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  • Romanian Society of Gastroenterology and Hepatology  (3)
  • 1
    Online Resource
    Online Resource
    Unfused Liver Segments: a Case Report of an Unknown Phenotype of the Conradi-Hünermann-Happle Syndrome
    Romanian Society of Gastroenterology and Hepatology ; 2016
    In:  Journal of Gastrointestinal and Liver Diseases Vol. 25, No. 4 ( 2016-12-01), p. 547-549
    Details
    In: Journal of Gastrointestinal and Liver Diseases, Romanian Society of Gastroenterology and Hepatology, Vol. 25, No. 4 ( 2016-12-01), p. 547-549
    Abstract: Background: Since its description in 1957, Couinaud`s classification of the segmental organization of the liver has remained valid. However, recent investigations by 3-dimensional computed tomography suggest a significant variability of the vascular anatomy and segment volume. Here, we report a surprise finding during the laparoscopic cholecystectomy of a patient with Conradi-Hünermann-Happle syndrome, in whom the liver segments were not fused. Case report: Laparoscopic cholecystectomy was performed because of recurrent biliary pancreatitis in a 47 year-old male patient, who had been diagnosed with Conradi-Hünermann-Happle syndrome. Upon direct view, the liver parenchyma appeared normal, but liver segments were separated and connected by fibrous bridges containing vascular structures, only. Since the hilar anatomy was unclear, an open cholecystectomy was performed without technical difficulties and the postoperative course was uneventful. Postoperatively, magnetic resonance imaging was performed, which revealed a trifurcation of the portal vein and a right bile duct draining into the left main duct. Intersegmental signal alterations corresponded to the fibrous bands seen during laparoscopy. Conclusions: The intraoperative findings of this case confirm the segmental organization of hepatic anatomy proposed by Couinaud. The first description of such an unusual anatomical variant in an extremely rare genetic disorder strongly suggests an association with the genetic background of the syndrome. The established abnormalities of cholesterol biosynthesis in patients with Conradi-Hünermann-Happle syndrome may well explain the observed liver anomaly, which is a novel phenotype of this syndrome. Based on this case, we suggest a potential involvement of the mutation in the emopamil-binding protein gene in liver development and regeneration. Abbreviations: CDPX2: X-linked dominant chondrodysplasia punctata; EPB: emopamil-binding protein; MRCP: magnetic resonance cholangiopancreatography; MRI: magnetic resonance imaging; RCDP: rhizomelic chondrodysplasia punctuata.
    Type of Medium: Online Resource
    ISSN: 1842-1121 , 1841-8724
    URL: Article
    DOI: 10.15403/jgld.2014.1121.254.sch
    Language: Unknown
    Publisher: Romanian Society of Gastroenterology and Hepatology
    Publication Date: 2016
    detail.hit.zdb_id: 2253255-9
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  • 2
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    Online Resource
    Cirrhosis Risk Score of the Donor Organ Predicts Early Fibrosis Progression after Liver Transplantation
    Romanian Society of Gastroenterology and Hepatology ; 2019
    In:  Journal of Gastrointestinal and Liver Diseases Vol. 28, No. 1 ( 2019-03-01), p. 53-61
    Details
    In: Journal of Gastrointestinal and Liver Diseases, Romanian Society of Gastroenterology and Hepatology, Vol. 28, No. 1 ( 2019-03-01), p. 53-61
    Abstract: Background & Aims: Fibrosis progression (FP) after liver transplantation (LT) increases morbidity and mortality. Biomarkers are needed for early prediction of FP. A recipient’s seven-gene cirrhosis risk score (CRS) has been associated with FP, especially in non-transplant cohorts. A broader validation of CRS, including the genotype of the donor-organ and HCV-negative patients is lacking. We therefore analyzed the impact of donor- and recipient-specific genotypes on FP after LT in a large cohort of HCV-positive and -negative patients.Method: Genotyping from liver biopsies (n=201 donors) and peripheral blood (n=442 recipients) was performed. Cirrhosis risk score was correlated with FP at 1 and 5 years after LT.Results: Fibrosis ≥F2 was documented in 26.5% of the recipients’ CRS group (R-CRS) (defined by recipient’s genotype) and in 23.4% of the donors’ CRS- group (D-CRS) (defined by donor’s genotype). Cumulative incidence for fibrosis ≥F2 was higher in patients with D-CRS 〉 0.7 (p=0.03). While the R-CRS showed no prognostic relevance, D-CRS 〉 0.7 was associated with higher hazard ratios (HRs) for fibrosis ≥F2 (HR=2.04; p=0.01), especially in HCV-negative patients (HR=2.59, p=0.03). Donors’ CRS 〉 0.7 was associated with higher risk for ≥F2 in 1-year protocol biopsies (p 〈 0.001). Among the patients in whom both the recipient’s and donor’s CRS were available, fibrosis ≥F2 was encountered more frequently in patients with a D-CRS 〉 0.7, in combination with any R-CRS, compared to patients with D-CRS scores ≤0.7 (p=0.034). Donors’ AZIN1, STXBP5L, TRPM5 genotypes carried a higher risk for fibrosis ≥F2 in subgroups.Conclusion: High D-CRS 〉 0.7 predicted early FP after LT, especially in HCV negative patients.
