Influence of Age and Type 1 Diabetes Mellitus on Serological Test for Celiac Disease in Children.

Purpose: Serological tests of tissue transglutaminase (TTG) and deamidated gliadin (DGP) antibodies for celiac disease diagnosis show conflicting correlation with histology in young children and in type 1 diabetes mellitus (T1DM). Tests' ability to predict histology and cutoff values based on age and T1DM was evaluated.

Methods: A retrospective study of children who had celiac serological tests between 6/1/2002 and 12/31/2014 at a pediatric hospital.

Birth-related subdural hemorrhage: prevalence and imaging morphology.

Background: Birth trauma accounts for 1-2% of the mortality in newborns with significant intracranial injuries presenting in the immediate postnatal period. However, a significant number of asymptomatic neonates harbor birth-related intracranial hemorrhage (ICH), with birth-related subdural hemorrhage (SDH) being a common occurrence on infant brain CT and MRI studies performed as a standard of care for a variety of reasons. Although clinically insignificant, birth-related SDH is frequently brought up in courts as an alternative explanation for SDH in suspected abusive head trauma.

Excellent Outcome for Pediatric Patients With High-Risk Hodgkin Lymphoma Treated With Brentuximab Vedotin and Risk-Adapted Residual Node Radiation.

Purpose: Brentuximab vedotin, an effective anti-CD30 antibody-drug conjugate approved for use in adults with classical Hodgkin lymphoma (HL), was introduced in this frontline trial to reduce prescribed radiation in children and adolescents with classical HL.

Methods: Open-label, single-arm, multicenter trial for patients (age ≤ 18 years) with stage IIB, IIIB, or IV classical HL was conducted. Brentuximab vedotin replaced each vincristine in the OEPA/COPDac (vincristine, etoposide, prednisone, and doxorubicin/cyclophosphamide, vincristine, prednisone, and dacarbazine) regimen according to GPOH-HD2002 treatment group 3 (TG3); two cycles of AEPA and four cycles of CAPDac.

Machine Learning Predicts the 3D Outcomes of Adolescent Idiopathic Scoliosis Surgery Using Patient-Surgeon Specific Parameters.

Study Design: Retrospective descriptive, multicenter study.

Objective: The aim of this study was to predict the three-dimensional (3D) radiographic outcomes of the spinal surgery in a cohort of adolescent idiopathic scoliosis (AIS) as a function preoperative spinal parameters and surgeon modifiable factors.

Summary Of Background Data: Current guidelines for posterior spinal fusion surgery (PSF) in AIS patients are based on two-dimensional classification of the spinal curves.

A Diagnostic Dilemma of Antiglutamic Acid Decarboxylase 65 (Anti-GAD 65) and Mycoplasma Pneumoniae Antibodies in a Girl Presenting with Acute-Onset Obsessive-Compulsive Disorder.

Acute-onset obsessive-compulsive disorder can be challenging, especially when triggered by an underlying disease process. Clinicians often turn to Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS), but it is important to consider a broad differential in these patients. We present a case of a 9-year-old girl with acute-onset obsessive-compulsive behavior likely triggered by a post-infectious phenomenon that ultimately resolved following treatment with plasmapheresis.

Monitoring retinal pathology and cerebral injury in sickle cell disease using spectral-domain optical coherence tomography in pediatric patients.

Purpose: This study aimed to confirm the correlation between sickle cell disease (SCD) genotype and retinal damage identified by spectral-domain optical coherence tomography (SD-OCT), and examine a potential link between hypoxic ischemic injury in the retina and brain.

Methods: In this prospective, observational case series, 117 patients (56 males) aged 5-20 years with SCD (36 SC, 68 SS, eight Sβ+ thalassemia, five Sβ0 thalassemia) underwent ophthalmologic examination including funduscopy and SD-OCT imaging. Comparison of SCD genotypes and association between ocular findings and cerebrovascular disease (CVD) in subjects with SS/Sβ0 genotype were investigated.

Generalized Arterial Calcification of Infancy.

Generalized arterial calcification of infancy (GACI) is a rare genetic disease that affects the circulatory system and the large- and medium-sized arteries throughout the body. GACI usually occurs during fetal development. Babies with GACI are diagnosed early, generally soon after birth and in some cases before birth by fetal ultrasound.

Cytomegalovirus and Herpes Simplex Virus Co-Infection in an HIV-Negative Patient: A Case Report.

Herpes simplex virus (HSV) and cytomegalovirus (CMV) infections are commonly seen in immunocompromised patients, particularly in patients with HIV. However, fulminant CMV infection and concurrent infection with HSV and CMV in non-HIV patients are quite rare. We present the case of a 72-year-old HIV-negative man with a history of oropharyngeal carcinoma in remission and recent treatment of immune thrombocytopenic purpura with high-dose steroids who was transferred from an outside hospital for Ear Nose and Throat (ENT) evaluation of a non-healing buccal ulcer.

Association Between Gray Matter Volume Variations and Energy Utilization in the Brain: Implications for Developmental Stuttering.

Purpose The biological mechanisms underlying developmental stuttering remain unclear. In a previous investigation, we showed that there is significant spatial correspondence between regional gray matter structural anomalies and the expression of genes linked to energy metabolism. In the current study, we sought to further examine the relationship between structural anomalies in the brain in children with persistent stuttering and brain regional energy metabolism.

