Atypical Hemolytic Uremic Syndrome (aHUS) and Adenosine Deaminase (ADA)-Deficient Severe Combined Immunodeficiency (SCID)-Two Diseases That Exacerbate Each Other: Case Report.

Hemolytic uremic syndrome (HUS) is defined by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury (AKI). Atypical HUS (aHUS), distinguished by its etiology, is caused by uncontrolled overactivation of the alternative complement pathway. The correct diagnosis of aHUS is complex and involves various gene mutations. Severe combined immunodeficiency (SCID), characterized by severe T-cell lymphocytopenia and a lack of antigen-specific T-cell and B-cell immune responses, is of seldom occurrence. In 10-15% of pediatric patients, SCID is caused by adenosine deaminase (ADA) deficiency. The authors describe the case of a boy who suffered from both aHUS and ADA-deficient SCID. At the age of 9 months, the patient presented acute kidney injury with anuria and coagulopathy. The diagnosis of aHUS was established on the basis of alternative complement pathway deregulation and disease-associated gene mutations. Further examination revealed immune system failure and, at the age of 13 months, the ADA deficiency was confirmed by genetic tests and the boy was diagnosed with ADA-SCID. ADA SCID has recently been described as a possible triggering factor of aHUS development and progression. However, more research is required in this field. Nevertheless, it is crucial in clinical practice to be aware of these two co-existing life-threatening diseases.

Ropivacaine has no advantage over bupivacaine in thoracic epidural analgesia for patients with pectus excavatum undergoing the Nuss procedure - a single blind randomized clinical trial comparing efficacy and safety.

INTRODUCTION: Pectus excavatum repair (Nuss procedure) is a painful procedure requiring effective postoperative analgesia. AIM: To establish whether thoracic epidural analgesia with ropivacaine is non-inferior to epidural analgesia with bupivacaine following the Nuss procedure in children. MATERIAL AND METHODS: The prospective, randomized, controlled, single blind study included 81 children. Computer-generated random numbers were used to allocate treatment. All children received general anesthesia. Intraoperative and postoperative analgesia was achieved with either 0.5% and 0.1% ropivacaine (group R) or 0.375% and 0.0625% bupivacaine (group B). The Numeric Rating Scale (NRS) and the Prince Henry Hospital Pain Score (PHHPS) were used to assess postoperative pain directly after and 1, 8, 20 and 24 hours after awakening from general anesthesia. NRS scores of more than 2 and a PHHPS score of more than 1 were considered as pain requiring intervention. Hemodynamic stability and side effects were also compared between the groups. RESULTS: The durations of the procedure and extubation times in groups R and B were similar (59 ±7 vs. 56 ±10 minutes and 9 ±5 vs. 10 ±5 minutes, respectively). Pain scores requiring intervention were below 10% and were recorded with similar frequency in both groups, except for one difference in the PHHPS score in favor of group R after 24 hours (12% vs. 40%, p < 0.05). The frequency of side effects and hemodynamic stability were similar in both groups. CONCLUSIONS: 0.1% epidural ropivacaine has no advantage over 0.0625% epidural bupivacaine for pectus excavatum repair in children.

Substance P and intensity of pruritus in hemodialysis and peritoneal dialysis patients.

BACKGROUND: Uremic pruritus is a common complication in patients undergoing dialysis. The pathophysiological mechanisms of pruritus in patients with end-stage renal disease remain unknown. Neuropeptides, including substance P, are postulated to play an important role in the pathogenesis of pruritus. The aim of this study was to evaluate the role of substance P in uremic pruritus in patients on hemodialysis and peritoneal dialysis. MATERIAL/METHODS: We included 197 patients with end-stage renal disease: 54 on continuous ambulatory peritoneal dialysis and 143 on hemodialysis. Substance P, calcium, phosphorus, iron, ferritin, CRP, albumin, hemoglobin, Ca×P product, and iPTH level were determined in all participants. The correlation between these parameters and self-reported itching was evaluated in patients on hemodialysis in comparison with peritoneal dialysis patients. RESULTS: The incidence of itching was similar in hemodialysis and peritoneal dialysis patients. No differences in substance P level between the 2 groups were found. There was no correlation between substance P level and the incidence or intensity of pruritus in dialyzed patients. CONCLUSIONS: This study demonstrates that substance P does not play any important role in pruritus in hemodialysed and peritoneal dialyzed patients. However, further studies are necessary to assess the exact role of neuropeptides in uremic pruritus.

Epidural and opioid analgesia following the Nuss procedure.

BACKGROUND: Parents have the right to decide on behalf of their children and deny consent to regional anaesthesia. The investigators decided to investigate quality of postoperative analgesia in adolescents undergoing epidural and opioid analgesia following the Nuss procedure. material/methods: The study subjects were 61 adolescents aged 11-18 years who underwent pectus excavatum repair with the Nuss procedure. Patients were divided into epidural (n=41) and opioid (n=20) groups, depending on their parents' consent to epidural catheter insertion. Intraoperatively, 0.5% epidural ropivacaine with fentanyl or intermittent intravenous injections of fentanyl were used. Postoperative analgesia was achieved with either epidural infusion of 0.1% ropivacaine with fentanyl, or subcutaneous morphine via an intraoperatively inserted "butterfly" cannula. Additionally, both groups received metamizol and paracetamol. Primary outcome variables were postoperative pain scores (Numeric Rating Scale and Prince Henry Hospital Pain Score). Secondary outcome variables included hemodynamic parameters, additional analgesia and side effects. RESULTS: Heart rate and blood pressure values in the postoperative period were significantly higher in the opioid group. Pain scores requiring intervention were noted almost exclusively in the opioid group. CONCLUSIONS: Denial of parental consent to epidural analgesia following the Nuss procedure results in significantly worse control of postoperative pain. Our data may be useful when discussing with parents the available anaesthetic techniques for exceptionally painful procedures.

Comparison of remifentanil or fentanyl administration during isoflurane anesthesia for coronary artery bypass surgery.

BACKGROUND: The combination of inhalational agents with moderate doses of fentanyl is popular in cardiac anesthesia. Remifentanil is a new opioid metabolized by non-specific esterases. The aim of our study was to assess whether remifentanil may be superior to fentanyl during isoflurane anesthesia for coronary artery surgery. MATERIAL/METHODS: Forty patients aged 40-70 years with stable coronary artery disease were randomly allocated to two groups: remifentanil was used in 20 patients (group I) and fentanyl was used in 20 patients (group II). Induction of anesthesia was performed with remifentanil infusion (0.5 microg/kg/min) or a bolus dose of fentanyl (5 microg/kg) followed by a bolus of etomidate and pancuronium. Maintenance of anesthesia was provided by isoflurane and infusion of the study opioid, with the rate adjusted according to systolic blood pressure values. Hemodynamic parameters were registered before and after the induction of anesthesia, skin incision, sternotomy, aortic cannulation, termination of cardiopulmonary bypass, chest closure, and skin closure. RESULTS: Heart rate and mean arterial pressure values were significantly lower in the remifentanil group, while systemic vascular resistance was higher in the fentanyl group in the measurements taken before the initiation of cardiopulmonary bypass. No differences were found between groups in the values of mean pulmonary pressure, pulmonary artery wedge pressure and transpulmonary pressure gradient, central venous pressure, and cardiac index. CONCLUSIONS: Remifentanil is safe during inhalation anesthesia for coronary artery surgery and it appears to be more effective than fentanyl in blunting hemodynamic response before the initiation of cardiopulmonary bypass surgery.