[Show abstract][Hide abstract] ABSTRACT: Background:
The aim of our study was to retrospectively assess any correlation between graft fibrosis and selected immunological factors in pediatric liver transplant recipients.
Material and methods:
The study was performed on 33 patients after living related donor transplantation, divided into 2 groups depending on history of acute rejection episodes after transplantation. We assessed liver biopsies for presence of fibrosis, signs of antibody-mediated rejection, inflammatory infiltrations, and changes in bile ducts. We correlated these findings with assessment of anti-HLA antibodies.
Among 14 patients with ACR, a history fibrosis was found in 8 patients (57%). In 19 patients without a history of ACR, fibrosis was found in 9 patients (47%). Anti-HLA antibodies were found in 47% of patients with fibrosis and in only 18.75% of patients without fibrosis. Among 3 patients with signs of antibody-mediated rejection, all had fibrosis in the graft 2 years after transplantation. We did not find any patient with chronic rejection or ductopenia.
We suggest that there is a correlation between ACR and development of graft fibrosis present in liver grafts from recipients with normal liver biochemistry. Anti-HLA antibodies class II seems to be most important in development of fibrosis.
Annals of transplantation: quarterly of the Polish Transplantation Society 05/2015; 20:279-84. DOI:10.12659/AOT.892544 · 1.26 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Background:
Antibody-mediated rejection (AMR), associated with the presence of C4d deposits, is well-defined in kidney transplantation but much less documented in liver transplantation (LTx). The aim of our study was to retrospectively analyze a group of pediatric liver transplant recipients who experienced episodes of acute rejection in the past, for the signs of AMR and its impact on liver histology.
Our study population consisted of 18 patients after living related donor liver transplantation with a history of acute cellular rejection (1-5/patient). In all of them, actual liver function was good at almost 2-year median follow-up after transplantation. We reassessed all liver biopsies taken from these children between 5 days to 5.7 years after transplantation for signs of acute cellular rejection and antibody-mediated rejection. In all patients, anti-HLA antibodies were also assessed at least 2 years after transplantation (2.18-12.27 years, median 6.795 years).
There were 27 episodes of acute rejection proved by liver biopsy. Signs of AMR were found in 6 of 18 patients (33.3%). In 5 of these patients, donor-specific (DSA) and non-specific anti-HLA antibodies were also identified. In the group of 12 patients with acute rejection without histochemical signs of AMR, anti-HLA antibodies were found in sera of only 5 of 12 patients after transplantation.
Our study shows some correlation between C4d-positive reaction in liver biopsies with acute cellular rejection and presence of anti-HLA antibodies, particularly against HLA class II. We did not find any difference in the late graft function, which could be correlated with the presence of AMR. Further studies on larger groups of patients are necessary.
Annals of transplantation: quarterly of the Polish Transplantation Society 03/2014; 19:119-23. DOI:10.12659/AOT.889921 · 1.26 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Undifferentiated embryonal sarcoma of the liver (UESL) represents less than 5% of all malignant hepatic tumors in childhood. It is considered an aggressive neoplasm with an unfavorable prognosis. The aim of this paper is to present a single center experience in the treatment of children with UESL.
Ten children with UESL were treated between 1981 and 2012. Age at diagnosis ranged from 4months to 17years (median age, 6years and 9months). Surgery after neoadjuvant chemotherapy (CHT) was performed in 7 patients, and in 3 patients primary surgery was done. Adjuvant chemotherapy was administered in all 10 patients (CYVADIC, CAV, CAV/ETIF/IF+ADM, CDDP/PLADO). Right hemihepatectomy was performed in 1 patient, extended right hemihepatectomy in 6, and partial resection of the right lobe (segments V-VI, segment V) in 2 patients. One patient with unresectable tumor affecting both lobes was listed for liver transplantation (LTx).
Follow-up from diagnosis ranged from 50 to 222months (mean 138months). Among 9 patients treated with partial liver resection, distant metastases/local recurrence was not observed in any, and disease-free survival in this group is 100% (9 patients alive). The patient that underwent liver transplantation died of multiorgan failure 4months postoperatively. However, this patient was misdiagnosed as having hepatoblastoma (HBL) and received PLADO chemotherapy. The overall survival rate is 90%.
Excellent results with long-term survival can be achieved in children with UESL with conventional therapy, including a combination of neoadjuvant and adjuvant chemotherapy and surgery, even in large extensively growing tumors.
