Does renal transplant in children with LUTD improve their bladder function?

Much is still unknown about LUT function after receiving renal graft. Graft function was the main focus of different studies discussing the same issue. However, these studies ignored the effects of the graft on lower tract function and more demand for bladder cycling and growth of the child. Therefore, we aimed at evaluating the LUT function after RT into patients with LUTD. We enrolled a retrospective cohort of 83 live renal transplant children with LUTD. The 44 patients in Group (A) had a defunctionalized bladder, and the 39 patients in Group (B) had underlying LUT pathology. All patients had clinical and urodynamic evaluation of LUT functions at least 1 year after RT. We found that the improvement in patients with impaired bladder compliance was 73% in Group (A) and 60% in Group (B), with no statistically significant difference between the study groups. In Group (B), there was statistically significant worsening of MFP (8.4%) and mean PVR (79.9%) after RT. In Group (A), mild but stable significant improvement of all clinical and urodynamic parameters was observed. Serum creatinine was significantly worse in patients with pathological LUTD compared with those with defunctionalized bladder but without significant effect on graft survival. All LUT variables seemed to have no adverse effect on graft survival except for use of CIC and augmented bladder. Incident UTI independent of LUT variables accounted for 20% of graft creatinine change.

Pediatric kidney transplantation in Egypt: Results of 10-year single-center experience.

Pediatric kidney transplantation is a multidisciplinary therapy that needs special consideration and experience. In this study, we aimed to present CUCH experience; over a 10-year period, as a specialized center of kidney transplantation in children. We studied 148 transplantations performed at a single center from 2009 to 2018. Pretransplant and follow-up data were collected and graft/patient survival rates were evaluated. A total of 48 patients developed at least one rejection episode during 688 patient-years of follow-up. Infections, recurrence of original disease, and malignancy were the most important encountered medical complications (20%, 2%, and 1.4%, respectively). One-year patient survival was 94.1%, while graft and patient survival was 91.9%. Graft/patient survival at 5, 7, and 9 years was 90%, 77%, and 58%, respectively. Infections were the main cause (69%) of mortality. Death with a functioning graft and CR were the main causes of graft loss (48% and 33%, respectively). Pediatric kidney transplantation in Egypt is still a challenging yet successful experience. Rejections and infections are the most frequent complications. Short-term outcomes surpass long-term ones and graft survival rates are similar to the international standard.

Combined liver-kidney transplantation for primary hyperoxaluria type I in children: Single Center Experience.

Primary hyperoxalurias are rare inborn errors of metabolism with deficiency of hepatic enzymes that lead to excessive urinary oxalate excretion and overproduction of oxalate which is deposited in various organs. Hyperoxaluria results in serious morbid-ity, end stage kidney disease (ESKD), and mortality if left untreated. Combined liver kidney transplantation (CLKT) is recognized as a management of ESKD for children with hyperoxaluria type 1 (PH1). This study aimed to report outcome of CLKT in a pediatric cohort of PH1 patients, through retrospective analysis of data of 8 children (2 girls and 6 boys) who presented by PH1 to Wadi El Nil Pediatric Living Related Liver Transplant Unit during 2001-2017. Mean age at transplant was 8.2 ± 4 years. Only three of the children underwent confirmatory genotyping. Three patients died prior to surgery on waiting list. The first attempt at CLKT was consecutive, and despite initial successful liver transplant, the girl died of biliary peritonitis prior to scheduled renal transplant. Of the four who underwent simultaneous CLKT, only two survived and are well, one with insignificant complications, and other suffered from abdominal Burkitt lymphoma managed by excision and resection anastomosis, four cycles of rituximab, cyclophosphamide, vincristine, and prednisone. The other two died, one due to uncontrollable bleeding within 36 hours of procedure, while the other died awaiting renal transplant after loss of renal graft to recurrent renal oxalosis 6 months post-transplant. PH1 with ESKD is a rare disease; simultaneous CLKT offers good quality of life for afflicted children. Graft shortage and renal graft loss to oxalosis challenge the outcome.

Risk factors for urological complications following living donor renal transplantation in children.

