Fibrous hamartoma of infancy (FHI) is a rare benign soft tissue tumor with a triphasic organoid histologic appearance. The authors present a case of a 21-month-old healthy girl with a slowly growing flesh-colored subcutaneous plaque 12cm in size on the lower back, with overlying hypertrichosis. A punch biopsy revealed a proliferation of spindle cells infiltrating the dermis and hypodermis organized in a dense storiform pattern with a strong diffuse positivity for CD34. The diagnosis of congenital dermatofibrosarcoma protuberans (DFSP) was considered and an excision was performed. Histopathologic analysis showed an extensive poorly demarcated mass infiltrating the dermis and hypodermis, composed of different components: a monomorphous fibroblastic/myofibroblastic component, a mature adipose component, and an immature mesenchymal basophilic component. The clinical aspects with the histologic and immunohistochemical features led to the diagnosis of giant fibrous hamartoma of infancy. In our case the strong diffuse CD34 positivity was a diagnostic pitfall leading to an incorrect hypothesis of congenital DFSP. The characteristic triphasic histology of FHI was missed owing to the small size of the punch biopsy. This article highlights the importance of being aware of the CD34+ dermal mesenchymal tumor differential diagnosis and the necessity of appropriate size biopsies to avoid sampling error.

BACKGROUND: Current techniques for donation after circulatory determination of death (DCD) heart procurement, through either direct procurement and machine perfusion or thoracoabdominal normothermic regional perfusion (NRP), have demonstrated excellent heart transplant outcomes. However, the impact of thoracoabdominal DCD (TA-DCD) heart procurement on liver allograft outcomes and utilization is poorly understood. METHODS: One hundred sixty simultaneous heart and liver DCD donors were identified using the United Network for Organ Sharing/Organ Procurement and Transplantation Network database between December 2019 and July 2021. Liver outcomes from TA-DCD donors were stratified by heart procurement technique and evaluated for organ utilization, graft survival, and patient survival. Results were compared with abdominal-only DCD (A-DCD; n = 1332) and donation after brain death (DBD; n = 12 891) liver transplants during the study interval. Kaplan-Meier methods with log-rank testing were used to evaluate patient and graft survival. RESULTS: One hundred thirty-three of 160 livers procured from TA-DCD donors proceeded to transplant. TA-DCD donors were younger (mean 28.26 y; P < 0.0001) with lower body mass index (mean 26.61; P < 0.0001) than A-DCD and DBD donors. TA-DCD livers had equivalent patient survival ( P = 0.893) and superior graft survival (P = 0.009) compared with A-DCD. TA-DCD livers had higher rates of organ discard for long warm ischemia time (37.0%) than A-DCD (20.5%) and DBD (0.5%; P < 0.0001), with direct procurement and machine perfusion procurements leading to a higher discard rate (18.5%) than NRP procurements (7.4%). CONCLUSIONS: Liver transplants after TA-DCD donation demonstrated equivalent patient outcomes and excellent graft outcomes. NRP procurements resulted in the lowest rate of organ discard after DCD donation and may represent an optimal strategy to maximize organ utilization.

INTRODUCTION: The impact of donation after circulatory death (DCD) heart procurement techniques on the utilization and outcomes of concurrently procured DCD livers and kidneys remains unclear. METHODS: Using the United Network for Organ Sharing database, we identified 246 DCD donors whose heart was procured using direct procurement and ex-situ machine perfusion and 128 DCD donors whose heart was procured using in-situ thoracoabdominal normothermic regional perfusion (12/2019-03/2022). We evaluated the transplantation rate of concurrently procured DCD livers and kidneys (defined as the number of organs transplanted/total number of organs available for procurement) and their post-transplant outcomes. RESULTS: The transplantation rate of concurrently procured DCD livers was higher with in-situ perfusion compared to direct procurement (67.1% vs 56.5%, p = 0.045). After excluding pediatric, multiorgan, and repeat transplant recipients, there was no difference in 6-month liver graft failure rate (direct procurement 0.9% vs in-situ perfusion 0%, p > 0.99). Recipients of kidneys procured with in-situ perfusion had less delayed graft function (11.3% vs 41.5%, p < 0.0001) shorter length of stay, and lower serum creatinine at discharge (both p < 0.05). Six-month recipient survival in the direct procurement and in-situ perfusion group were similar after DCD liver and kidney transplantation (p = 0.24 and 0.79 respectively). CONCLUSIONS: Compared to direct procurement, DCD heart procurement with in-situ thoracoabdominal normothermic regional perfusion was associated with increased utilization of DCD livers and a lower incidence of delayed graft function in concurrently procured DCD kidneys. Broader implementation of DCD heart transplantation must maximize the transplant potential of concurrently procured abdominal organs and ensure their successful outcomes.

