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?

A study of maternal mortality due to non-obstetric causes

Background: Pregnancy, although being considered a physiological state, carries the risk of serious maternal morbidity and at times mortality, due to various complications that may arise during pregnancy, labour or thereafter. The existing medical condition, infection, and surgical condition which is collectively called as non- obstetric cause pre disposes a women for more complication than a non-pregnant women, so much so that it can lead to maternal mortality. Thus, Pregnancy is more vulnerable state and present study was carried out to study, analyse and review various non-obstetrics causes of death of women during pregnancy or within 42 days of termination of pregnancy in Tertiary care centre.Methods: This was an Observational study, conducted in the department of obstetrics and gynaecology, at a tertiary care hospital attached with medical college, from October 2016 to October 2018. The details of maternal deaths were collected from various departments with non- obstetric causes and analyzed.Results: The total number of deliveries in my study period was 15,208. There were 197 maternal mortality in our study period, of which 51 women died of non-obstetric causes. The most common cause of maternal mortality in our study was hepatic cause i.e. 33.33% amongst which viral hepatitis was the most common cause followed by respiratory (19.60%), infectious (15.18%), heamoglobinopathy (13.72%), cardiac (5.88%), neurological (5.88%), surgical (5.88%) causes.Conclusions: Looking into our study, maternal mortality can be reduced by identifying various different indirect medical causes which are preventable by proper pre-pregnancy evaluation for pre-existing comorbid conditions.

A case report: 46 XY-disorder of sexual development

Authors report a case of a 6-year-old child with syndromic 46, XY disorder of sexual development. From the birth patient was assigned female. Physical examination showed dysmorphic features and ambiguous external genitalia. Cytogenetic analysis of cultured peripheral blood lymphocytes revealed a male karyotype. The result of the chromosomal investigation showing male genetic sex, together with the ambivalent aspect of the external genitalia and gonads that are exclusively testes led to the diagnosis of 46, XY disorder of sexual development. The clinical management will help the child and the family deal effectively with this condition A multidisciplinary approach to this problem involving pediatricians, specialists in the field of endocrinology, genetics, surgery and psychiatry is necessary in order to reach a prompt and correct diagnosis and treatment.

A case report of HELLP syndrome.

HELLP syndrome is a serious complication in pregnancy characterized by hemolysis, elevated liver enzymes and low platelet count. This case demonstrated the importance of rapid and early diagnosis and treatment of HELLP syndrome to reduce maternal and perinatal mortality and morbidity. 26 years old, 2nd gravida with 31 weeks of gestation with severe pre-eclampsia was admitted to Department of Obstetrics and Gynecology at Civil Hospital, Ahmedabad. Patient suddenly developed epigastric pain, blood tinge urine (not frank hematuria)and decrease urine output within 24 hours of admission. Investigations revealed platelet count 44,300, serum bilirubin 12, direct bilirubin 3.44, and indirect bilirubin 8.56, SGPT 193.5 and was diagnosed as a HELLP syndrome class 1 She underwent cesarean section and there was dramatic improvement of her symptoms and all blood investigations (S. bilirubin, Platelet count) were declined to normal limit within 48 hours post operatively. HELLP syndrome, a variant of severe pre-eclampasia, if diagnosed and manage timely ensure favorable maternal and perinatal outcome.

Cardiac diseases with pregnancy - A study of maternal and fetal outcome.

Background: Total 0.2% to 4% of all pregnancies are complicated by cardiac diseases. In non western countries, rheumatic heart diseases (RHD) constitute 56-89% of the cases and congenital heart diseases only 9-19%. Aim and objectives: To review all obstetric patients with co-existing cardiac diseases admitted to a tertiary care center over a period of one year and ascertain the causes of admissions and the maternal and perinatal morbidity and mortality. Material and methods: A retrospective, analytical study of all patients with cardiac diseases who delivered over a period of January 2013 to December 2013 was conducted. A tabulated representation of the data was done. The various cardiac diseases were categorized according to their severity, NYHA category, type of pathology, the maternal and perinatal outcome was assessed and maternal mortality and perinatal mortality was recorded. Conclusion: Total 69% cardiac lesions in pregnancy were rheumatic in origin and 15% belonged to high risk category while 13.1% were NYHA type IV. Total 69% deliveries were by vaginal route Epidural analgesia preferred in LSCS of cardiac patients. Perinatal and maternal complications were observed to increase with increase in NYHA class.

Study Of Malformations And Down Syndrome In India (SOMDI) : Baroda Region.

This was a period prevalence study carried out in 4 hospitals in Baroda from October 93 to February 97 covering over 30,000 deliveries. This study, a part of the multicentric SOMDI Project, aimed at dermining the prevalence of malformations in the population and the overall risk figures for Down Syndrom (DS) as well as its maternal age specific prevalence. The hospitals chosen for the study had delivery rates such that the study in the end was expected to comprise of 50% Government i.e. poor socioeconomic strata (SES) and 50% Private sector i.e, an upper SES. Total number of births recorded were 31,775, with the Government Sector having 15,652 and the Private sector have 16,123. The total number of malformations was 651 with the overall incidence of malformation being 2.05% and the incidence the Government and private sectors being 2.57% and 1.54% respectively. The significantly lower incidence in the private sector was probably because of an upper SES and because of early detection and termination. Increasing maternal age showed a rising trend in the percentage of malformations with incidence in the age group from 15-19 years being 2.07% that at an age more than or equal to 40 being 4.92%. Still births had 6.3 times higher incidence of malformations than that in live births (10.43% in still births Vs 1.68% in live births). Malformations were found to be significantly higher in rural (3.1%) compared to urban (1.8%) populations and in children of Consanguinous (5.0%) compared to non-consanguinous marriages (2.06%). Pre terms had a significantly higher (5.6%) incidence of malformations compared to term (1.75%) babies. In male and female babies, incidence of malformations was not significantly different (2.12% and 1.75% respectively). A previous history of malformations was present in 53 incidences (out of total deliveries); out of 53, as many as 31 had a previous history of a neural tube defect (NTD) and in 2 of these there was a recurrence of NTD in this particular pregnancy. In the systemwise distribution of malformation, CNS anomalies were the most common, followed by the musculoskeletal system and gastrointestinal system. An interesting association noted was a large number of babies having a combination of midline defects viz. cleft lip and/or cleft palate and NTD and/or hyrocephalus. A total of 33 Down syndrome cases were encountered with an overall prevalence of 1.04% per 1000 and an overall risk of DS of 1 per 962 births. Maternal age specific prevalence of DS increased from 0.54/1000 at age 15-19 years to 15.6/1000 at age > 40 years. The corresponding age specific risks for DS were 1/1825 births and 1/64 births respectively.