Syndrome In Pediatric Patients Research Articles

Using liver elastography to diagnose sinusoidal obstruction syndrome in pediatric patients undergoing hematopoetic stem cell transplant.

Sinusoidal obstruction syndrome (SOS) is a potentially fatal complication of hematopoietic stem cell transplantation (HSCT). Traditional ultrasound (US) has poor sensitivity and specificity. US shear wave elastography (SWE) is a newer technology that measures liver stiffness. This is a single-institution, prospective cohort study evaluating SWE in patients younger than 21 years who received HSCT from December 2015 through June 2017. SOS was defined using the modified Seattle criteria. Subjects had US with SWE at three scheduled time points. t-tests were used to assess for difference between the groups and ROC curves were generated. Twenty-five patients were included. Five subjects developed SOS. At day +5 HSCT, SOS patients had SWE velocities that increased by 0.25 ± 0.21 m/s compared to 0.02 ± 0.18 in patients without SOS (p = 0.020). At day +14, SOS patients had SWE velocities that significantly increased by 0.91 m/s ± 1.14 m/s compared to 0.03 m/s ± 0.23 m/s in patients without SOS (p = 0.010). SWE SOS diagnosis occurred on average 9 and 11 days before clinical and conventional US diagnosis, respectively. Patients who develop SOS have increased liver stiffness compared to patients who do not develop SOS. SWE changes occur before other imaging and clinical findings of SOS.

Outcomes following operative management of thoracic outlet syndrome in the pediatric patients.

Objectives Thoracic outlet syndrome, a condition commonly reported in adults, occurs infrequently in the pediatric population. The objective of this study was to assess the outcome of surgical interventions of thoracic outlet syndrome in pediatric patients. Methods Clinical records of all pediatric patients with thoracic outlet syndrome who underwent operative repair from 2002 to 2015 in a tertiary pediatric hospital were reviewed. Pertinent clinical variables and treatment outcomes were analyzed. Results Sixty-eight patients underwent a total of 72 thoracic outlet syndrome operations (mean age 15.7 years). Venous, neurogenic, and arterial thoracic outlet syndromes occurred in 39 (57%), 21 (31%), and 8 (12%) patients, respectively. Common risk factors for children with venous thoracic outlet syndrome included sports-related injuries (40%) and hypercoagulable disorders (33%). Thirty-five patients (90%) with venous thoracic outlet syndrome underwent catheter-based interventions followed by surgical decompression. All patients underwent first rib resection with scalenectomy via either a supraclavicular approach (n = 60, 88%) or combined supraclavicular and infraclavicular incisions (n = 8, 12%). Concomitant temporary arteriovenous fistula creation was performed in 14 patients (36%). Three patients with arterial thoracic outlet syndrome underwent first rib resection with concomitant subclavian artery aneurysm repair. The mean follow-up duration was 38.4 ± 11.6 months. Long-term symptomatic relief was achieved in 94% of patients. Conclusions Venous thoracic outlet syndrome is the most common form of thoracic outlet syndrome in children, followed by neurogenic and arterial thoracic outlet syndromes. Competitive sports-related injuries remain the most common risk factor for venous and neurogenic thoracic outlet syndromes. Temporary arteriovenous fistula creation was useful in venous thoracic outlet syndrome patients in selective children. Surgical decompression provides durable treatment success in children with all subtypes of thoracic outlet syndrome.

Guillain Barre syndrome in pediatric patients: Is the electrophysiological pattern different than the adult

Guillain Barre syndrome in pediatric patients: Is the electrophysiological pattern different than the adult

Prophylactic levosimendan for the prevention of low cardiac output syndrome and mortality in paediatric patients undergoing surgery for congenital heart disease.

