Unusual case of 'scorbutic proptosis'.

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.

Axillary Lymph Node in Extra-Ocular Retinoblastoma - Benign or Malignant?

A 1-year-old male child presented with whitish discoloration of pupil of the left eye and swelling over the left axilla. A contrast-enhanced magnetic resonance imaging of the brain and orbits performed revealed left eye extra-ocular retinoblastoma. 18F-fluorodeoxyglucose positron emission tomography/computed tomography scan was done in this child as a part of baseline staging of retinoblastoma in an ongoing research project. The scan revealed left eye extra-ocular retinoblastoma along with calcified left axillary level I lymph node.

Paediatric orbital ultrasound: Tips and tricks.

Background: The orbital structures are ideally suited for ultrasound examination due to their superficial location and cystic composition of the eye. However, orbital ultrasound remains an underutilised modality due to preference for other cross-sectional modalities in general practice. Aim: In this article, we review the basic principles, clinical uses and technique of orbital ultrasound in peadiatric patients. Materials and methods: The clinical utility of orbital ultrasound in peadiatric patients is demonstrated using selected cases. Results: Ultrasound is useful in the diagnosis of various posterior segment pathologies, especially in conditions causing opacification of light-conducting media of the eye. It is also beneficial in diagnosing various orbital pathologies, particularly in differentiating solid from cystic lesions. Discussion: The added advantages of its use in children include lack of ionising radiation and reduced requirement of sedation or general anesthesia. Ultrasound is the most practical initial investigation in cases where ophthalmoscopy is limited by opacification of ocular media. The addition of color Doppler on ultrasound can give additional information about the vascularity of the lesion. Conclusion: Use of ultrasound can be streamlined into the workup of various orbital and ocular pathologies either as an initial investigation or as a problem-solving tool in cases with a diagnostic dilemma on other modalities.

Intussusception in a child with Acute Lymphoblastic Leukemia: a remarkable presentation with literature review - a case report.

BACKGROUND: 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. CASE PRESENTATION: A 14 year old male presented with an acute abdomen. Initial routine investigations revealed a deranged blood picture. On further examination of bone marrow aspirate, biopsy and detailed immunohistochemical studies a diagnosis of B-Acute Lymphoblastic Leukemia (B-ALL) was made. Concurrent ultrasound of the abdomen to find a cause for severe abdominal pain revealed an Ileo-colic intussusception. The patient was started on steroids; however he succumbed to his illness after two days, before surgery could be attempted. CONCLUSION: 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.

Predictors of severe dengue amongst children as per the revised WHO classification.

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.

Psychiatric Problems Amongst Adolescents Living With HIV at a Tertiary Care Centre in India.

OBJECTIVE: To assess the prevalence of psychiatric problems among adolescents living with HIV (ALHIV). METHODS: Questionnaire-based cross-sectional study conducted at pediatric HIV clinic of a tertiary care hospital. Participants: 101 ALHIV between 10-18 years of age. RESULTS: Of the 101 ALHIV, 12 (11.88%) met criteria for psychiatric disorders, of which dysthymi (5,41.6%) and oppositional defiant disorder (6,50%) were the commonest. Father of 7 (58.34%) and mother of 8 (66.6%) screen positive patients were dead as compared to 22 (24.7%) and 13 (14.6%) of screen negative patients (P=0.016 and P=0.0003, respectively). CONCLUSION: Psychiatric problems are common in ALHIV in the age group more than 15 years.

Acute lymphoblastic leukemia presenting as nephromegaly in a child: A rare case report.

Arora SK, Swarnim S, Hemal A, Bidhuri N. Acute lymphoblastic leukemia presenting as nephromegaly in a child: A rare case report. Turk J Pediatr 2019; 61: 97-101. 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.

Pediatric HIV Disclosure in Northern India: Evaluation of Its Prevalence, Perceptions amongst Caregivers, and Its Impact on CLHIV.

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.

Microbiome: Paediatricians' perspective.

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.

Defining and Measuring Vulnerability in Young People.

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.

Comparison of Bone Mineral Density in Thalassemia Major Patients with Healthy Controls.

