Intractable ascites is a rare condition in children mainly caused by cirrhosis or lymphatic disorders. Internal drainage may be considered as rescue therapy. In our department, 4 patients ages from 2 months to 15 years old underwent a peritoneovenous shunt (PVS) placement between 2010 and 2020. The surgically inserted device was a pumping device that enabled to drain ascites from the peritoneum into the venous system via the internal jugular vein (Denver shunt, BD Company, NJ). Immediate efficient drainage was achieved in all cases and lasted up to 9 years. Two major complications occurred a postoperative fat embolism requiring urgent temporary ligation of the shunt and endocarditis shortly after inguinal hernia repair performed 16 months after placement of the shunt. Implementation of a PVS may be a useful procedure in patients with refractory ascites. Chylous ascites should be drained and washed totally before activating the device to avoid fat embolism. https://www.selleckchem.com/products/dyngo-4a.html Antibiotic prophylaxis is required when abdominal siotic prophylaxis is required when abdominal surgery is planned while the device is in place.
The aim of our study was to examine longitudinal changes in bone mineral density (BMD) of children and adolescents with Crohn's disease, and risk factors related to low BMD.
All patients aged from 2 to 18 years with CD who underwent dual-energy X-ray absorptiometry (DXA) at diagnosis and at the end of follow-up between 1999 and 2018 were considered for inclusion in this retrospective study. Factors related to changes in BMD at diagnosis and during follow-up were investigated.
193 patients had the 2 DXA required.At diagnosis 36 patients (18.7%) had a low BMD.At the end of follow-up, 31 patients (16%).164 patients did not have the 2 DXA required.In included CD, BMD values were lower in lumbar spine (LS) than in total body less head (TBLH), as well at diagnosis (p < 0.0001) or at the end of follow-up (p = 0.001).At diagnosis, only growth impairment or low BMI was associated with low BMD (p < 0.0001), only cumulative dose of corticosteroid at the end of follow-up (p = 0.01).
The high prevalence of low BMD in children and adolescents with IBD highlights the importance of evaluating BMD in these patients at the time of diagnosis and throughout the course of their treatment. Special attention must be given to patients with height delay or low BMI at diagnosis. Long-term glucocorticoid therapy is the main clinical risk factor associated with low BMD at the end of follow-up.
The high prevalence of low BMD in children and adolescents with IBD highlights the importance of evaluating BMD in these patients at the time of diagnosis and throughout the course of their treatment. Special attention must be given to patients with height delay or low BMI at diagnosis. Long-term glucocorticoid therapy is the main clinical risk factor associated with low BMD at the end of follow-up.
Celiac disease (CD) is a common intestinal autoimmune disorder with diverse presenting features. We aimed to determine age-dependent patterns in CD presentation, diagnosis and management at a large tertiary referral center.
A retrospective review of electronic medical records of pediatric patients diagnosed with CD between January 1999 and December 2018 at Schneider Children's Medical Center of Israel. We compared demographics, clinical and laboratory parameters between four age groups at CD presentation.
A cohort of 932 children was divided into four groups by age (in years) at diagnosis 0-3 (17.9%), 3-6 (31.8%), 6-12 (34.5%), 12-18 (15.8%). The youngest age group presented more frequently with diarrhea, weight loss, abdominal distention, vomiting and lower weight z scores, P < 0.01. Hypoalbuminemia and zinc deficiency were also more frequent in this age group, compared to older patients (P < 0.05, each). Rates of anemia were higher in younger age groups (0-3 and 3-6 years), compared to older age groups, P < 0.05. Patients in the younger age groups (0-3 and 3-6 years) presented more frequently with tissue transglutaminase (TTG) levels above 10 times the upper limit of normal (ULN; P < 0.05), and more often normalized their CD serologies by 24 months of gluten-free diets (GFD) compared to older age groups (P < 0.05).
There is an age-dependent variation in CD presentation during childhood. Younger patients present more often with malabsorptive features, and higher TTG levels, yet normalize TTG while on GFD more rapidly than older patients. Clinicians should be aware of the diversity in CD presentation and course at the various presentation age.
There is an age-dependent variation in CD presentation during childhood. Younger patients present more often with malabsorptive features, and higher TTG levels, yet normalize TTG while on GFD more rapidly than older patients. Clinicians should be aware of the diversity in CD presentation and course at the various presentation age.
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.
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.
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.
Our results suggest that serum L-FABP could be a novel biomarker of liver fibrosis in WD children.
Our results suggest that serum L-FABP could be a novel biomarker of liver fibrosis in WD children.
