Publications by authors named "Piotr Socha"

169 Publications

Quantitative multiparametric MRI as a non-invasive stratification tool in children and adolescents with autoimmune liver disease.

Sci Rep 2021 Jul 27;11(1):15261. Epub 2021 Jul 27.

Department of Gastroenterology, Hepatology, Nutritional Disorders and Pediatrics, The Children's Memorial Health Institute, Warsaw, Poland.

Autoimmune hepatitis (AIH) and autoimmune sclerosing cholangitis (ASC) are two very closely related autoimmune liver diseases with overlapping clinical features and similar management strategies. The purpose of this study was to assess the utility of quantitative imaging markers to distinguish ASC from AIH in paediatrics. 66 participants (N = 52 AIH, N = 14 ASC) aged 14.4 ± 3.3 years scheduled to undergo routine biopsy and baseline serum liver biochemistry testing were invited to undergo MRI (non-contrast abdominal MRI and 3D fast spin-echo MRCP). Multiparametric MRI was used to measure fibro-inflammation with corrected T1 (cT1), while the biliary tree was modelled   using quantitative MRCP (MRCP +). Mann-Whitney U tests were performed to compare liver function tests with imaging markers between patient groups (ASC vs AIH). Receiver operating characteristic curves and stepwise logistic regressions were used to identify the best combination of markers to discriminate between ASC and AIH. Correlations between liver function tests and imaging markers were performed using Spearman's rank correlation. cT1 was significantly correlated with liver function tests (range 0.33 ≤ R ≤ 56, p < 0.05), as well as with fibrosis, lobular and portal inflammation (range 0.31 ≤ R ≤ 42, p < 0.05). 19 MRCP + metrics correlated significantly with liver function tests (range 0.29 ≤ R ≤ 0.43, p < 0.05). GGT and MRCP + metrics were significantly higher in ASC compared to those with AIH. The best multivariable model for distinguishing ASC from AIH included total number of ducts and the sum of relative severity of both strictures and dilatations AUC: 0.91 (95% CI 0.78-1). Quantitative MRCP metrics are a good discriminator of ASC from AIH.
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http://dx.doi.org/10.1038/s41598-021-94754-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8316432PMC
July 2021

Assessment of Lactobacillus casei rhamnosus (LGG) therapy in children with biliary atresia - randomized placebo controlled trial.

Clin Res Hepatol Gastroenterol 2021 Jul 23:101753. Epub 2021 Jul 23.

Department of Gastroenterology, Hepatology, Nutritional Disorders and Pediatrics, The Children's Memorial Health Institute, Warsaw, Poland.

Background: The role of microbiota in biliary atresia (BA) remains unclear. The aim of our study was to assess efficacy and safety of LGG treatment in children with BA after HPE with special focus on bacterial cholangitis (BCH) and quantitative assessment of the gut microbiota composition and metabolism.

Methods: We performed double-blind placebo controlled trial with patients randomized into treatment group who received LGG (n=14) and placebo (n=16). The gut microbiota and short-chain fatty acids (SCFA) were assessed at baseline and after 6 months of treatment. Clinical and laboratory parameters including episodes of bacterial cholangitis (BCH) were collected during the study period and after 2-year follow-up. Additionally, stool composition of BA patients was compared with healthy age-matched control group.

Results: There were lower concentration of SCFA in children with BA compared to control group and significant increase in the number of Enterococcus bacteria. After 6 months of treatment, neither laboratory parameters nor gut microbiota composition differed between LGG group and placebo. PP analysis results were similar to ITT analysis, no significant differences between study and control group. Overall, there were 11 (36%) patients who developed at least one episode of bacterial cholangitis; 3 (21%) in the LGG group compared to 8 (50%) placebo group (p=0.14). Bacterial cultures were positive in 22% of cases and recurrence after the first episode was observed in 27% of patients. The level of total bilirubin decreased below 2 mg/dl after 6 months of the study in 6 (42.8%) patients in the LGG group and in 8 (50%) patients in the placebo group (p=0.73). During 2-year follow-up 6 out of 14 patients (42.8%) in the LGG group and 11 out of 16 placebo patients (68.7%) underwent liver transplantation (p=0.27).

Conclusions: Patients with BA present with specific microbiota profiles and decreased SCFA what gives opportunities to implement novel therapeutic options based on modulation of microbiota. Whether LGG is an effective therapy needs to be studied in a larger group with similar outcome parameters.
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http://dx.doi.org/10.1016/j.clinre.2021.101753DOI Listing
July 2021

Liver Involvement in Congenital Disorders of Glycosylation and Deglycosylation.

Front Pediatr 2021 5;9:696918. Epub 2021 Jul 5.

Department of Pediatrics, Nutrition and Metabolic Diseases, Children's Memorial Health Institute, Warsaw, Poland.

Congenital disorders of glycosylation (CDG) and NGLY1-CDDG (NGLY1-congenital disorder of deglycosylation) usually represent multisystem (especially neurovisceral) diseases with liver involvement reported in some of them. The aim of the study was to characterize the liver phenotype in CDG and NGLY1-CDDG patients hospitalized in our Institute, and to find the most specific features of liver disease among them. The study involved 39 patients (from 35 families) with CDG, and two patients (from two families) with NGLY1-CDDG, confirmed molecularly, for whom detailed characteristics of liver involvement were available. They were enrolled based on the retrospective analysis of their medical records. At the time of the first consultation, 13/32 patients were diagnosed with hepatomegaly; none of them with splenomegaly. As many as 23/32 persons had elevated serum transaminases, including 16 (70%) who had mildly elevated levels. During the long-term follow-up (available for 19 patients), serum transaminases normalized in 15/19 (79%) of them, including a spontaneous normalization in 12/15 (80%) of them. The GGT activity was observed to be normal in all study cases. Protein C, protein S and antithrombin activities in plasma were observed in 16 patients, and they were decreased in all of them. It is necessary to conduct a long-term follow-up of liver disease in CDG to obtain comprehensive data.
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http://dx.doi.org/10.3389/fped.2021.696918DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8286991PMC
July 2021

A Report of 2 Infant Siblings with Progressive Intrahepatic Familial Cholestasis Type 1 and a Novel Homozygous Mutation in the ATP8B1 Gene Treated with Partial External Biliary Diversion and Liver Transplant.

Am J Case Rep 2021 Jul 20;22:e932374. Epub 2021 Jul 20.

Department of Gastroenterology, Hepatology, Nutritional Disorders and Pediatrics, The Children's Memorial Health Institute, Warsaw, Poland.

