Progressive esophageal leiomyomatosis with respiratory compromise

Leiomyomatosis is a rare neoplastic condition of the pediatric esophagus. Presenting symptoms usually overlap with more common esophageal disorders, namely, gastroesophageal reflux. A patient is presented in whom leiomyomatosis progressed to the point of causing cachexia and respiratory compromise.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/42053/1/247-30-4-247_00300247.pd

Diverse manifestations of tumorigenicity and immunogenicity displayed by the poorly immunogenic B16-BL6 melanoma transduced with cytokine genes

 We evaluated the in vivo response to the poorly immunogenic B16-BL6 (BL6) murine melanoma genetically altered to secrete interleukin-2 (IL-2), IL-4, interferon γ (IFNγ) and granulocyte/macrophage-colony-stimulating factor (GM-CSF). Three parameters were evaluated: (1) tumorigenicity, (2) vaccination of naive animals, and (3) assessment of antitumor reactivity of T cells derived from tumor-draining lymph nodes (TDLN). Secretion of IL-2 abrogated the tumorigenicity of BL6, while IFNγ and IL-4 partially reduced tumorigenicity, and GM-CSF had no effect. Protective immunity to wild-type tumor challenge could not be achieved by vaccination with irradiated cytokine-secreting tumors, although IL-2 and IL-4 secretion appeared to retard the growth of the challenge inoculum significantly. An alternative method to evaluate the immunogenicity of the cytokine-secreting tumors was to measure the ability of T cells obtained from TDLN to mediate regression of wild-type tumor in adoptive immunotherapy. Neither IL-2 nor IFNγ secretion resulted in the induction of immune T cells. By contrast, GM-CSF and IL-4 secretion were found to induce immune T cells in the TDLN with GM-CSF being superior to IL-4. The combined secretion of GM-CSF and IL-4 did not lead to enhanced induction of immune T cells. GM-CSF secretion was found to up-regulate B7-1 expression in TDLN, consistent with an increase in the population of antigen-presenting cells. These studies demonstrated that reduced tumorigenicity by cytokine secretion did not correlate with increased immunogenicity. With the cytokines examined, there was limited capability of developing protective immunity against the BL6 tumor. Nevertheless, GM-CSF and IL-4 secretion significantly enhanced T cell immune reactivity to the poorly immunogenic BL6 tumor.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/42080/1/262-42-4-237_60420237.pd

Alveolar capillary dysplasia: A logical approach to a fatal disease

Purpose: The aim of the study was to review the authors' experience with alveolar capillary dysplasia (ACD), a cause of persistent pulmonary hypertension of the neonate (PPHN) caused by decreased alveolar units, dilated anomalous pulmonary veins, thick-walled arterioles, and thickened interalveolar septa. Methods: The records of all neonates with ACD were reviewed from Children's Hospital, Columbus, Ohio, and Sophia's Children's Hospital, Rotterdam, The Netherlands. The clinical characteristics and pathological findings are discussed. Results: Eight neonates were diagnosed with ACD from 1994 to 2002. Twenty-five percent (2/8) experienced respiratory distress immediately after birth, whereas 75% (6/8) had normal Apgar scores but deteriorated 1.5 hours to 30 days after birth. All infants required conventional ventilation initially; 50% (4/8) were placed on high-frequency oscillating ventilation and 87% (7/8) on extracorporeal membrane oxygenation. A premorbid diagnosis was established in 3 patients by open lung biopsy. The diagnosis of ACD was confirmed at autopsy in all patients. Conclusions: ACD is a fatal disease that should be suspected in all neonates with respiratory failure and PPHN who fail conventional therapy. Prompt diagnosis helps to avoid prolongation of costly treatment modalities in a uniformly fatal disease. An algorithm is proposed in which neonates with PPHN who fail treatment with extracorporeal membrane oxygenation are managed by open lung biopsy

