New therapeutics based on emerging concepts in pulmonary fibrosis

Introduction: Fibrosis is an irreversible pathological endpoint in many chronic diseases, including pulmonary fibrosis. Idiopathic pulmonary fibrosis (IPF) is a progressive and often fatal condition characterized by (myo)fibroblast proliferation and transformation in the lung, expansion of the extracellular matrix, and extensive remodeling of the lung parenchyma. Recent evidence indicates that IPF prevalence and mortality rates are growing in the United States and elsewhere. Despite decades of research on the pathogenic mechanisms of pulmonary fibrosis, few therapeutics have succeeded in the clinic, and they have failed to improve IPF patient survival.

Areas covered: Based on a literature search and our own results, we discuss the key cellular and molecular responses that contribute to (myo)fibroblast actions and pulmonary fibrosis pathogenesis; this includes signaling pathways in various cells that aberrantly and persistently activate (myo)fibroblasts in fibrotic lesions and promote scar tissue formation in the lung.

Expert opinion: Lessons learned from recent failures and successes with new therapeutics point toward approaches that can target multiple pro-fibrotic processes in IPF. Advances in preclinical modeling and single-cell genomics will also accelerate novel discoveries for effective treatment of IPF.     

Abstracts

Abstract

The efficacies of four bowel care regimens (bisacodyl suppositories, glycerin suppositories, mineral oil enemas and docusate sodium mini-enemas) were compared in seven subjects with traumatic spinal cord injury. Efficacy was assessed in terms of colonic transit time, bowel evacuation time and subjective responses to a questionnaire. Both docusate sodium mini-enemas and mineral oil enemas decreased total and left-sided colonic transit time. However, docusate sodium mini-enemas were superior to mineral oil enemas in terms of the decrease in bowel evacuation time and symptom reduction. Results in this small group of subjects suggest that docusate sodium mini-enemas may have advantages in the management of bowel evacuation in individuals with spinal cord injury. (J Spinal Cord Med 1998;21 -24)     

Echocardiography in adult patients with PDA: A simplified approach

Patent ductus arteriosus is a common congenital heart anomaly in the newborn and children. It is often diagnosed and treated in childhood; however, there are a subset of patients who survive to adulthood undiagnosed, and it is present in adulthood with various clinical presentations. The clinical presentation, hemodynamics, and management of PDA presentation in adults depend on various factors, primarily the size of PDA, magnitude of shunting, and status of pulmonary vasculature. Echocardiography is often the primary imaging modality in the evaluation of adult patients with PDA and is an important tool that provides diagnostic and hemodynamic assessment for the initial evaluation and routine follow-up after PDA closure. In this review, we present a simplified approach of basic echocardiographic assessment of various types of PDA presentations in adults.     

A Unique Association of Unilateral Idiopathic Calcinosis Cutis with Ipsilateral Porokeratotic Eccrine Ostial and Dermal Duct Nevus

An 11‐year‐old boy presented with complaints of multiple skin‐colored hard lumps on the right side of his body and progressive deformity of the right leg of 7‐years duration. His parents had also noticed multiple asymptomatic pits over his right arm, palms, and soles since childhood. Examination revealed skin‐colored nontender nodules on the right half of his body and shortening of his right leg. The multiple hyperpigmented pits over the right arm, palm, and sole raised diagnostic difficulties, but histopathologic, radiologic, and biochemical investigations confirmed the features of idiopathic calcinosis cutis and porokeratotic eccrine ostial and dermal duct nevus. Unilateral idiopathic calcinosis cutis has not been previously reported in the literature, and the association with ipsilateral porokeratotic eccrine ostial and dermal duct nevus makes this case unique. Diagnostic difficulties and limited options for treatment make this case interesting academically.