It has been reported that 5-50% of patients with primary immune deficiencies (PID) may present with or develop gastrointestinal (GI) manifestations.
This study was aimed at analyzing GI and related ...endoscopic, histopathological findings in children with PID.
Children with PID who were evaluated by endoscopy between 2005 and 2016 were enrolled in this study. Demographic data, growth parameters, signs and symptoms at diagnosis were obtained.
Of 425 children with PID, 195 had GI manifestations. Forty-seven of 195 children required endoscopic investigation, 30 (63.8%) were male, and the mean age was 7.7 ± 5 years. The rate of consanguinity was 61.7%, and the most common symptom was chronic diarrhea (57.4%). Seventy-two percent of the patients were malnourished. Giardia intestinalis was detected in 4, and Helicobacter pylori was confirmed in 8/45 (17.7%) patients. Non-celiac villous flatting was discovered in 15.5% of patients. Twelve patients were diagnosed as having immunodeficiency associated inflammatory bowel disease (IBD)-like colitis.
PID may present with GI manifestations or develop during the course of the disease. Investigating immunodeficiency in patients with atypical GI symptoms can provide an appropriate therapeutic option, and an improved quality of life, particularly in populations with a high rate of consanguinity.
Abstract
Background and aims.
Syndecan-1 (CD138) is a transmembrane heparan sulfate proteoglycan expressed in the liver which may exert metabolic effects by mediating the hepatic clearance of ...triglyceride-rich lipoproteins. In the present study, we assayed serum levels and the hepatic expression of syndecan-1 and examined their association with clinical, biochemical, and histologic phenotypes in patients with histology-proven nonalcoholic fatty liver disease (NAFLD).
Methods.
A total of 59 patients with biopsy-proven NAFLD and 54 matched controls were enrolled. The analysis of syndecan-1 expression in liver biopsies was performed by immunohistochemistry on formalin-fixed, paraffin-embedded samples. Serum syndecan-1 levels were measured by ELISA.
Results.
NAFLD patients had significantly higher serum syndecan-1 levels median: 61 ng/mL (interquartile range: 36-97 ng/mL) than controls median: 37 ng/mL (interquartile range: 25-59 ng/mL, Mann-Whitney U test, p < 0.001. However, we did not find any significant association between serum syndecan-1 and the mean syndecan-1 immunohistochemical score (n = 59, r = 0.064, p = 0.63). Interestingly, the syndecan-1 immunohistochemical score was an independent predictor of HDL cholesterol in NAFLD patients (β = 0.27; t = 1.99, p < 0.05).
Conclusions.
Our data suggest that serum syndecan-1 levels are raised in patients with NAFLD. Moreover, the syndecan-1 immunohistochemical score in the liver is independently associated with HDL cholesterol in this group of patients. These pilot results support further investigation of this molecule in metabolic liver diseases.
Background and Aim: The coexistence of metabolic-associated fatty liver disease (MAFLD) in the course of chronic hepatitis B virus infection increases liver-related morbidity. A positive correlation ...was found between positive hepatitis B core antibody (anti-HBc) and the risk of cirrhosis and hepatocellular carcinoma (HCC) in MAFLD. The relationship between anti-HBc positivity and MAFLD progression to fibrosis, cirrhosis, and liver-related outcomes was determined. Material and Methods: This is a retrospective study including 242 patients with biopsy-proven MAFLD, 130 patients with clinically diagnosed MAFLD-related cirrhosis, and 62 patients with MAFLD-related or cryptogenic HCC. Anti-HBc antibody results were compared with clinical outcomes. Results: Anti-HBc positivity was associated with fibrosis severity (p=0.005). Anti-HBc was positive in 19 (20.2%), 33 (25.8%), 53 (35.3%), and 27 (43.5%) patients with F0–F1 fibrosis, F2–F3 fibrosis, cirrhosis (F4), and HCC, respectively. Median steatosis score was grade 3 in anti-HBc positive patients and grade 2 in negative patients (p=0.07). Anti-HBc positivity was not associated with significant fibrosis (≥F2), cirrhosis, and any liver related complications including HCC. Conclusion: Higher anti-HBc positivity was found in MAFLD patients with advanced fibrosis and cirrhosis compared to patients with early stage fibrosis. No relation was found between anti-HBc positivity and development of cirrhosis, HCC or other liver related complications.
