Multidetector CT diagnosis of massive hemobilia due to gallbladder polyposis in a child with metachromatic leukodystrophy.

Hemobilia secondary to gallbladder polyposis is rare in children but has been reported in a few children with metachromatic leukodystrophy. We present a case with preoperative multidetector computed tomography (MDCT) diagnosis of massive hemobilia caused by gallbladder polyposis in a patient with metachromatic leukodystrophy. Our report highlights the importance of both awareness of the association of gallbladder polyposis with other syndromes such as metachromatic leukodystrophy as well as the possibility of this entity presenting with life-threatening bleeding.

Neuroenteric Staining as a Tool in the Evaluation of Pediatric Motility Disorders.

The diagnosis of enteric neuromuscular disorders has come a long way since the first description of an enteric neuropathic disorder by the Danish physician Harald Hirschsprung in 1886. Advances in specialized enteric histopathological staining techniques have made it possible to identify subtle neuropathies and myopathies that cause intestinal motility disorders, from the common and now better understood and relatively easily diagnosed Hirschsprung's disease to the less common and more severe and not well-characterized chronic idiopathic intestinal pseudoobstruction, which continues to present a diagnostic challenge to the gastroenterologist and histopathologist alike. This article will discuss the common gastrointestinal motility disorders and some of the specialized histological stains, such as the relatively common enzyme stain, acetylcholinesterase, used to diagnose Hirschsprung's disease; advanced tinctorial stains, such as Masson trichrome, which may aid in diagnosis of enteric myopathies causing pseudoobstruction; and immunohistochemical stains such as C-Kit or PG 9.

Corrigendum to "High Dose Dexmedetomidine: Effective as a Sole Agent Sedation for Children Undergoing MRI".

[This corrects the article DOI: 10.1155/2015/397372.].

Screening and Prophylaxis for Varices in Children with Liver Disease.

Esophageal varices in children with portal hypertension are quite common. Bleeding from these varices frequently occurs. Prophylactic measures to prevent such bleeding can be undertaken either before ("primary," prompted by a screening endoscopy) or after ("secondary") an initial variceal bleed.

Treatment of Adolescent Substance Use Disorders and Co-Occurring Internalizing Disorders: A Critical Review and Proposed Model.

Background: The past several decades have seen dramatic growth in empirically supported treatments for adolescent substance use disorders (SUDs), yet even the most well-established approaches struggle to produce large or long-lasting improvements. These difficulties may stem, in part, from the high rates of comorbidity between SUDs and other psychiatric disorders.

Method: We critically reviewed the treatment outcome literature for adolescents with co-occurring SUDs and internalizing disorders.

A comparison of the Monti and spiral Monti procedures: A long-term analysis.

Introduction/background: The Monti ileovesicostomy provides an excellent substitution for an appendicovesicostomy when the appendix is unavailable or suitable for use. The spiral Monti is a useful modification to the traditional Monti as it allows creation of a longer channel when needed. In 2007, the short-term outcomes were reported; they compared traditional and spiral Monti in 188 patients with an average follow-up of 43 months.

Spitzoid melanoma of childhood: a case series and review.

Spitzoid melanomas (SM) and atypical Spitz tumors (AST) are rare pediatric neoplasms. We performed a retrospective, single-institution review and report our institutional experience. We identified 10 patients (median age: 12.

Surgical treatment of the brow and upper eyelid.

Surgical management of the aging upper face has taken on a critical role in total facial rejuvenation, with a variety of techniques available. The hallmarks of the aging upper third of the face and periorbital region most commonly manifest as rhytids, brow descent, prolapse of periorbital fat, dermatochalasis, and volume loss and hollowing. The surrounding structures should be assessed individually and their relationships carefully analyzed to guide selection of the appropriate treatment.

Central Precocious Puberty: Update on Diagnosis and Treatment.

Central precocious puberty (CPP) is characterized by the same biochemical and physical features as normally timed puberty but occurs at an abnormally early age. Most cases of CPP are seen in girls, in whom it is usually idiopathic. In contrast, ~50% of boys with CPP have an identifiable cause.

Ultrasound classification of solitary renal cysts in children.

Introduction: Solitary renal cysts are typically incidentally found in children who have undergone renal ultrasound (US). The main concern is a cystic tumor. There is no US-based grading system for children to guide management.

Clinical significance of monocyte heterogeneity.

Monocytes are primitive hematopoietic cells that primarily arise from the bone marrow, circulate in the peripheral blood and give rise to differentiated macrophages. Over the past two decades, considerable attention to monocyte diversity and macrophage polarization has provided contextual clues into the role of myelomonocytic derivatives in human disease. Until recently, human monocytes were subdivided based on expression of the surface marker CD16.

