Defective kinase activity of IKKα leads to combined immunodeficiency and disruption of immune tolerance in humans.

IKKα is a multifunctional serine/threonine kinase that controls various biological processes, either dependent on or independent of its kinase activity. However, the importance of the kinase function of IKKα in human physiology remains unknown since no biallelic variants disrupting its kinase activity have been reported. In this study, we present a homozygous germline missense variant in the kinase domain of IKKα, which is present in three children from two Turkish families.

Arterial anastomosis in LDLT: techniques and risks.

Reconstructing the hepatic artery in adult living donor liver transplantation is technically challenging, with complications leading to significant morbidity and mortality. Early arterial thrombosis can result in a mortality rate up to 50%, often necessitating re-transplantation. The most common techniques for arterial anastomosis include end-to-end reconstruction with interrupted or continuous sutures, either using magnifying loupes or a microscope.

Analysis of factors influencing target PASI responses and side effects of methotrexate monotherapy in plaque psoriasis: a multicenter study of 1521 patients.

Methotrexate (MTX) is commonly used as first-line systemic treatment agent in psoriasis. We aimed to evaluate the clinical characteristics and treatment responses of patients with psoriasis undergoing MTX monotherapy. Data from adult patients with plaque psoriasis who received MTX monotherapy for at least 3 months between April 2012 and April 2022 were retrospectively evaluated in 19 tertiary care centers.

Assessing hepatic steatosis by magnetic resonance in potential living liver donors.

Purpose: To determine the accuracy of magnetic resonance imaging-proton density fat fraction (MRI-PDFF) measurements for detecting liver fat content in potential living liver donors and to compare these results using liver biopsy findings.

Methods: A total of 139 living liver donors (men/women: 83/56) who underwent MRI between January 2017 and September 2021 were included in this analysis retrospectively. The PDFFs were measured using both MR spectroscopy (MRS) and chemical shift-based MRI (CS-MRI) for each donor in a blinded manner.

Indications to LDLT: a changing pathway?

Liver transplantation (LT) revolutionized the outlook for cirrhotic patients, offering a potential cure with over 80% life expectancy after 5 years. Cirrhosis, with or without hepatocellular carcinoma (HCC), is the primary LT indication. Living donor LT (LDLT) initially explored as an alternative, declined due to poorer outcomes.

Biliary complications after adult-to-adult living-donor liver transplantation: An international multicenter study of 3633 cases.

In living-donor liver transplantation, biliary complications including bile leaks and biliary anastomotic strictures remain significant challenges, with incidences varying across different centers. This multicentric retrospective study (2016-2020) included 3633 adult patients from 18 centers and aimed to identify risk factors for these biliary complications and their impact on patient survival. Incidences of bile leaks and biliary strictures were 11.

Risankizumab for the Treatment of the Patients with Moderate to Severe Plaque Psoriasis During a 24-Week Period: Real-Life Experience.

Introduction: Several authors have reported their experiences in real-world clinical practice, confirming the therapeutic efficacy of risankizumab in plaque psoriasis. We aimed to reflect our experience with risankizumab treatment in patients with psoriasis.

Materials And Methods: Patients who presented to the dermatology outpatient clinics of two tertiary care centers between November 2021 and August 2022, diagnosed with psoriasis and treated with risankizumab, constituted the target population.

Burning questions: Current practices and critical gaps in evaluating removal of per- and polyfluoroalkyl substances (PFAS) during pyrolysis treatments of biosolids.

Concerns surrounding potential health and environmental impacts of per- and polyfluoroalkyl substances (PFAS) are growing at tremendous rates because adverse health impacts are expected with trace-level exposures. Extreme measures are required to mitigate potential PFAS contamination and minimize exposures. Extensive PFAS use results in the release of diverse PFAS species from domestic, industrial, and municipal effluents to wastewater, which partition to biosolids throughout secondary treatment.

Management of Established Small-for-size Syndrome in Post Living Donor Liver Transplantation: Medical, Radiological, and Surgical Interventions: Guidelines From the ILTS-iLDLT-LTSI Consensus Conference.

Small-for-size syndrome (SFSS) following living donor liver transplantation is a complication that can lead to devastating outcomes such as prolonged poor graft function and possibly graft loss. Because of the concern about the syndrome, some transplants of mismatched grafts may not be performed. Portal hyperperfusion of a small graft and hyperdynamic splanchnic circulation are recognized as main pathogenic factors for the syndrome.

Effectiveness and safety of ustekinumab for the treatment of psoriasis; six years of clinical experience.

Background: This work aimed to investigate the long-term clinical experience with ustekinumab in cases with psoriasis.

Materials And Methods: This retrospective cohort research group consisted of cases who presented to the dermatology outpatient clinics between January 2015 and January 2021, diagnosed with psoriasis, and were treated with ustekinumab. Data including gender, age, weight, disease duration, naïve and non-naïve status, psoriasis type, duration of medication, comorbidities, psoriasis area and severity index scores, the causes of treatment discontinuation, and previous treatments were retrospectively reviewed and analyzed.