Low cardiac output syndrome remains a serious complication, and accounts for substantial morbidity and mortality in the postoperative course of paediatric patients undergoing surgery for congenital heart disease. Standard prophylactic and therapeutic strategies for low cardiac output syndrome are based mainly on catecholamines, which are effective drugs, but have considerable side effects. Levosimendan, a calcium sensitiser, enhances the myocardial function by generating more energy-efficient myocardial contractility than achieved via adrenergic stimulation with catecholamines. Thus potentially, levosimendan is a beneficial alternative to standard medication for the prevention of low cardiac output syndrome in paediatric patients after open heart surgery. To review the efficacy and safety of the postoperative prophylactic use of levosimendan for the prevention of low cardiac output syndrome and mortality in paediatric patients undergoing surgery for congenital heart disease. We identified trials via systematic searches of CENTRAL, MEDLINE, Embase, and Web of Science, as well as clinical trial registries, in June 2016. Reference lists from primary studies and review articles were checked for additional references. We only included randomised controlled trials (RCT) in our analysis that compared prophylactic levosimendan with standard medication or placebo, in infants and children up to 18 years of age, who were undergoing surgery for congenital heart disease. Two review authors independently extracted data and assessed risk of bias according to a pre-defined protocol. We obtained additional information from all but one of the study authors of the included studies. We used the five GRADE considerations (study limitations, consistency of effect, imprecision, indirectness, and publication bias) to assess the quality of evidence from the studies that contributed data to the meta-analyses for the prespecified outcomes. We created a 'Summary of findings' table to summarise the results and the quality of evidence for each outcome. We included five randomised controlled trials with a total of 212 participants in the analyses. All included participants were under five years of age. Using GRADE, we assessed there was low-quality evidence for all analysed outcomes. We assessed high risk of performance and detection bias for two studies due to their unblinded setting. Levosimendan showed no clear effect on risk of mortality (risk ratio (RR) 0.47, 95% confidence interval (CI) 0.12 to 1.82; participants = 123; studies = 3) and no clear effect on low cardiac output syndrome (RR 0.64, 95% CI 0.39 to 1.04; participants = 83; studies = 2) compared to standard treatments. Data on time-to-death were not available from any of the included studies.There was no conclusive evidence on the effect of levosimendan on the secondary outcomes. The length of intensive care unit stays (mean difference (MD) 0.33 days, 95% CI -1.16 to 1.82; participants = 188; studies = 4), length of hospital stays (MD 0.26 days, 95% CI -3.50 to 4.03; participants = 75; studies = 2), duration of mechanical ventilation (MD -0.04 days, 95% CI -0.08 to 0.00; participants = 208; studies = 5), and the risk of mechanical circulatory support or cardiac transplantation (RR 1.49, 95% CI 0.19 to 11.37; participants = 60; studies = 2) did not clearly differ between the groups. Published data about adverse effects of levosimendan were limited. A meta-analysis of hypotension, one of the most feared side effects of levosimendan, was not feasible because of the heterogeneous expression of blood pressure values. The current level of evidence is insufficient to judge whether prophylactic levosimendan prevents low cardiac output syndrome and mortality in paediatric patients undergoing surgery for congenital heart disease. So far, no significant differences have been detected between levosimendan and standard inotrope treatments in this setting.The authors evaluated the quality of evidence as low, using the GRADE approach. Reasons for downgrading were serious risk of bias (performance and detection bias due to unblinded setting of two RCTs), serious risk of inconsistency, and serious to very serious risk of imprecision (small number of included patients, low event rates).

Historia del síndrome de apnea-hipopnea obstructiva del sueño (SAHOS)

La historia de la apnea obstructiva del sueño es larga y complicada desde sus primeras descripciones en el siglo XIX con el relato de Pickwick en la primera novela de Charles Dickens. La definición de este término por parte de diferentes investigadores resalta la importancia de comprender las entidades asociadas al síndrome, como la hipoventilación alveolar y la somnolencia diurna excesiva. De esta forma, los estudios neurofisiológicos adelantados desde entonces, el desarrollo de la conferencia de la sociedad europea de neurología de 1964, la descripción del síndrome en pacientes pediátricos, la confirmación de la comorbilidad cardiovascular asociada a la enfermedad, la traqueostomía como tratamiento y la creación de implantes de estimuladores del nervio hipogloso —pasando por el descubrimiento de Sullivan de la presión positiva como tratamiento en casa— han sido esenciales para la comprensión de este síndrome.

Prophylactic levosimendan for the prevention of low cardiac output syndrome and mortality in paediatric patients undergoing surgery for congenital heart disease.

