Childhood achalasia: A comprehensive review of disease, diagnosis and therapeutic management

Achalasia is an esophageal motility disorder characterized by failure of lower esophageal sphincter (LES) relaxation and is rare in children. The most common symptoms are vomiting, dysphagia, regurgitation, and weight loss. Definitive diagnosis is made with barium swallow study and esophageal manometry. In adults, endoscopic biopsy is recommended to exclude malignancy however; it is not as often indicated in children. Medical management often fails resulting in recurrent symptoms and the ultimate definitive treatment is surgical. Laparoscopic Heller myotomy with or without an anti-reflux procedure is the treatment of choice and has become standard of care for children with achalasia. Peroral endoscopic myotomy is a novel therapy utilized with increasing frequency for achalasia treatment in adults. More experience is needed to determine the safety, efficacy, and feasibility of peroral endoscopic myotomy in children

Prediction of Patients with Acute Cholecystitis Requiring Emergent Cholecystectomy: A Simple Score

The objective was to develop a score, to stratify patients with acute cholecystitis into high, intermediate, or low probability of gangrenous cholecystitis. The probability of gangrenous cholecystitis (score) was derived from a logistic regression of a clinical and pathological review of 245 patients undergoing urgent cholecystectomy. Sixty-eight patients had gangrenous inflammation, 132 acute, and 45 no inflammation. The score comprised of: age > 45 years (1 point), heart rate > 90 beats/min (1 point), male (2 points), Leucocytosis > 13,000/mm3 (1.5 points), and ultrasound gallbladder wall thickness > 4.5 mm (1 point). The prevalence of gangrenous cholecystitis was 13% in the low-probability (0–2 points), 33% in the intermediate-probability (2–4.5 points), and 87% in the high probability category (>4.5 points). A cutoff score of 2 identified 31 (69%) patients with no acute inflammation (PPV 90%). This scoring system can prioritize patients for emergent cholecystectomy based on their expected pathology

Natural Progression of Biochemical Markers of Biliary Tract Obstruction in Patients with Gallstone Pancreatitis

The presenting pattern and natural progression of biochemical markers of biliary tract obstruction in patients with gallstone pancreatitis have not been elucidated. We analyzed serial values of bilirubin levels following admission to discharge in 143 patients. Ninety-four of patients demonstrated a Decrescendo (falling) pattern of bilirubin levels from admission until normalization at 21 hours (median). Forty-nine patients demonstrated a Crescendo-Decrescendo (initially rising) pattern with peak levels of bilirubin occurring at 39 hours after admission followed by a subsequent normalization after a median of 119 hours. Patients in the Decrescendo group were significantly younger (33 versus 41 years, P = .02) and more patients had experienced symptoms for greater than 48 hours (65% versus 47%, P = .05). Ten percent of patients in the Decrescendo group and 29% of patients in the Crescendo-Decrescendo group underwent ERCP (P = .02). Normalization of biochemical markers after ERCP was significantly delayed in both groups compared to no ERCP. Older patients present earlier, with higher bilirubin levels and normalize slower than younger patients, perhaps due to fibrosis of the ampulla and decreased compliance of the common bile duct. Patients who disobstruct spontaneously (90%) normalize quicker than patients undergoing ERCP

Dissemination of Strongyloides stercoralis in a patient with systemic lupus erythematosus after initiation of albendazole: a case report

<p>Abstract</p> <p>Introduction</p> <p><it>Strongyloides stercoralis </it>infection affects hundreds of millions of people worldwide. As immigration rates and international travel increase, so does the number of cases of strongyloidiasis in the United States. Although described both in immigrant and in immunosuppressed populations, hyperinfection and dissemination of <it>S. stercoralis </it>following the initiation of antiparasitic medication is a previously unreported phenomenon.</p> <p>Case presentation</p> <p>Here we describe the case of a 38-year-old immunocompromised woman with systemic lupus erythematosus, who developed disseminated disease following treatment with albendazole (400 mg every 12 hours). Notably the patient was receiving oral prednisone (10 mg once daily), azathioprine (50 mg twice daily), and hydroxychloroquine (400 mg daily) at the time of hospitalization. The patient was subsequently treated successfully with ivermectin (200 mcg/kg daily).</p> <p>Conclusion</p> <p>The reader should be aware that dissemination of <it>S. stercoralis </it>can occur even after the initiation of antiparasitic medication.</p

Lateral Pancreaticojejunostomy for Chronic Pancreatitis and Pancreatic Ductal Dilation in Children.

© Copyright 2018, Mary Ann Liebert, Inc., publishers 2018. Background: Pancreatic ductal obstruction leading to ductal dilation and recurrent pancreatitis is uncommon in children. Treatment is dependent upon etiology but consists of decompression of the pancreatic duct (PD) proximally, if possible, by endoscopic retrograde cholangiopancreatography (ERCP) intervention or surgical decompression with pancreaticojejunal anastomosis. Methods: After institutional review board approval, we retrospectively reviewed the records for 2 children who underwent lateral pancreaticojejunostomy for pancreatic ductal dilation. Data, including demographics, diagnostic studies, operative details, complications, outcomes, and follow-up, were analyzed. Results: Case 1 was a 4-year-old female with pancreatic ductal obstruction with multiple episodes of recurrent pancreatitis and failure of ERCP to clear her PD of stones. She underwent a laparoscopic cholecystectomy with a lateral pancreaticojejunostomy (Puestow procedure). She recovered well with no further episodes of pancreatitis and normal pancreatic function 4 years later. Case 2 was a 2-year-old female who developed recurrent pancreatitis and was found to have papillary stenosis and long common bile-PD channel. Despite multiple sphincterotomies, laparoscopic cholecystectomy, and laparoscopic hepaticoduodenostomy, she continued to experience episodes of pancreatitis. She underwent a laparoscopy converted to open lateral pancreaticojejunostomy. Her recovery was also smooth having had no episodes of pancreatitis or hospital admissions for over 2 years following the Puestow. Conclusions: Indication for lateral pancreaticojejunostomy or Puestow procedure is rare in children and even less often performed using laparoscopy. In our small experience, both patients with pancreatic ductal obstruction managed with Puestow\u27s procedure enjoy durable symptom and pain relief in the long term

Current concepts regarding the pathogenesis of necrotizing enterocolitis

Necrotizing enterocolitis (NEC) is a devastating disease that predominantly affects premature neonates. The mortality associated with NEC has not changed appreciably over the past several decades. The underlying etiology of NEC remains elusive, although bacterial colonization of the gut, formula feeding, and perinatal stress have been implicated as putative risk factors. The disease is characterized by massive epithelial destruction, which results in gut barrier failure. The exact molecular and cellular mechanisms involved in this complex disease are poorly understood. Recent studies have provided significant insight into our understanding of the pathogenesis of NEC. Endogenous mediators such as prostanoids, cyclooxygenases, and nitric oxide may play a role in the development of gut barrier failure. Understanding the structural architecture of the gut barrier and the cellular mechanisms that are responsible for gut epithelial damage could lead to the development of novel diagnostic, prophylactic and therapeutic strategies in NEC