Double Lumen Polyamide Tube-stent for the Treatment of Recurrent Postcorrosive Esophageal Stenosis.

This article presents the results of endoscopic treatment for recurrent postcorrosive esophageal stenosis with a tube-stent developed at our institution. The tube-stent was implanted in 5 children with corrosive esophageal injury at the age of 2 to 8.5 years after 7 to 64 dilatation sessions during 5 to 118 months. In total, 13 tube-stents were implanted. One patient had undergone 9 procedures during 2.5 years and the tube-stent remained in place for 14 to 250 days. This patient was tube-stent-dependent due to the lack of any possibility of surgical reconstruction. Two patients had the tube-stent removed after 150 to 205 days and they remain free from esophageal restenosis. One patient did not tolerate the tube-stent, evacuated it after 1 day and was referred for surgical esophagus replacement. One patient is currently still being treated with the tube-stent. Tube-stent was well tolerated and it may be effective in children with recurrent critical postcorrosive esophageal stenosis.

Neutrophil gelatinase-associated lipocalin in blood in children with inflammatory bowel disease.

BACKGROUND: Neutrophil gelatinase-associated lipocalin (NGAL) is a 25 kDa glycoprotein present in the bodily fluids and tissues. It is secreted by neutrophils, epithelial cells, hepatocytes and adipocytes, and its expression is highly increased in response to cellular stress. The role of NGAL in the pathophysiology of inflammatory bowel disease including Crohn's disease and ulcerative colitis in children has thus far not been studied. METHODS: The following groups of children were included: (i) inflammatory bowel disease group, n = 36, aged from 1 to 18 years with Crohn's disease (n = 19) and ulcerative colitis (n = 17); (ii) control group, n = 126; and (iii) disease control group, n = 27, without inflammatory bowel disease, with a food and/or inhalant allergy. RESULTS: Healthy children aged from 1 to 8 years exhibited lower NGAL level than those of 9 to 18 years old (39.0; 18.1-83.7 ng/mL vs 57.6; 28.7-107 ng/mL, P = 0.001). In the younger, but not in the older children, the serum NGAL level correlated with their age, r = 0.334, P = 0.001. In children with inflammatory bowel disease, serum NGAL level was higher (108; 37.3-245 ng/mL) than in healthy (42.0; 18.1-107 ng/mL) and allergic, noninflammatory bowel disease children (49.3; 19.3-107 ng/mL), P = 0.001. Serum NGAL levels in Crohn's disease and ulcerative colitis children did not correlate with age, gender, disease activity, and indices of the inflammation. CONCLUSION: Serum NAGL levels are highly elevated in Crohn's disease and ulcerative colitis in children compared to the healthy control group. Systematic studies are needed to explain the role of this protein in the inflammatory bowel disease.

High Resolution Melting analysis as a rapid and efficient method of screening for small mutations in the STK11 gene in patients with Peutz-Jeghers syndrome.

BACKGROUND: Peutz-Jeghers syndrome (PJS) is a rare hereditary syndrome characterized by the occurrence of hamartomatous polyps in the gastrointestinal tract, mucocutaneous pigmentation and increased risk of cancer in multiple internal organs. Depending on the studied population, its incidence has been estimated to range from 1:200 000 even up to 1:50 000 births. Being an autosomal disease, PJS is caused in most cases by mutations in the STK11 gene. METHODS: The majority of causative DNA changes identified in patients with PJS are small mutations and, therefore, developing a method of their detection is a key aspect in the advancement of genetic diagnostics of PJS patients. We designed 13 pairs of primers, which amplify at the same temperature and enable examination of all coding exons of the STK11 gene by the HRM analysis. RESULTS: In our group of 41 families with PJS small mutations of the STK11 gene were detected in 22 families (54%). In the remaining cases all of the coding exons were sequenced. However, this has not allowed to detect any additional mutations. CONCLUSIONS: The developed methodology is a rapid and cost-effective screening tool for small mutations in PJS patients and makes it possible to detect all the STK11 gene sequence changes occurring in this group.

High- versus low-volume polyethylene glycol plus laxative versus sennosides for colonoscopy preparation in children.

