Overview of Current Hepatocellular Carcinoma Staging Systems: Is There an Optimal System?

Multiple hepatocellular carcinoma (HCC) staging systems have been proposed and used clinically over time. These may consider clinical, pathological, radiological, or treatment response factors, depending on the model. Given the heterogeneity of HCC treatment in its different stages and the validation of the systems in different populations, they are not universal. Likewise, the improvement in diagnostic tools, as well as novel therapeutic alternatives, have made these models more complex. Despite this, some have been modified over time in line with advances in the field, and although there is no universally accepted one, each has its usefulness, strengths, and weaknesses.

Liver transplantation in Latin America: reality and challenges.

Healthcare systems in Latin America are broadly heterogeneous, but all of them are burdened by a dramatic rise in liver disease. Some challenges that these countries face include an increase in patients requiring a transplant, insufficient rates of organ donation, delayed referral, and inequitable or suboptimal access to liver transplant programs and post-transplant care. This could be improved by expanding the donor pool through the implementation of education programs for citizens and referring physicians, as well as the inclusion of extended criteria donors, living donors and split liver transplantation. Addressing these shortcomings will require national shifts aimed at improving infrastructure, increasing awareness of organ donation, training medical personnel, and providing equitable access to care for all patients.

Liver transplantation for unresectable colorectal liver metastasis.

PURPOSE OF REVIEW: To summarize the current state of liver transplantation (LT) for unresectable colorectal liver metastases (uCRLM), and to address future directions. RECENT FINDINGS: The Norwegian secondary cancer (SECA) I and SECA II studies demonstrated that after LT the 5-year survival of a highly selected group of patients with uCRLM could be as high as 60% and 83%, respectively. After long-term follow-up, the 5- and 10-year survival was shown to be 43% and 26%, respectively. Furthermore, data has accumulated in other countries and a North American study reported a 1.5-year survival of 100%. In addition, steady growth has been demonstrated in the US, with 46 patients transplanted to date and 19 centers enrolling patients for this indication. Lastly, although recurrence is almost universal in patients with a high tumor burden, it has not been an accurate surrogate for survival, reflecting the relatively indolent nature of recurrence after LT. SUMMARY: Growing evidence has shown that excellent survival and even cure can be achieved in highly selected patients with uCRLM, with survival rates far superior than in patients treated with chemotherapy. The next step is to create national registries to standardize selection criteria and establish the optimal approach and best practices for incorporating LT for uCRLM into the treatment armamentarium.

Recomendaciones para el cuidado, prevención de infecciones y quimioprofilaxis en los errores innatos de la inmunidad / Recommendations for care, prevention of infections and chemoprophylaxis in inborn errors of immunity

Los errores innatos de la inmunidad (EII), antes llamados inmunodeficiencias primarias (IDP), son un grupo heterogéneo de trastornos genéticos con defectos en uno o más componentes del sistema inmune. Los pacientes afectados por EII presentan aumentada susceptibilidad a microorganismos únicos o múltiples que se manifestará con infecciones recurrentes de diferente tipo y gravedad dependiendo del tipo de la localización del defecto. La prevención de infecciones es uno de los pilares fundamentales en el abordaje integral de los pacientes con EII. En este trabajo se resumen las conclusiones consensuadas en el Grupo de Trabajo de Inmunología Pediátrica de la Sociedad Argentina de Pediatría, sobre la base de la revisión de la evidencia disponible, respecto a los principios esenciales para el cuidado, la prevención de infecciones y la quimioprofilaxis en los errores innatos de la inmunidad para la orientación del pediatra y especialista dedicados al seguimiento de estas enfermedades.

Inborn errors of immunity, previously named primary immunodeficiency are a heterogeneous group of genetic defects of different components of the immune system. Patients present high susceptibility to an only or several microorganisms, developing recurrent infections; the severity is related to the specific genetic type of immunity defect. The main strategy on the management of these illness is the prevention of infections. These consensus guidelines made by the Pediatric Immunology Work Group of Sociedad Argentina de Pediatría, givese main approaches of infection prevention in order to provide a useful tool for all practitioners who are involved in the management of these patients, based on scientific evidence and broad consensus of a specialized panel expert.