The impact of scoliosis surgery on pulmonary function in spinal muscular atrophy: a systematic review.

Scoliosis often occurs coincident with pulmonary function deterioration in spinal muscular atrophy but a causal relationship has not yet been reliably established. A systematic literature review was performed, with pulmonary function testing being the primary outcome pre- and post-scoliosis surgery. Levels of evidence were determined and GRADE recommendations made.

Skeletal Muscle in Cerebral Palsy: From Belly to Myofibril.

This review will provide a comprehensive, up-to-date review of the current knowledge regarding the pathophysiology of muscle contractures in cerebral palsy. Although much has been known about the clinical manifestations of both dynamic and static muscle contractures, until recently, little was known about the underlying mechanisms for the development of such contractures. In particular, recent basic science and imaging studies have reported an upregulation of collagen content associated with muscle stiffness.

A Longitudinal Comparison of Alternatives to Body Mass Index Z-Scores for Children with Very High Body Mass Indexes.

Objective: The current Centers for Disease Control and Prevention (CDC) body mass index (BMI) z-scores are inaccurate for BMIs of ≥97th percentile. We, therefore, considered 5 alternatives that can be used across the entire BMI distribution: modified BMI-for-age z-score (BMIz), BMI expressed as a percentage of the 95th percentile (%CDC95th percentile), extended BMIz, BMI expressed as a percentage of the median (%median), and %median adjusted for the dispersion of BMIs.

Study Design: We illustrate the behavior of the metrics among children of different ages and BMIs.

Serum Biomarkers of Regeneration and Plasticity are Associated with Functional Outcome in Pediatric Neurocritical Illness: An Exploratory Study.

Background/objective: Pediatric neurocritical care survivorship is frequently accompanied by functional impairments. Lack of prognostic biomarkers is a barrier to early identification and management of impairment. We explored the association between blood biomarkers and functional impairment in children with acute acquired brain injury.

Specific sagittal alignment patterns are already present in mild adolescent idiopathic scoliosis.

Purpose: The complex three-dimensional spinal deformity in AIS consists of rotated, lordotic apical areas and neutral junctional zones that modify the spine's sagittal profile. Recently, three specific patterns of thoracic sagittal 'malalignment' were described for severe AIS. The aim of this study is to define whether specific patterns of pathological sagittal alignment are already present in mild AIS.

Tracheal narrowing in children and adults with mucopolysaccharidosis type IVA: evaluation with computed tomography angiography.

Background: Mucopolysaccharidosis type IVA (MPS IVA) is characterized by progressive skeletal dysplasia and respiratory issues with difficult airway management during anesthesia.

Objective: To characterize tracheal abnormalities in children and adults with MPS IVA including interplay of the trachea, vasculature, bones and thyroid at the thoracic inlet.

Materials And Methods: Computed tomography (CT) angiograms of the chest were analyzed for trachea shape, narrowing and deviation at the thoracic inlet, course of vasculature, bone alignment and thyroid location.

The value-added benefit of utilizing two attending surgeons for patients with scoliosis secondary to cerebral palsy.

Purpose: The use of two attending surgeons during posterior spinal fusion (PSF) for cerebral palsy (CP) patients has been shown to improve perioperative outcomes. This study aims to determine if the use of two surgeons is associated with an increase in the number of subsequent surgeries that can be performed in the same operating room (OR) during business hours.

Methods: Patients with scoliosis and CP treated with PSF with minimum 90-day follow-up were included.

Epidemiology of Mucopolysaccharidoses Update.

Mucopolysaccharidoses (MPS) are a group of lysosomal storage disorders caused by a lysosomal enzyme deficiency or malfunction, which leads to the accumulation of glycosaminoglycans in tissues and organs. If not treated at an early stage, patients have various health problems, affecting their quality of life and life-span. Two therapeutic options for MPS are widely used in practice: enzyme replacement therapy and hematopoietic stem cell transplantation.

The Impact of Spinal Fusion on Hip Displacement in Cerebral Palsy.

Background And Study Aims: The aims of this study were to determine the risk of progressive hip subluxation in children with CP after spinal fusion for scoliosis and how frequent the hips follow-up should be scheduled.

Patients And Methods: Pelvis radiography [migration index (MI) and pelvic obliquity (PO)] of Gross Motor Function Classification System (GMFCS) levels IV and V children with CP who received spinal fusion and pelvic fixation were reviewed retrospectively. This population was categorized into three groups based on the MI at spinal fusion: G1 = 0-29%; G2 = 30-59%; and G3 = 60-100%.

Engaging Frontline Providers Prevents Hypothermia and Improves Communication in the Postoperative Neonate.

Background: Neonates undergoing surgery are at high risk for perioperative hypothermia. Hypothermia has been associated with increased adverse events. Transfer of care from the operating room (OR) to the neonatal intensive care unit (NICU) adds another layer of risk for this population introducing the potential for miscommunication leading to preventable adverse events.

Association of Positional Plagiocephaly and Developmental Delay Within a Primary Care Network.

Objective: Previous research has suggested an association between plagiocephaly and developmental delay. However, study samples drawn from children seen in subspecialty clinics increase the potential for selection and referral bias. Our study evaluates the association between plagiocephaly and developmental delay and the timing of these diagnoses in a primary care setting, where plagiocephaly is commonly diagnosed and managed.