BACKGROUND Current treatment options for progressive intrahepatic familial cholestasis type 1 (PFIC-1) comprise ursodeoxycholic acid (UDCA), partial external biliary diversion (PEBD), and liver transplantation (LTx). The role and timing of LTx in PFIC-1 remains debated. We present 2 case reports of male siblings with PFIC-1 who benefited from different treatments. CASE REPORT Both siblings harbored a homozygous truncating mutation in ATP8B1 characteristic for PFIC-1 and both underwent PEBD after unsuccessful UDCA treatment at the age of 7 and 5 months, respectively. The older brother, after initial improvement of symptoms, developed severe pruritus, cholestasis, and diarrhea 9 months after PEBD and underwent LTx at the age of 16 months. Chronic diarrhea and abnormal transaminases activity appeared soon after transplantation. A liver biopsy was performed 3 months after LTx and showed severe macrovesicular steatosis (95%). Sixteen months after LTx, total biliary diversion was performed, with rapid relief from diarrhea and significant regression of graft steatosis by <30%. In his brother we observed persistent severe pruritus and cholestasis after PEBD, but we decided to postpone LTx due to lack of a living related donor and risk of graft steatosis. Eight months after PEBD, bilirubin and bile acids significantly decreased and pruritus disappeared completely. Currently, in 5-year follow-up, liver function is stable and he has no pruritus. CONCLUSIONS The good effect of PEBD may be delayed in PFIC-1, even in severe mutation; thus, the decision to perform LTx should be made cautiously. Total biliary diversion is an efficient procedure in case of persistent symptoms after LTx and can reverse graft steatosis in children with PFIC-1.
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http://dx.doi.org/10.12659/AJCR.932374DOI Listing
July 2021

Aglepristone Administration in Mid-Proestrus Reduces the LH Peak but Does Not Prevent Ovulation in the Bitch.

Animals (Basel) 2021 Jun 28;11(7). Epub 2021 Jun 28.

Department of Animal Reproduction with Clinic, University of Warmia and Mazury, Oczapowskiego 14, 10-719 Olsztyn, Poland.

The aim of the study was to evaluate the influence of administration of aglepristone in mid-proestrus on progesterone concentration, LH release, and occurrence of ovulation in the bitch. Experimental bitches ( = 7) were treated on days 4 and 5 of proestrus with aglepristone at the dose of 10 mg/kg body weight s.c. (i.e., the two treatments were 24 h apart). Control animals ( = 7) received s.c. injections of saline. For progesterone determination, blood was collected daily until the first day of cytological diestrus. For LH determination, blood was collected daily and in the periovulatory phase every 8 h. The progesterone concentration showed a similar pattern in both groups. The LH peak value in bitches treated with aglepristone was significantly lower ( < 0.05) than that in control bitches (4.83 ± 1.20 vs. 13.66 ± 1.21 ng/mL). The area under the curve (AUC) for LH was significantly ( < 0.05) lower in treated than in control animals (6.85 ± 1.21 ng/mL/d vs. 12.25 ± 1.35 ng/mL/d). The ovulation occurred in all animals in both groups. The study showed that administration of aglepristone in the mid-proestrus significantly reduced the preovulatory LH surge, but it had no effect on progesterone concentration and the occurrence of ovulation.
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http://dx.doi.org/10.3390/ani11071922DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8300317PMC
June 2021

Dietary recommendations during the Covid-19 pandemic. Statement of the Committee of Human Nutrition Science of the Polish Academy of Sciences.

Rocz Panstw Zakl Hig 2021 ;72(2):209-220

Department of Dietetics, Warsaw University of Life Sciences, Warsaw, Poland.

During the COVID-19 pandemic, care for an adequate diet, well adapted to the body's needs and the current level of physical activity, becomes of particular importance. Many dietary compounds participate in the functioning of the immune system, while vitamins D, C, A (including beta-carotene), E, B6, B12, folic acid, zinc, copper, selenium, iron, amino acids, n-3 and n-6 polyunsaturated fatty acids and intestinal microbiota are crucial in various types of defence processes. There has been no evidence that consumed food and its compounds, including those with pro-/prebiotic properties, play a significant role in preventing SARS-CoV-2 infection or alleviating its course. However, in terms of the nutritional value of food and the prevention of dysbiosis, recommending a varied diet with a high proportion of plant-based foods and an adequate amount of animal-based foods has a sound scientific basis. Malnutrition, underweight and obesity are considered independent and prognostic risk factors of severe SARS-CoV-2 infection, which reduce a patient's chances of survival. Therefore, ensuring good nutritional status, including healthy body weight, is a reasonable approach in the prevention of viral infection SARS-CoV-2 or alleviating its course. The document is accompanied by two catalogues of practical nutritional recommendations during the COVID-19 pandemic, addressed to the general population and children.
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http://dx.doi.org/10.32394/rpzh.2021.0166DOI Listing
June 2021

Complementary Feeding and Overweight in European Preschoolers: The ToyBox-Study.

Nutrients 2021 Apr 5;13(4). Epub 2021 Apr 5.

Department of Nutrition and Dietetics, Harokopio University, 176 76 Athens, Greece.

Complementary feeding (CF) should start between 4-6 months of age to ensure infants' growth but is also linked to childhood obesity. This study aimed to investigate the association of the timing of CF, breastfeeding and overweight in preschool children. Infant-feeding practices were self-reported in 2012 via a validated questionnaire by >7500 parents from six European countries participating in the ToyBox-study. The proportion of children who received breast milk and CF at 4-6 months was 51.2%. There was a positive association between timing of solid food (SF) introduction and duration of breastfeeding, as well as socioeconomic status and a negative association with smoking throughout pregnancy ( < 0.005). No significant risk to become overweight was observed among preschoolers who were introduced to SF at 1-3 months of age compared to those introduced at 4-6 months regardless of the type of milk feeding. Similarly, no significant association was observed between the early introduction of SF and risk for overweight in preschoolers who were breastfed for ≥4 months or were formula-fed. The study did not identify any significant association between the timing of introducing SF and obesity in childhood. It is likely that other factors than timing of SF introduction may have impact on childhood obesity.
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http://dx.doi.org/10.3390/nu13041199DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8066073PMC
April 2021

Fatty Acid-Binding Protein 1 as a Potential New Serological Marker of Liver Status in Children with Wilson Disease.

J Pediatr Gastroenterol Nutr 2021 Mar 17. Epub 2021 Mar 17.

*Department of Pathomorphology, IPCZD, Warsaw, Poland †Department of Gastroenterology, Hepatology, Nutritional Disorders and Pediatrics, IPCZD, Warsaw, Poland ‡Department of Biochemistry, Radioimmunology and Experimental Medicine, IPCZD, Warsaw, Poland §Department of Nephrology and Arterial Hypertension, IPCZD, Warsaw, Poland ||Department of Radiology, IPCZD, Warsaw, Poland.

Objectives: Wilson's disease (WD) is a copper metabolism disorder with toxic copper accumulation in the liver leading to liver steatosis or fibrosis. In vitro studies suggest that fatty acid-binding protein 1 (L-FABP) and lipid droplet-associated protein 5 (PLIN5) may have an impact on both processes, but knowledge about these potential biomarkers is insufficient in the case of WD. Thus, the aim of this study was to determine L-FABP and PLIN5 levels in sera of WD patients in relation to liver steatosis/fibrosis.