The High Incidence of Occult Carcinoma in Total Hepatectomy Specimens of Patients Treated for Unresectable Colorectal Liver Metastases With Liver Transplant.

OBJECTIVE: To describe the rate of occult carcinoma deposits in total hepatectomy specimens from patients treated with liver transplant (LT) for colorectal liver metastases (CRLM). BACKGROUND: Previous studies have shown that patients with CRLM treated with systemic therapy demonstrate a high rate of complete radiographic response or may have disappearing liver metastases. However, this does not necessarily translate into a complete pathologic response, and residual invasive cancer may be found in up to 80% of the disappearing tumors after resection. METHODS: Retrospective review of 14 patients who underwent LT for CRLM, at 2 centers. Radiographic and pathologic correlation of the number of tumors and their viability before and after LT was performed. RESULTS: The median (interquartile range) number of tumors at diagnosis was 11 (4-23). The median number of chemotherapy cycles was 24 (16-37). Hepatic artery infusion was used in 5 patients (35.7%); 6 (42.9%) underwent surgical resection, and 5 (35.7%) received locoregional therapy. The indication for LT was unresectability in 8 patients (57.1%) and liver failure secondary to oncologic treatment in the remaining 6 (42.9%). Before LT, 7 patients (50%) demonstrated fluorodeoxyglucose-avid tumors and 7 (50%) had a complete radiographic response. Histopathologically, 11 patients (78.6%) had a viable tumor. Nine (64.2%) of the 14 patients were found to have undiagnosed metastases on explant pathology, with at least 22 unaccounted viable tumors before LT. Furthermore, 4 (57.1%) of the 7 patients who demonstrated complete radiographic response harbored viable carcinoma on explant pathology. CONCLUSIONS: A complete radiographic response does not reliably predict a complete pathologic response. In patients with unresectable CRLM, total hepatectomy and LT represent a promising treatment options to prevent indolent disease progression from disappearing CRLM.

[Recommendations for care, prevention of infections and chemoprophylaxis in inborn errors of immunity]. / Recomendaciones para el cuidado, prevención de infecciones y quimioprofilaxis en los errores innatos de la inmunidad.

Inborn errors of immunity, previously named primary immunodeficiency are a heterogeneous group of genetic defects of different components of the immune system. Patients present high susceptibility to an only or several microorganisms, developing recurrent infections; the severity is related to the specific genetic type of immunity defect. The main strategy on the management of these illness is the prevention of infections. These consensus guidelines made by the Pediatric Immunology Work Group of Sociedad Argentina de Pediatría, givese main approaches of infection prevention in order to provide a useful tool for all practitioners who are involved in the management of these patients, based on scientific evidence and broad consensus of a specialized panel expert..

Los errores innatos de la inmunidad (EII), antes llamados inmunodeficiencias primarias (IDP), son un grupo heterogéneo de trastornos genéticos con defectos en uno o más componentes del sistema inmune. Los pacientes afectados por EII presentan aumentada susceptibilidad a microorganismos únicos o múltiples que se manifestará con infecciones recurrentes de diferente tipo y gravedad dependiendo del tipo de la localización del defecto. La prevención de infecciones es uno de los pilares fundamentales en el abordaje integral de los pacientes con EII. En este trabajo se resumen las conclusiones consensuadas en el Grupo de Trabajo de Inmunología Pediátrica de la Sociedad Argentina de Pediatría, sobre la base de la revisión de la evidencia disponible, respecto a los principios esenciales para el cuidado, la prevención de infecciones y la quimioprofilaxis en los errores innatos de la inmunidad para la orientación del pediatra y especialista dedicados al seguimiento de estas enfermedades.

Expanding spectrum, intrafamilial diversity, and therapeutic challenges from 15 patients with heterozygous CARD11-associated diseases: A single center experience.

