Not only a small liver - The pathologist's perspective in the pediatric liver transplant setting.

Pediatric liver transplantation represents a safe and long-lasting treatment option for various disease types, requiring the pathologist's input. Indeed, an accurate and timely diagnosis is crucial in reporting and grading native liver diseases, evaluating donor liver eligibility and identifying signs of organ injury in the post-transplant follow-up. However, as the procedure is more frequently and widely performed, deceptive and unexplored histopathologic features have emerged with relevant consequences on patient management, particularly when dealing with long-term treatment and weaning of immunosuppression.In this complex and challenging scenario, this review aims to depict the most relevant histopathologic conditions which could be encountered in pediatric liver transplantation. We will tackle the conditions representing the main indications for transplantation in childhood as well as the complications burdening the post-transplant phases, either immunologically (i.e., rejection) or non-immunologically mediated. Lastly, we hope to provide concise, yet significant, suggestions related to innovative pathology techniques in pediatric liver transplantation.

[Management of the adult with sickle cell disease]. / Prise en charge de l'adulte drépanocytaire.

Sickle cell disease is a chronic disease with multisystemic complications. Follow-up requires specialised and multidisciplinary care. The consultation allows for the screening of complications.

[Pregnancy planning and follow-up of a pregnant woman with sickle cell disease]. / Projet de grossesse et suivi d'une femme enceinte porteuse de la drépanocytose.

Pregnancy is a high-risk situation in sickle cell patients, both for the mother and the foetus. It considerably increases the risk of an acute complication (vaso-occlusive crisis, acute chest syndrome, infection, thrombosis) of sickle cell disease. In addition, this condition increases the risk of placental vascular complications (in utero growth retardation, pre-eclampsia, retroplacental haematoma and in utero foetal death).

Left atrial appendage perforation during appendage angiography treated by percutaneous left atrial appendage closure: a case report.

BACKGROUND: Pericardial effusion is a common complication of percutaneous left atrial appendage (LAA) closure. Acute management is the cornerstone of pericardial effusion treatment and interrupting the intervention is often required. CASE SUMMARY: A 65-year-old man presented an acute 10 mm pericardial effusion following pigtail contrast appendage injection. A rapid Watchman Flex 24 mm (Boston Scientific) deployment permitted bleeding interruption. A needle pericardiocentesis was achieved in order to prevent any haemodynamical instability. DISCUSSION: This case report describes an atypical cause of pericardial effusion and a technique for bleeding control with LAA closure device deployment.

Massive retroperitoneal hematoma as an acute complication of retrograde intrarenal surgery: A case report.

BACKGROUND: Retrograde intrarenal surgery (RIRS) has been proven to be a safe and effective treatment modality in large-scale quantitative studies. However, although its safety profile has been established, it also has a potential risk of life-threatening complications. We here describe our experience with a patient who developed a huge periureteral hematoma after RIRS with holmium laser lithotripsy. CASE SUMMARY: A 73-year-old woman visited our center with a complaint of gross hematuria. An enhanced computed tomography (CT) scan revealed a 1.5-cm left renal pelvis stone with hydronephrosis. The patient underwent RIRS. During the surgery, a 12/14-Fr ureteral access sheath was applied and a 6-Fr ureteral catheter was indwelled thereafter. On postoperative day 1, she experienced aggravated left flank pain and left lower-quadrant tenderness without rebound tenderness. A follow-up CT scan was taken, which revealed a huge hematoma in the periureteral space, not in the perirenal space, with suspicious contrast medium extravasation. Immediate angiography was performed; however, it showed no evidence of active bleeding. She was conservatively managed with hydration and antibiotic and nonsteroidal anti-inflammatory drug therapy, and was discharged on postoperative day 7. However, she visited our outpatient department with recurrent left flank pain at 5 d from discharge. Ultrasonography confirmed that the double J-stent was intact. To rule out stent malfunction, the stent was changed. Decreased size of the hematoma was observed in the imaging studies, and conservative management for candiduria was performed for 1 wk. CONCLUSION: Although RIRS is an effective and safe procedure for the management of renal stones, clinicians should be aware of its potential complications.

Effects of Genotypes and Treatment on Oxygenscan Parameters in Sickle Cell Disease.

