Is chronic pancreatitis in children an autoimmune disease?
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Adolescent↗
Biomedical subjects
Jerzy Socha
Publications and source records attributed to Jerzy Socha.
Normal pancreatic secretion in children with progressive familial intrahepatic cholestasis type 1.
OBJECTIVE: Progressive familial intrahepatic cholestasis type 1 (PFIC1) is a rare, autosomal, recessive, inherited disease resulting from mutations in the ATP8B1 gene which is expressed at high levels in the small intestine and pancreas and at lower levels in the liver. Given this expression pattern, patients might be expected to have a pancreatic phenotype. Although pancreatitis and steatorrhea have been reported in patients with PFIC1, the available data on pancreatic function are not fully convincing. Therefore, the objective of this study was to assess exocrine pancreatic function in patients with PFIC1. MATERIAL AND METHODS: Three subjects with a diagnosis of PFIC1 were included in the study. The diagnosis was confirmed by molecular analysis of ATP8B1. Prior to surgical treatment (biliary diversion), two patients had steatorrhea and in the third patient, a borderline value for fecal fat excretion was documented. In one patient, liver transplantation also was subsequently performed. Exocrine pancreatic secretion was assessed by the use of fecal elastase-1 and chymotrypsin tests. Fecal lipase concentrations were determined in order to exclude isolated lipase deficiency. Other typical diagnostic procedures were performed annually. RESULTS: The results of the fecal tests were within the normal range. None of the three patients experienced any episodes that could be related to acute or chronic pancreatitis. Laboratory tests including serum amylase and lipase tests were always normal. Abdominal ultrasonography findings did not show any pancreatic pathology. CONCLUSIONS: Pancreatic secretion in the study patients with progressive familial intrahepatic cholestasis type 1 was normal. The observed steatorrhea was not related to pancreatic insufficiency.
Adenosine Triphosphatases↗
Exocrine pancreatic function in children with progressive familial intrahepatic cholestasis type 2.
In progressive familial intrahepatic cholestasis type 2 (PFIC-2), severe steatorrhea is often documented. However, pancreatic exocrine secretion has not yet been studied. In 14 children with PFIC-2, pancreatic function was assessed using standard fecal tests. Normal fecal lipase concentrations excluded isolated lipase deficiency. No differences in fecal elastase-1 concentrations and chymotrypsin activities were detected between PFIC-2 patients with or without steatorrhea, nor between these patients and healthy subjects. In conclusion, pancreatic exocrine function in patients with PFIC-2 is normal. Steatorrhea observed in those patients is not related to pancreatic insufficiency.
Adolescent↗
Analysis of CFTR, SPINK1, PRSS1 and AAT mutations in children with acute or chronic pancreatitis.
OBJECTIVES: Defects of PRSS1, SPINK1, CFTR and AAT are considered causative or predisposing to pancreatitis. The aim of this study was to evaluate the impact of these defects into molecular pathology of chronic pancreatitis (CP) and acute recurrent pancreatitis (ARP). METHODS: Ninety-two children with CP or ARP, 55 family members and 50 controls were investigated. The subjects were screened for PRSS1 mutations: R122H, R122C, A16V, N29I; SPINK1 N34S variant; panel of 14 CFTR defects: INNOLiPA CFTR12, CFTRdele2,3 and IVS8-T variant or panel of 3 CFTR defects-F508del, CFTRdele2,3 and IVS8-T; AAT mutations: E264V, E342K. RESULTS: We identified 1 mutated allele in at least 1 of 4 genes in 31 of 92 patients and 12 of 50 controls (P = 0.157). Mutations in SPINK1 and PRSS1 were most frequent. PRSS1 mutations were identified mainly in CP patients (9.6% of CP vs 2.5% of ARP alleles, P = 0.094), whereas N34S SPINK1 mutation was present with comparable frequency in CP and ARP patients (7.7% vs 10.0%, P = 0.768). The frequency of mutations in CFTR alleles was similar to controls (4.9% vs 5%, P = 0.587). Overall frequency of AAT mutations was lower than in the controls. Family studies showed that defects in the examined genes did not always segregate with disease. CONCLUSIONS: PRSS1 defects seem to be causative for pancreatitis, whereas defects in SPINK1 are suggested to be associated with the disease. No association between CFTR mutations and pancreatitis was observed. The importance of AAT variants remains speculative.
