O transplante hepatico (TH) pediatrico e atualmente tratamento de rotina para criancas sofrendo de insuficiencia hepatica terminal em paises desenvolvidos e subdesenvolvidos. As indicacoes para transplante incluem insuficiencia hepatica agudae cronica, doencahepaticametabolica e hereditaria e tumores hepaticos nao ressecaveis. Em termosmundiais, a principal indicacao ocorre em criancas com atresia biliar submetidas a portoenterostomia de Kasai sem sucesso, alcancando um indice de mais de 50% de criancas transplantadas abaixo dos 2 anos de idade na Europa e nos EUA. Insuficiencia hepatica fulminante secundaria a hepatite viral ou toxica e a principal indicacao de insuficiencia hepatica aguda.

http://www.scielo.br/pdf/jped/v84n5/en_v84n5a01.pdf

Liver transplantation in children

This article aims to review recent advances in the diagnosis and treatment of pulmonary arterial hypertension in neonates and children with congenital heart disease. Articles on pulmonary arterial hypertension in congenital heart disease were retrieved from PubMed and MEDLINE published after 1958. A diagnosis of primary (or idiopathic) pulmonary arterial hypertension is made when no known risk factor is identified. Pulmonary arterial hypertension associated with congenital heart disease constitutes a heterogenous group of conditions and has been characterized by congenital systemic-to-pulmonary shunts. Despite the similarities in histologic appearance of pulmonary vascular disease, there are differences between pulmonary arterial hypertension secondary to congenital systemic-to-pulmonary shunts and those with other conditions with respect to pathophysiology, therapeutic strategies, and prognosis. Revision and subclassification within the category of secondary pulmonary arterial hypertension based on pathophysiology were conducted to improve specific treatments. The timing of surgical repair is crucial to prevent and minimize risk of postoperative pulmonary arterial hypertension. Drug therapies including prostacyclin, endothelin-receptor antagonist, phosphodiesterase inhibitor, and nitric oxide have been evolved with promising results in neonates and children. Among the different forms of congenital heart diseases, an early correction generally prevents subsequent development of pulmonary arterial hypertension. Emerging therapies for treatment of patients with idiopathic pulmonary arterial hypertension also improve quality of life and survival in neonates and children with congenital heart disease associated with pulmonary arterial hypertension. Heart and lung transplantation or lung transplantation in combination with repair of the underlying cardiac defect is a therapeutic option in a minority of patients. Partial repair options are also beneficial in some selected cases. Randomized controlled trials are needed to evaluate the safety and efficacy of these therapies including survival and long-term outcome.

Advances in diagnosis and treatment of pulmonary arterial hypertension in neonates and children with congenital heart disease

Objective: In this Consensus Statement, we review the etiology and pathophysiology of inflammatory processes seen in critically ill children with cardiac disease. Immunomodulatory therapies aimed at improving outcomes in patients with myocarditis, heart failure, and transplantation are extensively reviewed. Data Sources: The author team experience and along with an extensive review of the medical literature were used as data sources. Data Synthesis: The authors synthesized the data in the literature to present current immumodulatory therapies. For each drug, the physiologic rationale, mechanism of action, and pharmacokinetics are synthesized, and the evidence in the literature to support the therapy is discussed. Conclusions: Immunomodulation has a crucial role in the treatment of certain pediatric cardiac diseases. Immunomodulatory treatments that have been used to treat myocarditis include corticosteroids, IV immunoglobulin, cyclosporine, and azathioprine. Contemporary outcomes of pediatric transplant recipients have improved over the past few decades, partly related to improvements in immunomodulatory therapy to prevent rejection of the donor heart. Immunosuppression therapy is commonly divided into induction, maintenance, and acute rejection therapy. Common induction medications include antithymocyte globulin, muromonab-CD3, and basiliximab. Maintenance therapy includes chronic medications that are used daily to prevent rejection episodes. Examples of maintenance medications are corticosteroids, cyclosporine, tacrolimus, sirolimus, everolimus, azathioprine, and mycophenolate mofetil. Rejection of the donor heart is diagnosed either by clinically or by biopsy and is treated with intensification of immunosuppression.

