Secondary hemophagocytic syndrome after renal transplantation: two case-reports / Síndrome hemofagocítica secundária após transplante renal: a propósito de dois casos

ABSTRACT Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH) is an infrequent and underdiagnosed condition caused by an overactive immune response, resulting in blood cells phagocytosis. After kidney transplantation (KTx), HLH is usually secondary (or reactive) to infectious and neoplastic processes and has a high mortality rate. No effective treatment is available for this condition. Usual procedures include detecting and treating the pathology triggering the immune system dysregulation, other than administration of intravenous human immunoglobulin (IVIG) and high doses of steroids, and plasmapheresis. The best protocol for maintenance immunosuppressive therapy is also unknown. This article presents two cases of post-KTx reactive HLH that underwent adjuvant IVIG treatment and obtained good clinical results. Despite the high morbidity and mortality associated with reactive HLH after KTx, the early and precise diagnosis and the administration of IVIG therapy along with the treatment of the triggering disease, was an effective strategy to control HLH.

RESUMO A síndrome hemofagocítica (SHF) ou linfo-histiocitose hemofagocítica é uma condição infrequente e subdiagnosticada que tem por base a ativação excessiva da resposta imune, resultando em fagocitose das células do sangue. Após o transplante renal (TxR), a SHF é habitualmente secundária (ou reativa) a processos infecciosos e neoplásicos, culminando em elevadas taxas de mortalidade. Não há evidências quanto ao tratamento ideal dessa condição. Além de investigação e tratamento da patologia desencadeante do processo de desregulação do sistema imune, há descrições do uso de imunoglobulina humana (IVIG), esteroides em altas doses e plasmaférese. Não há evidências quanto à melhor forma de delinear a imunossupressão de manutenção. Este artigo apresenta dois casos de SHF reativa pós-TxR que realizaram tratamento adjuvante com IVIG, obtendo bons resultados clínicos. Apesar da elevada morbimortalidade associada à SHF reativa após o TxR, o diagnóstico ágil e preciso, associado à instituição de terapia com IVIG adjuvante ao tratamento da doença desencadeante, foi uma estratégia eficaz em conter o processo.

Secondary hemophagocytic syndrome after renal transplantation: two case-reports.

Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH) is an infrequent and underdiagnosed condition caused by an overactive immune response, resulting in blood cells phagocytosis. After kidney transplantation (KTx), HLH is usually secondary (or reactive) to infectious and neoplastic processes and has a high mortality rate. No effective treatment is available for this condition. Usual procedures include detecting and treating the pathology triggering the immune system dysregulation, other than administration of intravenous human immunoglobulin (IVIG) and high doses of steroids, and plasmapheresis. The best protocol for maintenance immunosuppressive therapy is also unknown. This article presents two cases of post-KTx reactive HLH that underwent adjuvant IVIG treatment and obtained good clinical results. Despite the high morbidity and mortality associated with reactive HLH after KTx, the early and precise diagnosis and the administration of IVIG therapy along with the treatment of the triggering disease, was an effective strategy to control HLH.

The Effect of Gabapentin on Postoperative Pain of Orthopedic Surgery of Lower Limb by Sciatic and Femoral Blockage in Children: A Clinical Trial.

BACKGROUND: There are meta-analyzes in adults demonstrating the benefits of using gabapentin to improve postoperative pain in orthopedic surgeries. In pediatrics, it has never been studied. OBJECTIVES: The aim of this study was to evaluate the use of gabapentin 10 mg/kg, orally, in postoperative analgesia, hemodynamic stability and its pre/postoperative anxiolytic effect in children subjected to unilateral inferior limb surgery. METHODS: We performed a double-blinded, randomized study. 84 patients in Albert Sabin Children's Hospital were selected for elective surgery that were divided into 2 groups: gabapentin group, who received gabapentin 1 to 2 hours before the procedure and the control group. Both groups were submitted to the same general anesthesia protocol with 0.125% bupivacaine femoral and sciatic block. Patients received scheduled dipyrone and morphine was used as the rescue analgesic up to 2/2 h. Postoperative pain was assessed using a scale appropriate for age (CRIES, CHIPPS or Wong-Baker face scale). We registered hemodynamic parameters, analgesic consumption and pre/postoperative anxiolytics. RESULTS: A decrease in pain intensity in the 4th and 8th postoperative hours was observed in gabapentin group, both groups had the same opioid consumption. Children in the gabapentin group had an odds ratio of 25.6 for preoperative sedation and gabapentin promoted reduction of postoperative agitation. During orotracheal intubation the gabapentin group exhibited attenuation of the hemodynamic response. CONCLUSIONS: Gabapentin was superior to placebo in reducing postoperative pain. Children who received gabapentin were more sedated in the operating room, less agitated in the postoperative period and the autonomic response to intubation was reduced.

Gabapentin in Improvement of Procedural Sedation and Analgesia in Oncologic Pediatric Patients: A Clinical Trial.

BACKGROUND: Preoperative anxiety and distress can produce significant psychological impacts on children undergoing oncologic care or investigation. Adjuvant therapy is used for pain management in children; however, pre-analgesia options are restricted because they can cause undesirable outcomes. OBJECTIVES: Our study aimed to investigate the use of gabapentin in procedural sedation as adjuvant therapy in children undergoing oncologic treatment. METHODS: We performed a double-blinded, randomized, clinical trial at Albert Sabin Infant's Hospital in Fortaleza, Brazil. Children aged 1 - 6 years who had myelogram or lumbar puncture (associated or not with intrathecal chemotherapy) received placebo or gabapentin syrups (15 mg/kg and 30 mg/kg) one to two hours before the procedure. Preoperative anxiety was evaluated by the Yale preoperative anxiety scale modified (m-YPAS scale). The pediatric anesthesia emergence delirium (PAED) and children and infants postoperative pain scale (CHIPP) scales were used for emergence delirium and pain intensity measurement, respectively. RESULTS: We evaluated 135 patients. We observed that the gabapentin groups presented lower m-YPAS scores than the placebo group at separation and induction times. Postoperatively, the gabapentin groups had lower PAED and CHIPP scores than the placebo group; however, only had PAED scores clinical relevance. No significant differences were found between the gabapentin groups. Furthermore, children with less than three prior similar procedures were more likely to benefit from gabapentin. Postoperative vomiting was prevented by 30 mg/kg gabapentin. CONCLUSIONS: Although gabapentin has little preoperative effects, it ameliorates anxiety before induction, improves anesthetic induction, and reduces the occurrence of emergence delirium and postoperative vomiting up to eight hours after the procedure. Thus, we indicate gabapentin as adjuvant therapy for procedural sedation.