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  • Grünert SC
  • Derks TGJ
  • Mundy H
  • Dalton RN
  • Donadieu J
  • et al.
Mol Genet Metab. 2024 Mar;141(3):108144 doi: 10.1016/j.ymgme.2024.108144.

Glycogen storage disease type Ib (GSD Ib, biallelic variants in SLC37A4) is a rare disorder of glycogen metabolism complicated by neutropenia/neutrophil dysfunction. Since 2019, the SGLT2-inhibitor empagliflozin has provided a mechanism-based treatment option for the symptoms caused by neutropenia/neutrophil dysfunction (e.g. mucosal lesions, inflammatory bowel disease). Because of the rarity of GSD Ib, the published evidence on safety and efficacy of empagliflozin is still limited and does not allow to develop evidence-based guidelines. Here, an international group of experts provides 14 best practice consensus treatment recommendations based on expert practice and review of the published evidence. We recommend to start empagliflozin in all GSD Ib individuals with clinical or laboratory signs related to neutropenia/neutrophil dysfunction with a dose of 0.3-0.4 mg/kg/d given as a single dose in the morning. Treatment can be started in an outpatient setting. The dose should be adapted to the weight and in case of inadequate clinical treatment response or side effects. We strongly recommend to pause empagliflozin immediately in case of threatening dehydration and before planned longer surgeries. Discontinuation of G-CSF therapy should be attempted in all individuals. If available, 1,5-AG should be monitored. Individuals who have previously not tolerated starches should be encouraged to make a new attempt to introduce starch in their diet after initiation of empagliflozin treatment. We advise to monitor certain safety and efficacy parameters and recommend continuous, alternatively frequent glucose measurements during the introduction of empagliflozin. We provide specific recommendations for special circumstances like pregnancy and liver transplantation.

  • Drefs M
  • Schoenberg MB
  • Börner N
  • Koliogiannis D
  • Koch DT
  • et al.
Eur J Surg Oncol. 2024 Mar;50(3):107952 doi: 10.1016/j.ejso.2024.107952.
BACKGROUND:

Hepatocellular Carcinoma (HCC) still is one of the most detrimental malignant diseases in the world. As two curative surgical therapies exist, the discussion whether to opt for liver resection (LR) or transplantation (LT) is ongoing, especially as novel techniques to improve outcome have emerged for both. The aim of the study was to investigate how the utilization and outcome of the respective modalities changed through time.

METHODS:

We searched Medline and PubMed for relevant publications comparing LT and LR in HCC patients during the time period from 1990 to 2022, prior to March 31, 2023. A total of 63 studies involving 19,804 patients - of whom 8178 patients received a liver graft and 11,626 underwent partial hepatectomy - were included in this meta-analysis.

RESULTS:

LT is associated with significantly better 5-year overall survival (OS) (64.83%) and recurrence-free survival (RFS) (70.20%) than LR (OS: 50.83%, OR: 1.79, p < 0.001; RFS: 34.46%, OR: 5.32, p < 0.001). However, these differences are not as evident in short-term intervals. Older cohorts showed comparable disparities between the outcome of the respective modalities, as did newer cohorts after 2005. This might be due to the similar improvement in survival rates that were observed for both, LT (15-23%) and LR (12-20%) during the last 30 years.

CONCLUSION:

LT still outperforms LR in the therapy of HCC in terms of long-term survival rates. Yet, LR outcome has remarkably improved which is of major importance in reference to the well-known limitations that occur in LT.

  • Aldrian D
  • Bochdansky C
  • Kavallar AM
  • Mayerhofer C
  • Deeb A
  • et al.
Liver Int. 2024 Mar;44(3):811-822 doi: 10.1111/liv.15834.
BACKGROUND AND AIMS:

To systematically review the literature for reports on Wolcott-Rallison syndrome, focusing on the spectrum and natural history, genotype-phenotype correlations, patient and native liver survival, and long-term outcomes.

METHODS:

PubMed, Livio, Google Scholar, Scopus and Web of Science databases were searched. Data on genotype, phenotype, therapy, cause of death and follow-up were extracted. Survival and correlation analyses were performed.

