Oral cyclophosphamide therapy in 100 children with steroid-sensitive nephrotic syndrome: experience from a developing country.

BACKGROUND: Oral cyclophosphamide (CYP) is an important therapeutic agent in treatment of steroid-sensitive nephrotic syndrome having a steroid-dependent (SD) or frequent relapsing (FR) course. This retrospective observational study aimed to determine response to oral CYP and factors associated with positive response in these patients. METHODS: We studied 100 children (male; 75) with FR (19%) and SD (81%) NS treated with CYP in the Pediatric Nephrology clinic. Responders were defined as children in whom steroids were stopped for at least 6 months following CYP and factors affecting response were analysed. Relapse-free survival was estimated by Kaplan-Meier method. RESULTS: Median age at onset of NS was 3 years (IQR 2-5.2). Median age at CYP was 5.7 years (IQR 3.7-7.9). Fifty percent of patients were in the responder group at 6 months of CYP. Relapse-free survival post CYP therapy was 31% at 1 year, 11% at 2 years. Factors predicting good response were age at onset of NS > 3 years with 61.2% response at 6 months (p = 0.028) and older age at CYP initiation (> 5 years) with 61% response (p = 0.008). Multivariate regression analysis showed age at start of CYP > 5 years was an independent factor for good response (p = 0.044, OR = 2.903, CI -1.03 to 8.18). CONCLUSIONS: Judicious selection of patients, especially with age of onset of NS more than 3 years and initiation of CYP after age of 5 years, can predict better response in this group of patents.

Comparison of Noninvasive Oscillometric and Intra-Arterial Blood Pressure Measurements in Children Admitted to the Pediatric Intensive Care Unit.

Intra-arterial blood pressure (IABP) measurement, although considered the gold standard in critically ill children, is associated with certain risks and lacks widespread availability. This study was conducted to determine the differences and agreements between oscillometric non-invasive blood pressure (NIBP) and invasive IABP measurements in children. Inclusion criteria consisted of children (from 1 month to 18 years) admitted to the pediatric intensive care unit (PICU) of a teaching hospital who required arterial catheter insertion for blood pressure (BP) monitoring. The comparison between IABP and NIBP was studied using paired t -test, Bland-Altman analysis, and Pearson's correlation coefficient. In total, 4,447 pairs of simultaneously recorded hourly NIBP and IABP measurements were collected from 65 children. Mean differences between IABP and NIBP were -3.6 ± 12.85, -4.7 ± 9.3, and -3.12 ± 9.30 mm Hg for systolic, diastolic, and mean arterial BP, respectively ( p < 0.001), with wide limits of agreement. NIBP significantly overestimated BP ( p < 0.001) in all three BP states (hypotensive, normotensive, and hypertensive), except systolic blood pressure (SBP) during hypertension where IABP was significantly higher. The difference in SBP was most pronounced during hypotension. The difference in SBP was significant in children <10 years ( p < 0.001), with the maximum difference being in infants. It was insignificant in adolescents ( p = 0.28) and underweight children ( p = 0.55). NIBP recorded significantly higher BP in all states of BP except SBP in the hypertensive state. SBP measured by NIBP tended to be the most reliable in adolescents and underweight children. NIBP was the most unreliable in infants, obese children, and during hypotension.

Clinical Profile and Outcome of Children Requiring Noninvasive Ventilation (NIV).

