Evaluation of quality of abstracts of randomized controlled trials on procedural sedation in children after publication of CONSORT guidelines for abstracts: A systematic review.

The extension of the Consolidated Standards of Reporting Trials (CONSORT) statement provides guidelines for abstracts of randomized controlled trials (RCTs). This study was done to assess the reporting quality of abstracts of RCTs, on procedural sedation in children and identify factors associated with better quality. A PubMed search was conducted from inception of database till July 2017 to identify RCTs on procedural sedation in children. Search terms used were (procedural [All Fields] AND sedation [All Fields]) AND ("child" [MeSH Terms] OR "child" [All Fields] OR "children" [All Fields]) were included in the analysis, while primary RCTs, published in the English language unstructured abstracts, secondary analysis of primary RCTs and studies not exclusively on children we excluded. Our search strategy initially yielded 582 abstracts. Out of these, 535 abstracts were excluded. 47 articles were included in the final analysis. We extracted basic information and data on CONSORT items from abstracts. Each abstract was assessed using a 16-item composite abstract score (CAS) based on the CONSORT guidelines. This abstract quality was further explored by Method Score and by Result Score. Regression analysis was conducted to analyze factors associated with reporting quality. In majority of the abstracts, only objectives and conclusion were adequately reported. Inadequately reported items in >90% of abstracts included randomization, trial status, registration & funding. There was no significant difference in the CAS of abstracts (mean ± SD) published in & before 2008 (12.63 ± 4.0), to those published after 2009 (12.48 ± 4.23). Similarly, there was no significant difference in Result Score and Method Score of the abstracts. After the publication of 'CONSORT for abstracts' guideline, the quality of abstracts of RCTs on procedural sedation has shown suboptimal improvement. We suggest stricter adherence to guidelines by editors and reviewers. A checklist for adherence to CONSORT guidelines could be introduced during submission for the same.

Absent phallus: issues in management.

Aphallia or complete absence of penis is a rare disorder, often associated with other congenital anomalies of the genitourinary and gastrointestinal systems. This disorder is difficult to manage and has a very significant psychosocial impact on the child as well as the family. Individualized assessment and formulation of management plan is important.

Metabolic Disorders among Children Presenting with Acute Encephalopathy.

OBJECTIVE: To study the clinicoetiological profile of children presenting with acute noninfectious encephalopathy (NIE) and identify the proportion of children having inborn errors of metabolism (IEM). METHOD: This descriptive cross sectional study was conducted in a tertiary care centre in Northern India. Consecutive children, aged more than 28 d and less than 12 y, with acute encephalopathy were enrolled after ruling out CNS infection. All children were evaluated on an internally validated structured proforma. A sequential pre-decided battery of tests was applied to determine the cause of encephalopathy. IEM suspects were subjected to TMS/GCMS followed by mutation analysis for confirmation. RESULTS: Fifty children with noninfectious encephalopathy (NIE) were recruited and metabolic causes were detected in 9 of these children (18%), aged 3 to 42 mo, with female preponderance. The IEMs included lactic acidosis (4), glutaric aciduria (3), isovaleric academia (1), and hyperhomocysteinemia (1). History of previously affected siblings and consanguinity between the parents were important indicators of IEM. MS/MS and mutation analysis were the mainstay of diagnosis in these patients. IEMs contributed to the most common cause amongst cases of NIE. CONCLUSION: IEMs constitute a significant proportion of NIE in India and a high index of suspicion is required to make the diagnosis.

Coeliac disease presenting atypically: A much wider spectrum.

Atypical coeliac disease in young children is frequently missed when it presents atypically as non-gastrointestinal presentations to different specialties. There was a greater delay (54 months) in establishing the diagnosis in those with atypical coeliac disease (p < 0.001). No difference was observed in the mode of delivery or duration of breast feeding, but significant difference was observed between gestational age at birth (p < 0.001). Most cases showed stunted growth and underweight. Irritability, anaemia, rickets, dermatitis herpetiformis, alopecia and intussusception were other common predictors of atypical coeliac disease. Because of a myriad spectrum of non-gastrointestinal symptoms, at any age with diverse presentation, a high index of suspicion is therefore required.

