Search Results (138)

Background/Objectives: Hypothermic oxygenated perfusion (HOPE) improves graft preservation in whole liver transplantation, yet evidence supporting its use in split grafts for pediatric transplantation remains limited. Mitochondrial injury assessed during HOPE through spectroscopic measurement of flavin mononucleotide (FMN) has been associated with graft function in whole-organ transplantation. Here, we evaluate a human liver assessment pathway integrating mitochondrial viability testing with ex situ liver splitting during HOPE. Methods: Following standard procurement and transport, twelve discarded extended criteria human donor livers were evaluated for split feasibility and underwent HOPE (VitaSmart^®). Donors were between 40 and 72 years with a BMI of 23.4–42.7 kg/m² and 4–22 h of cold storage prior to HOPE. After two hours of portal-venous HOPE treatment, different split procedures were performed. Perfusates and tissues were analyzed for mitochondrial injury and inflammatory responses. Results: Four grafts met previously reported FMN thresholds for transplant suitability in whole-graft HOPE studies (FMN ≤ 0.02 μg/mL at 60 min). Livers with low FMN release demonstrated lower Complex I and II injury, greater ATP recovery, and reduced inflammatory signaling during HOPE. Conclusions: These findings indicate that mitochondrial injury during HOPE can be monitored during ex situ splitting and suggest that FMN-guided metabolic assessment may support graft evaluation within split liver transplantation pathways for pediatric recipients. Full article

Background: Kasai hepatoportoenterostomy (KPE) remains first-line therapy for biliary atresia(BA), yet the majority of patients will ultimately require liver transplantation. In centres where both KPE and paediatric liver transplantation are performed, KPE has been understood since the early 2000s as the first stage of a two-stage strategy, and the individual technical elements described here are established practice. What is reported is their uniform application as a single written protocol from the first case of the series, together with native liver and transplant outcomes in the resulting cohort. The technical conduct of KPE directly influences the safety and complexity of subsequent transplantation, yet operative decisions at the time of KPE have rarely been evaluated from a transplant-optimisation perspective. We describe a standardised KPE approach incorporating three technical modifications intended to preserve favourable conditions for eventual hepatic replacement and report native liver and transplant outcomes in the resulting cohort. Methods: A retrospective analysis was conducted of 74 consecutive KPE procedures performed by a single surgeon between 2014 and 2025 at Schneider Children’s Medical Center, Israel. The operative approach incorporated three deliberate modifications applied uniformly from the first case: a transverse subcostal incision aligned with future transplant access, avoidance of liver mobilisation and exteriorisation, and standardisation of the Roux limb at 50 cm. Primary outcomes were native liver survival and transplant operative parameters. Results: Of the 74 patients, 39 (52.7%) maintained their native liver throughout follow-up, while 35 (47.3%) required liver transplantation. No peri-operative mortality occurred. Median age at KPE was 53 days. In the Cox model, post-KPE portal hypertension (adjusted hazard ratio (aHR) 3.63, 95% confidence interval (CI) 1.40–9.42, p = 0.008) and hepatopulmonary syndrome (adjusted HR 10.23, 95% CI 2.19–47.85, p = 0.003) were associated with eventual transplantation; complications were modelled as fixed (ever/never) covariates because onset dates were not consistently retrievable, and these estimates may be subject to immortal-time bias. Among transplanted patients, the median operative time was 8.0 h (interquartile range (IQR) 6.0–10.2) (n = 34); intraoperative blood loss was documented in 15 of 35 patients (median 300 mL (IQR 205–450)). Conclusions: A standardised, transplant-orientated KPE approach was applied uniformly across 74 consecutive cases; 52.7% maintained their native liver, and transplantation in the remainder proceeded without peri-operative mortality. This series was descriptive by design and included no comparator group that operated without these modifications; it therefore cannot establish whether the modifications influence the complexity of subsequent transplantation, and no such inference should be drawn. Post-KPE portal hypertension and hepatopulmonary syndrome are markers associated with transplant requirement and should prompt intensified surveillance. The approach represents the standardisation of existing practice rather than a new technique. Full article

