Does Disruption of Optic Atrophy-1 (OPA1) Contribute to Cell Death in HL-1 Cardiomyocytes Subjected to Lethal Ischemia-Reperfusion Injury?

Disruption of mitochondrial structure/function is well-recognized to be a determinant of cell death in cardiomyocytes subjected to lethal episodes of ischemia-reperfusion (IR). However, the precise mitochondrial event(s) that precipitate lethal IR injury remain incompletely resolved. Using the in vitro HL-1 cardiomyocyte model, our aims were to establish whether: (1) proteolytic processing of optic atrophy protein-1 (OPA1), the inner mitochondrial membrane protein responsible for maintaining cristae junction integrity, plays a causal, mechanistic role in determining cardiomyocyte fate in cells subjected to lethal IR injury; and (2) preservation of OPA1 may contribute to the well-documented cardioprotection achieved with ischemic preconditioning (IPC) and remote ischemic conditioning.

Bortezomib treatment of steroid-refractory Evans syndrome in children.

Treatment of refractory Evans syndrome (ES) remains a challenge in hematology practice. Due to rarity of this condition, evidence-based approaches are limited and often treatment choices stem from small case series or anecdotal experiences. There is mounting evidence that some patients have genetic defects that could be targeted with promising preliminary results.

Migration of a ventriculo-peritoneal shunt catheter into a back incision of a patient with previous spinal fusion.

Introduction: This case examines a unique, longitudinal presentation of an abandoned, migrating VP shunt which presents as multiple complications, including a weeping abscess in the patients back. We believe that the latter complication was potentially caused by the wound from the patient's previous history of spinal fusion surgery.

Case Presentation: The patient presents with an associated type 2 Chiari malformation, hydrocephalus, and a previous history of posterior spinal fusion (T4-L5 anterior fusion and T2-L5 posterior fusion) at age 11.

Avoiding the pocket: A case report of coiling of distal shunt catheter into subcutaneous pocket.

Introduction: Hydrocephalus is one of the most common disorders of neurosurgery and ventricular shunting, the primary surgical intervention, malfunctions in 85% of patients by 10 years.

Presentation Of Case: Here we present a case of a 12-year-old girl with history of a vagal nerve stimulator (VNS) and ventricular shunt, most recently revised from ventriculoatrial (VA) to ventriculoperitoneal (VP) shunt at an outside hospital. The patient presented with a new left chest bulge, nausea, emesis, and seizures.

Effect of Preoperative Molding Helmet in Patients With Sagittal Synostosis.

Background: In our practice, the authors found that molding helmet used for plagiocephaly preoperatively, in patients with sagittal synostosis, decreased bathrocephaly, forehead bossing, and improved posterior vertex, as well as Cephalic Index (CI). This prompted us to investigate the impact of preoperative molding helmet in patients with sagittal synostosis.

Methods: A prospective study was performed on patients undergoing surgical correction of sagittal synostosis, over a 5-year period.

Randomized placebo-controlled trial of sucrose analgesia on neonatal skin blood flow and pain response during heel lance.

Objectives: To evaluate the effect of oral sucrose on skin blood flow (SBF; perfusion units; PU) measured by Laser Doppler Imager (LDI) in term newborns and pain response (Neonatal Infant Pain Scale score; NIPS score) during heel lance; (2) determine SBF changes during heel lance; and (3) the relationship between SBF and NIPS.

Materials And Methods: Term infants ≤7 days old (n=56) undergoing routine heel lance were randomized to pretreatment with 2.0 mL oral 24% sucrose (n=29) or sterile water (n=27) in a double-blinded, placebo-controlled trial.

Unusual physical features and heat stroke presentation for hypohydrotic ectodermal dysplasia.

Hypohidrotic/ anhidrotic ectodermal dysplasia (HED) is characterized by hypotrichosis (sparseness of scalp and body hair), hypohidrosis (reduced ability to sweat), and hypodontia (decreased tooth development). Sweating is either absent or greatly deficient leading to an increased risk for hyperthermia. This is a relatively common genetic condition; it is estimated that 1 in approximately 5000–10 000 newborns has HED (Wright et al.