    Type of Medium: Online Resource
    ISSN: 1842-1121 , 1841-8724
    URL: Article
    DOI: 10.15403/jgld-158
    Language: Unknown
    Publisher: Romanian Society of Gastroenterology and Hepatology
    Publication Date: 2019
    detail.hit.zdb_id: 2253255-9
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  • 3
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    Online Resource
    Different Prevalence of Alarm, Dyspeptic and Reflux Symptoms in Patients with Cardia and Non-cardia Gastric Cancer
    Romanian Society of Gastroenterology and Hepatology ; 2021
    In:  Journal of Gastrointestinal and Liver Diseases Vol. 30, No. 4 ( 2021-12-21), p. 431-437
    Details
    In: Journal of Gastrointestinal and Liver Diseases, Romanian Society of Gastroenterology and Hepatology, Vol. 30, No. 4 ( 2021-12-21), p. 431-437
    Abstract: Background and Aims: Symptoms of patients with gastric cancer (GC) are often unspecific and differences in symptoms between patients with cardia and non-cardia GC have been poorly investigated. We aimed to characterize symptoms of patients with cardia and non-cardia GC. Methods: Patients with cardia (Siewert type II and III) and non-cardia GC were recruited in the German multicenter cohort of the Gastric Cancer Research (staR) study between 2013 and 2017. Alarm, dyspeptic and reflux symptoms at the time of presentation were documented using a self-administered questionnaire. Results: A completed self-administered questionnaire was available for 568/759 recruited patients (132 cardia GC, 436 non-cardia GC, male 61%, mean age 64 years). Dyspeptic symptoms were more common in patients with non-cardia GC (69.0 vs. 54.5%, p=0.0024). Cardia GC patients reported more frequently alarm symptoms (69.7 vs. 44.7%, p 〈 0.0001), and were more likely to have Union for International Cancer Control (UICC) stage III-IV (54.1vs. 38.9%, p=0.0034). Especially, dysphagia and weight loss were more common in patients with cardia GC (49.2 vs. 6.4 %, p 〈 0.0001 and 37.1 vs. 25.7%, p=0.02, respectively). No differences between the two groups were observed with respect to reflux symptoms. Patients with alarm symptoms were more likely to have UICC stage III-IV at presentation (69.4 vs. 42.9%, p 〈 0.0001). Conclusions: In clinical practice the symptom pattern at presentation may serve as a hint for tumor localization. Despite the fact that they are common in the general population, dyspeptic symptoms offer a chance for earlier GC detection. Thus, in patients with dyspeptic symptoms who fail empiric approaches, endoscopy should not be delayed.
    Type of Medium: Online Resource
    ISSN: 1842-1121 , 1841-8724
    URL: Article
    DOI: 10.15403/jgld-3795
    Language: Unknown
    Publisher: Romanian Society of Gastroenterology and Hepatology
    Publication Date: 2021
    detail.hit.zdb_id: 2253255-9
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