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1.
Rev. med. Chile ; 150(9): 1260-1265, sept. 2022. ilus
Artículo en Español | LILACS | ID: biblio-1431894

RESUMEN

Hereditary transthyretin amyloidosis is a multisystemic autosomal dominant genetic disorder characterized by progressive distal sensory-motor polyneuropathy or restrictive cardiomyopathy, secondary to amyloid deposits. Its pathogenesis lies in the TTR gene mutation, and the Val50Met mutation is the most frequent. Patients have significant differences in the onset and severity of clinical presentation according to their country of origin. The diagnosis of this pathology is complex, even more in countries where it is not considered endemic. However, early suspicion and management are essential to improve survival and avoid unnecessary diagnostic and therapeutic strategies. We report a 69-year-old woman who presented a sensory-motor polyneuropathy, predominantly sensory, associated with distal neuropathic pain and bilateral vitritis. The history of her Italian father with polyneuropathy of unspecified etiology stood out. A vitreous biopsy identified amyloid substance deposits (congo red positive). These were also confirmed on a superficial peroneal nerve biopsy. During the etiological study of her polyneuropathy, an increased Kappa/Lambda index of 2.55 mg/L stood out. Therefore, light chain amyloidosis was suspected, and chemotherapy treatment was indicated without favorable response. After 10 years of progressive neurological and ophthalmological involvement, a genetic study confirmed the first case of late-onset hereditary transthyretin amyloidosis Val50Met with polyneuropathy in Chile.


Asunto(s)
Humanos , Femenino , Anciano , Polineuropatías/etiología , Polineuropatías/genética , Neuropatías Amiloides Familiares/complicaciones , Neuropatías Amiloides Familiares/diagnóstico , Neuropatías Amiloides Familiares/genética , Prealbúmina/genética , Mutación
2.
Rev. med. Chile ; 150(6): 828-831, jun. 2022. ilus
Artículo en Español | LILACS | ID: biblio-1424126

RESUMEN

Renal involvement in COVID-19 infection is varied and worsens its outcome and prognosis. However, the association of COVID-19 infection with glomerulonephritis is exceptional. We report a 46-year-old woman with COVID-19 who had an acute kidney injury and ANCA associated glomerulonephritis two weeks after the onset of the disease. The kidney biopsy showed a crescentic glomerulo-nephritis and the presence of anti-glomerular basement membrane antibodies (GBM-Abs). She was treated with steroids and oral cyclophosphamide with good response without requiring plasmapheresis. Plasma anti GBM-Abs were negative. This case suggests that the presence of anti-GBM-Abs in the kidney, was temporally related to COVID-19 pulmonary damage. The absence of plasma antibodies is probably due to transient production and glomerular adsorption, but with unknown pathogenic role.


Asunto(s)
Humanos , Femenino , Persona de Mediana Edad , COVID-19/complicaciones , Glomerulonefritis/complicaciones , Autoanticuerpos , Membrana Basal/patología , Anticuerpos Anticitoplasma de Neutrófilos
3.
Rev. méd. Chile ; 149(6): 934-938, jun. 2021. ilus, tab
Artículo en Español | LILACS | ID: biblio-1389531

RESUMEN

Acute phosphate nephropathy (APN) is an acute renal failure secondary to the use of oral sodium phosphate (OSP) laxatives, with a high risk of progression to chronicity. We report a 60-year-old woman with mixed connective tissue disease whose serum creatinine increased up to 2.0 mg/dL in her regular control tests, without an evident causative factor. Kidney biopsy showed numerous intratubular calcium phosphate deposits, consistent with APN. She had a history of OSP laxative intake, and a sodium phosphate enema was used before a colonoscopy performed six months earlier. The temporal association between the use of OSP laxatives and acute kidney injury, should lead to the suspicion of APN. The urine sediment is generally normal or with mild to moderate proteinuria. The diagnosis is confirmed with a kidney biopsy. Until now, there is no specific treatment for APN, thus prevention is essential. In high-risk patients for developing APN, the administration of these laxatives should be avoided.


Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Laxativos/efectos adversos , Lesión Renal Aguda , Fosfatos/efectos adversos , Catárticos/efectos adversos
4.
Rev. méd. Chile ; 148(8)ago. 2020.
Artículo en Español | LILACS | ID: biblio-1389302

RESUMEN

Background: One of the devastating consequences of monoclonal gammopathies is the development of end-stage kidney disease, which can be prevented with an early diagnosis. Renal involvement can be secondary to saturation of paraproteins with intratubular precipitation or the glomerular deposition of paraproteins with secondary inflammation and destruction. These conditions can also be associated with monoclonal gammopathies that do not meet hematological treatment criteria, called monoclonal gammopathies of renal significance (MGRS). Aim: To report a retrospective analysis of patients who underwent a renal biopsy and whose final diagnosis was a form of monoclonal gammopathy. Material and Methods: We reviewed the clinical and laboratory features and response to treatment of 22 patients aged 63 ± 12 years (55% women) with a pathological diagnosis of a nephropathy associated with paraproteinemia. Results: The most common hematological diagnosis was amyloidosis in 50% of patients, followed by cast nephropathy. The predominant clinical presentations were proteinuria (without nephrotic syndrome) and nephritic syndrome. Classic criteria such as erythrocyte sedimentation rate > 100 mm/h and protein-albumin gap were unusual. Serum light chain quantification was the test with the best yield to detect paraproteins. Conclusions: In this group of patients, light chains tend to affect the kidney more commonly than heavy chains. The prognosis of multiple myeloma is much worse than MGRS.


Asunto(s)
Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Paraproteinemias , Enfermedades Renales , Paraproteinemias/complicaciones , Paraproteinemias/diagnóstico , Paraproteínas , Estudios Retrospectivos , Riñón , Enfermedades Renales/diagnóstico , Enfermedades Renales/etiología
5.
Rev. méd. Chile ; 148(5): 702-706, mayo 2020. tab, graf
Artículo en Español | LILACS | ID: biblio-1139356

RESUMEN

C1q nephropathy is a rare glomerulopathy characterized by mesangial deposition of the complement component C1q. These deposits can be isolated or associated with immunoglobulins or complement fractions, which are observed by immunofluorescence or immunohistochemical microscopy. In ultramicroscopy, dense mesangial deposits and alterations of the podocyte are observed. Clinically it presents as a nephrotic syndrome (NS) or by alterations of the urinalysis such as proteinuria and/or hematuria in children and young adults. In light microscopy, it is expressed with a morphological pattern of minimal change disease (MCD), mesangial proliferative glomerulonephritis or focal segmental glomerulosclerosis (FSGS). The NS during its evolution usually evolve in steroid resistance or steroid dependency, often requiring the association of immunosuppressants to obtain remission. We report a 14 years old male with a history of NS and its evolution under various treatments during a 12-year follow-up.


Asunto(s)
Humanos , Masculino , Adolescente , Complemento C1q/metabolismo , Glomerulonefritis/diagnóstico
6.
Rev. méd. Chile ; 147(12): 1510-1517, dic. 2019. tab, graf
Artículo en Español | LILACS | ID: biblio-1094184