CARD11-associated diseases are monogenic inborn errors of immunity involving immunodeficiency, predisposition to malignancy and immune dysregulation such as lymphoproliferation, inflammation, atopic and autoimmune manifestations. Defects in CARD11 can present as mutations that confer a complete or a partial loss of function (LOF) or contrarily, a gain of function (GOF) of the affected gene product. We report clinical characteristics, immunophenotypes and genotypes of 15 patients from our center presenting with CARD11-associated diseases. Index cases are pediatric patients followed in our immunology division who had access to next generation sequencing studies. Variant significance was defined by functional analysis in cultured cells transfected with a wild type and/or with mutated hCARD11 constructs. Cytoplasmic aggregation of CARD11 products was evaluated by immunofluorescence. Nine index patients with 9 unique heterozygous CARD11 variants were identified. At the time of the identification, 7 variants previously unreported required functional validation. Altogether, four variants showed a GOF effect as well a spontaneous aggregation in the cytoplasm, leading to B cell expansion with NF-κB and T cell anergy (BENTA) diagnosis. Additional four variants showing a LOF activity were considered as causative of CARD11-associated atopy with dominant interference of NF-kB signaling (CADINS). The remaining variant exhibited a neutral functional assay excluding its carrier from further analysis. Family segregation studies expanded to 15 individuals the number of patients presenting CARD11-associated disease. A thorough clinical, immunophenotypical, and therapeutic management evaluation was performed on these patients (5 BENTA and 10 CADINS). A remarkable variability of disease expression was clearly noted among BENTA as well as in CADINS patients, even within multiplex families. Identification of novel CARD11 variants required functional studies to validate their pathogenic activity. In our cohort BENTA phenotype exhibited a more severe and expanded clinical spectrum than previously reported, e.g., severe hematological and extra hematological autoimmunity and 3 fatal outcomes. The growing number of patients with dysmorphic facial features strengthen the inclusion of extra-immune characteristics as part of the CADINS spectrum. CARD11-associated diseases represent a challenging group of disorders from the diagnostic and therapeutic standpoint, especially BENTA cases that can undergo a more severe progression than previously described.

Humoral response to different SARS-CoV-2 vaccines in orthotopic liver transplant recipients.

BACKGROUND: The safety and efficacy data of the different types of available vaccines is still needed. The goal of the present analysis was to evaluate the humoral response to the COVID-19 vaccines in orthotopic liver transplant (OLT) recipients. METHODS: Participants were included from February to September 2021. No prioritized vaccination roll call applied for OLT patients. Controls were otherwise healthy people. Blood samples were drawn after 15 days of the complete vaccine doses. The samples were analyzed according to the manufacturer's instructions using the Liaison XL platform from DiaSorin (DiaSorin S.p.A., Italy), and SARS-COV-2 IgG II Quant (Abbott Diagnostics, IL, USA). RESULTS: A total of 187 participants (133 OLT, 54 controls, median age: 60 years, 58.8% women) were included for the analysis; 74.3% had at least one comorbidity. The serologic response in OLT patients was lower than in controls (median 549 AU/mL vs. 3450 AU/mL, respectively; p = 0.001). A positive humoral response was found in 133 OLT individuals: 89.2% with BNT162b2 (Pfizer-BioNTech), 60% ChAdOx1 nCOV-19 (Oxford-AstraZeneca), 76.9% with CoronaVac (Sinovac, Life Sciences, China), 55.6% Ad5-nCov (Cansino, Biologics), 68.2% Gam-COVID-Vac (Sputnik V) and 100% with mRNA-1273. In controls the serological response was 100%, except for Cansino (75%). In a multivariable model, personal history of COVID-19 and BNT162b2 inoculation were associated with the serologic response, while the use of prednisone (vs. other immunosuppressants) reduced this response. CONCLUSION: The serologic response to COVID-19 vaccines in OLT patients is lower than in healthy controls. The BNT162b2 vaccine was associated with a higher serologic response.

Low-dose aspirin confers protection against acute cellular allograft rejection after primary liver transplantation.

