Hypogonadotropic Hypogonadism in Infants with Congenital Hypopituitarism: A Challenge to Diagnose at an Early Stage

Braslavsky, Débora; Grinspon, Romina; Ballerini, Marı́a Gabriela; Bedecarrás, Patricia; Loreti, Nazareth; Bastida, Gabriela; Ropelato, María Gabriela; Keselman, Ana; Campo, Stella; Rey, Rodolfo; Bergadá, Ignacio

doi:10.1159/000439051

Cited by 40 publications

(30 citation statements)

References 26 publications

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“…A maximal GH-stimulated level <6.0 ng/mL (calibrated against WHO 80/505, IMMULITE® 2000 system, Siemens Healthcare Diagnostics Products Ltd., Gwynedd, UK) or <4.7 ng/mL (calibrated against WHO 98/574, IMMULITE® 2000 system, Siemens Healthcare Diagnostics Products Ltd.) after pharmacological test categorized short children with GHD ( n = 31) [13, 14]. Alternatively, during the newborn period, a random GH <10 ng/mL under hypoglycemia identified GHD in neonates when associated with other pituitary deficiencies [15]. Thirty-seven children with GHD were included.…”

Section: Methodsmentioning

confidence: 99%

Circulating IGF-I, IGFBP-3 and the IGF-I/IGFBP-3 Molar Ratio Concentration and Height Outcome in Prepubertal Short Children on rhGH Treatment over Two Years of Therapy

et al. 2017

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Objective: To investigate the occurrence of abnormally elevated values of biomarkers of growth hormone (GH) action in short children on recombinant human GH (rhGH) therapy. Methods: Sixty-three prepubertal short children were examined: 31 with GH deficiency (GHD), 25 small for gestational age (SGA), and 9 with Turner syndrome (TS). The main outcomes were the following: standard deviation score (SDS) values of IGF-I, IGFBP-3, and IGF-I/IGFBP-3 molar ratio before, at the 1st and at the 2nd year on rhGH and Δheight (Ht)-SDS to evaluate GH treatment efficacy (adequate 1st-year ΔHt SDS: >0.4 SDS for GHD and >0.3 SDS for non-GHD). Results: Seventy-eight percent of GHD, 78% of SGA and 55% of TS children had adequate 1st-year ΔHt SDS. In GHD, 88% of IGF-I SDS and IGFBP-3 SDS that were ≤–2.0 SDS at baseline normalized on treatment. Abnormal IGF-I values >+2.0 SDS were observed in 52% of SGA and in 55% of TS patients on rhGH. Within each group, the IGF-I/IGFBP-3 molar ratio increased significantly from pretreatment and throughout therapy, remaining within normal range for most patients. ΔIGF-I/IGFBP-3 molar ratio SDS were significantly higher in children with an adequate response (p < 0.01). Conclusion: Non-GHD groups presented markedly elevated concentrations of GH biomarkers on rhGH and normal IGF-I/IGFBP-3 molar ratio in most patients. Since there is a lack of consensus regarding the molar ratio usefulness, we think that interventions towards a more physiological IGF-I serum profile should be implemented.

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Section: Methodsmentioning

confidence: 99%

Circulating IGF-I, IGFBP-3 and the IGF-I/IGFBP-3 Molar Ratio Concentration and Height Outcome in Prepubertal Short Children on rhGH Treatment over Two Years of Therapy

et al. 2017

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“… 21 The condition can be isolated and it is known as isolated hypogonadotropic hypogonadism, 22 or associated to other pituitary hormone deficiencies. 23 Several gene defects have been described as the cause of gonadotropin insufficiency. The pathogenesis may be related to: 1) altered GnRH neuron formation in the olfactory placode or its migration to the hypothalamus (usually associated with hyposmia/anosmia and known as Kallmann syndrome), 2) abnormal GnRH production due to defects in regulatory factors or in the GnRH1 gene, and 3) defective action in the gonadotroph owing to abnormalities in the GnRH receptor or its transduction pathway.…”

Section: Central Hypogonadism In Boysmentioning

confidence: 99%

“…During the post-natal active period, or mini-puberty, low LH and testosterone have high positive predictive values for central hypogonadism. 23 , 27 – 29 …”

