Hipertensión arterial maligna e hiperaldosteronismo hiperreninémico: reporte de un caso / Malignant arterial hypertension and hyperreninemic hyperaldosteronism: a case report

Se presenta el caso de una mujer de 28 años de edad, hospitalizada por hipertensión arterial severa, edema pulmonar e hipokalemia. se encontró hiperaldosteronismo hiperreninémico e imagen de una lesión nodular en la glándula suprarrenal izquierda, la cual fue extirpada mediante cirugía laparoscópica. El estudio histológico demostró hiperplasia macronodular cortical y en el postoperatorio, hubo retorno progresivo de la hipertensión e hipokalemia

Assessing adrenal function in primary care settings with a single sample subcutaneous glucagon test.

OBJECTIVE: To test the efficacy of the low-dose glucagon test in assessing adrenal gland function. STUDY DESIGN: Subcutaneous glucagon was used to assess the hypothalamo-pituitary-adrenal gland (HPA) axis in 215 healthy children. Concordance of this test with the low-dose intravenous ACTH test was established for 42 children. Glucagon testing was conducted for 150 minutes after subcutaneous glucagon administration and for 30 minutes after 1 microg intravenous ACTH. RESULTS: Mean peak serum cortisol concentrations were 22.4 +/- 0.6 microg/dL (SEM) after subcutaneous glucagon and 20.0 +/- 0.6 microg/dL after intravenous ACTH. Specificity of 95% was found at peak cortisol concentrations of 9.5 and 12.5 microg/dL for the glucagon and ACTH tests, respectively. Concordance between the glucagon and ACTH tests was 90.5%. CONCLUSIONS: The glucagon test was found to be as good a test of the HPA axis as the ACTH test and had a 90.5% concordance with it. The ease of performing the glucagon test, namely, obtaining a single sample of blood 150 minutes after the subcutaneous administration of glucagon, makes it a useful method of assessing the HPA axis in primary care settings.

Adrenocorticotropin-dependent precocious puberty of testicular origin in a boy with X-linked adrenal hypoplasia congenita due to a novel mutation in the DAX1 gene.

Primary adrenal insufficiency is a rare condition in pediatric age, and its association with precocious sexual development is very uncommon. We report a 2-yr-old Brazilian boy with DAX1 gene mutation whose first clinical manifestation was isosexual gonadotropin-independent precocious puberty. He presented with pubic hair, enlarged penis and testes, and advanced bone age. T levels were elevated, whereas basal and GnRH-stimulated LH levels were compatible with a prepubertal pattern. Chronic GnRH agonist therapy did not reduce T levels, supporting the diagnosis of gonadotropin-independent precocious puberty. Testotoxicosis was ruled out after normal sequencing of exon 11 of the LH receptor gene. At age 3 yr he developed clinical and hormonal features of severe primary adrenal insufficiency. The entire coding region of the DAX1 gene was analyzed through direct sequencing. A nucleotide G insertion between nucleotides 430 and 431 in exon 1, resulting in a novel frameshift mutation and a premature stop codon at position 71 of DAX-1, was identified. Surprisingly, steroid replacement therapy induced a clear decrease in testicular size and T levels to the prepubertal range. These findings suggest that chronic excessive ACTH levels resulting from adrenal insufficiency may stimulate Leydig cells and lead to gonadotropin-independent precocious puberty in some boys with DAX1 gene mutations.

Hipoadrenalismo en el síndrome antifosfolípidos/cofactores / Hypoadrenalism and Antiphospholipids/Cofactors

El síndrome antifosfolipidos/cofactores (SACF) se define como la presencia de anticuerpos antifosfolípidos (anticardiolipinas y/o anticoagulante lúpico), trombocitopenia, y asociación con manifestaciones tromboembólicas en territorio arterial y/o venoso. La hemorragia adrenal es una complicación poco común pero con riesgo potencial para la vida. Durante los últimos años ha ido en aumento el reporte de la asociación de hipoadrenalismo y enfermedad de Addison en presencia de anticuerpos antifosfolípidos. Su difícil diagnóstico está probablemente relacionado a las manifestaciones clínicas inespecíficas de la insuficiencia suprarrenal dentro de una enfermedad principal concurrente. Se realizó una revisión de las características más importantes tanto clínicas como de laboratorio, así como las bases patogénicas que incluyen la confluencia del binomio SAFC-hemorragia adrenal. Reconocer que un paciente está en riesgo de hemorragia suprarrenal podría incrementar la posibilidad de un diagnóstico temprano, tratamiento y supervivencia de estos desórdenes

Acometimento da supra-renal associado a paracoccidioidomicose / Adrenal gland involvement associated with paracoccidioidomycosis

A supra-renal foi estudada em 60 pacientes com paracoccidioidomicose. Dentre eles, 10 (16,7%) apresentavam alteraçöes anatômicas ou funcionais das supra-renais. As lesöes glandulares associaram-se à paracoccidioidomicose disseminada, com evoluçäo da doença de pelo menos cinco anos sem tratamento e com hipotensäo arterial sistêmica. Näo houve associaçäo entre alteraçöes anatomoclínicas supra-renais e alteraçöes radiológicas pulmonares. Esses dados revelam a importância da avaliaçäo sistemática da funçäo supra-renal em portadores de formas disseminadas da paracoccidioidomicose

Supra-renalite em chagásicos crônicos / Adrenalitis in patients with chronic Chagas' disease

O estudo das supra-renais de pacientes näo chagásicos mostrou infiltrados linfocitários sempre discretos na cortical (30%) e na medular (50%). Nos chagásicos foram vistos infiltrados mononucleares no tecido perissupra-renálico em 83%, na cortical em 53% e na medular em 83%. Enquanto na cortical o exsudato foi sempre discreto ou moderado, na medular foi moderado em 30% e acentuado em 20%. Em 27% dos chagásicos na medular das supra-renais havia também fibrose fina. Estes dados mostram que nos chagásicos crônicos a medular das supra-renais é acometida preferencialmente à cortical. É possível que o "parentesco" embriológico e funcional com o sistema nervoso autônomo esteja concorrendo para a existência de "caminhos patogenéticos" semelhantes para a ganglionite e medulite supra-renálica da tripanossomose crônica