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Male neonate was born by normal vaginal delivery at 38 weeks. He was antenatally diagnosed to have dextrocardia, congenitally corrected transposition of great arteries, moderate ventricular septal defect and pulmonary atresia. He was born in good condition and cried at birth. However, he was noted to have saturation of 65%–70% in air and started on continuous positive airway pressure and transferred to neonatal intensive care unit for further monitoring. Postnatal echocardiography confirmed the diagnosis, and he was started on prostaglandin E1 (PGE1) at 5 nanogram/kg/min. On day 3 of life, he underwent a cardiac gated CT angiography (CTA) for further understanding of anatomy and confirmation of diagnosis. In view of need for PGE1 until transfer to tertiary cardiac surgical centre, a …

Chronic myeloid leukemia (CML) is a stem cell disorder with the characteristic presence of chromosomal translocation resulting in the formation of Bcr-Abl protein tyrosine kinase. Within bone marrow, leukemia stem cells (LSCs) share a niche with mesenchymal stem cells (MSCs). Therefore, there exists a strong signalling traffic between MSC and LSC that may direct therapeutic outcome of the CML patients. Imatinib is commonly used as the first line of therapy for CML. However, therapeutic resistance appears to be a major challenge for treatment of CML. Autophagy is one of the key phenomena that may direct cells towards survival or death. However, current understanding on the regulation of autophagy in CML cells by bone marrow niche cells is limited. Therefore, the present study is focused on the regulation of imatinib-induced autophagy in CML cells by MSCs. Here, we cocultured bone marrow-derived MSCs with chronic myeloid leukemia cells, K562 to understand the signaling axis that controls autophagy and subsequent apoptosis of CML cells. We studied K562 cell viability and proliferation by MTT and CFSE assay. For autophagy determination, we performed Western blot analysis and live cell confocal microscopy with K562 cells transfected with ptfLC3 plasmid (the microtubule-associated protein 1 light chain 3, LC3 protein fused to mRFP and EGFP plasmid). Results revealed that there was a reduction in the viability of K562 cells when cocultured with MSC for 48 h. Further reduction in the viability was observed when cells were exposed to 5 µM imatinib for 48 h. The presence of MSCs caused ~2-fold higher accumulation of the lipidated form of LC3 (LC3BII) in K562 cells as compared to control. In addition, imatinib treatment resulted in ~10-fold higher accumulation of LC3BII in K562 cells. Interestingly, as compared to imatinib-treated K562 cells, ~2-fold lower autophagic flux was observed when imatinib was administered to K562 cells in the presence of MSCs. However, enhanced expression of pro-apoptotic protein Bcl-xl was observed. Involvements of ATG5 and Beclin-1 were also detected. This was also microscopically confirmed in K562 cells using mRFP and GFP tagged plasmid. In conclusion, all these studies suggest that bone marrow MSCs play a pivotal role in regulating imatinib-induced autophagy in CML cells. This might provide an important clue to effectively formulate a therapeutic strategy involving MSCs to eliminate imatinib-resistant CML cells from bone marrow niche. Citation Format: Devdeep Mukherjee, Joyjyoti Das, Prantar Chakrabarti, Tapas Kumar Maiti. Bone marrow mesenchymal stem cells tune autophagic circuitry of chronic myeloid leukemia cells in response to imatinib treatment [abstract]. In: Proceedings of the AACR-NCI-EORTC International Conference: Molecular Targets and Cancer Therapeutics; 2017 Oct 26-30; Philadelphia, PA. Philadelphia (PA): AACR; Mol Cancer Ther 2018;17(1 Suppl):Abstract nr A021.

On 1 September 2015, the UK became the world’s first country to introduce a novel meningococcal B vaccine (Bexsero) into its national childhood immunisation programme. Bexsero is the only market authorised meningococcal B vaccine in the UK. Meningococcal B strains accounts for ~80% of all laboratory confirmed cases of invasive meningococcal disease (IMD) in England and Wales.1 Bexsero is estimated to protect against 73%–88% of the meningococcal B strains causing invasive disease in England and Wales.2 After observing more instances of clinical instability in our immunised neonates following the introduction of the new …

(Key words: Tuberculosis, Pott’s spine, pyrexia of unknown origin, infant) Sri Lanka Journal of Child Health , 2017; 46 (2): 184-186

(Key words: Cardio renal syndrome, congestive cardiac failure, acute kidney injury, renin–angiotensin–aldosterone system, ventricular septal defect) Sri Lanka Journal of Child Health , 2017; 46 (2): 175-177