The aim of this study was to detect possible risk factors for UC and UTI following pediatric renal Tx and effect of these complications on outcome. One hundred and eight children who underwent living donor Tx between 2009 and 2015 were retrospectively included. Extraperitoneal approach was used with stented tunneled extravesical procedure. Mean recipient age was 9.89 ± 3.46 years while mean weight was 25.22 ± 10.43 kg. Seventy-three (67.6%) recipients were boys while 92 (85.2%) were related to donors. Urological causes of ESRD were present in 33 (30.6%) recipients (14 [13%] posterior urethral valve, 16 [14.8%] VUR, and 3 [2.8%] neurogenic bladder). Augmentation ileocystoplasty was performed in 9 (8.3%) patients. Mean follow-up was 39.3 ± 17.33 months. UC were detected in 10 (9.3%) children (leakage 4 [3.7%], obstruction 3 [2.8%], and VUR 3 [2.8%]) while UTIs were reported in 40 (37%) children. After logistic regression analysis, UC were significantly higher in children with cystoplasty (44.4% vs 6.1%; P = .001). UTIs were significantly higher in girls (51.4% vs 30.1%; P = .001) and in children with urological causes of ESRD (51.5% vs 30.7%; P = .049). UC and UTI were not significantly associated with increased graft loss or mortality. UC were significantly higher in children with cystoplasty while UTIs were significantly higher in girls and children with urological causes of ESRD. Presence of UC did not affect the rate of graft loss or mortality due to its early detection and proper management.

Levels of cystatin C in low- and high-flux hemodialysis in children with end-stage renal disease.

BACKGROUND: Cystatin-C (CyC) is a middle molecule that is freely filtered at the glomerulus and almost completely reabsorbed by the proximal tubules. The aim of this study was to evaluate serum CyC and its reduction ratio as a biomarker for assessing the adequacy of the hemodialysis (HD) sessions in children with end-stage renal disease on maintenance HD. We also compared levels of CyC in patients on low-flux HD (LFH) and high-flux HD (HFH). METHODS: Forty patients were included in the study and divided into two groups, with one group (16 patients) receiving HFH and the other group receiving LFH (24 patients) (high-flux and low-flux polysulfone filters, respectively). Before and after each dialysis session serum CyC and beta-2-microglobulin (B2M) levels were measured using an ELISA technique, and routine laboratory tests were performed for each patient. RESULTS: Pre-dialytic levels of CyC were significantly lower in the patients receiving HFH than in those receiving LFH (7.33 ± 1.35 vs. 9.73 ± 0.93, respectively; p < 0.0001). In the HFH group, post-dialytic levels of serum CyC were significantly lower than pre-dialytic levels (4.49 ± 0.71 vs. 7.33 ± 1.35, respectively; p < 0.0001). The reduction ratio (RR) of CyC was significantly higher in the HFH group than in the LFH group (38.2 ± 3.91 vs. -6.49 ± 5.05, respectively; p < 0.0001). Serum CyC level significantly correlated with B2M, urea and creatinine levels in both the LFH and HFH groups, whereas its RR significantly correlated with the RRs of urea, creatinine, and B2M in the HFH group. CONCLUSION: The results of our study emphasize the role of CyC as a good marker for assessing the adequacy of HD sessions in children on HFH and show that the CyC RR may be used as an index of middle-molecule toxin clearance following HFH sessions.

Outcomes of living donor renal transplantation in children with lower urinary tract dysfunction: a comparative retrospective study.