Background: Liver transplantation is the standard treatment for children with advanced hepato-blastoma when complete resection is not feasible. However, transplantation may be contraindicated because of persistent metastatic disease, severe comorbidities, poor clinical condition, or donor-related limitations. Associating Liver Partition and Portal Vein Ligation for Staged Hepatectomy (ALPPS) has emerged as a potential rescue strategy in highly selected patients. This study evaluated the feasibility, safety, and oncological outcomes of ALPPS in pediatric hepatoblastoma. Methods: A retrospective analysis was performed of four consecutive children with advanced hepatoblastoma who underwent classical ALPPS between 2013 and 2025 in two specialized centers. Patient characteristics, indications, future liver remnant (FLR) volumetry, perioperative outcomes, complications, and oncological follow-up were reviewed. Results: The median age at surgery was 22 months. In all patients, the FLR was considered insufficient for one-stage hepatectomy, leading to ALPPS. Rapid hypertrophy of the FLR was achieved in every case, allowing completion of the second stage after a median of 8.5 days (range, 7–11 days). FLR volume increased by 50–89% following the first stage. Despite successful liver hypertrophy, outcomes remained poor in three patients. One patient died intraoperatively from venous air embolism during the second stage, two died from disease recurrence despite aggressive multimodal treatment, and one remains in complete clinical and radiological remission following ALPPS and adjuvant chemotherapy. Conclusions: ALPPS reliably induces rapid FLR hypertrophy and may provide a potentially curative option for carefully selected children when liver transplantation is not feasible. However, its substantial perioperative risk and generally unfavorable oncological outcomes support its role only as a rescue procedure in experienced pediatric hepatobiliary centers. Further multicenter studies are needed to better define indications and patient selection. Full article

Pediatric acute liver failure (PALF) is a rare but life-threatening condition characterized by rapid clinical deterioration and high mortality. Viral infections represent a major etiology of PALF, although the causative agent remains unidentified in a substantial proportion of cases. Human Enteroviruses (EVs) are typically associated with self-limiting illnesses; however, they may rarely cause severe systemic disease, including fulminant hepatitis, particularly in neonates and young children. We describe the case of a 4-year-old previously healthy male who presented with acute fulminant hepatitis secondary to systemic Echovirus 25 (E25) infection, with concomitant Epstein–Barr virus (EBV) co-infection of recent onset. The diagnosis was established through multiplex PCR on cerebrospinal fluid, blood, stool, and nasopharyngeal aspirate, with serotype confirmation by the Italian National Institute of Health. The patient required intensive supportive care including therapeutic plasma exchange (TPE), continuous kidney replacement therapy (CKRT), and intravenous immunoglobulins (IGIV). Despite initial clinical deterioration and placement on the liver transplant list, the patient achieved complete hepatic recovery and was discharged after fourteen days of hospitalization without requiring transplantation. This case highlights the importance of prompt virological workup including enterovirus PCR in children presenting with acute liver failure of undetermined etiology and supports the use of extracorporeal therapies as a bridge to recovery. Full article

Background and Objectives: This study aimed to examine the level of depression in children who had undergone pediatric liver transplantation and to evaluate the potential role of liver enzymes as biomarkers of depression. Materials and Methods: The study was conducted with 50 pediatric liver transplant recipients followed at the Liver Transplantation Institute of İnönü University, and data were collected through face-to-face interviews. The Personal and Transplant Information Form, Child Revised Impact of Event Scale, and Patient Health Questionnaire–Depression were used as data collection tools. In addition to descriptive statistics, Student’s t-test, Mann–Whitney U test, correlation analyses, and regression analyses were performed. Results: The median PHQ-9 score was 1.00 (Q1–Q3: 0.00–5.00), indicating generally low levels of depression. A significant positive correlation was found between CRIES and PHQ-9 scores (r = 0.414, p < 0.01). In contrast, no consistent significant associations were observed between liver enzyme levels and depression scores in multivariate analyses, although bilirubin showed a modest negative correlation with PHQ-9 scores. In the multivariate analysis, although the overall regression model was not statistically significant, the CRIES score showed an individual association with PHQ-9 scores within the model (B = 0.117, p = 0.037). Conclusions: Liver enzymes cannot be considered strong biomarkers of depression in pediatric liver transplant recipients; however, post-traumatic stress symptoms may be an important clinical indicator for assessing psychological adjustment. Full article