Journal of Pediatric Surgery 11/2013; 48(11):2202-6. DOI:10.1016/j.jpedsurg.2013.05.020 · 1.39 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Despite prospective crossmatching and modern immunosuppression, early acute rejection is still present in cadaveric renal transplantation. The purpose of this study was to evaluate the incidence of preformed anti-donor antibodies, detected by 2 solid-phase techniques, and to analyze their impact on early renal allograft outcome.
Flow crossmatch detecting the presence of anti-donor IgG and IgM antibodies was performed in pre-transplant sera of 279 patients with negative cytotoxic crossmatch. Screening for IgG antibodies detected by bead-based multiplex technique was performed in sera of 69 patients from the FCXM group. The incidence of early biopsy-proven rejection and graft failure within 3 months after transplantation was analyzed.
Anti-donor IgG antibodies were detected in 33 patients (11.8%) by flow crossmatch and in 10 patients by multiplex (14.5%). IgM antibodies were detected in 23 patients (8.2%). All multiplex-positive sera were also positive for IgG by flow crossmatch, but in 18 cases no antibodies were found by multiplex technique. Biopsy-proven acute rejection within 3 months after transplantation was observed in 16 patients, and 5 allografts were lost due to immunological reasons. Presence of IgG antibodies was found to have no effect on early outcome, while the presence of IgM antibodies was associated with significantly higher rejection rate and immune-related graft failure.
Anti-donor IgG antibodies detected by bead-based and cell-based technique have no impact on biopsy-proven rejection rate or graft failure. Anti-donor IgM detected by flow crossmatch have significant impact on early transplantation outcome.
Annals of transplantation: quarterly of the Polish Transplantation Society 12/2011; 16(4):32-9. DOI:10.12659/AOT.882216 · 1.26 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The most common causes of clinical symptoms of bowel obstruction in newborn are the various dysmotylity disorders. The aim of the study was a retrospective analysis of the newborn's case history with the diagnosis of ganglion cells immaturity, their clinical course, diagnostic imagine and treatment in our own material. Material and methods. In the years 1981-2007 we treated in the Department of Pediatric Surgery in Children's Memorial Health Institute 420 children with various dysmotylity disorders so-called dysganglionosis. Among them were 15 infants, who finally were diagnosed ganglion cells immaturity. In all clinical symptoms associated with impaired motoric function of large bowel occurred in the first week of life. We performed a retrospective analysis of the case history of these children's including: demographic data, perinatal anamnesis, the dominant clinical symptoms, how to conduct an emergency and a final treatment, and long term follow-up. Results. The children were born at 28-40 week of pregnancy (mean 35,9 weeks) with the body mass between 0.95 and 4.15 kg (mean 2,72 kg), 7 of them were premature infant in 28-36 week of pregnancy and body mass 950-2900 g (mean 1970 g). All infants after birth were evaluated by the Apgar's scale of the 1-10 point (mean 8.5). The first meconium in the first 24 hours of life passed only 1 infant, the remaining 12 meconium passed delayed from 2 to 4 day of life. In 5 children were present concomitant diseases and malformations making it difficult to establish early correct diagnosis: congenital gastroschisis (1 child), intestinal volvulus (1 child) and necrotizing enterocolitis (NEC) (3 children). Diagnosis of ganglion cells immaturity was found at age from 1 to 365 day on the basis of the clinical course, radiological imagine (11 children) and histopathological test of rectal biopsy specimens (9 children) or surgical biopsy specimens (6 children). Four children were treated medically; the others require the temporary emergence colostomy or ileostomy. Functional maturity of ganglion cells identified between 2 and 16.5 month on the basis of electromanometric study (12 children), and/or rectal biopsy specimens (7 children) and in 3 only on clinical course. GI tract reconstruction was performed in the other 11 children at aged from 4.2 to 20.3 month of life, mean 10.6 month. After GI tract reconstruction in 1 child it was mechanical adhesive ileus and malabsorption of unknown origin requiring partial parenteral nutrition. In another child remained ileus and chronic cholestasis, this patient died after next laparotomy because of bleeding and failure liver function. Other patients live in a good general condition with a normal motoric function of the digestive tract from long term follow-up 1.8 to 17.8 years (mean 6.7 years). Conclusions. Early differential diagnosis of dysganglionosis is difficult, but possible subject to the full panel of diagnostic (radiological imagine, electromanometric and histopathological study) already in newborn with impaired bowel motoric function. Only in such cases with correct diagnosis patient can be adequately treated and to guarantee good initial and definitive treatment.