OBJECTIVE: Many protocols of bowel preparation are available for use in children; however, none of them is commonly accepted. The aim of the study was to evaluate the efficacy and acceptability of high-volume polyethylene glycol (PEG) versus low-volume PEG combined with bisacodyl (BPEG) versus sennosides for colonoscopy preparation in children. METHODS: Participants ages 10 to 18 years were randomly assigned to receive either PEG 60 or PEG 30 mL kg⁻¹ day⁻¹ plus oral bisacodyl 10 to 15 mg/day or sennosides 2 mg kg⁻¹ day⁻¹ for 2 days. A blinded assessment of bowel cleansing was made by the endoscopist according to the Aronchick and Ottawa scales. Patient acceptability was evaluated with the visual-analog scale. Analysis was done on an available case analysis basis. RESULTS: Of 240 patients enrolled in the study 234 patients were available for analysis of the efficacy of colon cleansing. There were no significant differences found among the 3 groups for the proportions of participants with excellent/good (PEG: 35/79, BPEG: 26/79, sennosides 25/76) and poor/inadequate (PEG: 20/79, BPEG: 28/79, sennosides 28/76) bowel preparation evaluated with the Aronchick scale and for the mean Ottawa total score (PEG: 5.47 ±â€Š3.63, BPEG: 6.22 ±â€Š3.3, sennosides: 6.18 ±â€Š3.53). Acceptability of bowel cleansing protocol was similar in all of the groups (P = 0.8). CONCLUSIONS: All 3 cleansing methods showed similar efficacy and tolerability; however, none of them was satisfactory.

Lipid metabolism and oxidative stress in children after liver transplantation treated with sirolimus.

Lipid disturbances are one of the most frequent side effects of SRL; however, clinical consequences are not known. The aim of the study was to evaluate the risk of AS in children after LTx treated with SRL. In 17 children with median age 13.2 yr (1.9-17.9) who received SRL on average for 4.1 yr (s.d. ± 2.9) we measured and compared with age-matched healthy control group (n = 45) lipid parameters and markers of AS: ADMA, oxyLDL, GSH, GPx, TC, TG, HDL cholesterol, LDL cholesterol, VLDL cholesterol, ApoAI, ApoB, ApoE, lipoprotein (a) (Lp(a)). We found no major differences in cholesterol, cholesterol in lipoprotein fractions and TG concentrations between patients receiving SRL and the control group. ApoE was markedly increased in the study group (19.1 g/L [±1.8]) when compared to controls (9.8 [±3.9]). ApoA1 was decreased in the study group: 1.30 g/L (±0.2) vs. 1.45 (±0.25), p = 0.04. ApoB and Lpa concentrations were similar in both groups. There were differences in oxidative stress markers: GSH 743 (±66.2) mol/mL vs. 780 (±48.2), p = 0.02 and GPx 32.8 (±5.5) U/gHb vs. 34.3(±2.6), p = 0.01. Markers of AS: ADMA did not differ between groups and oxidized LDLc was significantly lower in SRL group: 190 mU/mL (±113) vs. 237 (±107) in control, p < 0.05. SRL does not significantly disturb lipid metabolism and oxidative status in children after LTx.

Successful sirolimus rescue in tacrolimus-induced thrombotic microangiopathy after living-related liver transplantation.

TMA is a rare complication of tacrolimus. Disruption of endothelial cells, platelet aggregation, and intravascular mechanical fragmentation of red cells are core mechanisms of injury; however, exact pathways of toxicity are not clear. The clinical presentation may vary but TMA is a potentially life-threatening condition usually demanding aggressive treatment. We present the case of TMA in a child after living-related liver transplantation (LRLTx) on tacrolimus-based immunosuppressive regiment successfully converted to sirolimus.

Bone metabolism in cholestatic children before and after living-related liver transplantation--a long-term prospective study.