BACKGROUND: Appropriate patient selection for simultaneous heart-kidney transplantation (sHK) in patients with moderate renal dysfunction remains challenging. METHODS: From the United Network for Organ Sharing database (2003-2020), we identified 5678 adults with an estimated pre-transplant glomerular filtration rate (eGFR) between 30 and 45 mL/min/1.73 m2 and no pre-transplant dialysis. Patients undergoing sHK (n = 293) were compared with those undergoing heart transplantation alone (n = 5385) using 1:3 propensity score matching. RESULTS: The sHK utilization rate increased from 1.8% in 2003 to 12.2% in 2020 (p < .001). After matching, 1 and 5-year survival was 87.7% (95% confidence interval [CI] 83.3-91.0) and 80.0% (95% CI 74.2-84.6) after sHK, and 87.3% (95% CI 85.2-89.1) and 71.8% (95% CI 68.4-74.9) after heart transplant alone (p = .04). In the subgroup analysis, sHK was associated with a 5-year survival benefit only in patients with 30 < eGFR ≤ 35 mL/min/1.73 m2 (p = .05) but not in those with 35 < eGFR < 45 mL/min/1.73 m2 (p = .45). Patients who underwent heart transplants alone also had a higher incidence of becoming chronic dialysis-dependent after transplant within 5-year follow-up (10.2%, 95% CI 8.0-12.6 vs. 3.8%, 95% CI 1.7-7.1, p = .004). The 5-year incidence of subsequent kidney waitlisting and transplants after heart transplants alone was 5.6% and 1.9%, respectively. CONCLUSION: Among propensity-matched patients without pre-transplant dialysis, compared to heart transplants alone, sHK had improved 5-year survival in those with 30 < eGFR ≤ 35 but not in those with 35 < eGFR < 45 mL/min/1.73 m2 . One-year survival was similar irrespective of eGFR. Receiving a kidney after a heart transplant alone is rare under the current allocation system.

BACKGROUND: Given ongoing donor shortages, appropriate patient selection for dual-organ transplantation is critical. We evaluated outcomes of heart retransplant with simultaneous kidney transplant (HRT-KT) vs isolated heart retransplant (HRT) across varying levels of renal dysfunction. METHODS: The United Network for Organ Sharing database identified 1189 adult patients undergoing heart retransplantation between 2005 and 2020. Recipients undergoing HRT-KT (n = 251) were compared to those undergoing HRT (n = 938). The primary outcome was 5-year survival; subgroup analyses and multivariable adjustment were performed utilizing the following 3 estimated glomerular filtration (eGFR) groups: <30 ml/min/1.73m2, 30-45 ml/min/1.73m2, and >45 ml/min/1.73m2. RESULTS: HRT-KT recipients were older and had longer waitlist times, longer inter-transplant periods, and lower eGFR levels. HRT-KT recipients were less likely to require pretransplant ventilator (1.2% vs 9.0%, p < 0.001) or ECMO (2.0% vs 8.3%, p < 0.001) support but were more likely to have severe functional limitation (63.4% vs 52.6%, p = 0.001). After retransplantation, HRT-KT recipients had less treated acute rejection (5.2% vs 9.3%, p = 0.02) and more dialysis requirement (29.1% vs 20.2%, p < 0.001) before discharge. Survival at 5-years was 69.1% after HRT and 80.5% after HRT-KT (p < 0.001). After adjustment, HRT-KT was associated with improved 5-year survival among recipients with eGFR <30 ml/min/1.73m2 (HR:0.42, 95% CI: 0.26-0.67) and 30 to 45 ml/min/1.73m2 (HR:0.29, 95% CI 0.13-0.65), but not among those with eGFR>45 ml/min/1.73sm2 (HR 0.68, 95% CI 0.30-1.54). CONCLUSION: Simultaneous kidney transplantation is associated with improved survival following heart retransplantation in patients with eGFR <45 ml/min/1.73m2 and should be strongly considered to optimize organ allocation stewardship.

