Renal cystic diseases (RCDs) can arise from utero to early adulthood and present with a variety of symptoms including renal, hepatic, and cardiovascular manifestations. It is well known that common RCDs such as autosomal polycystic kidney disease and autosomal recessive kidney disease are linked to genes such as PKD1 and PKHD1, respectively. However, it is important to investigate the genetic pathophysiology of how these gene mutations lead to clinical symptoms and include some of the less-studied RCDs, such as autosomal dominant tubulointerstitial kidney disease, multicystic dysplastic kidney, Zellweger syndrome, calyceal diverticula, and more. We plan to take a thorough look into the genetic involvement and clinical sequalae of a number of RCDs with the goal of helping to guide diagnosis, counseling, and treatment.

A marked increase in the morbidity and mortality of a large number of broncho-pulmonary diseases has been documented in relation to smoking. The influence of tobacco smoking on various respiratory conditions. is discussed: incidence, severity or natural history modification of some respiratory illnesses: obstructive lung diseases (COPD, asthma), lung cancer, bacterial, viral respiratory infections, with the impact of smoking on tuberculosis. Finally, the relationship of tobacco with diffuse interstitial lung disease: protective role of smoking (controversial in sarcoidosis, real in hypersensitivity pneumonitis). The benefits of smoking cessation are described.

OBJECTIVE: This study was conducted to evaluate the value of sonographic B-lines (previously called \"comet tail artifacts\") in assessment of pulmonary hypertension in patients with interstitial lung diseases.
METHODS: One hundred thirty-four patients with clinically diagnosed interstitial lung diseases complicated by pulmonary hypertension underwent transthoracic lung sonography and Doppler echocardiography for assessment of the presence of B-lines, the distance between them, and the pulmonary artery (PA) systolic pressure. A correlation analysis and a receiver operating characteristic curve analysis were performed.
RESULTS: All patients had diffuse bilateral B-lines. The maximum number of B-lines seen in any positive zone (not a summation) was significantly correlated with the severity of PA systolic pressure (r= 0.812; P< .0001), and a linear regression equation could be demonstrated: that is, y = 6.06 x + 17.57, where x and y represent the number of B-lines and PA systolic pressure, respectively. A cutoff of more than 4 B-lines seen in any positive zone had 89.5% sensitivity, 85.0% specificity, and 87.2% accuracy in predicting elevated PA pressure (>30 mm Hg).
CONCLUSIONS: The number of B-lines is useful in assessment of pulmonary hypertension, especially when tricuspid regurgitation and pulmonary valve regurgitation do not exist or cannot be satisfactorily measured by Doppler echocardiography.