Bone disorders are common in children with end-stage liver diseases, especially those associated with cholestasis. Abnormal hepatocyte function, disordered vitamin D metabolism and calcium-phosphorous homeostasis, malnutrition, and immunosuppressive treatment are potential risk factors of bone tissue pathology before and after transplantation. The aim of the study was to analyze the long-term effect of successful living-related liver transplantation (LRLTx) on skeletal status and bone metabolism in cholestatic children. Eighteen cholestatic children (1.4±0.5yr old; 12 females [F]/6 males [M]) qualified for LRLTx were analyzed; 16 (5F/11M) of them participated in long-term observation (V4). Serum levels of osteocalcin (OC), procollagen type 1 N-terminal propeptide (P1NP), cross-linked telopeptide of type 1 collagen (CTx), insulin-like growth factor I (IGF-I), IGF-I binding protein 3 (IGFBP-3), parathyroid hormone (PTH), 25-hydroxyvitamin D (25(OH)D), and 1,25-dihydroxyvitamin D (1,25(OH)(2)D) were assayed before (V0) and 6mo (V1), 12mo (V2), 18mo (V3), and 4.4yr (V4) after LRLTx. Total body bone mineral content (TBBMC) and total body bone mineral density (TBBMD) were measured by dual-energy X-ray absorptiometry (DXA) at the same pattern. Before LRLTx, the OC, P1NP, CTx, IGF-I, and IGFBP-3 levels as well as TBBMC and TBBMD were decreased compared with age-matched control group. The mean serum levels of 25(OH)D and 1,25(OH)(2)D were within reference ranges from V0 to V4. After LRLTx, the OC, P1NP, CTx, IGF-I, and IGFBP-3 as well as TBBMC and TBBMD reached the age-matched reference values. At V4, the level of P1NP decreased below and the PTH increased above the reference range that coincided with reduced Z-scores of both TBBMC (-1.11±1.24) and TBBMD (-1.00±1.19). P1NP and CTx, both measured at V3, correlated with IGF-I at V2 (R=0.86, p=0.014 and R=0.78, p=0.021, respectively) and PTH at V3 for P1NP and V1 for CTx (R=0.64, p=0.048 and R=0.54, p=0.038, respectively). The TBBMC changes between V0 and V4 correlated with IGF-I (R=0.68, p=0.015) and 1,25(OH)(2)D (R=0.54, p=0.025), both assayed at V1. The change of TBBMC Z-scores between V0 and V4 correlated with P1NP at V1 (R=0.69, p=0.002). The TBBMD changes between V0 and V4 correlated with CTx at V1 (R=0.54, p=0.027) and P1NP change between V0 and V1 (R=0.51, p=0.038). In short-term observation, successful LRLTx led to bone metabolism normalization triggered by probable anabolic action of IGF-I and PTH and manifested by TBBMC and TBBMD increases. In long-term horizon, moderately impaired DXA assessed bone status coincided with disturbances in bone metabolism. Bone metabolism markers, especially P1NP and CTx, appeared to be good predictors of changes in bone status evaluated by DXA.

The Course of Ulcerative Colitis After Pediatric Liver Transplantation for Sclerosing Cholangitis.

BACKGROUND: Primary sclerosing cholangitis (PSC) and autoimmune sclerosing cholangitis (ASC) are often associated with ulcerative colitis (UC). The impact on the course of UC remains unclear, and up-to-date evidence in pediatric populations is scarce. The aim of the study was to analyze the course of UC in pediatric patients transplanted owing to PSC or ASC. MATERIAL AND METHODS: We retrospectively reviewed data from children with PSC/ASC and UC who underwent orthotopic liver transplantation (OLT). In all patients UC diagnosis was based on clinical presentation, endoscopy, and histology. RESULTS: Seventeen patients (9 female) with PSC or ASC underwent OLT from deceased donors at a median age of 16.8 years (range = 11.5-18.2 years). In 15 patients, UC was diagnosed before OLT (median age of diagnosis = 10.6 years; range = 6.6-18.0 years), and 2 patients developed UC after OLT. Ten patients (59%) presented with pancolitis on initial endoscopy. Disease activity was severe in 9 patients (53%) and most patients improved after initial treatment with steroids. Before OLT only 2 patients (13%) had severe disease activity. After OLT, 4 patients developed flares of the disease. These patients were successfully treated and remained in remission at the end of the posttransplant follow-up period (median = 3.76 years; range = 0.4-15.5 years). None of the patients developed colorectal cancer or underwent colectomy during 3.7 years of post-OLT follow-up. CONCLUSION: In our experience, the course of UC was not aggravated by OLT for PSC, and UC did not adversely affect patient or graft survival.

Occurrence of Portal Hypertension and Its Clinical Course in Patients With Molecularly Confirmed Autosomal Recessive Polycystic Kidney Disease (ARPKD).