Biliary atresia (BA) is the leading indication for pediatric liver transplantation. In the absence of surgical treatment, BA progresses rapidly toward hepatic fibrosis and cirrhosis. Although liver biopsy remains the gold standard for histological evaluation, its utility is limited by invasiveness, associated risks, and sampling variability. These limitations have spurred the development and validation of noninvasive tools to evaluate liver fibrosis in this patient population. Multiple imaging techniques have been developed to assess liver fibrosis and cirrhosis. In recent years, additional BA-related biomarkers have been identified, showing significant potential for diagnosis, assessment of fibrosis severity, and prediction of native liver survival outcomes. This article reviews the roles and potential clinical applications of the following biomarkers: matrix metalloproteinase-7 (MMP-7), fibroblast growth factor 19 (FGF-19), interleukin-33 (IL-33), clusterin, and osteopontin. Further research is needed to confirm the utility of these prognostic biomarkers in predicting and improving outcomes in BA. Full article

Background/Objectives: Early mortality after pediatric liver transplantation remains a clinical challenge, yet few studies have specifically addressed 30-day outcomes. Conventional pretransplant scores such as the age-appropriate MELD/PELD score were not designed for post-transplant risk prediction. We aimed to evaluate whether dynamic postoperative biomarker trajectories and novel composite ratios can identify high-risk patients. Methods: This single-center retrospective cohort study included 140 consecutive pediatric patients (<18 years) who underwent primary liver transplantation between 2015 and 2023. Patients were classified as deceased (≤30 days, n = 11) or survivors (>30 days, n = 129). PRISM-III, PELOD-2, and age-appropriate MELD/PELD scores were evaluated. Serial laboratory parameters were collected at pretransplant and at 0, 24, and 72 h. Delta (Δ) values and composite ratios—including lactate clearance, lactate-to-albumin ratio (LAR), INR×lactate product, platelet ratio, and fibrinogen/INR—were calculated. Penalized logistic regression (Firth method) was used for multivariate analysis. Internal validation was performed using bootstrap resampling (1000 iterations) and leave-one-out cross-validation (LOO-CV). Because two of the three components of the multivariable model (ΔINR, ΔALT) were derived from 72-h values, the model is best understood as a 72-h landmark risk model rather than as an immediate post-transplant early-warning tool. Results: The 30-day mortality rate was 7.9% (11/140), with central nervous system complications as the leading cause (36.4%). PRISM-III demonstrated excellent discrimination (AUROC 0.957; cut-off ≥ 14); the age-appropriate MELD/PELD score, a pretransplant tool not designed for post-transplant prediction, showed near-chance performance (AUROC 0.513; p = 0.576). A distinctive biomarker crossover pattern was observed: non-survivors had paradoxically lower pretransplant INR, ALT, and LAR values, but trajectories diverged sharply by 24 h. The INR×lactate product achieved an AUROC of 0.981 at 72 h. LAR at 24 h achieved 0.909, and lactate clearance at 0 → 72 h achieved 0.783. Postoperative hypernatremia emerged as a strong predictor (AUROC 0.884). In multivariate analysis, PRISM-III (OR 4.00), ΔINR (OR 3.28), and ΔALT (OR 3.46) were independent predictors (apparent AUROC 0.989). Internal validation confirmed model stability: bootstrap-corrected AUROC was 0.978; LOO-CV AUROC was 0.957 (sensitivity 90.9%, specificity 96.9%). Conclusions: Dynamic postoperative factors—rather than pretransplant disease severity—appeared more strongly associated with 30-day mortality after pediatric liver transplantation in this single-center exploratory analysis. The INR×lactate product, a novel two-variable composite, showed very high apparent discrimination (AUROC 0.981) and is proposed as a hypothesis-generating candidate marker requiring prospective external validation before any clinical use. The combined PRISM-III + ΔINR + ΔALT model (best understood as a 72-h landmark risk model, since two of its three components are defined at 72 h postoperatively) demonstrated robust internal validation performance (LOO-CV AUROC 0.957); however, given the small number of events (n = 11) and the absence of external validation, the model should be regarded as exploratory. Full article