RESUMEN

Background Lupus nephritis (LN) is a complication of systemic lupus erythematosus that requires renal biopsy (RB). Proliferative classes III, IV-S, IV-G have especial clinical and pathological characteristics. Aim To determine the association between pathological features in RB with serum creatinine and urine protein levels. Material and Methods We analyzed 186 RB performed in adults aged 18 to 73 years, from a renal pathology reference center. Histopathological variables such as class and subclass of proliferative LN, endocapillary and extracapillary proliferation, activity and chronicity indexes, and vascular sclerosis were correlated with serum creatinine and urine protein levels, at the time of diagnosis. Results As compared with LN III, all the morphological and laboratory values were significantly more deteriorated in LN IV, with special focus on vascular sclerosis. Serum creatinine was the only variable that significantly differentiated LN IV-S from LN IV-G. Proteinuria was non-significantly higher in LN IV-G compared to LN IV-S. However, the difference became significant when proteinuria was compared between LN IV-G and LN III. Conclusions The significant difference in serum creatinine between LN IV-S and LN IV-G supports the concept that they are different subclasses. Proteinuria is a variable that differentiates classes III from IV-G, being significantly higher in the second. Severe arteriosclerosis is a constant and significant finding that differentiates LN III from LN IV. Thus, we propose its usefulness for distinguishing LN classes, and eventually, to be considered in the chronicity index.


Asunto(s)
Humanos , Masculino , Femenino , Adolescente , Adulto , Persona de Mediana Edad , Anciano , Adulto Joven , Nefritis Lúpica/patología , Riñón/patología , Proteinuria/patología , Biopsia , Índice de Severidad de la Enfermedad , Estudios Retrospectivos , Creatinina/sangre
7.
Rev. chil. infectol ; 36(2): 238-242, abr. 2019. ilus
Artículo en Español | LILACS | ID: biblio-1020631

RESUMEN

Resumen La esquistomiasis urinaria es producida por Schistosoma haematobium. Es una enfermedad endémica en muchas regiones del mundo, no existente en Chile. Se presenta el caso de un hombre joven que viajó a Malawi, en África meridional, y que a su regreso al país, años después, presentó un síndrome miccional con hematuria macroscópica. La biopsia de vejiga mostró una cistitis granulomatosa y eosinofílica con huevos de Schistosoma haematobium.


Urinary schistosomiasis is produced by Schistosoma haematobium. It is an endemic disease in many regions of the world, non-existent in Chile. We report a case of a young man who traveled to Malawi, in southern Africa, and who returned to Chile. Few years later, he presented a urinary syndrome with macroscopic hematuria. The bladder biopsy showed a granulomatous and eosinophilic cystitis with eggs of Schistosoma haematobium.


Asunto(s)
Humanos , Masculino , Adulto , Esquistosomiasis Urinaria/diagnóstico , Esquistosomiasis Urinaria/patología , Schistosoma haematobium , Chile , Malaui
8.
Rev. méd. Chile ; 147(3): 390-394, mar. 2019. graf
Artículo en Español | LILACS | ID: biblio-1004362

RESUMEN

Goodpasture Syndrome is described as a single episode disease entity. It is diagnosed with the demonstration of antiglomerular basement (anti-GBM) antibodies in plasma or renal tissue. Although the recurrence of anti-GBM disease is rare, it has been reported in up to 3% of cases. Recurrence with negative anti-GBM antibodies in plasma is even less frequent We report a 63 years old male in whom anti-GBM disease recurred without detectable anti-GBM antibodies in plasma, despite having positive antibodies at the onset.


Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Autoanticuerpos/análisis , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/patología , Recurrencia , Biopsia , Prednisona/uso terapéutico , Combinación Trimetoprim y Sulfametoxazol/uso terapéutico , Técnica del Anticuerpo Fluorescente , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/tratamiento farmacológico , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/diagnóstico por imagen , Ciclofosfamida/uso terapéutico , Enfermedades Renales/patología , Glomérulos Renales/patología , Antibacterianos/uso terapéutico
9.
Rev. méd. Chile ; 146(12): 1486-1492, dic. 2018. graf
Artículo en Español | LILACS | ID: biblio-991361

