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Acute leukemia is the commonest pediatric malignancy with acute lymphoblastic leukemia (ALL) constituting about 75% of all leukemias. ALL commonly presents as fever, bleeding, bone pains, anemia, lymphadenopathy and hepatosplenomegaly. The liver, spleen or lymph nodes are the most common sites of extramedullary involvement in ALL, with renal involvement being relatively uncommon. The usual differential diagnosis of acquired massive bilateral nephromegaly in children includes pyelonephritis, obstructive uropathy, infections such as HIV nephropathy, mucormycosis, glycogen storage diseases, myelofibrosis with extramedullary hematopoiesis, kidney tumors and rarely hematological malignancies like ALL. Here we report a 2 years old child who presented with abdominal distention, low grade fever and constipation. Clinical examination revealed massive bilateral nephromegaly. Preliminary investigations showed severe anemia and slightly elevated WBC counts with presence of reactive changes in lymphocytes along with few atypical cells (9%). Abdominal ultrasonography revealed bilaterally enlarged kidneys which was later confirmed by CT abdomen. He was eventually diagnosed with CALLA positive B cell ALL for which treatment was started in accordance with the International network for cancer treatment and research (INCTR) protocol 02 04. At present, he is on maintenance phase and responding well to the treatment with regression of kidney size to normal on follow up ultrasonogram. Thus, leukemia should be considered in a child presenting with bilateral nephromegaly after exclusion of above mentioned differential diagnosis. Bone marrow aspiration must be done before doing a more invasive investigation like renal biopsy.

Proptosis is a frequent presenting symptom/sign of many paediatric malignancies. Acute-onset proptosis is an ophthalmic emergency that can endanger vision if not treated promptly. Appropriate treatment must be instituted only after investigating for the underlying aetiology. Here, we report a developmentally delayed boy in middle childhood who presented with recent onset bilateral proptosis. Clinical examination followed by radiological evaluation suggested scurvy to be the underlying cause and vitamin C supplementation led to prompt reversal of proptosis. The relevant literature has been reviewed and presented here to apprise the paediatric oncologists about this rare but easily treatable cause of proptosis.

Background & objectives: World Health Organization (WHO) revised its guidelines for classification and management of dengue in 2009. This revised system was found out to have good sensitivity and negative predictive value but poor specificity as well as positive predictive value. Methods: This retrospective study was carried out in a tertiary care hospital of Delhi, India to assess factors predicting the occurrence of severe dengue in children as per the revised classification. A total of 647 suspected dengue cases were admitted in the hospital in the year 2015. Detailed clinical and epidemiological data of 170 patients who were confirmed as dengue either by NS1 antigen test or by serology (Ig M positive) were recorded and statistically analyzed. Results: The number of laboratory-confirmed cases was 170 and included thirty (17.65%) dengue fever (DF), 106 (62.35%) dengue with warning signs (DWS) and 34 (20.0%) severe dengue (SD) patients. Regression analysis revealed that presence of vomiting, altered sensorium, shock, peri-orbital edema, hepatomegaly, splenomegaly, severe anemia, thrombocytopenia, elevated urea and creatinine, decreased total protein and globulin were significantly associated with occurrence of severe disease. Interpretation & conclusion: The addition of clinical features (peri-orbital edema and splenomegaly); and laboratory findings (elevated urea and creatinine, decreased serum protein and globulin) might help improve the sensitivity and specificity of the revised WHO dengue classification in predicting severe dengue.

Adolescents and youth, together addressed as \"young people\", form the future building blocks of any society. They being most energetic and dynamic, tend to get involved in high-risk behaviors making themselves susceptible to criminal offences, accidents, physical injuries, emotional trauma, and medical problems - some of them extremely serious like transmission of human immunodeficiency virus (HIV). The concept of vulnerability is applicable to all the people who are more exposed to risks than their peers like the young people. In order to deal with social evils like criminal offences, domestic violence, sexual abuse, HIV, etc. we need to define vulnerability and understand the factors that influence it. This review also attempts to summarize the indicators of vulnerability and the data currently available to estimate its burden in India. Measuring the magnitude of vulnerability by means of certain indicators/variables might help us in devising tools to assess this poorly defined entity. This may also evolve a conceptual framework on which targeted remedial interventions can be devised and implemented.

Gastrointestinal complications are not uncommon in patients of Acute Leukemia. Intussusception as a complication in leukemia, although described, is exceedingly rare. Also, it is usually seen after chemotherapy and not as a part of the native disease process. This case report aims to highlight such a rare association which warrants clinical and pathological attention. Rare presentations of relatively common diseases are a hurdle for timely and effective medical intervention. Although a rare condition in itself in leukemic patients, the occurrence of Intussusception in this particular patient, especially when no chemotherapy was initiated, is a very rare event. This case report was made to add to the relatively scarce literature available on this particular association. As it is a surgically treatable condition and since delay in diagnosis may lead to poorer prognosis, possibility of co-existence of ALL and intussusception should be borne in mind by all treating physicians and hematopathologists for effective patient care.