CAH Newborn Screening in India: Challenges and Opportunities.

Congenital adrenal hyperplasia (CAH) is a common treatable disorder which is associated with life-threatening adrenal crisis, sexual ambiguity, and/or abnormal growth if undiagnosed. Newborn screening is a cost-effective tool to detect affected babies early after birth to optimize their treatment and follow-up. Newborn screening however is in its nascent stage in India where it is not yet introduced universally for all babies. The following review briefly highlights the challenges (e.g., lack of universal screening, healthcare resources) and opportunities (e.g., reduction in morbidity and early correct gender assignment in females) associated with newborn screening for CAH in a large Indian birth cohort.

Effect of intramuscular cholecalciferol megadose in children with nutritional rickets.

BACKGROUND: The treatment practices for vitamin D deficiency rickets are highly variable. Though a single intramuscular (IM) megadose of vitamin D is economical, and ensures good compliance, it poses the risk of hypervitaminosis D. This observational study was conducted to assess the duration of effect and safety of single IM megadose of cholecalciferol in the treatment of vitamin D deficiency rickets. METHODS: Children younger than 14 years with rickets were enrolled. Baseline investigations included radiograph of wrists and estimation of serum calcium, phosphate, alkaline phosphatase (ALP), 25(OH) vitamin D and parathormone (PTH) levels. All children received a single IM megadose of vitamin D3. Biochemical parameters were re-evaluated at 1.5, 3 and 6 months after the megadose and the values were compared to the baseline. RESULTS: We enrolled 21 children, out of which nine remained under active follow-up till 6 months. Radiological evidence of rickets was present in all 21 children, 14 had hypocalcemia at the time of presentation. After IM cholecalciferol megadose, median 25 hydroxy vitamin D [25(OH)D] level remained significantly more than the baseline till 6 months after the megadose. At 1.5 months after the vitamin D megadose, three (30%) of the children were found to develop toxic levels of vitamin D (>150 ng/mL), although none had hypercalcemia or any clinical manifestation of vitamin D toxicity. At 3 months and 6 months after the megadose, 25(OH)D levels remained in the sufficient range (20-100 ng/mL) in seven out of the eight children who came for follow-up. CONCLUSIONS: A single IM megadose of vitamin D may be effective in significantly increasing the 25(OH)D levels for at least 6 months in children with rickets, but elevation of 25(OH)D to toxic range raises concern regarding its safety.

Disseminated cryptococcosis.

BACKGROUND: Fungal infections, especially in immunocompetent children are uncommon causes of fever of unknown origin. CASE CHARACTERISTICS: A 5-year-old boy with prolonged fever and no evidence of immunosuppression. OBSERVATION: Ultrasound-guided retroperitoneal lymph node biopsy showed granulomas and intracytoplamic fungal yeasts; staining charactristics were suggestive of cryptococci. Clinical and radiological improvement was seen after treatment with amphoterecin-B. OUTCOME: Disseminated fungal infection should be suspected as a cause of pyrexia of unknown origin after ruling out the commoner causes. Biopsy from enlarged lymph node or organomegaly may yield the diagnosis when non-invasive tests fail.

Evaluation of predictors of adverse outcome in febrile neutropenic episodes in pediatric oncology patients.

OBJECTIVES: To identify predictors associated with adverse outcome in febrile neutropenic episodes among pediatric oncology patients between 1 and 18 y age, to ascertain the prevalence of invasive bacterial or fungal infection/mortality, to determine the common organisms causing invasive bacterial infection in children with febrile neutropenia and to evaluate their current antimicrobial sensitivity pattern. METHODS: It was an observational descriptive study conducted between February 2009 through July 2010. Febrile neutropenic episodes satisfying the inclusion criteria were enrolled. Relevant history was taken followed by a detailed clinical examination and laboratory examination. Logistic Regression analysis was used to identify significant predictors of adverse outcome in febrile neutropenic episodes. RESULTS: Out of the 155 febrile neutropenic episodes studied, adverse outcome occurred in 53(34 %) of the episodes. History of three or more previous episodes of febrile neutropenia, child being already on oral antibiotics and Chest Radiograph abnormality at presentation were found to be significantly associated with adverse outcome on multivariate logistic regression analysis. Documented invasive bacterial and fungal infection was seen in 27.8 % and 14.2 % episodes. Mortality occurred in 8 (5 %) of episodes. Gram negative bacterial infections were more common. Most common bacteria isolated was Escherichia coli and the commonest gram positive organism isolated was Staphylococcus aureus (MSSA). CONCLUSIONS: On multivariate analysis, the variables found to be significantly associated with adverse outcome in febrile neutropenic episodes were three or more previous episodes of febrile neutropenia, child being already on oral antibiotics and Chest Radioraph abnormality at presentation.

