Impact of Hemoglobin Level in Ex Vivo Heart Perfusion on Donation After Circulatory Death Hearts: A Juvenile Porcine Experimental Model.

Background: Ex vivo heart perfusion (EVHP) of donation after circulatory death (DCD) hearts has become an effective strategy in adults; however, the small circulating volume in pediatrics poses the challenge of a low-hemoglobin (Hb) perfusate. We aimed to determine the impact of perfusate Hb levels during EVHP on DCD hearts using a juvenile porcine model.

Methods: Sixteen DCD piglet hearts (11-14 kg) were reperfused for 4 h in unloaded mode followed by working mode.

Short Cilia, Immunodeficiency, and Cystic Fibrosis in a Mother-Daughter Pair.

Patients with chronic rhinosinusitis (CRS) that is refractory to maximal medical and surgical therapy should be evaluated for other primary conditions. Cystic fibrosis (CF), primary immunodeficiency (PID), and primary ciliary dyskinesia (PCD) are potential risk factors for refractory CRS. These conditions present with variable disease severity and diagnosis may be delayed into adulthood.

A Pediatric Primary Cardiac Spindle Cell Neoplasm With a Rare PDGFRA::USP8 Gene Fusion: A Case Report.

We report a case of a primary cardiac spindle cell neoplasm with concerning histological features and a rare gene fusion in a 3 year old boy. The patient presented with a large cardiac mass predominantly in the right ventricle, originating from the ventricular septum. The mass was resected with grossly negative margins.

A Canadian children's hospital's experience with cystic echinococcosis over 30 years: A case series.

Background: Cystic echinococcosis (CE) or hydatid disease caused by the cestode is an uncommon infection in Canada especially among children. There are limited reports describing the clinical presentation and management in Canadian children.

Methods: The medical records of all children diagnosed with CE at a quaternary paediatric centre in Ontario between January 1988 and August 2021 were retrospectively reviewed.

L-citrulline attenuates lipopolysaccharide-induced inflammatory lung injury in neonatal rats.

Background: Prenatal or postnatal lung inflammation and oxidative stress disrupt alveolo-vascular development leading to bronchopulmonary dysplasia (BPD) with and without pulmonary hypertension. L-citrulline (L-CIT), a nonessential amino acid, alleviates inflammatory and hyperoxic lung injury in preclinical models of BPD. L-CIT modulates signaling pathways mediating inflammation, oxidative stress, and mitochondrial biogenesis-processes operative in the development of BPD.

Cardioprotective Actions of a Glucagon-like Peptide-1 Receptor Agonist on Hearts Donated After Circulatory Death.

Background Heart transplantation with a donation after circulatory death (DCD) heart is complicated by substantial organ ischemia and ischemia-reperfusion injury. Exenatide, a glucagon-like peptide-1 receptor agonist, manifests protection against cardiac ischemia-reperfusion injury in other settings. Here we evaluate the effects of exenatide on DCD hearts in juvenile pigs.

How I approach: The workup and management of patients with progressive transformation of the germinal center.

Progressive transformation of the germinal center (PTGC) is a common and underrecognized cause of pediatric lymphadenopathy. PTGC may be associated with numerous systemic medical conditions that require further workup and management, including malignancy, autoimmune conditions, lymphoproliferative conditions, immunodeficiency, and infection. Given the breadth and rarity of the associated conditions, workup should be tailored to the individual patient and occur in a tiered approach.

Fatal Pediatric Streptococcal Infection: A Clinico-Pathological Study.

Purpose And Context: Streptococcal Infection (SI) is an important cause of pediatric death in children, yet limited reports exist on autopsy findings in fatal SI cases.

Method: Case records (1997-2019) of SI with no pre-existing risk factors were reviewed and selected. Their clinical and pathological findings in the autopsy reports were analyzed.

[Cronkhite–Canada syndrome].

Összefoglaló. A Cronkhite-Canada-szindróma egy extrém ritka, nem öröklődő, gyomor-bél rendszeri polyposissal, fehérjevesztő enteropathiával és ectodermalis elváltozásokkal járó megbetegedés. A világon eddig összesen körülbelül 500 esetet jegyeztek fel.

Round Cell Sarcomas: Newcomers and Diagnostic Approaches.

Undifferentiated sarcomas of soft tissue and bone have been defined as tumors with no identifiable morphologic, immunohistochemical, or molecular features indicating tumor cell origin. In young patients, these tumors frequently have a round or spindle cell morphology. Recently described recurrent translocations within this category have led to the recognition of new molecular subtypes of round cell sarcomas, and several of them have a more aggressive clinical course and less chemosensitivity.

Atypical malignancies after intestinal transplantation in children: A European single-centre experience.

Background: Post-transplant malignancies, that is, lymphomas, are a recognized complication in intestinal transplant recipients but are mostly secondary to EBV infection. There is an increased risk for malignancies in unusual sites in intestinal transplant recipients as compared to other solid organ transplants and the general population.

Objective: To evaluate the incidence, course, and outcome of unusual malignancies in children after ITx.

Plaque-like Myofibroblastic Tumor: 2 Cases of This Unusual Dermal Tumor Which Occurs in Infancy and Early Childhood.

Plaque-like myofibroblastic tumor (PLMT) is a rare dermal spindle cell tumor which occurs in infancy or childhood within the first 4 years of life. The tumor is often pruritic and mostly presents on the lower back. We describe 2 cases with characteristic clinical and histological features of this entity, thus adding to the 10 cases which have so far been reported.

Congenital cutaneous lymphadenoma.

Cutaneous lymphadenoma is an uncommon benign neoplasm often considered to be an adamantinoid variant of trichoblastoma. Lesions present in both sexes, between 14 and 87 years of age, and are mainly located on the head and neck. Cases in children are rare and there is only 1 previous case of a congenital lymphadenoma.

A microscopically calcified Meckel's diverticulum: a histopathological perspective of a case of both gastric and pancreatic mucosae.

A 2-year-old child presented with a 1-week history of abdominal pain and non-bilious vomiting. Upon examination, the abdomen was distended and faecal aspirate was noted from a nasogastric tube. Ultrasound scan and a failed air enema demonstrated intestinal intussusception warranting a surgical intervention.

Clinical significance of eosinophilia and chronic inflammatory infiltrate in children's rectal biopsies.

Aims: The aim of the present study was to determine the clinical significance of an incidental finding of eosinophilia (EOS) and chronic inflammatory infiltrate (CINF) in rectal biopsies of children investigated for suspected Hirschsprung disease (HSCR).

Methods: A retrospective study (2000-2010) of children incidentally found to have EOS and CINF was performed. HSCR cases were excluded.

Meningeal and brainstem infiltration by a malignant mesothelioma.

Malignant mesothelioma is an uncommon neoplasia which primarily involves the pleura or peritoneum. Central nervous system involvement is rare. A rare presentation of metastatic pleural mesothelioma, which had infiltrated the meninges and brainstem, is described.