Consensus Report by the Pediatric Acute Lung Injury and Sepsis Investigators and Pediatric Blood and Marrow Transplant Consortium Joint Working Committees on Supportive Care Guidelines for Management of Veno-Occlusive Disease in Children and Adolescents: Part 2-Focus on Ascites, Fluid and Electrolytes, Renal, and Transfusion Issues

Even though hepatic veno-occlusive disease (VOD) is a potentially fatal complication of hematopoietic cell transplantation (HCT), there is paucity of research on the management of associated multiorgan dysfunction. To help provide standardized care for the management of these patients, the HCT Subgroup of the Pediatric Acute Lung Injury and Sepsis Investigators and the Supportive Care Committee of the Pediatric Blood and Marrow Transplant Consortium, collaborated to develop evidence-based consensus guidelines. After conducting an extensive literature search, in part 2 of this series we discuss the management of fluids and electrolytes, renal dysfunction; ascites, pleural effusion, and transfusion and coagulopathy issues in patients with VOD. We consider the available evidence using the GRADE criteria.

Premedication in an autistic, combative child: Challenges and nuances.

Children with autistic spectrum disorders are often encountered in anesthesia practice mainly for outdoor procedural sedation or anesthesia in endoscopy and magnetic resonance imaging suites. We describe a case of a 7-year-old autistic boy who required management of dental caries. He had a phobia to intravenous cannulation, displayed increasing anxiety and became combative on the day of surgery. With parental involvement and distraction, we succeeded in giving oral midazolam by concealing it, with the intent of avoiding intramuscular injection or unnecessary restraint. Lack of knowledge about the medical condition of such a patient can lead to inadequate preoperative preparation and use of restraint on the patient, which might cause anxiety or panic attacks in the operative room. To effectively manage children with special needs one needs to have clear guidelines on the management of uncooperative children, involve parents perioperatively, plan ahead with an emphasis on perioperative analgesia and sometimes incorporate the ethical use of restraint.

Multidisciplinary management of colorectal adenocarcinoma associated with anal fistula: an Indian series.

AIM: Adenocarcinomas associated with anal fistula are rare and often present at an advanced stage. They are often mistaken for commonly occurring benign diseases, leading to delayed diagnosis. Previous reports have predicted inferior oncological outcomes for these cases compared with sporadic rectal cancers. We are presenting our series of patients with colorectal adenocarcinoma associated with anal fistula who were treated with multimodality therapy at a tertiary cancer centre in India. METHOD: This was a retrospective review of a prospectively maintained database of patients treated at our centre between 1 July 2013 and 31 March 2015. Of the 15 patients included in the study, 11 had prior intervention in the form of seton placement or fistulotomy. Fourteen patients had circumferential resection margin (CRM) involvement at initial workup and hence were given neoadjuvant chemoradiotherapy (NACRT). None of the patients had distant metastasis and only 15% had regional nodal involvement. RESULTS: All 13 patients included in the final analysis underwent abdominoperineal excision (APE). Ten patients (73%) underwent extralevator APE. Plastic reconstruction in the form of a V-Y advancement flap for perineal closure was required in six patients (46%). On histopathological examination, a mucinous component was found to be present in 11 patients (73%). The quality of total mesorectal excision was complete in 92% patients. The CRM was free in 92% of patients. Median overall survival and disease-free survival were not reached. CONCLUSION: Colorectal adenocarcinomas associated with fistula are locally aggressive malignancies with a low incidence of lymph node involvement and distant metastasis. NACRT, wider resection in the form of extralevator APE, and liberal use of plastic reconstruction may result in favourable outcomes.

Serum and salivary sialic acid as a biomarker in oral potentially malignant disorders and oral cancer.

Background: Aberrant glycosylation is the universal feature of cancer and components of various glycoconjugates, such as sialic acid is found to rise in various malignancies. The objective of this study was to evaluate the serum and salivary sialic acid in oral potentially malignant disorders (OPMD) and oral cancer (OC) to investigate the possibility of using this as a diagnostic marker. Materials and Methods: The study included 85 subjects, who were grouped as control (30), OPMD patients (25), and oral cancer patients (30). Serum and unstimulated whole saliva was collected from subjects of all groups and sialic acid estimation was done using spectrophotometry. The results were tabulated and analyzed statistically. Results: The mean serum sialic acid levels in normal, OPMD, and oral cancer group were 7.515, 19.620, and 55.235 mg/dL, respectively, whereas the levels of salivary sialic acid were 1.5113, 2.3302, and 9.0304 mg/dL, respectively. A very highly significant rise (P < 0.005) in serum and salivary sialic acid was observed in the study subjects compared with that of the control. Conclusions: The present study showed a significant and gradual increase in serum and salivary sialic acid from control to oral potentially malignant disorders to oral cancer. From this study we can suggest that sialic acid can be used as a reliable biomarker. As this monosaccharide is observed in saliva in detectable quantity, saliva can be used as a diagnostic medium for screening and early detection of oral cancer.

Practice-based, clinical trial of a silorane-based composite resin system in posterior teeth.

INTRODUCTION: Newer monomer systems have been introduced in an attempt to improve the success rate of direct composite restorations by minimising shrinkage. AIMS AND OBJECTIVES: To evaluate the in vivo performance of a silorane-based resin composite system (Filtek Silorane, also known as P90), used together with a two-step self-etch bonding system (P90 System Adhesive, Self-Etch Primer and Bond), for the restoration of Class I and Class II cavities in human permanent posterior teeth. MATERIALS AND METHODS: A total of 53 restorations were completed and patients were recalled one year later. The restorations were assessed by two ndependent clinicians, using the Modified U.S. Public Health Service evaluation criteria for the following parameters: retention, colour match, marginal discolouration, secondary caries, anatomic form, marginal adaptation, surface texture, occlusal and interproximal contacts. RESULTS: Of the 41 restorations assessed at the one year follow-up, 92.7% showed clinically acceptable scores for all the parameters evaluated. Cronbach's alpha values ranged from 0.72-1.00 showing a good inter-examiner agreement and reliability. CONCLUSION: Silorane-based composite, Filtek Silorane, when used in conjunction with the recommended adhesive system showed a clinically acceptable performance in posterior teeth restorations over a one year clinical service.