Background/Objectives: Legume allergy is increasingly recognized as plant-based diets expand and legume proteins are widely used in processed foods. We aimed to characterize the clinical features, sensitization profiles, and management outcomes of IgE-mediated legume allergy in Italian children. Methods: This retrospective single-center study (January 2022–January 2024) included children (<18 years) allergic to ≥1 index legume (pea, lentil, chickpea, common bean, or soy). Diagnosis required a compatible clinical history and evidence of IgE sensitization. Clinical and allergy characteristics were analyzed. Results: Fifty-five children (63.6% male) were included; all had atopic comorbidities, and 96.4% had additional food allergies. Median age at first reaction was 18 months; anaphylaxis occurred at onset in 12.7%, most frequently triggered by pea. Pea (70.9%) and lentil (69.1%) were the most prevalent allergies, with pea causing 50% of index-legume anaphylaxis. Multi-legume allergy predominated (74.5%), with frequent co-allergy among pea, lentil, and chickpea (56–86%). Soy allergy was less frequent and mainly associated with Gly m 4 sensitization. Single-legume allergy (25.5%) was associated with later onset (54 vs. 15 months; p = 0.013) and liver transplantation (21% vs. 2%; p = 0.047). Peanut co-allergy occurred in 25.5%. Among 34 oral food challenges (OFCs), 23.5% were positive, including one case of pea-induced anaphylaxis. Of 16 oral immunotherapy (OIT) protocols initiated, 31.3% reached the full target maintenance dose, 37.5% remained on a lower, partial maintenance dose, and 31.3% were discontinued due to oral allergy syndrome (OAS). Conclusions: Pediatric legume allergy is characterized by early onset, frequent multi-legume involvement, and common co-allergies. In this cohort, pea allergy was associated with the highest proportion of severe reactions. Species-specific differences in severity, patterns of multi-legume involvement, and OIT outcomes should be interpreted cautiously given the limited sample size, while highlighting the need for tailored management and improved risk assessment across legume species. Full article

Organoids are three-dimensional culture systems that self-organize and partially recapitulate the architecture, cellular composition, and functional properties of native tissues. In pediatric congenital hepatobiliary diseases, persistent cholestasis, bile duct maldevelopment, epithelial injury, and progressive fibrosis often lead to cirrhosis, liver failure, or the necessity for liver transplantation. Compared with conventional two-dimensional cell culture and animal models, hepatobiliary organoids provide patient-derived, human-relevant platforms for modeling disease mechanisms, evaluating therapeutic responses, and exploring regenerative strategies. Unlike previous reviews that mainly discuss general organoid culture systems or broad liver disease modeling, this review is organized around clinically oriented translational endpoints, including mechanistic target discovery, prognostic stratification, therapeutic validation, and regenerative reconstruction. We further discuss current barriers to clinical translation, including reproducibility, scalability, vascularization, immune integration, manufacturing standardization, and patient-specific genetic, environmental, and dietary modifiers. By integrating disease-specific mechanisms with translational applications, this review provides a framework for understanding how organoid-based platforms may contribute to future diagnosis, risk assessment, therapeutic decision-making, and regenerative medicine in pediatric congenital hepatobiliary disorders. Full article

Background/Objectives: Survival rates after pediatric liver transplantation have improved substantially over recent decades, yet the psychiatric consequences for recipients remain a concern that warrants closer attention. We sought to map the psychiatric symptom burden across multiple domains in this population and to determine which symptom clusters carry the greatest impact on health-related quality of life (HRQOL). Materials and Methods: Fifty liver transplant recipients between the ages of 8 and 18 were enrolled at a single center. Children and their parents completed four psychiatric measures—the CBCL, CDI, SCARED, and CRIES-13—alongside the parent-proxy PedsQL to capture HRQOL across physical, emotional, social, and school functioning domains. Correlations between instruments were calculated, and linear regression was used to determine which psychiatric variables independently predicted PedsQL Total scores. Results: Across all psychiatric measures, higher symptom scores were associated with lower HRQOL, with school functioning recording the lowest absolute PedsQL domain score, while emotional functioning demonstrated the strongest and most consistent inverse correlations with all psychiatric symptom measures across instruments. CBCL Total (r = −0.607), SCARED Total (r = −0.557), and CRIES-13 Total (r = −0.548) scores all correlated meaningfully with overall HRQOL. When entered into multivariable analysis, anxiety symptoms measured by the SCARED (β = −0.295, p = 0.032) and post-traumatic stress symptoms measured by the CRIES-13 (β = −0.400, p = 0.004) stood out as the two independent predictors of worse PedsQL Total scores. Conclusions: Even in medically stable recipients, anxiety and post-traumatic stress symptoms were independently associated with lower daily functioning scores and overall quality of life. These findings suggest that routine psychosocial screening and trauma-informed approaches may warrant integration into post-transplant care protocols, and that prospective, adequately powered studies are needed to confirm and extend these associations. Full article