Anodic sites like grain-boundaries, micro-voids, micro-inclusions and other stress concentration points such as dislocations, vacancies, etc., take an active part in the galvanic interactions occurring on the surface of metals and alloys. This paper discusses the role of low concentration reinforcement of metals like Zn, Pb and Sn, alloy matrices like Cu-Zn, Cu-Mn, Zn-Al and Al-Zn and non-metals like polyaniline, along with the amorphous Ni-P-B class of current-assisted electroless deposits. It is assumed that ultrafine ceramic particulates in low concentration ranges (1–1.5 wt%) may cover the anodic defect sites such that these anodic boundaries start behaving cathodically as a network, within the already existing cathodic grain matrices, because they are predominantly more cathodic, compared to the grain boundaries, voids, etc. As such, there is a considerable reduction of surface dissolution and in the anodic current of the matrix. It has been shown that surface dissolution is a minimum for a critical threshold concentration of the particulates, above which there is drastic dissolution of the surface. It appears that particulates above that critical concentration cannot be accommodated within the available anodic sites, such that they are dispersed on the grain proper in a random fashion, creating stress spikes and subsequent enhanced matrix dissolution. It further appears that it will thus be possible either to decrease or increase the dissolution of the composite matrices for selective tailor-made applications, by changing the concentration of these ultrafine particulates around the threshold concentration. It has been shown that such a technique may eventually reduce the leaching of copper from conventional brass matrices and also reduce the dissolution of tin in the packaging industries. Grain boundary structures of the metallic matrices have also been correlated with the particulate trapping capacity and their corresponding galvanic stress factors. It has also been applied to amorphous Ni-P-B types of metal-metalloid coatings, for enhancement of surface corrosion resistance. Particulation of epoxy and epoxy-silicone classes of barrier with ultrafine SiC has revealed a more positive potential and lower galvanic currents. Reinforcement of the permalloy type Ni-Fe-Mo class barrier with fine Al2O3 particulates has indicated considerable improvement of the polarization resistance values.

304 SS substrates have been covered with polymeric class low melting barriers, like polyurethane, acrylate and epoxy with and without incorporation of eco‐friendly (non‐toxic) ceramic particular of antifouling origin. The results of corrosion resistance tests are encouraging. Other physical parameters like hardness, adhesion, scratch resistance are also studied, for these synergistically organized low melting point barrier layers.

Discusses the particulation of organic barrier layers, based on laboratory‐bench data, obtained from the exposure of painted panels in low, high or neutral pHelectrolytes. It also discusses their compatibility with the succeeding and preceding layers and the galvanic interactions at the layer substrate interfaces. Aspects like substrate effect, leaching of the micaceous iron powder, role of high temperature and hydrodynamic disturbances and distribution of ultrafine particulates have been highlighted using potential versus time plot, AAS data, and galvanic current measurements and XRD analysis. Exposure tests conducted under ambient conditions and under very strong hydrodynamic disturbances, revealed superior properties of the SiC particulated epoxy based barrier layers.

Kawasaki disease is an acute febrile vasculitis of childhood. Macrophage activation syndrome is a rare life threatening complication.4-year-old boy with Kawasaki Disease treated with intravenous immunoglobulins.He developed encephalopathy, hepatosplenomegaly and pancytopenia. Blood investigations and bone marrow aspiration suggested macrophage activation syndrome.Good response to pulse methylprednisolone (30 mg/kg/d) for 5 days.Macrophage activation syndrome may complicate Kawasaki disease.

AIM To ascertain the frequency of hyponatremic hypertensive syndrome (HHS) in a cohort of children with hypertensive emergency in a tertiary pediatric hospital. METHODS A retrospective review was undertaken among children with hypertensive emergency admitted in our tertiary children hospital between June 2014 and December 2015 with an aim to identify any children with HHS. Three children with HHS were identified during this period. RESULTS The 3 patients with HHS presented with hypertensive emergency. They were initially managed with Labetalol infusion and thereafter switched to oral anti-hypertensives (combination of Nifedipine sustained release, Hydralazine and Beta Blocker). All 3 were diagnosed to have unilateral renal artery stenosis. One child was lost to follow up, whereas the other 2 underwent renal angioplasty which was followed with normalization of blood pressure. CONCLUSION Despite activation of renin angiotensin axis secondary to renal artery stenosis, these groups of children have significant hyponatremia. Renal re-vascularisation produces excellent results in most of them.

Early infantile epileptic encephalopathy has a grave outcome. Dravet syndrome (DS), characterized by early onset, refractory seizures, and intellectual deficit is one of the variants of the condition. SCN1B gene mutation is one of the lesser known variants of DS. Increased awareness of genetic analysis has increased the early diagnosis of DS for an early prognostication as well as genetic counselling of parents. We present the case of a 7-month old male child who started having recurrent febrile, and thereafter, afebrile seizures, following administration of a vaccination at 3 months. He developed global developmental delay, and is presently on multiple anticonvulsants. Genetic analysis was suggestive of SCN1B gene mutation associated with DS.

A 7 day old term, appropriate for gestational age, girl was delivered by normal vaginal delivery at home. There was no history of any topical application over the umbilicus. Baby presented to our hospital with a history of worms coming out of the umbilicus along with excessive cry. She also had a history of yellow discolouration of skin for the last 2 days. There was no history of poor feeding, lethargy, distension of abdomen or fever.

Purpura fulminans (PF) may be the presenting symptom in a patient with protein C (PC) deficiency. It is a hematological emergency and presents with extensive areas of hemorrhagic necrosis of the skin. PC deficiency is usually genetically inherited. However, we report a 1 year and 4 months boy, who presented with acquired PC deficiency possibly of postinfectious etiology and developed PF.

Sri Lanka Journal of Child Health , 2014; 43 (3): 168-170 (Key words: Posterior reversible encephalopathy syndrome; PRES; renal artery stenosis; hypertension) DOI: http://dx.doi.org/10.4038/sljch.v43i3.7378