OBJECTIVES: To determine the clinical profile and outcome of children requiring noninvasive ventilation (NIV) in a PICU. METHODS: This prospective observational study was conducted in the PICU at Dayanand Medical College and Hospital, Punjab. Children (1 mo-18 y) with moderate-to-severe respiratory distress who received NIV during one-year period were included. Failure was defined as the need for endotracheal intubation. The patients received bilevel positive airway pressure (BiPAP) with inspiratory and expiratory positive airway pressure (8-18 cm H2O and 4-8 cm), respectively and indigenous continuous positive airway pressure (CPAP) were included. Vital signs (heart rate, respiratory rate) and gasometric parameters (pH, HCO3, pCO2, pO2) were recorded. RESULTS: Out of total 115 patients, 81.7% were successfully treated by NIV whereas 18.3% constituted NIV failure group. Two types of NIV were used, 65.2% were started on BiPAP and 34.8% on indigenous bubble CPAP. Most common diagnosis was tropical fever (24.3%), bronchopneumonia (20%), and sepsis with multiple organ dysfunction syndrome (MODS) (7.8%). Commonest indication of NIV was respiratory distress (70.4%) and prevention of postextubation respiratory failure (20.8%). Seven patients (6.9%) died during the study. NIV failure is higher in children with sepsis with MODS, abnormal blood gas (acidosis), and moderate-to-severe acute respiratory distress syndrome (ARDS). CONCLUSIONS: This study demonstrates that NIV is an effective form of respiratory support for children with acute respiratory distress/failure. Sepsis with MODS, acidosis and ARDS (moderate to severe) were predictors of NIV failure. Careful patient selection may help in judicious use of NIV in PICU.

Role of montelukast in multitrigger wheezers attending chest clinic in Punjab, India.

BACKGROUND: Wheeze is seen in 40% of preschool children, one-third of these may develop recurrent wheeze. Montelukast is an oral, once a day, easy to give therapy but there is no definite evidence to support its use in a recent meta-analysis. Present study was done to evaluate role of daily montelukast and various factors affecting the outcome after therapy in multitrigger wheeze (MTW). METHODS: A prospective study conducted in Pediatric chest clinic over 18 months at DMCH, Ludhiana. Children from 6 months to 5 years, diagnosed cases of MTW were started on montelukast. Diagnosis based on symptoms of recurrent wheeze triggered by various allergens/precipitants was made by pediatrician in charge of chest clinic. Children were followed up at 1 and 3 months. They were labeled as controlled, partially controlled, or uncontrolled as per global initiative for asthma guidelines. Data were used to compare the outcome related to various factors. RESULTS: Total 139 out of 150 children came for regular follow-up. At the end of 3 months, 94 (67.7%) were controlled, 8 (5.7%) partially controlled, and 37 (26.6%) children remained uncontrolled on montelukast. Factors associated with poor control were onset of symptoms at younger age (<6 months of age), family history of allergies, prior multiple visits or hospitalizations due to MTW, use of MDI in the past. No significant side effects were reported by parents. CONCLUSION: Symptomatically two-third of children became better after starting montelukast. There were few factors which resulted in poorer control in subset of patients.

Comparison of Clinical Features and Outcome of Dengue Fever and Multisystem Inflammatory Syndrome in Children Associated With COVID-19 (MIS-C).

OBJECTIVE: To identify clinical and laboratory features that differentiate dengue fever patients from MIS-C patients and determine their outcomes. Methods: This comparative cross-sectional study was done at a tertiary care teaching institute. We enrolled all hospitalized children aged 1 month - 18 years and diagnosed with either MIS-C and/or dengue fever according to WHO criteria between June and December, 2020. Clinical and laboratory features and outcomes were recorded on a structured proforma. RESULTS: During the study period 34 cases of MIS-C and 83 cases of Dengue fever were enrolled. Mean age of MIS-C cases (male, 86.3%) was 7.89 (4.61) years. MIS-C with shock was seen in 15 cases (44%), MIS-C without shock in 17 cases (50%) and Kawasaki disease-like presentation in 2 cases (6%). Patients of MIS-C were younger as compared to dengue fever (P=0.002). Abdominal pain and erythematous rash were more common in dengue fever. Of the inflammatory markers, mean C reactive protein was higher in MIS-C patients [100.2 (85.1) vs 16.9 (29.3) mg/dL] (P<0.001). In contrast, serum ferritin levels were higher in dengue fever patients (P=0.03). Mean hospital stay (patient days) was longer in MIS- C compared to dengue fever (8.6 vs 6.5 days; P=0.014). CONCLUSION: Clinical and laboratory features can give important clues to differentiate dengue fever and MIS-C and help initiate specific treatment.