Implementing an Evidence-Based,Asthma Decision Support Tool for Children Younger Than 5 Years Old

Introduction

Asthma is underdiagnosed, particularly for children younger than 5 years old. Clinical practice guidelines have been shown to improve asthma diagnosis and management, but are underutilized. This evidence-based practice project aimed to develop, implement, and evaluate a three-page decision support tool (DST) to improve the asthma diagnosis process among children younger than 5 years old.

Midterm Outcomes of the Dissected Aorta Repair Through Stent Implantation Trial

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Myofibroma—A Rare Entity with Unique Clinical Presentation

Myofibroma and myofibromatosis is a well-recognized spindle cell neoplasm that occurs predominantly in infants and young children. They have been described under different names since 1951. These lesions are a benign fibroblast and myofibroblast proliferation containing a biphasic presentation of spindle shaped cells surrounding a central zone of less differentiated cells focally arranged in a hemangiopericytoma like pattern. Classically these lesions are described in children younger than two, with 2/3rd present at birth and rarely in adults. Controversy exists as to an autosomal dominant or recessive inheritance or to a sporadic occurrence. Presented here is a unique case of myofibroma involving the mandible in a 11 year-old male patient. Clinically it mimicked more like a beningn tumor and not exhibiting any of its classical signs. The diagnosis could be established only after complete excision of the lesion and histopathological examination. There was no recurrence after a follow up period of 4 months.     

Idiopathic gingival fibromatosis. A case report.

Gingival fibromatosis is a progressive gingival enlargement caused by an over growth of the collagenous element of the gingival fibrous connective tissue. Pharmacologically induced, hereditary (familial) and idiopathic forms of gingival fibromatosis are recognized. This paper reports a case of idiopathic gingival fibromatosis in a 13 year old boy involving the right maxillary and mandibular arches who had also been treated 3 years back for a gingival enlargement involving the left maxillary and mandibular arches. The enlargement was quite sever and caused significant esthetic and functional problems on both occasions.     

Argininosuccinate Synthetase-1 (ASS1) Loss in High-Grade Neuroendocrine Carcinomas of the Urinary Bladder: Implications for Targeted Therapy with ADI-PEG 20

High-grade neuroendocrine carcinomas (HGNECs) of the urinary bladder encompass small cell (SCNEC) and large cell neuroendocrine carcinomas (LCNEC). Currently, recommended initial management is with systemic chemotherapy, followed by consolidative therapy with either radical cystectomy or radiotherapy in patients with localized disease. Nevertheless, survival in this setting remains poor. We therefore evaluated the potential to modify arginine metabolism as an alternative, targeted therapy approach in these carcinomas. In humans, arginine is a semi-essential amino acid and its synthesis enzyme argininosuccinate synthetase (ASS1) represents the rate-limiting step in arginine biosynthesis. Neoplasms that show low to absent ASS1 expression require extracellular arginine for cancer cell survival, and thus can be targeted using arginine-degrading enzymes such as pegylated arginine deiminase (ADI-PEG 20). An initial study by our group of 19 patients demonstrated that a high percentage of SCNEC lack ASS1 expression. Herein, we evaluated an expanded cohort of 74 radical cystectomy patients with HGNEC, including 63 SCNEC, 5 LCNEC, and 6 mixed morphology HGNEC patients. ASS1 expression was assessed through immunohistochemistry. Fifty-eight (of 74, 78%) patients with HGNEC showed absent ASS1 expression, including all patients with LCNEC and mixed morphology (11 of 11, 100%). Ten-year survival from disease-specific death was not statistically significant between ASS1-expressing and ASS1-deficient cases (p?=?0.75). Our results show that HGNEC of the bladder may be candidates for arginine deprivation therapy using drugs such as ADI-PEG 20. Further studies are needed to validate these findings and to determine the therapeutic efficacy of such agents.