Methods: The final study involved 74 WD children in whom liver steatosis (WD1 subgroup, n = 28) and fibrosis (WD2 subgroup, n = 13) were assessed with the use of transient elastography. Control groups included WD children without steatosis and fibrosis (WD0 subgroup, n = 33) and healthy children (n = 75). L-FABP and PLIN5 measurements were performed in sera with the use of the immunoenzymatic method.

Results: L-FABP was significantly higher in the WD2 subgroup, and the correlation between L-FABP concentration and liver fibrosis was confirmed statistically by regression analysis (p = 0.04) with Pearson's coefficient r = 0.24. L-FABP was significantly correlated with aminotransferase ALT (r = 0.42) and AST (r = 0.37) activity. PLIN5 concentration was similar in all groups and was not related to steatosis and fibrosis.

Conclusions: Our results suggest that serum L-FABP could be a novel biomarker of liver fibrosis in WD children.
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http://dx.doi.org/10.1097/MPG.0000000000003128DOI Listing
March 2021

Congenital disorders of glycosylation in children - Histopathological and ultrastructural changes in the liver.

Pediatr Neonatol 2021 May 5;62(3):278-283. Epub 2021 Feb 5.

Department of Pediatrics, Nutrition and Metabolic Diseases, The Children's Memorial Health Institute, Warsaw, Poland. Electronic address:

Background: Congenital disorders of glycosylation (CDG) result from defects in the synthesis of glycans and their attachment to proteins and lipids. Histologically, liver steatosis, fibrosis and cirrhosis have been reported in CDG. The aim of the study was to characterize the histopathological and ultrastructural liver changes in CDG patients hospitalized in our Institute, and to find the most characteristic features, as articles concerning the liver microscopic features in CDG are sparse.

Methods: Out of 32 CDG patients diagnosed and followed-up in our Institute, the liver biopsy was performed in 4 of them, including 2 with MPI-CDG, 1 with SRD5A3-CDG, and 1 with PGM1-CDG, as a part of diagnostic process. In one patient, diagnosed post mortem with PMM2-CDG, the histopathological study comprised liver autopsy samples.

Results: The most common histopathological liver finding was the presence of steatosis (4/5) of varying severity, the mixed macro- and microvesicular type as well as the foamy degeneration of hepatocytes. In two patients, liver steatosis was associated with fibrosis, stage 4 (cirrhosis) and 2 according to Batts and Ludwig classification, respectively. In two patients, besides steatosis, mild inflammatory infiltrates composed of lymphoid cells in portal tracts were observed. No correlation between the patient's age and histopathological features was observed.

Conclusions: The histopathological changes in the liver of CDG patients are miscellaneous; thus, based on the microscopic examination only, we can not identify (even suspect) the exact CDG. The most common histopathologic finding in our cohort of CDG patients was the presence of liver steatosis (of various severity) and foamy degeneration of hepatocytes.
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http://dx.doi.org/10.1016/j.pedneo.2021.01.017DOI Listing
May 2021

Sterol 27-Hydroxylase Deficiency as a Cause of Neonatal Cholestasis: Report of 2 Cases and Review of the Literature.

Front Pediatr 2020 13;8:616582. Epub 2021 Jan 13.

Department of Gastroenterology, Hepatology, Feeding Disorders and Pediatrics, The Children's Memorial Health Institute, Warsaw, Poland.

Inborn errors of primary bile acid (BA) synthesis are rare autosomal recessive disorders responsible for 1-2% of cases of neonatal cholestasis. Among them, cerebrotendinous xanthomatosis (CTX) is caused by mutations in the gene resulting in the impairment of sterol 27-hydroxylase enzyme activity. Here we present the study on two siblings with neonatal cholestasis diagnosed with sterol 27-hydroxylase deficiency. The clinical, biochemical, histological, and molecular presentation at the time of diagnosis and detailed follow-up were described. An extensive overview of the literature regarding patients with sterol 27-hydroxylase deficiency presenting with neonatal cholestasis was also provided. Patient 1 presented with cholestatic jaundice since 10 weeks of age and developed the end-stage liver disease requiring liver transplantation at 8 months of age but finally succumbed 3 years post-transplantation due to autoimmune hemolytic anemia and multiorgan failure development. Next-generation sequencing performed , revealed him to be homozygous for the known pathogenic splicing variant c.1184+1G>A in the gene. Patient 2 (sibling) presented with cholestatic jaundice since the first day of life. Sanger sequencing of revealed the same results. Chenodeoxycholic acid treatment was introduced just after diagnosis, at 4 months of age. Fourteen patients with sterol 27-hydroxylase deficiency presenting with neonatal cholestasis were reported in the literature, in most of them presenting as a self-limiting disease. An early recognition and treatment initiation in CTX is essential.
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http://dx.doi.org/10.3389/fped.2020.616582DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7838534PMC
January 2021

Pediatric Liver Disease Patients and Secondary Glycosylation Abnormalities.

Front Pediatr 2020 13;8:613224. Epub 2021 Jan 13.

Department of Paediatrics, Nutrition and Metabolic Diseases, The Children's Memorial Health Institute, Warsaw, Poland.

Isoelectric focusing (IEF) of serum transferrin (Tf) is still the method of choice for diagnosis of congenital disorders of glycosylation (CDG). An abnormal glycosylation is also a known phenomenon in adult liver disease patients. The aim of this study was to characterize glycosylation disturbances in pediatric patients with primary liver disease. However, there are no reports of this phenomenon in children. Between 1995 and 2019, circa 2,000 serum Tf isoform analyses have been performed in children with primary liver diseases; some of them underwent subsequent analyses. We enrolled in this study 19 patients who developed an acute liver injury (ALI)/failure (ALF) or exhibited a chronic liver disease (CLD) and were evaluated and listed for liver transplantation (LTx) or had just undergone this procedure, and secondary abnormal serum Tf isoform profile. Among 12 patients with ALI/ALF, 10 had an increased percentage of asialo-, monosialo-, and disialo-Tf isoforms. All patients with CLD had an increased percentage of asialo- and monosialo-Tf isoform. Two patients diagnosed with recurrent ALF had very specific serum Tf profile with a huge increase in the asialo- and monosialo-Tf isoform. On follow-up analyses (available in some patients), serum Tf IEF profile normalized in parallel to normalization of liver function tests, spontaneously or during treatment, including glucocorticosteroids in AIH, LTx in CLD. All pediatric patients with primary liver disease had increased asialo-Tf as well as monosialo-Tf isoforms. None of them had elevated percentage of trisialo-Tf isoform.
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http://dx.doi.org/10.3389/fped.2020.613224DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7838542PMC
January 2021

Clinical, biochemical and molecular phenotype of congenital disorders of glycosylation: long-term follow-up.

