Pediatric TAFRO syndrome: A multi-institution case series illustrating clinical challenges and excellent outcomes.

A rare lymphoproliferative disorder involving thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), renal dysfunction (R), and organomegaly (O), called TAFRO syndrome, was first reported in 2010. Considered a variant of idiopathic multicentric Castleman's disease, the recent discovery and rarity of this syndrome pose challenges to diagnosis and management. Herein, we review three pediatric cases, including an infant, that illustrate the heterogeneity of TAFRO syndrome.

Fetal and Neonatal Autopsy in the Molecular Age: Exploring Tissue Selection for Testing Success.

While conventional autopsy is the gold-standard for determining cause of demise in the fetal and neonatal population, molecular analysis is increasingly used as an ancillary tool. Testing methods and tissue selection should be optimized to provide informative genetic results. This institutional review compares testing modalities and postmortem tissue type in 53 demises occurring between 20 weeks of gestation and 28 days of life.

The importance of fetal autopsy: An institutional review and development of best practices for reporting size and estimating gestational age at demise.

Objectives: Fetal and neonatal autopsy offers critical insight into disease processes and clinical decision-making in reproductive medicine. Elucidating the cause of death and gaining a deeper understanding of the entities leading to fetal demise aids in anticipatory guidance for physicians and patients. Accurate assessment of growth and dating of fetuses is an important aspect of classifying pathology in the fetal and neonatal population.

Long-Term Effect of Germline Mutation on Pulmonary Clinico-Histopathologic Phenotype.

Tbx4 protein, expressed in mesenchyme of the developing lung, contributes to airway branching and distal lung growth. An association between pediatric onset of pulmonary arterial hypertension (PAH) and genetic variations coding for the T-box transcription factor 4 gene () has been increasingly recognized. Tbx4-related PAH onset has a bimodal age distribution, including severe to lethal PAH in newborns and later onset PAH.

Small cell carcinoma of the ovary hypercalcemic type (SCCOHT): A review and novel case with dual germline SMARCA4 and BRCA2 mutations.

Small cell carcinoma of the ovary hypercalcemic type (SCCOHT) is a rare and aggressive disease. While classically linked to mutations in we describe a case in a patient with both and germline mutations. We describe her disease presentation, histopathology and treatment with adjuvant systemic chemotherapy, interval hyperthermic intraperitoneal chemotherapy, high dose chemotherapy with stem cell rescue, and maintenance with a poly-ADP-ribose polymerase inhibitor (PARPi).

The effect of low-intensity pulsed ultrasound on autologous osteochondral plugs in a canine model.

Background: Low-intensity pulsed ultrasound promotes the enchondral portion of fracture healing, which has a direct stimulatory effect on cartilage formation and maturation.

Hypothesis: Daily ultrasound treatment positively affects the repair and incorporation of modified autologous osteochondral plugs in a canine model.

Study Design: Controlled laboratory study.