Insuficiencia suprarrenal relativa en pacientes cirróticos / Adrenal insufficiency in cirrhotic patients

La insuficiencia suprarrenal relativa (ISR) es frecuente en pacientes cirróticos con sepsis grave, asociándose a un pobre pronóstico. Se desconoce su importancia en condiciones de enfermedad estable. El objetivo del trabajo ha sido evaluar la prevalencia de la ISR en una serie de pacientes cirróticos estables y su relación con el deterioro de la función hepática. Se determinó el impacto de la ISR en la supervivencia y se correlacionaron los niveles entre el cortisol basal en plasma y saliva en sujetos controles y cirróticos. Fueron incluidos 47 pacientes ambulatorios y 16 controles. La funcionalidad del eje hipotalámico-pituitario-suprarrenal se valoró mediante la prueba de estimulación con 250 μg de ACTH sintética EV, definiendo la ISR como delta cortisol < 9 μg/dl. Respecto al grado de deterioro de la función hepática, 22 tenían un Child-Pugh ≤ 8 y 25 pacientes = 9. La prevalencia de ISR fue de un 22%, siendo significativamente más elevada en aquellos con mayor deterioro de la función hepática (8/32 vs. 3/13, p < 0.05). Se observó correlación entre el cortisol salival y el plasmático basal (r = 0.6, p < 0.0004). Por último, la supervivencia fue más elevada en los pacientes sin ISR al año (97%) y a los tres años (91%) que aquellos que desarrollaron esta complicación (79 % y 51%, p < 0.05, respectivamente). En resumen, la prevalencia de ISR es elevada en los pacientes con cirrosis estable y se relaciona con un deterioro de la función hepática y una mayor mortalidad.

Relative adrenal insufficiency (RAI) is a common finding in cirrhotic patients with severe sepsis, and increased mortality. Its significance is unknown in stable conditions. The aim of this study was to evaluate the prevalence of RAI in stable cirrhotic patients at different stages of the disease. Also, the impact of RAI on the survival was evaluated and basal cortisol levels between plasma and saliva was correlated in control subjects and cirrhotic patients. Forty seven ambulatory patients and 16 control subjects were studied. RAI was defined as a serum cortisol increase of less than 9 μg/dl from baseline after the stimulation with 250 mg of synthetic ACTH. Twenty two had Child-Pugh ≤ 8 and 25 = 9. The prevalence of RAI in patients with stable cirrhosis was 22%. A higher incidence of RAI was observed in patients with a Child-Pugh = 9 (8/32) than in those with ≤ 8 (3/13, p < 0.05). A correlation between salivary cortisol and basal plasma cortisol (r = 0.6, p < 0.0004) was observed. Finally, survival at 1 year (97%) and 3 years (91%) was significantly higher without RAI than those who developed this complication (79% and 51%, p < 0.05, respectively). In summary, the prevalence of RAI is frequent in patients with stable cirrhosis and that it is related to the severity of liver diseaseand increased mortality.

La educación terapéutica en pacientes con insuficiencia adrenal crónica / Therapeutic education of patients with chronic adrenal insufficiency

Las enfermedades adrenales tienen una incidencia y prevalencia no despreciable. Se presentan como situaciones agudas y crónicas. La cronicidad y su desarrollo están relacionados con los comportamientos y estilos de vida, e imponen afrontar exigencias terapéuticas que descansan en las decisiones que la persona asume ante su cuidado. Esta condición crónica puede transitar sin síntomas, y demanda una persona activa y responsable ante su cuidado diario, capaz y formada en los requerimientos de su enfermedad, adiestrada para los ajustes terapéuticos necesarios, y capacitada para actuar ante la presencia de un evento agudo. Los proveedores de salud deben establecer un contrato terapéutico con el enfermo. Este hecho requiere un saber hacer, en el cual el enfoque biosocial, las destrezas en la comunicación y las técnicas de enseñanza/aprendizaje resultan tan necesarias como la actualización clínica. La insuficiencia adrenal aguda puede presentarse de novo, o ser consecuencia del mal manejo de una condición crónica ya conocida. Se citan diversas causas, dentro de las que la omisión inadvertida del fármaco o el desarrollo concomitante de un padecimiento precipitante infeccioso o no, resultan las más frecuentes. Los pacientes con insuficiencia adrenal crónica necesitan estar educados para que sean capaces de desarrollar conocimientos, habilidades y conductas que permitan tomar decisiones acertadas ante posibles descompensaciones(AU)

