Short bowel syndrome (SBS) is the most common cause of intestinal failure in infants. In neonates and young infants, necrotizing enterocolitis, gastroschisis, intestinal atresia and intestinal malrotation/volvulus are the leading causes of SBS. Following an acute post-surgical phase, the residual gastrointestinal tract adapts with reorganization of the crypt-villus histoarchitecture and functional changes in nutrient absorption and motility. A cohesive, multidisciplinary approach can allow most neonates with SBS to transition to full enteral feeds and achieve normal growth and development. In this article, we review the clinical features, management, complications, and prognostic factors in SBS.
Objective
Up to a third of all infants who develop necrotizing enterocolitis (NEC) require surgical resection of necrotic bowel. We hypothesized that the histopathological findings in surgically-resected bowel can predict the clinical outcome of these infants.
Study design
We reviewed the medical records and archived pathology specimens from all patients who underwent bowel resection/autopsy for NEC at a regional referral center over a 10-year period. Pathology specimens were graded for the depth and severity of necrosis, inflammation, bacteria invasion, and pneumatosis, and histopathological findings were correlated with clinical outcomes.
Results
We performed clinico-pathological analysis on 33 infants with confirmed NEC, of which 18 (54.5%) died. Depth of bacterial invasion in resected intestinal tissue predicted death from NEC (odds ratio 5.39 per unit change in the depth of bacterial invasion, 95% confidence interval 1.33-21.73). The presence of transmural necrosis and bacteria in the surgical margins of resected bowel was also associated with increased mortality.
Conclusions
Depth of bacterial invasion in resected intestinal tissue predicts mortality in surgical NEC.
Hemophagocytic lymphohistiocytosis (HLH) is extremely rare in the neonatal period. The incidence of neonatal HLH is not confirmed and may range from 1 in 50,000 to 150,000. The incidence varies based on ethnicity, particularly in populations in which consanguinity is common. HLH is associated with a high fatality rate and poor prognosis, making it important to recognize and diagnose it early. This review will concentrate primarily on the diagnosis and management of neonatal HLH.
Several case reports and retrospective studies have reported a temporal association between RBC transfusions and necrotizing enterocolitis. In this article, we summarize the clinical evidence and biological plausibility of the association between RBC transfusions and NEC. We also review the clinical presentation, management, and outcomes in infants with “transfusion-associated” NEC.
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