Surviving complications of retroperitoneal fibrosis with intermittent relapses over a period of 20 years: case report and literature review / Sobrevivir a las complicaciones de la fibrosis retroperitoneal con recaídas intermitentes durante un período de 20 años: reporte de un caso y revisión de la literatura

Idiopathic retroperitoneal fibrosis is a rare fibro-inflammatory disease of varied etiology which usually originates around aorta and spreads caudally along Iliac vessels into adjacent retroperitoneum causing ureteral obstruction as the most frequent complication.A 53-year-old male patient presented with complaint of mild pain in both the legs off and on. On investigating further, we found that he had been struggling with intermittent relapses every 3-4 years for last 20 years since he was first diagnosed with Idiopathic Retroperitoneal Fibrosis. He was 33-year-old when he first developed the symptoms of anuria for 48 hours and was diagnosed with Idiopathic retroperitoneal fibrosis. This was followed by atrophy of left kidney and hypertension 6 years later, then hypothyroidism after another 3years and finally involvement of Inferior Vena Cava and acute Deep Vein Thrombosis of lower limbs after another 3-4 years. His deep vein thrombosis was well managed in time. He was put on glucocorticoids everytime he had a relapse and a complication.We did a review of literature to understand recent advances about its pathogenesis, diagnosis, investigations and management. We searched in PubMed using terms like retroperitoneal fibrosis alone and in combination with related terms such as Inferior Vena Cava thrombosis, Deep Vein Thrombosis, Tamoxifen, Methotrexate. This case is unique as it is very rare to find acute Deep Vein Thrombosis in Idiopathic retroperitoneal fibrosis without development of any collaterals when Inferior Vena Cava lumen is compromised to almost complete obstruction.After a follow up of 20 years patient is doing well in terms of physical activity and psychological wellbeing with anti-hypertensives, thyroxine and anti-coagulants. Is the disease-free interval actually free of the disease or it just subsided with immunosuppressants to become active after some time?

La fibrosis retroperitoneal idiopática es una enfermedad fibroinflamatoria rara, de etiología variada que generalmente se origina alrededor de la aorta y se propaga caudalmente a lo largo de los vasos ilíacos en retroperitoneo adyacente causando obstrucción ureteral como la complicación más frecuente.Reportamos el caso de un paciente varón de 53 años que se presentó con un dolor leve en ambas piernas. Al investigar más a fondo, descubrimos que había estado luchando con recaídas intermitentes cada 3-4 años durante los últimos 20 años desde que se le diagnosticó por primera vez fibrosis retroperitoneal idiopática. Tenía 33 años cuando desarrolló por primera vez los síntomas de anuria durante 48 horas y se le diagnosticó fibrosis retroperitoneal idiopática. Esto fue seguido por atrofia del riñón izquierdo e hipertensión 6 años después, luego hipotiroidismo después de otros 3 años y finalmente afectación de la vena cava inferior y trombosis venosa profunda aguda de las extremidades inferiores después de otros 3-4 años. Su trombosis venosa profunda se controló bien a tiempo. Le recetaron glucocorticoides cada vez que tenía una recaída y una complicación.Hicimos una revisión de la literatura para comprender los avances recientes sobre su patogenia, diagnóstico, investigaciones y manejo. Se realizaron búsquedas en PubMed utilizando términos como fibrosis retroperitoneal sola y en combinación con términos relacionados como trombosis de la vena cava inferior, trombosis venosa profunda, tamoxifeno, metotrexato. Este caso es único, ya que es muy raro encontrar trombosis venosa profunda aguda en fibrosis retroperitoneal idiopática sin desarrollo de colaterales cuando la luz de la vena cava inferior está comprometida hasta una obstrucción casi completa.Después de un seguimiento de 20 años, el paciente se encuentra bien en términos de actividad física y bienestar psicológico con antihipertensivos, tiroxina y anticoagulantes. ¿El intervalo libre de enfermedad está realmente libre de la enfermedad o simplemente disminuyó con inmunosupresores para activarse después de algún tiempo?

Primary carcinoid of posterior mediastinum: Truth or myth.

Mediastinal masses present challenging problems in thoracic practice. Most of them remain asymptomatic for long and by the time the pressure symptoms develop, these are quite advanced. Carcinoids arising from the mediastinum are invariably related to thymus. Non-thymic origin of mediastinal carcinoids is rare, especially in the posterior mediastinum. Only two cases of posterior mediastinal carcinoids have been reported so far. These were assumed to be arising from ectopic thymus tissue. We report a case of a 45-year-old woman who presented with dyspnoea and dry cough due to giant carcinoid tumour of the mediastinum, the pedicle originating from the posterior mediastinum, not related to thymus. She underwent thoracotomy and resection that provided relief. The immunochemical studies revealed positive reaction to cytokeratin, chromogranins and synaptophysin, and negative reaction to S100, CD99 (MIC2) confirming the tumour being neuroendocrine in nature.

Serological prevalence of antibodies to human immunodeficiency virus (HIV) and hepatitis B virus (HBV) among healthy Nepalese males--a retrospective study.

A retrospective data analysis for sero-prevalence of antibodies to HIV, HBV and syphilis was carried out during the period 3 notrd September 2003 to 3rd June 2004 in 627 apparently healthy Nepalese males aged 17 to 48 years and association of the diseases with age group and blood group were analyzed. The study showed the prevalence of HIV, HBV and syphilis being 1.6%, 2.7% and 0.6% respectively. Analysis of blood group showed that 97.76% of total male population having Rhesus positive blood. Among them, O "positive" was the most common blood group with 31.9% followed by A "positive", B "positive" and AB "positive" respectively. The analysis of relationship showed a tendency of high affinity of those diseases in the subjects with O "positive" blood group. However, no real association of those infections was found with the blood group (HIV: X2=0.902, P=0.99; HBsAg: X2=1.212, P=0.99; RPR: X2=3.975, P=0.789).