RESUMEN

We report a 23 year old woman presenting with a nephrotic syndrome due to minimal change disease, central diabetes insipidus, primary hypothyroidism, vitiligo and universal alopecia. Eleven years later, she presented secondary amenorrhea due to hypogonadotropic hypogonadism, with mild hyperprolactinemia and central adrenal insufficiency. A magnetic resonance imaging of the sella turcica showed a pituitary mass with suprasellar extension that was resected using a transsphenoidal approach. Pathology confirmed the presence of a lymphoplasmacytic hypophysitis. She needed a second surgical resection due to mass growth and neuro-ophthalmologic impairment. One year later, systemic lupus erythematosus, arterial hypertension and type 2 diabetes mellitus were diagnosed. Two years later, due to back pain, constipation and renal failure, retroperitoneal fibrosis was found, satisfactorily treated with glucocorticoids and colchicine. Hence, this clinical vignette shows the coexistence of autoimmune polyglandular syndrome with retroperitoneal fibrosis and lymphoplasmacytic hypophysitis. Tissue analysis showed the presence of IgG4 producing plasma cells in the pituitary and retroperitoneum, which constitute a basis for the diagnosis of IgG4 related disease.


Asunto(s)
Humanos , Femenino , Adulto Joven , Fibrosis Retroperitoneal/complicaciones , Poliendocrinopatías Autoinmunes/complicaciones , Hipofisitis/complicaciones , Enfermedad Relacionada con Inmunoglobulina G4/complicaciones , Fibrosis Retroperitoneal/patología , Fibrosis Retroperitoneal/diagnóstico por imagen , Imagen por Resonancia Magnética , Poliendocrinopatías Autoinmunes/patología , Poliendocrinopatías Autoinmunes/diagnóstico por imagen , Hipofisitis/patología , Hipofisitis/diagnóstico por imagen , Enfermedad Relacionada con Inmunoglobulina G4/patología , Enfermedad Relacionada con Inmunoglobulina G4/diagnóstico por imagen
10.
Rev. méd. Chile ; 146(2): 241-248, feb. 2018.
Artículo en Español | LILACS | ID: biblio-961383

RESUMEN

Renal involvement is a frequent complication in antineutrophil cytoplasmic antibodies (ANCA)associated vasculitides, adding morbidity and mortality, such as chronic kidney disease and the need for renal replacement therapy. With the aim of reaching a consensus on relevant issues regarding the diagnosis, treatment and follow-up of patients with these diseases, the Chilean Societies of Nephrology and Rheumatology formed a working group that, based on a critical review of the available literature and their experience, raised and answered consensually a set of questions relevant to the subject. This document includes aspects related to the clinical diagnosis, the histological characteristics, the therapeutic alternatives to induce and maintain the remission of the disease, relapse surveillance strategies and complementary therapies.


Asunto(s)
Humanos , Anticuerpos Anticitoplasma de Neutrófilos/sangre , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/complicaciones , Enfermedades Renales/etiología , Enfermedades Renales/terapia , Sociedades Médicas , Inducción de Remisión , Chile , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/diagnóstico , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/sangre , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/terapia , Quimioterapia de Mantención
12.
Rev. méd. Chile ; 145(1): 131-134, ene. 2017. ilus
Artículo en Español | LILACS | ID: biblio-845513

RESUMEN

IgG4 disease is a multi-systemic condition involving pancreas, salivary glands and lymph nodes. Less frequently, it causes interstitial nephritis and involves the lungs. We report a 58 years old male with a four years history of hemoptysis and renal dysfunction characterized by hematuria and proteinuria, responsive to steroidal therapy. The renal biopsy established the diagnosis of IgG4 associated interstitial nephritis. Lung involvement was considered secondary to the same systemic disease.


Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Enfermedades Autoinmunes/complicaciones , Inmunoglobulina G , Hemoptisis/etiología , Nefritis Intersticial/complicaciones , Nefritis Intersticial/diagnóstico , Recurrencia , Enfermedades Autoinmunes/diagnóstico , Diagnóstico Diferencial , Hemoptisis/diagnóstico
13.
Rev. méd. Chile ; 144(10): 1351-1355, oct. 2016. ilus
Artículo en Español | LILACS | ID: biblio-845451

RESUMEN

ANCA mediated vasculitis mainly occur between the fourth and fifth decade of life; therefore, it is very uncommon to see pregnant patients with the disease. Vasculitis may affect significantly the course of pregnancy; in turn pregnancy can change the course of vasculitis. We report a 20 years old woman with ANCA-mediated renal vasculitis lasting 10 years who consulted with a pregnancy of 15 weeks. She was in remission and had amenorrhea attributed to ovarian toxicity due to cyclophosphamide. Pregnancy had an uneventful course with spontaneous delivery at the 37th week, giving birth to a healthy newborn. Proteinuria increased during the course of pregnancy with a mild deterioration of kidney function. During the year after delivery, she had nephrotic proteinuria and a worsening of renal function.


Asunto(s)
Humanos , Femenino , Embarazo , Adulto Joven , Complicaciones del Embarazo/patología , Vasculitis/patología , Anticuerpos Anticitoplasma de Neutrófilos , Enfermedades Renales/patología , Complicaciones del Embarazo/etiología , Complicaciones del Embarazo/sangre , Proteinuria , Factores de Tiempo , Vasculitis/etiología , Vasculitis/sangre , Biopsia , Resultado del Embarazo , Edad Gestacional , Tasa de Filtración Glomerular , Enfermedades Renales/etiología , Enfermedades Renales/sangre
14.
Rev. méd. Chile ; 144(9): 1218-1221, set. 2016. ilus
Artículo en Español | LILACS | ID: biblio-830631

RESUMEN

HIV infection has different clinical presentations. We report a 21-year-old male with longstanding isolated microscopic hematuria attributed to thin glomerular basement membrane disease, who after 15 years of follow-up presented with significant proteinuria. A kidney biopsy was performed, revealing the presence of tubulo-reticular inclusions in the glomerular endothelial cells. This finding led to suspect an HIV infection, which was verified. Antiretroviral therapy, angiotensin-converting enzyme and angiotensin II receptor blockers were prescribed. At 6 years of diagnosis the patient is asymptomatic and has normal kidney function. Microscopic hematuria and low level proteinuria persists.


Asunto(s)
Humanos , Masculino , Adulto , Adulto Joven , Nefropatía Asociada a SIDA/diagnóstico , Hematuria/diagnóstico , Proteinuria/orina , Factores de Tiempo , Biopsia , Nefropatía Asociada a SIDA/complicaciones , Hematuria/complicaciones , Túbulos Renales/ultraestructura
15.
Rev. méd. Urug ; 31(3): 214-220, set. 2015. ilus, tab
Artículo en Español | LILACS | ID: lil-763431

RESUMEN

La dermatomiositis (DM) al igual que otras enfermedades autoinmunes ha sido reportada en varios estudios como un factor de riesgo para enfermedad tromboembólica venosa (ETEV). A su vez, debido a las alteraciones en la inmunidad causadas por la propia enfermedad, sumado a la inmunodepresión propia del tratamiento, estos pacientes son propensos a complicaciones infecciosas, muchas de ellas por gérmenes oportunistas como el Pneumocistis jirovecci (PJ). Los criterios diagnósticos de fiebre de origen desconocido (FOD) han estado durante largo tiempo en debate. En los últimos años se han propuesto nuevas categorías distintas a la FOD clásica, como ser la que afecta a pacientes inmunodeprimidos, donde tanto las etiologías como las formas de presentación de las mismas varía. Se han descrito más de 200 causas de FOD dentro de las cuales se incluye PJ. Se presenta el caso clínico de una paciente a la cual se le realizó diagnóstico de DM y que pese a la tromboprofilaxis presentó un episodio de tromboembolismo pulmonar. A su vez, durante la internación presentó una FOD. Luego de una búsqueda exhaustiva se aisló PJ y se realizó tratamiento para el mismo con excelente respuesta clínica.