Objective: Gastric intestinal metaplasia (GIM) is considered as a predisposing lesion for the development of gastric cancer and isrecommended to be kept under surveillance in designated intervals. We ...aimed to assess the natural course of GIM in a large Turkishcohort.Materials and Methods: We retrospectively reviewed findings from pathology reports of gastric biopsies conducted between 2011 to2018 to reveal patients diagnosed with solitary GIM in their index pathology report. Progression of GIM was pre-defined as; lowgradedysplasia (LGD), high-grade dysplasia (HGD), or gastric malignancy.Results: The median follow-up period of the study population was 34 (12-128) months. Out of 109 patients with GIM at the entry, 54(49.6%) patients had stable GIM, whereas 53 (48.6%) cases had no signs of GIM at their final endoscopy. Only two (1.8%) patientsprogressed to LGD, but no HGD or malignancy was detected in the follow-up.Conclusion: Although, considered as a premalignant lesion and offered surveillance globally, progression of GIM was very low in alarge Turkish cohort. Further prospective studies in larger cohorts are required to enlighten the obscure strategies in the surveillanceof gastric malignancy.
Abstract The novel adipokines vaspin, obestatin, and apelin-36 are associated with insulin resistance and the components of the metabolic syndrome. We assayed circulating levels of these molecules ...and examined their association with clinical, biochemical, and histologic phenotypes in patients with nonalcoholic fatty liver disease (NAFLD). Serum levels of vaspin, obestatin, and apelin-36 were assayed by enzyme-linked immunosorbent assay in 91 patients with biopsy-proven NAFLD and 81 controls. We analyzed associations between adipokines and the characteristics of patients with NAFLD using multivariable linear regression models. Univariable analysis showed that concentrations of vaspin and apelin-36 were significantly higher in patients with NAFLD than in controls, whereas no differences in obestatin levels were found. Serum vaspin levels showed a statistically significant association with C-reactive protein ( r = 0.378, P < .001) and liver fibrosis scores ( r = 0.401, P < .001), whereas apelin-36 levels showed a modest association with homeostasis model assessment of insulin resistance ( r = 0.204, P < .01). After stepwise linear regression analysis, serum vaspin levels were the only independent predictor of liver fibrosis scores in patients with NAFLD ( β = 0.37, t = 3.99, P < .01). Serum vaspin levels are raised in patients with NAFLD regardless of potential confounders and represent an independent predictor of liver fibrosis scores. These findings support further investigation of this novel adipokine in metabolic liver diseases.
Metabolic dysfunction-associated steatotic liver disease (MASLD) is a growing global epidemic in Türkiye and worldwide. The aim of this study was to evaluate the prevalence and predictors of MASLD ...and steatohepatitis among adults who died of unnatural causes including sudden death and non-burn trauma.
We conducted a prospective review of all consecutive adult forensic autopsies for natural (sudden unexpected) and non-natural (Suicidal, homicidal and accidental) suspicious deaths carried out at the Istanbul Council of Forensic Medicine from February to May 2022. Four wedge biopsies were extracted from sagittal sectioned liver specimens. A liver pathologist with 20 years of experience examined each case for steatosis, ballooning, inflammation, and fibrosis.
Among 1797 autopsies, 62 met inclusion criteria. Overall, 43.3 % (n = 26) of autopsies showed evidence of steatotic liver disease, with a distribution of steatosis severity as: Grade I (28.3 %), Grade II (6.6 %), and Grade III (8.3 %). All these cases met at least one cardiometabolic criteria and diagnosed with MASLD. Ballooning was observed in 20.0 % of cases (5 cases grades 1 and 7 cases grade 2), and Inflammation was present in 51.7 % (9 cases with grade 0–1, 12 with 1–2, 7 with 2–3, and 3 with 5–6). Notably, 46.1 % (n = 12) of MASLD cases and 20.0 % (n = 12) of all cases were diagnosed with steatohepatitis, with three cases exhibiting delicate perisinusoidal fibrosis and one case showing portal fibrosis.
The histopathological findings from this autopsy study confirmed the markedly high prevalence of MASLD and steatohepatitis within the general adult population, highlighting the concerning burden of steatotic liver disease in Türkiye.
