RESUMO
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.
Assuntos
Hiperplasia Suprarrenal Congênita/genética , Hiperplasia Suprarrenal Congênita/cirurgia , Adrenalectomia , Hipertensão/cirurgia , Esteroide 11-beta-Hidroxilase/genética , Glândulas Suprarrenais/patologia , Glândulas Suprarrenais/cirurgia , Hiperplasia Suprarrenal Congênita/complicações , Adulto , Anti-Hipertensivos/uso terapêutico , Encéfalo/patologia , DNA/biossíntese , DNA/genética , Seguimentos , Antagonistas de Hormônios/uso terapêutico , Humanos , Hipertensão/etiologia , Sistema Hipotálamo-Hipofisário/patologia , Masculino , Doenças Retinianas/etiologia , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Maturidade SexualRESUMO
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.