Section: Central Hypogonadism In Boysmentioning

confidence: 99%

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Male Central Hypogonadism in Paediatrics – the Relevance of Follicle-stimulating Hormone and Sertoli Cell Markers

Grinspon

Urrutia

Rey

2018

European Endocrinology

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The definition of male hypogonadism, used in adult endocrinology, is not fully applicable to paediatrics. A clear understanding of the developmental physiology of the hypothalamic-pituitary-testicular axis is essential for the comprehension of the pathogenesis of hypogonadal states in boys and for the establishment of adequate definitions and classifications in paediatric ages. This is particularly true for central hypogonadism, usually called hypogonadotropic in adults. Because childhood is a period characterised by a physiological state of low gonadotropin and testosterone production, these markers of hypogonadism, typically used in adult endocrinology, are uninformative in the child. This review is focused on the physiological importance of prepubertal Sertoli cell markers – anti-Müllerian hormone (AMH) and inhibin B – and of the intratesticular actions of follicle-stimulating hormone (FSH) and testosterone during early infancy and the first stages of pubertal development. We discuss the role of FSH in regulating the proliferation of Sertoli cells – the main determinant of prepubertal testicular volume – and the secretion of AMH and inhibin B. We also address how intratesticular testosterone concentrations have different effects on the seminiferous tubule function in early infancy and during pubertal development.

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“…Temperature instability and prolonged physiological jaundice is present in cases with neonatal TSH deficiency. The development of female genitalia is independent of hormone secretion; hence congenital hypogonadotropic hypogonadism is not expected to affect the normal development of female external genitalia (29). Micropenis is defined according to a -2.5 SD border value from the mean value.…”

Section: Neonatal Clinical Findings In Congenital Hypopituitarism Casesmentioning

confidence: 99%

Neonatal Hypopituitarism: Diagnosis and Treatment Approaches

Kurtoğlu¹,

Özdemır²,

Hatipoğlu³

2018

Jcrpe

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What is already known on this topic?The pituitary gland is the central regulator of growth, metabolism, reproduction and homeostasis. Hypopituitarism is defined as a decreased release of hypophysis hormones, which may be caused by pituitary gland disease or hypothalamus disease. Clinical findings for neonatal hypopituitarism depend on causes and hormonal deficiency type and degree. If early diagnosis is not made, it may cause pituitary hormone deficiencies. What this study adds?We aim to contribute to the literature through a review of etiological factors, clinical findings, diagnoses and treatment approaches for neonatal hypopituitarism. We also aim to increase awareness of neonatal hypopituitarism. We also want to emphasize the importance of early recognition. AbstractHypopituitarism is defined as a decreased release of hypophysis hormones, which may be caused by pituitary gland disease or hypothalamus disease. Clinical findings for neonatal hypopituitarism depend on causes and hormonal deficiency type and degree. Patients may be asymptomatic or may demonstrate non-specific symptoms, but may still be under risk for development of hypophysis hormone deficiency with time. Anamnesis, physical examination, endocrinological, radiological and genetic evaluations are all important for early diagnosis and treatment. Treatment of hypopituitarism patients is possible through treatment of hypophysis and hypothalamus disorders. The aim of this article is to contribute to the literature through a review of etiological factors, clinical findings, diagnoses and treatment approaches for neonatal hypopituitarism.

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Hypogonadotropic Hypogonadism in Infants with Congenital Hypopituitarism: A Challenge to Diagnose at an Early Stage

Cited by 40 publications

References 26 publications

Circulating IGF-I, IGFBP-3 and the IGF-I/IGFBP-3 Molar Ratio Concentration and Height Outcome in Prepubertal Short Children on rhGH Treatment over Two Years of Therapy

Circulating IGF-I, IGFBP-3 and the IGF-I/IGFBP-3 Molar Ratio Concentration and Height Outcome in Prepubertal Short Children on rhGH Treatment over Two Years of Therapy

Male Central Hypogonadism in Paediatrics – the Relevance of Follicle-stimulating Hormone and Sertoli Cell Markers

Neonatal Hypopituitarism: Diagnosis and Treatment Approaches

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