Ektacytometry Analysis of Post-splenectomy Red Blood Cell Properties Identifies Cell Membrane Stability Test as a Novel Biomarker of Membrane Health in Hereditary Spherocytosis.

Hereditary spherocytosis (HS) is the most common form of hereditary chronic hemolytic anemia. It is caused by mutations in red blood cell (RBC) membrane and cytoskeletal proteins, which compromise membrane integrity, leading to vesiculation. Eventually, this leads to entrapment of poorly deformable spherocytes in the spleen. Splenectomy is a procedure often performed in HS. The clinical benefit results from removing the primary site of destruction, thereby improving RBC survival. But whether changes in RBC properties contribute to the clinical benefit of splenectomy is unknown. In this study we used ektacytometry to investigate the longitudinal effects of splenectomy on RBC properties in five well-characterized HS patients at four different time points and in a case-control cohort of 26 HS patients. Osmotic gradient ektacytometry showed that splenectomy resulted in improved intracellular viscosity (hydration state) whereas total surface area and surface-to-volume ratio remained essentially unchanged. The cell membrane stability test (CMST), which assesses the in vitro response to shear stress, showed that after splenectomy, HS RBCs had partly regained the ability to shed membrane, a property of healthy RBCs, which was confirmed in the case-control cohort. In particular the CMST holds promise as a novel biomarker in HS that reflects RBC membrane health and may be used to asses treatment response in HS.

Quality of life after esophageal replacement in children.

PURPOSE: Assessing quality of life (QoL) after esophageal replacement (ER) for long gap esophageal atresia (LGEA). METHODS: All patients after ER for LGEA with gastric pull-up (GPU n = 9) or jejunum interposition (JI n = 14) at the University Medical Center Groningen and Utrecht (1985-2007) were included. QoL was assessed with 1) gastrointestinal-related QoL using the Gastrointestinal Quality of Life Index (GIQLI)), 2) general QoL (Child Health questionnaire CHF87-BREF (children)/World Health Organization questionnaire WHOQOL-BREF (adults)), and 3) health-related QoL (HRQoL) (TNO AZL TACQoL/TAAQoL). Association of morbidity (heartburn, dysphagia, dyspnea on exertion, recurrent cough) and (HR)QoL was evaluated. RESULTS: Six patients after GPU (75%) and eight patients after JI (57%) responded to the questionnaires (mean age 15.7, SD 5.9, 12 male, two female). Mean gastrointestinal, general and health-related QoL total scores of the patients were comparable to healthy controls. However, young adults reported a worse physical functioning (p = 0.02) but better social functioning compared to peers (p = 0.01). Morbidity was not associated with significant differences in (HR)QoL. CONCLUSIONS: With the current validated QoL most patients after ER with GPU and JI for LGEA have normal generic and disease specific QoL scores. Postoperative morbidity does not seem to influence (HR)QoL. TYPE OF STUDY: Prognosis Study. LEVEL OF EVIDENCE: III.

Effects and efficacy of laparoscopic fundoplication in children with GERD: a prospective, multicenter study.

INTRODUCTION: Laparoscopic antireflux surgery (LARS) in children primarily aims to decrease reflux events and reduce reflux symptoms in children with therapy-resistant gastroesophageal reflux disease (GERD). The aim was to objectively assess the effect and efficacy of LARS in pediatric GERD patients and to identify parameters associated with failure of LARS. METHODS: Twenty-five children with GERD [12 males, median age 6 (2-18) years] were included prospectively. Reflux-specific questionnaires, stationary manometry, 24-h multichannel intraluminal impedance pH monitoring (MII-pH monitoring) and a 13C-labeled Na-octanoate breath test were used for clinical assessment before and 3 months after LARS. RESULTS: After LARS, three of 25 patients had persisting/recurrent reflux symptoms (one also had persistent pathological acid exposure on MII-pH monitoring). New-onset dysphagia was present in three patients after LARS. Total acid exposure time (AET) (8.5-0.8 %; p < 0.0001) and total number of reflux episodes (p < 0.001) significantly decreased and lower esophageal sphincter (LES) resting pressure significantly increased (10-24 mmHg, p < 0.0001) after LARS. LES relaxation, peristaltic contractions and gastric emptying time did not change. The total number of reflux episodes on MII-pH monitoring before LARS was a significant predictor for the effect of the procedure on reflux reduction (p < 0.0001). CONCLUSIONS: In children with therapy-resistant GERD, LARS significantly reduces reflux symptoms, total acid exposure time (AET) and number of acidic as well as weakly acidic reflux episodes. LES resting pressure increases after LARS, but esophageal function and gastric emptying are not affected. LARS showed better reflux reduction in children with a higher number of reflux episodes on preoperative MII-pH monitoring.