Pediatric Cardiac Intensive Care Society 2014 Consensus Statement: Pharmacotherapies in Cardiac Critical Care Immune Therapy.

Each year in the United Kingdom approximately 240 children benefit from solid organ transplantation.1 Eleven paediatric centres variously perform kidney, heart, lung, liver and small bowel transplant operations. With current survival data approaching 90% or better at one year for most paediatric transplants, it is probable that at some point, these children will present to their local hospital or general practitioner (GP) for management of non-transplant issues or for shared care. Long term health and survival is dependent on careful adjustment of life long immunosuppressant medication. All of these children will be discharged from hospital on a combination of immunosuppression medications. The aim of immunosuppression is to prevent acute rejection, using a combination of drugs, while avoiding infection and toxicity. The combination of drugs (type and number) will depend on the organ transplanted, the time post-transplant, the patient’s rejection history, and side effects. There is no “one size fits all” immunosuppression protocol. Drugs and doses are adjusted according to clinical need. Children are seen regularly by the transplant centre during the first three months post-transplant, during which time most will be stabilised on their medication. This article focuses on maintenance immunosuppression that paediatricians, GPs, nurses, and pharmacists may encounter while caring for children in hospital or in the community.

In adult transplantation, there are many more randomised controlled trials (RCTs) published in the field of renal transplantation than there are in the other fields of transplantation. This is probably because enough kidney transplants are performed to recruit a sufficient number of patients to the trials. In heart transplantation, there are only four randomised controlled studies despite the first operations being done back in 1967. Drug immunosuppression protocols have evolved based on results seen in renal transplantation, and empirical observation. Generally, immunosuppression protocols include a calcineurin inhibitor, an anti-metabolite, and …

https://ep.bmj.com/content/edpract/91/3/ep87.full.pdf

Paediatric immunosuppression following solid organ transplantation

Pulmonary artery hypertension (PAH) is a rare progressive disease of the pulmonary vasculature. Although it affects fewer than two adults per million population, and probably fewer children, the clinical course can be one of rapid deterioration, with death occurring within three years of diagnosis for adults, and less than one year for children.1,2 In the last decade, greater understanding of the disease has led to rethinking the diagnostic classification of pulmonary hypertension, and the identification of therapeutic targets for drug development. A number of new drugs have been studied in randomised controlled trials, and are licensed to treat PAH. A key message from the recent World Symposium on Pulmonary Arterial Hypertension was to refer patients to a centre with expertise in managing PAH; however, paediatricians may encounter patients already commenced on these novel drugs and delivery systems.3 This review will summarise the key trials of the new drugs recently marketed for PAH, some of which are not yet available and none of which are licensed for children in the UK.

Until recently, primary pulmonary hypertension (PPH) was the term used to describe familial disease, or sporadic disease of unknown cause. At the World Pulmonary Arterial Hypertension Symposium in 2003, the term “primary pulmonary hypertension” was officially abandoned and replaced with idiopathic pulmonary artery hypertension (IPAH). This term more accurately indicates that the diagnosis is by exclusion, and exact causes are not known. Where genetic information supports a hereditary basis, the term familial pulmonary artery hypertension (FPAH) is to be used. In this classification, pulmonary artery hypertension (PAH) is differentiated from pulmonary hypertension caused by left heart disease; or chronic lung disease or hypoxaemia; or chronic thrombotic or embolic disorders affecting the lungs; or other infectious or inflammatory triggers (table 1). This classification system is useful in that …

https://ep.bmj.com/content/edpract/90/1/ep15.full.pdf

Pulmonary artery hypertension: new drug treatment in children

Pulmonary artery hypertension : New drug treatment in children K Ford

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Paediatric immunosuppression following solid organ transplantation

Pulmonary artery hypertension: new drug treatment in children

Pulmonary artery hypertension : New drug treatment in children K Ford