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1/24. Disseminated intravascular meconium in a newborn with meconium peritonitis.

    A 3-day-old premature infant with meconium peritonitis, periventricular leukomalacia, and pulmonary hypertension died with respiratory insufficiency. An autopsy disclosed intravascular squamous cells in the lungs, brain, liver, pancreas, and kidneys. Numerous pulmonary capillaries and arterioles were occluded by squamous cells, accounting for pulmonary hypertension. brain parenchyma surrounding occluded cerebral vessels showed infarct and gliosis. A mediastinal lymph node filled with squamous cells alluded to the mechanism by which these cells from the peritoneal cavity likely entered the bloodstream--namely, via diaphragmatic pores connecting with lymphatics. Thus, disseminated intravascular meconium rarely may complicate meconium peritonitis and have devastating consequences.
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2/24. A case of protein-losing enteropathy caused by intestinal lymphangiectasia in a preterm infant.

    Intestinal lymphangiectasia is characterized by obstruction of lymph drainage from the small intestine and lacteal dilation that distorts the villus architecture. Lymphatic vessel obstruction and elevated intestinal lymphatic pressure in turn cause lymphatic leakage into the intestinal lumen, thus resulting in malabsorption and protein-losing enteropathy. Intestinal lymphangiectasia can be congenital or secondary to a disease that blocks intestinal lymph drainage. We describe the first case of intestinal lymphangiectasia in a premature infant. The infant presented with peripheral edema and low serum albumin; high fecal concentration of alpha(1)-antitrypsin documented intestinal protein loss. endoscopy showed white opaque spots on the duodenal mucosa, which indicates dilated lacteal vessels. histology confirmed dilated lacteals and also showed villus blunting. A formula containing a high concentration of medium chain triglycerides resulted in a rapid clinical improvement and normalization of biochemical variables. These features should alert neonatologists to the possibility of intestinal lymphangiectasia in newborns with hypoalbuminemia and peripheral edema. The intestinal tract should be examined for enteric protein losses if other causes (ie, malnutrition and protein loss from other sites) are excluded. The diagnosis rests on jejunal biopsy demonstrating dilated lymphatic lacteal vessels.
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3/24. Segmental arterial mediolysis in a preterm.

    We firstly report on a dystrophic preterm infant with segmental arterial mediolysis (SAM) found in arteries of placental, umbilical and cerebral tissues. These arterial lesions of unknown etiology developing in the elderly are characterized by segmental lysis of the abdominal splanchnic arteries followed by aneurysms and acute bleeding. Typically, the lesions occur in a skip pattern. We could find a small number of SAM in the spleen but much more in placental and umbilical tissues. Rarely, a vascular elastosis and splitting of individual vessels in the spleen and lung could be detected. The histological findings are similar to that of adult patients.
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4/24. Management of infiltration injury in neonates using duoderm hydroactive gel.

    Neonates are at increased risk of injury from infiltration of intravenous fluids because of small vessel size and immature skin structure. Until recently, hyaluronidase injection was used to prevent tissue injury following the infiltration of intravenous solutions in neonates. The production of hyaluronidase injection was discontinued in 2001. The alternative, compounded hyaluronidase injection is not regulated by the U.S. food and Drug Administration and is subject to variation in quality assurance practices. Amorphous hydrogels have been used as wound dressings for sloughy or necrotic wounds in a variety of clinical settings. hydrogels facilitate autodebridement of the wound by rehydrating slough and enhancing the rate of autolysis. No adverse effects or increased infection rates have been associated with the use of hydrogel dressings. DuoDerm Hydroactive gel is a sterile, preservative-free, viscous, hydrating gel composed of natural hydrocolloids. We report our experience using DuoDerm Hydroactive gel for management of injury secondary to the infiltration of total parenteral nutrition solution and lipid emulsion in three neonates.
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5/24. Neonatal microscopic polyangiitis secondary to transfer of maternal myeloperoxidase-antineutrophil cytoplasmic antibody resulting in neonatal pulmonary hemorrhage and renal involvement.