Impact of laparoscopic antireflux surgery on belching in pediatric GERD patients.

BACKGROUND: Laparoscopic antireflux surgery (LARS) is a well-established treatment option for children with proton pomp inhibitor (PPI)-resistant gastroesophageal reflux disease (GERD). Besides preventing reflux of gastric fluid and solid content, LARS may also impair the ability of the stomach to vent intragastric air (i.e. gastric belching) and induce gas-related complications, such as bloating and/or hyperflatulence. Furthermore, it was previously hypothesized that LARS induces a behavioral type of belching, not originating from the stomach, called supragastric belching. The aim of this study was to objectively evaluate the impact of LARS on gastric (GB) and supragastric belching (SGB) in children with GERD. METHODS: We performed a prospective, Dutch multicenter cohort study including 25 patients (12 males, median age 6 (range 2-18) years) with PPI-resistant GERD who were scheduled for LARS. Twenty-four-hour multichannel intraluminal impedance pH monitoring (MII-pH monitoring) was performed before and 3 months after fundoplication. Impedance pH tracings were analyzed for reflux episodes and GBs and SGBs. KEY RESULTS: LARS reduced acid exposure time from 8.5% (6.0-16.2%) to 0.8% (0.2-2.8%), p < 0.001. The number of GBs also significantly decreased after LARS (59 [43-77] VS 5 [2-12], p < 0.001). The number of air swallows remained unchanged after LARS. SGBs were infrequent before LARS with no change in the number of SGB observed after the procedure. Postoperative belching symptoms were associated with GBs, not with SGBs. CONCLUSION & INFERENCES: LARS significantly reduces the number of GBs in children with GERD, whereas the number of air swallows remains unchanged. Postoperative symptomatic belching is associated with GBs, but not with SGBs. These findings suggest that LARS does not induce the occurrence of SGBs in children, but longer follow-up is required.

Effect of Vagus Nerve Integrity on Short and Long-Term Efficacy of Antireflux Surgery.

OBJECTIVES: Vagus nerve injury is a feared complication of antireflux surgery (ARS) that may negatively affect reflux control. The aim of the present prospective study was to evaluate short-term and long-term impact of vagus nerve injury, evaluated by pancreatic polypeptide response to insulin-induced hypoglycemia (PP-IH), on the outcome of ARS. METHODS: In the period from 1990 until 2000, 125 patients with gastroesophageal reflux disease (GERD) underwent ARS at a single center. Before and 6 months after surgery, vagus nerve integrity testing (PP-IH), 24-h pH-monitoring, gastric emptying, and reflux-associated symptoms were evaluated. In 2014, 14-25 years after surgery, 110 patients were contacted again for evaluation of long-term symptomatic outcome using two validated questionnaires (Gastrointestinal Symptom Rating Scale (GSRS) and GERD-Health Related Quality of Life (HRQL)). RESULTS: Short-term follow-up: vagus nerve injury (PP peak ≤47 pmol/l) was observed in 23 patients (18%) 6 months after fundoplication. In both groups, a comparable decrease in reflux parameters and symptoms was observed at 6-month follow-up. Postoperative gastric emptying was significantly delayed in the vagus nerve injury group compared with the vagus nerve intact group. Long-term follow-up: patients with vagus nerve injury showed significantly less effective reflux control and a higher re-operation rate. CONCLUSIONS: Vagus nerve injury occurs in up to 20% of patients after ARS. Reflux control 6 months after surgery was not affected by vagus nerve injury. However, long-term follow-up showed a negative effect on reflux symptom control and re-operation rate in patients with vagus nerve injury.

Complete versus partial fundoplication in children with gastroesophageal reflux disease: results of a systematic review and meta-analysis.

Complete fundoplication (Nissen) has long been accepted as the gold standard surgical procedure in children with therapy-resistant gastroesophageal reflux disease (GERD); however, increasingly more evidence has become available for partial fundoplication as an alternative. The aim of this study was to perform a systematic review and meta-analysis comparing complete versus partial fundoplication in children with therapy-resistant GERD. PubMed (1960 to 2011), EMBASE (from 1980 to 2011), and the Cochrane Library (issue 3, 2011) were systematically searched according to the PRISMA statement. Results were pooled in meta-analyses and expressed as risk ratios (RRs). In total, eight original trials comparing complete to partial fundoplication were identified. Seven of these studies had a retrospective study design. Short-term (RR 0.64; p = 0.28) and long-term (RR 0.85; p = 0.42) postoperative reflux control was similar for complete and partial fundoplication. Complete fundoplication required significantly more endoscopic dilatations for severe dysphagia (RR 7.26; p = 0.007) than partial fundoplication. This systematic review and meta-analysis showed that reflux control is similar after both complete and partial fundoplication, while partial fundoplication significantly reduces the number of dilatations to treat severe dysphagia. However, because of the lack of a well-designed study, we have to be cautious in making definitive conclusions. To decide which type of fundoplication is the best practice in pediatric GERD patients, more randomized controlled trials comparing complete to partial fundoplication in children with GERD are warranted.