INTRODUCTION: The Organ Care System (OCS) is an ex vivo perfusion platform for donor heart preservation. Short/mid-term post-transplant outcomes after its use are comparable to standard cold storage (CS). We evaluated long-term outcomes following its use. METHODS: Between 2011 and 2013, 38 patients from a single center were randomized as a part of the PROCEED II trial to receive allografts preserved with CS (n = 19) or OCS (n = 19). Endpoints included 8-year survival, survival free from graft-related deaths, freedom from cardiac allograft vasculopathy (CAV), non-fatal major adverse cardiac events (NF-MACE), and rejections. RESULTS: Eight-year survival was 57.9% in the OCS group and 73.7% in the CS group (p = .24). Freedom from CAV was 89.5% in the OCS group and 67.8% in the CS group (p = .13). Freedom from NF-MACE was 89.5% in the OCS group and 67.5% in the CS group (p = .14). Eight-year survival free from graft-related death was equivalent between the two groups (84.2% vs. 84.2%, p = .93). No differences in rejection episodes were observed (all p > .5). CONCLUSIONS: In select patients receiving OCS preserved allografts, late post-transplant survival trended lower than those transplanted with an allograft preserved with CS. This is based on a small single-center series, and larger numbers are needed to confirm these findings.

Prenatal micronutrient deficiencies are associated with negative maternal and birth outcomes. Multiple micronutrient supplementation (MMS) during pregnancy is a cost-effective intervention to reduce these adverse outcomes. However, important knowledge gaps remain in the implementation of MMS interventions. The Child Health and Nutrition Research Initiative (CHNRI) methodology was applied to inform the direction of research and investments needed to support the implementation of MMS interventions for pregnant women in low- and middle-income countries (LMIC). Following CHNRI methodology guidelines, a group of international experts in nutrition and maternal health provided and ranked the research questions that most urgently need to be resolved for prenatal MMS interventions to be successfully implemented. Seventy-three research questions were received, analyzed, and reorganized, resulting in 35 consolidated research questions. These were scored against four criteria, yielding a priority ranking where the top 10 research options focused on strategies to increase antenatal care attendance and MMS adherence, methods needed to identify populations more likely to benefit from MMS interventions and some discovery issues (e.g., potential benefit of extending MMS through lactation). This exercise prioritized 35 discrete research questions that merit serious consideration for the potential of MMS during pregnancy to be optimized in LMIC.

The evolution of land flora transformed the terrestrial environment. Land plants evolved from an ancestral charophycean alga from which they inherited developmental, biochemical, and cell biological attributes. Additional biochemical and physiological adaptations to land, and a life cycle with an alternation between multicellular haploid and diploid generations that facilitated efficient dispersal of desiccation tolerant spores, evolved in the ancestral land plant. We analyzed the genome of the liverwort Marchantia polymorpha, a member of a basal land plant lineage. Relative to charophycean algae, land plant genomes are characterized by genes encoding novel biochemical pathways, new phytohormone signaling pathways (notably auxin), expanded repertoires of signaling pathways, and increased diversity in some transcription factor families. Compared with other sequenced land plants, M. polymorpha exhibits low genetic redundancy in most regulatory pathways, with this portion of its genome resembling that predicted for the ancestral land plant. PAPERCLIP.