Acute Disease↗
Persistence of HBV-DNA in children with chronic hepatitis B who seroconverted to anti-HBs antibodies after interferon-alpha therapy: correlation with specific IgG subclass responses to HBsAg.
BACKGROUND/AIMS: We examined the persistence of HBV-DNA in sera collected 4-10 years after IFN-alpha therapy from patients with chronic hepatitis B who had seroconverted to anti-HBs antibodies. We also wanted to assess whether any association exists between HBV-DNA status and the IgG anti-HBs subclass responses. METHODS: Sera were obtained from 38 patients and the following parameters were determined in each of them: (1) serological markers of HBV; (2) concentrations of IgM, IgG, IgA; (3) total IgG subclasses. HBV-DNA and IgG anti-HBs subclasses were determined in anti-HBs positive sera. RESULTS: Four to 10 years after therapy, anti-HBs were found in 37 of 38 patients (GMT: 775IU/L). In 13 of them (35.1%) free and/or bound HBV-DNA was present in sera. Significant differences in the profiles of IgG anti-HBs were observed when the HBV-DNA status was considered. Patients with undetectable HBV-DNA responded mainly with IgG1 and/or IgG3, while in the HBV-DNA-positive group, a high contribution of IgG4 was found. CONCLUSIONS: Our study showed that HBV-DNA may persist for a long time after IFN-alpha therapy despite the appearance of anti-HBs antibodies. The monitoring of specific IgG subclasses may be of predictive value for HBV-DNA persistence.
Adolescent↗
Essential fatty acid depletion in children with inflammatory bowel disease.
OBJECTIVE: Children with inflammatory bowel disease (IBD) suffer from malabsorption and malnutrition and therefore may be at risk of developing polyunsaturated fatty acid (PUFA) deficiency. The aim of this study was to investigate PUFA status in children with IBD and the possible relationship to disease activity and nutritional status. MATERIAL AND METHODS: We assessed the fatty acid composition of plasma phospholipids (%wt/wt) of 21 children aged 5.5-18 years with IBD (ulcerative colitis, 15; Crohn's disease, 6) with mild or moderate disease activity. The clinical symptoms and biochemical indices of disease activity and nutritional status (lean and fat body mass, Hb, albumin serum conc.) were also determined. RESULTS: The patients had lower phospholipid PUFAs than 13 healthy, aged-matched controls (25.8+/-5.2 versus 34.2+/-5.7, M+/-SD, p<0.001), mainly due to lower values of linoleic acid (18:2n-6, 14.0+/-3.8 versus 18.3+/-4.3, p<0.01) and its major metabolite arachidonic acid (20:4n-6, 5.3+/-2.0 versus 9.3+/-1.9, p<0.0001). There were also higher values of a-linolenic acid (18:3n-3, 0.3+/-0.4 versus 0.2+/-0.1, p<0.01) while the long-chain n-3 PUFA-eicosapentaenoic and docosahexaenoic acids were normal. Total n-6 PUFA correlated inversely to erythrocyte sedimentation rate (p<0.01), seromucoid (p<0.05) and positively to Hb concentration (p<0.01). CONCLUSIONS: Children with inflammatory bowel disease have a high risk of n-6 PUFA depletion, which is related to disease activity.
Adolescent↗
[Diagnostic and therapeutic difficulties in management of patients with autoimmune hepatitis and HCV coinfection--case reports].
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Adolescent↗
[Complications in a patient with liver cirrhosis and HCV coinfection during treatment with peginterferon and ribavirin].
Authors present a case of 15-years old girl with liver cirrhosis due to biliary atresia with HCV coinfection with portal hypertension after several episodes of variceal bleeding treated primarily with sclerotherapy and later with transjugular intrahepatic porto-systemic stent shunt. In a patient during the combined peginterferon alfa-2b and ribavirin treatment a decompensation of liver occurred with necessity for liver transplantation.
Adolescent↗
[Persistence of hepatitis B virus in children after interferon-alpha therapy despite the seroconversion in HBsAg/anti-HBs system].