Low cardiac output syndrome remains a serious complication, and accounts for substantial morbidity and mortality in the postoperative course of paediatric patients undergoing surgery for congenital heart disease. Standard prophylactic and therapeutic strategies for low cardiac output syndrome are based mainly on catecholamines, which are effective drugs, but have considerable side effects. Levosimendan, a calcium sensitiser, enhances the myocardial function by generating more energy-efficient myocardial contractility than achieved via adrenergic stimulation with catecholamines. Thus potentially, levosimendan is a beneficial alternative to standard medication for the prevention of low cardiac output syndrome in paediatric patients after open heart surgery. To review the efficacy and safety of the postoperative prophylactic use of levosimendan for the prevention of low cardiac output syndrome and mortality in paediatric patients undergoing surgery for congenital heart disease. We identified trials via systematic searches of CENTRAL, MEDLINE, Embase, and Web of Science, as well as clinical trial registries, in June 2016. Reference lists from primary studies and review articles were checked for additional references. We only included randomised controlled trials (RCT) in our analysis that compared prophylactic levosimendan with standard medication or placebo, in infants and children up to 18 years of age, who were undergoing surgery for congenital heart disease. Two review authors independently extracted data and assessed risk of bias according to a pre-defined protocol. We obtained additional information from all but one of the study authors of the included studies. We used the five GRADE considerations (study limitations, consistency of effect, imprecision, indirectness, and publication bias) to assess the quality of evidence from the studies that contributed data to the meta-analyses for the prespecified outcomes. We created a 'Summary of findings' table to summarise the results and the quality of evidence for each outcome. We included five randomised controlled trials with a total of 212 participants in the analyses. All included participants were under five years of age. Using GRADE, we assessed there was low-quality evidence for all analysed outcomes. We assessed high risk of performance and detection bias for two studies due to their unblinded setting. Levosimendan showed no clear effect on risk of mortality (risk ratio (RR) 0.47, 95% confidence interval (CI) 0.12 to 1.82; participants = 123; studies = 3) and no clear effect on low cardiac output syndrome (RR 0.64, 95% CI 0.39 to 1.04; participants = 83; studies = 2) compared to standard treatments. Data on time-to-death were not available from any of the included studies.There was no conclusive evidence on the effect of levosimendan on the secondary outcomes. The levosimendan groups had shorter length of intensive care unit stays (mean difference (MD) 0.33 days, 95% CI -1.16 to 1.82; participants = 188; studies = 4; I² = 35%), length of hospital stays (0.26 days, 95% CI -3.50 to 4.03; participants = 75; studies = 2), and duration of mechanical ventilation (MD -0.04 days, 95% CI -0.08 to 0.00; participants = 208; studies = 5; I² = 0%). The risk of mechanical circulatory support or cardiac transplantation favoured the levosimendan groups (RR 1.49, 95% CI 0.19 to 11.37; participants = 60; studies = 2). Published data about adverse effects of levosimendan were limited. A meta-analysis of hypotension, one of the most feared side effects of levosimendan, was not feasible because of the heterogeneous expression of blood pressure values. The current level of evidence is insufficient to judge whether prophylactic levosimendan prevents low cardiac output syndrome and mortality in paediatric patients undergoing surgery for congenital heart disease. So far, no significant differences have been detected between levosimendan and standard inotrope treatments in this setting.The authors evaluated the quality of evidence as low, using the GRADE approach. Reasons for downgrading were serious risk of bias (performance and detection bias due to unblinded setting of two RCTs), serious risk of inconsistency, and serious to very serious risk of imprecision (small number of included patients, low event rates).

Transcriptional Categorization of the Etiology of Pneumonia Syndrome in Pediatric Patients in Malaria-Endemic Areas.

Pediatric acute respiratory distress in tropical settings is very common. Bacterial pneumonia is a major contributor to morbidity and mortality rates and requires adequate diagnosis for correct treatment. A rapid test that could identify bacterial (vs other) infections would have great clinical utility. We performed RNA (RNA-seq) sequencing and analyzed the transcriptomes of 68 pediatric patients with well-characterized clinical phenotype to identify transcriptional features associated with each disease class. We refined the features to predictive models (support vector machine, elastic net) and validated those models in an independent test set of 37 patients (80%-85% accuracy). We have identified sets of genes that are differentially expressed in pediatric patients with pneumonia syndrome attributable to different infections and requiring different therapeutic interventions. Findings of this study demonstrate that human transcription signatures in infected patients recapitulate the underlying biology and provide models for predicting a bacterial diagnosis to inform treatment.

Outcome of tumor lysis syndrome in pediatric patients with hematologic malignancies – a single-center experience from Pakistan

Outcome of tumor lysis syndrome in pediatric patients with hematologic malignancies – a single-center experience from Pakistan

Accuracy of Point-of-care Lung Ultrasonography for Diagnosis of Acute Chest Syndrome in Pediatric Patients with Sickle Cell Disease and Fever.