Purpose: Liver involvement in autosomal recessive polycystic kidney disease (ARPKD) leads to the development of portal hypertension and its complications. The aim of this study was to analyze the occurrence of the portal hypertension and its clinical course and the dynamics in patients with molecularly confirmed ARPKD in a large Polish center. Moreover, the available options in diagnostics, prevention and management of portal hypertension in ARPKD will be discussed. Materials and Methods: The study group consisted of 17 patients aged 2.5-42 years. All patients had ARPKD diagnosis confirmed by molecular tests. Retrospective analysis included laboratory tests, ultrasound and endoscopic examinations, transient elastography and clinical evaluation. Results: Any symptom of portal hypertension was established in 71% of patients. Hypersplenism, splenomegaly, decreased portal flow and esophageal varices were found in 47, 59, 56, and 92% of patients, respectively. Gastrointestinal bleeding occurred in four of 17 patients. Endoscopic variceal ligation (EVL) was performed at least once in nine patients with esophageal varices. Conclusions: Portal hypertension and its complications are present in a significant percentage of ARPKD patients. They should be under the care of multidisciplinary nephrology-gastroenterology/hepatology team. Complications of portal hypertension may occur early in life. Endoscopic methods of preventing gastroesophageal bleeding, such as endoscopic variceal ligation, are effective and surgical techniques, including liver transplantation, are required rarely.

Percutaneous Treatment of Biliary Strictures After Pediatric Liver Transplantation.

BACKGROUND Biliary strictures (BS) are frequent after pediatric liver transplantation (LTx) and in spite of ongoing progress, they remain a significant cause of morbidity. In children, the majority of reconstruction is hepatico-jejunal anastomosis (HJA). The aim of this study was to analyze our experience in percutaneous transhepatic treatment of BS. MATERIAL AND METHODS Between 1998 and 2014, 589 (269 living donor) pediatric LTx were performed in our institution. We retrospectively reviewed clinical data of patients with HJA who developed BS and who underwent percutaneous transhepatic biliary drainage (PTBD). RESULTS Out of 400 patients with HJA, 35 patients developed BS. There were 27 cases (77%) of anastomotic BS (ABS) and 8 cases (23%) of multilevel BS (MBS). Ninety-two PTBD sessions (2.5 per patient) were performed, with successful outcomes in 20 cases (57%). Fifteen patients, after failed PTBD, underwent surgery which was successful in 11 cases. Overall good outcomes were achieved in 31 cases (88.5%). The most common complication of PTBD was cholangitis which occurred in 5.4% of the cases. We did not find any risk factors for PTBD failure, except for treatment occurring before 2007. CONCLUSIONS Percutaneous treatment is effective and safe in BS and is recommended as a first-line approach. The majority of patients in our study required multiple interventions, however, the overall risk of complications was low. Surgery is essential in selected cases and always should be considered if PTBD fails.

Bone mineral metabolism in children with biliary atresia after living related liver transplantation. Evaluation of selected parameters.

BACKGROUND: Aim of the study was to analyze the effect of living related liver transplantation on selected parameters of bone formation and resorption in children with liver cirrhosis caused by biliary atresia. MATERIAL/METHODS: 20 children (13F/7M) with biliary atresia aged from 6 month to 2.4 years were enrolled into the study 4-9 days before liver transplantation. Osteocalcin, procollagen 1 aminoterminal propeptide, collagen type 1 crosslinked C-telopeptide, parathyroid hormone and metabolites of vitamin D: 25(OH)D3, 1,25(OH)2D3 were measured before, 3, 6 and 12 months after liver transplantation. RESULTS: Three months after living related liver transplantation statistically significant increase of osteocalcin, collagen type 1 crosslinked C-telopeptide, parathyroid hormone and 1,25(OH)2D3 levels were found. We didn't observe further increase of these parameters during the next 9 months after liver transplantation. There was no difference in 25(OH)D3 levels in patients before and after liver transplantation. CONCLUSIONS: In children after successful living related liver transplantation we observed improvement of selected parameters of bone formation and resorption which indicate stimulation of growing processes and mechanisms of bone geometry modelling.

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.

[Preliminary results and complications of HCV treatment after liver transplantation]. / Wstepne wyniki i ocena powiklan leczenia zakazenia wirusem zapalenia watroby typu C (HCV) u pacjentów po transplantacji watroby.

