ABSTRACT
Pruritus is a disabling symptom accompanying chronic cholestasis. In extreme cases, the refractory nature of pruritus can result in a need for invasive therapies including liver transplantation. The pathogenesis of pruritus in cholestatic disease is poorly understood. It may involve a specific neural pathway (similar to that associated with pain) regulated by several pruritogenic substances such as bile acids, opioids, serotonin, and the more recently identified lysophosphatidic acid. While the therapeutic management of cholestatic pruritus is well established in adults, there is no consensus in children, in light of the difficulty of conducting controlled clinical studies. The currently recommended strategy to manage cholestatic pruritus in children is based on several lines of specific therapies that should be associated with skin hydration and with non-specific treatment of cholestasis including ursodeoxycholic acid. Pruritus should be assessed as objectively as possible between each line of therapy. Rifampicin, a potent CYP3A4 inducer, is the first-line treatment of cholestatic pruritus. Second-line therapies require evaluation of the child in an expert center and are discussed on a case-by-case basis depending on the underlying disease and the experience of the center. These include inhibitors of serotonin reuptake (sertraline), opioid antagonists (naloxone), or ASBT inhibitors. Invasive therapies such as biliary diversion or liver transplantation can also be proposed in the most severe cases. The aim of the current update is to review the physiopathologic mechanisms implicated in cholestatic pruritus and to propose potential therapeutic strategies in children.
Subject(s)
Cholestasis/physiopathology , Cholestasis/therapy , Pruritus/physiopathology , Pruritus/therapy , Child , Cholestasis/complications , Humans , Practice Guidelines as Topic , Pruritus/etiologyABSTRACT
Pruritus is a disabling symptom accompanying chronic cholestasis. In some cases, refractory pruritus may require invasive therapies including liver transplantation. The pathogenesis of pruritus in cholestatic disease is poorly understood. It may involve a specific neural pathway and several pruritogenic substances such as bile acids, opioids, serotonin, and the more recently identified lysophosphatidic acid. While the therapeutic management of cholestatic pruritus is well established in adult patients, there is no consensus in children, given the difficulty in conducting controlled clinical studies. The currently recommended strategy to manage cholestatic pruritus in children is based on several lines of therapy that should always be associated with local cutaneous care and with nonspecific treatment of cholestasis including ursodeoxycholic acid therapy. Pruritus should be assessed as objectively as possible between each therapeutic step. Rifampicin, an enzyme inducer, is the specific first-line treatment of cholestatic pruritus. The second-line therapies require evaluation of the child in an expert center and are discussed on a case-by-case basis depending on the underlying disease, the experience of the center and the will of the child and his family. It could be inhibitors of serotonin reuptake (sertraline) or an opioid antagonist (naloxone). Invasive therapies such as biliary diversion or liver transplantation can also be proposed in the most severe cases.
Subject(s)
Cholestasis/complications , Pruritus/etiology , Pruritus/therapy , Anion Exchange Resins/therapeutic use , Biliary Tract Surgical Procedures , Child , Cholagogues and Choleretics/therapeutic use , Cholestyramine Resin/therapeutic use , Chronic Disease , Cytochrome P-450 CYP3A Inducers/therapeutic use , Humans , Liver Transplantation , Narcotic Antagonists/therapeutic use , Rifampin/therapeutic use , Selective Serotonin Reuptake Inhibitors/therapeutic use , Sertraline/therapeutic use , Sorption Detoxification , Ursodeoxycholic Acid/therapeutic useABSTRACT
The onset of puberty is the sum of complex and multifactorial mechanisms resulting from the action of both activating and inhibiting factors, leading to the maturation of the gonads and the ability to reproduce. Many contributors to pubertal development are involved in fat mass acquisition and their action is relayed through the hypothalamus. It is therefore easy to understand how chronic diseases can affect the development of puberty and fertility apart from the specific impact of their molecular alteration. We have chosen cystic fibrosis and chronic renal disease as examples of chronic disorders affecting puberty through distinct mechanisms. As drugs are undistinguishable from chronic diseases, we also describe the impact of corticosteroids and chemotherapy on reproductive function. Last, we describe the surveillance and care of pubertal delay and its consequences (growth and bone mineralization) of patients affected with chronic disorders during adolescence.