RESULTS:

Sixty-two studies with 159 patients met the inclusion criteria and additional 30 WRS individuals were collected by personal contact. The median age of presentation was 2.5 months (IQR 2) and of death was 36 months (IQR 50.75). The most frequent clinical feature was neonatal diabetes in all patients, followed by liver impairment in 73%, impaired growth in 72%, skeletal abnormalities in 59.8%, the nervous system in 37.6%, the kidney in 35.4%, insufficient haematopoiesis in 34.4%, hypothyroidism in 14.8% and exocrine pancreas insufficiency in 10.6%. Episodes of acute liver failure were frequently reported. Liver transplantation was performed in six, combined liver-pancreas in one and combined liver-pancreas-kidney transplantation in two individuals. Patient survival was significantly better in the transplant cohort (p = .0057). One-, five- and ten-year patient survival rates were 89.4%, 65.5% and 53.1%, respectively. Liver failure was reported as the leading cause of death in 17.9% of cases. Overall survival was better in individuals with missense mutations (p = .013).

CONCLUSION:

Wolcott-Rallison syndrome has variable clinical courses. Overall survival is better in individuals with missense mutations. Liver- or multi-organ transplantation is a feasible treatment option to improve survival.

  • Raina R
  • Jothi S
  • Haffner D
  • Somers M
  • Filler G
  • et al.
Kidney Int. 2024 Mar;105(3):450-463 doi: 10.1016/j.kint.2023.10.017.

Focal segmental glomerular sclerosis (FSGS) is 1 of the primary causes of nephrotic syndrome in both pediatric and adult patients, which can lead to end-stage kidney disease. Recurrence of FSGS after kidney transplantation significantly increases allograft loss, leading to morbidity and mortality. Currently, there are no consensus guidelines for identifying those patients who are at risk for recurrence or for the management of recurrent FSGS. Our work group performed a literature search on PubMed/Medline, Embase, and Cochrane, and recommendations were proposed and graded for strength of evidence. Of the 614 initially identified studies, 221 were found suitable to formulate consensus guidelines for recurrent FSGS. These guidelines focus on the definition, epidemiology, risk factors, pathogenesis, and management of recurrent FSGS. We conclude that additional studies are required to strengthen the recommendations proposed in this review.

  • Peng S
  • Liang W
  • Liu Z
  • Ye S
  • Peng Z
  • et al.
Hum Cell. 2024 Mar;37(2):420-434 doi: 10.1007/s13577-023-01012-3.

Hypothermic machine perfusion (HMP) has been demonstrated to be more effective in mitigating ischemia-reperfusion injury (IRI) of donation after circulatory death (DCD) organs than cold storage (CS), yet the underlying mechanism remains obscure. We aimed to propose a novel therapeutic approach to ameliorate IRI in DCD liver transplantation. Twelve clinical liver samples were randomly assigned to HMP or CS treatment and subsequent transcriptomics analysis was performed. By combining in vivo HMP models, we discovered that HMP attenuated inflammation, oxidative stress, and apoptosis in DCD liver through a SEPRINA3-mediated PI3Kδ/AKT signaling cascade. Moreover, in the hypoxia/reoxygenation (H/R) model of BRL-3A, overexpression of SERPINA3 mitigated H/R-induced apoptosis, while SERPINA3 knockdown exacerbated cell injury. Idelalisib (IDE) treatment also reversed the protective effect of SERPINA3 overexpression. Overall, our research provided new insights into therapeutic strategies and identified potential novel molecular targets for therapeutic intervention against DCD liver.

  • Yasir MB
  • Man RK
  • Gogikar A
  • Nanda A
  • Niharika Janga LS
  • et al.
Ann Vasc Surg. 2024 Mar;100:67-80 doi: 10.1016/j.avsg.2023.10.010.
BACKGROUND:

Cardiovascular disease is the most common cause of death in renal transplant recipients (RTrs). High-output heart failure (HoHF) is a classic problem of RTrs with patent arteriovenous fistulae (AVF). Central to the entire discipline of transplant nephrology is the ligation of AVF in RTrs, with a patent AVF presenting with signs and symptoms of HoHF. AVF ligation has long been a topic of great interest in this population. To date, little attention has been paid to the effects of arteriovenous graft ligation on HoHF. This study systematically reviews the data for AVF ligation, aiming to provide its impact on HoHF in RTrs.

