal males. In seven of 14 boys aged 10.1–19 yr, INSL3, testosterone, and LH were low (37.4 ± 19.4 pg/ml, 1.44 ± 0.46 nmol/liter, 0.3 ± 0.6 IU/liter). The other seven with higher INSL3, testosterone, and LH (693.1 ± 305.8 pg/ml, 5.91 ± 2.77 nmol/liter, 2.7 ±1.9 IU/liter) had more advanced pubertal development. INSL3 was normal in seven of nine males aged older than 19 yr, despite low testosterone in six. After controlling for age, INSL3 correlated with LH (P = 0.005) and testosterone (P < 0.001) but not with FSH, AMH, or inhibin B. Conclusions: Most PWS males have normal INSL3 levels. By contrast, testosterone levels after infancy are low. These findings suggest a specific defect in Leydig cell function. |
Author | Eldar-Geva, Talia Hirsch, Harry J Gross-Tsur, Varda Roger, Marc Lahlou, Najiba Benarroch, Fortu |
AuthorAffiliation | Neuropediatric Unit (H.J.H., V.G.-T.), Department of Pediatrics, and Reproductive Endocrinology and Genetics Unit (T. E.-G.), Department of Obstetrics and Gynecology, Shaare Zedek Medical Center, the Hebrew University, Jerusalem 91031, Israel; Herman Dana Division of Child and Adolescent Psychiatry (F.B.), Hadassah Mount Scopus Hospital, Jerusalem 91120, Israel; and Department of Hormone Biology and Metabolic Disorders (N.L.) and Institute for Endocrine and Metabolic Research (N.L., M.R.), Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris-Descartes University, 75014 Paris, France |
AuthorAffiliation_xml | – name: Neuropediatric Unit (H.J.H., V.G.-T.), Department of Pediatrics, and Reproductive Endocrinology and Genetics Unit (T. E.-G.), Department of Obstetrics and Gynecology, Shaare Zedek Medical Center, the Hebrew University, Jerusalem 91031, Israel; Herman Dana Division of Child and Adolescent Psychiatry (F.B.), Hadassah Mount Scopus Hospital, Jerusalem 91120, Israel; and Department of Hormone Biology and Metabolic Disorders (N.L.) and Institute for Endocrine and Metabolic Research (N.L., M.R.), Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris-Descartes University, 75014 Paris, France |
Author_xml | – sequence: 1 givenname: Harry J surname: Hirsch fullname: Hirsch, Harry J email: hirschmd@gmail.com – sequence: 2 givenname: Talia surname: Eldar-Geva fullname: Eldar-Geva, Talia – sequence: 3 givenname: Varda surname: Gross-Tsur fullname: Gross-Tsur, Varda – sequence: 4 givenname: Fortu surname: Benarroch fullname: Benarroch, Fortu – sequence: 5 givenname: Marc surname: Roger fullname: Roger, Marc – sequence: 6 givenname: Najiba surname: Lahlou fullname: Lahlou, Najiba |
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Cryptorchidism, incomplete pubertal development, and low testosterone are manifestations of hypogonadism in Prader-Willi syndrome (PWS).... BACKGROUND:Cryptorchidism, incomplete pubertal development, and low testosterone are manifestations of hypogonadism in Prader-Willi syndrome (PWS).... Cryptorchidism, incomplete pubertal development, and low testosterone are manifestations of hypogonadism in Prader-Willi syndrome (PWS). Insulin-like peptide-3... BACKGROUNDCryptorchidism, incomplete pubertal development, and low testosterone are manifestations of hypogonadism in Prader-Willi syndrome (PWS). Insulin-like... |
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SubjectTerms | Adolescent Adult Aging - blood Aging - physiology Case-Control Studies Child Child, Preschool Humans Infant Insulin - blood Insulin - metabolism Leydig Cells - metabolism Leydig Cells - physiology Male Prader-Willi Syndrome - blood Prader-Willi Syndrome - physiopathology Proteins - metabolism Testosterone - blood Young Adult |
Title | Normal Insulin-Like Peptide-3 Levels Despite Low Testosterone in Adult Males with Prader-Willi Syndrome: Variations in Leydig Cell Function from Infancy through Adulthood |
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