Intractable ascites is a rare condition in children mainly caused by cirrhosis or lymphatic disorders. Internal drainage may be considered as rescue therapy. In our department, 4 patients ages from 2 months to 15 years old underwent a peritoneovenous shunt (PVS) placement between 2010 and 2020. The surgically inserted device was a pumping device that enabled to drain ascites from the peritoneum into the venous system via the internal jugular vein (Denver shunt, BD Company, NJ). Immediate efficient drainage was achieved in all cases and lasted up to 9 years. Two major complications occurred a postoperative fat embolism requiring urgent temporary ligation of the shunt and endocarditis shortly after inguinal hernia repair performed 16 months after placement of the shunt. Implementation of a PVS may be a useful procedure in patients with refractory ascites. Chylous ascites should be drained and washed totally before activating the device to avoid fat embolism. https://www.selleckchem.com/products/dyngo-4a.html Antibiotic prophylaxis is required when abdominal siotic prophylaxis is required when abdominal surgery is planned while the device is in place.
The aim of our study was to examine longitudinal changes in bone mineral density (BMD) of children and adolescents with Crohn's disease, and risk factors related to low BMD.
All patients aged from 2 to 18 years with CD who underwent dual-energy X-ray absorptiometry (DXA) at diagnosis and at the end of follow-up between 1999 and 2018 were considered for inclusion in this retrospective study. Factors related to changes in BMD at diagnosis and during follow-up were investigated.
193 patients had the 2 DXA required.At diagnosis 36 patients (18.7%) had a low BMD.At the end of follow-up, 31 patients (16%).164 patients did not have the 2 DXA required.In included CD, BMD values were lower in lumbar spine (LS) than in total body less head (TBLH), as well at diagnosis (p < 0.0001) or at the end of follow-up (p = 0.001).At diagnosis, only growth impairment or low BMI was associated with low BMD (p < 0.0001), only cumulative dose of corticosteroid at the end of follow-up (p = 0.01).
The high prevalence of low BMD in children and adolescents with IBD highlights the importance of evaluating BMD in these patients at the time of diagnosis and throughout the course of their treatment. Special attention must be given to patients with height delay or low BMI at diagnosis. Long-term glucocorticoid therapy is the main clinical risk factor associated with low BMD at the end of follow-up.
The high prevalence of low BMD in children and adolescents with IBD highlights the importance of evaluating BMD in these patients at the time of diagnosis and throughout the course of their treatment. Special attention must be given to patients with height delay or low BMI at diagnosis. Long-term glucocorticoid therapy is the main clinical risk factor associated with low BMD at the end of follow-up.
Celiac disease (CD) is a common intestinal autoimmune disorder with diverse presenting features. We aimed to determine age-dependent patterns in CD presentation, diagnosis and management at a large tertiary referral center.
A retrospective review of electronic medical records of pediatric patients diagnosed with CD between January 1999 and December 2018 at Schneider Children's Medical Center of Israel. We compared demographics, clinical and laboratory parameters between four age groups at CD presentation.
A cohort of 932 children was divided into four groups by age (in years) at diagnosis 0-3 (17.9%), 3-6 (31.8%), 6-12 (34.5%), 12-18 (15.8%). The youngest age group presented more frequently with diarrhea, weight loss, abdominal distention, vomiting and lower weight z scores, P < 0.01. Hypoalbuminemia and zinc deficiency were also more frequent in this age group, compared to older patients (P < 0.05, each). Rates of anemia were higher in younger age groups (0-3 and 3-6 years), compared to older age groups, P < 0.05. Patients in the younger age groups (0-3 and 3-6 years) presented more frequently with tissue transglutaminase (TTG) levels above 10 times the upper limit of normal (ULN; P < 0.05), and more often normalized their CD serologies by 24 months of gluten-free diets (GFD) compared to older age groups (P < 0.05).
There is an age-dependent variation in CD presentation during childhood. Younger patients present more often with malabsorptive features, and higher TTG levels, yet normalize TTG while on GFD more rapidly than older patients. Clinicians should be aware of the diversity in CD presentation and course at the various presentation age.
There is an age-dependent variation in CD presentation during childhood. Younger patients present more often with malabsorptive features, and higher TTG levels, yet normalize TTG while on GFD more rapidly than older patients. Clinicians should be aware of the diversity in CD presentation and course at the various presentation age.
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.
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.
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.
Our results suggest that serum L-FABP could be a novel biomarker of liver fibrosis in WD children.
Our results suggest that serum L-FABP could be a novel biomarker of liver fibrosis in WD children.
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