Ovarian tumors in children: 10‑year experience from a tertiary care center in South India.

BACKGROUND: Ovarian tumors are uncommon in childhood and constitute around 1% of childhood malignancies. Two thirds of pediatric ovarian tumors are germ cell tumors. Epithelial ovarian tumors and stromal tumors are less frequent. We share our experience in childhood ovarian cancers, analyzing a series of cases with respect to the clinical profile, treatment and survival. METHODS: All newly diagnosed ovarian tumors in children up to 14 years of age, registered in our Pediatric Oncology Division between January 2000 and December 2009 were retrospectively reviewed. OBSERVATIONS: There were 47 patients with newly diagnosed ovarian tumors. The mean age at presentation was 10.0 ± 3.4 years. The most common symptoms at presentation were acute abdominal pain (48.9%) and abdominal mass (40.4%). Precocious puberty was uncommon (6.3%). Histology was germ cell tumors in 44 cases and nongerm cell tumors in three cases. The benign teratomas (mature and immature grade 1 and 2; n=9) underwent complete surgical resection alone; none had recurrence on follow up. Of the remaining 35 GCTs, 31 patients were given chemotherapy and 4 refused treatment.26 out of the 31 patients completed chemotherapy with BEP (bleomycin, etoposide, cisplatin) regimen with acceptable toxicities. 5 children i.e.; (19.2%) developed recurrence. At a median follow up of 80 months, 10 year disease free survival was 80.8 ± 7.7% and 10 year overall survival was 92.7 ± 4.9%. CONCLUSION: Germ cell tumors are the most common ovarian malignancy in children. With surgery and chemotherapy using BEP, good outcome can be expected in these patients.

Osteosclerotic bone dysplasia of the craniofacial bones.

Melorheostosis is a rare sclerosing bone dysplasia which is characterized by a localized, diffuse thickening of the cortical bone mainly affecting diaphysis of long bones, and bones of hand and feet unilaterally. Involvement of the maxillofacial region is rare and the diagnosis is mainly based on radiographic features. We report a rare case of a 5 year old girl with Osteosclerotic Bone Dysplasia (Melorheotosis) of multiple craniofacial bones.

RTI v medical ethics: some questions arising from the recent decision of the Chief Information Commissioner under the RTI Act.

Medical ethics attaches the utmost priority to the confidentiality of medical records. Hence, the decision of the Chief Information Commissioner (CIC) rendered on April 10, 2015 in Case No: CIC/KY/A/2014/001348SA Ms Jyoti Jeena v. PIO, Institute of Human Behaviour & Allied Science (hereinafter referred to as Jyoti Jeena), that the wife-applicant is entitled to get copies of the medical records of her estranged husband has raised many eyebrows.

INCLEN Diagnostic Tool for Neuromotor Impairments (INDT-NMI) for Primary Care Physician: Development and Validation.

Objectives: To develop and validate a diagnostic tool for use by primary care physicians for diagnosing neuro-motor impairment among 2-9 year old children in primary care settings. Study design: Modified Delphi technique involving national (n=49) and international (n=6) experts was used for development of INDT-NMI. The tool was then validated through a cross sectional study. Setting: Neurology specialty clinics of three tertiary care pediatric centers in New Delhi, India. Participants: 454 children aged 2-9 years [mean (SD) age: 60.4 (23.7) mo], selected through systematic random sampling, underwent assessment for identification and classification of neuromotor impairments (NMI). Intervention: All study subjects were first administered INDTNMI (candidate test) by a trained physician followed by expert assessment for NMI and other neurodevelopment disorders (NDD) by team of two pediatric neurologists (Gold standard). Results: According to expert evaluation, 171 (37.8%) children had neuromotor impairments. There were four categories of subjects: NMI alone (n=66); NMI+other NDDs (n=105); Other NDDs without NMI (n=225) and ‘Normal’ group (n=58). Using expert evaluation as gold standard, overall sensitivity of the INDTNMI was 75.4% and specificity was 86.8%. INDT-NMI helped graduate physicians to correctly classify 86.6% (112/129) children with NMI into different types (cerebral palsy, neuromotor diseases and other NMI). Graduate physicians assigned 40 children (8.8%) as ‘indeterminate’, 38 (95%) of whom had either NDD and/or NMI and thus merited referral. Misclassification of NMI occurred in those with mild changes in muscle tone, dystonia, or ataxia and associated NDDs. Conclusion: Graduate primary care physicians with a structured short training can administer the new tool and diagnose NMI in 2-9 year old children with high validity. INDT-NMI requires further evaluation in actual primary care settings.

INCLEN Diagnostic Tool for Epilepsy (INDT-EPI) for Primary Care Physicians: Development and Validation.