This study investigated the effect of low-dose aspirin in primary adult liver transplantation (LT) on acute cellular rejection (ACR) as well as arterial patency rates. The use of low-dose aspirin after LT is practiced by many transplant centers to minimize the risk of hepatic artery thrombosis (HAT), although solid recommendations do not exist. However, aspirin also possesses potent anti-inflammatory properties and might mitigate inflammatory processes after LT, such as rejection. Therefore, we hypothesized that the use of aspirin after LT has a protective effect against ACR. This is an international, multicenter cohort study of primary adult deceased donor LT. The study included 17 high-volume LT centers and covered the 3-year period from 2013 to 2015 to allow a minimum 5-year follow-up. In this cohort of 2365 patients, prophylactic antiplatelet therapy with low-dose aspirin was administered in 1436 recipients (61%). The 1-year rejection-free survival rate was 89% in the aspirin group versus 82% in the no-aspirin group (hazard ratio [HR], 0.77; 95% confidence interval [CI], 0.63-0.94; p = 0.01). The 1-year primary arterial patency rates were 99% in the aspirin group and 96% in the no-aspirin group with an HR of 0.23 (95% CI, 0.13-0.40; p < 0.001). Low-dose aspirin was associated with a lower risk of ACR and HAT after LT, especially in the first vulnerable year after transplantation. Therefore, low-dose aspirin use after primary LT should be evaluated to protect the liver graft from ACR and to maintain arterial patency.

Pulmonary Alveolar Proteinosis and Multiple Infectious Diseases in a Child with Autosomal Recessive Complete IRF8 Deficiency.

BACKGROUND: Autosomal recessive (AR) complete IRF8 deficiency is a rare severe inborn error of immunity underlying an absence of blood myeloid mononuclear cells, intracerebral calcifications, and multiple infections. Only three unrelated patients have been reported. MATERIALS AND METHODS: We studied an Argentinian child with multiple infectious diseases and severe pulmonary alveolar proteinosis (PAP). We performed whole-exome sequencing (WES) and characterized his condition by genetic, immunological, and clinical means. RESULTS: The patient was born and lived in Argentina. He had a history of viral pulmonary diseases, disseminated disease due to bacillus Calmette-Guérin (BCG), PAP, and cerebral calcifications. He died at the age of 10 months from refractory PAP. WES identified two compound heterozygous variants in IRF8: c.55del and p.R111*. In an overexpression system, the p.R111* cDNA was loss-of-expression, whereas the c.55del cDNA yielded a protein with a slightly lower molecular weight than the wild-type protein. The mutagenesis of methionine residues downstream from c.55del revealed a re-initiation of translation. However, both variants were loss-of-function in a luciferase assay, suggesting that the patient had AR complete IRF8 deficiency. The patient had no blood monocytes or dendritic cells, associated with neutrophilia, and normal counts of NK and other lymphoid cell subsets. CONCLUSION: We describe the fourth patient with AR complete IRF8 deficiency. This diagnosis should be considered in children with PAP, which is probably due to the defective development or function of alveolar macrophages.

Liver Transplantation as Definitive Treatment of Post-cholecystectomy Bile Duct Injury: Experience in a High-volume Repair Center.

OBJECTIVE: To analyze the perioperative and long-term outcomes of patients undergoing LT due to BDI in a tertiary care center. BACKGROUND: BDI is associated with significant morbidity and long-term impact on quality of life. LT represents the only possibility of a cure in patients with BDI who develop SBC. METHODS: Retrospective cohort study from a prospective LT database. Between 2008 and 2019, patients with SBC due to BDI after cholecystectomy and requiring LT were identified. Perioperative and long-term outcomes were analyzed. RESULTS: Among 354 LT, 12 patients underwent LT to treat post-cholecystectomy BDI and accounted for 3.4% of all LT. The median time from BDI to SBC diagnosis was 9.3 years (2.4-14). The mean time from SBC to inclusion on the waitlist was 2.4years (± 2.2). Postoperative complications occurred in 11 patients (91.6%); mainly infectious (9/12 patients, 75%), followed by renal complications (4/12 patients, 33.3%). Only 2 patients developed major complications, which were the patients who died, resulting in a 90-day mortality of 16.7%. After a mean follow-up of 40.3 months (± 42.2) survival at 1, 3, and 5 years was 83%. CONCLUSIONS: Although BDI is an unusual indication for LT worldwide, it accounted for 3.4% of all LT in our center. Although postoperative mortality remains high, LT is the only possibility of a cure, with acceptable long-term outcomes. Early referral to a tertiary care center is essential to avoid long-term complications of BDI, such as SBC.