Background. With improving standards of care of children living with HIV (CLHIV), pediatric HIV related mortality rates are declining. New challenges like HIV status disclosure are emerging which need to be addressed to ensure their smooth transition into adulthood. Poor disease disclosure rates are observed in CLHIV globally. Aims. This study was done to assess the prevalence of HIV disclosure in North Indian CLHIV, know the perceptions of caregivers regarding disclosure, and evaluate the impact of disclosure on CLHIV. Methods. It was a questionnaire based cross-sectional study carried out amongst 144 caregivers of CLHIV aged 6-16 years attending the pediatric HIV clinic of a tertiary care teaching hospital. Results. Though the majority (93.8%) caregivers felt that it is important to disclose but only 33% of the children were actually disclosed. Eighty five percent felt that disclosure must be done by one of the family members and correspondingly 73% of the disclosed children were actually disclosed by their parents. Forty seven percent believed that the most appropriate age for disclosure is 10-12 years. The mean age at which disclosure was actually done was 11.06 ± 1.62 years. Comparison of the disclosed and undisclosed CLHIV revealed that the disclosed group had significantly higher age, longer duration of taking ART, and higher proportion of paternal orphans. Age of the CLHIV was the only significant factor for disclosure. Several reasons were cited by the caregivers for nondisclosure. The caregivers observed improved drug adherence in 47.9% of the children following disclosure. Conclusions. There is a need to develop region specific pediatric HIV disclosure guidelines keeping in mind the caregivers’ perceptions. The guidelines must be age appropriate, systematic, and socioculturally acceptable. The most suitable age for disclosure appears to be 10-12 years. Involvement of caregivers and health care providers in the process is a must.

Chronic hemoglobinopathies like thalassemia are associated with many osteopathies like osteoporosis. Methods. This observational study was carried out to compare the bone mineral density (BMD) in transfusion dependent thalassemics with that of healthy controls. Thirty-two thalassemia patients, aged 2–18 years, and 32 age and sex matched controls were studied. The bone mineral concentration (BMC) and BMD were assessed at lumbar spine, distal radius, and neck of femur. Biochemical parameters like serum calcium and vitamin D levels were also assessed. Results. The BMC of neck of femur was significantly low in cases in comparison to controls. We also observed significantly lower BMD at the lumbar spine in cases in comparison to controls. A significantly positive correlation was observed between serum calcium levels and BMD at neck of femur. Conclusion. Hence, low serum calcium may be used as a predictor of low BMD especially in populations where incidence of hypovitaminosis D is very high.

Objective To estimate the change in serum hepcidin levels and its correlation with change in hemoglobin (Hb) level during the initial two weeks of oral iron therapy in children with iron deficiency anemia (IDA). Methods A prospective observational study was carried out in children aged 2–12 years with IDA. Children with severe anemia (Hb < 7 g/dL), those with fever, infections, history of oral iron intake or blood transfusion within the preceding three months, or intolerant to oral iron were excluded. Serum hepcidin-25 was assessed using ELISA-based kits on day 0 (pre-therapy), after 24 hours and 14 days of starting oral iron therapy. Results Out of 78 children who were screened, we included 64 children with IDA with a mean (SD) hemoglobin of 8.81 (1.22) g/dL. The baseline mean (SD) serum hepcidin-25 levels [7.81 (4.88) ng/mL] increased significantly to 8.38 (4.96) ng/mL at 24 hours and 9.51 (5.2) ng/mL on day 14 of oral iron therapy ( P < 0.001). 63 children showed a good response to oral iron therapy. No significant correlation was observed between baseline hepcidin levels with change in hemoglobin on day 1 (r = −0.10, P = 0.40) or day 14 (r = −0.10, P = 0.43) of therapy. Conclusion Serum hepcidin levels rise significantly as early as 24 hours after starting oral iron therapy and should be explored to assess response to oral iron therapy in children with anemia.

An aneurysmal bone cyst (ABC) is a hyperplastic reactive lesion of unknown etiology consisting of cystic cavities containing blood lined by mesenchymal reactive tissue. It occurs more often in females (2:1), usually before the age of 20 years, and most commonly in long bones [1]. The extension of the primary ABC to the epiphysis after crossing the viable physis is an exceptional finding, and very few such cases have been reported in the literature [2–6]. Although uncommon, ABC can cause severe destruction of long bones in children and young adults. The suggested treatment for such aggressive cases ranges from simple curettage to wide resection and structural reconstruction [7, 8]. Here, we report the follow-up for a 10-year-old girl with large, aggressive ABC of the proximal tibia with transphyseal extension to the epiphysis, which was treated with intralesional excision, curettage and bone grafting.

Millions of microorganisms inhabit the human body and affect its homeostasis in multiple ways. Alterations in this microbial community have implications for the health and survival of the human hosts. It is believed that these microorganisms should be included as part of the human genome because of their influence on human physiology hence the term \"microbiome\" is commonly used to refer to these microbes along with their genetic make-up and their environmental interactions. In this article we attempt to provide an insight into this recently discovered vital organ of the human body which is yet to be fully explored. We herein discuss the composition and role of microbiome in human health and disease with a special emphasis in children and culture-independent techniques employed in mapping of the microbiome. Alteration in the gut microbiome has been associated with causation of several paediatric diseases like infantile colic, necrotizing enterocolitis, asthma, atopy, obesity, type -1 diabetes, and autism. Atopic dermatitis and psoriasis have also been associated with changes in the cutaneous microbiome. Respiratory microbial imbalances during infancy have been linked with wheezing and bronchial asthma. Dysbiosis in the regional microbiome has been linked with caries, periodontitis, and chronic rhinosinusitis. The future therapeutic implications of this rapidly evolving area of research are also highlighted.