Orphanet J Rare Dis 2021 01 6;16(1):17. Epub 2021 Jan 6.

Department of Pediatrics, Nutrition and Metabolic Diseases, The Children's Memorial Health Institute, Warsaw, Poland.

Background: Congenital disorders of glycosylation (CDG) result from defects in the synthesis of glycans and the attachment of glycans to proteins and lipids. Our study aimed to describe the clinical, biochemical, and molecular findings of CDG patients, and to present the long-term follow-up.

Material And Methods: A single-center study (1995-2019 years) of patients with congenital disorders of N-glycosylation and combined N- and O-hypoglycosylation was performed.

Results: Among 32 patients included into the study, there were 12 PMM2-CDG, 3 ALG13-CDG, 3 ALG1-CDG, 1 ALG3-CDG, 3 MPI-CDG, 1 PGM1-CDG, 4 SRD5A3-CDG, 1 DPAGT1-CDG, 3 ATP6AP1-CDG, 1 ATP6V0A2-CDG. The phenotypic and genotypic spectrum during long-term (in some cases over 20 years) observation was characterised and several measurements of serum Tf isoforms taken. Statistical analysis revealed strong negative correlation between asialo-Tf and tetrasialo-Tf, as well as between disialo-Tf and tetrasialo-Tf. Within CDG type I, no difference in % Tf isoforms was revealed between PMM2-CDG and non-PMM2-CDG patients. However, these two groups differed significantly in such diagnostic features as: cerebellar ataxia, failure to thrive, hypothyroidism, pericardial effusion, cardiomyopathy, inverted nipples, prolonged INR. The effect of treatment with mannose in 2 patients with MPI-CDG was assessed and we found that % of asialo-Tf, monosialo-Tf, and disialo-Tf was significantly lowered, whereas tetrasialo-Tf and pentasialo-Tf rose, coming closer or falling into the reference range.

Conclusions: The novel finding was an abnormal Tf IEF pattern in two ALG13-CDG patients and normal in one ALG1-CDG patient. Clinical manifestation of presented CDG patients was similar to that reported in the literature. Mannose supplementation in MPI-CDG patients, as well as galactose supplementation in PGM1-CDG patient, improved patients' clinical picture and Tf isoform profiles.
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http://dx.doi.org/10.1186/s13023-020-01657-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7789416PMC
January 2021

[Development of a large intraprostatic cyst following the use of a GnRH agonist-implant in a male dog with benign prostatic hyperplasia].

Tierarztl Prax Ausg K Kleintiere Heimtiere 2020 Dec 4;48(6):443-446. Epub 2020 Dec 4.

Department für Reproduktion der Tiere, Ermländisch-Masurische Universität in Olsztyn.

A male dog with benign prostatic hyperplasia and several small intraprostatic cysts was treated with a GnRH-agonist implant containing 4,7 mg deslorelin (Suprelorin). Within 2 weeks after the implantation, the prior urethral bleeding worsened. A large intraprostatic cyst was detected sonographically. The patient was subsequently treated with osaterone acetate (0.4 mg/kg p. o. once a day for 7 days) and enrofloxacin (5 mg/kg p. o. once a day for 21 days). The clinical symptoms receded within 10 days. Within one month, the cyst regressed completely. The mechanisms of cyst enlargement are discussed.
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http://dx.doi.org/10.1055/a-1295-2748DOI Listing
December 2020

Cardiovascular Risk Assessment in Children With Chronic Cholestatic Liver Diseases.

J Pediatr Gastroenterol Nutr 2020 11;71(5):647-654

Department of Gastroenterology, Hepatology, Nutritional Disorders and Pediatrics.

Objectives: Chronic cholestatic liver diseases are often associated with disturbed lipid metabolism, which may potentially increase cardiovascular (CV) risk but the evidence is scarce. The aim of the study was to assess factors associated with increased CV risk in children with Alagille syndrome (AGS) and biliary atresia (BA).

Methods: We investigated 17 patients with AGS, ages 11.0 years (8.4-13.4) and 19 with BA, ages 13.5 years (10.4-15.1) in whom we performed thorough biochemical assessment including lipid profiles and oxidative stress biomarkers, blood pressure (BP)-systolic, diastolic and mean, carotid intima-media thickness (cIMT), and pulse wave velocity (PWV).

Results: There were abnormal lipid profiles in 82% of children with AGS and 52.6% with BA. In AGS group, we observed significantly higher levels of TC, LDL C, APO B, lower glutathione concentration and glutathione peroxidase activity and lower blood pressure, lower cIMT (P = 0.02), cIMT-SDS (P = 0.04), and PWV (P = 0.04). We, however, observed elevated blood pressure in 2/19 patients with BA and none-with AGS (BA vs AGS: P = 0.12), whereas cIMT-SDS was increased only in 2/17 patients with AGS and in 6/19 with BA (P = 0.24), and abnormal PWV-SDS values were detected in 3/17 of AGS and 8/19 of BA patients (P = 0.15). Neither presence of dyslipidemia nor Lp-X correlated with vascular parameters.

Conclusions: Children with BA and AGS may present with increased cardiovascular risk factors but vascular parameters are not directly related to lipid abnormalities. cIMT and BP should be considered for clinical practice in these cholestatic disorders so as to determine individuals with potential CV risk.
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http://dx.doi.org/10.1097/MPG.0000000000002874DOI Listing
November 2020

Combining Effect and Process Evaluation on European Preschool Children's Snacking Behavior in a Kindergarten-Based, Family-Involved Cluster Randomized Controlled Trial: The ToyBox Study.

Int J Environ Res Public Health 2020 10 7;17(19). Epub 2020 Oct 7.

Department of Movement and Sports Sciences, Ghent University, 9000 Ghent, Belgium.

This study aimed at (1) studying the effect of the standardized ToyBox intervention on European preschoolers' snacking behavior, and (2) studying whether a higher process evaluation score from teachers and parents/caregivers was associated with a more positive result for preschoolers' snack intake. A sample of 4970 preschoolers (51.4% boys, 4.74 ± 0.44 years) from six European countries provided information on snack intake with the use of a Food Frequency Questionnaire. To investigate the effect of the intervention, multilevel repeated measures analyses were executed for the total sample and the six country-specific samples. Furthermore, questionnaires to measure process evaluation were used to compute a total process evaluation score for teachers and parents/caregivers. No significant intervention effects on preschoolers' snack intake were found (all > 0.003). In general, no different effects of the intervention on snack intake were found according to kindergarten teachers' and parents'/caregivers' process evaluation scores. The lack of effects could be due to limited intervention duration and dose. To induce larger effects on preschoolers' snack intake, a less standardized intervention which is more tailored to the local needs might be needed.
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http://dx.doi.org/10.3390/ijerph17197312DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7579655PMC
October 2020

Expression of Matrix Metalloproteinases and Their Tissue Inhibitors in Peripheral Blood Leukocytes and Plasma of Children with Nonalcoholic Fatty Liver Disease.