Identification of Germline Non-coding Deletions in XIAP Gene Causing XIAP Deficiency Reveals a Key Promoter Sequence.

PURPOSE: X-linked inhibitor of apoptosis protein (XIAP) deficiency, also known as the X-linked lymphoproliferative syndrome of type 2 (XLP-2), is a rare immunodeficiency characterized by recurrent hemophagocytic lymphohistiocytosis, splenomegaly, and inflammatory bowel disease. Variants in XIAP including missense, non-sense, frameshift, and deletions of coding exons have been reported to cause XIAP deficiency. We studied three young boys with immunodeficiency displaying XLP-2-like clinical features. No genetic variation in the coding exons of XIAP was identified by whole-exome sequencing (WES), although the patients exhibited a complete loss of XIAP expression. METHODS: Targeted next-generation sequencing (NGS) of the entire locus of XIAP was performed on DNA samples from the three patients. Molecular investigations were assessed by gene reporter expression assays in HEK cells and CRISPR-Cas9 genome editing in primary T cells. RESULTS: NGS of XIAP identified three distinct non-coding deletions in the patients that were predicted to be driven by repetitive DNA sequences. These deletions share a common region of 839 bp that encompassed the first non-coding exon of XIAP and contained regulatory elements and marks specific of an active promoter. Moreover, we showed that among the 839 bp, the exon was transcriptionally active. Finally, deletion of the exon by CRISPR-Cas9 in primary cells reduced XIAP protein expression. CONCLUSIONS: These results identify a key promoter sequence contained in the first non-coding exon of XIAP. Importantly, this study highlights that sequencing of the non-coding exons that are not currently captured by WES should be considered in the genetic diagnosis when no variation is found in coding exons.

Subtotal Cholecystectomy After Failed Critical View of Safety Is an Effective and Safe Bail Out Strategy.

BACKGROUND: Bile duct injury (BDI) is accompanied by significant morbidity and long-term impact in quality of life. Subtotal cholecystectomy (STC) is an alternative to prevent this outcome but is associated with other complications. The aim of this work is to demonstrate that BDI associated morbidity exceeds STC associated morbidity, underscoring STC as a reasonable bail out strategy. METHODS: We compared 115 patients who underwent STC with 293 patients who were referred to our center with BDI type E1-E3 and underwent surgical repair. The groups were comparable because in both instances the surgeon had the opportunity to decide not to perform a total cholecystectomy once critical view of safety (CVS) was not achieved. RESULTS: Bile leakage was found in 21% of the STC group with only one BDI (0.9%). More Accordion ≥ 4 were found in the STC group (10.4% vs 4.8%, p = 0.035); however, reoperations were more frequent in the BDI group (8.2% vs 0.9%, p = 0.006). No patient in the STC group required reintervention for completion cholecystectomy. After 3.8 years follow-up, 2.4% of patients had secondary biliary cirrhosis in the BDI group; none in the STC group. CONCLUSIONS: Despite complications of STC, morbidity associated with BDI is much higher due to high long-term reoperation rate, in addition to secondary biliary cirrhosis. STC is a safe alternative that can prevent BDI if properly and timely performed in the context of difficult cholecystectomy.