METHODS:

The present study adopts the Preferred Reporting Items for Systematic Reviews and Meta-analysis 2020 guidelines. Published studies were identified using a search strategy in PubMed, Scopus, PubMed Central, Science Direct, and Medline. The primary inclusion criterion for this review was RTrs with a patent AVF who exhibited clinical or imaging findings of HoHF. Articles dating back to the last decade that involved the human species were included in our review, and the search was restricted to the English language. Studies involving both male and female genders and those describing the adult population (aged > 19 years) were also a part of our inclusion criteria.

RESULTS:

After applying eligibility criteria, our electronic search yielded 1,461 articles. A total of 16 studies that involved 18,735 subjects were included in our review, which comprised 6 cohort studies, 4 case reports, 2 randomized control trials, 2 narrative reviews, 1 meta-analysis, and 1 case series. While the risk of bias of the narrative reviews was low, 1 of the randomized control trials had some overall concerns. The meta-analysis included in our review had moderate risk of bias, while 4 of the 6 cohort studies were of good quality. All of the case reports and series included in our review were of good quality. Of the 12 studies that reported genders, 10,949 were male and 6,416 were female. There was a notable reduction in left ventricular mass, left ventricular mass index, left ventricular end diastolic dimension, cardiac output, velocity index, and systemic vascular resistance index upon AVF ligation.

CONCLUSIONS:

A complete resolution of the clinical signs and symptoms of HoHF can be anticipated after AVF ligation in RTrs. Clinicians should always be on the lookout for signs and symptoms of cardiovascular decompensation in asymptomatic RTrs.

  • Kobashigawa J
  • VanWagner LB
  • Hall S
  • Emamaullee J
  • Entwistle JW
  • et al.
Am J Transplant. 2024 Mar;24(3):380-390 doi: 10.1016/j.ajt.2023.12.002.

Patients with severe heart disease may have coexisting liver disease from various causes. The incidence of combined heart-liver transplant (CHLT) is increasing as more patients with congenital heart disease survive to adulthood and develop advanced heart failure with associated liver disease from chronic right-sided heart or Fontan failure. However, the criteria for CHLT have not been established. To address this unmet need, a virtual consensus conference was organized on June 10, 2022, endorsed by the American Society of Transplantation. The conference represented a collaborative effort by experts in cardiothoracic and liver transplantation from across the United States to assess interdisciplinary criteria for liver transplantation in the CHLT candidate, surgical considerations of CHLT, current allocation system that generally results in the liver following the heart for CHLT, and optimal post-CHLT management. The conference served as a forum to unify criteria between the different specialties and to forge a pathway for patients who may need dual organ transplantation. Due to the continuing shortage of available donor organs, ethical issues related to multiorgan transplantation were also debated. The findings and consensus statements are presented.

  • Wu TC
  • Smith CP
  • Li JS
  • Burton J
  • Jackson NJ
  • et al.
J Surg Oncol. 2024 Mar;129(3):574-583 doi: 10.1002/jso.27511.
UNLABELLED:

BACKGROUND AND OBJECTIVES: Many heterogenous orthotopic liver transplant (OLT) protocols exist for patients with unresectable cholangiocarcinoma. Little is known about the incidence, predictors for, and the significance of achieving a pathologic complete response (pCR).

METHODS:

We performed a systematic review through September 2022 of the PubMed, Embase, and Web of Science databases. A random-effect meta-analysis was conducted to pool data across studies with reported pCR rates. Heterogeneity between treatment protocols was assessed via subgroup analysis. The pCR and 1-, 3-, and 5-year recurrence-free survival (RFS) and overall survival (OS) rates were extracted as outcomes of interest.