Tobramycin for the treatment of bacterial pneumonia in children.

INTRODUCTION: Common etiological agents for community-acquired lower respiratory tract infection (LRTI) include Streptococcus pneumoniae, Hemophilus influenzae and Mycoplasma pneumoniae and can be easily managed with oral or intravenous antibiotics. However, LRTI in patients with underlying illnesses, such as cystic fibrosis (CF) and immune deficiency, or on ventilator support is difficult to manage because these are caused by Gram-negative bacilli. Tobramycin has been shown to be effective in the management of these patients. AREAS COVERED: Information about the antimicrobial activity, pharmacological aspects (including pharmacokinetics and pharmacodynamics), clinical efficacy, safety and side effects of tobramycin have been covered in this review. EXPERT OPINION: A major advance for the use of tobramycin has occurred with its use by the inhalational route, in children with CF. The inhalation route provides the advantage of ease of administration for prolonged periods at home and allows use of very high doses. Systematic reviews suggest that tobramycin inhalation improves outcome, decreases the need for hospitalization and decreases the need for use of frequent systemic antibiotics in CF patients colonized with pseudomonas. Data on the efficacy of inhaled tobramycin in non-CF bronchiectasis are scarce, as are data on the prevention and treatment of ventilator-associated pneumonia, and on the role of combining inhaled tobramycin with systemic tobramycin. Despite limitations, this drug has the potential to be used in various conditions other than CF.

Frontonasal dysplasia (Median cleft face syndrome).

This is a report of a rare case of frontonasal dysplasia (FND) in a full-term girl with birth weight of 2.750 kg. The baby had the classical features of FND. There were no other associated anomalies. There was no history of consanguinity and no family history of similar conditions. So inheritance of this case could be considered sporadic. Maxillofacial surgery should be considered for all patients for whom improvement is possible. However, in developing countries where there are considerable limitations in provision of social services, with economic and educational constraints, correction of such major defects remains a challenging task.

Clinical profile of Langerhans Cell Histiocytosis at a tertiary centre: a prospective study.

OBJECTIVE: To study the varied presentations of Langerhans Cell Histiocytosis (LCH), the differential diagnosis of the varied presentations and the time lag in achieving the diagnosis. Prospective analysis of children diagnosed to have LCH over a period of 51 mo was done. A complete history and physical examination was undertaken in all patients, followed by relevant laboratory and radiological evaluation. Biopsy of the appropriate specimen was done. The extent of the disease was documented, accordingly treated and followed up. RESULTS: There were 16 children with LCH from October 2005 through December 2009. The age ranged from 8 mo to 72 mo. Diagnosis was confirmed by CD1a/S 100 in 15 children (93.75%). The mean time to arrive at the diagnosis was 9.9 mo. Multisystem disease was documented in 11 (68.75%) children and there were 4 (25.0%) cases of pulmonary LCH. The mean time of follow-up was 14.4 mo (range, 1 mo to 50.6 mo). Most common referral diagnoses in LCH patients was recurrent pneumonia and immunodeficiency. CONCLUSIONS: There is a need for high index of suspicion for diagnosis of LCH; misdiagnosis is frequent. Pulmonary involvement in children with LCH appears common. It is possibly still underdiagnosed. Nail changes are uncommon, but may act as a marker for multisystem disease. In addition to survival data and analysis of prognostic factors, the prospective collection of data on diverse presentations is essential, along with a high index of suspicion for the diagnosis of LCH.