Objective: To evaluate the diagnostic accuracy of a new diagnostic instrument for epilepsy – INCLEN Diagnostic Tool for Epilepsy (INDT-EPI) – with evaluation by expert pediatric neurologists. Study design: Evaluation of diagnostic test. Setting: Tertiary care pediatric referral centers in India. Methods: Children aged 2-9 years, enrolled by systematic random sampling at pediatric neurology out-patient clinics of three tertiary care centers were independently evaluated in a blinded manner by primary care physicians trained to administer the test, and by teams of two pediatric neurologists. Outcomes: A 13-item questionnaire administered by trained primary care physicians (candidate test) and comprehensive subject evaluation by pediatric neurologists (gold standard). Results: There were 240 children with epilepsy and 274 without epilepsy. The candidate test for epilepsy had sensitivity and specificity of 85.8% and 95.3%; positive and negative predictive values of 94.0% and 88.5%; and positive and negative likelihood ratios of 18.25 and 0.15, respectively. Conclusion: The INDT-EPI has high validity to identify children with epilepsy when used by primary care physicians.

INCLEN Diagnostic Tool for Attention Deficit Hyperactivity Disorder (INDT-ADHD): Development and Validation.

Objective: To develop and validate INCLEN Diagnostic Tool for Attention Deficit Hyperactivity Disorder (INDT-ADHD). Design: Diagnostic test evaluation by cross sectional design. Setting: Tertiary care pediatric centers. Participants: 156 children aged 65-117 months. Methods: After randomization, INDT-ADHD and Connor’s 3 Parent Rating Scale (C3PS) were administered, followed by an expert evaluation by DSM-IV-TR diagnostic criteria. Main outcome measures: Psychometric evaluation of diagnostic accuracy, validity (construct, criterion and convergent) and internal consistency. Results: INDT-ADHD had 18 items that quantified symptoms and impairment. Attention deficit hyperactivity disorder was identified in 57, 87 and 116 children by expert evaluation, INDT-ADHD and C3PS, respectively. Psychometric parameters of INDT-ADHD for differentiating attention deficit hyperactivity disorder and normal children were: sensitivity 87.7%, specificity 97.2%, positive predictive value 98.0% and negative predictive value 83.3%, whereas for differentiating from other neuro-developmental disorders were 87.7%, 42.9%, 58.1% and 79.4%, respectively. Internal consistency was 0.91. INDT-ADHD has a 4-factor structure explaining 60.4% of the variance. Convergent validity with Conner’s Parents Rating Scale was moderate (r =0.73, P= 0.001). Conclusions: INDT-ADHD is suitable for diagnosing attention deficit hyperactivity disorder in Indian children between the ages of 6 to 9 years.

INCLEN Diagnostic Tool for Autism Spectrum Disorder (INDT-ASD): Development and Validation.

Objective: To develop and validate INCLEN Diagnostic Tool for Autism Spectrum Disorder (INDT-ASD). Design: Diagnostic test evaluation by cross sectional design Setting: Four tertiary pediatric neurology centers in Delhi and Thiruvanthapuram, India. Methods: Children aged 2-9 years were enrolled in the study. INDT-ASD and Childhood Autism Rating Scale (CARS) were administered in a randomly decided sequence by trained psychologist, followed by an expert evaluation by DSM-IV TR diagnostic criteria (gold standard). Main outcome measures: Psychometric parameters of diagnostic accuracy, validity (construct, criterion and convergent) and internal consistency. Results: 154 children (110 boys, mean age 64.2 mo) were enrolled. The overall diagnostic accuracy (AUC=0.97, 95% CI 0.93, 0.99; P<0.001) and validity (sensitivity 98%, specificity 95%, positive predictive value 91%, negative predictive value 99%) of INDT-ASD for Autism spectrum disorder were high, taking expert diagnosis using DSM-IV-TR as gold standard. The concordance rate between the INDT-ASD and expert diagnosis for ‘ASD group’ was 82.52% [Cohen’s κ=0.89; 95% CI (0.82, 0.97); P=0.001]. The internal consistency of INDT-ASD was 0.96. The convergent validity with CARS (r = 0.73, P= 0.001) and divergent validity with Binet-Kamat Test of intelligence (r = -0.37; P=0.004) were significantly high. INDT-ASD has a 4-factor structure explaining 85.3% of the variance. Conclusion: INDT-ASD has high diagnostic accuracy, adequate content validity, good internal consistency high criterion validity and high to moderate convergent validity and 4-factor construct validity for diagnosis of Autistm spectrum disorder.

Management of Children with Severe Acute Malnutrition: Experience of Nutrition Rehabilitation Centers in Uttar Pradesh, India.

