Solid organ transplantation is a widely recognized treatment for end-stage organ failure

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2 Basic information Solid organ transplantation is a widely recognized treatment for end-stage organ failure Children account for 5-10% of recipients of solid organ transplants

3 History Pediatric transplant history World 1954r- kidney transplant liver transplant bowel transplant a heart transplant Poland- childrens Memorial Health Institute 1984 kidney transplant liver transplant simultaneous liver and kidney transplants 2002r- multiple organ transplantation (liver, intestine, duodenum, pancreas )

4 The death of brain stem The criterion for brain death or brain death as a whole does not need to recognize the performance of other studies outside clinical trials with properly made statements and exclusions The diagnosis of brain death is based on the finding irreversible loss of its function Qualification procedure is in two phases: Stage I : suspicion of brain death Stage II : the performance of studies confirming brain stem death

5 Suspicion of brain death The death of the brain stem suspicion Declare that: Foreclosure : patient is in a coma patient is artificially ventilated diagnosed the cause of coma demonstrated primary or secondary structural brain damage structural damage to the brain is irreversible exhaustion of the possibilities of treatment and the passage of time patients poisoned and under the influence of certain pharmacological agents (drugs, antypsychotics, sedatives, anesthetic agents, striated muscle relaxants) able hypothermic patients ( 35 warmth surface ) patients with metabolic or endocrine disorders full-term newborns with less than 7 days of life

6 The death of brain stem No brain stem reflexes : pupil reaction to light corneal reflex spontaneous eye movements, eye movements when trying to caloric cold water motor response to pain stimulus in the range of innervation of cranial, peripheral, and no response in the face movement in response to pain stimuli in the spinal innervation retching and cough brain - ocular reflex persistence of apnea DOUBLE try again - IN ADULTS AFTER 3 HOURS - IN NEWBORNS AFTER 72 H - IN CHILDREN FOR 2 yo AFTER 24 H

7 The death of brain stem Additional tests for instrumental diagnostic difficulties ( extensive damage to the facial skeleton, infratentorial brain damage the current abnormal reflexes ) EEG cerebral circulation Rating angioct of cerebral arteries brain perfusion scintigraphy Transcranial Doppler Ultrasound Multimodal evoked potentials

8 The death of brain stem The panel, consisting of three doctors expert- at least one specialist in the field : Anaesthesiology and Intensive Care neurologist or neurosurgeon Upon determination of brain death = time of death is to cease further, intentional treatment = disconnection from the ventilator, the exception is to prepare the body for removal of organs for transplantation.

9 Kidney transplant I -st kidney transplant in a child - Vaysse performed in Paris in 1954 Poland 1984 Approx. 2-4 in age 0-19 yrs / 1 Mln develops chronic kindey disease The demand for organ transplants / year Kidney transplant

10 Kidney transplant Causes of CRF Birth defects ( hypoplasia, dysplasia and polycystic kidney disease, posterior urethral valves in boys ) gastroesophageal reflux nephropathy glomerulonephritis Haemolytic uraemic syndrome Cystinosis, and other oxalosis Renal replacement therapy ( CAPD, ADO, HD) - in children should be as short as possible ( complications, stunting physical ) Prior qualification : remove organic changes, eliminate potential sources of infection ; - Treatment of Functional disorders of the lower urinary tract - neurogenic bladder, diverticulosis of the bladder, the bladder reconstruction = formation of alternative ways of urine discharge - examination - Cystographic miction - if reflux = removal of the kidney with the ureter There is no age limit for the child, but a kidney transplant is performed usually in a child over 1 yo( = technical difficulties much larger donor kidney, the relative hypoperfusion transplant, high propensity for thrombotic complications and increased risk of rejection )

11 Kidney transplant Preparation for surgery Kidney obtained can be stored in a liquid preservative for hours at a temperature of 4C At this time, it takes the typical transplant recipient At the price of the selected recipient performs a series of tests to exclude infection and iliac vessels doppler ultrasound is performed

12 Kidney transplant Surgery Catheter into the bladder Arched incision over the bottom hip retroperitoneal access Launching artery and external iliac vein Unveiling of a fragment of the summit and the posterior lateral wall of the bladder On the table beside the organ it is prepared for implantation

13 Kidney transplant Fixation / anastomoses Venous renal vein- iliac vein external / common Arterial renal artery - iliac artery external / common or utensils pole Ureteral - blistering technique extravesical antyreflux ureteral implantation modo Lich- Gregoire In a child weighing less than kg, adult kidney from a donor is placed intraperitoneally ( Renal vascular anastomosis to the aorta and IVC )

14 Kidney transplant Management after transplantation Suitable blood flow through the organ catecholamines Diuresis stymulation- hydratation, furosemide, dopamine Immunosuppressive drugs (schemes based on calcineurin inhibitors : CsA, tacrolimus and steroids ) Prevention of bacterial and viral infections Clots prevention - low molecular weight heparin Measurements of the level of immunosuppressive drugs Doppler ultrasound organ Any biopsy to assess the severity of rejection