(1) Background: The aim of the present study was to compare oxygen gradient ektacytometry parameters between sickle cell patients of different genotypes (SS, SC, and S/ß+) or under different treatments (hydroxyurea or chronic red blood cell exchange). (2) Methods: Oxygen gradient ektacytometry was performed in 167 adults and children at steady state. In addition, five SS patients had oxygenscan measurements at steady state and during an acute complication requiring hospitalization. (3) Results: Red blood cell (RBC) deformability upon deoxygenation (EImin) and in normoxia (EImax) was increased, and the susceptibility of RBC to sickle upon deoxygenation was decreased in SC patients when compared to untreated SS patients older than 5 years old. SS patients under chronic red blood cell exchange had higher EImin and EImax and lower susceptibility of RBC to sickle upon deoxygenation compared to untreated SS patients, SS patients younger than 5 years old, and hydroxyurea-treated SS and SC patients. The susceptibility of RBC to sickle upon deoxygenation was increased in the five SS patients during acute complication compared to steady state, although the difference between steady state and acute complication was variable from one patient to another. (4) Conclusions: The present study demonstrates that oxygen gradient ektacytometry parameters are affected by sickle cell disease (SCD) genotype and treatment.

Immediate aortic dissection after transcatheter aortic valve replacement: A case report and review of the literature.

-Aortic dissection of descending aorta was detected by intraoperative TEE in a case of 67-year-old man with symptomatic severe aortic stenosis after TAVR. -Transesophageal echocardiogram after TAVR procedure is helpful to detect this rare complication.

Multicentre analysis of hyperglycaemic hyperosmolar state and diabetic ketoacidosis in type 1 and type 2 diabetes.

AIMS: To compare diabetes patients with hyperglycaemic hyperosmolar state (HHS), diabetic ketoacidosis (DKA), and patients without decompensation (ND). METHODS: In total, 500,973 patients with type 1 or type 2 diabetes of all ages registered in the diabetes patient follow-up (DPV) were included. Analysis was stratified by age (≤ / > 20 years) and by manifestation/follow-up. Patients were categorized into three groups: HHS or DKA-during follow-up according to the most recent episode-or ND. RESULTS: At onset of diabetes, HHS criteria were met by 345 (68.4% T1D) and DKA by 9824 (97.6% T1D) patients. DKA patients had a lower BMI(-SDS) in both diabetes types compared to ND. HbA1c was higher in HHS/DKA. During follow-up, HHS occurred in 1451 (42.2% T1D) and DKA in 8389 patients (76.7% T1D). In paediatric T1D, HHS/DKA was associated with younger age, depression, and dyslipidemia. Pump usage was less frequent in DKA patients. In adult T1D/T2D subjects, metabolic control was worse in patients with HHS/DKA. HHS and DKA were also associated with excessive alcohol intake, dementia, stroke, chronic kidney disease, and depression. CONCLUSIONS: HHS/DKA occurred mostly in T1D and younger patients. However, both also occurred in T2D, which is of great importance in the treatment of diabetes. Better education programmes are necessary to prevent decompensation and comorbidities.

Ventricular Septal Defect Complicating ST-Elevation Myocardial Infarctions: A Call for Action.

BACKGROUND: Ventricular septal defect is a lethal complication after an acute myocardial infarction, which has become infrequent with the advent of reperfusion strategies; however, it remains a major contributor to mortality. METHODS: We identified patients using the International Classification of Diseases, 9th Revision, Clinical Modification procedure codes from the Nationwide Inpatient Sample between the years 2001 and 2013. A multivariate hierarchical logistic regression model was used to identify significant predictors of in-hospital mortality. RESULTS: We identified 3,373,206 ST-elevation myocardial infarctions, out of which 10,012 (0.3%) were complicated with ventricular septal defects. Most of the patients (60%) were older than 65 years, male (55%), and white (63%). Inferior (49.7%) and anterior (41.1%) myocardial infarctions were more commonly implicated with the development of ventricular septal defects. The median (interquartile range) hospitalization length was 7 (3.0-13.5) days. Only 7.65% of patients underwent some intervention, with 7% surgical and 0.65% minimally invasive. Mechanical support devices were used in 36.5% of patients, with intra-aortic balloon pump (96%) being the most common. In-hospital mortality remained high at 30.5% (downward trending from 41.6% in 2001 to 23.3% in 2013). Age, cardiogenic shock, and in-hospital cardiac arrest were statistically significant predictors of in-hospital mortality. The utilization of corrective procedures significantly declined. The use of mechanical support devices and performing a corrective procedure were associated with higher mortality, length of stay, and cost. CONCLUSIONS: Ventricular septal defects after acute myocardial infarctions remain associated with significantly high mortality rates. Highly specialized regional centers with individual expertise in the management of septal ruptures are required to improve outcomes of these patients.