OBJECTIVES: To compare outcomes of renal transplantation (RTx) in children with end-stage renal disease (ESRD) resulting from lower urinary tract dysfunction (LUTD) vs other causes. PATIENTS AND METHODS: A database of children (<18 years old) who underwent RTx between May 2008 and April 2012 was reviewed. Patients were divided into those with LUTD (group A, n = 29) and those with other causes of ESRD (group B, n = 74). RTx was performed after achieving low intravesical pressure (<30 cmH2 O) with adequate bladder capacity and drainage. The groups were compared using Student's t-test, Mann-Whitney, chi-squared or exact tests. Graft survival rates (GSRs) were evaluated using Kaplan-Meier curves and the log-rank test. RESULTS: The mean ± sd (range) age of the study cohort was 5.05 ± 12.4 (2.2-18) years. Causes of LUTD were posterior urethral valve (PUV; 41.4%), vesico-ureteric reflux (VUR; 37.9%), neurogenic bladder (10.3%), prune belly syndrome (3.4%), obstructive megaureter (3.4%) and urethral stricture disease (3.4%). There was no significant difference in age, dialysis duration or donor type. In group A, 25 of the 29 patients (86.2%) underwent ≥1 surgery to optimize the urinary tract for allograft. Pretransplant nephrectomy was performed in 15 of the 29 patients (51.7%), PUV ablation in nine patients (31%) and ileocystoplasty in four patients (13.7%). The mean ± sd follow-up was 4.52 ± 1.55 and 4.07 ± 1.27 years in groups A and B, respectively. There was no significant difference in creatinine and eGFR between the groups at different points of follow-up. The GSRs at the end of the study were 93.1 and 91.1% in groups A and B, respectively (P = 1.00). According to Kaplan-Meier survival curves, there was no significant difference in the GSR between the groups using the log-rank test (P = 0.503). No graft was lost as a result of urological complications. In group B, one child died from septicaemia. The rate of urinary tract infections was 24 and 12% in groups A and B, respectively, but was not significant. No significant difference was found between the groups with regard to the incidence of post-transplantation hydronephrosis. Of the 22 patients who had hydronephrosis after transplantation, three were complicated by UTI. Injection of bulking agents was required in two patients for treatment of grade 3 VUR. In the third patient, augmentation cystoplasty was needed. CONCLUSION: Acceptable graft function, survival and UTI rates can be achieved in children with ESRD attributable to LUTD. Thorough assessment and optimization of LUT, together with close follow-up, are key for successful RTx.

Caudal extradural catheterization in pediatric renal transplant and its effect on perioperative hemodynamics and pain scoring: a prospective randomized study.

PURPOSE: 'Hockey stick incision' used in renal transplant is large enough to cause severe postoperative morbidity especially in pediatric recipients. Although epidural analgesia is known to be effective in pain control, the resulting sympathectomy might affect hemodynamics interfering with the transplant process. In our study, we evaluated the feasibility and safety of inserting an epidural catheter to the thoracic level via the caudal route, and the effect of using epidural local anesthetics at low concentrations on hemodynamics. METHODS: After approval from the ethical committee at Kasr Al Ainy University Hospital and consent from parents/legal guardians, sixty patients aged 3-12 years who were scheduled for renal transplant were randomly divided into two equal groups. Group I (epidural group) received continuous caudal epidural bupivacaine 0.125 % with fentanyl together with intravenous (IV) fentanyl and paracetamol. Group II (control group) received only IV fentanyl and paracetamol. Intraoperative data included heart rate (HR), mean arterial blood pressure (MAP) and central venous pressure (CVP). Postoperative variables included HR, MAP, CVP, pain score and complications. RESULTS: Threading failure via the caudal route occurred in 6.67 % of cases. Intraoperative differences in hemodynamics and CVP were not clinically significant between groups. Postoperative HR, MAP, and CVP were generally higher in the control group. Pain control was more satisfactory and postoperative complications were less in the epidural group. CONCLUSION: Caudal epidural anesthesia in pediatric renal transplant is a valuable addition to general anesthesia as it provides stable perioperative hemodynamics, excellent postoperative analgesia and is associated with fewer complications than narcotic-dependent analgesia. CLINICAL TRIAL REGISTRATION NUMBER: NCT02037802.

Clinical and ultrasonographical characterization of childhood cystic kidney diseases in Egypt.

BACKGROUND: Renal cystic disorders (RCD) constitute an important and leading cause of end-stage renal disease (ESRD) in children. It can be acquired or inherited; isolated or associated with extrarenal manifestations. The precise diagnosis represents a difficult clinical challenge. METHODS: The aim of this study was to define the pattern of clinical phenotypes of children with renal cystic diseases in Pediatric Nephrology Center, Cairo University. We have studied the clinical phenotypes of 105 children with RCD [45 (43%) of them had extrarenal manifestations]. RESULTS: The most common disorders were the presumably inherited renal cystic diseases (65.7%) mainly nephronophthisis and related ciliopathies (36.2%), as well as polycystic kidney diseases (29.5%). Moreover, multicystic dysplastic kidneys accounted for 18% of study cases. Interestingly, eight syndromic cases are described, yet unclassified as none had been previously reported in the literature. CONCLUSION: RCD in this study had an expanded and complex spectrum and were largely due to presumably inherited/genetic disorders (65.7%). Moreover, we propose a modified algorithm for clinical and diagnostic approach to patients with RCD.