A study of standardized regimens of steroid treatment in reactions in leprosy at a referral centre.

It is a well known fact that reactions and nerve function impairment (NFI) account for majority of disabilities and morbidities in leprosy. Steroids are the principal agents administered for treatment of reactions and NFI. In this study, we compared the efficacy of two regimens namely high dose (60 mg) regimen tapered over 28 weeks and low dose (40 mg) regimen tapered over 22 weeks in treatment of reactions and early NFI as regards to incidence of recurrence of reactions in 209 patients. Concerns about the side-effects of steroids were also addressed byfollowing a strict pre-steroid investigation protocol. We observed that the low dose regimen was associated with a higher incidence of recurrence (48.3%) as compared to high dose regimen (16%) signifying the efficacy of longer duration of therapy with a higher starting dose. No patient developed side-effects which necessitated withdrawal of steroids. Early detection with prompt and adequate therapy with proper dose and duration is the key to reduce recurrence of reactions and to minimize deformities due to reactions and NFI in leprosy.

Twice daily i.v. bolus tacrolimus infusion for GVHD prophylaxis in children undergoing stem cell transplantation.

Tacrolimus is routinely administered for GVHD prophylaxis as a 24-h continuous infusion that requires a dedicated i.v. line and thus becomes logistically difficult to administer, especially in young pediatric patients. We investigated the safety and efficacy of twice daily bolus infusions of i.v. tacrolimus in 33 children undergoing hematopoietic stem cell transplantation (HSCT) at our institution. Tacrolimus was started at an initial dose of 0.015 mg/kg i.v. bolus administered as a 2-h infusion and then given at every 12 h to maintain a trough drug level between 5-15 ng/mL. Patients also received short-course MTX (66%) or mycophenolate mofetil (34%) in combination with tacrolimus. No acute infusional toxicities were observed with bolus infusions of i.v. tacrolimus. Nephrotoxicity occurred in 14/33 (42%) patients and 48% developed hypertension (HT). Almost all (94%) patients required magnesium supplements to maintain magnesium (Mg) levels 1.5 mg/dL. In all, 3 (9%) patients developed severe sinusoidal obstruction syndrome (SOS). One patient developed posterior reversible leuko-encephalopathy syndrome (PRES) and one additional patient had tremors. The prevelance of these side-effects was similar to those reported for continuous i.v. administration. In all, 28% of the evaluable patients developed acute GVHDgrade II, though the incidence of severe (grade III-IV) GVHD was only 7%. These results suggest that intermittent bolus i.v. tacrolimus administration is a safe and effective method of GVHD prophylaxis in children.

A retrospective cohort study of dermatological problems observed in paediatric intensive care unit.

BACKGROUND: Dermatological manifestations are often encountered in paediatric intensive care units (PICU). Spectrum of dermatological problems that may arise in critically ill children in intensive care unit remains unknown. OBJECTIVES: The aim of this study was to find out the burden of dermatological problems and to describe the proportional distribution of paediatric dermatoses in ICU set-up. METHODS: In a retrospective cohort study, we analysed all types of paediatric dermatological conditions manifesting in children admitted to a tertiary level ICU in South India. RESULTS: During the study period of 25 months, 1180 new cases were admitted to PICU. A total of 318 children with 361 skin manifestations were observed. Majority of the skin lesions were minor and were secondary to systemic disease. Infection was the leading cause of dermatoses in ICU. Dengue infection was detected in 64% of total cases included in the study. Stevens-Johnson syndrome was the only primary dermatological condition leading to PICU admission in the present cohort. CONCLUSIONS: The spectrum and proportional distribution of skin conditions in children differ from adult ICU-data. Further large-scale investigations are needed to define the characteristics and distribution of infections along with other disease conditions leading to ICU-admissions and mortality among critically ill paediatric patients.

PCATMIP: enhancing signal intensity in diffusion-weighted magnetic resonance imaging.

Diffusion-weighted MRI studies generally lose signal intensity to physiological motion, which can adversely affect quantification/diagnosis. Averaging over multiple repetitions, often used to improve image quality, does not eliminate the signal loss. In this article, PCATMIP, a combined principal component analysis and temporal maximum intensity projection approach, is developed to address this problem. Data are first acquired for a fixed number of repetitions. Assuming that physiological fluctuations of image intensities locally are likely temporally correlated unlike random noise, a local moving boxcar in the spatial domain is used to reconstruct low-noise images by considering the most relevant principal components in the temporal domain. Subsequently, a temporal maximum intensity projection yields a high signal-intensity image. Numerical and experimental studies were performed for validation and to determine optimal parameters for increasing signal intensity and minimizing noise. Subsequently, a combined principal component analysis and temporal maximum intensity projection approach was used to analyze diffusion-weighted porcine liver MRI scans. In these scans, the variability of apparent diffusion coefficient values among repeated measurements was reduced by 59% relative to averaging, and there was an increase in the signal intensity with higher intensity differences observed at higher b-values. In summary, a combined principal component analysis and temporal maximum intensity projection approach is a postprocessing approach that corrects for bulk motion-induced signal loss and improves apparent diffusion coefficient measurement reproducibility.