Rare diseases · Sign or symptom
Hypogonadotropic hypogonadism
HP:0000044
What it means
Hypogonadotropic hypogonadism is characterized by reduced function of the gonads (testes in males or ovaries in females) and results from the absence of the gonadal stimulating pituitary hormones: follicle stimulating hormone (FSH) and luteinizing hormone (LH).
Rare diseases that can present with this66
Always100%
2Very common80–99%
20- 3-hydroxyisobutyric aciduria
- 46,XY complete gonadal dysgenesis
- 4H leukodystrophy
- 8p11.2deletion syndrome
- Ataxia-hypogonadism-choroidal dystrophy syndrome
- Biemond syndrome type 2
- Cataract-intellectual disability-hypogonadism syndrome
- Cerebellar ataxia-hypogonadism syndrome
- CHARGE syndrome
- Congenital cataracts-facial dysmorphism-neuropathy syndrome
- Erdheim-Chester disease
- Hypogonadotropic hypogonadism-severe microcephaly-sensorineural hearing loss-dysmorphism syndrome
- Intellectual disability-myopathy-short stature-endocrine defect syndrome
- Isolated follicle stimulating hormone deficiency
- Kallmann syndrome
- Moebius syndrome-axonal neuropathy-hypogonadotropic hypogonadism syndrome
- Noonan syndrome
- Normosmic congenital hypogonadotropic hypogonadism
- Prolactinoma
- Xp21deletion syndrome
Common30–79%
27- Acromegaly
- ANE syndrome
- Aromatase excess syndrome
- Beta-thalassemia
- Brachytelephalangy-dysmorphism-Kallmann syndrome
- Combined pituitary hormone deficiencies, genetic forms
- Craniopharyngioma
- Hyposmia-nasal and ocular hypoplasia-hypogonadotropic hypogonadism syndrome
- Keratoderma hereditarium mutilans
- Meningioma
- Non-acquired panhypopituitarism
- Non-functioning pituitary adenoma
- Polyendocrine-polyneuropathy syndrome
- Prader-Willi syndrome due to maternal uniparental disomy of chromosome 15
- Prader-Willi syndrome due to paternal 15q11q13 deletion
- Prader-Willi syndrome due to paternal deletion of 15q11q13 type 1
- Prader-Willi syndrome due to paternal deletion of 15q11q13 type 2
- Prader-Willi syndrome due to translocation
- Roifman syndrome
- SIM1-related Prader-Willi-like syndrome
- Somatomammotropinoma
- Tremor-ataxia-central hypomyelination syndrome
- TSH-secreting pituitary adenoma
- Wiedemann-Rautenstrauch syndrome
- Wilson-Turner syndrome
- X-linked adrenal hypoplasia congenita
- Xp22.3microdeletion syndrome
Sometimes5–29%
14- Classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency
- Congenital fibrosis of extraocular muscles
- Gorlin syndrome
- Hypothyroidism due to deficient transcription factors involved in pituitary development or function
- Mitochondrial neurogastrointestinal encephalomyopathy
- Moebius syndrome
- Pituicytoma
- Pituitary deficiency due to Rathke cleft cysts
and 6 more in this range
The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions. A sign on this page is a conversation to have with a clinician, not a conclusion.
Part of the broader category
Also called: Hypogonadotrophic hypogonadism · Isolated hypogonadotropic hypogonadism · Low gonadotropins (secondary hypogonadism)
Tracking symptoms like this for someone? Eleplan keeps symptoms, diagnoses, medications and every specialist in one plan.
Start free with EleplanThis page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.