Pradeep Deivasigamani, Suprit Basu, Rashmi Rikhi, Kanika Arora, Vibhu Joshi, Richa Jain, Amit Rawat, Surjit Singh, Deepti Suri
{"title":"Screening for Wiskott-Aldrich Syndrome in Chronic Idiopathic Thrombocytopenic Purpura.","authors":"Pradeep Deivasigamani, Suprit Basu, Rashmi Rikhi, Kanika Arora, Vibhu Joshi, Richa Jain, Amit Rawat, Surjit Singh, Deepti Suri","doi":"10.1007/s12098-025-05712-6","DOIUrl":"10.1007/s12098-025-05712-6","url":null,"abstract":"<p><p>The clinical spectrum of X-linked thrombocytopenia (XLT)/ Wiskott-Aldrich syndrome (WAS) is broad, ranging from mild, intermittent thrombocytopenia to the classical severe phenotype characterized by eczema, infections, and thrombocytopenia. Patients with XLT often lack the complete triad but manifest with thrombocytopenia-associated bleeding and are hence misdiagnosed as chronic immune thrombocytopenia (ITP). Moreover, the clinical picture is further complicated by the development of autoimmune cytopenia. Early identification is critical, as definitive treatment with hematopoietic stem cell transplantation (HSCT) can be offered, and unnecessary immunosuppressive therapies, including corticosteroids, can be avoided. In this study, the authors screened a cohort of boys with chronic ITP using flow cytometry-based WASp protein expression analysis, followed by genetic testing. Among 38 patients evaluated, 5 (13.5%) had pathogenic variants in the WAS gene. Clinical features such as X-linked family history, eczema, low mean platelet volume (MPV), or reduced WASp expression in chronic ITP patients with suboptimal response to immunomodulatory drugs should prompt genetic evaluation for WAS.</p>","PeriodicalId":13320,"journal":{"name":"Indian Journal of Pediatrics","volume":" ","pages":"1104-1106"},"PeriodicalIF":2.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144821371","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Nomograms and Reference Ranges for Intra-Cranial Ventricular Dimensions in Indian Neonates.","authors":"Ayush Khare, Ajay Kumar Gaur, Megha Mittal, Satvik Chaitanya Bansal","doi":"10.1007/s12098-024-05274-z","DOIUrl":"10.1007/s12098-024-05274-z","url":null,"abstract":"<p><strong>Objectives: </strong>To establish normal reference values for commonly used ventricular indices - Anterior Horn Width (AHW), Ventricular Index (VI) and Thalamo-Occipital Distance (TOD)-against common variables including gestational age, birth weight and head circumference in Indian newborns.</p><p><strong>Methods: </strong>This was a cross-sectional study. Cranial ultrasound was performed between post-natal days 3 to 7. Anterior fontanelle was taken as the acoustic window. Data were analyzed to determine correlation, coefficient of determination (R2), regression equations and plotted against gestational age (GA).</p><p><strong>Results: </strong>Total 2200 newborns were included in the study. The study observed that there is an increase in VI, AHW and TOD with an increase in gestational age (p-value < 0.001) and birth weight (p-value < 0.001). No significant correlation of gender and singleton or multiple gestation-with the intraventricular dimensions was observed (p-value > 0.05). All the three ventricular parameters were found to have a significantly lower mean value in the normal vaginal delivery group as compared to the LSCS group. There was a good correlation between ventricular indices of the left and right side. Nomograms of AHW, TOD, and VI were prepared with respect to gestational age.</p><p><strong>Conclusions: </strong>Intracranial ventricular size dimensions vary significantly with increasing gestational age and birth weight. The new nomograms for various ventricular indices of Indian neonates can assist in objectively assessing ventricular sizes.</p>","PeriodicalId":13320,"journal":{"name":"Indian Journal of Pediatrics","volume":" ","pages":"1085-1091"},"PeriodicalIF":2.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142390183","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Insulin Like Growth Factor-1 as a Screening Tool to Differentiate Between Idiopathic Short Stature and Growth Hormone Deficiency.","authors":"Vaman Khadilkar, Madhura Karguppikar, Aboli Bhalerao, Karishma Bhade, Anuradha V Khadilkar","doi":"10.1007/s12098-025-05687-4","DOIUrl":"10.1007/s12098-025-05687-4","url":null,"abstract":"<p><p>Measurement of serum insulin-like growth factor-1 (IGF-1) is a useful screening test for growth hormone deficiency (GHD) because unlike growth hormone, it has minimal circadian variation. In this retrospective study, the authors compared IGF-1 concentrations in children with GHD vs. idiopathic short stature (ISS) to define IGF-1 cut-off to differentiate the two. Of the 75 children studied, 38 were GHD and 37 ISS. Weight, height, BMI Z-scores, bone age: age ratio, IGF-1 concentrations and IGF-1 Z-scores were significantly lower in the GHD group. The best IGF-1 Z-score cut-off was -2.41 (sensitivity: 74.3% and specificity: 89.2%). Many children who are non-GH deficient fail growth hormone stimulation tests and get wrongly diagnosed as GHD and receive a lesser dose of GH leading to suboptimal response. In such patients, if the IGF-1 is well above the cut-off (-2.41), other diagnostic possibilities including ISS may be considered for GH dose adjustment.</p>","PeriodicalId":13320,"journal":{"name":"Indian Journal of Pediatrics","volume":" ","pages":"1098-1100"},"PeriodicalIF":2.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144730140","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Acute Myocardial Infarction in an Adolescent: Correspondence.","authors":"Jagabandhu Ghosh","doi":"10.1007/s12098-025-05698-1","DOIUrl":"10.1007/s12098-025-05698-1","url":null,"abstract":"","PeriodicalId":13320,"journal":{"name":"Indian Journal of Pediatrics","volume":" ","pages":"1135"},"PeriodicalIF":2.