Dermatomyositis (DM), the same as other auto-immune diseases, has been reported in several studies as a risk factor for a venous thromboembolic disease. Also, given immunity alterations caused by the disease itself, along with the immunodepression that characterizes treatment, these patients are likely to present infectious complications, many of them due to opportunistic germs such as Pneumocystis jirovecci (PJ). Diagnostic criteria for fever of unknown origin (FUO) have been long discussed. Lately, new categories that are different to the classic FUO have been proposed, as is the one affecting immunodepressed patients, where both the etiology and presentation vary. Over 200 causes for FUO have been described, PJ being among them. The clinical case of a female patient diagnosed with DM is described in the study, who, in spite of thromboprofilaxis presented a pulmonary thromboembolism episode. Likewise, during hospitalization, the patient evidenced FUO. After an extensive search, PJ was isolated and treatment was applied, resulting in an excellent clinical response.


A dermatomiosite (DM), assim como outras patologias, tem sido descrita como um fator de risco para doença tromboembólica venosa (DTEV). As alterações da imunidade causadas poresta patologia, associadas à imunodepressão própria do tratamento fazem com que os pacientes sejam propensos a complicações infecciosas, muitas delas por microrganismos oportunistas como o Pneumocistis jirovecci (Pj). Durante muito tempo os critérios diagnósticos de febre de origem indeterminada (FOI) foram causa de discussão. Nos últimos anos foram propostas novas categorias diferentes da FOI clássica, como por exemplo, a que afeta pacientes imunodeprimidos, nas quais, tanto as etiologias como as formas de apresentação variam. Mais de 200 causas de FOI, incluindo Pj, foram descritas. Apresenta-se o caso clínico de uma paciente diagnosticada com DM e que, apesar da profilaxia tromboembólica, apresentou um episódio de tromboembolismo pulmonar. Durante a internação apresentou FOI; Pj foi isolado depois de uma exaustiva busca. A paciente recebeu o tratamento correspondente com excelente resposta clínica.


Asunto(s)
Humanos , Dermatomiositis , Embolia Pulmonar , Fiebre de Origen Desconocido , Pneumocystis carinii
16.
Rev. méd. Chile ; 141(6): 797-802, jun. 2013. ilus
Artículo en Español | LILACS | ID: lil-687212

RESUMEN

Gemcitabine is a widely used drug in the treatment of advanced pancreatic cancer and other malignancies. It is generally well tolerated and exceptionally its use has been associated with hemolytic-uremic syndrome, causing acute kidney injury, hipertension, chronic renal failure requiring dialysis, and death. We report a 60-year-old man with pancreatic carcinoma and regional lymph node invasion, whom after four months of therapy with gemcitabine and after dose number 11, suddenly developed an acute nephritic syndrome with moderate renal impairment, associated with severe anemia (hemoglobin 6.0 g/dL) and thrombocytopenia (20,000 mm³). Renal biopsy showed the classic findings of thrombotic micro angiopathy Gemcitabine was discontinued and renal function and hematological parameters gradually improved.


Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Antimetabolitos Antineoplásicos/efectos adversos , Desoxicitidina/análogos & derivados , Síndrome Hemolítico-Urémico/inducido químicamente , Antimetabolitos Antineoplásicos/uso terapéutico , Desoxicitidina/efectos adversos , Desoxicitidina/uso terapéutico , Neoplasias Pancreáticas/tratamiento farmacológico
17.
Rev. méd. Chile ; 141(3): 381-387, mar. 2013. ilus
Artículo en Español | LILACS | ID: lil-677348

RESUMEN

Celiac disease may be associated with other autoimmune diseases and exceptionally with glomerulopathies and nephrotic syndrome. Associations have been reported with IgA nephropathy, membranoproliferative glomerulonephritis, membranous glomerulopathy and minimal change disease. We report a 63-year-old woman who simultaneously presented with massive nephrotic syndrome (proteinuria 46 g/day) and cachexia due to a malabsorption syndrome secondary to celiac disease. The course of her diseases was complicated with cardiomyopathy due to severe malnutrition, septic shock, acute kidney injury that required dialysis for seven weeks and severe hypertension. A renal biopsy showed a membranoproliferative pattern of injury secondary to a thrombotic microangiopathy and diffusepodocyte damage. Fouryears later, the patient was in good general health, the glomerular filtration rate was 30 ml/min/1.73m² and there was non-nephrotic proteinuria.


