Do You C What I C: Emergency Department Evaluation and Diagnosis of Pediatric Scurvy in an Autistic Child With a Restricted Diet.

Scurvy in modern times may not be as rare as previously thought. The link between adequate intake of vitamin C and scurvy has been known since ancient times and is recorded in Ebers Papyrus. Recent reports indicate that, with restricted diets, vitamin C deficiency is being seen in infants exclusively fed plant-based formula and children with oral aversion, autism, restricted diets, and cerebral palsy. Additional at-risk groups include the older adults and patients having alcoholism. Often costly, emergency department visits and elaborate diagnostic studies lead to fruitless results when a simple diet history is often overlooked. Here, we report a case of pediatric scurvy in an 11-year-old autistic child with a restricted diet who presented with refusal to walk, fatigue, a purpuric rash, and gingival bleeding. The diagnosis was made based on diet history, physical examination findings, and symptom resolution with vitamin C supplementation. Our case report reaffirms that vitamin C deficiency still occurs and should be considered in children with restrictive diets. Early recognition of this disease by physicians provides early diagnosis, avoids costly diagnostic workup and hospitalization, and expedites effective treatment.

Emergency Department Diagnosis of Dietl Crisis in a 7-Year-Old Girl With Abdominal Pain.

Children with Dietl crisis often experience a delay in diagnosis, with the clinical entity being underdiagnosed. Pain is caused by compression of an aberrant artery crossing dilated kidney. Pain is often worsened after the consumption of liquids and resolves after fluid reabsorption. There are no clear criteria for evaluating ureter obstruction in childhood abdominal pain in the emergency department setting; however, it has been suggested that ultrasound may aid in the diagnosis. As renal parenchyma is typically preserved, and there is a paucity of associated urological complaints, once properly diagnosed, most patients are well served by a pyeloplasty.

Upregulation of alveolar epithelial active Na+ transport is dependent on beta2-adrenergic receptor signaling.

Alveolar epithelial beta-adrenergic receptor (betaAR) activation accelerates active Na+ transport in lung epithelial cells in vitro and speeds alveolar edema resolution in human lung tissue and normal and injured animal lungs. Whether these receptors are essential for alveolar fluid clearance (AFC) or if other mechanisms are sufficient to regulate active transport is unknown. In this study, we report that mice with no beta1- or beta2-adrenergic receptors (beta1AR-/-/beta2AR-/-) have reduced distal lung Na,K-ATPase function and diminished basal and amiloride-sensitive AFC. Total lung water content in these animals was not different from wild-type controls, suggesting that betaAR signaling may not be required for alveolar fluid homeostasis in uninjured lungs. Comparison of isoproterenol-sensitive AFC in mice with beta1- but not beta2-adrenergic receptors to beta1AR-/-/beta2AR-/- mice indicates that the beta2AR mediates the bulk of beta-adrenergic-sensitive alveolar active Na+ transport. To test the necessity of betaAR signaling in acute lung injury, beta1AR-/-/beta2AR-/-, beta1AR+/+/beta2AR-/-, and beta1AR+/+/beta2AR+/+ mice were exposed to 100% oxygen for up to 204 hours. beta1AR-/-/beta2AR-/- and beta1AR+/+/beta2AR-/- mice had more lung water and worse survival from this form of acute lung injury than wild-type controls. Adenoviral-mediated rescue of beta2-adrenergic receptor (beta2AR) function into the alveolar epithelium of beta1AR-/-/beta2AR-/- and beta1AR+/+/beta2AR-/- mice normalized distal lung beta2AR function, alveolar epithelial active Na+ transport, and survival from hyperoxia. These findings indicate that betaAR signaling may not be necessary for basal AFC, and that beta2AR is essential for the adaptive physiological response needed to clear excess fluid from the alveolar airspace of normal and injured lungs.

Laparoscopic bariatric surgery can be performed safely in the community hospital setting.