Mediators Inflamm 2020 10;2020:8327945. Epub 2020 Sep 10.

Department of Gastroenterology, Hepatology, Nutritional Disorders and Paediatric, The Children's Memorial Health Institute, Warsaw, Poland.

Gene expression profiles of matrix metalloproteinases () and their tissue inhibitors () were evaluated in peripheral blood leukocytes of children with nonalcoholic fatty liver disease (NAFLD). Gene expression patterns were correlated with their plasma protein counterparts, systemic parameters of liver injury, and selected markers of inflammation. The -, -, -, -, -, -, -, and - transcripts levels were tested by the real-time PCR. Plasma concentrations of MMP-9, TIMP-1, MMP-9/TIMP-1 ratio, MMP-2/TIMP-2 ratio, sCD14, leptin, resistin, IL-1 beta, and IL-6 and serum markers of liver injury were estimated by ELISA. The -, - expression levels, plasma amounts of MMP-9, TIMP-1, and the MMP-9/TIMP-1 ratio were increased in children with NAFLD. Concentrations of AST, ALT, GGT, and leptin were elevated in serum patients with NAFLD, while concentration of other inflammatory or liver injury markers was unchanged. The - and - levels correlated with serum liver injury parameters (ALT and GGT concentrations, respectively); there were no other correlations between gene expression profiles, their plasma counterparts, and serum inflammatory markers. Association of - and -9 expression with serum liver injury parameters (ALT, GGT) may suggest leukocyte engagement in the early stages of NAFLD development which possibly precedes subsequent systemic inflammatory responses.
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http://dx.doi.org/10.1155/2020/8327945DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7501567PMC
August 2021

Multiparametric MRI as a Noninvasive Monitoring Tool for Children With Autoimmune Hepatitis.

J Pediatr Gastroenterol Nutr 2021 01;72(1):108-114

Department of Gastroenterology, Hepatology, Nutritional Disorders and Pediatrics, The Children's Memorial Health Institute, Warsaw, Poland.

Objectives: Autoimmune hepatitis (AIH) is a progressive liver disease managed with corticosteroids and immunosuppression and monitored using a combination of liver biochemistry and histology. However, liver biopsy is invasive with risk of pain and bleeding. The aim of the present study was to investigate the utility of noninvasive imaging with multiparametric magnetic resonance imaging (MRI) (mpMRI) to provide clinically useful information on the presence and extent of hepatic inflammation, potentially guiding immunosuppression.

Methods: Eighty-one participants (aged 6-18), 21 healthy and 60 AIH patients, underwent multiparametric MRI to measure fibro-inflammation with iron-corrected T1 (cT1) at the Children's Memorial Health Institute in Warsaw alongside other clinical blood tests and liver biopsy at recruitment and after an average of 16-month follow-up (range 9-22 months). Correlation analyses were used to investigate the associations between cT1 with blood serum markers and histological scores.

Results: At recruitment, patients with AIH had a higher cT1 value than healthy controls (P < 0.01). cT1 correlated significantly with key histopathological features of disease. Treatment naïve AIH patients showed evidence of inflammation and heterogeneity across the liver compared to healthy controls.At follow-up, cT1 showed utility in monitoring disease regression as most patients showed significantly reduced fibro-inflammation with treatment (P < 0.0001) over the observational period. Six patients had histological fibrosis and clear fibro-inflammation on MR despite biochemical remission (normalized aspartate aminotransferase (AST), alanine aminotransferase (ALT), and immunoglobulin G [IgG]).

Conclusions: Multiparametric MRI can measure disease burden in pediatric AIH and can show changes over time in response to therapy. Active disease can be seen even in biochemical remission in children.
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http://dx.doi.org/10.1097/MPG.0000000000002930DOI Listing
January 2021

Targeted Next-Generation Sequencing in Diagnostic Approach to Monogenic Cholestatic Liver Disorders-Single-Center Experience.

Front Pediatr 2020 24;8:414. Epub 2020 Jul 24.

Department of Gastroenterology, Hepatology, Feeding Disorders and Pediatrics, The Children's Memorial Health Institute, Warsaw, Poland.

To evaluate the clinical utility of panel-based NGS in the diagnostic approach of monogenic cholestatic liver diseases. Patients with diagnosis of chronic cholestatic liver disease of an unknown etiology underwent NGS of targeted genes panel. Group 1 included five patients (prospectively recruited) hospitalized from January to December 2017 while group 2 included seventeen patients (retrospectively recruited) hospitalized from 2010 to 2017 presenting with low-GGT PFIC phenotype (group 2a, 11 patients) or indeterminant cholestatic liver cirrhosis (group 2b, 6 patients). Among 22 patients enrolled into the study, 21 various pathogenic variants (including 11 novel) in 5 different genes (including ) were identified. The molecular confirmation was obtained in 15 out of 22 patients (68%). In group 1, two out of five patients presented with low-GGT cholestasis, and were diagnosed with BSEP deficiency. Out of three patients presenting with high-GGT cholestasis, one patient was diagnosed with PFIC-3, and the remaining two were not molecularly diagnosed. In group 2a, seven out of eleven patients, were diagnosed with BSEP deficiency and two with TJP-2 deficiency. In group 2b, three out of six patients were molecularly diagnosed; one with PFIC-3, one with CYP27A1 deficiency, and one with DGUOK deficiency. Panel-based NGS appears to be a very useful tool in diagnosis of monogenic cholestatic liver disorders in cases when extrahepatic causes have been primarily excluded. NGS presented the highest diagnosis rate to identify the molecular background of cholestatic liver diseases presenting with a low-GGT PFIC phenotype.
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http://dx.doi.org/10.3389/fped.2020.00414DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7393978PMC
July 2020

Teriflunomide inhibits activation-induced CD25 expression on T cells and may affect Foxp3-expressing regulatory T cells.

Res Vet Sci 2020 Oct 17;132:17-27. Epub 2020 May 17.

Department of Animal Reproduction with Clinic, Faculty of Veterinary Medicine, University of Warmia and Mazury in Olsztyn, Oczapowskiego 14, 10-719 Olsztyn, Poland.