Asunto(s)
Femenino , Humanos , Persona de Mediana Edad , Lesión Renal Aguda/complicaciones , Enfermedad Celíaca/complicaciones , Glomerulonefritis/complicaciones , Síndrome Nefrótico/complicaciones , Microangiopatías Trombóticas/complicaciones , Lesión Renal Aguda/patología , Enfermedad Celíaca/patología , Glomerulonefritis/patología , Síndrome Nefrótico/patología , Microangiopatías Trombóticas/patología
18.
Arch. med. interna (Montevideo) ; 35(1): 19-22, mar. 2013. ilus
Artículo en Español | LILACS | ID: lil-754128

RESUMEN

Resumen: Arch Med Interna 2013 - 35(1):19-22 La estenosis de la válvula pulmonar es una patología infrecuente constituyendo entre 8 y 10% de las cardiopatías congénitas. Se clasifica según sus características anatómicas en: típica, con displasia leve/moderada y con displasia severa. Su severidad se mide según el gradiente de presión siendo: leve < 30 mmHg, moderada entre 30 y 80 mmHg y severa > 80 mmHg. La valvuloplastia pulmonar percutánea es el procedimiento de elección en todas las edades y en todos las variantes anatómicas. Está indicada en casos de estenosis moderada a severa o en presencia de sintomatología, siendo menos efectiva en casos con válvulas displásicas. Es una técnica segura y efectiva con baja incidencia de mortalidad y complicaciones. En caso de ser ineficaz está indicada la cirugía. Los aneurismas de la arteria pulmonar presentan una baja prevalencia, estando descritos únicamente casos clínicos aislados y pequeñas series de casos lo que no permite estimar su prevalencia. Generalmente se presentan en pacientes asintomáticos o con síntomas inespecíficos y el diagnóstico se realiza como hallazgo en estudios de imagen. Pueden ser congénitos o adquiridos, asociando los congénitos otras malformaciones cardiovasculares. Los adquiridos son secundarios a: hipertensión pulmonar ya sea primaria o secundaria; estenosis valvular pulmonar; vasculitis; infecciones; neoplasias o iatrogénicas. La complicación más temida es la rotura del aneurisma con una elevada mortalidad. El tratamiento médico es limitado, atendiendo al control de la hipertensión pulmonar y a la etiología causal en el caso de los secundarios. El tratamiento quirúrgico queda reservado para los aneurismas inestables, con hemoptisis, o aneurismas mayores a 60 mm.


Abstract: Arch Med Interna 2013 - 35(1):19-22 The Pulmonary Artery Aneurysm is a rare condition that accounts for 8 to 10% of all congenital heart diseases. It is classified according to its anatomy as typical, with mild/moderate dysplasia and with severe dysplasia. Severity is defined based on the pressure gradient, being: mild < 30 mmHg, moderate between 30 and 80 mmHg and severe > 80 mmHg. The percutaneous pulmonary valve repair is a safe and effective technique that entails a low incidence of complications and a low mortality rate. It is the procedure of choice at all ages, regardless of the anatomical type. It is indicated in cases of moderate to severe stenosis or in the presence of symptoms, being less effective in cases that occur with dysplastic valves. Surgery is indicated when the percutaneous route has failed. Pulmonary artery aneurysms have a low prevalence; being there only isolated reports and small case series, their prevalence is difficult to estimate. They usually occur in patients that are either asymptomatic or presenting with non specific symptoms; diagnosis typically results from incidental imaging findings. These aneurysms may be congenital or acquired, being the former associated to additional cardiovascular malformations. Acquired PA aneurysms may be secondary to pulmonary hypertension, which may in turn be primary or secondary to episodes of pulmonary thromboembolism, pulmonary valve stenosis, vasculitis, infections, malignancies or resulting from iatrogenia. The most feared complication of the aneurysm is its rupture, a highly lethal event. Medical therapy is limited, and requires keeping pulmonary hypertension under control and acting upon the root cause in the secondary cases. Surgical therapy is reserved only to unstable aneurysms, patients with haemoptysis, or aneurysms over 60.