Autoimmune pancreatitis has been described as a pancreatic manifestation of immunoglobulin G4-related disease, which is characterized by typical histopathologic, radiologic, and clinical features. ...Immunoglobulin G4-related disease is usually accompanied by elevated serum immunoglobulin G4 level, and can involve multiple organ/systems. Immunoglobulin G4-related disease has rarely been reported in pediatric population. There are few reports of inflammatory bowel disease in association with immunoglobulin G4-related disease. We describe a 7-year-old girl who presented with pancreatitis and concurrent sclerosing cholangitis, and developed bloody diarrhea during follow-up. An endoscopic examination revealed inflammatory bowel disease, and later lacrimal gland involvement was also recognized. She was diagnosed as having immunoglobulin G4-related disease, and her clinical signs and symptoms improved dramatically after steroid treatment. Hence, awareness of the clinical picture is important and early diagnosis can prevent fibrosis and organ damage.
Chronic hepatitis C virus (HCV) infection is a major health problem worldwide. The majority of cases involving HCV infection develop into chronic hepatitis because of a failure to develop an ...effective immune response. Apoptosis of the hepatocytes plays a significant role in the pathogenesis of HCV infection: the interaction between the Fas antigen on hepatocytes and the Fas ligand on T cells corresponds to the main mechanism for hepatocyte damage. Interferon (IFN)-α has antiviral, immunoregulatory, and antiproliferative properties, and apoptosis seems to be a critical event in the action mechanisms of both IFNs. In this study, we aimed to detect any relationship between apoptotic markers in the liver and the response to the treatment.
The study included 180 chronic HCV patients treated with IFN and ribavirin in four centers. Apoptotic markers (Fas, Fas ligand, Fas-associated death domain, caspases 3, 8, and 9, and in-situ apoptosis) were studied in the liver. The age, sex of the patients, response to therapy, ALT level, viral load, and genotype were recorded.
The results of the study showed that the histological activity index and fibrosis correlated with CD95 staining density, caspase-8 intensiveness, and portal and parenchymal Fas ligand scores. The apoptotic parameters of the responsive cases were not significantly different from those of the unresponsive cases.
The apoptotic parameters studied in liver tissue are associated with inflammation and fibrosis; however, these parameters may not predict response to treatment.
Objective
To analyze the relationship between fibrosis staged by Ishak stage and quantified by digital image analysis (DIA), and to reveal the optimum performance of shear‐wave elastography (SWE) ...using quantitative DIA measurements as a comparative histological standard.
Methods
The proportionate area (PA) of fibrosis was measured by DIA from images of the PA of trichrome‐stain (TPA) of 168 chronic hepatitis patients. SWE was performed in 105 patients. The accuracy of SWE for predicting the fibrosis defined by quantitative PA thresholds (≥2.5%, ≥5%, ≥10% and ≥20%, respectively) and by Ishak stages was measured using the area under the receiver operating characteristic curve (AUROC).
Results
DIA was proven to be highly reproducible (interclass correlation coefficient 0.926). The TPA range corresponding to each Ishak stage was large, widened as stages progressed, and reached its greatest extent in cirrhosis. TPA magnified at ×50 ranges 11.9–56% for Ishak stage F5–6. A good correlation between TPA and elasticity was presented for more advanced fibrosis (TPA ≥10%, rs = 0.732, P = 0.000) than milder fibrosis (TPA <10%, rs = 0.308, P = 0.006). With the advance of fibrosis either by stages or PA thresholds the discriminative accuracy of SWE gradually increased, but was less satisfactory for milder fibrosis.
Conclusions
DIA may serve as a reproducible and reliable quantitative standard for surrogate tests for liver fibrosis. The performance and correlation of SWE with the fibrotic extent were better for advanced fibrosis, but less satisfactory for milder fibrosis.
Morphological evaluation of the liver continues to play a centralrole for the diagnosis, grading and staging of chronic viral hepatitis.The defining morphology is necroinflammation, that is ...hepatocyteinjury and inflammation. Hepatocyte injury is usually irreversible,and presents as apoptosis and/or necrosis. Mononuclear cellinfiltration of the portal tracts, that is usually accompanied byperiportal (interface) and lobular inflammation is typical. Continuednecroinflammatory activity at the limiting plate destroyingperiportal parenchyma initiates fibrogenesis leading to cirrhosis.Fibrosis can be reversible with fragmentation of scar tissue,resolving vascular derangements and parenchymal regeneration.Grading is a measure of the intensity of necroinflammatory activityand staging is a measure of fibrosis and architectural alteration.Besides staging, Laennec scoring system, subdividing cirrhosisthat is based on histologic parameters of fibrous septa width andnumber, has been advised to be used in reporting chronic viralhepatitis.Keywords: Viral hepatitis, Grading, Staging, Biopsy
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