Incidence of Kawasaki disease among children in Chandigarh, India during 2015-2019: a trend analysis.

Background: Only limited information exists regarding the epidemiology of Kawasaki disease (KD) in low-income and middle-income countries. The present study provides the incidence of KD during 2015-2019 in Chandigarh, north India. Our centre follows the largest KD cohort in India. Methods: Children with KD at Chandigarh diagnosed during January 2015-December 2019 were enrolled in the study. Annual incidence rates were determined using decadal growth rates of the National Census 2011. We computed the incidence of KD in children aged <5, and <15 years. We also undertook linear trend analysis using our incidence data from 1994 to 2019. Findings: During 2015-2019, 83 patients (66 males, 17 females) were diagnosed with KD in Chandigarh. Incidence rates during these 5 years were 5.64, 9.25, 9.11, 9.87, and 9.72/100,000 in children aged <5 years, and 2.65, 4.44, 3.86, 5.07, 4.74/100,000 in children aged <15 years. The median age at diagnosis was 48 months (range: 12 days to 15 years). Compared to previous data (2009-2014), there was a 53.1% increase in annual incidence of KD in children aged <5 years, and a 53.7% increase in children aged <15 years. Coronary artery abnormalities during acute phase were noted in 16.9%, and in 7.2% of patients at 6 weeks of follow-up. The trend analysis indicated a monthly rise of 0.002 cases per 100,000 children aged <5 years, and 0.0165 cases per 100,000 children aged <15 years. Interpretation: The incidence of KD has continued to show an upward trend in Chandigarh over the period 2015-2019. This may indicate a true rise in the occurrence of KD or may reflect better disease ascertainment as a result of greater awareness about KD amongst healthcare professionals. Funding: None.

Isolated ventricular inversion with hypoplastic right ventricle and pulmonary stenosis in an adolescent with situs solitus: a rare combination.

Isolated ventricular inversion with situs solitus is a severe and rare congenital cardiac malformation characterised by an atrioventricular discordance but with ventriculo-arterial concordance. Here, we present the rare case of an adolescent with isolated ventricular inversion and hypoplasia of the left-sided morphological right ventricle and pulmonary stenosis, a first of its kind to be reported in the literature.

Pericardial effusion in anti-complement factor H antibody-associated atypical hemolytic uremic syndrome: two case reports.

Hemolytic uremic syndrome (HUS), a cause of pediatric acute kidney injury (AKI), has a spectrum of extra-renal manifestations. While neurological and gastrointestinal system involvement is common, cardiac involvement is rare. This is more so with pericardial involvement, though it has been reported in a handful of HUS cases associated with shiga toxin-producing Escherichia coli (STEC HUS). However, this complication has scarcely been reported in atypical HUS (aHUS) where there is alternate complement abnormality or DKGE (diacylglycerol kinase epsilon) mutation. We describe two children diagnosed with anti-complement factor H (CFH) antibody-associated aHUS who had pericardial involvement. Two boys, one 10-year-old and another 8-year-old, presented with pallor, oliguria and hypertension. They both had microangiopathic haemolytic anemia, thrombocytopenia and AKI suggestive of HUS. Complement workup revealed elevated anti-CFH antibody titres. With a diagnosis of anti-CFH antibody aHUS, they were started on plasmapheresis, pulse methylprednisolone and cyclophosphamide. The first case developed cardiac tamponade during the second week of hospital stay for which he needed pigtail drainage and further immunosuppression with rituximab. He gradually improved and pigtail was removed. The second case presented with pericardial effusion which subsequently resolved during the course of treatment. Thus, our patients developed pericardial effusion, with one of them progressing to life-threatening cardiac tamponade. Therefore, it is prudent that we are aware of this complication while treating children with aHUS.

Transcatheter closure of Patent ductus arteriosus in a child with IVC interruption through standard femoral access: a case report.

BACKGROUND: Portsmann and co. performed the first PDA device closure in 1967. The technique and the devices used have evolved since then and are the first choice in managing anatomically feasible patent ductus arteriosus (PDA) for the last 20 years. Though catheter-based closure of PDA is generally a simple procedure, there are instances when the interventionist faces challenges, especially in smaller children, with syndromic features and venous anomalies even when defects are small and pulmonary artery pressures are normal. Although the femoral vein is the relatively risk-free standard access, internal jugular vein, femoral artery, and transhepatic IVC can be used to close the PDA in different anomalies. The rare venous anomaly of infrahepatic interruption of the IVC with azygous continuation poses technical challenges when percutaneous closure of PDA was attempted through the standard femoral access. CASE PRESENTATION: We report a rare case of PDA device closure in a syndromic child with a short neck having interrupted IVC via femoral-azygous venous approach. CONCLUSION: Knowledge of the IVC course and its anomalies should be known to the operator before the percutaneous closure of PDA. Although other approaches are available, femoral vein approach can be used in case of interrupted IVC for percutaneous closure of PDA.

Ventricular Septal Defect Pulmonary Atresia: Provisional Bifurcation Strategy for Confluence Stenosis During Patent Ductus Arteriosis Stenting.

