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RESUMEN La enfermedad relacionada con la inmunoglobulina G4 (IgG4) es un proceso fibroinflamatorio relacionado con la inmumomediación. En las últimas décadas, esta enfermedad ha sido reconocida como un trastorno sistémico que engloba afecciones individuales de órganos, antes no relacionadas y conocidas como entidades independientes. La afectación renal de la enfermedad relacionada con la IgG4 puede ser tanto sincrónica como metacrónica al compromiso de otro órgano como el que se da en la pancreatitis autoinmune, la colangitis esclerosante, la fibrosis retroperitoneal o la linfadenopatía relacionadas con IgG4. En esta revisión presentamos las manifestaciones más frecuentes de la afectación renal por la enfermedad relacionada con IgG4, destacando el papel que tienen las pruebas de imagen. El tratamiento tanto de la enfermedad relacionada con IgG4 en general como de la afectación renal en particular son los glucocorticoides. Es importante conocer esta enfermedad, sospecharla y realizar un diagnóstico precoz y preciso.
ABSTRACT Immunoglobulin G4-related disease (IgG4-RD) is an immunomediated fibroinflammatory process. In the last few decades, this disease has been recognized as a systemic disorder encompassing individual involvement of organs, previously unrelated and known as independent entities. Renal involvement in IgG4-RD may be both synchronous and metachronous to other organ compromise, such as that seen in autoimmune pancreatitis, sclerosing cholangitis, retroperitoneal fibrosis, or IgG4-related lymphadenopathy. In this review we present the most frequent manifestations of renal involvement due to IgG4-RD, highlighting the role of imaging tests. The treatment of both IgG4-RD in general and renal involvement in particular are glucocorticoids. It is important to know about this disease, be suspicious about it and make an early and accurate diagnosis.
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La pancreatitis autoinmune (PAI) es una patología poco frecuente y una entidad a tener en cuenta en el diagnóstico diferencial de ictericia obstructiva y masa pancreática. Es una enfermedad inflamatoria crónica del páncreas con características clínicas, radiológicas, serológicas e histopatológicas establecidas. El tratamiento se basó en el uso de corticoides y suele tener una buena respuesta, con resolución completa de parámetros clínicos, analíticos y radiológicos. Se presenta el caso de una mujer de 62 años con dolor abdominal en hipocondrio derecho y epigastrio asociado a baja de peso. Pruebas de laboratorio normales. TEM abdominal: páncreas aumentado de volumen difusamente con halo peripancreático. EUS: lesión heterogénea extensa que compromete cabeza y cuerpo, se realiza PAAF. AP: Infiltrado linfo-plasmocitario. IgG4: 520 mg/dL. Se determinó que se trata de una pancreatitis autoinmune tipo I probable y se decide realizar prueba terapéutica con corticoides. Se realizó control tomográfico a las 4 semanas con adecuada respuesta.
Autoimmune pancreatitis (PAI) is a rare pathology and an entity to consider in the differential diagnosis of obstructive jaundice and pancreatic mass. It is a chronic inflammatory disease of the pancreas with established clinical, radiological, serological and histopathological characteristics. The treatment is based on the use of corticosteroids and usually has a good response, with complete resolution of clinical, analytical and radiological parameters. We present the case of a 62-year-old woman with abdominal pain in the right hypochondrium and epigastrium associated with low weight. Normal laboratory tests. Abdominal TEM: pancreas increased in volume diffusely with peripancreatic halo. EUS: extensive heterogeneous lesion involving the head and body, FNA is performed. AP: lympho-plasmocitary infiltrate. IgG4: 520 mg / dL. It is determined that it is a probable type I autoimmune pancreatitis and it is decided to perform a therapeutic trial with corticosteroids. Tomographic control is performed at 4 weeks with adequate response.