OBJECTIVE: The aim of our study was to assess: (1) whether seroconversion to IgG anti-HBs, induced by IFN-alpha therapy in children with chronic active hepatitis B, is maintained 4-10 years after treatment; and (2) whether HBV-DNA is present in circulation despite the synthesis of anti-HBs. METHODS: Serum samples were collected from 38 patients and serological markers of HBV were determined in each of them. HBV-DNA was determined by PCR in anti-HBs positive sera. Serum samples obtained from 19 subjects with a complete spontaneous seroconversion after acute HBV served as controls. RESULTS: Four to 10 years after therapy, anti-HBs were present in ca. 97.4% patients with GMT value of 775 IU/L vs 127 IU/L found in controls. HBsAg was found in 2/38 subjects. In 13 out of 37 patients (35.1%) free and/or bound HBV-DNA was present. CONCLUSIONS: This study showed that seroconversion induced by IFN-alpha may not be a sustained phenomenon. HBV-DNA may persist for a long time after therapy despite the anti-HBs synthesis.
Adolescent↗
Magnesium status in children and adolescents with coeliac disease without malabsorption symptoms.
BACKGROUND & AIMS: Magnesium deficiency has been reported in coeliac disease (CD) with clinical symptoms. The aim of this study was to evaluate magnesium status in CD without malabsorption symptoms in patients aged 5-18 years. In the study participated 41 coeliac patients on a gluten-free diet (GFD) for a mean 11 years with IgAEmA (-) and normal villi, 28 patients with untreated subclinical (atypical) CD with IgAEmA (+) and villous atrophy, and 8 healthy subjects as a control group. METHOD: Magnesium status was examined by an intravenous Mg loading test, determination of serum and erythrocyte Mg concentrations, and urinary excretion. Steatorrhea was assessed by fecal fat excretion. Magnesium, calcium, protein, and fat intake were evaluated by a food frequency questionnaire. RESULTS: The frequency of magnesium deficiency as assessed by the magnesium loading test was: 19.6% in treated, 21.4% in untreated CD patients, and 25% in control group, similar in both sexes, sporadic in small towns (3.8%), frequent in large cities and rural villages (23.5% vs. 32.3%). Fat excretion was within normal limits. The following results were found, respectively: decreased serum magnesium concentration, 7.3% vs. 3.6% vs. 0%, decreased erythrocyte Mg concentration, 14.6% vs. 25% vs. 12.5%; decreased intake of magnesium, 29% vs. 32% vs. 0%. CONCLUSIONS: The frequency of magnesium deficiency was similar in pediatric patients who were treated for CD or in patients with subclinical CD, and in control group; magnesium deficiency in patients participating in our study depended on an imbalanced diet.
Adolescent↗
Endoscopic ligation of esophageal varices for prophylaxis of first bleeding in children and adolescents with portal hypertension: preliminary results of a prospective study.
BACKGROUND/PURPOSE: Endoscopic variceal ligation (EVL) is effective in controlling rebleeding from esophageal varices in children, but there is no data on the use of EVL to prevent initial bleeding. The objective of this study was to prospectively evaluate the efficacy of EVL in preventing the first hemorrhage from esophageal varices in children. METHODS: Thirty-seven children with portal hypertension (22 liver cirrhosis, 15 portal vein thrombosis), aged 4 to 17 years (M = 9.5 +/- 4.4 years) were included in the study. The criteria for inclusion were (1) no previous variceal bleeding; (2) the presence of esophageal varices classified grade II or more, and (3) their enlargement by at least I grade after 6 months of observation without endoscopic treatment or appearance of endoscopic signs of high bleeding risk. A Multi-Band Ligator was used, and 2 to 6 bands were fixed under general anesthesia during one procedure depending on the number and size of varices. Follow-up examinations were performed every 3 months, repeating the procedure if necessary. In total, 75 procedures of EVL were performed, from one to 5 in each patient RESULTS: Four patients underwent liver transplantation before eradication of varices. Two others were excluded from the observation because of lack of compliance to the protocol. Of the remaining 31 patients, eradication of varices was achieved in 28 children (90.3%) after 2.0 EVL sessions performed at 3-month intervals. The average time of follow-up after cessation of treatment is 16 months. No bleeding from varices occurred in any child during or after treatment. There were no differences in results between children with liver cirrhosis and portal vein thrombosis. Development of hypertensive gastropathy was observed in 2 children with one episode of bleeding. Recurrence of varices without bleeding occurred in 3 children after 12, 13, and 28 months from eradication. CONCLUSIONS: The study results confirmed that endoscopic variceal ligation is a safe and highly effective procedure in children with portal hypertension, regardless of its etiology. Eradication of esophageal varices was followed by 16 months free of bleeding. Prolonged observation is mandatory to conclude if preventive EVL influences the natural history of disease and diminishes the risk of first bleeding onset.
