Discovery of novel predisposing coding and noncoding variants in familial Hodgkin lymphoma.

Familial aggregation of Hodgkin lymphoma (HL) has been demonstrated in large population studies, pointing to genetic predisposition to this hematological malignancy. To understand the genetic variants associated with the development of HL, we performed whole genome sequencing on 234 individuals with and without HL from 36 pedigrees that had 2 or more first-degree relatives with HL. Our pedigree selection criteria also required at least 1 affected individual aged <21 years, with the median age at diagnosis of 21.

Ross-Konno Operation in an Infant With a Quadricuspid Pulmonary Valve and Anomalous Aortic Origin of the Right Coronary Artery.

Anomalous aortic origin of the right coronary artery from the left aortic sinus is a rare congenital anomaly that is generally repaired during adolescence when the condition is associated with symptoms. It is rarely diagnosed in infancy. Similarly, a quadricuspid pulmonary valve is also a rare finding, and there are scant data to evaluate whether this malformation of the pulmonary valve is suitable to be used for a Ross operation.

Sepsis management and the pediatric surgeon.

Sepsis is a life-threatening condition that requires aggressive, timely, and multi-disciplinary care. Understanding the changes in national guidelines regarding definitions, diagnosis and the management of pediatric sepsis is critical for the pediatric surgeon participating in the care of these patients. The purpose of this article is to review the essential steps for the timely management of pediatric sepsis, including fluid resuscitation, antibiotics, vasopressors, and steroids.

Vaginal Anatomy on MRI: New Information Obtained Using Distention.

Objectives: To demonstrate and confirm the presence of three anatomic zones of the vagina (a superficial sphincteric zone; a central wedge shaped transition zone; and a deep, expanded forniceal zone) using pelvic magnetic resonance imaging with contrast distention of the vagina.

Methods: A total of 107 consecutive female pelvic magnetic resonance imaging scans using vaginal contrast distention were retrospectively reviewed. The images were observed for the three-zone configuration.

Laparoscopic Surgical Algorithm to Triage the Timing of Tumor Reductive Surgery in Advanced Ovarian Cancer.

Objective: To estimate the effects of a laparoscopic scoring algorithm to triage patients with advanced ovarian cancer to immediate or delayed debulking to improve complete gross surgical resection rates and determine the resulting clinical outcomes.

Methods: We prospectively performed laparoscopic assessment on patients with suspected advanced-stage ovarian cancer from April 2013 to December 2016 to determine primary resectability at tumor reductive surgery. Patients with medically inoperable or distant metastatic disease received neoadjuvant chemotherapy.

Effect of an Enhanced Recovery After Surgery Program on Opioid Use and Patient-Reported Outcomes.

Objective: To investigate the effect of an enhanced recovery after surgery (ERAS) program on perioperative outcomes with an emphasis on opioid consumption and patient-reported outcomes in the immediate and extended postoperative periods.

Methods: We initiated our ERAS program as part of a quality improvement initiative in November 2014. We compared clinical outcomes among a cohort of 607 women undergoing open gynecologic surgery before or after implementation of ERAS.

Central neurogenic hyperventilation and renal tubular acidosis in children with pontine gliomas.

Central neurogenic hyperventilation (CNH) is a rare neurologic condition that develops secondary to brainstem involvement. Approximately 35 noncomatose patients with CNH have been reported, including 7 with either a diffuse (patients 2–10 in the table) or focal (patient 1 in the table) brainstem glioma. Although diffuse intrinsic pontine glioma (DIPG) is the most common pediatric brainstem cancer, only 1 case report described a child with DIPG who experienced CNH.