Antimicrobial resistance landscape and COVID-19 impact in Egypt, Iraq, Jordan, and Lebanon: A survey-based study and expert opinion.

OBJECTIVES: The objective of this study was to assess the antimicrobial resistance (AMR) landscape and the impact of COVID-19 on AMR in Egypt, Iraq, Jordan, and Lebanon, and to gather expert opinions on the barriers to the implementation of antimicrobial stewardship (AMS) initiatives in the region. METHODS: A cross-sectional questionnaire survey was used to assess the current AMR landscape, existing AMS initiatives, barriers to implementing AMS initiatives, and the impact of COVID-19 on AMR in the four countries. RESULTS: The survey was completed by 204 physicians from Egypt (n = 82), Lebanon (n = 49), Iraq (n = 43), and Jordan (n = 30). Previous antibiotic use and previous bacterial colonization were perceived as the most common risk factors for an increase in AMR. According to the survey, multidrug-resistant (MDR) gram-negative bacteria were most common in lower respiratory tract infections, and Klebsiella pneumoniae and Escherichia coli were the most commonly identified gram-negative bacteria in hospital-acquired infections. Only 14.8% of pediatric physicians and 28.6% of adult physicians reported that target pathogen genotyping and phenotyping were done in hospitals, and the most commonly reported reasons for the lack of testing were technological and resource constraints. These constraints, coupled with the scarcity and high cost of newer antibiotics, have been identified as the most significant barriers to the successful management of MDR gram-negative bacterial infections in the region. It was reported that the spectrum of activity and safety of the antibiotic, the site of infection, the presence of comorbidities, and published guidelines and local antibiograms determined the choice of empirical antibiotic therapy for patients in the region. The four countries experienced a significant rise in AMR due to several factors during the COVID-19 pandemic, including an increase in hospital occupancy, a shift in priorities away from AMR surveillance, and changes in AMR epidemiology. Additionally, the large volumes of unnecessary and unsubstantiated antibiotic prescriptions during the COVID-19 pandemic has led to subsequent antibiotic shortages and significant increases in AMR in the region. Physicians also noted that the majority of COVID-19 patients were already on antibiotics before visiting the healthcare facility. MDR gram-negative bacteria were found in the majority of COVID-19 patients admitted to the intensive care unit. Despite the fact that various AMS initiatives have been implemented, they are not standardized across the region. Some of the main barriers to AMS implementation in the region are a lack of adequately trained AMS staff, lack of AMS knowledge and training among healthcare professionals, financial constraints, and the lack of AMR surveillance systems. CONCLUSION: These survey results provide valuable insights into the existing AMR and AMS landscape in the region, as well as the barriers that impede efficient AMS and AMR management. Based on these findings, the authors developed a call to action that suggests ways for each country in the region to address these challenges.

Renal involvement in childhood-onset systemic lupus erythematosus in Egypt.

Lupus nephritis has been described as the most serious complication of systemic lupus erythematosus (SLE) and the strongest predictor of poor outcome. While the incidence of childhood SLE is relatively low, renal involvement appears to be more common and more severe in childhood SLE. This study aims to characterize the features and outcome of renal involvement in childhood-onset SLE based on a study of 100 Egyptian patients (mean age at diagnosis 10.1 years, range 2-17 years). Initial data regarding disease manifestations and biopsy findings were reviewed. Disease activity was assessed using SLEDAI scores. Follow-up data (mean duration 6 years) were noted regarding specific treatment, response, complications and renal survival. Initial renal involvement was present in 78 patients, including 66 with hypertension and 23 with renal impairment. Pathologically, class IV nephropathy was found in 18 patients, class V in 9 and low-grade lesions (class II-III) in 49. Twenty patients required follow-up biopsy, and all transformations were observed. SLEDAI scores significantly decreased from initial (mean ± SD) of 21.4 ± 7.3 to 13.4 ± 7.8, in association with response to therapy (P < 0.0001). Poor response was associated with initial hypertension and renal impairment but not with initial SLEDAI score or pathological class. The projected renal survival was 82.4 and 64.7% 5 and 10 years from diagnosis. Early renal involvement in childhood SLE is common, serious and requires proper evaluation and management.