Implementação de protocolo de cuidados para realização segura de raio-x no leito em pacientes críticos / Implementation of a care protocol for safe bedside x-rays in critically ill patients

A segurança do paciente no momento da realização do exame de raio-x no leito em pacientes críticos é fundamental, o que exige a implementação de um protocolo de cuidados para esta prática que contemple as ações que os profissionais envolvidos devem executar antes, durante e após o exame. Assim, o objetivo dessa pesquisa é implementar um protocolo de cuidados para realização segura de raio-x no leito em pacientes críticos na Unidade de Terapia Intensiva em um hospital público. Realizou-se uma Pesquisa Convergente Assistencial (PCA), desenvolvida em unidade de terapia intensiva e unidade de diagnóstico por imagem, de um Hospital de Ensino do Sul do Brasil, no período de julho de 2019 a julho de 2020, com enfermeiros, técnicos de enfermagem e técnicos de radiologia, envolvidos na realização do exame de raio-x no leito. Na fase de concepção da PCA, em decorrência da atuação profissional da pesquisadora em unidade de terapia intensiva, despertou-se interesse em implementar um protocolo já existente para realização desses exames. Na fase de instrumentação definiu-se o local e os participantes da pesquisa. A fase de perscrutação foi dividida em cinco etapas: na primeira, observou-se a realização do exame de raio-x no leito o que permitiu identificar fragilidades e potencialidades da prática; na segunda etapa, ocorreu a divulgação do protocolo a ser implementado por meio de flayers e cartões com o intuito de despertar curiosidade e interesse nos profissionais; na terceira etapa, os profissionais foram capacitados para implementação do protocolo, com utilização de vídeo didático que possibilitou um maior envolvimento das equipes; na quarta etapa, os participantes foram convidados a dialogar com a pesquisadora sobre a prática da realização do exame e na quinta etapa os profissionais responderam a um formulário de quais ações começaram a realizar após a implementação do protocolo. Na fase de análise os dados foram agrupados por similaridade e analisados com literatura correlata. Esta pesquisa foi aprovada em Comitê de Ética e Pesquisa com parecer número 3.182.887 e CAAE: 64812117.0.0000.0096. Após análise dos dados, foi possível apontar oito aspectos que foram base para discussão da implementação do protocolo, a citar: a importância de um protocolo para a realização segura de raio-x no leito; a capacitação ao implementar novos cuidados para garantir uma prática segura; a comunicação entre setores, profissionais e paciente no momento da realização do exame; o fechamento da sonda enteral antes do exame; a desinfecção das mãos e equipamentos; o papel da enfermagem durante o exame, exposição corporal desnecessária do paciente e cuidados com diferentes dispositivos. Concluiu-se que é importante padronizar as ações desenvolvidas pelos profissionais com a implementação de um protocolo, trazendo como recomendação a necessidade constante de atualização da equipe de saúde, olhar humanizado dos profissionais e a valorização do uso de protocolos pelas instituições.

The patient safety at the moment an x-ray examination in bed in critically ill patients is essential, it must indicate the most appropriate practice, contemplating the care actions that the professionals involved must perform before, during and after the examination, which requires the implementation of a care protocol for this practice. Therefore, the objective of this research is to implement a care protocol for safely performing x-ray in bed in critically ill patients in the Intensive Care Unit in a public hospital. A Convergent Care Research (PCA) was carried out, developed in an intensive care unit and diagnostic imaging unit, of a Teaching Hospital in the South of Brazil, from July 2019 to July 2020, with nurses, technicians from nursing and radiology technicians, involved in performing the bed x-ray examination. In the PCA design phase, as a result of the researcher's professional performance in an intensive care unit, interest was aroused in implementing an already existing protocol for carrying out these exams. In the instrumentation phase, the location and research participants were defined. The screening phase was divided into five stages: in the first, the x-ray examination in bed was observed, which allowed the identification of weaknesses and potentialities of the practice; in the second stage, there was the disclosure of the protocol to be implemented through flayers and cards in order to arouse curiosity and interest in the professionals; in the third stage, the professionals were trained to implement the protocol, using didactic video that enabled greater involvement of the teams; in the fourth stage, the participants were invited to talk with the researcher about the practice of taking the exam and in the fifth stage, the professionals responded to a form of what actions they started to perform after the protocol implementation. In the analysis phase, data were grouped by similarity and analyzed with related literature. This research was approved by the Ethics and Research Committee with opinion number 3,182,887 and CAAE: 64812117.0.0000.0096. After analyzing the data, it was possible to point out eight aspects that were the basis for discussing the implementation of the protocol, to mention: the importance of a protocol for the safe performance of xrays in bed; training in implementing new care to ensure safe practice; communication between sectors, professionals and the patient at the time of the exam; closing the enteral tube before the exam; disinfection of hands and equipment; the role of nursing during the examination, unnecessary body exposure of the patient and care for different devices. It was concluded that it is important to standardize the actions developed by the professionals with the implementation of a protocol, bringing as a recommendation the constant need for updating the health team, a humanized view of the professionals and the valorization of the use of protocols by institutions.