Analysis of submitted abstracts for the American Academy of Pediatrics, Section on Surgery, National Conference (AAP SoSU).

Purpose: In this study, we characterized the topics and type of research performed for abstracts submitted to the AAP SoSU national conferences from 2010-2013.

Methods: All abstracts submitted to the AAP SoSU Program Committee from 2010-2013 were analyzed. Abstracts were classified as basic science, clinical, domestic, international, as well as by disease processes, single, multicenter, retrospective, prospective, registry data, or other.

Psychological assessment of mothers and their daughters at the time of diagnosis of precocious puberty.

Background: Concerns about psychological distress are often used to justify treatment of girls with precocious puberty, but there is little evidence to support these concerns. The extent to which psychological problems are associated with central precocious puberty (CPP) compared with other forms of early puberty in girls has likewise not been established.

Methods: Girls presenting with untreated CPP, premature adrenarche (PA) or early normal puberty (ENP) were recruited from our pediatric endocrine clinic along with their mothers.

The Effects of Pulmonary Valve Replacement for Severe Pulmonary Regurgitation on Exercise Capacity and Cardiac Function.

Patients may develop hemodynamic abnormalities after right ventricular outflow tract (RVOT) repair. Re-intervention timing remains a dilemma. This study evaluates exercise capacity and RV function before and after intervention using age-related comparisons.

High dose dexmedetomidine: effective as a sole agent sedation for children undergoing MRI.

Objective. To determine the efficacy and safety of high dose dexmedetomidine as a sole sedative agent for MRI. We report our institution's experience.

Loss of periostin/OSF-2 in ErbB2/Neu-driven tumors results in androgen receptor-positive molecular apocrine-like tumors with reduced Notch1 activity.

Introduction: Periostin (Postn) is a secreted cell adhesion protein that activates signaling pathways to promote cancer cell survival, angiogenesis, invasion, and metastasis. Interestingly, Postn is frequently overexpressed in numerous human cancers, including breast, lung, colon, pancreatic, and ovarian cancer.

Methods: Using transgenic mice expressing the Neu oncogene in the mammary epithelium crossed into Postn-deficient animals, we have assessed the effect of Postn gene deletion on Neu-driven mammary tumorigenesis.

Relative cerebral blood volume from dynamic susceptibility contrast perfusion in the grading of pediatric primary brain tumors.

Introduction: The aim of this study is to evaluate the utility of relative cerebral blood volume (rCBV) data from dynamic susceptibility contrast (DSC) perfusion in grading pediatric primary brain tumors.

Methods: A retrospective blinded review of 63 pediatric brain tumors with DSC perfusion was performed independently by two neuroradiologists. A diagnosis of low- versus high-grade tumor was obtained from conventional imaging alone.

Multipotent hematopoietic progenitors divide asymmetrically to create progenitors of the lymphomyeloid and erythromyeloid lineages.

Hematopoietic stem and progenitor cells (HSPCs) can self-renew and create committed progenitors, a process supposed to involve asymmetric cell divisions (ACDs). Previously, we had linked the kinetics of CD133 expression with ACDs but failed to detect asymmetric segregation of classical CD133 epitopes on fixed, mitotic HSPCs. Now, by using a novel anti-CD133 antibody (HC7), we confirmed the occurrence of asymmetric CD133 segregation on paraformaldehyde-fixed and living HSPCs.

The success of stainless steel crowns placed with the Hall technique: a retrospective study.

Background: In this retrospective study, the authors evaluated the clinical and radiographic success of stainless steel crowns (SSCs) used to restore primary molars with caries lesions, placed by means of both the traditional technique (involving complete caries removal and tooth reduction before placement of the SSC) and the Hall technique (involving no caries removal, no crown preparation and no use of local anesthetic before placement of the SSC).

Methods: The authors conducted a retrospective chart review by using the patient records at a private pediatric dental practice at which the Hall technique had been introduced in June 2010 as an alternative treatment to traditional SSC placement. The inclusion criteria were caries lesions on a primary molar with no clinical or radiographic evidence of pulpitis, necrosis or abscess, as well as follow-up of at least six months or until failure, whichever came first.

More than words: the emotional maltreatment of children.

Emotional maltreatment may be the most complex, prevalent, and damaging form of child maltreatment and can occur simultaneously with other forms of abuse. Children in the first few years of life seem to be at the greatest risk of suffering the most negative outcomes. Medical professionals can help identify and protect victims of emotional maltreatment by carefully observing caregiver-child interactions, paying attention to a family's social history, making referrals to community or counseling programs when necessary, and reporting any suspicions of maltreatment to Child Protective Services.