Neonates with pulmonary atresia survive only if duct patency is maintained before staged surgical repair. Ductal stenting is an effective alternative to conventional shunt surgery, as it avoids thoracotomy. With today's generation of coronary stents having better profile, flexibility, and trackability, it may be achieved safely, with less difficulty than previously described. The strategy during patent ductus arteriosus (PDA) stenting with trifurcation narrowing is a real challenge. We present an underweight newborn with ventricular septal defect pulmonary atresia, restrictive PDA, and bilateral branch stenosis at the ostia. We performed a successful PDA stenting as well as addressed the ostia of branch pulmonary arteries.

Mystery still unresolved: Untouched "Blue heart" presenting at 40yrs of age.

The modern-day surgical techniques and strategies have changed the outlook of patients with dextro-transposition of great arteries (d-TGA). The survival of an unrepaired d-TGA into late adulthood is difficult to explain. Even when large intracardiac shunts are present, it still remains a lethal cyanotic congenital heart disease if it is not surgically corrected soon after birth. Here, we report an extremely rare case of d-TGA presenting at 40 years of age, with moderately elevated pulmonary artery pressures and relatively stable symptoms.

Bloodstream infections and trends of antimicrobial sensitivity patterns at Port Blair.

PURPOSE: Bloodstream infection can range from inapparent bacteremia until fulminant septic shock with high mortality. Microorganisms present in circulating blood whether continuously, intermittently, or transiently are a threat to every organ in the body. Culture of blood is a vital tool to diagnose such infections. Drug susceptibility patterns help in rationalizing therapy. OBJECTIVE: The objective of this study was to perform bacteriological analysis and assess drug sensitivity patterns of isolates from blood stream infections. DESIGN: Retrospective observational study was conducted from May 2015 to February 2017 at a tertiary care hospital, Port Blair, India. Blood samples were collected with aseptic guidelines and cultured for 7 days. Growths were identified using standard biochemical tests and subjected to sensitivity testing according to Modified Kirby-Bauer's disk diffusion method. Data for the source of blood collection and duration of incubation were noted and compared. RESULTS: A total of 270 (14.24%) pathogens were isolated from 1895 bacteremia suspect patient blood specimens. Contamination was observed at a rate of 1.63%. Gram-positive cocci (60.37%) were predominant organisms recovered followed by Gram-negative Bacilli (36.29%) and Yeasts (3.33%). Staphylococcus aureus, CoNS, and Acinetobacter spp. were the primary pathogens isolated. Aminoglycosides, carbapenems, and glycopeptides were the most effective drugs for treating bacteremia. CONCLUSIONS: Successful treatment of sepsis depends on early diagnosis and proper antimicrobial therapy. Local knowledge of bacteriological profile and antimicrobial sensitivity patterns helps rationalize empiric treatment strategies.

An Unusual Combination of Truncus Arteriosus, Interrupted Aortic Arch, and Hypoplastic Left Ventricle.

Truncus arteriosus (common arterial trunk) is known to be associated with interrupted aortic arch in 10% to 15% of cases. However, the association of either of these lesions with a hypoplastic left ventricle is rare. The combination of all three of these lesions along with an intact interventricular septum is virtually unknown with only two cases reported in the literature. We report such a case with its anatomical and surgical management aspects.

Comparative evaluations of powder and mechanical properties of low crystallinity celluloses, microcrystalline celluloses, and powdered celluloses.

The purpose of this study was to examine and compare the powder and mechanical properties of different batches of low crystallinity powdered cellulose (LCPC-S1 to LCPC-S5) with those of commercial microcrystalline celluloses (MCC) (Avicel PH-101, Avicel PH-102, Avicel PH-103, Avicel PH-301, Avicel PH-302, and Emcocel 90m) and powdered celluloses (PC) (Solka Floc BW-40 and Solka Floc BW-100). Both the LCPC and MCC products were aggregated powders, whereas, the PC materials showed a fibrous structure. The primary particles forming the LCPC aggregates, however, were smaller in size and showed a greater degree of coalescence between boundaries, than those forming the MCC aggregates. The LCPC materials had significantly higher bulk and tap densities and lower porosity values compared with the MCC materials. The yield pressure value calculated from the linear region of the Heckel curve for LCPC varied between 48 and 70 MPa, for Avicel and PC materials between, 80 and 106 MPa, and for Emcocel 90m was 48 MPa. These results suggest that the LCPC products and Emcocel 90m, compared with commercial MCC and PC excipients, undergo plastic deformation at relatively lower compression pressures. The total volume reduction (i.e. compressibility), determined by calculating the area under the Heckel curve (AUHC), however, was comparable for all materials, with the exception of the LCPC-S3, which owing to the low yield pressure value, showed the largest reduction in volume. With the exception of LCPC-S1 and Solka Floc BW-40, all the other materials formed compacts, whose strength ranged from about 522 to 799 MPa2. The strengths of LCPC-S1 and Solka Floc BW-40 compacts, in contrast, were 214 and 257 MPa2, respectively. Irrespective of the solid fraction levels, the LCPC compacts, in general, disintegrated much faster than the MCC and PC compacts. In conclusion, the results suggest that the new LCPC materials reported herein have powder properties that are quite different from the MCC and PC materials evaluated, and show clear potential as direct compression excipients.