Teriflunomide (TER) is an immunomodulatory agent. Although the first reports on the use of TER in dogs have already appeared, immune mechanisms underlying the immunomodulatory effect of TER do not seem to have been fully elucidated yet. There were two aspects of this study. First, further insight into the mode of action of TER was gained by investigating its effect on the expression of IL-2 receptor α-chain (CD25) and Forkhead box P3 (Foxp3) by CD4 and CD8 T cells and apoptosis of these cells. Second, in view in the earlier lack of data on the effect of TER on T cells in dogs, the results of this study filled in this gap. TER at a concentration which can be achieved in vivo prevented or reduced the activation-induced CD25 expression on CD4 and CD8 T cells, respectively. Taking into consideration the role of CD25 in T cell proliferation, this effect may constitute an additional mechanism responsible for the antiproliferative effect of the drug. Under stimulation conditions, TER induced Foxp3 expression in Foxp3-negative CD4 and CD8 T cells, while down-regulating it under unstimulated conditions. These results suggest that TER may generate iTreg cells, but this process requires cell activation. TER was not found to affect on the absolute count and apoptosis of CD4 and CD8 T cells. The results suggest that the impairment of CD25 expression during T cell activation and generation of iTreg cells may constitute additional mechanisms, besides the principal one, underlying the immunomodulatory effect of TER.
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http://dx.doi.org/10.1016/j.rvsc.2020.05.011DOI Listing
October 2020

Neonatal cholestasis due to citrin deficiency: diagnostic pitfalls.

Acta Biochim Pol 2020 May;67(2):225-228

Department of Gastroenterology, Hepatology, Feeding Disorders and Pediatrics, The Children's Memorial Health Institute, Warsaw, Poland.

Citrin deficiency can manifest in newborns or infants as neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD). The paper presents a case of Polish NICCD patient presenting with low birth weight, failure to thrive, prolonged cholestatic jaundice with coagulopathy and hypoalbuminemia with normal results of MS/MS newborn screening but with high blood citrulline level observed at 3 months of age. Unreported findings included N-hypoglycosylation and increased serum very-long-chain fatty acids (VLCFA), probably secondary to liver impairment. Final diagnosis was established based on whole-exome sequencing (WES) analysis.
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http://dx.doi.org/10.18388/abp.2020_5202DOI Listing
May 2020

Liver involvement in NGLY1 congenital disorder of deglycosylation.

Pol J Pathol 2020 ;71(1):66-68

Department of Pediatrics, Nutrition and Metabolic Diseases, The Children's Memorial Health Institute, Warsaw, Poland.

N-glycanase 1 deficiency is a congenital disorder of deglycosylation, which has been diagnosed in 27 patients, including 2 of them from Poland. The most characteristic symptoms include global developmental disability, hyperkinetic movement disorder, hypo-/alacrimia, and elevated serum transaminases. We reported on a patient in whom the liver biopsy done at the age of 3 years revealed the presence of steatosis, fibrosis, and an amorphous periodic acid-Schiff staining positive diastases-digested material in the cytoplasm.
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http://dx.doi.org/10.5114/pjp.2020.92994DOI Listing
September 2020

Long-Term Effects of Vitamin D Supplementation in Obese Children During Integrated Weight-Loss Programme-A Double Blind Randomized Placebo-Controlled Trial.

Nutrients 2020 Apr 15;12(4). Epub 2020 Apr 15.

Department of Paediatrics, Gastroenterology, Allergology and Paediatric Nutrition, Medical University of Gdańsk, 80-803 Gdańsk, Poland.

Background: Vitamin D was studied in regards to its possible impact on body mass reduction and metabolic changes in adults and children with obesity yet there were no studies assessing the impact of vitamin D supplementation during a weight management program in children and adolescence. The aim of our study was to assess the influence of 26 weeks of vitamin D supplementation in overweight and obese children undergoing an integrated 12-months' long weight loss program on body mass reduction, body composition and bone mineral density.

Methods: A double-blind randomized placebo-controlled trial. Vitamin D deficient patients (<30 ng/ml level of vitamin D) aged 6-14, participating in multidisciplinary weight management program were randomly allocated to receiving vitamin D (1200 IU) or placebo for the first 26 weeks of the intervention.

Results: Out of the 152 qualified patients, 109 (72%) completed a full cycle of four visits scheduled in the program. There were no difference in the level of BMI (body mass index) change - both raw BMI and BMI centiles. Although the reduction of BMI centiles was greater in the vitamin D vs. placebo group (-4.28 ± 8.43 vs. -2.53 ± 6.10) the difference was not statistically significant ( = 0.319). Similarly the reduction in fat mass-assessed both using bioimpedance and DEXa was achieved, yet the differences between the groups were not statistically significant.

Conclusions: Our study ads substantial results to support the thesis on no effect of vitamin D supplementation on body weight reduction in children and adolescents with vitamin D insufficiency undergoing a weight management program.
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http://dx.doi.org/10.3390/nu12041093DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7230345PMC
April 2020

Atherosclerotic Risk Factors in Children with Celiac Disease.

Gastroenterol Res Pract 2020 27;2020:6138243. Epub 2020 Mar 27.

Department of Gastroenterology, Hepatology and Feeding Disorders, Children's Memorial Health Institute, Warsaw, Poland.

Results: We found significantly lower concentrations of total cholesterol, lipoprotein LDL-C, apolipoproteins A1 and B, as well as hCRP in all children with CD. We showed decreased level (<5 ng/mL) of folic acid among 46% of children treated for >5 years. Moreover, we showed significant decrease of folic acid level already after 1 year of a GFD (12 . 5.6 ng/mL; < 0.001). We also found significant negative correlation of -score body mass index (BMI) with HDL and APOA1 level ( = -0.33; = 0.015 and = -0.28; = 0.038, respectively) and modest positive correlation of -score BMI with atherogenic factor of total cholesterol-HDL ratio and LDL-HDL ratio ( = 0.40; = 0.002 and = 0.36; = 0.006, respectively). Analysis of physical activity showed an increase in the insulin levels with inactivity ( = 0.36; = 0.0025). We also found positive correlation of the sleep duration with the adiponectin level ( = 0.41; = 0.011).

Conclusions: In children with CD treated with a GFD, decreased level of folic acid together with increased BMI, sedentary behavior, and an improper lipid profile may predispose them to atherosclerosis in the long run. This data suggests the need of further studies to determine the need for metabolic cardiovascular risk screening in children with CD.
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http://dx.doi.org/10.1155/2020/6138243DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7140124PMC
March 2020

Acute liver failure due to DGUOK deficiency-is liver transplantation justified?

Clin Res Hepatol Gastroenterol 2021 Jan 8;45(1):101408. Epub 2020 Apr 8.

Department of Gastroenterology, Hepatology, Feeding Disorders and Pediatrics, Children's Memorial Health Institute, Al. Dzieci Polskich 20, Warsaw, Poland.

Background: Deoxyguanosine kinase (DGUOK) deficiency is one of the causes of the hepatocerebral form of mitochondrial depletion syndrome (MDS). It is characterized by an early onset of liver failure with concomitant neurological deterioration. In the current literature, there are only few reports regarding long-term observation of children with DGUOK deficiency. Liver transplantation (LTx) is controversial due to extrahepatic involvement and unpredictable outcome.