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Objective To detect the serum level of transglutaminase 2 (TG2)-specific IgE (slgE) in patients with atopic dermatitis (AD),and to analyze its correlation with the disease condition.Methods A total of 77 patients with AD were enrolled into this study,including 44 patients aged ≥ 12 years and 33 patients aged ＜ 12 years.Of the 77 patients,20 were diagnosed with intrinsic AD,which was characterized by the absence of sIgE and total serum IgE values ＜ 150 kU/L,and 49 with extrinsic AD characterized by positive (++) or even strongly positive slgE for more than 1 kind of exogenous allergens,or total serum lgE values ≥ 150 kU/L.[mmunocapture-biotinylated detector enzyme immunoassay was performed to detect the serum level of TG2-sIgE in 77 patients with AD,40 adult patients with psoriasis vulgaris (PV) and 30 healthy adult controls.Clinical data on the AD patients were recorded,including age,disease duration,SCORAD score,blood eosinophil count,levels of total IgE and TG2-sIgE.Results The serum levels of TG2-sIgE in AD patients aged ≥ 12 years,AD patients aged ＜ 12 years,PV patients and healthy controls were 1.02 ± 0.2,1.04 ± 0.044,0.93 ± 0.25 and 0.71 ± 0.13,respectively.Additionally,the serum level of TG2-sIgE significantly differed among AD patients aged ≥ 12 years,PV patients and healthy controls (x2 =37.407,P ＜ 0.001),and was significantly higher in both AD patients aged ≥ 12 years and PV patients than in the healthy controls (t =7.38,4.83,respectively,both P ＜ 0.001).Moreover,the intrinsic AD group showed significantly higher TG2-sIgE levels compared with the extrinsic AD group (1.16 ± 0.03 vs.1.02 ± 0.20,t =2.27,P =0.02).The TG2-sIgE level was uncorrelated with the patients' age,disease duration,SCORAD score,blood eosinophil count or serum total IgE levels in AD patients (r =0.03,0.14,-0.04,-0.08,0.06,respectively,all P ＞ 0.05).Conclusion The serum level of TG2-sIgE obviously increases in AD patients,so TG2 may be one kind of autoantigen in AD patients,but there is no significant correlation between the TG2-sIgE level and AD severity.
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El Síndrome de Guillain Barre es una polineuropatía autoinmune que causa desmielinización motora y sensitiva, frecuentemente con antecedente de una infección. El diagnóstico se realiza mediante sospecha clínica, aunque el líquido cefalorraquídeo y estudios electrodiagnósticos ayudan a soportarlo. El Médico debe estar familiarizado con las variantes clínicas tales como la diplejía facial para hacer un diagnóstico preciso. La inmunoglobulina intravenosa y la plasmaferesis son tratamientos eficaces. El cuidado de soporte durante y después de la hospitalización es crucial.
Guillain Barre Syndrome is an autoimmune polyneuropathy that causes motor and sensory demyelination, often with a history of infection. The diagnosis is made by clinical suspicion, although the cerebrospinal fluid and electrodiagnostic studies help support it. The Physician should be familiar with the clinical variants such as facial diplegia and make an accurate diagnosis. IVIG and plasmapheresis are effective treatments. Supportive care during and after hospitalization is crucial.
A síndrome de Guillain Barre é uma polineuropatia autoimune que provoca desmielinização motora e sensitiva, frequentemente com antecedentes de infecção. O diagnostico é realizado através de suspeita clinica, embora o líquido cefalorraquidiano e estudos eletrodiagnósticos ajudam a apoiá-lo. O médico deve estar familiarizado com as variantes clínicas tais como a diplegia facial para estabelecer um diagnóstico preciso. A imunoglobulina intravenosa e a plasmaférese são tratamentos eficazes. O tratamento de suporte durante e após a hospitalização é crucial.
Subject(s)
Humans , Cerebral Palsy , Immunoglobulins , Guillain-Barre Syndrome , Facial ParalysisABSTRACT
La Enfermedad de Kawasaki (EK) es una de las causas principales de enfermedad cardiaca adquirida durante la niñez. Para una subpoblación de pacientes en especial los menores de 1 año; los síntomas y los signos que forman parte de los criterios actuales para llegar al diagnóstico clínico de la enfermedad pueden aparecer luego de un período prolongado de tiempo haciendo que la identificación de la enfermedad sea más difícil e impidiendo el diagnóstico. Estudios recientes resaltan la importancia del diagnóstico oportuno; reportando que la identificación de la Enfermedad de Kawasaki luego del décimo día de ebre esta asociado con un incremento en la frecuencia de aneurismas de las arterias coronarias. El caso que reportamos es de un lactante masculino de 3 meses quien desarrolla las manifestaciones características de la Enfermedad de Kawasaki a los 20 días de fiebre persistente y además presentó la complicación más temida de dicha enfermedad. Este caso destaca la dificultad para hacer el diagnóstico en los menores de un año, quienes son la población de mayor riesgo para el desarrollo aneurismas coronarios.