Profile of patients visiting the pediatric emergency service in an Egyptian university hospital.

BACKGROUND: The emergency department (ED) is an essential component of the medical service offered in any hospital. Yet, the published information about patients' profile and utilization of emergency services in both developing and developed countries is scarce. AIM: This study aimed to characterize the presentation and outcome of patients visiting the ED of a university-based tertiary pediatric hospital. METHODS: This is a descriptive study reporting the profile of patients visiting the ED for 1 year. RESULTS: The total number of patients visiting the hospital was 249,552, with a monthly average of 20,046 patients, including 3639 monthly ED visits. "Treat-and-release" cases represented 46.7% and infants represented the largest age group (44.4%). The main presentation was respiratory distress (24.4%) and wheezy chest (17.3%), followed by convulsions (15%) and then pallor (10.7%). Long-term illnesses were predominantly congenital heart diseases, cerebral palsy, chronic hemolytic anemia, and asthma. The overall mortality rate was 0.8%. CONCLUSIONS: Appropriate and ongoing data collection and analysis could guide more efficient utilization of pediatric emergency services to achieve better outcomes.

Assessment of Tissue Perfusion Using the Peripheral Perfusion Index and Lactate Clearance in Shock in Pediatric Patients.

BACKGROUND: Pediatric shock has a high mortality rate because many of the early clinical signs are subtle and have poor sensitivity and specificity. Pediatric shock was categorized either: compensated with normal blood pressure, poor skin perfusion (CRT >2 s, mottled, cool peripheries, peripheral cyanosis), weak peripheral pulse, age specific tachycardia, tachypnoea, and oliguria or decompensated with hypotension (SBP < 70 + (2× age in years) mm Hg and decreased mental status. The perfusion index is a non-invasive method for assessing peripheral perfusion and may be a useful marker for identifying shock early in pediatric patients. OBJECTIVE: This prospective cohort study (November 2019 to August 2020) evaluated whether the perfusion index, lactate, and/or lactate clearance could predict mortality among pediatric shock patients. METHODS: Fifty children (68% male) with shock underwent assessments at presentation to the emergency room to evaluate their heart rate, blood pressure, capillary refill time, central venous pressure, perfusion index, cardiac index, systemic vascular resistance, central venous oxygen saturation, and lactate clearance. RESULTS: The perfusion index range was 0.03 to 2.2 and ≤0.18 as the cut-off for mortality prediction providing 74% sensitivity and 78% specificity. The serum lactate concentration range was 0 to 16 mmol/L and >5.7 mmol/L as the cut-off for mortality prediction provided 70% sensitivity and 96% specificity at presentation to the emergency room. The lactate clearance range was 3% to 75% and >10% as the cut-off for survival prediction after resuscitation and at 6 h later. CONCLUSION: Perfusion index (PI), lactate, and lactate clearance provided comparable sensitivity and specificity for predicting outcomes among pediatric patients with shock Therefore, we suggest that the PI is an inexpensive, rapid, and non-invasive tool that can be used to predict illness severity and mortality in busy pediatric intensive care units and emergency departments. This tool may guide better patient triage and an earlier diagnosis of shock in this setting.

Prognostic value of baseline carotid blood flow in critically ill children with septic shock.