The objective was to determine the test performance characteristics for point-of-care lung ultrasonography (LUS) performed by pediatric emergency medicine (PEM) physicians compared with radiographic diagnosis of acute chest syndrome (ACS) in patients with sickle cell disease (SCD) and fever. This was a prospective study of patients up to 21 years with SCD and fever requiring chest X-ray (CXR) evaluation for ACS. Before obtaining CXR, a blinded PEM physician performed LUS using a standardized scanning protocol. Positive LUS for ACS was defined as lung consolidation. All patients received CXR and follow-up. The criterion standard for ACS was consolidation on CXR as determined by a blinded radiologist. LUS clips were reviewed by a blinded expert PEM sonologist. A total of 116 febrile events from 91 patients with a median age of 5.7 years were enrolled by 15 PEM sonologists. CXR was positive for ACS in 15 (13%) patients, and LUS was positive for ACS in 19 (16%) patients. Positive LUS had a sensitivity of 87% (95% confidence interval [CI] = 62% to 96%), specificity of 94% (95% CI = 88% to 97%), positive likelihood ratio of 14.6 (95% CI = 6.5 to 32.5), and negative likelihood ratio of 0.14 (95% CI = 0.04 to 0.52) for ACS. The interobserver agreement (kappa) was 0.77. There were two missed cases of ACS on LUS. LUS may be sensitive and specific for diagnosis of ACS in pediatric patients with SCD and fever. LUS may reduce the need for routine CXR and associated ionizing radiation exposure in this population.

Surgical Treatment of Chronic Exertional Compartment Syndrome in Pediatric Patients

Background: Chronic exertional compartment syndrome (CECS) is a cause of leg pain in running athletes and is treated with fasciotomy after failure of nonoperative management. CECS is being seen with increased frequency in younger patients. The demographics and outcomes of fasciotomy for CECS in pediatric patients, including risk factors for treatment failure, have not been described. Purpose: To describe characteristics of pediatric patients with CECS and determine surgical outcomes of the condition in this population. Study Design: Case series; Level of evidence, 4. Methods: A retrospective review was performed for patients 18 years and younger treated surgically for CECS with compartment release at a single institution from 1995 to 2014. Demographic and condition characteristics, operative procedure, postoperative course, and clinical outcomes were recorded for 286 legs of 155 patients. Compartment pressure testing using the Pedowitz criteria confirmed the diagnosis in all patients. Results: A total of 155 patients were included in the study (average patient age at presentation, 16.4 ± 1.38 years); 136 (88%) were female. All 155 patients presented with leg pain; of these patients, 8 (5%) also had neurologic symptoms, and 131 (85%) presented with bilateral symptoms requiring bilateral compartment release. Symptoms were chronic in nature, with duration over 1 year in 63% of patients. The primary sport was most commonly reported as running (25%), soccer (23%), or field hockey (12%); 50% of patients were multisport athletes. Of 286 legs, 138 (48%) had only anterior and/or lateral compartments released, while 84 (29.4%) had all 4 compartments released. Documented return to sport was seen in 79.5% of patients. Outcomes analysis was performed for 250 of 286 legs. Of these 250 legs, 47 (18.8%) had recurrent CECS requiring reoperation at a median of 1.3 years (interquartile range, 0.8-3.5) after initial compartment release. For each additional month between presentation and release, the odds of recurrence decreased by 12% (P = .04). Legs with only anterior and/or lateral compartment released had 3.4 times (95% CI, 1.29-9.14) the odds of reoperation compared with legs that had all 4 compartments released (P = .01). Twenty-eight of 250 legs (11.2%) had wound issues that resolved with nonoperative management. No wounds required repeat surgical management. Conclusion: CECS in pediatric patients most commonly occurs in adolescent females participating in running sports. Fasciotomy results in a 79.5% return-to-sports rate. Recurrence occurs in 18.8% of patients, more commonly in patients undergoing anterior and lateral release only.