Skip segment Hirschsprung disease and Waardenburg syndrome

Skip segment Hirschsprung disease describes a segment of ganglionated bowel between two segments of aganglionated bowel. It is a rare phenomenon that is difficult to diagnose. We describe a recent case of skip segment Hirschsprung disease in a neonate with a family history of Waardenburg syndrome and the genetic profile that was identified

Antibiotics and appendicitis in the pediatric population: An American pediatric surgical association outcomes and clinical trials committee systematic review

Objective: The aim of the study was to review evidence-based data regarding the use of antibiotics for the treatment of appendicitis in children.Data source: Data were obtained from PubMed, MEDLINE, and citation review.Study selection: We conducted a literature search using appendicitis combined with antibiotics with children as the target patient population. Studies were selected based on relevance for the following questions: (1) What perioperative antibiotics should be used for pediatric patients with nonperforated appendicitis? (2) For patients with perforated appendicitis treated with appendectomy: a. What perioperative intravenous antibiotics should be used? b. How long should perioperative intravenous antibiotics be used? c. Should oral antibiotics be used? (3) For patients with perforated appendicitis treated with initial nonoperative management, what antibiotics should be used in the initial management?Results: Children with nonperforated appendicitis should receive preoperative, broad-spectrum antibiotics. In children with perforated appendicitis who had undergone appendectomy, intravenous antibiotic duration should be based on clinical criteria. Furthermore, broad-spectrum, single, or double agent therapy is as equally efficacious as but is more cost-effective than triple agent therapy. If intravenous antibiotics are administered for less than 5 days, oral antibiotics should be administered for a total antibiotic course of 7 days. For children with perforated appendicitis who did not initially undergo an appendectomy, the duration of broad-spectrum, intravenous antibiotics should be based on clinical symptoms.Conclusions: Current evidence supports the use of guidelines as described above for antibiotic therapy in children with acute and perforated appendicitis

Optimizing resources for the surgical care of children: An American pediatric surgical association outcomes and clinical trials committee consensus statement

The United States\u27 healthcare system is facing unprecedented pressures: the healthcare cost curve is not sustainable while the bar of standards and expectations for the quality of care continues to rise. Systems committed to the surgical treatment of children will likely require changes and reorganization. Regardless of these mounting pressures, hospitals must remain focused on providing the best possible care to each child at every encounter. Available clinical expertise and hospital resources should be optimized to match the complexity of the treated condition. Although precise criteria are lacking, there is a growing consensus that the optimal combination of clinical experience and hospital resources must be defined, and efforts toward this goal have been supported by the Regents of the American College of Surgeons, the members of the American Pediatric Surgical Association, and the Society for Pediatric Anesthesia (SPA) Board of Directors. The topic of optimizing outcomes and the discussion of the concepts involved have unfortunately become divisive. Our goals, therefore, are 1) to provide a review of the literature that can provide context for the discussion of regionalization, volume, and optimal resources and promote mutual understanding of these important terms, 2) to review the evidence that has been published to date in pediatric surgery associated with regionalization, volume, and resource, 3) to focus on a specific resource (anesthesia), and the association that this may have with outcomes, and 4) to provide a framework for future research and policy efforts

Outcomes research in pediatric surgery. Part 1: Overview and resources

Outcomes research in pediatric surgery can be defined as the analysis of pediatric surgical outcomes and their predictors at different levels in the health care delivery system. The objectives of this article are to understand the differences between outcomes research and clinical trials as well as to gain familiarity with public multispecialty and specialty-specific databases. The utility of outcomes research extends to benchmarking the quality of care, refinement of management strategies, patient education, and marketing. Assessment of the integration of a new surgical technique into the health care system is best determined by examining a population-based registry, whereas comparative efficacy of surgical procedures is best assessed by randomized clinical trials. In the first part of this 2-part series, an overview and brief outline of available resources for outcomes research in pediatric surgery are reviewed. In part 2, a template is presented on how to structure and design an outcomes research question