Objective: To assess the effectiveness of facility-based care for children with severe acute malnutrition (SAM) in Nutrition Rehabilitation Centers (NRC). Design: Review of data. Setting: 12 NRCs in Uttar Pradesh, India. Participants: Children admitted to NRCs (Jan 1, 2010 - Dec 31, 2011). Intervention: Detection and treatment of SAM with locallyadapted protocols. Outcomes: Survival, default, discharge, and recovery rates. Results: 54.6% of the total 1,229 children admitted were boys, 81.6% were in the age group 6-23 months old, 86% belonged to scheduled tribes, scheduled castes, or other backward castes, and 42% had edema or medical complications. Of the 1,181 program exits, 14 (1.2%) children died, 657 (47.2%) children defaulted, and 610 (51.7%) children were discharged The average (SD) weight gain was 12.1 (7.3) g/kg body weight/day and the average (SD) length of stay was 13.2 (5.6) days. 206 (46.8%) children were discharged after recovery (weight gain ≥15%) while 324 (53.2%) were discharged, non-recovered (weight gain <15%) Conclusions: NRCs provide life-saving care for children with SAM; however, the protocols and therapeutic foods currently used need to be improved to ensure the full recovery of all children admitted.

Development and Validation of Language Evaluation Scale Trivandrum for Children Aged 0-3 years - LEST (0-3).

Objective: To develop and validate a simple screening tool which can be used in the Community to identify delay in language development among children of 0-3 years of age. Methods: The normal range for the 33-items of “Language Evaluation Scale Trivandrum for 0-3years-LEST(0-3)” were carefully selected from various existing language development charts and scales, by experts keeping in mind the face validity and content validity. The criterion validity was assessed using a community sample of 643 children of 0 to 3 years of age, including 340 (52.9%) boys. LEST (0-3) was validated against Receptive Expressive Energent Language Scale, for screening delay in language development among children of 0-3 years. Results: When one item delay was taken as ‘LEST delay’ (test positive), the sensitivity and specificity of LEST(0-3), was found to be 95.85% and 77.5%, respectively with a negative predictive value of 99.8% and LR (negative) of 0.05.When two item delay was taken as ‘LEST delay’ (test positive), the sensitivity and specificity of LEST(0-3), was found to be 66.7% and 94.8% respectively with a negative predictive value of 98.7% and LR (negative) of 0.35. The test-retest and inter-rater reliability were good and acceptable (Inter-class correlation of 0.69 for testretest and 0.94 for inter-rater). Conclusion: LEST (0-3) is a simple, reliable and valid screening tool for use in the community to identify children between 0-3 years with delay in language development, enabling early intervention practices.

Tumefactive demyelinating lesions: A Clinicopathological correlative study.

Tumefactive demyelinating (TDL) lesions are focal zones of demyelination in the central nervous system and they often mimic the neuroimaging features of an intraxial neoplasm. In this report we describe the clinical, neuroimaging and neuropathological features of six cases of TDL. Only in two patients the neuroimaging features in MRI (magnetic resonance imaging) scans were suggestive of TDL while in the other four cases a diagnosis of glioma was suggested. In order to establish a confirmatory diagnosis neuronavigation/stereotactic biopsy was undertaken and the diagnosis of TDL was established in all six cases at histopathology. Two out of six patients did not respond to the conventional corticosteroid therapy and they were treated with plasma exchange. It is being concluded that neuronavigation biopsy, though provide only a small amount of tissue, and is extremely useful in making the diagnosis of TDL.

Iron Deficiency as a Risk Factor for Simple Febrile Seizures–A Case Control Study.

Objective: To study the role of iron deficiency as a risk factor for simple febrile seizures. Design: Case control study. Setting: Pediatric department of a tertiary care teaching hospital. Participants: 154 cases and 154 controls were included in the study. Consecutive cases and concurrent controls were selected. Cases were children of age group 6 months to 3 years presenting with simple febrile seizures. Controls were children of same age group presenting with short febrile illness but without any seizures. Methods: After informed consent, detailed history was taken and clinical examination done in both cases and controls and blood investigations were done to diagnose iron-deficiency in both cases and controls. Iron deficiency was diagnosed as per WHO criteria (hemoglobin value <11g%, red cell distribution width of >15% and serum ferritin value < 12ng/mL). Other explanatory variables, which can be the potential confounders were also included in the study and considered for analysis. Results: Highly significant association was found between iron deficiency and simple febrile seizures in both univariate and multivariate analysis. Crude odds ratio was 5.34 (CI 3.27- 8.73, P<0.001) and adjusted odds ratio in the logistic regression analysis was 4.5 (CI 2.69- 7.53, P <0.001). Conclusions: Iron deficiency is a significant risk factor for simple febrile seizures in children of age group 6 months to 3 years.

Antibacterial and antialgal studies of some lanthanide schiff base complexes.

