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Biomedical subjects

A Kamiński

Publications and source records attributed to A Kamiński.

At least 19 recordsLinked to original sources

Vascular complications after pediatric liver transplantation from the living donors.

Early arterial or portal vein thrombosis is a complications that can lead to graft loss and patient death or need of immediate retransplantation. The aim of the study was to assess the incidence, causes, treatment, and outcome of vascular thrombosis after living related donor liver transplantation (LRdLTx). Between 1999 and 2004 71 LRdLTx were performed in children aged from 6 months to 10 years. Vascular thrombosis was found in 12 recipients. Hepatic artery thrombosis (HAT) occurred in 4 (5.6%), portal vein thrombosis (PVT) in 8 (11.2%) cases. HAT occurred 5 to 8 days, PVT 1 to 22 days after LTx. Diagnosis of vascular thrombosis was confirmed by routine Doppler ultrasound examination. Thrombectomy was successful in one patient with HAT and in three patients with PVT. Venous conduit was performed in one patient with PVT after second thrombosis. Two children developed biliary strictures as a late complication of HAT and required additional surgical interventions. Two children with PVT developed portal hypertension with esophageal bleeding, which required surgical intervention; one another underwent endoscopic variceal ligation for grade III varices. Follow-up ranged from 7 to 60 months. One patient died as a result of HAT after retransplantation due to multiple intrahepatic abscesses 2 months after first transplant. Any risk factors of vascular thrombosis that can be controlled should be avoided after transplantation. Routine posttransplant Doppler examination should be performed at least twice a day within 7 to 14 posttransplant days. Immediate thrombectomy should be always carried out to avoid late complications and even mortality.

Child↗

Harvesting liver fragments from living-related donors: a single-center experience.

The aim of the study was to estimate the risk of harvesting a liver fragment from a living-related adult donor. Liver fragments were harvested from 44 donors. Liver segments II and III were harvested from 36 donors. Liver segments II, III, IV were harvested from 6 donors, 2 donors gave segments V, VI, VII, and VIII. After preliminary donor selection volumetric assessment of liver segments by computed tomography and arteriography was performed to visualize the cenac trunk and superior mesenteric artery. None of the donors died. No complications were observed during the operation. Only one case, a bile collection, was observed after surgery. We treated this patient with a satisfactory result by sonography-guided drainage. We observed temporary elevation of bilirubin and transaminase levels and a decrease in prothrombin index value. Blood transfusion was not necessary during any of the procedures. Mean hospitalization time after the surgery was 9.4 days. Mean graft weight/recipient weight ratio was 2.54%. The risk of the harvesting liver fragment from a living-related adult donor seems to be minimal.

Body Weight↗

Surgical treatment of progressive familial intrahepatic cholestasis: comparison of partial external biliary diversion and ileal bypass.

AIMS: Progressive familial intrahepatic cholestasis (PFIC, Byler's disease) is an autosomal recessive disorder resulting in liver fibrosis/cirrhosis and liver insufficiency. Before the 1990s, liver transplantation was the only effective therapy for these children. During the last 12 years, two alternative methods of surgical treatment have been proposed: partial external biliary diversion (PEBD) and ileal bypass procedure (IB), which allow for effective elimination of bile acids accumulated in the body. In this study, we compare the efficacy of these surgical techniques for PFIC. METHODS: During the last 20 years, we have treated 52 children with PFIC. PEBD was done in 21 patients (since 1995), and IB in 5 patients (since 1998), transplantation was performed in 9 patients (since 1990). The efficacy of non-transplantation surgical treatment was assessed by patients' clinical outcome, liver biochemistry, and survival without transplantation during a follow-up period of 12 to 48 months. RESULTS: In 15 out of 21 patients clinical symptoms improved after PEBD and liver function tests normalised (blood bile acids), 1 patient had to be converted to IB due to too high output biliary fistula, 2 patients were transplanted and 3 are considered for transplantation. Out of the 5 children after IB, 4 improved clinically and biochemically, but, after 12 months, symptoms recurred in 3 patients, one patient was converted successfully to PEBD. No significant influence on growth was observed, irrespective of the type of treatment in this group of patients. CONCLUSIONS: PEBD is more effective than IB for the permanent improvement of symptoms of PFIC. Ileal bypass procedure, although initially effective, does not ensure good long-term results in more than 50 % of patients, probably due to intestinal re-absorption of bile acids increasing over time.

Adolescent↗

Hepatobiliary scanning in the diagnosis of biliary atresia.

Early diagnosis is vital in the neonatal cholestasis. The aim of this study was to assess the usefulness of hepatobiliary scanning in the diagnosis of biliary atresia. 33 hepatobiliary scannings performed in 30 children with cholestasis over the last two years were analysed. The mean age at the diagnosis was 6.6 weeks. The investigation was carried out with Multispect camera using intravenous infusion of 99mTc-MBrIDA. In 23 patients there was no passage of the radiolabelled substance into the intestinal tract. In 18 patients biliary atresia was diagnosed. One patient with a clinical suspicion of Alagille syndrome had two scannings performed at the interval of two weeks. In 1 child a common biliary tract cyst with total obstruction of extrahepatic biliary tree was diagnosed. In 18 children with biliary atresia the diagnosis was confirmed during the operation and Kasai procedure was performed. In 2 children the second scanning showed bile drainage. In 3 children intrahepatic cholestasis was diagnosed in addition to the bile passage failure. Hepatobiliary scanning in the diagnosis of neonatal cholestasis was characterised by high sensitivity (100%) but lower specificity (75%). In difficult cases the final diagnosis should be made on a basis of complex clinical, biochemical and radiological techniques and, if necessary, it should be verified by intraoperative cholangiography.