Kawasaki disease (KD) is one of the leading causes of acquired heart disease in childhood.For a subpopulation of patients especially those under 1 year; the symptoms and signs that are part of the current criteria for the diagnosis of clinical disease may occur after a prolonged period of time making the identi cation of the disease more di cult and hindering the diagnosis. Recent studies highlight the importance of early diagnosis, reporting that the identi cation of Kawasaki disease after the tenth day of fever is associated with an increased frequency of coronary artery aneurysms. This case report is about a male infant of three months who develops the characteristic manifestations of Kawasaki disease at 20 days of persistent fever and also presented the most feared complication of this disease. This case highlights the di culty in making a diagnosis in children under one year of age, who are those most at risk for developing coronary aneurysms.
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ObjectiveTo investigate the changes and functions of T lymphocyte subsets,immune globulin and complement in children with mycoplasma pneumoniae pneumonia(MPP) on different disease stages.MethodsThe levels of T Iymphocyte subsets of CD3,CD4,CD8 and immunoglobulin ( IgG,IgA IgM),and complement ( C3,C4 ) in the peripheral blood were detected on acute and recovery stages in 28 children with MPP by flow cytometry and immune nephelometry.Twenty-five healthy children were recruited as control group.ResultsAmong these subjects of MPP children on acute stage,the levels of CD3,CD4,CD8,and CD4/CD8 in the peripheral blood were (58.71 ± 11.63)％,(32.36 ± 8.06)％,(28.19±6.23 ) ％ and 1.15 ± 0.41 respectively,and on recovery stage,the levels of CD3,CD4,CD8,and CD4/CD8 were (61.29 ±10.17)％,(34.14 ±7.22)％,(26.47 ±6.01)％,and 1.29 ±0.37 respectively.Both on acute stage and on recovery stage of MPP children,the levels of CD4,CD4/CD8 were significantly lower than those in control group [ (39.53 ± 6.16 ) ％,1.83 ± 0.49 ],and CD8 was significantly higher compared to thecontrol group( 1.83 ± 0.49 ),P＜0.01.CD3 were lower than that in control group [ (63.03 ± 12.32) ％ ] on acute stage (P＜0.01 ),and no significant difference on recovery stage (P＞0.05).During the acute stage of MPP,IgG [ ( 14.50 ±3.86) g/L] and IgM [ ( 1.67 ±0.56) g/L] were obviously higher than those in control group [ (7.92 ± 2.62 ) g/L,( 1.06 ± 0.32 ) g/L,P＜0.01 ],and C3 [ ( 0.83 ± 0.42 ) g/L ] were obviously lower compared to the control group [ ( 1.37 ± 0.33 ) g/L,P＜0.05].There were no significant differences of IgA and C4 between MPP and control groups ( P＞0.05 ).ConclusionChildren with MPP had celhilar immune and humoral immune disorders.Through the detection of T lymphocyte subsets,immunoglobulin and complement,it will be helpful to judge the effectiveness of clinical treatment,which provides a theoretical basis for the clinical application of immune regulators.
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Por más de 30 años se han usado ampliamente las fluoroquinolonas a las cuales ha habidobuena tolerancia; son de amplio espectro contra gérmenes grampositivos y gramnegativos,aunque la actividad de la norfloxacina, la ciprofloxacina y la ofloxacina contra estreptococos yalgunos anaerobios es limitada; tienen, además, buena biodisponibilidad oral y adecuadapenetración en los tejidos. Se presentan reacciones características de hipersensibilidadaproximadamente en 1 por cada 50.000. tratamientos. Se describe un caso de desensibilizaciónoral exitosa con ciprofloxacina en una paciente con infección urinaria crónica parcialmentetratada. Esta es la primera desensibilización con quinolonas reportada en Colombia.