A new series of rare earth complexes of the Schiff base 4– hydroxybenzilidene–2–aminophenol having the general formula [Ln(HL)2(H2O)3]NO3 have been synthesised and characterized by elemental analysis, conductance studies, infrared and electronic spectral analysis and magnetic studies. The Schiff base and the complexes were screened for their biological activity against the bacteria, E.coli, B.subtilis, S.aureus and P.aeruginosa and the algae, Oscillaforia subrivis and Chlorella pyrenoiclosa. All the complexes were highly active against the bacterial species; Schiff base being less active comparatively. Algal growth was effectively inhibited by the ligand and the complexes, exhibiting total inhibition against the unicellular green algae, Chlorella pyrenoiclosa.

Retrospective study of severe cases of leptospirosis admitted in the intensive care unit.

OBJECTIVES: Evaluate patient demographics, risk factors, complications, seropositivity, treatment and outcome among leptospirosis patients. DESIGN: Retrospective analysis of 104 patients admitted in the intensive care unit (ICU) with a clinical suspicion of leptopirosis. SETTING: Ten-bedded medical ICU in a medical school situated in a rural area endemic for leptospirosis. MAIN OUTCOME MEASURES: Seropositivity for leptospirosis, patient demographics, risk factors, complications, treatment and survival. RESULTS: One hundred and four patients were admitted with a clinical suspicion of leptospirosis. Fifty-three (50.7%) were serologically confirmed cases. Males dominated both groups. Most of the admissions were in the monsoon season. Exposure to moist soil was the main risk factor. The mortality in the seronegative group was 26.8% while it was only 3.8% in the seropositive group. Multi-organ dysfunction syndrome, primarily acute respiratory distress syndrome with thromboctyopenia and renal failure were the causes for mortality. All the patients who died presented late into the illness. CONCLUSIONS: The initial diagnosis of leptospirosis depends on a high index of clinical suspicion, routinely available diagnostic tests being unreliable in the initial period. A reliable, unsophisticated test should be developed for early detection of this disease. As leptospirosis in its early stage mimics other tropical infections, both medical professionals and the general public (especially with risk of occupational exposure) should be educated about the disease and the need to seek early medical intervention.

Carotid artery stenting: results and long-term follow-up.

BACKGROUND AND PURPOSE: The role of carotid artery stenting (CAS) as an alternative to carotid endarterectomy in the treatment of for symptomatic carotid artery stenosis is investigated. MATERIALS AND METHODS: Forty-seven patients underwent CAS over 10-year period. Forty-nine vessels were treated. Stenosis quantification was done using North American symptomatic carotid endarterectomy trial method. The mean follow-up period by clinical and Duplex examination ranged is 5.6 years. RESULTS: The technical success rate was 100%. There were four deaths (8.1%) and two (4.1%) minor strokes within thirty days of procedure. There was no major strokes. All patients with minor stroke achieved complete recovery at 1-month follow up. Two deaths occurred probably due to hyperperfusion syndrome (HS) and two due to cardiac arrest. CONCLUSION: CAS is an effective treatment modality of symptomatic carotid artery disease but should be carefully done in high-risk groups having severe medical ailments and those having severe bilateral stenosis of the carotid arteries.

Early detection of HIV-1 in infants by PCR.

Infants of HIV-infected mothers are at great risk of becoming infected with HIV during childbirth. Many infants acquire HIV during labor and delivery. Others can acquire HIV through the mixing of fetal and maternal blood as the placenta separates. The duration of membrane rupture, acute chorioamnionitis and invasive delivery techniques that increase the baby's contact with the mother's blood have been associated with higher risks of MTCT during labor and delivery. HIV is present in breast milk and risk of its transmission during breastfeeding depends on several factors, including: infant age, pattern of breastfeeding, breastfeeding duration, breast health, maternal viral load and maternal immune status. Infants born to HIV infected mothers carry their mother's antibodies in their blood into the second year of life, even if the infants themselves are not infected. For this reason, standard HIV antibody tests cannot reliably confirm HIV infection in infants until after the maternal antibodies have disappeared. Tests that can diagnose pediatric HIV infection accurately during the first year of life include HIV-PCR, CD4/CD8 counts, P24 antigen tests, and viral cultures. PCR offers an effective tool to reliably diagnose HIV in a pediatric age group. Nineteen infants born by normal delivery to HIV-1 seropositive mothers were studied by PCR for the HIV1 env gene. Thirteen babies (68.5%) were negative whereas 6 babies were found to be positive (31.5%). Although PCR is one of the most useful tests for this clinical situation, it is not definitive. Therefore, PCR should be interpreted with caution and repeated at regular defined intervals, preferably lasting until the HIV antibody status of the infant is resolved.