Vascular Tumor with Kasabach Merritt Phenomenon Treated with Steroids and Vincristine: A Retrospective Study.

Vascular tumours (VT) with Kasabach-Merritt phenomenon (KMP) are rare and aggressive tumors. In absence of evidence based treatment guidelines, we studied varied presentation and response to therapy with vincristine and steroids in VT with KMP at our center. In this retrospective observational study, infants with a symptomatic/disfiguring rapidly growing VT with features of KMP were included. Demographic, treatment and outcome data was retrieved from patient file. Complete response (CR) was defined as complete clinical regression of VT with normalization of coagulopathy and thrombocytopenia. Partial response (PR) was defined as decrease in size of VT by more than 80%, absence of clinical bleed with normalization of coagulopathy and platelet count > 50,000/cumm. Five infants (2-male, 3-female) with age range (0-7 month) treated with daily prednisolone and weekly vincristine were included. The location of VT was: face (2), hemi-thorax (2) and urinary bladder (1). Four of five infants showed PR within two months; while two of these attained CR to treatment. There were no significant adverse effects over 9-32 (range) month follow-up. Two children (one in PR, one immediately after presentation) succumbed to intra-cranial hemorrhage. Combination therapy of steroids with vincristine is effective and safe in management of VT with KMP.

Assessment of knowledge, attitude and practice (KAP) of parents/caregivers towards epilepsy in children - A cross- sectional observational study.

Objectives: The study aimed to assess the knowledge, attitude, and practice (KAP) of parents/caregivers toward epilepsy in paediatric patients at a tertiary care centre of North India. Methods: A cross sectional study was carried out among 418 parents or caregivers using convenience sampling technique with 16-item questionnaire in English language and also translated to local language that is Hindi. Children with epilepsy who visited the paediatric outpatient department within a year were included in the study (January 2021-22). A total of 450 children visited the clinic, 32 of whom were excluded for various reasons, and the final analysis was conducted among the 418 parents or caregivers who completed the questionnaire. Results: The male and female patients were 56.7% (n = 237) and 43.3% (n = 181) respectively. The age distribution of patients with less than 5 years, 6-10 years and more than 10 years were 35.6% (n = 149), 54.5% (n = 228), 9.8% (n = 41) respectively. Only one third of parents and caregivers did not consider epilepsy as psychiatric illness. Most of the parents and caregivers think that epilepsy affects school performance (77.2%) and hinders family life (71.0%). More than half of the parents or caregivers believes that the society discriminates against person with epilepsy and around 46.6% consider that alternative medicine can cure epilepsy. The parents or caregivers felt financial burden due to epilepsy was in 72.5% and approximately 78.5% perceived that their work is affected because of their child's epilepsy. Perception of epilepsy as a psychiatric illness was found to be significantly higher in parents with primary and secondary level education, when compared to parents who were graduates. The practice of the parents or caregivers towards administration of drugs to their child was good, however around 36.6% (n = 153) missed the dose of anti-seizure medications. Conclusion: The study highlights the substantial knowledge, attitude and practice gap amongst parents and caregivers for children with epilepsy which indirectly has huge impact on the management of epilepsy. Thus it becomes utmost important to educate the family as well as the community regarding epilepsy which will help in improving the therapeutic outcomes, overall quality of life and interpersonal and social relationships of these children.

Impact of pre-hydration duration on high-dose methotrexate induced nephrotoxicity in childhood acute lymphoblastic leukaemia in resource constraint centers: a randomized crossover study.

PURPOSE: Hydration before starting high-dose methotrexate (HD-MTX) ensures good renal perfusion and alkaline urinary pH. The duration of pre-hydration is not uniform across protocols. We compared 6-h versus 12-h of pre-hydration for HD-MTX therapy in childhood acute lymphoblastic leukaemia (ALL) at our centre where serial MTX level monitoring is not feasible. METHODS: This randomised cross-over study consecutively enrolled children < 12 years with ALL receiving HD-MTX. Children with pre-existing renal disease or those exposed to nephrotoxic drugs were excluded. Two groups receiving 6-h versus 12-h pre-hydration on alternate basis in same patient (each exposed to four cycles of 2-5 g/m2 of HD-MTX) were compared for HD-MTX induced nephrotoxicity (primary outcome) and other HD-MTX toxicities (HMT) as per common terminology criteria for adverse events (CTCAE-4.0). HD-MTX was administered over 24 h as per BFM-protocol-2009. Solitary MTX levels at 36-h (MTX36) were outsourced and leucovorin (LV) was started at 36 h at 15 mg/m2/dose for 6-8 doses 6-hourly depending on MTX36. Hydration fluid was dextrose normal saline with sodium-bicarbonate and administered till last LV dose. RESULTS: Total 136 HD-MTX cycles in 34 patients (age range 5-144 months) were evaluated. Nephrotoxicity [2/68 (2.9%) in 6-h versus 1/68 (1.5%) in 12-h] and HMT incidence was comparable in two pre-hydration groups. Median MTX36 levels were not affected by duration of hydration irrespective of administered dose of HD-MTX. Median serum creatinine at baseline, post-pre-hydration and at 36-h post start of HD-MTX were comparable. CONCLUSION: Reduction of pre-hydration duration does not affect HD-MTX induced nephrotoxicity and MTX36 levels in children < 12 years.