BACKGROUND: Bariatric surgery is being performed commonly in various practice settings. To evaluate safety and efficacy, we reviewed the results of our first 516 laparoscopic bariatric operations performed in a community hospital setting. METHODS: We reviewed retrospectively the results of our first 516 consecutive laparoscopic bariatric procedures. RESULTS: Between December 2001 and December 2004, we performed 516 bariatric surgical procedures. Ninety-nine percent of these were accomplished laparoscopically. Thirty-day mortality in our series of 516 patients is 0%. Of these patients, 431 had laparoscopic gastric bypass. The mean BMI in these patients was 51. Mean percent excess weight loss in the laparoscopic gastric bypass patients was 70% at 1 year, 79% at 2 years and 84% at 3 years. Complications in the laparoscopic bypass group requiring reoperation included 11 bowel obstructions (2.5%), 5 episodes of bleeding (1.1%), and 2 laparoscopies for benign reasons. There were 8 anastomotic leaks (1.9%)-7 requiring reoperation, 1 managed nonoperatively. Eighty-five patients had adjustable gastric banding. Mean BMI was 45. Mean percent excess weight loss in the adjustable gastric banding patients was 39% at 1 year and 57% at 2 years. Complications in the adjustable gastric band patients requiring reoperation included 3 access port malfunctions (3.5%), 3 prolapsed bands (3.5%), 1 punctured band requiring replacement (1.2%) and 1 band causing complete obstruction requiring removal (1.2%). CONCLUSION: Laparoscopic bariatric surgery can be performed safely in the community hospital setting with a very low operative morbidity and mortality. This requires an experienced team of bariatric surgeons leading a multidisciplinary team of other health care professionals. Surgeon experience and super obesity can influence the risks.

Effects of beta2-adrenergic receptor overexpression on alveolar epithelial active transport.

beta-Adrenergic receptor (betaAR) agonists accelerate the clearance of edema from the alveolar airspace by increasing the function of epithelial transport proteins, including epithelial Na(+) channels and Na,K-adenosinetriphosphatases. To improve our understanding of the role of the beta(2)AR in regulating alveolar fluid clearance, we used an adenoviral-mediated gene transfer strategy to effect significant increases in membrane-bound beta(2)AR number and function in the alveolar epithelium of normal rats. Alveolar fluid clearance in beta(2)AR-overexpressing lungs, measured by means of an isolated lung model in the absence of catecholamine supplementation, was 100% greater than in controls. These findings were associated with significant increases of epithelial Na(+) channel function and Na,K-adenosine triphosphatase function in the peripheral lung. Experiments performed with adrenalectomized rats, a beta(2)-agonist (procaterol), and a nonspecific beta-antagonist (propranolol) indicate that overexpression maximally up-regulates beta(2)-adrenergic-responsive alveolar fluid clearance and improves responsiveness to endogenous catecholamines. Mechanistic studies in human lung epithelial cells (A549) indicate that receptor overexpression prevents homologous receptor desensitization, possibly by overwhelming endogenous regulatory pathways. Our studies demonstrate that overexpression of beta(2)AR in lung epithelial cells can be used to study the role and regulation of alveolar beta(2)ARs. They also suggest a therapeutic role for the beta(2)AR in the treatment of pulmonary edema.

In vivo timing of onset of transgene expression following adenoviral-mediated gene transfer.

Recombinant adenoviruses are efficient gene transfer vehicles that could be used for treatment of acute diseases. However, the time required for adenoviruses to produce physiologically relevant levels of transgene in vivo is unknown. To address this question rat lungs were infected with an E1a(-)/E3a(-) adenovirus that contains an hCMV-driven human beta(2)-adrenergic receptor (beta(2)AR) cDNA. Human beta(2)AR message and protein expression were noted 2-4 h postinfection without evidence of pseudotransduction. beta(2)AR function (cAMP production) was increased at 6 h postinfection. To determine when beta(2)AR gene transfer affects downstream catecholamine-sensitive pathways, we measured lung Na,K-ATPase expression and alveolar fluid clearance (AFC). beta(2)AR gene transfer increased Na,K-ATPase number by 80% at 6 h, and AFC by 20% at 8 h postinfection. These data indicate that recombinant adenoviruses can produce physiologically significant levels of transgene within hours of infection and that they may be suitable for gene therapies for acute, rapidly progressive diseases.