BACKGROUND AND AIM: Hemodynamic monitoring and cardiac output (CO) assessment in the ICU have been trending toward less invasive methods. Carotid blood flow (CBF) was suggested as a candidate for CO assessment. The present study aimed to test the value of carotid artery ultrasound analysis in prediction of mortality in pediatric patients with septic shock. METHODOLOGY/PRINCIPAL FINDING: Forty children with septic shock were included in the study. Upon admission, patients were subjected to careful history taking and thorough clinical examination. The consciousness level was assessed by the Glasgow Coma Scale (GCS). Laboratory assessment included complete blood count, C-reactive protein, arterial blood gases, serum electrolytes, and liver and kidney function tests. Electrical cardiometry was used to evaluate hemodynamic parameters. Patients were also subjected to transthoracic 2-D echocardiography. CBF was evaluated using GE Vivid S5 ultrasound device through dedicated software. At the end of study, 14 patients (35.0%) died. It was found that survivors had significantly higher CBF when compared non-survivors [median (IQR): 166.0 (150.0-187.3) versus 141.0 (112.8-174.3), p = 0.033]. In addition, it was noted that survivors had longer ICU stay when compared with non-survivors [16.5 (9.8-31.5) versus 6.5 (3.0-19.5) days, p = 0.005]. ROC curve analysis showed that CBF could significantly distinguish survivors from non-survivors [AUC (95% CI): 0.3 (0.11-0.48), p = 0.035] (Fig 2). Univariate logistic regression analysis identified type of shock [OR (95% CI): 28.1 (4.9-162.4), p<0.001], CI [OR (95% CI): 0.6 (0.43-0.84), p = 0.003] and CBF [OR (95% CI): 0.98 (0.96-0.99), p = 0.031]. However, in multivariate analysis, only type of shock significantly predicted mortality. CONCLUSIONS: CBF assessment may be a useful prognostic marker in children with septic shock.

Pediatric focal segmental glomerulosclerosis: favorable transplantation outcome with plasma exchange.

BACKGROUND: Although kidney transplantation (KTX) is the treatment of choice for pediatric end stage kidney disease (ESKD); concerns for recurrence in cases of focal segmental glomerulosclerosis (FSGS) are still present. This study aimed to investigate the outcome of KTX in children with ESKD secondary to FSGS, with implementation of preemptive perioperative plasma exchange (PE) for non-genetically proven patients. METHODS: Forty FSGS pediatric kidney transplant recipients were studied. Of them: 12 patients (30%) had genetically proven NPHS2 mutations/familial and 28 (70%) were sporadic FSGS patients. All sporadic patients electively received 6 perioperative PE sessions. Patients with recurrence of proteinuria (n = 13; including 3 patients with genetic/familial and 10 patients with sporadic FSGS) were managed with PE and Rituximab (RTX). Kaplan-Meier curves were used to analyze graft and recurrence free survival data. RESULTS: The mean follow-up duration after KTX was 3.8 ± 2.86 years. Recurrence of proteinuria was encountered early postoperative in 11 patients (27.5%) and late (1.6 and 2.9 years after KTX) in 2 patients (5%). All patients with early recurrence achieved complete remission, while patients with late recurrence developed graft failure. Current serum creatinine and proteinuria levels were not different in patients received PE (n = 31) and patients did not PE (n = 9) (p = 0.308 and 0.287 respectively). Current serum creatinine and proteinuria levels in sporadic patients (n = 28) after prophylactic perioperative PE were not different from those of genetic/ familial patients (n = 12) (p = 0.303 and 0.144 respectively). Proteinuria was less in patients underwent native nephrectomy than others immediately postoperative and at assessment (p = 0.002 & 0.0031 respectively). One-year graft and patient survival was 93.8% with a mean 1-year serum creatinine of 0.67 ± 0.25 mg/dl. Three graft losses (7.5%) were due to chronic rejection 3.3, 3.75 and 4.17 years after KTX and 2 patients' mortality (5%) occurred early postoperative (first 2 weeks). CONCLUSION: FSGS transplanted children have favorable outcomes with perioperative PE for non-genetically proven cases. Early recurrence after KTX can be successfully managed with PE and RTX.

Glucose tolerance in a cohort of Egyptian children after kidney transplantation.

BACKGROUND: Post- transplantation diabetes mellitus (PTDM) in children is a serious metabolic complication that can endanger both graft and patient survival. These complications can be partially reduced by early diagnosis & prompt treatment of impaired glucose tolerance. The aim of this study was to assess glucose tolerance & insulin resistance among a cohort of kidney transplanted children. METHODS: Thirty consecutive pediatric kidney transplant recipients were subjected to basal evaluation of plasma glucose and insulin then underwent oral glucose tolerance test (OGTT). RESULTS: Abnormal glucose metabolism was detected in 7 (23.3%) patients; 3 (10%) patients with PTDM; 3 (10%) patients with impaired fasting glucose (IFG) and 1 (3.3%) patient with IFG and impaired glucose tolerance (IGT). Four (13.3%) patients had high Homeostatic model assessment of insulin resistance (HOMA-IR). Patients with abnormal glucose metabolism had significantly higher tacrolimus trough levels and higher maintainence steroid doses (p values = 0.003,0.026). Significant positive correlation existed between pre-transplantation glucose level and post-transplantation fasting glucose (p = 0.001, r = 0.69), glucose at 120 min (p = 0.018, r = 0.429) and HOMA-IR (p = 0.008, r = 0.47). CONCLUSION: Abnormalities in glucose metabolism (IFG, IGT &PTDM) are frequent in Egyptian pediatric kidney transplant recipients. OGTT is the gold standard for assessment of abnormalities in glucose metabolism.