Safety and outcome of ultrasound-guided tunneled central venous catheter in children with cancers from low middle-income country: A prospective study.

BACKGROUND: Central venous access devices (CVAD) are vital for cancer therapeutics in pediatric oncology. Tunneled vascular access devices (TVAD) are preferred in children for prolonged and frequent vascular access. Data on insertion, care, and complications of CVAD in children from low middle-income countries (LMIC) are scarce, heterogeneous, and retrospective. PROCEDURE: This prospective observational study on eligible children <12 years with pediatric malignancies requiring chemotherapy for minimum 6 months from diagnosis excluded children with mucosal bleeding, coagulopathy, and infections. TVAD insertion was ultrasound (USG) guided. Number of catheter-days, surgical and nonsurgical complications, and risk factors for catheter-related bloodstream infections (CRBSI) were noted TVAD removal due to complications, therapy completion, tumor progression, or death. RESULTS: Data from 61 of 86 eligible children with median age 42 months (range 1-144) were analyzed. Hematological malignancy and severe thrombocytopenia were seen in 37/61 (61%) and 18/61 (30%) children, respectively. First-attempt success rate was 74%. Surgical complications were seen in four of 61 (7%). Nonsurgical complications were seen in 33/61 (54%) children; CRBSI was commonest 24/61 (39%), causing removal of TVAD in 14/61 (23%). Incidence per 1000 catheter-days for CRBSI was 3.24. Antibiotic lock therapy could salvage nine of 24 TVAD with CRBSI. Thrombus and accidental removal was seen in six of 61 (10%) and four of 61 (7%). None of the studied risk factors were significantly associated with CRBSI. The mean insertion duration of TVAD was 121 ± 90 days. CONCLUSION: USG-guided TVAD insertion is safe and reliable way for chemotherapy administration with acceptable complications in children with malignancies in LMIC, including children with severe thrombocytopenia.

Delirium in Pediatric Intensive Care Unit: Frequency, Causes, and Interventions.

Delirium is an acute neurologic dysfunction characterized by fluctuating alteration in awareness, attention, and cognition and is a result of an underlying medical condition or its treatment. It is associated with increased morbidity and mortality in the intensive care setting and may lead to long-term cognitive impairment in survivors. Pediatric delirium (PD) occurs in as many as 25% of critically ill children. The pathophysiology of delirium is complex and widespread screening of this entity is lacking. To diagnose PD, a high index of suspicion should be kept in the patients who are at high risk, and appropriate screening tools should be applied, as the symptoms of PD overlap with other commonly observed neurological phenomena in pediatric intensive care unit. How to cite this article: Dhingra S, Delirium in Pediatric Intensive Care Unit: Frequency, Causes, and Interventions. Indian J Crit Care Med 2021;25(6):620-621.

Electrocardiometry Fluid Responsiveness in Pediatric Septic Shock.

Hemodynamic monitoring and categorization of patients based on fluid responsiveness is the key to decisions prompting the use of fluids and vasoactive agents in septic shock. Distinguishing patients who are going to benefit from fluids from those who will not is of paramount importance as large amounts of fluids used conventionally based on surviving sepsis guidelines may be detrimental. Noninvasive monitoring techniques for the assessment of various cardiovascular parameters are increasingly accepted as the current medical practice. Electrical cardiometry (EC) is one such method for the determination of stroke volume, cardiac output (CO), and other hemodynamic parameters and is based on changes in electrical conductivity within the thorax. It has been validated against gold standard methods such as thermodilution [Malik V, Subramanian A, Chauhan S, et al. World J 2014;4(7):101-108] and is being used more often as a point-of-care noninvasive technique for hemodynamic monitoring. EC is Food and Drug Administration approved and validated for use in neonates, children, and adults. A meta-analysis in 2016, including 20 studies and 624 patients comparing the accuracy of CO measurement by using EC with other noninvasive technologies, demonstrated that EC was the device that offered the most correct measurements. The article in the current issue of IJCCM by Rao et al. (2020) has extended the use of EC to categorize pediatric patients with septic shock into vasodilated and vasoconstricted states based on systemic vascular resistance and correlate the categorization clinically. The authors also studied the changes in hemodynamic parameters after an isotonic fluid bolus of 20 mL/kg was administered. This is a pilot prospective observational study of 30 patients, which has given an insight into physiological rearrangements following fluid administration in patients with septic shock. How to cite this article: Gupta D, Dhingra. Electrocardiometry Fluid Responsiveness in Pediatric Septic Shock. Indian J Crit Care Med 2021;25(2):123-125.

