ABSTRACT
INTRODUCTION: Development of pulmonary artery hypertension (PAH) worsens the prognosis of systemic sclerosis (SSc). There is paucity of data on PAH in patients with SSc in India. We have attempted to determine the prevalence and predictors of pulmonary artery hypertension in systemic sclerosis using noninvasive cardiopulmonary evaluation. OBJECTIVES: (1) To study the prevalence of PAH in SSc (2) To study the predictors of PAH in SSc (gender, age of onset of disease, duration of disease, extent of skin involvement, digital infarcts/ulcer, interstitial lung disease. MATERIAL AND METHODS: Clinical and functional characteristics of 100 patients of systemic sclerosis who had undergone screening echocardiography to detect pulmonary artery hypertension were studied. RESULTS: PAH was found in 32% patients on 2D-echocardiography. Prevalence of PAH did not differ between patients with limited cutaneous SSc (lcSSc) and patients with diffuse cutaneous SSc (dcSSc). On multiple logistic regression analysis, none of the studied variables was found to be independent predictor of PAH in SSc. CONCLUSION: PAH in SSc occurs in significant proportion of patients without any "red flag signs" in early stages. Non-invasive screening of patients with SSc for PAH will help in early diagnosis and appropriate timely therapeutic intervention before significant end-organ damage occurs.
Subject(s)
Adult , Age of Onset , Cohort Studies , Cross-Sectional Studies , Echocardiography , Female , Forecasting , Humans , Hypertension, Pulmonary/epidemiology , India/epidemiology , Logistic Models , Male , Middle Aged , Prevalence , Pulmonary Artery , Scleroderma, Systemic/complications , Sex Factors , Tricuspid Valve Insufficiency/epidemiologyABSTRACT
Autosomal Recessive Polycystic Kidney (ARPKD) is a very rare entity (1 in 15,000 live births) and mostly not compatible with life. Early diagnosis and genetic councelling may help prevent such births. Two interested cases are presented.