Evaluation and Treatment of Adrenal Dysfunction in the Primary Care Environment.

Adrenal insufficiency (Addison's disease) and Cushing's syndrome are rare disorders characterized by abnormal secretion of adrenal hormones. All patients with adrenal insufficiency and many with Cushing's syndrome require life-long therapy with the potential to impact the quality of life. Management requires gain of a significant amount of knowledge related to treatment, self-care, and how to react quickly in critical situations. Knowledge deficits related to management may cause patients to become critically ill and may even cause death. Ongoing patient/family teaching is crucial for proper disease management and sustaining the quality of life.

Perioperative management of pheochromocytoma/paraganglioma: is there a state of the art?

Pheochromocytoma and paraganglioma are rare tumors of sympathetic or parasympathetic origin, presenting with a highly variable clinical picture. Rarity, as well as biological, clinical, and genetic heterogeneity are barriers to initiate prospective studies that help to establish clinical guidelines. The best management of these patients relies on the experience of a multidisciplinary team. The ultimate outcome can benefit from adequate pre-surgical evaluation and treatment as well as an accurate post-surgical follow-up. Long-term follow-up is mandatory in all patients, but is particularly important in specific familial cases such as those with an SDHB mutation where the risks of recurrence are higher. The surgical approach varies depending on tumor size, location, and surgeon's personal attitude and experience. In this paper, we summarize recommendations, based mostly on authors' and other experts' personal experiences, for the best possible management of patients prior, during and after surgery, as well as when pheochromocytoma is diagnosed during pregnancy.

Long-term postoperative follow-up in patients with apparently benign pheochromocytoma and paraganglioma.

Patients with pheochromocytoma or paraganglioma are at risk of developing tumor recurrences or new tumors after successful resection of the primary tumor. This review summarizes current knowledge concerning the incidence and risk factors for such events. The overall incidence exceeds 15%. Patients with inherited tumors have a higher probability of recurrence or new tumors. Most recurrences are metastatic, particularly in patients with SDHB mutations or nonhereditary tumors. We recommend the determination of plasma or urinary metanephrines (normetanephrine and metanephrine) 1 month after surgery. In patients with sporadic, single tumors ≤5 cm in diameter, clinical and biochemical follow-up should be performed every 2 years. However, this follow-up period can be reduced to yearly, if it is more simple and more convenient for patients and physicians. Patients with larger or multiple but apparently benign tumors and/or inherited disease should be tested 6 months after surgery and then every year for the rest of their lives. Imaging follow-up is also required in patients with inherited or malignant tumors.

[Loïs or the end of life of a child suffering from cancer]. / Loïs ou la fin de vie d'un enfant atteint de cancer.

Loïs, suffering from a malignant tumour, was supported until his death by a general paediatric department, with the collaboration of a mobile continuing care and support team and surrounded by his parents. The particularity of this multi-disciplinary support lies in the fact that the family experienced the birth of their daughter at the same time.

[The nutritional care of a child suffering from neuroblastoma]. / La prise en charge nutritionnelle d'un enfant atteint de neuroblastome.

Children are growing people. When they are suffering from cancer, they are often malnourished. Their nutritional care must be adapted and form an integral part of the overall treatment. The diet of Léa, a young girl suffering from neuroblastoma, was enriched and adjusted in order to take into account the side effects of her treatment.

Pheochromocytoma: challenges in diagnosis and nursing care.

Pheochromocytomas are catecholamine-secreting tumors arising from chromaffin cells of the sympathoadrenal system, which includes the adrenal medulla and sympathetic ganglionic tissue. The effects of catecholamine excess cause potentially fatal symptomologies and end-organ damage if not diagnosed and treated. If diagnosed and removed surgically, most of these patients can be cures. Pheochromocytomas are rare and affect from two to eight per million people. There are 800 deaths in the United States annually as a result of complications. Of patients who have pheochromocytomas diagnosed at autopsy, 75% died suddenly from myocardial infarction or cerebral vascular catastrophe. Challenges in diagnosis, tumor location, and treatment are considerable.

Surgical treatment of pheochromocytomas.

Pheochromocytomas are tumors that develop from chromaffin tissue of the embryonic sympathoadrenal system. These tumors may occur anywhere chromaffin tissue exists but most often develop in the adrenal medulla. Less than 50% of patients are diagnosed with pheochromocytomas while alive, and most of these tumors are found on autopsy. The classic signs and symptoms of pheochromocytomas are headache, perspiration, palpitations, pallor, and paroxysmal hypertension. Elevated levels of vanillylmandelic acid and metanephrines in patients' 24-hour urine collections are the most reliable diagnostic indicators of pheochromocytomas. Most patients with pheochromocytomas can be cured if diagnoses and surgical resections of tumors occur before irreversible cardiovascular disease and end-organ damage from hypertension develop.