Methylene blue compared to norepinephrine in the management of vasoplegic syndrome in pediatric patients after cardiopulmonary bypass: a randomized controlled study

Background and aimVasoplegic syndrome (VS) is a frequent complication following cardiopulmonary bypass (CPB) requiring escalating dose of vasopressor support. The guanylate cyclase inhibitor methylene blue (MB) could be an attractive alternative treatment in such cases. This study examines the efficacy and safety of using MB compared to the commonly used norepinephrine in VS in pediatric population following CPB.MethodsForty patients of pediatric age group who developed VS following CPB for elective corrective cardiac surgeries received 0.5 μg/kg/min norepinephrine intravenous infusion for 5 min without improvement (Time 1). Patients were randomly assigned to two equal groups. Group MB received 1.5 mg/kg methylene blue by intravenous infusion over 20 min. Group N did not receive MB. Norepinephrine infusion was continued in both groups and titrated according to the response of patients with a maximum dose of 2 μg/kg/min (Time 2). Heart rate, mean arterial pressure (MAP), central venous pressure (CVP), cardiac output (CO), cardiac index (CI), mean pulmonary artery pressure (MPAP), systemic vascular resistance (SVR) and systemic vascular resistance index (SVRI) were calculated in both groups. Side effects related to the study drug were recorded.ResultsTime 2 values of norepinephrine dose were significantly lower in MB group compared to N group. Time 2 values of MAP were significantly higher in MB group compared to N group with a significant decrease in HR in MB group compared to N group. No change in the rhythm was detected in the two groups. Time 2 values of CVP were higher in MB group compared to N group. Time 2 values of CO and CI were significantly lower in MB group compared to N group and SVR and SVRI were significantly higher in MB group compared to N group. Time 2 values of MPAP were comparable in both groups and showed no significant change. No side effects from using MB were recorded as pulmonary edema and respiratory distress.ConclusionIn this study, MB showed superior efficacy and safety in managing VS in pediatrics following CPB compared to the conventionally used norepinephrine.

Incidence and long-term outcome of postradiotherapy moyamoya syndrome in pediatric patients with primary brain tumors: a single institute experience in Taiwan.

We aimed to investigate the incidence and long‐term outcome of moyamoya syndrome in pediatric patients with primary brain tumors after receiving cranial radiotherapy (RT) in a single institute in Taiwan. The complete medical records, medical images, and RT notes of 391 pediatric patients with primary brain tumors treated with cranial RT between January 1975 and December 2005 in Taipei Veterans General Hospital (TVGH), Taiwan, were entered into an electronic registry and reviewed. Eight (2%) cases of post‐RT moyamoya syndrome were identified in the sample of 391 patients. The median latency was 3 years post‐RT. Among the eight patients, three had craniopharyngioma, two had optic glioma, two had medulloblastoma, and one had a suprasellar astrocytoma. The prescribed physical doses of RT were in the range of 40–54 Gy. The incidence was highest in those with optic glioma (0.039/person‐year), followed by craniopharyngioma (0.013/person‐year), astrocytoma (0.003/person‐year), and medulloblastoma (0.002/person‐year). No patients died of vasculopathy. No difference in crude incidence was found between our results and those of other series. The incidence of moyamoya syndrome was diagnosis dependent, with the highest incidence among patients with optic glioma. No regional difference in incidence was found. Long‐term, stable neurological function may be achieved following timely surgical intervention.

The Pharmacological Management of Complex Regional Pain Syndrome in Pediatric Patients.

The awareness of complex regional pain syndrome (CRPS) in the pediatric population is increasing. The condition involves regional pain that is out of proportion to any initiating event (if there is one) and is associated with sensory, functional, autonomic, and inflammatory changes in the region of the pain. The signs and symptoms of CRPS can vary between patients and stage of the disease process. Like many chronic pain conditions, it is often associated with significant disability and a detrimental effect on quality of life. It has a complex pathophysiology that remains poorly understood but provides many potential targets for treatments. Management involves a biopsychosocial formulation that encompasses physical and psychological interventions alongside pharmacological strategies. We review the current evidence for the treatment of this condition in children, with particular reference to pharmacological management.

Steroid Resistant Nephrotic Syndrome in Pediatric Patients : Histopathological Patterns at King Hussein Medical Center

Steroid Resistant Nephrotic Syndrome in Pediatric Patients : Histopathological Patterns at King Hussein Medical Center

Incidence of Vancomycin-Induced Red Man Syndrome in Pediatric Patients

The weight-based dose of vancomycin has increased for children with red man syndrome (RMS), but the duration of infusion has not. Therefore, there is presumed increased risk of RMS. Of 179 pediatric patients who received vancomycin between January 2010 and August 2011, 5 (2.8%) experienced RMS. Due to low incidence, empiric prolongation of infusion is not warranted unless the patient has a history of RMS.