Desensitization with ciprofloxacin in a patient with partially treated urinary tract infectionFluoroquinolones have been widely used for over 30 years and tolerance to them has beengood. They are of broad spectrum against both gram positive and gram negative bacteria, althoughthe activity of norfloxacin, ciprofloxacin and ofloxacin against streptococci and some anaerobicbacteria is rather limited. Their oral bioavailability is good and tissue penetration is adequate.68IATREIA / VOL 23/No. 1/ MARZO/ 2010Hipersensitivity reactions occur in about 1 per 50.000treatments. We report a case of successful oraldesensitization with ciprofloxacin in a patient withchronic partially treated urinary tract infection. This isthe first desensitization with quinolones reported inColombia.
Subject(s)
Humans , Desensitization, Immunologic , Drug Hypersensitivity/drug therapy , Urinary Tract Infections/therapyABSTRACT
Se describe el caso de un paciente de 50 años, masculino, con el diagnóstico de un mieloma IgD, que debuta con la presencia de anemia y dolores óseos generalizados. Se destaca la baja frecuencia de este subtipo de mieloma y su comportamiento biológico. El mieloma IgD constituye solamente el 2.1 % de todos los mielomas. El peor pronóstico del MM IgD con respecto a otro tipo de mielomas está en relación con la mayor frecuencia de insuficiencia renal, invasiones extraóseas y amiloidosis que se presentan. Se describen las características clínicas y humorales del paciente. Se resalta el estudio diagnóstico y la terapéutica empleada.
A case of a patient of 50 years, male, with diagnosis of a myeloma IgD is described, that debuts with the presence of anemia and generalized bony pains. The low frequency of this subtype of myeloma and its biological behavior stands out. The myeloma IgD constitutes only the 2.1% of all the myelomas. The worst prognostic of the MM IgD regarding to another type of myelomas is in relation to the greater frequency of renal insufficiency, extraosseus and amyloidosis invasions that are presented. The clinical and humoral characteristics of the patient are described. The diagnostic study and the employed therapeutic stands out.
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El virus del Oeste del Nilo (VON) es un virus ARN perteneciente a la familia Flaviviridae del género Flavivirus que causa infección en aves, equinos y humanos. La infección por VON es transmitida por el mosquito Culex sp. El ciclo de vida del virus incluye a los mosquitos como vectores y a las aves como huéspedes naturales. El virus mantiene un ciclo de transmisión mosquitoave-mosquito. Los seres humanos son huéspedes accidentales. Se han reportado epidemias en Rumania, Nueva York e Israel. Mediante el programa de vigilancia epidemiológica en nuestro país, se han reportado 90 muestras positivas en 1,223 casos estudiados en aves hasta el 15 de Septiembre del 2005. La enfermedad por el VON se presenta con fiebre, malestar general, anorexia, nausea, vómito, cefalea, mialgia, erupción cutánea y linfadenopatía. La principal entidad clínica descrita es la encefalitis y la parálisis flácida. A mayor edad, es mayor el riesgo de enfermedad neurológica y muerte. Los métodos diagnósticos incluyen determinación de anticuerpos IgM e IgG en suero y/o liquido cerebroespinal. No existe tratamiento antiviral para la infección por VON. Algunas terapias que se han utilizado incluyen interferón α2b e inmunoglobulina específica contra VON. La prevención juega un papel crucial.
West Nile virus (WNV) is a RNA virus of the Flaviridae, genus flavivirus family. It is a neuropathogenic virus causing disease in birds, horses and humans. WNVis transmitted by the vector mosquito Culex sp. The virus life 's cycle includes mosquitoes as vectors and birds as natural hosts. Humans are accidental hosts. Since the introduction of the Epidemiological Surveillance Program at the Ministry ofHealth. we have documented 90 positive test results among birds out of 1,223 cases studied in Mexico as of September IS. 2005. The incubation period in humans after a mosquito bite ranges from 3 to 14 days. Disease is characterized by early onset fever, general malaise, decreased appetite, nausea, vomiting, headaches, myalgias, enlarged lymph nodes andrash. Neurological manifestations include encephalitis andflaccid paralysis, which are present in less than 1% of subjects infected with WNV. Older patients display more adverse outcomes including death. The diagnosis is made by the determination of specific IgM and JgG antibodies in serum and/or cerebrospinal fluid. There is no antiviral treatment to date against WNV but interferon ?2b, and WNVspec4ic-immunoglobulin have been used Prevention is therefore the key to control the infection.