Rectoperineal congenital anorectal malformations (CARMs) are diagnosed by examining the perineum, combined with electric stimulation (ES) of the anal sphincter performed under anesthesia. This ...procedure may be troublesome because it is based on the observed contractibility of the sphincter. We aimed to add 3-dimensional high-resolution anorectal manometry (3D-HRAM) to the procedure and to assess its value for diagnosing rectoperineal forms of CARM.
We prospectively included 66 patients younger than 24 months who presented with constipation and were suspected of CARM, idiopathic constipation, or Hirschsprung disease. The patients were assessed between 2015 and 2021 at University Medical Center Groningen, the Netherlands. All patients underwent 3D-HRAM, followed by the standard diagnostic procedure for either CARM or Hirschsprung disease.
Of the 51 patients who underwent both 3D-HRAM and ES, we observed that patients with rectoperineal CARM revealed a pressure gap along the anterior part of the anal sphincter. Based on this observation, we diagnosed 35 patients with CARM. Subsequently, all 35 diagnoses were confirmed with ES. Sixteen patients were diagnosed as not having CARM, 100% of whom were in agreement with the gold standard-ES. Both the specificity and sensitivity of 3D-HRAM for diagnosing rectoperineal CARM were 100%.
We consider 3D-HRAM a reliable tool for diagnosing and excluding rectoperineal CARM. Using this method in patients suspected of CARM might keep infants from undergoing unnecessary interventions requiring anesthetics, such as ES. By providing objective insight into the functional capabilities of the anal sphincter, anorectal manometry adds to the diagnosis.
ObjectiveWhile National Surgical, Obstetric and Anaesthesia Plans (NSOAPs) have emerged as a strategy to strengthen and scale up surgical healthcare systems in low/middle-income countries (LMICs), ...the degree to which children’s surgery is addressed is not well-known. This study aims to assess the inclusion of children’s surgical care among existing NSOAPs, identify practice examples and provide recommendations to guide inclusion of children’s surgical care in future policies.DesignWe performed two qualitative content analyses to assess the inclusion of children’s surgical care among NSOAPs. We applied a conventional (inductive) content analysis approach to identify themes and patterns, and developed a framework based on the Global Initiative for Children’s Surgery’s Optimal Resources for Children’s Surgery document. We then used this framework to conduct a directed (deductive) content analysis of the NSOAPs of Ethiopia, Nigeria, Rwanda, Senegal, Tanzania and Zambia.ResultsOur framework for the inclusion of children’s surgical care in NSOAPs included seven domains. We evaluated six NSOAPs with all addressing at least two of the domains. All six NSOAPs addressed ‘human resources and training’ and ‘infrastructure’, four addressed ‘service delivery’, three addressed ‘governance and financing’, two included ‘research, evaluation and quality improvement’, and one NSOAP addressed ‘equipment and supplies’ and ‘advocacy and awareness’.ConclusionsAdditional focus must be placed on the development of surgical healthcare systems for children in LMICs. This requires a focus on children’s surgical care separate from adult surgical care in the scaling up of surgical healthcare systems, including children-focused needs assessments and the inclusion of children’s surgery providers in the process. This study proposes a framework for evaluating NSOAPs, highlights practice examples and suggests recommendations for the development of future policies.
Metastatic rectal cancer patients could benefit from novel therapeutic approaches. The signaling network formed by chemokines and their receptors can promote metastasis and resistance to current ...anticancer treatments. This study assessed the expression of chemokine receptor 4 (CXCR4) and its ligand CXCL12 immuhistochemically in stage IV rectal tumors. Paraffin-embedded primary tumor collected before and after local radiotherapy and systemic treatment with bevacizumab, oxaliplatin and capecitabine was analyzed. Receptor and ligand expression was assessed in the cytoplasm and nucleus of tumor, stromal and normal rectal crypt cells. Baseline expression of CXCR4 and CXCL12 was correlated with patients' pathologic response to treatment. At diagnosis (n=46), 89% of the rectal tumors expressed cytoplasmic CXCR4 and 81% CXCL12. Nuclear CXCR4 expression in tumor cells was present in 30% and nuclear CXCL12 expression in 35% of the tumors. After radiochemotherapy and administration of bevacizumab, nuclear CXCL12 expression was observed in 79% of residual tumors, as compared to 31% of the paired tumor samples expressing nuclear CXCL12 before treatment (P=0.001). There were no differences in CXCR4 or CXCL12 expression at baseline between the patients who had (n=9) and did not have (n=30) a pathologic complete response. Our results show that CXCR4 and CXCL12 are extensively expressed in primary rectal tumors of patients presenting with metastatic disease, while radiochemotherapy and bevacizumab further upregulate CXCL12 expression. These data indicate the importance of the CXCR4/CXCL12 axis in rectal tumor biology, and may suggest the CXCR4/CXCL12 receptor-ligand pair as a potential therapeutic target in metastatic rectal cancer.
In 2012, we started the KLANKbord-study. A quality of life (QoL) study that follows patients with an anorectal malformation (ARM) or Hirschsprung disease (HD) and their parents from diagnosis till ...the age of 18 years. We hypothesized that the diagnosis of ARM or HD initially has a negative influence on QoL and anxiety levels of parents, but that this influence will diminish over time. The aim of this study is to see whether QoL and anxiety levels of parents change within the first year after the diagnosis.
