Autoimmune Polyendocrine Syndromes in the Pediatric Age

Paparella, Roberto; Menghi, Michela; Micangeli, Ginevra; Leonardi, Lucia; Profeta, Giovanni; Tarani, Francesca; Petrella, Carla; Ferraguti, Giampiero; Fiore, M.; Tarani, Luigi

doi:10.3390/children10030588

Cited by 9 publications

(3 citation statements)

References 67 publications

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“…Type 3 stands firstly for not involving adrenal glands, but for having the combination of an autoimmune thyroid disease with another entity such as type 1 diabetes (type 3A), chronic atrophic gastritis, and/or pernicious anemia (type 3B) or vitiligo, alopecia, and myasthenia gravis (type 3C). Type 4 introduces other associations than the mentioned types [ 130 , 131 , 132 ].…”

Section: Discussionmentioning

confidence: 99%

Endocrine Petrified Ear: Associated Endocrine Conditions in Auricular Calcification/Ossification (A Sample-Focused Analysis)

Valea,

Nistor,

Ciobica

et al. 2024

Diagnostics

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Petrified ear (PE), an exceptional entity, stands for the calcification ± ossification of auricular cartilage (CAC/OAC); its pathogenic traits are still an open matter. Endocrine panel represents one of the most important; yet, no standard protocol of assessments is available. Our objective was to highlight most recent PE data and associated endocrine (versus non-endocrine) ailments in terms of presentation, imagery tools, hormonal assessments, biopsy, outcome, pathogenic features. This was a comprehensive review via PubMed search (January 2000–March 2024). A total of 75 PE subjects included: 46 case reports/series (N = 49) and two imagery-based retrospective studies (N = 26) with CAC/OAC prevalence of 7–23% (N = 251) amid routine head/temporal bone CT scans. Endocrine PE (EPE): N = 23, male/female ratio = 10.5; average age = 56.78, ranges: 22–79; non-EPE cohort: N = 26; male/female ratio = 1.88, mean age = 49.44; ranges: 18–75 (+a single pediatric case).The longest post-diagnosis follow-up was of 6–7 years. The diagnosis of PE and endocrine anomalies was synchronous or not (time gap of 10–20 years). A novel case in point (calcified EPE amid autoimmune poly-endocrine syndrome type 2 with a 10-year post-diagnosis documented follow-up) was introduced. We re-analyzed EPE and re-classified another five subjects as such. Hence, the final EPE cohort (N = 50) showed: adrenal insufficiency was the most frequent endocrine condition (36%) followed by hypopituitarism (22%) and hypothyroidism (18%); 39% of the patients with adrenal failure had Addison’s disease; primary type represented 72% of all cases with hypothyroidism; an endocrine autoimmune (any type) component was diagnosed in 18%. We propose the term of “endocrine petrified ear” and a workflow algorithm to assess the potential hormonal/metabolic background in PE.

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Section: Discussionmentioning

confidence: 99%

Endocrine Petrified Ear: Associated Endocrine Conditions in Auricular Calcification/Ossification (A Sample-Focused Analysis)

Valea,

Nistor,

Ciobica

et al. 2024

Diagnostics

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“…Além disso, bebês podem necessitar de suplementação de cloreto de sódio até os 12 meses de idade para garantir a homeostase eletrolítica adequada. Essas diretrizes visam garantir um tratamento seguro e eficaz da PAI durante a infância, promovendo o crescimento e desenvolvimento saudáveis (PAPARELLA et al, 2023).…”

Section: Tratamentounclassified

Endocrinologia e Imunologia: O caminho para compreender as doenças metabólicas, hormonais e autoimunes - Edição IX

Barroso Langoni de Freitas,

De Vasconcelos Eigenheer,

Henrique Carneiro da Cunha Guimarães

et al. 2024

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“…More rare is the occurrence of vaginal discharge, or the development of a sterile abscess at the injection site or at the subcutaneous implant site, which may lead to loss of efficacy of the therapy [3,76]. Weight gain has been documented during therapy in some patients; however, the data available to date indicate that long-term GnRHa treatment does not affect body composition and the onset of obesity or the increased incidence of polycystic ovary syndrome (PCOS) in adulthood [81,82].…”

Section: Treatmentmentioning

confidence: 99%

Clinical Management and Therapy of Precocious Puberty in the Sapienza University Pediatrics Hospital of Rome, Italy

Micangeli,

Paparella,

Tarani

et al. 2023

Children

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Puberty identifies the transition from childhood to adulthood. Precocious puberty is the onset of signs of pubertal development before age eight in girls and before age nine in boys, it has an incidence of 1/5000–1/10,000 with an F:M ratio ranging from 3:1 to 20:1. Precocious puberty can be divided into central, also known as gonadotropin-dependent precocious puberty or true precocious puberty, and peripheral, also recognized as gonadotropin-independent precocious puberty or precocious pseudopuberty. Thus, the main aim of this narrative report is to describe the standard clinical management and therapy of precocious puberty according to the experience and expertise of pediatricians and pediatric endocrinologists at Policlinico Umberto I, Sapienza University of Rome, Italy. In the suspicion of early sexual maturation, it is important to collect information regarding the age of onset, the speed of maturation of secondary sexual features, exposure to exogenous sex steroids and the presence of neurological symptoms. The objective examination, in addition to the evaluation of secondary sexual characteristics, must also include the evaluation of auxological parameters. Initial laboratory investigations should include serum gonadotropin levels (LH and FSH) and serum levels of the sex steroids. Brain MRI should be performed as indicated by the 2009 Consensus Statement in all boys regardless of chronological age and in all girls with onset of pubertal signs before 6 years of age. The gold standard in the treatment of central precocious puberty is represented by GnRH analogs, whereas, as far as peripheral forms are concerned, the triggering cause must be identified and treated. At the moment there are no reliable data establishing the criteria for discontinuation of GnRH analog therapy. However, numerous pieces of evidence suggest that the therapy should be suspended at the physiological age at which puberty occurs.

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Autoimmune Polyendocrine Syndromes in the Pediatric Age

Cited by 9 publications

References 67 publications

Endocrine Petrified Ear: Associated Endocrine Conditions in Auricular Calcification/Ossification (A Sample-Focused Analysis)

Endocrine Petrified Ear: Associated Endocrine Conditions in Auricular Calcification/Ossification (A Sample-Focused Analysis)

Endocrinologia e Imunologia: O caminho para compreender as doenças metabólicas, hormonais e autoimunes - Edição IX

Clinical Management and Therapy of Precocious Puberty in the Sapienza University Pediatrics Hospital of Rome, Italy

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