Deep vein thrombosis and pulmonary embolism in cirrhotic patients:Systematic review

Patients with liver cirrhosis were traditionally believed to be protected against development of blood clots.Lately,studies have shown that these patients may probably be at an increased risk of venous thrombotic complications.Although the hemostatic changes in the chronic liver disease patients and the factors that may predict bleeding vs thrombotic complications remains an area of active research,it is believed that the coagulation cascade is delicately balanced in these patients because of parallel reduced hepatic synthesis of pro and anticoagulant factors.Thrombotic state in cirrhotic patients is responsible for not only portal or non-portal thrombosis[deep vein thrombosis(DVT)and pulmonary embolism(PE)];it has also been associated with progression of liver fibrosis.The use of anticoagulants in cirrhosis patients is a challenging,and often a scary situation.This review summarizes the current literature on the prevalence of venous thrombosis(DVT and PE),risk factors and safety of prophylactic and therapeutic anticoagulation in patients with chronic liver disease.     

Hybrid procedure of bilateral pulmonary artery banding and bilateral ductal stenting in an infant with aortic atresia and interrupted aortic arch

We report an infant with aortic valve atresia, interrupted aortic arch, ventricular septal defect, confluent pulmonary arteries, bilateral arterial ducts, absent common carotid arteries, and anomalous coronary arteries arising from main pulmonary artery. Hybrid procedure consisting of bilateral pulmonary artery banding and bilateral arterial duct stenting was performed at 4 weeks of age. Hybrid procedure can be an alternative palliative approach in an infant with this complex cardiac anatomy. © 2014 Wiley Periodicals, Inc.     

Inclusion body myositis in the rheumatology clinic

Inclusion body myositis is a rare sporadic inflammatory‐degenerative myopathy of the elderly. Despite being the commonest type of acquired myopathy after the age of 50, misdiagnosis is extremely common. The most frequent hurdle in identifying new cases is the wrong diagnosis of polymyositis or motor neuron disease. Novel insights into pathogenic mechanisms have heralded the quest for newer therapeutics as well as drug repurposing in this otherwise progressive disorder.     

Bilateral bifid ureter with unilateral renal vasculature variations

During routine dissection of 82-year-old female cadaver with no known unfavorable medical history, we observed bilateral bifid ureter and variation in arterial supply of left kidney only. Careful examination revealed that there were bifid ureters on both sides enclosed in single facial sheath. It was also observed that both the ureter have different pattern of origin. On the right side, both the ureters were seen to be emerging from the hilum, one below another and joined together at the brim of the lesser pelvis just before crossing the right external iliac artery. Right kidney was supplied by single renal artery lying anterior to both the ureters. On the left side one ureter emerged from the hilum while the second one exited the kidney from a prominent lobule present below the inferior pole.     

Genetic Analysis in Bartter Syndrome from India

Bartter syndrome is a group of inherited, salt-losing tubulopathies presenting as hypokalemic metabolic alkalosis with normotensive hyperreninemia and hyperaldosteronism. Around 150 cases have been reported in literature till now. Mutations leading to salt losing tubulopathies are not routinely tested in Indian population. The authors have done the genetic analysis for the first time in the Bartter syndrome on two cases from India. First case was antenatal Bartter syndrome presenting with massive polyuria and hyperkalemia. Mutational analysis revealed compound heterozygous mutations in KCNJ1(ROMK) gene [p(Leu220Phe), p(Thr191Pro)]. Second case had a phenotypic presentation of classical Bartter syndrome however, genetic analysis revealed only heterozygous novel mutation in SLC12A gene p(Ala232Thr). Bartter syndrome is a clinical diagnosis and genetic analysis is recommended for prognostication and genetic counseling.