The effect of high fat dietary modification and nutritional status on the outcome of critically ill ventilated children: single-center study.

BACKGROUND: Ventilator dependency constitutes a major problem in the intensive care setting. Malnutrition is considered a major determinant of extubation failure, however, attention has been attracted to modulating carbon dioxide production through decreasing carbohydrate loading and increasing the percent of fat in enteral feeds. The detected interrelation between substrate oxidation and ventilation outcome became the base of several research to determine the appropriate composition of the nonprotein calories of diet in ventilated patients. PURPOSE: We aimed to assess the effect of high-fat dietary modification and nutritional status on ventilatory and final outcomes of pediatric intensive care. METHODS: Fifty-one ventilated children (1 month to 12 years of age) with pulmonary disease who could be enterally fed, in the Cairo University Pediatric intensive care unit, were divided into 2 groups: group A included 25 patients who received isocaloric high-fat, low-carbohydrate diet; group B included 26 patients who received standard isocaloric diet. Comprehensive nutritional assessment was done for all patients. RESULTS: Group A had a significant reduction in carbon dioxide tension, but no similar reduction in the duration or level of ventilatory support. Assisted minute ventilation was predicted by weight-for-age and caloric intake rather than the type of diet. Poor nutritional status was associated with higher mortality and lower extubation rates. Mild hypertriglyceridemia and some gastrointestinal intolerance were significant in group A, with no impact on the adequacy of energy or protein delivery. CONCLUSION: The high-fat enteral feeding protocol may contribute to reducing carbon dioxide tension, with mild hypertriglyceridemia and negligible gastrointestinal intolerance as potential adverse effects. Optimization of nutritional status rather than dietary modification may improve ventilatory and survival outcomes in critically ill-ventilated children.

Initial Response and Outcome of Critically Ill Children With Guillain Barre' Syndrome.

Background: Guillain-Barre syndrome is the most common cause of acute flaccid paralysis worldwide since the eradication of poliomyelitis. Severe cases may require intensive care and mechanical ventilation. Purpose: was to study pediatric patients with severe GBS requiring intensive care unit (ICU) admission, to assess their course and response to initial treatment modality plasma exchange (PE) or intravenous immunoglobulins (IVIg) and their final outcome. Methods: children with severe GBS who had either actual or impending respiratory failure, bulbar involvement or rapid progression of acute flaccid paralysis with trunk, upper limb and neck involvement within 24 h of the onset of weakness were enrolled. Results: 40 children were included. Following the initial treatment (33 subjects had 5 PE sessions each and IVIg in 7), 16 patients improved (40%), two died and 22 (55%) showed initial treatment failure. Axonal neuropathy, rapid progression and severe motor weakness significantly predicted poor response to therapy. At discharge, favorable outcomes (patient can walk unaided) were present in 22 cases (58%). Conclusion: Despite relatively low mortality, critically ill children with severe GBS have increased prevalence of axonal neuropathy and guarded response to initial therapy with PE or IVIg.

Central venous catheters as a vascular access modality for pediatric hemodialysis.