Methods: Five patients (2 boys) from 4 different families with hepatocerebral MDS associated with DGUOK mutations diagnosed with liver failure were treated in our hospital between 2010-2019.

Results: In all children clinical symptoms developed within the first days of live and hypoglycemia (hypoketotic), conjugated hyperbilirubinemia (cholestasis), severe lactic acidosis, and coagulopathy were observed. Two neonates had low birth-weight for gestational age and failed to thrive. Mild neurological involvement as hypotonia was observed in all children. Three children died at the age of 2, 6 months and 6,5 months of age, respectively, due to end-stage liver failure. In one case, LTx was not considered, in two patients (sisters) parents did not agree to this procedure. LTx was subsequently performed in two patients at the age of 6 and 7 months, respectively, one from deceased, and one from living related donor, in both before the final confirmation of DGUOK mutations. One boy died 2 months after LTx due to post-LTx procedure-related complications; one is still alive with 3years of follow-up, with good liver function and mild neurological disturbances. The diagnosis of DGUOK deficiency was confirmed by biallelic DGUOK mutations detection. Equally, patients were compound heterozygotes (three cases) and homozygotes (two cases). Three known molecular variants, including regulatory substitutions (c.1A>G, c.3G>A) and in-frame insertion (c.813_814insTTT) were identified.

Conclusions: Prognosis in patients with DGUOK deficiency is generally poor. Based on a review of the literature and our experience liver transplantation in selected patients with DGUOK mutation does not appear to be contraindicated, especially in those without or with minimal neurologic abnormalities.
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http://dx.doi.org/10.1016/j.clinre.2020.02.018DOI Listing
January 2021

Mutations in the V-ATPase Assembly Factor VMA21 Cause a Congenital Disorder of Glycosylation With Autophagic Liver Disease.

Hepatology 2020 12;72(6):1968-1986

Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands.

Background And Aims: Vacuolar H+-ATP complex (V-ATPase) is a multisubunit protein complex required for acidification of intracellular compartments. At least five different factors are known to be essential for its assembly in the endoplasmic reticulum (ER). Genetic defects in four of these V-ATPase assembly factors show overlapping clinical features, including steatotic liver disease and mild hypercholesterolemia. An exception is the assembly factor vacuolar ATPase assembly integral membrane protein (VMA21), whose X-linked mutations lead to autophagic myopathy.

Approach And Results: Here, we report pathogenic variants in VMA21 in male patients with abnormal protein glycosylation that result in mild cholestasis, chronic elevation of aminotransferases, elevation of (low-density lipoprotein) cholesterol and steatosis in hepatocytes. We also show that the VMA21 variants lead to V-ATPase misassembly and dysfunction. As a consequence, lysosomal acidification and degradation of phagocytosed materials are impaired, causing lipid droplet (LD) accumulation in autolysosomes. Moreover, VMA21 deficiency triggers ER stress and sequestration of unesterified cholesterol in lysosomes, thereby activating the sterol response element-binding protein-mediated cholesterol synthesis pathways.

Conclusions: Together, our data suggest that impaired lipophagy, ER stress, and increased cholesterol synthesis lead to LD accumulation and hepatic steatosis. V-ATPase assembly defects are thus a form of hereditary liver disease with implications for the pathogenesis of nonalcoholic fatty liver disease.
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http://dx.doi.org/10.1002/hep.31218DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7483274PMC
December 2020

Effects of screen time and playing outside on anthropometric measures in preschool aged children.

PLoS One 2020 2;15(3):e0229708. Epub 2020 Mar 2.

Division of Metabolic and Nutritional Medicine, Department of Pediatrics, Dr. von Hauner Children's Hospital, University Hospital, LMU, Munich, Germany.

Objective: In view of the current obesity epidemic, studies focusing on the interplay of playing outside (PO), screen time (ST) and anthropometric measures in preschool age are necessary to guide evidence-based public health planning. We therefore investigated the relationship between average time spent PO and ST from the ages 3 to 6 years and anthropometric measures at 6 years of age.

Methods: PO and ST of 526 children of the European Childhood Obesity Project (CHOP) were annually assessed by questionnaire from 3 until 6 years of age. Body weight, waist circumference and height were measured at 3 and 6 years of age to calculate Body-Mass-Index z-Scores (zBMI) and waist-to-height ratio (WTH). Linear, logistic and quantile regressions were used to test whether average time spent PO and ST in the 4 year period had an effect on anthropometric measures at age 6 years.

Results: Longer daily ST was associated with a higher zBMI (P = 0.002) and WTH (P = 0.001) at 6 years of age. No significant associations were found for time spent PO. Each additional hour of average ST during the 4 year period resulted in a 66% higher risk of having a zBMI score over 1 (P < 0.001) and almost twice the risk (94% higher risk) of having an zBMI score over 2 (P < 0.001) at 6 years.

Conclusions: Excessive ST during preschool age is a risk factor for increased zBMI at 6 years, regardless of time spent PO. Reducing high levels of ST during preschool age, for e.g. at least 1h per week, could help preventing childhood obesity.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0229708PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7051070PMC
June 2020

Influences of Parental Snacking-Related Attitudes, Behaviours and Nutritional Knowledge on Young Children's Healthy and Unhealthy Snacking: The ToyBox Study.

Nutrients 2020 Feb 7;12(2). Epub 2020 Feb 7.

Department of Nutrition and Dietetics, School of Health Science and Education, Harokopio University, 17671 Athens, Greece.

This study investigated parental influences on preschool children's healthy and unhealthy snacking in relation to child obesity in a large cross-sectional multinational sample. Parents and 3-5 year-old child dyads ( = 5185) in a kindergarten-based study provided extensive sociodemographic, dietary practice and food intake data. Parental feeding practices that were derived from questionnaires were examined for associations with child healthy and unhealthy snacking in adjusted multilevel models, including child estimated energy expenditure, parental education, and nutritional knowledge. Parental healthy and unhealthy snacking was respectively associated with their children's snacking (both < 0.0001). Making healthy snacks available to their children was specifically associated with greater child healthy snack intake ( < 0.0001). Conversely, practices that were related to unhealthy snacking, i.e., being permissive about unhealthy snacking and acceding to child demands for unhealthy snacks, were associated with greater consumption of unhealthy snacks by children, but also less intake of healthy snacks (all < 0.0001). Parents having more education and greater nutritional knowledge of snack food recommendations had children who ate more healthy snacks (all < 0.0001) and fewer unhealthy snacks ( = 0.002, < 0.0001, respectively). In the adjusted models, child obesity was not related to healthy or unhealthy snack intake in these young children. The findings support interventions that address parental practices and distinguish between healthy and unhealthy snacking to influence young children's dietary patterns.
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http://dx.doi.org/10.3390/nu12020432DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7071198PMC
February 2020

Controlled attenuation parameter and liver stiffness measurements using transient elastography by FibroScan in Gaucher disease.