Adolescent↗
Long-chain PUFA supplementation improves PUFA profile in infants with cholestasis.
Long-chain PUFA (LCP) deficiency is a frequent complication in cholestatic infants. We investigated the effects of LCP-supplemented formula on EFA status in infants with cholestasis. Twenty-three infants with cholestasis (biliary atresia after surgery, 8; intrahepatic cholestasis, 15) aged 1.9 to 4.9 mon (median 3.1 mon) were randomized to receive commercial infant formulas either without LCP or with LCP from egg phospholipids for 1 mon. Liver tests, nutrient intakes, and plasma phospholipid FA (%w/w) were determined at baseline and after intervention. At baseline, patients had high serum direct bilirubin levels (5.9 +/- 3.0 mg/dL; mean +/- SD), they were malnourished (body fat mass: 40 +/- 13% of normal) and presented with PUFA deficiency [plasma phospholipid PUFA: 28.43%w/w (26.56-30.53) in patients vs. 37.02%w/w (34.53-39.58) in controls; median (1st-3rd quartile)] with elevated Mead acid and palmitoleic acid. LCP-supplemented (n = 11) and -nonsupplemented groups (n = 12) did not differ in age, indicators of liver function, and EFA status at baseline. After the intervention, LCP-supplemented infants had higher levels of arachidonic acid [7.2 (5.9-8.8) vs. 4.2 (3.0-5.3) %w/w; P < 0.001] and DHA [2.8 (2.2-3.2) vs. 1.6 (1.0-2.1) %w/w; P < 0.05], accompanied by increased TBARS concentration: 1.9 (1.4-2.2) vs. 1.3 (1.1-1.6) nmol/mL; P < 0.05]. We concluded that LCP-supplemented formulae improve LCP status of infants with severe cholestasis but may enhance lipid peroxidation.
Bile Acids and Salts↗
Neutral endopeptidase activity is not elevated in serum in children with cholestatic liver disease: a unique role of aminopeptidase-m in sequential hydrolysis of peptides.
The aim of this study was to determine whether neutral endopeptidase activity is elevated in serum in children with cholestatic liver disease (Alagille syndrome), and whether the enzyme cooperates with the serum aminopeptidase-M in degradation of peptides. Our data suggest that neutral endopeptidase activity remains at a very low level,.undetectable with the assays we have applied, both in the serum from healthy children and those with cholestasis. In contrast, the serum aminopeptidase-M activity is highly increased in cholestasis. We have demonstrated that aminopeptidase-M alone is capable of sequential and complete hydrolysis of enkephalins and low-molecular-weight, nonspecific peptides. The rate of release of free amino acids from both exogenous or endogenous substrate peptides was statistically significantly higher in serum in children with cholestasis compared with healthy children (P < 0.05). Substance P (Ki = 2.5 micromol/liter) and bradykinin (Ki = 27 micromol/liter) were shown to be potent inhibitors of the serum aminopeptidase-M. We postulate that aminopeptidase-M, except when regulating the activity of bioactive peptides, may serve as a scavenger of short, nonspecific peptides in the circulation. Our data seem to provide new insights for further studies on the role of serum peptidases both in physiology and pathophysiology.
Adolescent↗
[Individual sensitivity of jejunal mucosa to small doses of gluten in coeliac disease ].
In coeliac patients the age of development of symptoms, clinical picture of the disease and complications depend on the dose of ingested gluten. The aim of the study was the evaluation of individual sensitivity to small doses of gluten in the group of 60 patients aged 2.65 to 17.92 (mean age 7.49) treated with gluten-free diet for at least 12 months due to coeliac disease diagnosed according to ESPGAN criteria (food allergy to gluten excluded). Gluten challenge with dose of 10 mg/kg body mass/day was controlled with serological tests (IgAEmA, IgAAGA, and IgGAGA antibodies) carried out every 3 to 6 months. Jejunal biopsy was performed before gluten challenge (normal mucosa), and after positive EmA/AGA antibodies tests to confirm diagnosis (flat mucosa). After 35 months of observation 53.7% of all patients presented of jejunal villious atrophy, and positive IgAEmA. In this group 3.7% presented symptoms after 3 months of gluten challenge, 5.5% after 6 months, 3.7% after 9 months, and 3.7% after 12 months. In some coeliac patients ingestion of small amounts of gluten (10 mg/kg/day) can lead to small intestinal villious atrophy.
