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Marilena Nakaguma, Fernanda A Correa, Lucas S Santana, Anna F F Benedetti, Ricardo V Perez, Martha K P Huayllas, Mirta B Miras, Mariana F A Funari, Antonio M Lerario, Berenice B Mendonca, Luciani R S Carvalho, Alexander A L Jorge and Ivo J P Arnhold

Introduction Congenital hypopituitarism (CH) is a rare disorder (incidence of 1:3500–10,000 births) defined by the deficiency of one or more pituitary hormones ( 1 , 2 ). Clinical presentation varies, ranging from isolated growth hormone

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Fernanda A Correa, Ericka B Trarbach, Cintia Tusset, Ana Claudia Latronico, Luciana R Montenegro, Luciani R Carvalho, Marcela M Franca, Aline P Otto, Everlayny F Costalonga, Vinicius N Brito, Ana Paula Abreu, Mirian Y Nishi, Alexander A L Jorge, Ivo J P Arnhold, Yisrael Sidis, Nelly Pitteloud and Berenice B Mendonca

cells. Plotted are means± s.e.m . of five independent experiments (*statistically significant ( P <0.05)). Discussion Congenital hypopituitarism (CH) is implicated in considerable morbidity and leads to premature mortality (18) . Although many genetic

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L M Mongioì, R A Condorelli, S La Vignera and A E Calogero

, mean age 41 ± 2.67 years) ( Table 1 ) and 9 patients with AI secondary to hypopituitarism (6 men, 3 women, mean age 53.2 ± 17.7 years) ( Table 2 ). Table 1 Biographical and clinical data of patients with primary adrenal insufficiency

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Luca Persani, Biagio Cangiano and Marco Bonomi

and USA, based on the combination of low TT4 and normal/low TSH ( 5 , 7 ). Despite the possible association with life-threatening adrenal crisis in congenital multiple pituitary hormone defeciencies (MPHDs), CeH is not a direct cause of death. The

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Rachel D C A Diniz, Renata M Souza, Roberto Salvatori, Alex Franca, Elenilde Gomes-Santos, Thiago O Ferrão, Carla R P Oliveira, João A M Santana, Francisco A Pereira, Rita A A Barbosa, Anita H O Souza, Rossana M C Pereira, Alécia A Oliveira-Santos, Allysson M P Silva, Francisco J Santana-Júnior, Eugênia H O Valença, Viviane C Campos and Manuel H Aguiar-Oliveira

congenital, lifetime, untreated IGHD, but with only a modest increase in transaminases, suggesting the lack of advanced forms of NAFLD. This finding contrasts with the association of severe forms of NAFLD in acquired hypopituitarism and weakens the hypothesis

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Elena Galazzi, Paolo Duminuco, Mirella Moro, Fabiana Guizzardi, Nicoletta Marazzi, Alessandro Sartorio, Sabrina Avignone, Marco Bonomi, Luca Persani and Maria Teresa Bonati

factors implicated in anterior pituitary development in the aetiology of congenital hypopituitarism . Annals of Medicine 2006 38 560 – 577 . ( ) 28 Pontecorvi M Goding CR Richardson WD Kessaris N

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Ursula M M Costa, Carla R P Oliveira, Roberto Salvatori, José A S Barreto-Filho, Viviane C Campos, Francielle T Oliveira, Ivina E S Rocha, Joselina L M Oliveira, Wersley A Silva and Manuel H Aguiar-Oliveira

brain tumors) and very recently adrenal crisis (7, 8, 28, 29, 30, 31, 32, 33) have been implicated in the increased mortality risk associated with hypopituitarism (28) . Accordingly, very recent national or multicenter studies have suggested that GHD

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Kunal Thakkar, Swati Ramteke-Jadhav, Rajeev Kasaliwal, Saba Samad Memon, Virendra Patil, Puja Thadani, Nilesh Lomte, Shilpa Sankhe, Atul Goel, Sridhar Epari, Naina Goel, Anurag Lila, Nalini S Shah and Tushar Bandgar

methodological details about imaging technique, hormonal evaluation, and definition of hypopituitarism have been described previously ( 7 ). Results Our series consisted of ten patients, five females and five males. Mean age of patients was 42 (range 16

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Kathrin R Frey, Tina Kienitz, Julia Schulz, Manfred Ventz, Kathrin Zopf and Marcus Quinkler

Introduction In states of adrenal insufficiency (AI), such as primary adrenal insufficiency (PAI) and congenital adrenal hyperplasia (CAH), glucocorticoids (GCs) are given in low doses as hormone replacement therapy. However, the daily intake

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Taísa A R Vicente, Ívina E S Rocha, Roberto Salvatori, Carla R P Oliveira, Rossana M C Pereira, Anita H O Souza, Viviane C Campos, Elenilde G Santos, Rachel D C Araújo Diniz, Eugênia H O Valença, Carlos C Epitácio-Pereira, Mario C P Oliveira, Andrea Mari and Manuel H Aguiar-Oliveira

congenital isolated GHD (IGHD) due to the c.57+1G>A mutation in the GHRH receptor (GHRHR) gene ( GHRHR ), who reside in Itabaianinha county in northeast Brazil (9) . Individuals homozygous for this mutation (MUT/MUT) have very low serum GH and IGF1 levels