RESUMEN
Ichthyosis prematurity syndrome (IPS) is a rare inherited skin disorder. Children are born prematurely with thick skin and have been found to develop neonatal asphyxia due to occlusions in the bronchial tree from debris in the amniotic fluid. At 31 weeks of gestation, separation of amniotic and chorionic membranes was identified as well as polyhydramnion. The child was born 2 weeks later, with thickened skin with a granular appearance and required immediate ventilation and intensive care. At 2 years of age, the patient has developed an atopic skin condition with severe itching, recurrent skin infections, food intolerance and periods of wheezing. Prenatal observation of separation of foetal membranes or dense amniotic fluid may be signs of IPS and severe complication immediately after birth.
Asunto(s)
Amnios/diagnóstico por imagen , Asfixia Neonatal/diagnóstico por imagen , Corion/diagnóstico por imagen , Ictiosis/diagnóstico por imagen , Enfermedades del Prematuro/diagnóstico por imagen , Adulto , Aniridia , Asfixia Neonatal/genética , Presentación de Nalgas , Cesárea , Preescolar , Codón sin Sentido , Diagnóstico Diferencial , Exones/genética , Proteínas de Transporte de Ácidos Grasos/genética , Femenino , Rotura Prematura de Membranas Fetales/diagnóstico por imagen , Rotura Prematura de Membranas Fetales/genética , Estudios de Seguimiento , Homocigoto , Humanos , Ictiosis/genética , Interpretación de Imagen Asistida por Computador , Imagenología Tridimensional , Lactante , Recién Nacido , Enfermedades del Prematuro/genética , Riñón/anomalías , Polihidramnios/diagnóstico por imagen , Polihidramnios/genética , Embarazo , Trastornos Psicomotores , Suecia , Ultrasonografía PrenatalRESUMEN
A 63 year old woman seeks medical attention for symptoms of fever, headache and increasing dyspnoea. She has a history of psoriatic-arthritis and uses 10 mg/week of methotrexate as arthritic treatment. There is also a history of severe alcohol abuse. She is in respiratory failure, with basal pulmonary crackles and increased serum inflammatory markers, a normal white-cell count but with a lowered lymphocyte count. A CT-scan reveals ground-glass lung changes. Bronchial biopsy reveals the fungus P. jiroveci which infects immunodeficient hosts. After extensive testing it was concluded that the patient's immunodeficiency was attributed to the combination of methotrexate, ethanol and psoriatic-arthritis. In this article, a case of pneumocystis pneumonia is reviewed as well as the infective mechanism of P. jiroveci and host-defence against the fungus. Additionally, symptoms and signs of the infection, diagnostic approach and treatment are reviewed. Because the combination of methotrexate and ethanol played a significant role in the immunodeficiency of the patient, their effect on the immune system is addressed.
Asunto(s)
Alcoholismo/complicaciones , Artritis Psoriásica/complicaciones , Huésped Inmunocomprometido , Inmunosupresores/efectos adversos , Metotrexato/efectos adversos , Pneumocystis carinii/patogenicidad , Neumonía por Pneumocystis/inmunología , Alcoholismo/inmunología , Artritis Psoriásica/tratamiento farmacológico , Artritis Psoriásica/inmunología , Femenino , Humanos , Sistema Inmunológico/efectos de los fármacos , Pulmón/diagnóstico por imagen , Pulmón/inmunología , Pulmón/microbiología , Persona de Mediana Edad , Pneumocystis carinii/aislamiento & purificación , Neumonía por Pneumocystis/diagnóstico , Neumonía por Pneumocystis/microbiología , Neumonía por Pneumocystis/terapia , Tomografía Computarizada por Rayos XRESUMEN
The knowledge of drug side-effects is an important part of modern medicine and it is thought that about 25% of all side effects are based on activation of the immune system. Unlike most other side effects, immune responses to drugs are usually unforeseen and minimally or not at all related to their dosage. Such activation is not only based on the pharmacological character of the drug but also various environmental factors and the individual?s genetic makeup. Allergy is traditionally categorized into the four groups of Gell and Coombs. Such allergy is usually based upon specific activation of certain cells through antibody receptors on the cell-surface but the immune system can also be activated unspecifically, irrespective of antibody receptors, through pharmacological actions or by unknown mechanisms. Non-steroidal anti inflammatory drugs (NSAIDs) can cause allergic reactions either directly or indirectly. Because of the extensive usage and usefulness of NSAIDs in medicine, these allergic side effects cause a large and difficult problem within the health system. This article discusses in depth the causes and pathology of the different disease forms caused by immune reactions to NSAIDs, with emphasis on describing why some people with asthma may feel a serious, temporary worsening of the disease after ingestion of NSAIDs. Finally, diagnostic approaches to NSAID allergies are discussed.