Studies on the Toxicity and Efficacy of a New Amino Acid Solution in Pediatric Parenteral Nutrition

Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/142223/1/jpen0368.pd

The definitive management of Hirschsprung's disease with the endorectal pull-through procedure

From 1 July 1974 to 31 August 1985, 99 patients with Hirschsprung's disease were treated at the University of Michigan Mott Children's Hospital; 75 were initially diagnosed and treated at our institution. Of these 75 patients, 55 had standard rectosigmoid disease and 20 had long-segment disease. The endorectal pull-through (ERPT) procedure was used consecutively in 73 of the 75 patients; the other two children died, one because of total intestinal aganglionosis and the other due to severe congenital anomalies, prior to a definitive procedure. Of the 24 patients referred by other institutions, 12 underwent repeat pull-through procedures and 12 required lesser operations to deal with the complications of prior procedures. There were no postoperative deaths and no permanent enterostomies among the 73 consecutive ERPT patients. The follow-up in this series ranges from 4 months to 10 years, and all patients have fecal continence with an average of 3–4 stools daily. Our treatment plan involves the use of suction rectal biopsy for diagnosis and leveling colostomy in the neonatal period. An endorectal pull-through procedure is then carried out between 8 months and 1 year of age. The excellent functional results, 0% operative mortality, low morbidity, and technical ease of performing the ERPT underscore our enthusiasm for continued use of this procedure in Hirschsprung's disease.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/47147/1/383_2004_Article_BF00166867.pd

Esophageal and tracheobronchial foreign bodies in infants and children

In an effort to improve the diagnosis and management of children with aspiration or ingestion of foreign bodies we reviewed 100 consecutive cases of esophageal (49) or tracheobronchial (51) foreign bodies occurring over a 6-year period. While the incidence of positive physical findings in the esophageal group was low, the combination of plain and contrast radiography was positive in 96% (47/49). Of the patients with tracheobronchial foreign body, 78% (40/51) had lateralizing signs on physical examination and 80% (41/51) had abnormal inspiratory/expiratory radiographs. Disimpaction of esophageal foreign bodies was carried out using a combination of techniques with 100% success and no complications. All cases of tracheobronchial foreign bodies were managed with the rigid bronchoscope with 98% success (50/51) using a variety of instruments. Complications secondary to the foreign body itself rather than its management were seen in 9 patients, and were often due to a delay in diagnosis. A careful history and physical examination along with appropriate radiographic studies will result in a correct diagnosis in virtually all cases of esophageal and tracheobronchial foreign bodies. A liberal indication for endoscopy using an approach tailored to the particular case will almost always be successful.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/47159/1/383_2004_Article_BF00175647.pd

Advances in short bowel syndrome: an updated review

Short bowel syndrome (SBS) continues to be an important clinical problem due to its high mortality and morbidity as well as its devastating socioeconomic effects. The past 3 years have witnessed many advances in the investigation of this condition, with the aim of elucidating the cellular and molecular mechanisms of intestinal adaptation. Such information may provide opportunities to exploit various factors that act as growth agents for the remaining bowel mucosa and may suggest new therapeutic strategies to maintain gut integrity, eliminate dependence on total parenteral nutrition, and avoid the need for intestinal transplantation. This review summarizes current research on SBS over the last few years.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/47168/1/383_2005_Article_1500.pd

Hirschsprung's Disease and Related neuromuscular Disorders of the Intestine

Hirschsprung's disease may present initially as perforated appendicitis, commonly in the newborn period.15,201,287 Serial biopsies of the colon are required in infants who present with such a clinical picture. In general, these perforations often denote longer lengths of aganglionosis. A great deal of controversy surrounds the use of the appendix to diagnose total colonic Hirschsprung's disease; however, histologic evaluation of the appendix is generally an excellent diagnostic tool.10 Innervation of the appendix differs slightly from that of the rest of the intestine; in cases of total colonic aganglionosis, the appendix has a paucity of nerve fibers and neuroendocrine cells.295 Evaluating the appendix at a relatively early stage in the initial surgical exploration of a child with Hirschsprung's disease often expedites the diagnosis. In one case, a child with presumed total colonic aganglionosis and aganglionosis of the appendix was subsequently found to have normal ganglion cells in the transverse colon.10 This extremely rare case represents segmental Hirschsprung's disease