Polish Journal of Surgery 03/2009; 81(2):95-102. DOI:10.2478/v10035-009-0013-1
[Show abstract][Hide abstract] ABSTRACT: There is a group of children with primary hepatic tumors which can not be resected by conventional partial liver resection. Total hepatectomy followed by liver transplantation may be the only solution in such cases. Authors reviewed own experience with the liver transplantation for unresectable tumors in children and assessed the possible indications and role of transplantation in these patients.
Liver transplantation was performed in 17 children with unresectable hepatic tumors out of total number of 350 children transplanted. Hepatocarcinoma was present in 8 children, hepatoblastoma in 6 and benign giant hemangioma in 3. There was no other option for the treatment which would lead to the oncological cure of children with malignant tumors. All patients with giant hemangiomas were infants transplanted urgently due to circulatory and then multiorgan failure.
Survival within whole group is 75.5% (13 of 17 pts), 3 children died of malignant tumor recurrence, one of other causes. All 3 children with benign tumors are alive and well. Actual follow-up is from 3 months to 7 years.
Liver transplantation should be considered as option in the treatment of all children with unresectable hepatic tumors. With the careful and individual patient selection significant chances for survival can be achieved in this group of patients which would otherwise not survive with the conventional treatment.
Annals of transplantation: quarterly of the Polish Transplantation Society 06/2008; 13(2):37-41. · 1.26 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Anti-CMV prophylaxis is currently a routine management in patients after organ transplantation. One of the clinical symptoms of CMV infection may be lesions in the oral cavity.The aim of this study was to determine the relationship between CMV infection, occurrence of Candida species and presence of oral mucosal ulceration in transplant recipients.
Twenty fi ve patients after kidney or liver transplantation (mean age 13 years +/-4 years), receiving immunosuppression (tacrolimus, cyclosporine or sirolimus), who presented minor and major recurrent aphtous stomatitis (RAS), atypical ulcerations or focal necrosis in the oral cavity were enrolled into the study. Mean duration of post-transplant follow-up was 4 years and 3 months (+/-3 years and 5 months). Clinical dental examination (Pl I, GI), mycological tests and blood tests for CMV infection (specific IgM antibodies and/or pp65 antygenemia) were performed in all patients. Eighteen specimens of oral mucosa were evaluated for CMV presence in situ.
CMV infection was confirmed in 13 patients with oral mucosal ulceration (46.43%); which were accompanied by gingivitis (average GI=1.34); in two cases Candida albicans was identified. DNA of HCMV was found in-situ in 5.5% of all biopsies, and in 9% of biopsies of patients with clinical CMV infection; changes did not show the presence of Candida spp.
There is a significant correlation between CMV infection and oral lesions; in some cases, CMV may be a direct cause.
Annals of transplantation: quarterly of the Polish Transplantation Society 02/2008; 13(4):28-33. · 1.26 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Intestinal transplantation is becoming more and more successful due to improved surgical techniques, immunosuppression medication and postoperative intensive care. It can be performed as an isolated transplantation or as part of a multivisceral graft including the liver in cases of associated liver cirrhosis requiring total parenteral nutrition (TPN). The authors describe the first intestinal and multivisceral (liver, small bowel, duodenum and pancreas) transplantation in Poland performed in an 11-month old infant with short bowel syndrome and TPN related liver failure. A composite graft was harvested from a deceased 4-month old infant. All transplanted organs showed good function; however, the recipient died of infectious complications one month after transplantation.
Polish Journal of Surgery 02/2007; 79(2):120-125. DOI:10.2478/v10035-007-0019-5
[Show abstract][Hide abstract] ABSTRACT: Thyroglossal duct remnants (TGDR), most often cysts, are the most common type of developmental abnormalities of the thyroid gland. In about 1 to 2% of TGDR neoplastic transformation occurs. Papillary carcinoma of the thyroid may be encountered in over 90% of such cases. Two cases of primary papillary carcinoma of the thyroid in TGDR in young girls are presented. The diagnostic and therapeutic problems are shared, and up-to-date management guidelines in similar cases are discussed.
Endokrynologia Polska 01/2007; 58(2):164-7. · 0.99 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Renal cancer accounts for about 2% of all cancers in general population. One of the risk factors is acquired cystic kidney disease developed in course of end- stage renal failure. About 2% of cases of renal cancer are associated with inherited syndromes. We present here the case of 18-year old boy with papillary bilateral renal cell carcinoma, which was found 3 years after renal transplantation, in the stage of graft failure, few days after graphtectomy, while looking for the reason of persistent fever.
