Eculizumab therapy in a child with hemolytic uremic syndrome and CFI mutation

Background

Hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure in childhood. It usually occurs after a prodromal episode of diarrhea and it leads to significant morbidity and mortality during the acute phase. However, cases that start as diarrhea-positive HUS whose renal function fail to recover should be screened for genetic disorders of the complement system, which is called atypical HUS (aHUS).

Case-Diagnosis/Treatment

We herein report a 10-year-old girl, who initially came with bloody diarrhea and had features of HUS with delayed renal and hematological recovery despite plasma therapy. Eculizumab (600 mg/week) was initiated on day 15 for atypical presentation and later a complement factor I (CFI) mutation was detected. The girl recovered diuresis within 24 h and after the third eculizumab infusion, hemoglobin, platelet, and C3 levels normalized; renal function improved; and proteinuria completely disappeared in 2 weeks.

Conclusion

It is our belief that eculizumab can be the treatment of choice in children who have plasma exchange-refractory HUS with defective regulation of the alternative complement pathway.     

Survival after Lung Metastasectomy in Colorectal Cancer Patients with Previously Resected Liver Metastases

Background  

Resection of hepatic metastases is indicated in selected stage IV colorectal cancer (CRC) patients. A minority will eventually develop pulmonary metastases and may undergo lung surgery with curative intent. The aims of the present study were to assess clinical outcome and identify parameters predicting survival after pulmonary metastasectomy in patients who underwent prior resection of hepatic CRC metastases.     

The Effectiveness of Nurse‐Led,Home‐Based Pulmonary Rehabilitation in Patients with COPD in Turkey

The goal of this study was to determine the effectiveness of nurse‐led, home‐based pulmonary rehabilitation in patients with stage 3 or 4 chronic obstructive pulmonary disease (COPD), according to the Global Initiative for COPD (GOLD) staging system. The study consisted of 32 patients—a nurse‐led, home‐based pulmonary rehabilitation group (16) and a control group (16). In the rehabilitation program, patients received education about their diseases and performed breathing exercises and lower‐ and upper‐extremity aerobic exercises at their homes during the 3‐month period of the study. There was meaningful improvement in the rehabilitation group in terms of pulmonary function tests (FEV₁% predicted value), artery blood gases (PaCO₂), quality of life, dyspnea, and functional capacity. On the other hand, no meaningful change was observed in the control group. The study showed that the nurse‐led, home‐based pulmonary rehabilitation program had positive effects on the patients with COPD.     

Comparison of paravertebral block versus fast-track general anesthesia via laryngeal mask airway in outpatient inguinal herniorrhaphy

Purpose  

Outpatient inguinal herniorrhaphy (IH) can be successfully performed under general, regional, or local anesthesia. In this study recovery profile, postoperative pain scores, incidence of adverse effects, and patient and surgeon satisfaction were compared between paravertebral block (PVB) and fast-track general anesthesia (GA) via laryngeal mask airway (LMA) for outpatient IH.     

The Assessment of Validity of Different Asthma Diagnostic Tools in Adults

The aim of this study was to evaluate the effect of house dust mite impermeable covers on asthma-specific health-related quality of life in adult asthmatic patients that were trained in guided self-management. In a 2-year randomized placebo-controlled clinical trial, information on the quality of life was collected. The improvement of Mini Asthma Quality of Life Questionnaire (AQLQ) score in the allergens-avoidance group (0.26) was comparable to the improvement in the placebo group (0.30) and not significant. HDM-impermeable covers for pillows, duvets, and mattresses did not result in improved health-related quality of life.     

Assessing the factor structure of posttraumatic stress disorder symptoms in war-exposed youths with and without Criterion A2 endorsement

Posttraumatic stress disorder's (PTSD) tripartite factor structure proposed by the DSM-IV is rarely empirically supported. Other four-factor models ( [King et al., 1998] and [Simms et al., 2002]) have proven to better account for PTSD's latent structure; however, results regarding model superiority are conflicting. The current study assessed whether endorsement of PTSD's Criterion A2 would impact on the factorial invariance of the King et al. (1998) model. Participants were 1572 war-exposed Bosnian secondary students who were assessed two years following the 1992-1995 Bosnian conflict. The sample was grouped by those endorsing both parts of the DSM-IV Criterion A (A2 Group) and those endorsing only A1 (Non-A2 Group). The factorial invariance of the King et al. (1998) model was not supported between the A2 vs. Non-A2 Groups; rather, the groups significantly differed on all model parameters. The impact of removing A2 on the factor structure of King et al. (1998) PTSD model is discussed in light of the proposed removal of Criterion A2 for the DSM-V.     

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Graefe's Archive for Clinical and Experimental Ophthalmology -     

Effect of caffeic acid phenethyl ester on the regression of endometrial explants in an experimental rat model

The objective of this study is to determine the effects of antioxidant and anti-inflammatory caffeic acid phenethyl ester (CAPE) on experimental endometriosis, peritoneal superoxide dismutase (SOD) and catalase (CAT) activities, and malondialdehyde (MDA) levels in the rat endometriosis model. Thirty rats with experimentally induced endometriosis were randomly divided into 2 groups and treated for 4 weeks with intraperitoneal CAPE (CAPE-treated group; 10 micromol/kg/d, n = 13) or vehicle (control group; n = 13). The volume and weight changes of the implants were calculated. Immunohistochemical and histologic examinations of endometriotic explants by semiquantitative analysis and measurements of peritoneal SOD, CAT, and MDA levels were made. Following 4 weeks of treatment with CAPE, there were significant differences in posttreatment spherical volumes (37.4 +/- 14.7 mm(3) vs 147.5 +/- 41.2 mm(3)) and explant weights (49.1 +/- 28.5 mg vs 158.9 +/- 50.3 mg) between the CAPE-treated groups and controls. The mean evaluation nomogram levels in glandular epithelium for COX-2 positivity by scoring system were 2.1 +/- 0.3 in the CAPE-treated group and 3.9 +/- 0.3 in the control group. In the CAPE-treated group, peritoneal levels of MDA and activities of SOD and CAT significantly decreased when compared with the control group (P < .01). Histologic analysis of the explants demonstrated mostly atrophy and regression in the treatment group, and semiquantitative analysis showed significantly lower scores in rats treated with CAPE compared with the control group. CAPE appeared to cause regression of experimental endometriosis.