ABSTRACT
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?
Subject(s)
Humans , Male , Middle Aged , Retroperitoneal Fibrosis/complications , Retroperitoneal Fibrosis/diagnosis , Retroperitoneal Fibrosis/therapy , Recurrence , Time Factors , Tomography, X-Ray Computed , Diagnosis, Differential , Hypothyroidism , Immunosuppressive Agents/therapeutic useABSTRACT
Background: Fetal arterial and venous doppler studies help in identification of the foetuses at risk for perinatal complications and may help in prediction of the fetal acid base status or neonatal complications. Aim: To estimate various color doppler indices in low and high risk preterm pregnancies. Material and methods: Study comprised of 60 patients which was an observational,descriptive hospital based study. The study was divided into 2 groups: High risk group (n=30) and Low risk group (n=30). Color doppler scanners (Philips envisor HD7, HD9 and GE logic P5 ultrasound machine), wasused with 3.5 Mhz curvilinear array and following Doppler Velocimetry were assessed- Uterine artery, Umbilical artery, Middle cerebral artery, Umbilical vein, Uterine vein and Ductus Venosus. Results: Gestational age (in weeks)at examination was (31.89+2.69) in low rsk group as compared to (29.25+1.96) in high risk group. Gestational age (in weeks) at delivery was (36.2+1.78) in low risk group as compared to (29.83+1.86) in high risk group. In low risk group uterine artery doppler show decrease in PI, RI and S/D ratio with increasing gestation as compared to increasing PI,RI and S/ratio were seen (p<0.001). Conclusion: Doppler investigation of the fetal circulation play an important role in monitoring high risk pregnancies and thereby help to determine optimal time for delivery. Hence, the use of doppler provides information that is not readily obtained from more conventional test for fetal wellbeing. Therefore it has a role to play in management of high risk pregnancies.