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.

Giant primary cerebral hydatid cyst: A rare cause of childhood seizure.

We report a 9-year-old girl who presented with focal seizures, hemiparesis, headache, vomiting and bilateral optic atrophy. CT scan revealed a giant solitary cyst in the left parietal lobe. Serology and histopathology of the excised cyst confirmed the diagnosis of neurohydatidosis which is a rare cause of childhood seizure.

Impact of an educational film on parental knowledge of children with cerebral palsy.

Parents of children with cerebral palsy (CP) must have knowledge about the disease and its management to improve the outcome. This uncontrolled interventional trial was carried out to evaluate the parental knowledge of CP and assess the impact of an educational programme on it. Preset questionnaires were filled before and 1 week after a single session educational programme using an educational film. Out of a total of 53 subjects, majority (75.5%) were from lower socioeconomic status. Initially, none knew the correct name of child's illness; afterwards 45.3% could name it. When compared to previous status, there occurred significant improvement in the knowledge of parents after viewing the film with regard to knowing the cause of CP, knowing that motor involvement was predominant in CP, knowledge regarding curability of the disease, and knowledge about special schooling (P < 0.05). Change in knowledge was not related to socioeconomic or educational status (P > 0.05). Majority (94.3%) found the film useful and 96.2% learned how they could help in the management of their children. Parental knowledge of CP is inadequate which can be improved by incorporating such educational programmes in special clinics to improve management.

Role of organochlorine pesticides in children with idiopathic seizures.

Background. Organochlorine pesticides (OCP) are persistent organic pollutants that have been implicated in causing several deleterious effects in humans. These are known neurotoxins in high doses, but the role of environmentally acquired OCPs in the body to induce seizures in children has not been investigated yet. Objectives. To assess the serum levels of OCPs in children aged 2-12 with idiopathic seizure and to find out any association between the two are our objectives. Methods. It was a cross-sectional pilot study. Twenty developmentally normal children aged 2-12, presenting with idiopathic generalized seizures, were recruited. Twenty age-matched controls without any history of seizures were also taken. Their serum levels of α, ß, and γ hexachlorocyclohexane (HCH); and aldrin; dieldrin; p,p-dichlorodiphenyltrichloroethane (DDT), o,p-DDT, and p,p dichlorodiphenyldichloroethylene (DDE); and α and ß endosulfan were analysed using gas chromatography (GC). Mann-Whitney U test was used to compare OCP levels between the groups. Spearman correlation was used to find the correlation between individual pesticide levels with age and seizure duration. Results. Levels of ß, γ, and total HCH were significantly higher among cases as compared to the control group (P ≤ 0.05). Conclusion. There exists a possible association between idiopathic seizures and high serum levels of OCPs, especially HCH.

Estimation of gestational age, using neonatal anthropometry: a cross-sectional study in India.

Prematurity is a significant contributor to neonatal mortality in India. Conventionally, assessment of gestational age of newborns is based on New Ballard Technique, for which a paediatric specialist is needed. Anthropometry of the newborn, especially birthweight, has been used in the past to predict the gestational age of the neonate in peripheral health facilities where a trained paediatrician is often not available. We aimed to determine if neonatal anthropometric parameters, viz. birthweight, crown heel-length, head-circumference, mid-upper arm-circumference, lower segment-length, foot-length, umbilical nipple distance, calf-circumference, intermammary distance, and hand-length, can reliably predict the gestational age. The study also aimed to derive an equation for the same. We also assessed if these neonatal anthropometric parameters had a better prediction of gestational age when used in combination compared to individual parameters. We evaluated 1,000 newborns in a cross-sectional study conducted in Guru Teg Bahadur Hospital in Delhi. Detailed anthropometric estimation of the neonates was done within 48 hours after birth, using standard techniques. Gestational age was estimated using New Ballard Scoring. Out of 1,250 consecutive neonates, 1,000 were included in the study. Of them, 800 randomly-selected newborns were used in devising the model, and the remaining 200 newborns were used in validating the final model. Quadratic regression analysis using stepwise selection was used in building the predictive model. Birthweight (R=0.72), head-circumference (R = 0.60), and mid-upper arm-circumference (R = 0.67) were found highly correlated with gestation. The final equation to assess gestational age was as follows: Gestational age (weeks) = 5.437 x W-0.781 x W(2) + 2.815 x HC-0.041 x HC(2) + 0.285 x MUAC-22.745 where W=Weight, HC=Head-circumference and MUAC=Mid-upper arm-circumference; Adjusted R = 0.76. On validation, the predictability of this equation is 46% (+/-1 week), 75.5% (+/- 2 weeks), and 91.5% (+/- 3 weeks). This mathematical model may be used in identifying preterm neonates.