Neurenteric cyst--a case report of this rare disorder.

Neurenteric cysts are rare congenital lesions that are believed to be the result of the split notochord syndrome. We report the clinical case of a 5-year-old boy presenting with vague gastrointestinal symptoms and fatigue, who had undergone resection of a small intestine duplication cyst as a newborn. Computed tomography revealed a mediastinal neurenteric cyst with partial destruction of several thoracic vertebrae. Resection of the tumor proved effective. Recognition of this disorder is important: because of its benign nature, the prognosis after surgical resection can be good. If the diagnosis is made in an early stage, unnecessary progressive destruction of surrounding structures may be prevented.

[Ancillary diagnostic imaging is undesirable if there is evidence of appendicitis]. / Aanvullend beeldvormend onderzoek is ongewenst bij aanwijzingen voor appendicitis.

CT and ultrasound have been advocated to improve the diagnostic accuracy and management of appendicitis. However, these were single-centre studies, performed by specialised radiologists and with a low level of evidence. Most of the literature shows no increase in diagnostic accuracy or decrease of negative appendectomies if radiographic imaging techniques are applied. However these techniques do lead to disadvantages such as radiation load and unnecessary prolongation of the diagnostic phase. Therefore, CT or ultrasound are of no use in the diagnosis and management of acute appendicitis.

The effect of laparoscopic partial fundoplication on dysphagia, esophageal and lower esophageal sphincter motility.

It has been suggested that dysphagia is less common after partial versus complete fundoplication. The mechanisms contributing to postoperative dysphagia remain unclear. The objective of the present prospective study was to investigate esophageal motility and the prevalence of dysphagia in patients who have undergone laparoscopic partial fundoplication. Symptoms, lower esophageal sphincter (LES) characteristics and esophageal body motility were evaluated prospectively in 62 patients before and after laparoscopic partial fundoplication: 33 women and 29 men with a mean age of 44 +/- 1.5 years (range, 21-71). The patients filled in symptom questionnaires and underwent stationary and ambulatory manometry and 24-h pH-metry before and after operation. A small but significant increase in LES pressure from 14.8 +/- 0.9 to 17.8 +/- 0.8 mmHg was seen after laparoscopic partial fundoplication. Further, LES characteristics and esophageal body motility were not different post- versus preoperation. Three months after surgery, dysphagia was present in eight patients. No differences in LES characteristics or body motility were present between patients with and without dysphagia. Six months after the operation dysphagia was present in only three patients (3.2% mild and 1.6% severe dysphagia). Adequate reflux control was obtained in 85% of the patients. Laparoscopic partial fundoplication offers adequate reflux control without affecting esophageal body motility and with a very low incidence of postoperative dysphagia.

Function of the proximal stomach after partial versus complete laparoscopic fundoplication.

OBJECTIVES: After antireflux surgery, more than 30% of patients develop dyspeptic symptoms such as fullness and early satiety. We have previously shown that these symptoms are related to fundoplication-induced changes in proximal gastric motor and sensory function, especially impaired postprandial relaxation. We hypothesize that impaired fundus relaxation may be more pronounced after complete versus partial fundoplication. METHODS: Fasting and postprandial proximal gastric motor and sensory function were measured with an electronic barostat in patients after laparoscopic partial (n = 14) and complete (n = 14) fundoplication, in gastroesophageal reflux disease (GERD) patients (n = 12), and in healthy control subjects (n = 15). Gastric emptying and vagus nerve function tests were performed in all patients. RESULTS: Minimal distending pressure (MDP) and proximal gastric compliance were not significantly different among patients after antireflux surgery, GERD patients, and healthy controls. Maximal postprandial fundus relaxation was significantly (p < 0.01) reduced in patients after partial (267 +/- 32 ml) and complete (294 +/- 34 ml) fundoplication compared with GERD patients (448 +/- 30 ml) and healthy controls (409 +/- 25 ml). Sensations of fullness were not significantly different between patients with partial and complete fundoplication. There was a significant positive correlation between the postoperative duration and the degree of postprandial fundus relaxation (r = 0.67; p < 0.001). CONCLUSIONS: Both after complete and after partial fundoplication, proximal gastric motor function is affected, with impaired postprandial relaxation and increased sensation of fullness. These alterations are not related to the type of fundoplication but correlate significantly with the duration of the postoperative period.