Background: Parents of children undergoing liver transplantation face substantial caregiving demands that may adversely affect their mental health across multiple domains. Systematic evaluation of psychosocial outcomes in this population remains limited, particularly in settings that include immigrant families. Method: This was a single-center, cross-sectional study including the parents of 50 children after liver transplantation. Major sociodemographic variables included parental age, sex, education, chronic disease, and immigration status. We also recorded children’s demographics, transplant-related data, follow-up findings, and mental health status. Instruments for psychiatric assessment included the Generalized Anxiety Disorder 7-item scale (GAD-7; anxiety), Patient Health Questionnaire-9 (PHQ-9; depression), Perceived Stress Scale-10 (PSS-10; stress), and Pittsburgh Sleep Quality Index (PSQI; sleep quality). Results: We enrolled 50 parents of 50 pediatric liver transplant recipients (43 Turkish citizens, 7 Syrian immigrants; 28 fathers, 22 mothers; mean age: 40.10 ± 6.65). Time since transplantation showed weak negative correlation with PHQ-9 and GAD-7. Stress (PSS) levels had weak to strong positive correlation with PSQI, PHQ-9, and GAD-7. Sleep quality (PSQI) was positively correlated with PHQ-9 and GAD-7. Depressive findings (PHQ-9) were strongly and positively correlated with GAD-7. In Firth-penalized multivariable models, high PHQ-9 scores were independently associated with shorter time since transplantation (p = 0.001) and high PSS (p = 0.003). High GAD-7 scores were independently associated with shorter time since transplantation (p = 0.025) and high PSS (p = 0.001). Conclusions: The parents of pediatric liver transplant recipients experience high levels of stress, sleep issues, depression, and anxiety, which demonstrate multiple correlations. Full article

Introduction: Presently, liver transplantation is becoming a more common treatment option for adults and children suffering from liver failure. Antibody-mediated rejection (AMR), a phenomenon that is exceedingly uncommon and inadequately comprehended, may induce graft dysfunction. The objective of the investigation was to evaluate the clinical and histopathological manifestations of AMR in pediatric patients. Material and methods: The retrospective study comprised sixty-two liver core biopsies from forty-two pediatric patients. In a total of 10 biopsies, 7 children were diagnosed with AMR, while 35 of them exhibited features of acute T-cell-mediated rejection (TCMR) in 52 biopsies. The C4d binding assay was conducted in all biopsies using the immunohistochemical (IHC) method. Bilirubinostasis, steatosis and acute and chronic rejection were re-assessed in all specimens. The 6-grade Ishak scale was employed to evaluate fibrosis. The TCMR activity was established using the Banff classification. AMR was assessed according to a novel histopathological grading system that was developed by the authors. Depending on the type of rejection, the relationship between histopathological grading, morphological characteristics, and laboratory parameters was established for each group. Standard methods were implemented to conduct statistical analysis. Results: At the time of biopsy, the median age of patients was 47.6 months (15.03–98.83) and the median time from transplantation was 0.9 months (0.3–7.6). The study’s findings provided evidence that histopathological lesions were the least specific manifestation, which supported the presence of AMR. A positive C4d staining statistically increases the likelihood of AMR diagnosis, whether or not there are associated morphological abnormalities. The type of rejection and laboratory tests did not exhibit any statistically significant correlation. Conclusions: The diagnosis of AMR in a transplanted liver is intricate and requires a multifaceted approach. However, the proposed histopathological grading may be a helpful method for selecting patients who should be assessed for donor-specific antibodies (DSAs) or in whom AMR should be suspected when DSAs cannot be determined. Full article