15 Kidney transplant complications : early thrombosis of the transplant vesells Acute hydronephrosis, urine outflow impairment, urinary fistula Acute renal tubular necrosis Acute rejection The recurrence of the primary disease late Chronic rejection, hypertension, CMV viral infection, recurrent UTI

16 Kidney transplant Results: 1-year survival of 90 % transplants 5-year survival of transplant % Patient survival after transplantation 1 - year 100 %, 5-year survival of the patient 95 %

17 Liver transplant I -st liver transplant in the world - in 1963 (Starzl) In Poland from a cadaveric donor in Poland donor family - so far 71 transplants from a living donor, simultaneous liver and kidney transplantation ( first Polish combined liver and kidney transplant r., thereafter 14 such operations )

18 Liver transplant The causes of end-stage liver failure Cholestasis ( congenital extrahepatic biliary atresia % of children will have TX) % of recipients Cirrhosis after inflammation, infection- HBS, HCV, AIH, idiopathic ) Congenital metabolic diseases, such as: CF, Wilson's disease, hemochromatosis, congenital deficiency of alpha antitrypsin Primary hepatic tumors - HBL, HCC Acute liver failure- poisoning, inflammation, viral, AIH Other- congenital cysts, cirrhosis of the liver after long-term TPN

19 Liver transplant eligibility Criteria nutritional status physical development basic disease Limiting quality of life: itching, bleeding from esophageal varices, hospitalizations ) Child-Pugh classification - Turcotte'a ( ascites, encephalopathy, INR, bilirubin, albumin ) Other classifications: eg. PELD - evaluate the patient's risk of death within 3 Mnths, neoplasms- individual assessment, Acute Liver Failure- criteria of King's College Hospital

20 Primary liver tumors and transplantation Actually more frequent indication Mainly, patients with so-called unresectable, despite aggressive chtx - HBL, HCC- extensive tumor or tumor location CONDITION : no extrahepatic tumor foci at the time of the transplant, and the presence of meta-foci in the lungs, which disappear after chtx, is not a contraindication The best prognosis : patients with a single tumor < 5-6cm or up to 3 foci of tumor, of which none is bigger than 3cm

21 Liver transplant Preparation of recipient : Improvement of nutritional status Correction of coagulation disorders Prevention of complications of portal hypertension (TIPS, banding varicose veins, propranolol, treatment of ascites) Prevention of renal dysfunction Prevention of hepatic encephalopathy vaccination dental treatment Selective decontamination of the digestive tract

22 Liver transplant selection of donors Compatibility ABO group urgency priority : LFA, retransplantation because of graft necrosis, acute decompensation of chronic liver failure Preferred is a whole organ transplant, if necessary 1-2 organ recipients Techniques variants : reduction of graft segments (left side panel II + III segment or left lobe, II + III + IV) or an individual II. They are used when the weight exceeds 2x the weight of donor recipient Family Giver - Segment II and III; more often the right lobe harvested

23 Liver transplantation Phase I: hepatectomy in organ recipient Preparation of the operating field: abdomen, chest, groin - may be the option: femoral axillary shunting : veno - venous and VP VCI and VCS Skin incision: bilateral transverse upper abdominal under the ribs The release of liver ligaments, VCI dissected above and below the liver and hepatoduodenal ligament Cutting of the common biliary duct and hepatic artery closing Closing of the portal vein, IVC clamped above and below the liver. In the case of hemodynamically unstable patient veno- venous shunt is performed

24 Liver transplant Phase II Non hepatic phase anastomosis of IVC when transplanted organ is with the IVC, or hepatic veins when only segment is transplanted Hepatic artery anastomosis and the portal vein end to end Clamp removal from the VCI above- and below the liver and simultaneous release of the hepatic artery and the portal vein Biliary anastomosis end to end ( CSF donor recipient PAA ) or Roux Y Phase III post-reperfusion phase

25 Liver transplant postoperative complications vascular Thrombosis Acute hepatic artery approx. 5 % Late: hepatic artery result in strictures of bile ducts Late: portal vein result in portal hypertension Bile Complications most common - leaks, fistulas, stenosis, recurrent cholangitis Acute rejection approx. 50 % in 1st year. - Symptoms: fever, increase in transaminases, liver biopsy Immunosuppressive Drugs : TCL, CsA, steroids

26 Liver transplant results: 1 - year- experience % Retransplantation 5-10%

27 Intestine transplant World's first : r Poland : One time 2001r- liver, small intestine, duodenum and pancreas, which is also the first Polish bowel transplant -to currently the only such operation in Poland. children account for % intestinal transplant recipients indications: SBS Extensive innervation of bowel disorders Idiopathic intestinal pseudoobstruction syndrome Long-term TPN- Tx + colorectal liver

28 Problem: Huge risk of infectious complications A great number of lymphatic tissue transplanted donor with intestine- episodes, graft vs. host rejection and GvHD Often % - post transplant limphatic - growth because of strong immunosuppression results: -5 Year 60 % -80 % Of patients without TPN