[Show abstract][Hide abstract] ABSTRACT: The aim of study was to report the preliminary results and complications of HCV infection treatment after liver transplantation.
Six patients after liver transplantation (one after combine liver and kidney) had been qualified to treatment with pegylated interferon and ribavirin.
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.
HCV treatement in solid organ recipients should be individualised.
[Show abstract][Hide abstract] ABSTRACT: We encountered on seven malignant hepatocellular tumors developing in older children and adolescents.
These tumors exhibit an unusual phenotype with respect to clinical presentation, histopathology, immunohistochemistry, and treatment response. As a working hypothesis, we suggest that these apparently novel, unusual, and aggressive tumors occurring in older children and adolescents may form a transition in the putative developmental pathway of hepatocarcinogenesis.
We therefore propose the term, transitional liver cell tumors (TLCT), to denote these lesions.
Medical and Pediatric Oncology 12/2002; 39(5):510-8. DOI:10.1002/mpo.10177
[Show abstract][Hide abstract] ABSTRACT: Hepatic vascular tumors (HVT) are the most common benign liver tumors present in infancy and childhood commonly associated with high output cardiac failure. Pediatric HVT usually are divided into infantile hemangioendothelioma (IHE), cavernous hemangioma (HC), and arteriovenous malformations (AVM). The aim of this study was to analyze clinical and histologic features of pediatric HVT in relation to treatment strategies.
During last 12 years, 17 children have been treated because of HVT. The diagnosis of HVT was established in all on the basis of clinical and imaging data. A retrospective analysis of clinical records and histopathology divided 17 into: Group 1, neonates; and Group 2, infants and older children.
Radiologic imaging revealed the vascular nature of the tumors in all patients. All nine from Group 1 were symptomatic from AVMs and seven were operated upon. Only one of eight children from Group 2 presented symptoms of AVM requiring surgery; four of five in this group had surgery because of the risk of malignancy. Within Group 1, a mixture of proliferating IHE with microscopic features of AVM was found in most. In three neonates with HVT immunologic and clinical features of cytomegalovirus (CMV) hepatitis were noted. In two Group 2 patients, pure HC was present and in another, the diagnosis of angiosarcoma was established after biopsy of a peritoneal metastasis.
HVT in children demonstrate internal morphologic heterogeneity and an age-related behavior of the disease. We also confirm the proliferative nature of all hemangioendotheliomas (HEs) in children. Further studies on the tumorigenesis of these lesions are needed.
Medical and Pediatric Oncology 12/2002; 39(5):524-9. DOI:10.1002/mpo.10179
[Show abstract][Hide abstract] ABSTRACT: Progressive intrahepatic familial cholestasis (PFIC), previously called Byler’s disease, is a syndrome in which children develop severe cholestasis progressing to biliary cirrhosis and chronic liver failure, usually during the first decade of life. Clinical features include jaundice, hepatomegaly, splenomegaly, growth retardation and severe pruritis. Laboratory tests demonstrate elevated bilirubin and bile acids, without an increase in serum gamma-glutamyl-transpeptidase or cholesterol. This study was performed to evaluate our experience with medical therapy as well as two types of surgical treatment used in children with PFIC, particularly partial external biliary diversion (PEBD) as an alternative method of therapy to liver transplantation (OLTx). Between 1979 and 1998 we have treated 46 children with PFIC (27 boys and 19 girls), aged 10 months to 19 yr (at the time of this study). Medical treatment with ursodeoxycholic (UDCA) was used in 39 patients for the period between 6 and 82 months. PEBD (cholecysto-jejuno-cutaneostomy) was performed in 16 patients, OLTx in eight children (including one after unsuccessful PEBD). Retrospective analysis of the clinical course and selected laboratory tests (bilirubin, ASPAT, ALAT, bile acids), and histopathological examinations were performed. Results of treatment were assessed by means of influence of the type of treatment on clinical symptoms, laboratory tests, progress of liver cirrhosis and hepatic failure, as well as physical development and survival. Medical therapy was effective in the long term in four (10%) of the patients resulting in clinical and biochemical normalization. Both surgical methods of therapy of PFIC, PEBD and OLTx, resulted in an 80% success rate and therefore should be used as complementary therapies. In patients before established liver cirrhosis, PEBD should be the first choice of treatment. Patients presenting with cirrhosis or after ineffective PEBD should qualify for OLTx. With this strategy most children with PIFC can be cured.