Hepatopulmonary syndrome in pediatric patients (clinical presentation)

Hepatopulmonary syndrome (HPS) is a rare complication of liver diseases, resulting in lung perfusion disturbances and reduction of blood oxygenation. We report a case of hepatopulmonary syndrome in an 11- year-old-boy with congenital liver fibrosis.

Utility of Lung Ultrasound for Evaluating Acute Chest Syndrome in Pediatric Patients with Sickle Cell Disease

Background: Acute chest syndrome (ACS) is a common complication in patients with sickle cell disease (SCD) and is a leading cause of morbidity and mortality. Physical assessment alone is not sensitive for diagnosing ACS and therefore Chest x-ray (CXR) is recommended because of the difficulty of diagnosing ACS on clinical grounds alone. Children with SCD are repeatedly exposed to diagnostic radiation for the evaluation of ACS. Focused chest ultrasound (US) has been used to evaluate for lung consolidation. If lung US can identify patients with ACS this application could potentially limit radiation exposure in patients with SCD at risk for ACS. We evaluated the utility of physician performed US as compared to CXR to identify patients with SCD who have ACS.Methods: This is a prospective observational study that took place from November 2014-July 2015 in 2 urban pediatric emergency departments (EDs). The study population consisted of a convenience sample of patients with SCD from birth to 18 years of age at risk for ACS and who received a CXR for suspected ACS. Medical students and clinicians with training in lung sonography consented patients and performed a focused study to evaluate for lung consolidation. A blinded expert in point-of-care US reviewed for quality assurance and agreement. Sensitivity, specificity, and likelihood ratios were calculated for test performance characteristics of ultrasound using CXR as a reference standard. Inter-observer agreement (κ) between enrolling sonologists and reviewer was also calculated.Results: 85 patients were enrolled for a total of 98 cases. Median age was 7 years (IQR 2-13 years) and 53% of patients were male. The prevalence of ACS by CXR was 14%. Lung US was able to detect consolidation with a sensitivity of 86% (95% CI, 56-97%), specificity of 95% (95% CI, 87%-98%), positive likelihood ratio (LR) 18 (95% CI, 7-48) and negative LR 0.2 (95% CI, 0.04-0.5). The agreement between enrolling novice sonologists' interpretation and blinded reviewer's interpretation was very good with a Cohen κ of 0.86 (95% CI, 0.7-1).Conclusions: Focused lung US was able to identify ACS with high specificity. There was very good agreement between novice and expert sonologist interpretation. Lung US may decrease the need for CXR in patients at risk for ACS. Further studies are needed to see how this test performs within current clinical practice guidelines. DisclosuresNo relevant conflicts of interest to declare.

Probiotic for irritable bowel syndrome in pediatric patients: a randomized controlled clinical trial.

Background:Irritable bowel syndrome (IBS) is a common gastrointestinal disorder in children. Recently, probiotics have been suggested as a treatment option for gastrointestinal disorders. The most effective species and the most appropriate doses are still unknown.Objective:The aim of this study was to assess the effects of Lactobacillus GG (LGG) for treating IBS in pediatric patients.Methods:In a controlled, double blind, randomized trial, patients with IBS diagnosed by Rome III criteria from August 2012 to September 2012 at Dr. Sheikh Hospital, Mashhad University of Medical Sciences, Iran, were assigned to one of two groups, i.e., intervention and control groups. For four weeks, the intervention group received a probiotic in capsule form that contained LGG at a concentration of 1×1010 cfu/ml bacteria. For the same period, the control group received a placebo capsule that had the same shape and color but only contained inulin, which also was present in the LGG capsules. The primary outcome was any change in the severity of the patients’ pain, and we used a five-point Likert scale to evaluate the severity of their pain. Secondary outcomes were ghanges of the functional scale, stool patterns, and associated problems.Results:Fifty-two patients participated in the study, and 26 patients were assigned randomly to each of the two groups. The severity of the patients’ pain decreased significantly in the intervention group after one, two, three, and four weeks of treatment, as indicated by P-values of 0.01, 0.00, 0.00, and 0.00, respectively. Also, there was significant improvement in the functional scale after two weeks of treatment (P-value ≤ 0.00).Conclusion:Lactobacillus GG at a concentration of 1×1010 cfu/ml for a period of four weeks can lessen the severity of the patients’ pain and improve the functional scale in patients with irritable bowel syndrome. Probiotics can have therapeutic effects for IBS patients.