Subject(s)
Chronic Disease , Fertility/physiology , Puberty/physiology , Adrenal Cortex Hormones/adverse effects , Cystic Fibrosis/complications , Cystic Fibrosis/physiopathology , Drug-Related Side Effects and Adverse Reactions , Gonadal Disorders/etiology , Humans , Hypothalamus/physiopathology , Puberty, Delayed/complications , Renal Insufficiency, Chronic/complications , Renal Insufficiency, Chronic/physiopathologyABSTRACT
Introducción/Objetivos: la Fundación CUDECA esta formada por un equipo asistencial multidisciplinar formado por médicos, enfermeros-as, trabajadora social, psicóloga y voluntarios cualificados, que atiende al paciente paliativo tanto en el Centro CUDECA, como en el propio domicilio del enfermo, consiguiendo así que se puedan cumplir los deseos de las personas en esta situación, enfermos y familiares, de permanecer en su propio hogar. Se analizan, en este estudio, los datos de 3.400 pacientes atendidos en el Programa Asistencial de Cuidados Paliativos de la Fundación CUDECA. Material y métodos: se procesaron los datos de 3.400 pacientes, incluidos en programa desde 1992 hasta 2005, mediante el programa estadístico SPSS 11.0, se realizó un estudio descriptivo de las siguientes características de los pacientes: sexo, edad de los pacientes, edad del cuidador, tiempo de permanencia en programa, tipo de tumor más frecuente y lugar de defunción de los pacientes, que figuraba codificado de la siguiente manera: domicilio, hospital, UCP (Unidad de Cuidados Paliativos), residencia y otros. Resultados: la media de edad es de 67 años. Acorde con la mayor prevalencia de tumores en pacientes mayores y también en relación con el mayor envejecimiento de la población. Predominan los varones: 59,4%. En relación a la mayor incidencia de tumores, destaca el de pulmón en varones, que es el tumor más prevalente en nuestra población. La media de estancia en programa es de 76 días. La mediana es de 36 días. Destaca el tumor de pulmón como el más frecuente con un 24%. Seguido del colorrectal con un 14%, cabeza y cuello y cáncer de mama con un 10%. La mayoría de nuestros pacientes, el 76,8%, fallecen en el domicilio. Conclusiones: la mayoría de nuestros pacientes, el 76,8% fallecen en el domicilio. Es un dato muy importante de calidad de asistencia en Cuidados Paliativos, ya que se consigue que los pacientes mueran en su ambiente familiar. Supone un importantísimo ahorro al Sistema Nacional de Salud y compañías privadas, ya que se evitan estancias prolongadas en hospital, con el ahorro que supone de coste cama/día. Los datos de alta frecuencia de cáncer de pulmón coinciden con los descritos en la literatura, ya que se trata del tumor más prevalente. En nuestra población destaca la alta frecuencia de tumores de cabeza y cuello, en posible relación al tabaco y al consumo de alcohol. La media de estancia en programa es corta, estos datos demuestran la sospecha inicial de que los pacientes son derivados a las Unidades de Cuidados Paliativos por los Servicios de Oncología en situación muy avanzada de su enfermedad, es necesario, por tanto, mejorar la coordinación entre las Unidades de Cuidados Paliativos y los Servicios de Oncología de Procedencia (AU)
Objectives: CUDECA Foundation is formed by a multidisciplinary team of doctors, nurses, social workers, psychologists, and qualified volunteers who care for patients at CUDECA's center and in their homes, so that patients can stay with their families in their own houses. We analyzed the data of 3,400 patients who entered the palliative care program of CUDECA Foundation. The main objectives of CUDECA are: to offer «specialised palliative care» to patients suffering from terminal cancer, and also to support their families during illness and the bereavement process. To constitute a study, training, investigation, and awareness program regarding palliative care. Our care is offered completely free of charge to those patients and families who need it and who live in Malaga province, Spain. Methods: We analyzed the data of 3400 patients who entered the palliative program of CUDECA Foundation using the statistical program SPSS 11.0. A descriptive study was performed of the following patient characteristics: sex, median age, median age of caregiver, time in the program, and place of death, which was coded with the following variables -nursing home, hospital, palliative care unit (PCU), home, other. Results: The majority of our patients, 76.8%, died in their homes. Median age of patients: 67 years. Median age of caregiver: 55 years. Males predominated (59.4%) in the prevalence of tumours, especially of the lungs. Mean time in the program: 76 days. Lung cancer was the most frequent malignancy (24%), followed by colorectal cancer (14%), and head and neck tumours (10%). Conclusions: Mean age of patients was high due to the greater prevalence of tumours in older patients and also in relation to population ageing. Mean time in the program was not very long (76 days); as patients are sent to palliative care units by oncology departments when disease is advanced, coordination between palliative care units and oncology departments. The high frequency of lung cancer is consistent with the data described in the literature, as this is the tumour with the highest prevalence. A majority of our patients, 76.8 %, died in their homes. This is avery important quality-of-life issue in palliative care, as we allowed patients to die with their families and in their homes according to their own wishes (AU)
Subject(s)
Humans , Male , Female , Middle Aged , Foundations/organization & administration , Palliative Care/organization & administration , Home Care Services/organization & administration , Home Nursing , Lung Neoplasms/epidemiology , Lung Neoplasms/prevention & control , Oncology Service, Hospital/trends , Retrospective StudiesABSTRACT
La disnea es un síntoma muy frecuente en medicina paliativa, especialmente en pacientes con diagnostico de cáncer de pulmón, que afecta seriamente la calidad de vida de los pacientes. Presentamos un caso clínico, de un paciente con disnea secundaria a un cáncer de pulmón avanzado, resistente a tratamiento convencional con morfina, donde la disnea se controla con el uso del citrato de fentanilo oral transmucoso. Se realiza una revisión amplia de la literatura donde apenas hay estudios del uso del citrato de fentanilo oral transmucoso en el tratamiento de la disnea, si hemos encontrado estudios con el uso del fentanilo nebulizado en el tratamiento de la disnea, con buenos resultados. Sería interesante la realización de ensayos fase II-III que respondieran a esta pregunta (AU)
Dyspnea is a frequent symptom in palliative medicine, and may seriously affect patient quality of life. We report a case of dyspnea in a patient with advanced lung cancer resistant to conventional treatment with morphine, which was controlled with oral transmucosal fentanyl citrate. We performed a systematic review of the literature, and found little evidence on the use of oral transmucosal fentanyl citrate in the treatment of dyspnea; however, we did find some evidence on the use of nebulized fentanyl with positive results. It is important that new phase II-III clinical studies are designed to answer this question (AU)