Adolescent↗
[Cholestatic liver disease in children].
Cholestatic liver disease constitutes a large part of chronic liver diseases during infancy. It is caused either by extrahepatic disorders (obstruction) or by intrahepatic cholestasis (functional). The differential diagnosis should be done as early as possible because the delayed surgical therapy in extrahepatic cholestasis has a very bad prognosis. Intrahepatic cholestasis may be caused by a broad spectrum of different disorders such as congenital infection, endocrine, chromosomal abnormalities or inborn errors of metabolism. Familial clustering is typical for the Byler's disease, Alagille's syndrome, PFIC, errors of bile acid synthesis or alpha-1-ATD. The established diagnosis allows to start etiological treatment: dietary--in metabolic diseases, antibiotics--in bacterial infections, antiviral--in viral infections etc. To lower bile acids level pharmacological treatment (UDCA) or surgical procedures (hepatoportoenterostomy, partial biliary diversion) should be performed. In progressive cholestasis the only effective therapy is liver transplantation.
Age Factors↗
[Focal nodular hyperplasia - own experiences].
INTRODUCTION: focal nodular hyperplasia (FNH) is a benign tumour of the liver, often discovered incidentally. It occurs mainly in women between 25-44 years old. Though it is rarefy reported in children the evidence has been growing in the recent years. THE AIM: of our work was to show our experience of focal nodular hyperplasia in pediatric cases. MATERIAL AND METHODS: 10 children, aged 6-17 years, were hospitalized in the Children's Memorial Health Institute between 2000 and 2005, with provisional diagnosis of focal nodular hyperplasia. In all children radiological examination was performed. In some cases laparotomy and operative liver biopsy was carried out. RESULTS: only in 3 children diagnosis of FNH was made from radiological examination. 6 children needed laparotomy and operative liver biopsy. Although operative resection of FNH lesions was carried out in some patients, the authors observed recurrence of hepatic lesions. CONCLUSIONS: our experiences confirm that nowadays the conservative approach should to be standard procedure in children with FNH. Only in doubtful cases laparotomy is recommended.
Adolescent↗
[Efficacy and safety of cyclosporine in the treatment of inflammatory bowel disease in children - a retrospective study].
AIM: the aim of this study was to report single centre experience with cyclosporine used in treatment of children with inflammatory bowel disease with regard to safety and efficacy. METHODS: retrospective analysis included 23 patients, 21 with ulcerative colitis and 2 with Crohn's disease, aged 2.75 to 18.5 years. They were treated with cyclosporine during the last 5 years. Before cyclosporine therapy was started they received steroids and azathioprine. Cyclosporine treatment was given in severe steroid-resistant exacerbation of the disease (n = 10) or steroid-dependence (n = 13). Cyclosporine dose was set to obtain therapeutic levels (serum concentration > 100 ng/ml and < 200 ng/ml). RESULTS: Cyclosporine treatment was continued up to 2 months in 6 cases, 2 to 6 months in 8 patients and more than 6 months in 9 patients. Complications were reported in 2 patients: hirsutism and gingival hypertrophy. Cyclosporine treatment was stopped in the second case. None of the two patients with Crohn's disease improved during the treatment. Short-term improvement was observed in 11 patients with ulcerative colitis. Long-term recovery (> 6 months) was obtained in 6 cases. In 10 patients with severe exacerbation of ulcerative colitis colectomy was performed, in 4 of them elective surgery was performed when the clinical status improved. CONCLUSION: cyclosporine appears to be a safe and relatively effective treatment of ulcerative colitis in children. Cyclosporine is less effective in maintaining remission and it did not allow to avoid colectomy in severe exacerbation. Still case controlled studies are needed to show the efficacy of this treatment.
Administration, Oral↗