Clinical course, laboratory parameters and outcome of TTP pediatric patients presenting with posterior reversible encephalopathy syndrome

Aim: The clinical course and outcome of children with thrombotic thrombocytopenic purpura and posterior reversible encephalopathy has not been observed and studied till date. The aim of the present study was to know the clinical course and outcome of children with thrombotic thrombocytopenic purpura and posterior reversible encephalopathy. Results from our observation invite potential insight for further research on this subject. Methods: From January 2005 to February 2013, seven children diagnosed with thrombotic thrombocytopenic purpura and posterior reversible encephalopathy syndromes were admitted in a tertiary care hospital in Srinagar, Kashmir. The demographic parameters, clinical characteristics and laboratory data were noted. The outcome was defined in the form of complete recovery or death. Thrombotic thrombocytopenic purpura was diagnosed on clinical grounds, laboratory parameters and renal biopsy. The diagnosis was established after an expert opinion from a hematologist. Posterior reversible encephalopathy syndrome was defined on neuroimaging. Results: The common clinical parameters which were shared by all the patients were hypertension and altered sensorium. Four (57.1%) patients showed clinical deterioration and died within one week of admission even after intensive management. Three (42.8%) patients improved clinically and recovered fully and were discharged in stable clinical condition. Repeat imaging on discharge was normal. Conclusion: This series of seven pediatric patients is the first series on this subject. The presence of posterior reversible encephalopathy syndrome in pediatric patients with thrombotic thrombocytopenic purpura complicates the clinical course and worsens the prognosis.

Encephaloduroarteriosynangiosis and encephalomyoarteriosynangiosis for treatment of moyamoya syndrome in pediatric patients with sickle cell disease.

OBJECT Pediatric patients with sickle cell disease (SCD) and moyamoya syndrome (MMS) are at significant risk for cerebrovascular accidents despite chronic transfusion therapy. Encephaloduroarteriosynangiosis (EDAS) and encephalomyoarteriosynangiosis (EMAS) are additional therapeutic options for these patients. To date, the incidence of complications after and efficacy of EDAS and EMAS in stroke prevention in this population have been described in several institutional case series reports, but no randomized prospective trials have been reported. METHODS The authors retrospectively reviewed the cases of all pediatric patients at the University of Alabama at Birmingham with a history of homozygous hemoglobin S (HbS) and sickle cell/β-thalassemia (SB0 thalassemia) and on chronic transfusion therapy, including 14 patients with MMS who underwent EDAS or EMAS. RESULTS Sixty-two patients with SCD and on chronic transfusion therapy were identified. After exclusion of patients on chronic transfusion therapy for indications other than stroke prevention, 48 patients (77.4%) remained. Of those patients, 14 (29.1%) underwent EDAS or EMAS. Nine (18.8%) and 25 (52.1%) patients were on chronic transfusion therapy for primary or secondary stroke prevention, respectively, but did not undergo EDAS or EMAS. The 14 patients with SCD and radiological evidence of MMS and on chronic transfusion therapy for primary or secondary stroke prevention underwent 21 EDAS or EMAS procedures for progressive vascular disease (92.9% of patients), stroke (71.4%), and/or seizure (7.1%). The mean (± SD) time from initiation of chronic transfusion therapy to EDAS or EMAS was 76.8 ± 58.8 months. Complications included 1 perioperative stroke, 1 symptomatic subdural hygroma, 1 postoperative seizure, and 1 case of intraoperative cerebral edema that required subsequent cranioplasty. Before EDAS or EMAS, the stroke rate was calculated to be 1 stroke per 7.8 patient-years. One additional stroke occurred during the follow-up period (mean follow-up time 33.7 ± 19.6 months), resulting in a post-EDAS/EMAS stroke rate of 1 stroke per 39.3 patient-years, a 5-fold reduction compared with that in the pre-EDAS/EMAS period. The patients' mean pre-EDAS/EMAS HbS level of 29.5% ± 6.4% was comparable to the mean post-EDAS/EMAS HbS level of 25.5% ± 6.1% (p = 0.104). CONCLUSIONS The results of this retrospective case series in a large cohort of pediatric patients with SCD and MMS suggest that EDAS/EMAS provides a stroke-prevention benefit with an acceptably low morbidity rate. Given the combined experience with EDAS and EMAS for this indication at this and other institutions, a prospective clinical trial to assess their efficacy compared with that of chronic transfusion therapy alone is warranted.