Parents of all children born with ARM or HD, were eligible for this study. Within 3 months after the diagnosis ARM or HD, parents received a set of validated QoL questionnaires (measurement 1). Measurement 2 was 12 months after the first questionnaire.
During measurement 1 mothers (n = 20) scored significantly higher on the social (p value, 0.01; 95% confidence interval CI, 0.3946-3.1528) and environmental domain (p value, 0.01; 95% CI, 0.4449-2.2851) of the World Health Organization Quality of Life-BREF (WHOQOL-BREF) compared with the known reference values. Fathers (n = 19) scored significantly higher on the physical (p value, 0.01; 95% CI, 0.2964-1.8072), psychological (p value, 0.001; 95% CI, 0.7697-2.4757), and environmental domain (p value, 0.003; 95% CI, 0.5586-2.4214) than the reference values. Comparison of all domains of the WHOQOL-BREF for mothers and fathers between measurements did not show a significant difference. Anxiety levels of mothers were lower during measurement 2 compared with measurement 1. Anxiety levels of fathers were higher during measurement 2 compared with measurement 1. These differences are not significant. Anxiety levels of mothers were significantly higher than anxiety levels of fathers during measurement 1 (p value, 0.002; 95% CI, 0.808-2,956). During measurement 2 this difference in anxiety of mothers versus fathers did not exist (p value, 0.373; 95% CI, -1.157 to 2.922).
A negative influence on the QoL of parents having a child with ARM or HD, compared with the reference population was not seen in this population. QoL did not change significantly during the first year for both fathers and mothers. Anxiety levels of mothers did decline during this first year. The number of parents included in this study is still small, which might influence our results. Therefore, we will continue the KLANKbord-study indefinitely.
Selective serotonin reuptake inhibitors (SSRIs) are the most common antidepressants used in first-world countries and are generally well tolerated. Specifically, less cardiovascular toxicity has been ...reported in comparison with tricyclic antidepressants. Here we report QT interval prolongation after an overdose of the SSRI sertraline.
A previously healthy female patient presented with an attempted suicide with overdoses sertraline (2250 mg), diazepam (200 mg), and temazepam (400 mg). Routine laboratory studies were normal and her ECG upon admission showed a normal QT interval. The next day, her ECG showed prolongation of the QTc interval up to 525 ms. After discontinuation of sertraline the QT interval normalized. Echocardiography and exercise electrocardiography were normal. After hospitalization, the patient resumed sertraline in the normally recommended dose and QT interval remained within normal ranges.
It seems that the SSRI sertraline in overdose may cause QT interval prolongation.
Treatment of patients with distal and locally advanced rectal cancer is challenging. In many series, abdominoperineal resection for distal rectal cancer is related to a high percentage of local ...recurrences. Some authors relate this high percentage of local recurrence to the abdominoperineal resection itself, considering it to be a poor operation for distal rectal cancer, while other authors relate it to technically inadequate resections: a high incidence of positive circumferential resection margins is seen because of coning of the specimen when the mesorectal fascia is followed or because of inadvertent perforation of the rectal wall. In many other series an acceptable low percentage of local recurrence after abdominoperineal resection is reported. These authors have consistently advocated a wide perineal resection, resecting the levator ani muscle en bloc with the specimen. These enhanced perineal resections are not standardized in the surgical world. We reviewed the literature and describe technical considerations for performing the perineal phase in abdominoperineal resection to reduce circumferential resection margin positivity and local recurrence rates.
Low molecular weight heparin in hemodialysis and hemofiltration patients. Low molecular weight (LMW)-heparin was used as the sole anticoagulant during hemodialysis and hemofiltration in a pilot study ...on 32 patients. A LMW-heparin dose corresponding to 50% of the patients usual unfractionated, standard (UF)-heparin dose was found to produce comparable plasma heparin levels (anti-FXa-activity). No thrombosis of the extracorporal system and no bleeding complications occurred at this LMW-heparin dose. In contrast to UF-heparin, LMW-heparin produced only slight increases in PTT and thrombin time in all patients. Lipoprotein lipase was stimulated only marginally by LMW-heparin, with a correspondingly reduced release of free fatty acids. Both heparin species caused similar elevations in factor VIII and fibrin monomers, thus excluding a difference in coagulation activation. On the basis of these results, long-term studies have been started at four nephrology centers. To date, 26 patients have been treated with LMW-heparin for 6 months. A LMW-heparin dose was used that produced plasma anti-FXa-activity of 0.5 to 0.9 U/ml (initial dose: 30 to 40; dose/hr: 8 to 15 anti-FXa-units/kg body wt). PTT and thrombin time were only increased by 5sec on average. Surprisingly, the elevated pre-dialysis levels of factor VIII and fibrin monomers decreased during this 6-month period. Bleeding complications did not occur and thrombotic complications were not observed when the anti-FXa levels were above 0.5 U/ml. LMW-heparin, therefore, appears to be a good alternative to UF-heparin for dialysis patients and may present less risk of bleeding because of its reduced effect on PTT, thrombin time, and thrombocytes.
Dialysances of urea, creatinine, ethylenediamine tetraacetic acid, and ouabain were determined in three different flat-bed dialysers during three successive uses on 15 patients. There were no ...untoward reactions. A significant decrease in dialysing efficiency was observed only with the Ab-Gambro dialyser, in which the dialysance of the small molecules decreased more than that of the large. This is thought to result from blood deposits on the membrane surface inhibiting exchange.
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