Clinicohematological profile and platelet trends in children with dengue during 2010 epidemic in north India.

OBJECTIVE: To study the clinicohematological profile and the platelet trends in children presenting with dengue infection during the 2010 dengue epidemic in north India and to compare the it with previous epidemics. METHODS: This retrospective study was carried out in a tertiary care hospital of North India. Of the 219 children admitted from August 2010 through November 2010 with suspected dengue infection, 135 were IgM positive and were included in the final analysis. RESULTS: These children were further categorized as dengue fever (8%), dengue hemorrhagic fever (51%) and dengue shock syndrome (42%). The mean age (± SD) of children were 8.3 ± 3.5 y with male: female ratio 1.32. Mean duration of fever (±SD) was 6.3 d ± 3.7 d. The clinical features included fever (100%), headache (63%), abdominal pain (71%), petechia (35.5%), rash (26.6%) and bleeding manifestations (48.8%). On examination, Hess test (33.3%), signs of fluid retention (23%), pallor (13.3%),signs of circulatory failure (43%), hepatomegaly (31.1%) and splenomegaly (27%) were positive. Laboratory investigations revealed mean (±SD) hemoglobin 11.5 g/dl (±1.7), hematocrit 36.1(±5.5), leucocyte count 7,551/mm(3) and platelet count 38,800 mm(3) on day of admission. A total of 92.6% of children had thrombocytopenia and 19.2% had abnormal leucocyte count. Deranged liver function tests were observed in 48 children. The mean (±SD) of hospitalization and platelet recovery were 4.2 ± 2.3 d and 3.6 ± 1.3 d respectively and did not vary according to disease category (P > 0.05). Bleeding manifestations were not related to platelet count (P > 0.05). There was no statistical difference in the demographic, clinical or laboratory observations according to disease category (P > 0.05). A total of 24 children had evidence of other co-infections and four had atypical complications. Dengue 2 virus was the strain reported in most of the cases. CONCLUSIONS: This study showed shift to higher age of presentation, more number of dengue hemorrhagic fever and associated co-infections in children. The complications and mortality was low and platelet recovery time was not influenced by disease category.

Immune response to second dose of MMR vaccine in Indian children.

BACKGROUND & OBJECTIVES: MMR vaccine in a two dose schedule has successfully eliminated measles, mumps and rubella from many developed countries. In India, it is not a part of national immunization programme but is included in the State immunization programme of Delhi as a single dose between 15-18 months. This prospective study was carried out to assess the extent of seroprotection against these three diseases in immunized children and to study the immune response to a second dose of MMR. METHODS: Consecutive children aged 4-6 yr, attending the immunization clinic of a tertiary care hospital in Delhi for routine DT vaccination, were enrolled. Second dose of MMR was given and pre- and post-vaccination antibody levels were compared. RESULTS: The pre-vaccination percentage seropositivity observed in the 103 children recruited, was 20.4 per cent for measles, 87.4 per cent for mumps and 75.7 per cent for rubella. Amongst the 84 children who were followed up after the second dose, the percentage seroprotection for measles rose from 21.4 (18/84) to 72.6 per cent (61/84) and 100 per cent became seroprotected to mumps and rubella. INTERPRETATION & CONCLUSIONS: The percentage of children protected against measles was found to be alarmingly low which needs to be investigated. Though the observed protection against mumps and rubella was adequate, its durability was not known. The need for re-appraisal of the current MMR immunization policy is called for by carrying out longitudinal studies on a larger sample.