19.
Rev. méd. Chile ; 140(12): 1580-1584, dic. 2012. ilus
Artículo en Español | LILACS | ID: lil-674030

RESUMEN

Nephrotic syndrome secondary to paraneoplastic glomerulopathies is exceptional. We are aware of only three cases reported of cervical carcinomas associated with nephrotic syndrome. Two women, aged 40 and 79 years, presented with nephrotic syndrome. The first had a membranous nephropathy and the second was not biopsied. The first women had a metrorrhagia after 8 months of unsuccessful therapy with corticosteroids and immunosuppressive drugs. An advanced cervical carcinoma with lymph node metastases was found. In the second patient, a cervical carcinoma and hematometra was discovered two months after diagnosis ofa nephrotic syndrome. The syndrome subsided completely, nine months after radiotherapy and chemotherapy in the first patient and 10 months after hysterectomy in the second patient.


Asunto(s)
Adulto , Anciano , Femenino , Humanos , Carcinoma/terapia , Síndrome Nefrótico/terapia , Neoplasias del Cuello Uterino/terapia , Carcinoma/complicaciones , Glomerulonefritis Membranosa/etiología , Síndrome Nefrótico/etiología , Síndromes Paraneoplásicos/etiología , Inducción de Remisión/métodos , Neoplasias del Cuello Uterino/complicaciones
20.
Arch. med. interna (Montevideo) ; 34(3): 80-87, dic. 2012. tab
Artículo en Español | LILACS | ID: lil-754120

RESUMEN

El ataque cerebrovascular hemorrágico o hemorragia cerebral no traumática es una importante causa de mortalidad y morbilidad a nivel mundial y constituye el efecto adverso más temido de la terapia anticoagulante. Cuando ésta ocurre es significativamente más letal que otras hemorragias constituyendo el 90% de las muertes ocasionadas por sangrados en pacientes que reciben warfarina. La enfermedad tromboembólica es una complicación frecuente en los pacientes que han sufrido un ataque cerebrovascular lo que determina que la profilaxis y tratamiento de eventos tromboembólicos versus el riesgo de recurrencia de sangrado intracraneano constituya un verdadero desafio clínico-terapéutico. El objetivo de esta revisión es analizar la importancia de la enfermedad tromboembólica en los pacientes con hemorragia cerebral, la tromboprofilaxis y el manejo de los pacientes tratados con anticoagulantes orales y la oportunidad de reinicio de los mismos luego de una hemorragia cerebral reciente.


ABSTRACT: Arch Med Interna 2012 - 34(3):80-87 Hemorrhagic stroke or non traumatic cerebral hemorrhage is a significant cause of morbi-mortality globally, and it is the most dreaded adverse effect of anticoagulant therapy. When it does occur, it is significantly more lethal than other hemorrhages, and it accounts for 90% of the deaths that result from bleeding in patients receiving warfarin. Thromboembolic disease is a frequent complication in patients with stroke; therefore, balancing the prophylaxis and therapy of thromboembolic events with the risk of recurrence of intracranial bleeding becomes a true clinical and therapeutic challenge. The objective of this review is to discuss the importance of thromboembolic disease in patients presenting with cerebral hemorrhage, thromboprophylaxis and the management of the patients treated with oral anticoagulants, as well as the timing for resuming the administration of such agents after a recent cerebral hemorrhage.

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