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

Trauma scores, accident deformity codes, and car restraints in children

The importance and effectiveness of the appropriate use of automobile restraints by young children has been emphasized in several studies. Once the child has entered the emergency care system, however, restraint use may not be the best predictor of injury severity. This study was undertaken to investigate the relationship of restraint status to morbidity and mortality in children examined in a hospital emergency facility following involvment in a motor vehicle crash (MVC). The emergency room charts of 101 children under 18 years of age, who were victims of MVCs, were reviewed and the following trauma scores were calculated: Glascow Coma Scale, Pediatric Trauma Score, Revised Trauma Score, Injury Severity Score, and Maximum Abbreviated Injury Score. In addition, the Traffic Accident Damage (TAD) score, an estimate of crash severity determined by the police at the accident scene, was recorded. The patients were age stratified as follows: 0 to 4 years (n = 24), 5 to 11 years (n = 29), and 12 to 17 years (n = 48). Fifty patients were appropriately restrained at the time of the crash. There was a significant correlation between mean trauma scores and mean TAD codes (P P > .05) across all age groups, and these children were grouped together as "unrestrained" in further analyses. In the 0 to 4 age group, there were no significant differences in mean trauma scores between restrained and improperly restrained or unrestrained children in contrast to the 5 to 11 and the 12 to 17 age groups. There were no significant difference between the distribution of restrained and unrestrained victims with regard to mean TAD scores (P = .5224) in the 0 to 4 age group. This study demonstrates a close correlation between mean trauma scores and vehicle deformity in all age groups, and shows that mean trauma scores appear to be independent of restraint use for the 0 to 4-year-old age subset. Therefore, a police-assigned crash severity score, such as the TAD, may be useful in the initial traige of pediatric trauma victims to an appropriate hospital or trauma center.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/31099/1/0000777.pd

Effects of lipid administration on liver apoptotic signals in a mouse model of total parenteral nutrition (TPN)

Lipids are an important component of total parenteral nutrition (TPN), contributing the largest caloric load per volume of solution and providing essential fatty acids necessary for survival. However, lipids are known to be causative factors in oxidative stress, which are expressed via the Bcl-2 family of proteins and/or Fas-mediated apoptosis in several tissues. Interestingly, we have recently observed an increase in hepatocyte apoptosis with administration of TPN. To address the mechanism of this apoptosis, we investigated the effects of parenteral lipid administration on apoptotic signaling in a mouse model. C57BL/6J male mice received physiologic saline and standard chow (control) or standard TPN solution with (TPN+L) or without lipid (TPN-L) emulsion. After 7 days of infusion, apoptosis increased in the TPN+L at a significantly higher rate compared with control and TPN-L groups ( p <0.05). Both TPN, with and without lipids, suppressed the pro-apoptotic signals Bid and Bcl-xs ( p <0.05). In contrast, TPN with lipid increased the expression of Fas and both the pro-apoptotic factor Bad and the anti-apoptotic factor Bcl-xl ( p <0.05). These changes may contribute to TPN-induced hepatocyte injury (apoptosis) or suppress the ability of liver hepatocytes to regenerate.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/47162/1/383_2003_Article_1115.pd

Quantitative imaging by pixel-based contrast-enhanced ultrasound reveals a linear relationship between synovial vascular perfusion and the recruitment of pathogenic IL-17A-F+IL-23+ CD161+ CD4+ T helper cells in psoriatic arthritis joints

To develop quantitative imaging biomarkers of synovial tissue perfusion by pixel-based contrast-enhanced ultrasound (CEUS), we studied the relationship between CEUS synovial vascular perfusion and the frequencies of pathogenic T helper (Th)-17 cells in psoriatic arthritis (PsA) joints. Eight consecutive patients with PsA were enrolled in this study. Gray scale CEUS evaluation was performed on the same joint immediately after joint aspiration, by automatic assessment perfusion data, using a new quantification approach of pixel-based analysis and the gamma-variate model. The set of perfusional parameters considered by the time intensity curve includes the maximum value (peak) of the signal intensity curve, the blood volume index or area under the curve, (BVI, AUC) and the contrast mean transit time (MTT). The direct ex vivo analysis of the frequencies of SF IL17A-F+CD161+IL23+ CD4+ T cells subsets were quantified by fluorescence-activated cell sorter (FACS). In cross-sectional analyses, when tested for multiple comparison setting, a false discovery rate at 10%, a common pattern of correlations between CEUS Peak, AUC (BVI) and MTT parameters with the IL17A-F+IL23+ - IL17A-F+CD161+ - and IL17A-F+CD161+IL23+ CD4+ T cells subsets, as well as lack of correlation between both peak and AUC values and both CD4+T and CD4+IL23+ T cells, was observed. The pixel-based CEUS assessment is a truly measure synovial inflammation, as a useful tool to develop quantitative imaging biomarker for monitoring target therapeutics in PsA. © 2016, International League of Associations for Rheumatology (ILAR)

Macrolactone Nuiapolide, Isolated from a Hawaiian Marine Cyanobacterium, Exhibits Anti-Chemotactic Activity

A new bioactive macrolactone, nuiapolide (1) was identified from a marine cyanobacterium collected off the coast of Niihau, near Lehua Rock. The natural product exhibits anti-chemotactic activity at concentrations as low as 1.3 μM against Jurkat cells, cancerous T lymphocytes, and induces a G2/M phase cell cycle shift. Structural characterization of the natural product revealed the compound to be a 40-membered macrolactone with nine hydroxyl functional groups and a rare tert-butyl carbinol residue

Dermatoglyphic patterns in children with chronic constipation

Analysis of the fine ridge configurations on the digits of the palms and soles (dermatoglyphics) may sometimes help in the diagnoses of certain medical disorders. Dermatoglyphic patterns have been reported to be associated with congenital anomalies, such as congenital heart disease, duodenal ulcer, abdominal pain, and constipation. The palmar dermatoglyphic patterns of 77 children with constipation (39 functional and 38 organic constipation) were recorded. The control group consisted of 84 children with inguinal hernia. Those patients with at least one arch identified on any digit of either hand were termed arch positive. There was no significant correlation between arch positivity and constipation (functional or organic), or inguinal hernia (chi square, P =0.9211). Therefore, the presence of palmar arches cannot be used as a screening device for children with chronic constipation, especially of organic etiology.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/44424/1/10620_2005_Article_BF02285186.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