Mol Genet Metab 2020 02 1;129(2):125-131. Epub 2019 Nov 1.

Department of Pediatrics, Nutrition and Metabolic Diseases, The Children's Memorial Health Institute, Warsaw, Poland. Electronic address:

Background: Liver involvement in Gaucher disease (GD) is a result of glucosylceramide (GL1) and its deacylated lysolipid, glucosylsphingosine (lyso-GL1) infiltration of macrophages. The long-term liver-related complications of GD could include liver fibrosis and cirrhosis. The aim of the study was to evaluate clinical utility and relevance of TE by FibroScan in GD patients by assessing two parameters: controlled attenuation parameter (CAP) and liver stiffness (LS), in regard of GD-related variables, type of GD, age of patients, enzymatic replacement therapy (ERT), and metabolic features.

Methods: 59 Polish patients (55 adults, 4 children) with GD (43 patients with type 1 and 16 patients with type 3) aged 7-86 years, underwent TE by FibroScan; elevated CAP was defined as >250 dB/m and elevated LS as >7 kPa. All patients, except five patients with type 1 GD (patients' refusal), were treated by ERT.

Results: Elevated CAP was present in 23% of GD1 patients and 19% of GD3 patients. Elevated LS was present in 21% of GD1 patients and 13% of GD3 patients. CAP was fairly, positively (ρ = 0.356) correlated with BMI. LS was fairly, positively (ρ = 0.4) correlated with patient's age, as well as the age at start of ERT (ρ = 0.326). CAP was strongly, negatively (ρ = -0.52) correlated with the age at start of ERT. LS and CAP were correlated (strongly, positively) only in GD3.

Conclusions: TE by FibroScan could be considered as an additional method for evaluating GD patients for non-invasive assessment of CAP and LS. The investigation of serial TE measurements in untreated as well as treated GD patients is needed to better determine whether this technology should be added to recommendations for monitoring GD patients. TE by FibroScan could be performed in GD patients with increased BMI and especially those with metabolic syndrome as they have other important risks for liver disease. After our analysis we think these risks factors are independent of GD but still very important for their overall health.
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http://dx.doi.org/10.1016/j.ymgme.2019.10.013DOI Listing
February 2020

Shiga toxin-producing Escherichia coli isolates from red deer (Cervus elaphus), roe deer (Capreolus capreolus) and fallow deer (Dama dama) in Poland.

Food Microbiol 2020 Apr 22;86:103352. Epub 2019 Oct 22.

Department of Epizootiology, Faculty of Veterinary Medicine, University of Warmia and Mazury in Olsztyn, Oczapowskiego 13, 10-718, Olsztyn, Poland.

Shiga toxin-producing Escherichia (E.) coli (STEC) pathogens are responsible for the outbreaks of serious diseases in humans, including haemolytic uraemic syndrome (HUS), bloody diarrhoea (BD) and diarrhoea (D), and they pose a significant public health concern. Wild ruminants are an important environmental reservoir of foodborne pathogens that can cause serious illnesses in humans and contaminate fresh products. There is a general scarcity of published data about wildlife as a reservoir of foodborne pathogens in Poland, which is why the potential epidemiological risk associated with red deer, roe deer and fallow deer as reservoirs of STEC/AE-STEC strains was evaluated in this study. The aim of the study was to investigate the prevalence of STEC strains in red deer (Cervus elaphus), roe deer (Capreolus capreolus) and fallow deer (Dama dama) populations in north-eastern Poland, and to evaluate the potential health risk associated with wild ruminants carrying STEC/AE-STEC strains. We examined 252 rectal swabs obtained from 134 roe deer (Capreolus capreolus), 97 red deer (Cervus elaphus) and 21 fallow deer (Dama dama) in north-eastern Poland. The samples were enriched in modified buffered peptone water. Polymerase chain reaction (PCR) assays were conducted to determine the virulence profile of stx1, stx2 and eae or aggR genes, to identify the subtypes of stx1 and stx2 genes, and to perform O and H serotyping. E. coli O157:H7 isolates were detected in the rectal swabs collected from 1/134 roe deer (0.75%) and 4/97 red deer (4.1%), and they were not detected in fallow deer (Dama dama). The remaining E. coli serogroups, namely O26, O103, O111 and O145 that belong to the "top five" non-O157 serogroups, were detected in 15/134 roe deer (11.19%), 18/97 red deer (18.56%) and 2/21 fallow deer (9.52%). STEC/AE-STEC strains were detected in 33 roe deer isolates (24.63%), 21 red deer isolates (21.65%) and 2 fallow deer isolates (9.52%). According to the most recent FAO/WHO report, stx2a and eae genes are the primary virulence traits associated with HUS, and these genes were identified in one roe deer isolate and one red deer isolate. Stx2 was the predominant stx gene, and it was detected in 78.79% of roe deer and in 71.43% of red deer isolates. The results of this study confirmed that red deer and roe deer in north-eastern Poland are carriers of STEC/AE-STEC strains that are potentially pathogenic for humans. This is the first report documenting the virulence of STEC/AE-STEC strains from wild ruminants in Poland.
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http://dx.doi.org/10.1016/j.fm.2019.103352DOI Listing
April 2020

Survival and fertility of bitches undergoing caesarean section.

Vet Rec 2020 Apr 3;186(13):416. Epub 2019 Oct 3.

Department of Animal Reproduction with Clinic, Uniwersytet Warminsko-Mazurski, Olsztyn, Poland.

Background: With the increasing popularity of planned caesarean section, the need for knowledge regarding this surgery has become increasingly important. The reported death and survival rates for caesarean sections vary widely. Another important aspect is the fertility rate in subsequent oestrous after caesarean section. The aim of this study was to investigate the mortality and survival rate of bitches during caesarean section. Additionally, the fertility of bitches after caesarean sections was determined.

Methods: Caesarean sections which were performed in the years 1997-2009 at two university clinics were evaluated retrospectively. A distinction was made between bitches in which a conservative caesarean section was performed and bitches with a caesarean section followed by an ovariohysterectomy.

Results: A total of 482 caesarean sections were included in the study. The overall mortality rate was 3.11 per cent, with 2.59 per cent during or after a conservative caesarean section and 4.19 per cent during or after caesarean section with ovariohysterectomy. The reason for ovariohysterectomy was the owner's preference in 63 bitches (47.01 per cent); in 71 (52.98 per cent) bitches, ovariohysterectomy was performed due to a medical indication. The fertility rate after caesarean section was 100 per cent.

Conclusion: The results show a high mortality rate during and after caesarean section. On the other hand, caesarean section does not seem to have a big impact on further fertility. Further studies are needed to investigate possible reduction of litter sizes and the suitability of caesarean section in subsequent pregnancies.
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http://dx.doi.org/10.1136/vr.105123DOI Listing
April 2020
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