BACKGROUND: The use of hemodialysis catheters is an essential component of dialysis practice. Children are particularly likely to require multiple courses of dialysis over their lifetime, hence the repeated need for vascular access. These catheters remain a significant source of morbidity and mortality. METHODS: All catheters inserted for hemodialysis at the Center of Pediatric Nephrology and Transplantation, Cairo University over a period of 40 months were studied. Patient data as well as data of catheter insertion, dwell, cause of removal and complications were reported. RESULTS: A total of 195 uncuffed central venous catheters were used for temporary access in 131 patients for a mean duration of 35.7 days. Of attempted insertions, 87.4% achieved successful access, of which 56% remained for the required period, 8.9% were accidentally dislodged, and 35.1% were removed due to complications--mostly infection. The overall rate of possible catheter-related bacteremia was 9.6 episodes/ 1,000 catheter days. Infection increased with longer catheter dwell. Nineteen cuffed tunneled catheters were surgically inserted and used for up to 11 months (mean 117 days). Loss of these catheters was attributed mainly to infection (ten episodes) and catheter thrombosis (six episodes). During the study, 317 femoral catheters were inserted. CONCLUSION: Uncuffed central venous catheters are both needed and useful for short-term hemodialysis. Vascular access for extended durations may be provided by cuffed tunneled catheters. Infection is the major serious concern with both uncuffed and cuffed catheters.

Clinical spectrum of primary hyperoxaluria type 1: Experience of a tertiary center.

BACKGROUND AND AIM: Primary hyperoxalurias are rare inborn errors of metabolism resulting in increased endogenous production of oxalate that leads to excessive urinary oxalate excretion. Diagnosis of primary hyperoxaluria type 1 (PH1) is a challenging issue and depends on diverse diagnostic tools including biochemical analysis of urine, stone analysis, renal biopsy, genetic studies and in some cases liver biopsy for enzyme assay. We characterized the clinical presentation as well as renal and extrarenal phenotypes in PH1 patients. METHODS: This descriptive cohort study included patients with presumable PH1 presenting with nephrolithiasis and/or nephrocalcinosis (NC). Precise clinical characterization of renal phenotype as well as systemic involvement is reported. AGXT mutational analysis was performed to confirm the diagnosis of PH1. RESULTS: The study cohort included 26 patients with presumable PH1 with male to female ratio of 1.4:1. The median age at time of diagnosis was 6 years, nevertheless the median age at initial symptoms was 3 years. Thirteen patients (50%) were diagnosed before the age of 5 years. Two patients had no symptoms and were diagnosed while screening siblings of index patients. Seventeen patients (65.4%) had reached end-stage renal disease (ESRD): 6/17 (35.3%) during infancy, 4/17 (23.5%) in early childhood and 7/17 (41.29%) in late childhood. Two patients (7.7%) had clinically manifest extra renal (retina, heart, bone, soft tissue) involvement. Mutational analysis of AGXT gene confirmed the diagnosis of PH1 in 15 out of 19 patients (79%) where analysis had been performed. Fifty percent of patients with maintained renal functions had projected 10 years renal survival. CONCLUSION: PH1 is a heterogeneous disease with wide spectrum of clinical, imaging and functional presentation. More than two-thirds of patients presented prior to the age of 5 years; half of them with the stormy course of infantile PH1. ESRD was the commonest presenting manifestation in two-thirds of our cohort.

Lymphocyte Activation Markers in Pediatric Kidney Transplant Recipients.

BACKGROUND AND OBJECTIVES: The role of CD4+CD25+ T regulatory cells (Tregs) in immune tolerance in experimental transplantation is very important but the clinical significance of circulating Tregs in the peripheral blood is undetermined. We evaluated the association between the frequency of T cell activation markers CD25 and CD71 and clinical parameters that may affect the level of these T cell markers. METHODS: In 47peditric kidney transplant (KT) recipients and 20 healthy controls, the frequency of T cell activation markers, CD25 and CD71 was measured with flow cytometry after transplantation. Two clinical protocols of induction immunosuppression were used: (1) anti-thymocyte globulin (THYMO) group (n =29) and Basiliximab (BSX) group (n=10). RESULTS: The percentage of circulating CD25 after KT was significantly lower than that in the controls. There is no significant difference between KT and the controls s regard to circulating CD71. The percentage of CD25 was significantly increased in children with acute rejection compared with those without acute rejection. Calcineurin inhibitors (CNIs) decreased the frequency of CD25 but mammalian target rapamycin (mTOR) inhibitor did not. The proportion of CD25 significantly decreased in THYMO group during the first year after transplantation. CONCLUSION: The frequency of circulating T cell activation marker CD25 in pediatric KT recipients is strongly affected by CNIs, and a high frequency of CD25 is associated with acute rejection during the early posttransplant period. The measurement of T cell activation markers, may become a useful immune monitoring tool after kidney transplantation.