RESUMEN
CONTEXT: Bilateral adrenalectomy has been recently proposed as a surgical treatment option for patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. There is however little documented data about the long-term efficiency and potential side effects of this treatment. Patients with 11beta-hydroxylase deficiency (11betaHD) have been also concerned by this new approach. OBJECTIVE: Our objective was to describe our experience with bilateral adrenalectomy as a treatment of severe hypertension in a patient with 11betaHD deficiency and to report the long term follow-up (72 months) results after surgery. PATIENT AND INTERVENTION: A 22-year-old genetically female patient with 11betaHD deficiency was raised as a male because of severe pseudohermaphroditism. The patient has been managed by conventional steroid suppressive therapy and antihypertensive drugs with limited success; hypertension remained uncontrolled and led to severe complications. Bilateral adrenalectomy was offered to him. RESULTS: The intervention was followed by immediate blood pressure normalization and resulted in remarkable clinical improvement. Good compliance with glucocorticoid and androgen substitutive therapies was noted. However, a high 11-deoxycortisol, presumably due to non-ovarian ectopic adrenal rests was noted 24 months after surgery. CONCLUSION: Bilateral adrenalectomy is a safe and efficient method of managing CAH with selected patients. Long-term clinical and biochemical follow-up of patients with CAH treated by bilateral adrenalectomy is needed to earlier detect ectopic adrenal rests.
Asunto(s)
Hiperplasia Suprarrenal Congénita/genética , Hiperplasia Suprarrenal Congénita/cirugía , Adrenalectomía , Hipertensión/cirugía , Esteroide 11-beta-Hidroxilasa/genética , Glándulas Suprarrenales/patología , Glándulas Suprarrenales/cirugía , Hiperplasia Suprarrenal Congénita/complicaciones , Adulto , Antihipertensivos/uso terapéutico , Encéfalo/patología , ADN/biosíntesis , ADN/genética , Estudios de Seguimiento , Antagonistas de Hormonas/uso terapéutico , Humanos , Hipertensión/etiología , Sistema Hipotálamo-Hipofisario/patología , Masculino , Enfermedades de la Retina/etiología , Reacción en Cadena de la Polimerasa de Transcriptasa Inversa , Maduración SexualRESUMEN
OBJECTIVE: To assess the effectiveness of a patient education program on diabetes control. METHODS: A pre-test post-test pre experimental study was performed with 87 insulin treated diabetic patients followed in the chronic disease unit of Kelibia district (Nabeul, Tunisia). Subjects received education in 6 monthly sessions. Outcome measures included glycated haemoglobin (HbA1c), anxiety level according to the Hamilton scale and Body Mass Index. Changes were assessed after the 6 month program. RESULTS: HbA1c decreased from 8.8% +/- 1.23 to 7,6 +/- 1.43 (P<10-6) with an increase in the prevalence of satisfactory HbA1c (< or = 8%) from 33% to 61.2% (P<0,001). The prevalence of severe anxiety decreased by about a third (P < 0,001). CONCLUSION: Education led to an improvement in diabetes control in insulin treated diabetic patients. More attention should be paid to such strategies in general practice.