Background: Percutaneous liver biopsy is a cornerstone in the diagnostic and therapeutic management of pediatric liver diseases. However, data on the optimal needle gauge for coaxial techniques in children remain scarce. Smaller-gauge needles may theoretically enhance safety but could potentially compromise diagnostic yield. Objectives: The primary objective of this study was to evaluate and compare the safety and diagnostic clinical adequacy of ultrasound-guided percutaneous liver biopsies performed with semi-automated 20G versus 18G coaxial needles in pediatric patients. Patients and Methods: This retrospective cohort study included consecutive patients aged ≤19 years who underwent percutaneous non-targeted liver biopsies at a tertiary medical center between 2006 and 2012. Patient demographics, biopsy technique parameters (including needle gauge, number of cores, and tract embolization), and procedure-related complications were analyzed. Procedural success was defined by diagnostic and clinical adequacy, requiring a definitive pathology report and the presence of ≥7 portal tracts (the widely accepted threshold for a reliable histologic diagnosis). Complications were classified according to the Society of Interventional Radiology guidelines. Results: A total of 320 biopsies were performed in 260 patients (44.6% female; mean age 7.4 ± 6.0 years). Common indications included post-liver transplantation surveillance (28.4%) and unexplained liver enzyme elevation (22.5%). Biopsies were performed using 18G (n = 148; 46.3%) or 20G (n = 172; 53.7%) coaxial needles. Diagnostic and clinical adequacy was achieved in 100% of the procedures, with biopsy results directly influencing clinical management in 39.7% of cases. The overall complication rate was 5.3% (3.4% minor, 1.9% major), with no procedure-related mortality. While raw complication rates were numerically higher in the 20G group (likely to reflect an operator-driven selection bias for younger or higher-risk patients), the differences between the 18G and 20G needles were not statistically significant. Notably, the use of the 20G needle was associated with a significantly reduced clinical need for post-biopsy tract embolization. Conclusions: Our findings demonstrate no statistically significant differences in complication rates or diagnostic clinical adequacy between 18G and 20G coaxial needles for pediatric percutaneous liver biopsies. When selected based on appropriate clinical judgment, the 20G needle provides a high diagnostic yield and serves as an effective option, particularly for reducing the need for tract embolization. However, both 18G and 20G needles represent acceptable clinical options within the pediatric interventional armamentarium. Ultimately, the choice of needle gauge should be meticulously tailored to individual patient characteristics, bleeding risk profiles, and specific clinical indications, rather than uniformly recommending a smaller gauge across all pediatric age groups. Full article

Background and Clinical Significance: Dyskeratosis congenita (DC) is a rare inherited disorder classified as a telomere biology disorder and characterized by multisystem involvement, including bone marrow failure and mucocutaneous abnormalities. Oral manifestations such as leukoplakia, increased susceptibility to infection, and abnormal dental development have been reported; however, detailed descriptions of long-term oral functional management in pediatric patients remain limited. This report aims to describe the longitudinal oral management and imaging-based assessment of dental development in a child with DC and to discuss the clinical implications in the context of existing literature. Case Presentation: A female patient diagnosed with dyskeratosis congenita due to a heterozygous TINF2 mutation was followed from early childhood. She underwent hematopoietic stem-cell transplantation at five years of age and later required lung and liver transplantation. Long-term oral management included regular professional oral care, preventive strategies, and periodic imaging evaluation. Panoramic radiographs obtained over several years demonstrated generalized delayed eruption and incomplete root formation relative to chronological age, with apparent early arrest of root elongation. Discussion: This case highlights the potential association between telomere dysfunction, intensive systemic therapy in early childhood, and arrested odontogenesis. These findings suggest a possible association between telomere dysfunction, early intensive systemic therapy, and impaired root formation. Despite severe systemic disease, continuous preventive oral care and imaging-based monitoring were effective in maintaining oral health and detecting mucosal changes. Conclusions: Long-term preventive oral management combined with noninvasive imaging assessment may play an important role in preserving oral function and monitoring dental development in pediatric patients with dyskeratosis congenita. This case adds to the limited literature on longitudinal oral outcomes in this rare disorder. Full article

A history of invasive mold disease (IMD) often delays or contraindicates allogeneic hematopoietic stem cell transplantation (allo-HSCT) in children. Given the limited data on pediatric patients with pre-allo-HSCT IMD, we aimed to describe the management and clinical outcomes of a cohort of children with IMD prior to allo-HSCT through day +100 post-transplantation. Between 2021 and 2024, ten pediatric patients were identified with proven or probable IMD. Their median age was 8.5 years. The most common pathogens were Aspergillus (n = 5) and Fusarium (n = 4). Infections most frequently involved the lungs followed by paranasal sinuses, bloodstream, liver, and skin. All patients demonstrated clinical improvement before transplantation, and by day +100 post-HSCT, no IMD relapses or infection-related mortality were observed. These findings suggest that complete radiologic or clinical resolution is not a prerequisite for proceeding with transplantation. Recent IMD should not be considered an absolute contraindication to urgent allo-HSCT when clinical improvement is evident, as transplantation facilitates immune reconstitution necessary for definitive infection control. Full article