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Spermatogenic failure 4(SPGF4)

MedGen UID:
68568
Concept ID:
C0232981
Pathologic Function
Synonyms: Arrest of spermatogenesis; Azoospermia due to Perturbations of Meiosis; AZOOSPERMIA WITH MATURATION ARREST; PREGNANCY LOSS 4; Spermatogenesis arrest
SNOMED CT: Spermatogenic arrest (85716005); Spermatogenesis arrest (85716005); Aspermatogenesis (85716005)
 
Gene (location): SYCP3 (12q23.2)
 
Monarch Initiative: MONDO:0010052
OMIM®: 270960

Definition

Azoospermia, a condition in which there are no sperm present in the ejaculate, has historically been divided into 2 broad categories, obstructive (e.g., 277180) and nonobstructive. Among the genetically based, inherited nonobstructive causes are defects of spermatogenesis, which may interrupt the development of the sperm at various stages, either before (e.g., 415000) or during meiosis. SPGF4 is a form of azoospermia due to perturbations of meiosis. For a discussion of phenotypic and genetic heterogeneity of spermatogenic failure, see SPGF1 (258150). Recurrent Pregnancy Loss Miscarriage, the commonest complication of pregnancy, is the spontaneous loss of a pregnancy before the fetus has reached viability. The term therefore includes all pregnancy losses from the time of conception until 24 weeks' gestation. Recurrent miscarriage, defined as 3 or more consecutive pregnancy losses, affects about 1% of couples; when defined as 2 or more losses, the scale of the problem increases to 5% of all couples trying to conceive (summary by Rai and Regan, 2006). Pregnancy losses have traditionally been designated 'spontaneous abortions' if they occur before 20 weeks' gestation and 'stillbirths' if they occur after 20 weeks. Subtypes of spontaneous abortions can be further distinguished on the basis of embryonic development and include anembryonic loss in the first 5 weeks after conception (so-called 'blighted ovum'), embryonic loss from 6 to 9 weeks' gestation, and fetal loss from 10 weeks' gestation through the remainder of the pregnancy. These distinctions are important because the causes of pregnancy loss vary over gestational ages, with anembryonic losses being more likely to be associated with chromosomal abnormalities, for example. Possible etiologies for recurrent pregnancy loss include uterine anatomic abnormalities, cytogenetic abnormalities in the parents or fetus, single gene disorders, thrombophilic conditions, and immunologic or endocrine factors as well as environmental or infectious agents (summary by Warren and Silver, 2008). For a discussion of genetic heterogeneity of recurrent pregnancy loss, see RPRGL1 (614389). [from OMIM]

Clinical features

From HPO
Azoospermia
MedGen UID:
2150
Concept ID:
C0004509
Disease or Syndrome
Absence of any measurable level of sperm,whereby spermatozoa cannot be observed even after centrifugation of the semen pellet.
Male infertility
MedGen UID:
5796
Concept ID:
C0021364
Disease or Syndrome
The inability of the male to effect fertilization of an ovum after a specified period of unprotected intercourse. Male sterility is permanent infertility.

Professional guidelines

PubMed

Pajarinen J, Savolainen V, Perola M, Penttilä A, Karhunen PJ
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Recent clinical studies

Etiology

Hardy JJ, Wyrwoll MJ, Mcfadden W, Malcher A, Rotte N, Pollock NC, Munyoki S, Veroli MV, Houston BJ, Xavier MJ, Kasak L, Punab M, Laan M, Kliesch S, Schlegel P, Jaffe T, Hwang K, Vukina J, Brieño-Enríquez MA, Orwig K, Yanowitz J, Buszczak M, Veltman JA, Oud M, Nagirnaja L, Olszewska M, O'Bryan MK, Conrad DF, Kurpisz M, Tüttelmann F, Yatsenko AN; GEMINI Consortium
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Halder A, Kumar P, Jain M, Iyer VK
Andrology 2017 May;5(3):460-472. Epub 2017 Feb 19 doi: 10.1111/andr.12330. PMID: 28217965
Zhu XB, Lu JQ, Zhi EL, Zhu Y, Zou SS, Zhu ZJ, Zhang F, Li Z
J Assist Reprod Genet 2016 Aug;33(8):1099-104. Epub 2016 May 27 doi: 10.1007/s10815-016-0738-9. PMID: 27233649Free PMC Article
Honke K
Proc Jpn Acad Ser B Phys Biol Sci 2013;89(4):129-38. doi: 10.2183/pjab.89.129. PMID: 23574804Free PMC Article

Diagnosis

Liu M, Wang L, Li Y, Zhi E, Shen G, Jiang X, Li D, Zhao X, Ruan T, Jiang C, Wang X, Zhang X, Zheng Y, Wu B, Ou N, Zhao G, Dai S, Zhou R, Yang L, Yang Y, Liu H, Shen Y
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Therapy

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Munkelwitz R, Gilbert BR
J Urol 1998 Oct;160(4):1329-33. PMID: 9751347
Pajarinen JT, Karhunen PJ
Int J Androl 1994 Dec;17(6):292-9. doi: 10.1111/j.1365-2605.1994.tb01259.x. PMID: 7744508

Prognosis

Teoli J, Mallet D, Renault L, Gay CL, Labrune E, Bretones P, Giscard D'Estaing S, Cuzin B, Dijoud F, Roucher-Boulez F, Plotton I
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Hum Genet 2021 Aug;140(8):1169-1182. Epub 2021 May 7 doi: 10.1007/s00439-021-02287-y. PMID: 33963445Free PMC Article
Hasani N, Mohseni Meybodi A, Rafaee A, Sadighi Gilani MA, Mohammadzadeh R, Sabbaghian M
Andrologia 2019 Nov;51(10):e13376. Epub 2019 Aug 2 doi: 10.1111/and.13376. PMID: 31373714
Nargund VH
Nat Rev Urol 2015 Jul;12(7):373-82. Epub 2015 Jun 9 doi: 10.1038/nrurol.2015.112. PMID: 26057063
Weedin JW, Bennett RC, Fenig DM, Lamb DJ, Lipshultz LI
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Clinical prediction guides

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Pan B, Yuan S, Mayernik L, Yap YT, Moin K, Chung CS, Maddipati K, Krawetz SA, Zhang Z, Hess RA, Chen X
FASEB J 2023 May;37(5):e22908. doi: 10.1096/fj.202201848R. PMID: 37039784Free PMC Article
Yao C, Zhao L, Tian R, Li P, Zhu Z, Xue Y, Chen H, Gong Y, Liu N, Yang C, He Z, Li Z
Tissue Cell 2019 Jun;58:24-32. Epub 2019 Apr 6 doi: 10.1016/j.tice.2019.04.003. PMID: 31133243
Razavi SM, Sabbaghian M, Jalili M, Divsalar A, Wolkenhauer O, Salehzadeh-Yazdi A
Sci Rep 2017 Nov 17;7(1):15778. doi: 10.1038/s41598-017-16005-0. PMID: 29150651Free PMC Article
Nargund VH
Nat Rev Urol 2015 Jul;12(7):373-82. Epub 2015 Jun 9 doi: 10.1038/nrurol.2015.112. PMID: 26057063

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