Immaturity of Ganglion Cells - A Study of Our Own Material

Markiewicz-Kijewska, Małgorzata; Kowalski, Adam; Bacewicz, Ludmiła; Drewniak, Tomasz; Ismail, Hor; Kluge, Przemysław; Kaliciński, Piotr

doi:10.2478/v10035-009-0013-1

Cited by 3 publications

(5 citation statements)

References 13 publications

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“…This was slightly similar to the study done by Mallick et al [24], who reported 3/8 patients (37.5%) with delayed meconium passage. Meanwhile, Markiewicz-Kijewska et al [25] reported 12/15 (80%) cases with this presentation. Our study included 2/8 (25%) cases who underwent pull-through surgery and 5/8 (62.5%) who underwent stoma formation.…”

Section: Discussionmentioning

confidence: 85%

Diagnostic utility of Bcl-2 immunohistochemical expression in pediatric functional bowel obstruction cases with ganglionated specimens

et al. 2022

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Background Functional disturbances of the gastrointestinal tract are caused by a number of neurodysplastic conditions, including diseases that are rarer than Hirschsprung’s disease (HSCR), such as ganglion cell immaturity and intestinal neuronal dysplasia (IND). Bcl-2 shows positive immunoreactivity in degenerative and immature ganglion cells (IGCs). This work evaluates the implication of the immunohistochemical expression of Bcl-2 in cases of pediatric intestinal pseudo-obstruction (IPO) with ganglionated specimens. This 2-year prospective observational study was conducted from January 2017 to December 2019 on all intestinal specimens for cases referred from a pediatric surgery department with clinical data suggesting IPO (abdominal distension > 3 months, intermittent bilious vomiting, no radiological evidence of the transitional zone of HSCR or evidence of mechanical obstruction). The exclusion criteria were patients with IPO related to myopathic disorders, aganglionic biopsies, and specimens with inadequate tissue blocks. The same number of intestinal specimens of cases without IPO was used as a control group. All specimens underwent a histologic examination of ganglion cells and nerve bundles and were also evaluated for Bcl-2 immunohistochemical expression. Results Twenty-one specimens were analyzed, including six colonic resection specimens and 15 intestinal biopsies taken by full-thickness transanal biopsy (n = 12) or incisional biopsy from inadequately functioning stomas (n = 3). The mean ± standard deviation age of the patient cohort was 22 ± 7.4 months (range, 19–153 months), and there were 13 (61.9%) male patients. Bcl-2 protein was strongly positive in 57.1% of the cases, weakly positive in 19%, and negative in 23.8% as well as in all 21 control specimens. A highly significant correlation was observed between Bcl-2 expression and ganglion cell number (P < 0.001), where all hyperganglionic specimens showed strong positivity compared with the hypoganglionic and adequately ganglionic specimens. A positive association was also found between Bcl-2 expression and IND compared with other disorders of dysganglionosis (P = 0.04) and post-HSCR cases (P = 0.002). Conclusion Bcl-2 immunohistochemistry is a valuable tool to diagnose allied disorders of HSCR through its expression in IGCs, which are difficult to identify by conventional hematoxylin and eosin staining.

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Section: Discussionmentioning

confidence: 85%

Diagnostic utility of Bcl-2 immunohistochemical expression in pediatric functional bowel obstruction cases with ganglionated specimens

et al. 2022

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“…Measures of frailty vary center to center. In liver transplantation, sarcopenia has been shown to be an objective measurement of frailty and is an independent predictor of mortality among wait listed liver transplant candidates with low model for end stage liver disease (MELD) scores 12,13 . Frailty has been shown to increase wait list mortality for liver transplant candidates 14 .…”

Section: Transplantationmentioning

confidence: 99%

“…Another recent report analyzed the impact of DSA on longer term LT outcomes. 13 In this series, starting in 2008 all patients (n=267) who were 6 months or more from LT underwent screening for DSA which was repeated annually through 2012. The report found 13% of patients had DSA at the first screen (using an MFI of >1000 as positive), and an additional 21 (9%) developed new DSA during the study period.…”

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confidence: 99%

“…County health rankings are assigned based on access to care, prevalence of comorbidity, environmental hazards, and behaviors of individuals within each U.S. county. Rankings range from 1-40 and were treated categorically to capture community risk: lowest risk (<10), low-intermediate risk(11)(12)(13)(14)(15)(16)(17)(18)(19)(20), high-intermediate risk(21)(22)(23)(24)(25)(26)(27)(28)(29)(30), and highest risk (>30). EHR was any readmission within 30 days of discharge following KT.…”

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confidence: 99%

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Abstracts

2015

American Journal of Transplantation

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All authors and co-authors and/or individuals involved with the abstract are required to disclose relevant financial relationshipswith respect to their abstract. All relevant disclosures are published following each abstract. Pre-MeetingAbstract #PM-1Many new challenges face surgeons in the field of liver transplantation. Increasingly complex surgical needs are present, and these impact day-to-day operations for transplantation. These include, but are not limited to, extended criteria donors and increasing use of vascular reconstruction, both in donors and in recipients. Much of fellowship training is devoted to mastering the basic skill set for liver transplantation, yet frequently patients undergoing liver transplantation may have significantly increased complexity. A critical skill for effective liver transplantation involves recognition and anticipation of technical challenges that must be anticipated prior to encountering such difficulties. Surgeons in these potential situations must have colleagues with sufficient skills and willingness to assist. While not regularly common, such situations may arise and lead to a critical need for a well-skilled team of surgeons for effective surgical intervention. The fully trained surgeon must be familiar with special techniques which may often be required, including the use of venovenous bypass, cut down techniques for split liver transplant, unique vascular and biliary reconstruction, and the judgment required for use of other teams, including interventional radiology. Each of these complex areas will be discussed in the course of this presentation. Abstract #PM-2Hepatocellular Carcinoma: Who Not to Transplant, That's the Question Theodore H. Welling, MD, University of MichiganThe incidence of hepatocellular carcinoma (HCC) is rising, and therapies designed to target early stage HCC which show curative efficacy include hepatic resection, liver transplantation (OLT), radiofrequency ablation, and other developing ablative options such as stereotactic body radiation therapy (SBRT) 1 . However, the donor liver supply remains flat over time despite the increasing incidence of HCC. It is estimated that the incidence of HCC in the U.S. is rising faster than other solid organ malignancies, greater than 5 cases/100,000 persons 2 , and is believed to be related to the HCV epidemic and rising burden of NASH. While OLT remains a useful therapy for patients with HCC and a significant burden of liver disease 3 , the donor organ supply, complication/cost rate, and risk of recurrence in patient subsets limits this modality from being used universally in HCC patients. Indeed, it has recently been brought to attention that OLT may be over-utilized in HCC patients when compared to non-HCC patients receiving OLT 4,5 . HCC biologic factors such as tumor stage, AFP, and response to therapy remain important criteria affecting overall outcomes following OLT 1,6,7 . Therefore the question remains: which patients are most likely to benefit from other therapies? Is there evidence supporting OLT ...

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“…The fi rst systematic classifi cation of dysganglionoses was proposed by Schärli in 1995 (2), and ganglion cell immaturity and ganglion cell degeneration were later included as a new type, dysmorphic type (3)(4)(5). Ganglion cell immaturity is a rare form of dysganglionosis.…”

Section: Introductionmentioning

confidence: 99%