Acute kidney injury in the pediatric intensive care unit at a tertiary care hospital of the Armed Forces: a cross-sectional observational study.

BACKGROUND: Acute kidney injury (AKI) is shown to be the commonest complication in critically ill children admitted to the pediatric intensive care unit (PICU). Kidney Disease: Improving Global Outcomes (KDIGO) classification and definition are now used universally. We undertook prospective observational study to study the etiology and maximum stage of AKI as defined by KDIGO and its complications and outcomes. METHODS: All children admitted to the PICU were included in the study. The diagnosis of sepsis and multiorgan dysfunction syndrome (MODS) was made according to the standard international guidelines. The patients were followed up till discharge/death. All children were screened for AKI at admission and subsequently using serum creatinine measured by modified Jaffe's method and urine output measurement. RESULTS: A total of 197 children were admitted to the PICU. 38 (19.28%) developed AKI, and 6 (15.78%) developed stage III AKI. Malignancies, serious neurological and renal disorders, and postsurgery complications accounted for most of the cases with AKI. Six were admitted with primary renal condition. Sepsis with or without MODS was seen in 12 patients with AKI and in 8 without AKI. Twenty-one children with AKI and 3 children without AKI were exposed to nephrotoxic drugs. Twenty-three children with AKI required inotropic support. The average length of stay (ALOS) of children with AKI in the PICU was 9.86 days, whereas ALOS of children without AKI was 6.23 days. Eighteen children with AKI (47.36%) and 36 (21.38%) with no AKI died. CONCLUSIONS: AKI in children in the PICUs of referral hospitals in the armed forces have varied etiologies and presentations. These children require early identification and management with close monitoring to prevent long-term renal morbidity and mortality.

Steroid resistant nephrotic syndrome in a child with chronic hepatitis B infection.

Steroid resistant nephrotic syndrome (SRNS) continues to be a challenge for pediatric nephrologists the world over. Secondary causes of nephrotic syndrome need to be searched for in all cases of steroid resistance. Hepatitis B virus (HBV) is associated with several types of glomerulonephritis, most commonly being membranous nephropathy (MN) in children. It is an important cause of secondary nephrotic syndrome in countries with high prevalence of chronic hepatitis B virus (HBV) infection. We present a case of SRNS in a 5-yr-old boy who had received 3 weeks of daily steroids before referral to our hospital. At presentation the child had urinary tract infection (UTI) which was adequately treated. The child had persistence of proteinuria, even after completing 4 weeks of daily steroids in adequate dose. Secondary causes of nephrotic syndrome were looked for which revealed presence of chronic HBV infection in the patient with a very high viral load. Kidney biopsy was characteristic of MN with predominant IgG, & minor IgM, and C3 deposits in subepithelial region. The child responded to treatment with Lamivudine with reduction in edema and proteinuria.

Crystal structure of zeolite MCM-68: a new three-dimensional framework with large pores.

The crystal structure of the aluminosilicate MCM-68 was solved from synchrotron powder diffraction data by the program FOCUS. The unit cell framework contains Si100.6Al11.4O224. This material crystallizes in space group P42/mnm, where, after Rietveld refinement, a=18.286(1) A and c=20.208(2) A. A three-dimensional framework is found that contains continuous 12-ring channels and two orthogonal, intersecting, undulating 10-ring channels. Rietveld refinement of the model coordinates optimizes the framework geometry, to match the observed intensity profile by Rwp=0.1371, R(F2)=0.1411. It is not possible to determine the location of approximately 0.84 K+ cations remaining in the unit cell after the material is steamed and then dehydrated. The framework model also successfully predicts observed electron diffraction data in two projections, and the tetragonal projection can be determined independently from these data by direct methods. The calculated density of the framework structure is 1.66 g/cm3, and the T-site framework density is 16.6 T/1000 A3.