Biliary Atresia↗

Scintiscanning in diagnostics of hepatopulmonary syndrome in children.

BACKGROUND: Arterial blood oxygenation disturbances accompanying liver cirrhosis are referred to as hepatopulmonary syndrome (HPS). HPS develops due to the formation of intrapulmonary arteriovenous shunts and dilatation of the vascular bed. The aim of the study was to assess the incidence of arteriovenous shunts in children with hepatic cirrhosis and HPS suspected on the basis of clinical signs, qualified for liver transplantation. MATERIAL AND METHODS: The study was carried out in a group of 21 children aged from 1.2 to 17.7 years (mean age 8.4); 8 girls and 13 boys. The patients were diagnosed as follows: biliary cirrhosis due to extrahepatic bile duct impatency--12, post-inflammatory liver cirrhosis due to infection with HVB--2, autoimmune hepatitis--2, fibrosis of the liver--2, alpha 1-antitrypsin deficiency--1, progressive familial intrahepatic cholestasis--1, cystic fibrosis--1. The presence of arteriovenous anastomoses in the lungs was detected by scintiscanning utilizing microspheres (albumin macroaggregates). Under physiologic conditions, technetium-labeled microspheres injected i.v. are accumulated in pulmonary capillaries. Radioactivity detected in other organs (kidneys, brain) indicates the presence of pathologic shunts omitting the alveoli. A percentage ratio of radioactivity detected in the brain to that present in the lungs serves as the index of blood flow through the anastomosis (SI--shunt index). According to Grimon, the mean SI value for healthy children amounts to 0.43%, the values ranging from 1% to 2% are regarded as borderline ones, and those above 2% allow unequivocal diagnosis of HPS. RESULTS: The SI values obtained in the study ranged from 0.06 to 51%. In 7 patients SI exceeded 1%, reaching 1.23% in one, and over 3% in the remaining 6 patients. No correlations were found either between SI value and the etiology of cirrhosis, or between the index and clinical condition of the patient assessed according to Child-Pugh scale. CONCLUSIONS: 1. Scintiscanning revealed the presence of arteriovenous shunts in the lungs of 6/21 (28.6%) investigated patients. 2. No correlation was found between the severity of HPS and the etiology and stage of the underlying disease.

Adolescent↗

Treatment of progressive familial intrahepatic cholestasis: liver transplantation or partial external biliary diversion.

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 pruritus. 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.

Adolescent↗

Primary vascular thrombosis after renal transplantation in children.

The aim of the study was to assess the incidence, causes, diagnostic and treatment modalities, and outcome of vascular thrombosis after kidney transplantation in children. Between 1984 and 1995 we performed 176 kidney transplants in pediatric recipients aged 1 to 18 years. Vascular thrombosis followed 7 transplants, 4 were renal vein and 3 arterial thromboses. Venous thromboses occurred 2 to 12 days after transplantation. All of the patients with a renal vein thrombosis lost their grafts. Arterial thrombosis developed in 2 cases of double renal arteries which were separately anastomosed into the recipient vessels. One graft was lost, but the other was saved by thrombolytic therapy (streptokinase). One child experienced intrarenal segmental artery thrombosis during acute vascular rejection, which resolved following combined anti-rejection and thrombolytic (intra-arterial streptokinase) treatment with full recovery of graft function. In all, vascular thrombosis complicated 7 out of 176 transplants (4.0%), and was the cause of 5 graft losses (2.8%). The incidence of vascular thrombosis was not increased in grafts with vascular anomalies (3/34 v. 4/142; p>0.05, chi sq.). We conclude that acute tubular necrosis, rejection and unstable volemia may predispose to vascular thrombosis. In selected cases, early diagnosis of vascular thrombosis may enable graft salvage by surgical or thrombolytic treatment.

Adolescent↗

Diagnosis and monitoring of cytomegalovirus infection after liver transplantation in children.

Cytomegalovirus infection (CMV) complicated the posttransplant course in 9 of 24 children after liver transplantation. We found specific antibodies (IgG and IgM) to be of very low value in diagnosis and monitoring of CMV infection after liver transplantation. Detection of CMV-DNA by PCR method in the blood or urine was very useful for diagnosis, but less for monitoring of the course of disease and its treatment. Measurements of early immediate CMV antigen (IEA), in peripheral blood leucocytes allowed for very early diagnosis of CMV infection and correlated well with the course of disease and response to treatment of the patient.

Antibodies, Viral↗

[Effect of bisphosphonates (HEBP, CL2MBP) on the process of biological mineralization in fresh bone and bone tissue de novo as a result of experimental osteogenesis induction in guinea pigs].

It is known from the literature that bisphosphonates inhibit mineralization process in skeletal tissues. To study in vivo the effects of HEBP (etidronate) and Cl2MBP (clodronate) both on preexisting bone mineral as well as on the early stages of the mineralization process the experimental model of heterotopical bone induction was chosen. Urinary bladder mucosa was used as an inductor of osteogenesis. Using this model it is possible to compare the effects of bisphosphonates on preexisting mineral on ortothopic bones as well as on the early stages of mineral deposition in the newly formed bone induced in heterotopic sites. HEBP and Cl2MBP doesn't inhibit heterotopic bone induction, but HEBP deeply inhibits the mineralization process; the small amount of deposited mineral does not contain crystalline fraction.

Animals↗

[Recurrent acute chylous peritonitis].

A case of acute chylous diffuse peritonitis with recurrences was treated by peritoneal drainage. During the first hospital stay this drainage resulted in cure. After 17 months another recurrence treated in the same way led to death with increasing renal failure, diabetes, cardiorespiratory failure.

Acute Disease↗