Adrenal diseases have significant incidence and prevalence; they occur as acute chronic situations. Chronicity and development are both related to behaviours and lifestyles and impose therapeutic demands that lie in the decisions of the person in charge of the patient care. This chronic condition may appear without any symptom and requires an active responsible person for daily care of patients, capable and trained in disease requirements and in necessary therapeutic adjustments, and prepared to act when an acute event occurs. The health care providers should set a therapeutic contract with the patient. This fact requires "know-how" that includes biosocial approach, communicative dexterities and teaching/learning techniques, which are as necessary as the clinical update. The acute adrenal insufficiency may present de novo or results from wrong management of already known chronic condition. There are several causes such as the involuntary omission of medical drug or the concomitant development of a precipitating infective or non-infective illness, which are the most common. The patients suffering chronic adrenal insufficiency need education so that they may be able to acquire new pieces of knowledge, skills and behaviors that allow taking right decisions in the face of possible decompensation(AU)

The etiologies of adrenal insufficiency in 73 Thai children: 20 years experience.

BACKGROUND: Adrenal insufficiency (AI) is an event caused by an inadequate secretion or action of adrenal hormones. It can be classified as primary (1 degree) and secondary (2 degree). AI may result in severe morbidity and mortality when undiagnosed or ineffectively treated. OBJECTIVE: To determine the etiologies of AI in Thai children. MATERIAL AND METHOD: Data of children with AI presented to the authors' pediatric endocrine service between 1982 and 2002 (20 years) were retrospectively collected and analyzed. RESULTS: AI was diagnosed by clinical and laboratory data in 73 children (31 boys and 42 girls). Sixty-two (84.9%) patients had 1degree AI while 11 (15.1%) had 2 degree AI. The majority of patients with 1 degree AI (87.1%) were diagnosed with congenital adrenal hyperplasia (CAH). Other causes of 1 degree AI were uncommon such as ACTH unresponsiveness (4.8%) and no definite diagnosis (8.1%). Most children with 1 degree AI presented with hyperpigmentation. Causes of 2 degree AI were as follows: panhypopituitarism (63.6%), isolated ACTH deficiency (9.1%), and low birth weight (27.3%). CONCLUSION: In the present study, CAH was the most common cause of 1 degree AI while panhypopituitarism was the most common cause of 2 degree AI. Other causes of AI were quite uncommon. Definite causes of AI have not yet been identified in some children. Further clinical observation and special tests including molecular studies in these children are warranted for diagnostic and prognostic importance.

Prevalence of endocrine complications in beta-thalassaemia major in the Islamic Republic of Iran

To identify the prevalence of endocrine dysfunction in Iranians with beta-thalassaemia, we assessed thyroid, parathyroid, pancreatic and adrenal function in 150 beta-thalassaemic patients aged 10-22 years at the Paediatrics Unit, Shiraz University of Medical Sciences. Primary hypothyroidism was found in 6.0% of patients [mean age: 14.6 +/- 1.9 years], hypoparathyroidism in 7.3% [14.5 +/- 3.2 years], type 1 diabetes mellitus in 7.3% [13.9 +/- 2.8 years] and adrenal insufficiency in 1 patient. The relatively high frequency of endocrine dysfunction found in our study may be a result of poor disease control and management in early life when irreversible tissue damage occurs due to iron overload. These findings reinforce the importance of regular follow-up of patients with beta-thalassaemia major for early detection and management of associated complications

Presença de hipocortisolismo clínico-laboratorial em pacientes com paracoccidioidomicose / Presence of clinic-laboratorial hypocortisolism in patients with paracoccidoidomycosis

Micoses sistêmicas se associam, com frequência, ao acometimento da glândula supra-renal. A revisao de prontuários de 135 pacientes com diagnóstico de paracoccidioidomicose permitiu estimar uma prevalência clínica da doença de Addison em 5,2 por cento dos casos. O diagnóstico de doença de Addison foi feito com base na sintomatologia clássica de insuficiência supra-renal (fadiga, perda de peso, fraqueza, hipotensao, hiperpigmentaçao) ou na vigência de um quadro de insuficiência supra-renal aguda. A investigaçao da funçao supra-renal, na ausência de manifestaçoes clínicas clássicas da doença, foi realizada em 9 pacientes. Nesses casos, mostrou resultados sugestivos de normalidade em relaçao ao eixo dos glicocorticóides. Chamam a atençao os baixos níveis de tensao arterial detectados, sendo a pressao diastólica inferior a 80 mmHg em 89,2 por cento dos casos, bem como o alto percentual de hiponatremia e hipercalemia, respectivamente 13,3 por cento e 7,4 por cento dos pacientes avaliados. Estes dados indicam a necessidade de investigaçao adequada do ponto de vista mineralocorticóide. Enfatiza-se que a utilizaçao de agentes antimicóticos inibidores da esteroidogênese deve ser cuidadosamente monitorizada, num contexto de possível hipofunçao supra-renal.