Novel Benchmark for Adult-to-Adult Living-donor Liver Transplantation: Integrating Eastern and Western Experiences.

Objective: To define benchmark values for adult-to-adult living-donor liver transplantation (LDLT).

Background: LDLT utilizes living-donor hemiliver grafts to expand the donor pool and reduce waitlist mortality. Although references have been established for donor hepatectomy, no such information exists for recipients to enable conclusive quality and comparative assessments.

Early Postoperative Serum Phosphate Drop Predicts Sufficient Hypertrophy After Liver Surgery.

Objective: The aim of this study was to assess the impact of postoperative hypophosphatemia on liver regeneration after major liver surgery in the scenario of Associating Liver Partition with Portal vein ligation for Staged hepatectomy (ALPPS) and living liver donation (LLD).

Background: Hypophosphatemia has been described to reflect the metabolic demands of regenerating hepatocytes. Both ALPPS and LLD are characterized by an exceptionally strong liver regeneration and may be of particular interest in the context of posthepatectomy hypophosphatemia.

Early period intensive care follow-up after liver transplantation in children: a single center experience.

Background: Liver transplantation (LT) is a well-established, life-saving treatment for children with irreversible acute and chronic liver failure (LF). We aimed to evaluate the factors associated with morbidity and mortality in the early period of LT in children by reviewing our pediatric intensive care unit (PICU) experience.

Methods: We reviewed children`s medical records followed in the PICU after LT between May 2015-August 2021, including demographic parameters, indications for LT, operative variables, respiratory and circulatory support requirements, LT-related complications and survival.

Revival of associating liver partition and portal vein ligation for staged hepatectomy for perihilar cholangiocarcinoma: An international multicenter study with promising outcomes.

Background: Associating liver partition and portal vein ligation for staged hepatectomy for perihilar cholangiocarcinoma has been considered to be contraindicated due to the initial poor results. Given the recent reports of improved outcomes, we aimed to collect the recent experiences of different centers performing associating liver partition and portal vein ligation for staged hepatectomy for perihilar cholangiocarcinoma to analyze factors related to improved outcomes.

Methods: This proof-of-concept study collected contemporary cases of associating liver partition and portal vein ligation for staged hepatectomy for perihilar cholangiocarcinoma and analyzed for morbidity, short and long-term survival, and factors associated with outcomes.

Technical challenges in LDLT - Overcoming small for size syndrome and venous outflow reconstruction.

Living Donor Liver Transplantation (LDLT) emerged as an alternative treatment option for patients with end-stage liver disease waiting for an organ from a deceased donor. In addition to allowing for a faster access to transplantation, LDLT provides improved recipient outcomes when compared to deceased donor LT. However, it represents a more complex and demanding procedure for the transplant surgeon.

Real-life efficacy and drug continuation of secukinumab in treating moderate to severe plaque psoriasis in Aegean region of Turkey: a multicenter retrospective study and systematic review of the literature.

Background: Secukinumab was demonstrated to be efficient in the treatment of moderate to severe plaque psoriasis in the phase studies. Real-life treatment experiences obtained from patients that do not meet the inclusion criteria of phase studies can provide valuable information on efficacy and safety results. Results may also differ in different ethnic populations.

Ixekizumab for the treatment of the patients with moderate to severe plaque psoriasis: Clinical data from a real-world experience.

Real-life data about any particular treatment is very helpful for clinicians, particularly when managing a chronic disease such as psoriasis. In our study, we aimed to reflect our clinical experience during 48 weeks with an IL-17 antagonist ixekizumab. This study was designed as a retrospective multi-center study.

Comparison of Anatomic and Non-Anatomic Liver Resection for Hepatocellular Carcinoma: A Retrospective Cohort Study.

: The survival benefit of anatomical liver resection for hepatocellular carcinoma has not been elucidated yet. In this study, we aimed to investigate the effects of anatomic and non-anatomic liver resection on surgical outcomes in patients with hepatocellular carcinoma. : A retrospective analysis of patients undergoing anatomic or non-anatomic resections due to hepatocellular carcinoma between March 2006 and October 2019 was conducted.

Living liver donor hilar anatomical variations and impact of variant anatomy on transplant outcomes.

Donor anatomy is an essential part of donor selection and operative planning in living donor liver transplantation. In this study, variations of hilar structures, and the effects of variant anatomy on donor and recipient outcomes were evaluated. Living donor liver transplantations in a single center between January 2013 and December 2020 were retrospectively reviewed.

Uses of a dedicated 3D reconstruction software with augmented and mixed reality in planning and performing advanced liver surgery and living donor liver transplantation (with videos).

The development of digital intelligent diagnostic and treatment technology has opened countless new opportunities for liver surgery from the era of digital anatomy to a new era of digital diagnostics, virtual surgery simulation and using the created scenarios in real-time surgery using mixed reality. In this article, we described our experience on developing a dedicated 3 dimensional visualization and reconstruction software for surgeons to be used in advanced liver surgery and living donor liver transplantation. Furthermore, we shared the recent developments in the field by explaining the outreach of the software from virtual reality to augmented reality and mixed reality.