UNLABELLED: The aim of study was to report the preliminary results and complications of HCV infection treatment after liver transplantation. MATERIAL: Six patients after liver transplantation (one after combine liver and kidney) had been qualified to treatment with pegylated interferon and ribavirin. RESULTS: In four patients the therapy was discontinued due to severe side effects (anaemia, cholestasis, sepsis, acute rejection). In two patients the normalization of biochemical parameters of liver function was achived after treatment. CONCLUSION: HCV treatement in solid organ recipients should be individualised.

[Amanita phalloides poisoning as an indication for liver transplantation in three family members]. / Zatrucie muchomorem sromotnikowym jako wskazanie do transplantacji watroby u trzech czlonków rodziny.

Amanita phalloides poisoning is one of the most dramatic medical invents. The course of the illness may vary from mild to the lethal, with signs of fulminant liver insufficiency with coma and multiorgan failure. When hepatic encephalopathy (III/IV degrees) occurs the prognosis is very poor. In definite cases the liver transplantation is necessary. The authors present severe Amanita phalloides poisoning in three family members, who due to fulminant hepatic failure underwent liver transplantation. The two of them (son and father) transplanted accordingly in fifth and seventh day after poisoning, survived. Mother, in whom transplantation started in ninth day after poisoning, died intraoperativel with signs of massive hemorrhage, and cardiac arrest.

Longitudinal study of renal function in pediatric liver transplant recipients.

OBJECTIVES: Study was performed to evaluate prospectively long-term renal function in pediatric liver transplant recipients. METHODS: In 50 children aged 1-18 years, renal function tests (sCr, GFR, ERPF, Cr clearance, renal Doppler ultrasound, ABPM) have been performed before and several times during 36 months follow-up after liver transplantation. RESULTS: Signifficant reduction of renal filtration function was found in studied children (increase of sCr, decrease of GFR, ERPF, Cr clearance) within 12 months after transplantation, which did not progress further in most children after this time. None of patients progressed to stage 4 or 5 renal failure according to CKD. Schwartz formula was not found reliable in assessing renal function in children after liver transplantation. There was no difference in renal function according to cyclosporine or tacrolimus treatment, however arterial hypertension was more common in cyclosporine treated group. CONCLUSIONS: Although renal damage may be a problem for an individual chuild after liver transplantation, majority of children have stable renal function 36 months after Tx. DTPA GFR should be performed yearly in all children until adulthood not to overlook progression of renal injury.

[Complications in a patient with liver cirrhosis and HCV coinfection during treatment with peginterferon and ribavirin]. / Powiklania leczenia interferonem pegylowanym i rybawiryna u pacjentki z marskoscia zólciowa watroby i nadkazeniem HCV.

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.

Nasogastric tube as protection for recurrent oesophageal stricture: a case report.

This report presents the case of an 8.5-year-old boy with Down syndrome after experiencing extensive caustic injury to the oesophagus and stomach resulting from the accidental ingestion of concentrated sulphuric acid. The patient had undergone 32 unsuccessful endoscopic oesophageal stricture dilatations and stenting procedures performed over a period of 15 mo following the accident. Surgical reconstruction of the oesophagus was not possible due to previous gastric and cardiac surgeries for congenital conditions. Before referring the patient for salivary fistula surgery, the patient received a nasogastric tube with perforations located above the upper margin of the oesophageal stenosis for the passage of saliva and fluid. The tube was well tolerated and improved swallowing; however the backflow of gastric contents caused recurrent infections of the respiratory tract. To overcome these problems, we developed a double lumen, varying diameter, perforated tube for protection of the oesophageal closure. This nasogastric tube was found to be safe and decreased the need for hospitalization and further endoscopic procedures. This newly developed tube can thus be considered as a treatment option for patients with recurrent oesophageal stenosis and contraindications for surgical oesophageal reconstruction.

The first case of Enterocytozoon bieneusi infection in Poland.

Microsporidia are intracellular parasites that cause opportunistic infections in humans of various immunological status. Only a few case reports exist on microsporidial infection in solid organ transplant recipients worldwide. The presented study demonstrates the first case in Poland of Enterocytozoon bieneusi infection in a liver transplant patient. Parasites were diagnosed in stool samples using both modified trichrome staining and PCR.