Use of Iliac Allograft from Cadaveric Donor as a Rescue Technique in Living Donor Kidney Transplant: Two Case Reports.

BACKGROUND: A short right renal vein (RRV) remains a challenge for renal transplant surgery, especially in the living donor. Different techniques exist to obtain an RRV with a suitable length in cadaveric donor; however, in living donors the options are limited. MATERIAL AND METHODS: We present 2 living kidney transplants in which we obtained a very short RRV, making the implantation very difficult. We describe our technique to overcome this problem by using cadaveric iliac vessels retrieved from previous cadaveric donations and preserved at 4°C in histidine-tryptophan-ketoglutarate (HTK) solution, without intraoperative or postoperative complications. We complied with the Helsinki Congress and the Istanbul Declaration regarding the donor source. RESULTS: In both cases, kidney grafts had optimal primary function, with good creatinine clearance after transplant and good patency of vascular anastomosis by Doppler ultrasounds. CONCLUSIONS: We believe the use of cadaveric vessel grafts in living donor kidney transplant is a valuable resource as a rescue tool in emergency situations like the ones being presented in this article in order to avoid discarding a kidney graft with damage or short vessels. This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Successful Multimodal Treatment of an IGF2-Producing Solitary Fibrous Tumor With Acromegaloid Changes and Hypoglycemia.

Doege-Potter syndrome with acromegaloid facial changes is extremely rare. Uncooked cornstarch along with glucocorticoids have been used as supportive care in patients with non-islet cell tumor hypoglycemia (NICTH). Preoperative embolization of hepatic solitary fibrous tumors (SFT) with NICTH has yielded unsatisfactory results. Herein we present the case of a 61-year-old man with a 3-month history of severe frequent hypoglycemic episodes and acromegaloid facial changes. During a spontaneous hypoglycemia (26 mg/dL), laboratory values showed a hypoinsulinemic pattern with low levels of GH, IGFPB3, and an IGF2/IGF1 ratio of 8.5:1. Cross-sectional imaging revealed a large (16 × 13 × 11 cm) hepatic tumor, and cytology was consistent with SFT. A preoperative right portal embolization was performed in an effort to induce normal remnant liver hypertrophy to allow for safe tumor resection. After the procedure, uncooked starch treatment followed by prednisone was started, achieving complete remission of hypoglycemic episodes in the preoperative setting. He subsequently underwent partial hepatectomy. The histologic diagnosis was compatible with a potentially malignant SFT. The patient had an excellent outcome with complete remission of hypoglycemia, improvement of facial acromegaloid changes, and no further evidence of disease. To our knowledge, this is the first case of a patient with Doege-Potter syndrome with acromegaloid facial changes induced by a potentially malignant liver SFT, treated successfully with a multimodal approach consisting of uncooked cornstarch, low-dose prednisone, preoperative embolization, and complete surgical resection. The use of cornstarch and low-dose glucocorticoids may be an adequate treatment in advance of undergoing surgery.

Recurrent eosinophilic cystitis in a child with chronic granulomatous disease.

Eosinophilic cystitis is an uncommon disease in children, and its association with chronic granulomatous disease (CGD) has been previously reported in only five patients. In all those patients the disease showed either a self-limited benign course or a rapid response to corticosteroid treatment. The authors describe a child with X-linked CGD who developed eosinophilic cystitis with a recurrent course and difficult therapeutic management. The authors also discuss the pathogenesis of granuloma formation in CGD and review the literature for current therapies for these complications.