First case report of multivisceral transplant from a deceased cardiac death donor.

The current shortage of pediatric multivisceral donors accounts for the long time and mortality on the waiting list of pediatric patients. The use of donors after cardiac death, especially after the outbreak of normothermic regional perfusion, has increased in recent years for all solid organs except the intestine, mainly because of its higher susceptibility to ischemia-reperfusion injury. We present the first literature case of multivisceral donors after cardiac death transplantation in a 13-month-old recipient from a 2.5-month-old donor. Once exitus was certified, an extracorporeal membrane oxygenation circuit was established, cannulating the aorta and infrarenal vena cava, while the supra-aortic branches were clamped. The abdominal organs completely recovered from ischemia through normothermic regional perfusion (extracorporeal membrane oxygenation initially and beating heart later). After perfusion with the preservation solution, the multivisceral graft was uneventfully implanted. Two months later, the patient was discharged without any complications. This case demonstrates the possibility of reducing the time spent on the waiting list for these patients.

Interocular Symmetry of Choroidal Thickness and Volume in Healthy Eyes on Optical Coherence Tomography.

BACKGROUND: We aimed to determine the physiological symmetry, with spectral-domain optical coherence tomography, of choroidal measurements in a healthy population in all the areas defined in the Early Treatment Diabetic Retinopathy Study (ETDRS). METHODS: One hundred and fifty-four eyes of 77 healthy young adults between the ages of 19 and 32 years were enrolled. Differences in choroidal thickness (CT) and volume (CV) between the left and right eyes were calculated. Normal ranges of absolute interocular differences were established as the 95th percentile. RESULTS: The mean ± SD subfoveal CT (SFCT) and total CV values in the right and left eyes were 342.03 ± 77.38 versus 361.64 ± 76.45 µm (correlation coefficient ρ = 0.820; p < 0.001) and 0.27 ± 0.06 versus 0.28 ± 0.06 mm3 (ρ = 0.830; p < 0.001), respectively. Differences in 5 of the 9 areas of the ETDRS map were statistically significant (p < 0.05), but with a strong interocular correlation (ρ > 0.8; p < 0.001). The 95th percentile of interocular tolerance limits for CT in the 1-, 3-, and 6-mm areas were 97, 70, and 57 µm, respectively; the 95th percentile for the volume values were 0.06, 0.51, and 1.73 mm3. CONCLUSIONS: CT and CV are highly correlated between eyes, statistically significant differences between them can be found, and absolute interocular differences may reach 97 µm in SFCT, and 1.73 mm3 in total CV.

CHOROIDAL THICKNESS AND VOLUME IN A HEALTHY PEDIATRIC POPULATION AND ITS RELATIONSHIP WITH AGE, AXIAL LENGTH, AMETROPIA, AND SEX.

PURPOSE: To evaluate choroidal thickness (CT) and volume in healthy pediatric individuals using enhanced depth imaging spectral domain optical coherence tomography (SD-OCT), as well as its association with age, sex, axial length (AL), and refractive error. METHODS: Ninety-three eyes from 93 healthy pediatric individuals were examined. An Early Treatment Diabetic Retinopathy Study grid was applied to analyze CT and volume map in each of its nine sectors. RESULTS: The mean subfoveal CT and volume were 314.22 ± 55.48 µm and 0.25 ± 0.04 mm, respectively. The nasal CT and volume of both the inner and the outer rings were significantly lower than the temporal area of the same ring and lower than the subfoveal choroidal thickness. A significant negative correlation between the subfoveal CT and AL (r = -0.250, P = 0.015) and a significant positive correlation between the subfoveal CT and refractive error (r = 0.238, P = 0.006) were found. The estimation of the variation in the subfoveal CT in relationship to the AL was -13.55 µm per millimeter. The variation in the subfoveal CT with refractive error was 7.52 µm per diopter. The estimation of the variation in the total choroidal volume related to the AL and ametropia was, respectively, -0.2354 mm per millimeter and 0.1412 mm per diopter. CONCLUSION: Healthy pediatric subjects exhibit choroidal differences in refractive error and AL. In the study population, CT and volume show an increase with age after adjusting for AL.

Descemet's Membrane Detachment during Phacocanaloplasty: Case Series and In-Depth Literature Review.

This article presents three cases of Descemet's membrane detachment (DMD) occurring during 'ab externo' phacocanaloplasty procedures in three patients with uncontrolled primary open-angle glaucoma (OAG) and discusses the management of this condition by reviewing the available literature. Following a successful 360° cannulation of Schlemm's canal (SC), the microcatheter was withdrawn while an ophthalmic viscosurgical device (OVD) was injected into the canal. During passage through the inferonasal quadrant, a spontaneous separation of the posterior layer of the cornea was observed. Each case was managed differently after diagnosis, with the third case being drained intraoperatively based on experience gained from the previous cases. On the first postoperative day, slit-lamp biomicroscopy (BMC) revealed multiple DMDs in case one and a hyphema in the lower third of a deep anterior chamber. In the other two cases, a single DMD was observed. The second case developed hemorrhagic Descemet membrane detachment (HDMD), while the other two were non-hemorrhagic. In all three cases, anterior segment optical coherence tomography (AS-OCT) revealed the presence of retrocorneal hyperreflective membranes indicative of DMDs. These membranes were located in the periphery of the cornea and did not impact the visual axis. After evaluation, a small incision was made in the inferotemporal DMD of the first case. However, for the two remaining cases, a strategy of watchful waiting was deemed appropriate due to the location and size of the DMDs, as they did not affect the best-corrected visual acuity (BCVA). Over time, the patients demonstrated progressive improvement with a gradual reduction in the size of the DMDs.

