A young female presented with evidence of right-sided heart failure and was subsequently found to have significant pulmonary artery hypertension (PAH). Because of her normal left ventricular function and pulmonary capillary wedge pressure, the most probable site of etiology seemed to be the pulmonary vasculature. All the common possible secondary causes of PAH were ruled out, but during the investigations, she was found to have elevated thyroid function tests compatible with the diagnosis of Grave's disease. The treatment of Grave's disease, initially by medications and subsequently by radioiodine therapy, was associated with a significant reduction in the pulmonary artery systolic pressure. The purpose of this case report is to highlight one of the unusual and underdiagnosed presentations of Grave's disease.
SUMMARYA 76 -year-old lady with a recent diagnosis of rheumatic heart disease (RHD), and a history of repeated lower respiratory tract infections, came with symptoms of gastritis unrelated to the primary disease but further diagnostic study in the hospital revealed poorly controlled atrial fibrillation, grossly dilated left atrium with two large left atrial thrombi and mitral valve area<1 cm 2 . It was decided that the best approach in our patient would be mitral valve replacement with mechanical prosthesis. Despite the usual trend of using bioprosthesis in the elderly, our decision was influenced by the fact that the patient would need chronic anticoagulation for atrial fibrillation in any case. The purpose of our case presentation is to illustrate a late-presenting case of RHD with unusual associations and the challenges to choose the best possible management.
BACKGROUND
Background: Pregnancy-related acute kidney injury (PRAKI) remains a large public health problem, with decreasing incidences in developing countries like India. However, some single centred studies from United States and Canada revealed an increasing incidence of PRAKI. This increase could be due to higher rates of hypertensive disorders of pregnancy.Methods: To assess the management and outcome of PRAKI. In this prospective, observational study, total 1021 cases of acute renal failure were observed.Results: 96 (9.4%) were of obstetric origin and enrolled as per inclusion criteria. Regarding management of PRAKI, 78 out of 96 (81.25%) required haemodialysis. 67 (69.79%) among them were managed with intermittent haemodialysis (IHD) while 10 (10.41%) who had hypotension at presentation were dialysed with slow, low efficiency dialysis (SLED). Continuous renal replacement therapy (CRRT) was done in 1 (10.4%) patient. Maternal mortality in this PRAKI study was 19 of 96 patients (19.79%). Sepsis accounted for 52.63% of deaths. Foetal death was observed in 58 out of 96 patients (60.41%) comprising of intrauterine death in 55 (55.29%) and abortion in 3 (3.13%) patients. 38 of 96 (39.58%) patients gave birth to live born child out of which 27 were at full term and 11 were preterm.Conclusions: In order to avoid further increase in PRAKI in India, treating obstetrician should remain aware of management and outcome of PRAKI. The better awareness of diagnosis and management protocols will ultimately lead to further reduction in prevalence of PRAKI in our country.
Background: Atypical Haemolytic Uremic Syndrome (aHUS) is a genetic or acquired disorder of regulatory component of the complement system. It is associated with mutations in genes coding for complement components. The abnormality in components of complement makes it susceptible and predispose to chronic uncontrolled hyperactivation of the alternative complement pathway, which results in endothelial damage and microvascular thrombosis. This case report describes a patient diagnosed with Thrombotic Microangiopathy (TMA) due to factor H autoantibody having haemolytic anemia, thrombocytopenia and acute kidney injury. Patient's anemia and renal parameters improved after treatment with plasma exchange therapy. Conclusion: Atypical HUS must be strongly suspected in any patient who presents with nonspecific abdominal or respiratory symptoms along with anemia and thrombocytopenia. As extrarenal involvement is a rare entity of aHUS, the clinician should also keep a high index of suspicion to the possibility of thrombotic microangiopathy manifestation in almost any organ system. In a suspected or diagnosed case of aHUS, the development of new non renal symptoms and signs should prompt clinician for further evaluation to rule out ongoing thrombotic microangiopathy process.
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