    BACKGROUND: microscopic polyangiitis is a systemic vasculitis characterized by small vessel involvement. Studies suggest myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) is involved in its pathogenesis, and the titer may reflect disease activity. OBJECTIVE: To report a case of transplacental transfer of MPO-ANCA from a mother to a 33-week gestational age neonate that resulted in neonatal pulmonary hemorrhage and renal involvement that was successfully treated with high-dose steroid therapy and exchange transfusion. methods: MPO-ANCA titers from the cord blood and the neonate on the 8th, 15th, and 25th days of life (DOLs) were obtained. Metabolic panels and chest x-ray examinations were performed for the neonate and mother and the following values were measured: ANCA, erythrocyte sedimentation rate, c-reactive protein, antinuclear antibody, serial urinalysis, and complete blood cell count. Anti-glomerular basement membrane, quantitative immunoglobulins, anticardiolipin antibody, and rheumatoid factor were also measured for the neonate. RESULTS: The neonate had elevated MPO-ANCA titers at birth. Pulmonary hemorrhage and renal involvement were seen on DOL 2. High-dose steroid therapy decreased symptoms within 1.5 hours of initiation. Exchange transfusion performed on DOL 5 removed all of the remaining MPO-ANCA by DOL 25. The child remains asymptomatic to date. CONCLUSIONS: To our knowledge, this is the first reported case of transplacental transfer of MPO-ANCA resulting in pulmonary-renal syndrome that was successfully treated with high-dose steroid therapy and exchange transfusion.
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6/24. air embolism and pulmonary interstitial emphysema in a preterm infant with hyaline membrane disease.

    A preterm infant with severe hyaline membrane disease requiring extreme mechanical ventilation developed pulmonary air leaks with consecutive shock. The chest roentgenogram showed bilateral pulmonary interstitial emphysema and gas within the heart silhouette as well as in the hepatic veins, inferior v. cava, portal vein, and many abdominal vessels. The respiratory and circulatory failure by massive systemic gas embolism resulted in death.
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7/24. cardiac tamponade and hydrothorax as complications of central venous parenteral nutrition in infants.

    The recent use of soft catheters made of silastic polymeric silicone or polyurethane has decreased the incidence of perforation of great veins or right-sided heart chambers but has not eliminated it as had been hoped. Two premature infants who presented with cardiac tamponade more than 24 hr after the insertion of a 23-gauge silastic catheter for total parenteral nutrition (TPN) administration are described. In one infant, bilateral hydrothorax preceded the occurrence of cardiac tamponade. Osmotic injury to great vessels and/or myocardium seems to be the common mechanism but which these complications of intraluminal catheters are produced. review of the pediatric literature revealed a proportion of low birth weight infants among the reported cases. Despite a prohibitively high mortality rate, early recognition can prevent a fatal outcome.
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8/24. malassezia furfur--disseminated infection in premature infants.

    Three infants, born prematurely, died after clinical illnesses of 67, 65, and 60 days from infection by malassezia furfur. Each infant had been nourished with lipid emulsions delivered through deep-line catheters. The infections, all discovered at autopsy, were characterized by massive involvement of lungs. Two of the three had endocardial vegetations containing M. furfur; all three had lesions in liver, kidney, and spleen, and two had lesions in adrenal, pancreas, and colon. In addition, one of the infants had acute meningoencephalitis caused by M. furfur. In some of the distant organs, yeast cells of M. furfur were growing in the lumina of small vessels, filling the lumina, but causing no vasculitis or infarction. In addition to these benign collections of yeasts within vessels, there were acute inflammatory lesions as well. These were consolidation, vasculitis, granulomatous inflammation, septic thrombosis, and septic infarction of lung and foci of necrosis and inflammation in kidney and liver. Two previously reported autopsies described neonates with lesions in lung and heart. The authors' three cases for which autopsies were performed had lesions in lung and heart too but, in addition, had dissemination with acute lesions in kidney and liver. Finally, one patient had a severe meningoencephalitis caused by M. furfur.
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9/24. Systemic to pulmonary shunting masquerading as patent ductus arteriosus: a pitfall in clinical diagnosis.

    Patent ductus arteriosus (PDA) frequently complicates the course of respiratory distress syndrome in premature infants. A 920-g, 28 weeks' gestation infant with clinical and radiographic evidence of left to right shunting, was presumed to have a patent ductus arteriosus. Following ductal ligation the signs persisted. Aortic angiogram demonstrated multiple collateral vessels arising from the systemic circulation and communicating with pulmonary vessels. Accurate diagnosis of left to right shunting is imperative if such collateral shunting is not to go undiagnosed.
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10/24. Systemic air embolism in the respiratory distress syndrome of the newborn.

    Details of 25 cases of neonatal systemic air embolism, including three of our patients, are reviewed. This rare complication of respiratory distress syndrome (RDS) was observed in 21 premature infants and 4 fullterm newborns, of whom 23 required intermittent positive pressure ventilation (IPPV). In 21 of these patients air embolism was preceded by pulmonary interstitial emphysema, pneumomediastinum, pneumothorax and/or pneumopericardium. air embolism occurred between 3 and 288 hours after delivery. The outcome was lethal in 24 cases, only one patient survived. Two main pathogenic mechanisms are discussed; entrance of air from ruptured alveoli into the lung capillaries and introduction of air via catheters in umbilical vessels.
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