Submucosal Necrotic Nodule of the Colon: An Enigmatic Entity Potentially Related to Anisakis Infection.

CONTEXT.­: Discrete submucosal necrotic nodules may rarely manifest as colon polyps. OBJECTIVE.­: To characterize the clinical and pathologic features of this lesion, which has been under-studied in the literature. DESIGN.­: We conducted an international search to compile a series. For each potential case, photomicrographs were centrally reviewed to confirm the diagnosis. We gathered clinical and pathologic information on each confirmed case. RESULTS.­: The final cohort included 25 patients, with 23 having 1 lesion and 2 having several (31 lesions total). Mean patient age was 62 years; 13 patients (52%) were male. Symptoms were nonspecific, although 4 patients (16%) had blood in stool; 14 patients were asymptomatic. Patient history and medications appeared noncontributory. Most cases were located in the right colon (n = 18; 58%). Mean lesion size was 0.4 cm (range, 0.1-1.7 cm). Histology typically showed a centrally necrotic nodule with peripheral fibrosis, chronic inflammation, and sometimes palisading granulomatous inflammation. Percent necrosis ranged from 5% to 95% (average, 70%), and percent fibrosis ranged from 3% to 70% (average, 25%). In 3 cases, degenerated parasitic structures consistent with Anisakis could be seen on hematoxylin-eosin and trichrome special stain. No patient experienced disease recurrence. CONCLUSIONS.­: Submucosal necrotic nodules can present as colon polyps. Most cases are unifocal, and patients do well on follow-up. At least some examples appear to be caused by Anisakis, implicating patient diet. Patients are often asymptomatic, and many cases show no histologic evidence of the causative agent.

Study protocol of a randomized controlled trial to assess safety of teleconsultation compared with face-to-face consultation: the ECASeT study.

BACKGROUND: The use of remote consultation modalities has exponentially grown in the past few years, particularly since the onset of the COVID-19 pandemic. Although a huge body of the literature has described the use of phone (tele) and video consultations, very few of the studies correspond to randomized controlled trials, and none of them has assessed the safety of these consultation modalities as the primary objective. The primary objective of this trial was to assess the safety of remote consultations (both video and teleconsultation) in the follow-up of patients in the hospital setting. METHODS: Multicenter, randomized controlled trial being conducted in four centers of an administrative healthcare area in Catalonia (North-East Spain). Participants will be screened from all individuals, irrespective of age and sex, who require follow-up in outpatient consultations of any of the departments involved in the study. Eligibility criteria have been established based on the local guidelines for screening patients for remote consultation. Participants will be randomly allocated into one of the two study arms: conventional face-to-face consultation (control) and remote consultation, either teleconsultation or video consultation (intervention). Routine follow-up visits will be scheduled at a frequency determined by the physician based on the diagnostic and therapy of the baseline disease (the one triggering enrollment). The primary outcome will be the number of adverse reactions and complications related to the baseline disease. Secondary outcomes will include non-scheduled visits and hospitalizations, as well as usability features of remote consultations. All data will either be recorded in an electronic clinical report form or retrieved from local electronic health records. Based on the complications and adverse reaction rates reported in the literature, we established a target sample size of 1068 participants per arm. Recruitment started in May 2022 and is expected to end in May 2024. DISCUSSION: The scarcity of precedents on the assessment of remote consultation modalities using randomized controlled designs challenges making design decisions, including recruitment, selection criteria, and outcome definition, which are discussed in the manuscript. TRIAL REGISTRATION: NCT05094180. The items of the WHO checklist for trial registration are available in Additional file 1. Registered on 24 November 2021.

Primary Pure Pancreatic -Type Acinar Cell Carcinoma of the Stomach: A Rare Entity with a Difficult Diagnosis and Review of the Literature.

Acinar cell carcinoma is an uncommon tumour, representing only 1% to 2% of all exocrine pancreatic tumours. Pancreatic-type acinar cell carcinoma can occur in other organs, including the stomach, but it is extraordinarily rare. We report a case of a 51-year-old woman with a pancreatic-type pure acinar carcinoma of the stomach coexisting with a large cell B lymphoma synchronously, and a literature review of gastric carcinomas with pancreatic cell differentiation. At present there is a preoperative underdiagnosis of these tumours that could be minimized by including this entity in the differential diagnosis of gastric cancer and by performing immunohistochemical analysis with neuroendocrine markers and exocrine pancreatic enzymes.

