IPLab:Lab 13:Cystic Fibrosis

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Contents

Clinical Summary

This white female infant was the product of an uncomplicated term delivery, though meconium stainingMeconium is a dark-green mucilaginous mixture of intestinal secretions and amniotic fluid which is found in the intestine of a full-term fetus. Meconium-stained amniotic fluid, found at delivery, may be an indication of perinatal asphyxia. was noted at birth. During the first day post-partum, the infant's abdomen became progressively distended and a meconium ileusMeconium ileus occurs when abnormally viscid meconium completely obstructs the ileum of a newborn. was suspected. Surgery confirmed the presence of a meconium ileusMeconium ileus occurs when abnormally viscid meconium completely obstructs the ileum of a newborn. and a section of perforated atretic jejunum proximal to the ileus was resected. Eight days later, the patient's condition had deteriorated. A second operation revealed a segment of necrotic bowel, which was removed. Subsequently the infant's pulmonary function deteriorated and she required frequent suctioning. She developed repeated episodes of pneumoniaIn alcoholics, aspiration pneumonia is common--bacteria enter the lung via aspiration of gastric contents. (E. coli and Pseudomonas grew out on cultures) complicated by atelectasisAtelectasis is the collapse of an airway and lung, regardless of the cause, resulting in reduced or absent gas exchange. secondary to pneumothoraxA pneumothorax is an accumulation of gas in the pleural space.. The patient died at 25-days-of-age in respiratory failure.

Autopsy Findings

Bilateral, extensive organizing bronchopneumonia was present with evidence of a pneumothoraxA pneumothorax is an accumulation of gas in the pleural space. and atelectasisAtelectasis is the collapse of an airway and lung, regardless of the cause, resulting in reduced or absent gas exchange.. There were significant changes in the pancreas consistent with cystic fibrosis as well as involvement of the small intestine and changes related to the surgical procedures.

Images

Virtual Microscopy

Pancreas

Duodenum

Study Questions


Additional Resources

Reference

Journal Articles

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