RESULTS:

A total of 15 studies reported pCR rates and were grouped by use of the Mayo protocol (4/15), stereotactic body radiation therapy (2/15), and an Other category (9/15). The pooled pCR rate among all studies was 32%. Both radiation technique and duration of CHT showed no significant association with pCR (p = 0.05 and 0.13, respectively). Pooled 1-year RFS and OS after any neoadjuvant therapy and OLT was 80% (95% confidence interval [CI], 0.61-0.91), and 91% (95% CI, 0.87-0.94), respectively. There was no 1-year OS difference detected among the three groups. pCR was not associated with OS in the meta-regression. Pooled 3- and 5-year OS among all studies was 72% and 61%, respectively.

CONCLUSIONS:

The pooled incidence of pCR was 32%. Differences in radiation technique did not appear to influence pCR rates and upon meta-regression, pCR was not a surrogate marker for survival.

  • Drachenberg CB
  • Buettner-Herold M
  • Aguiar PV
  • Horsfield C
  • Mikhailov AV
  • et al.
Am J Transplant. 2024 Mar;24(3):362-379 doi: 10.1016/j.ajt.2023.10.011.

The Banff pancreas working schema for diagnosis and grading of rejection is widely used for treatment guidance and risk stratification in centers that perform pancreas allograft biopsies. Since the last update, various studies have provided additional insight regarding the application of the schema and enhanced our understanding of additional clinicopathologic entities. This update aims to clarify terminology and lesion description for T cell-mediated and antibody-mediated allograft rejections, in both active and chronic forms. In addition, morphologic and immunohistochemical tools are described to help distinguish rejection from nonrejection pathologies. For the first time, a clinicopathologic approach to islet pathology in the early and late posttransplant periods is discussed. This update also includes a discussion and recommendations on the utilization of endoscopic duodenal donor cuff biopsies as surrogates for pancreas biopsies in various clinical settings. Finally, an analysis and recommendations on the use of donor-derived cell-free DNA for monitoring pancreas graft recipients are provided. This multidisciplinary effort assesses the current role of pancreas allograft biopsies and offers practical guidelines that can be helpful to pancreas transplant practitioners as well as experienced pathologists and pathologists in training.

  • Lu LY
  • Lee HM
  • Burke A
  • Li Bassi G
  • Torres A
  • et al.
Chest. 2024 Mar;165(3):540-558 doi: 10.1016/j.chest.2023.09.019.
BACKGROUND:

Influenza-associated pulmonary aspergillosis (IAPA) increasingly is being reported in critically ill patients. We conducted this systematic review and meta-analysis to examine the prevalence, risk factors, clinical features, and outcomes of IAPA.

STUDY QUESTION:

What are the prevalence, risk factors, clinical features, and outcomes of IAPA in critically ill patients?

STUDY DESIGN AND METHODS:

Studies reporting IAPA were searched in the following databases: PubMed MEDLINE, CINAHL, Cochrane Library, Embase, Scopus, Cochrane Trials, and ClinicalTrials.gov. We performed one-group meta-analysis on risk factors, clinical features, morbidity, and mortality using random effects models.

RESULTS:

We included 10 observational studies with 1,720 critically ill patients with influenza, resulting in an IAPA prevalence of 19.2% (331 of 1,720). Patients who had undergone organ transplantation (OR, 4.8; 95% CI, 1.7-13.8; I2 = 45%), harbored a hematogenous malignancy (OR, 2.5; 95% CI, 1.5-4.1; I2 = 0%), were immunocompromised (OR, 2.2; 95% CI, 1.6-3.1; I2 = 0%), and underwent prolonged corticosteroid use before admission (OR, 2.4; 95% CI, 1.4-4.3; I2 = 51%) were found to be at a higher risk of IAPA developing. Commonly reported clinical and imaging features were not particularly associated with IAPA. However, IAPA was associated with more severe disease progression, a higher complication rate, and longer ICU stays and required more organ supports. Overall, IAPA was associated with a significantly elevated ICU mortality rate (OR, 2.6; 95% CI, 1.8-3.8; I2 = 0%).

INTERPRETATION:

IAPA is a common complication of severe influenza and is associated with increased mortality. Early diagnosis of IAPA and initiation of antifungal treatment are essential, and future research should focus on developing a clinical algorithm.

TRIAL REGISTRY:

International Prospective Register of Systematic Reviews; No.: CRD42022284536; URL: https://www.crd.york.ac.uk/prospero/.