Modification of Corneal Biomechanics and Intraocular Pressure Following Non-Penetrating Deep Sclerectomy.

Changes in the cornea can influence outcomes in patients with primary open-angle glaucoma (POAG). We aimed to evaluate the relevance of changes in corneal biomechanics and intraocular pressure (IOP) in patients undergoing non-penetrating deep sclerectomy (NPDS) with the Esnoper V2000 implant® (AJL Ophthalmic S.A., Gasteiz, Spain). We included 42 eyes of 42 patients with POAG scheduled for NPDS with the Esnoper V2000 implant. Biomechanical properties were measured by Ocular Response Analyzer® G3 (ORA; Reichert Inc., Depew, NY, USA). Corneal hysteresis (CH), corneal resistance factor (CRF), corneal compensated IOP (IOPcc), and Goldmann-correlated IOP (IOPg) were measured the day before surgery and on day 1, 7, and 30 and 2 and 3 months after surgery. CH initially increased, fell below the presurgical value at 30 days after the surgery, and increased again at 2 and 3 months. CRF, IOPcc, and IOPg decreased on the first day after surgery, then followed a trend of increasing but stayed below pre-surgery levels. All values reached statistical significance. While observed changes in corneal biomechanics after NPDS and Esnoper V2000 implant were significant, more studies are needed if we are to understand their influence on corneal biomechanics and their clinical relevance in POAG.

Low-dose sirolimus combined with angiotensin-converting enzyme inhibitor and statin stabilizes renal function and reduces glomerular proliferation in poor prognosis IgA nephropathy.

BACKGROUND: There is a lack of new therapeutic strategies for IgA nephropathy. Low-dose sirolimus inhibits mesangial cell proliferation and renal fibrosis in animal models. METHODS: We performed a pilot, randomized controlled trial to evaluate the efficacy and safety of low-dose sirolimus in patients with a high-risk IgA nephropathy. Twenty-three patients with a glomerular filtration rate (GFR) within 30-60 mL/min and/or proteinuria >1 g/day were randomly assigned to low-dose sirolimus plus enalapril and atorvastatin (SRL group, n = 14) or enalapril plus atorvastatin (CONTROL group, n = 9). Primary composite end point was variation of haematuria, proteinuria and blood pressure. Secondary end points were isotopic GFR, renal histology evaluated by Oxford classification and safety parameters evaluated at 6 and 12 months. RESULTS: Primary end point improved significantly in the SRL group at 12 months. Regarding isotopic GFR, patients included in the CONTROL group lost 8 mL/min/1.73 m(2), whereas those in the SRL arm improved 5 mL/min/1.73 m(2) (P = 0.03). Proteinuria decreased similarly in both study groups. At 1 year, SRL treatment was associated with a significant reduction of mesangial and endocapillary proliferation, whereas glomerular sclerosis, tubular atrophy and interstitial fibrosis were similar. Sirolimus was well tolerated; all patients remained on therapy at 12 months. CONCLUSION: The addition of low-dose sirolimus to enalapril and statin is safe, stabilizes renal function and reduces glomerular proliferative lesions in patients with poor prognosis IgA nephropathy.

Broad spectrum of Fabry disease manifestation in an extended Spanish family with a new deletion in the GLA gene.

BACKGROUND: Fabry disease (FD) is an X-linked inherited disease based on the absence or reduction of lysosomal-galactosidase (Gla) activity. The enzymatic defect results in progressive impairment of cerebrovascular, renal and cardiac function. Normally, female heterozygote mutation carriers are less strongly affected than male hemizygotes aggravating disease diagnosis. METHOD: Close examination of the patients by renal biopsy, echo- and electrocardiography and MRI. Blood work and subsequent DNA analysis were carried out utilizing approved protocols for PCR and Sequencing. MLPA analysis was done to unveil deletions within the GLA gene locus. Quantitative detection of Glycolipids in patient plasma and urine were carried out using HPLC/MS-MS and ESI-MS. RESULTS: In the presented case, a female index patient led to the examination of three generations of a Spanish family. She presented with severe oto-cochlear symptoms and covert renal and cardiac involvement. While conventional sequencing failed to detect a causative mutation, MLPA analysis revealed a deletion within the GLA gene locus, which we were able to map to a region spanning exon 2 and adjacent intronic parts. The analysis of different biomarkers revealed elevated lyso-Gb3 levels in all affected family members. CONCLUSION: Our findings highlight the broad intrafamilial spectrum of symptoms of FD and emphasise the need to use MLPA screening in symptomatic females without conclusive sequencing result. Finally, plasma lyso-Gb3 proved to be a reliable biomarker for the diagnosis of FD.

Renal involvement as the first manifestation of hypereosinophilic syndrome: a case report.

Idiopathic hypereosinophilic syndrome is characterized by elevated and prolonged blood eosinophilia along with organ system involvement and malfunction. The heart is the most frequently involved organ, and renal participation is extremely rare. Herein, we report on a case of idiopathic hypereosinophilic syndrome with renal involvement as the first manifestation.