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144742056","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Open versus Closed Suctioning Among Mechanically Ventilated Pediatric Patients: A Randomised Control Trial - Correspondence-2.","authors":"K S Yatheesh, Joseph John","doi":"10.1007/s12098-025-05731-3","DOIUrl":"10.1007/s12098-025-05731-3","url":null,"abstract":"","PeriodicalId":13320,"journal":{"name":"Indian Journal of Pediatrics","volume":" ","pages":"1150"},"PeriodicalIF":2.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144952331","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"RAM Cannula versus Short Binasal Prongs for Non-invasive Ventilation in Preterm Infants: An Updated Systematic Review and Meta-analysis.","authors":"Jogender Kumar, Jitendra Meena, Pradeep Debata, Venkataseshan Sundaram, Sourabh Dutta, Praveen Kumar","doi":"10.1007/s12098-024-05211-0","DOIUrl":"10.1007/s12098-024-05211-0","url":null,"abstract":"<p><strong>Objectives: </strong>To compare the efficacy and safety of RAM cannula with short binasal prongs (SBPs) as nasal interfaces in preterm infants requiring nasal continuous positive airway pressure (nCPAP) or nasal intermittent positive pressure ventilation (NIPPV).</p><p><strong>Methods: </strong>The authors searched electronic databases (Medline, Embase, and Web of Science) and trial registries from inception until March 15, 2024, for randomized controlled trials (RCTs) comparing the RAM cannula with SBP for delivering nCPAP/NIPPV. They performed a random-effects meta-analysis using RevMan 5.4 software. The primary outcome was failure of nCPAP/NIPPV. Secondary outcomes included nasal injury, mechanical ventilation, air leaks, and mortality.</p><p><strong>Results: </strong>Five RCTs (825 participants) were included. There was no significant difference in nCPAP/NIPPV failure (RR: 1.04; 95% CI: 0.58 to 1.87) or the need for invasive mechanical ventilation (RR: 1.23; 95% CI: 0.75 to 2.01) between the RAM cannula and SBP groups (low to very low certainty). Compared with infants in the SBP group, those in the RAM cannula group had a significantly lower incidence of moderate to severe nasal injury [(5 RCTs, 825 participants; RR: 0.34; 95% CI: 0.18 to 0.66); low certainty] and any nasal injury [(RR: 0.44; 95% CI: 0.26 to 0.76; very low certainty)]. There was no significant difference in the other clinical outcomes.</p><p><strong>Conclusions: </strong>In comparison to SBP, the RAM cannula may have little to no effect on nCPAP/NIPPV failure, but the evidence is very uncertain. Low-certainty evidence suggests that the use of RAM cannula possibly results in reduction in moderate to severe nasal trauma in preterm infants receiving nCPAP/NIPPV.</p>","PeriodicalId":13320,"journal":{"name":"Indian Journal of Pediatrics","volume":" ","pages":"1075-1084"},"PeriodicalIF":2.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141859581","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Management and Outcomes of Children with Malignant Germ Cell Tumor.","authors":"Kaushal Kulkarni, Sandeep Agarwala, Vishesh Jain, Anjan Dhua, Devender Kumar Yadav, Prabudh Goel, M Srinivas, Sameer Bakhshi","doi":"10.1007/s12098-024-05223-w","DOIUrl":"10.1007/s12098-024-05223-w","url":null,"abstract":"<p><strong>Objectives: </strong>To assess the clinico-pathological features, management and outcomes, amongst extracranial malignant germ cell tumors (MGCTs) in children treated primarily at a tertiary care center in a resource-challenged nation.</p><p><strong>Methods: </strong>The prospectively maintained data for children below 14 y of age treated for extracranial MGCT from May 1994 to January 2023 was analyzed for patient characteristics, management, event-free survival (EFS) and overall survival (OS) and factors effecting survival. Events was defined as death, recurrence and progression. Multivariate logistic regression analysis was performed to identify the factors independently predicting unfavorable outcomes.</p><p><strong>Results: </strong>One hundred and seventy-seven children (37% males) with a median (IQR) age at presentation of 30 mo (range 2-168 mo) were included. The cohort consisted of 87 (49%) extra-gonadal and 90 (51%) gonadal cases. Disease was metastatic at presentation in 48 (27%) with lungs being the most common site. Neoadjuvant chemotherapy (NACT) was given to 119 (67%) and finally 162/177 (92%) had undergone resection of the primary tumor. Endodermal sinus tumor (EST) was the commonest histological subtype in 141 children (73%). Twenty-two (12%) patients had died giving a 5-y OS of 84.7% (95% CI 78.3- 91.1). Recurrence occurred in 25 patients, and an additional 5 patients had progression giving a 5-y EFS of 69.9% (95% CI 62.5- 77.3). Stage III (p = 0.05), Stage IV (p = 0.006) and extra-gonadal site (p = 0.05) were significantly associated with poorer EFS.</p><p><strong>Conclusions: </strong>Children with MGCT have a favorable outcome with 5-y OS of 84.7% and EFS of 69.9%. Stage III and IV disease and extra-gonadal sites were independent predictors of a poor outcome.</p>","PeriodicalId":13320,"journal":{"name":"Indian Journal of Pediatrics","volume":" ","pages":"1069-1074"},"PeriodicalIF":2.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141889067","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Population Specific Nomograms for Ventricular Dimensions - A Step Forward in the Management of Neonates with Ventricular Dilatation.","authors":"Bharathi Balachander, Ballambattu Vishnu Bhat","doi":"10.1007/s12098-025-05656-x","DOIUrl":"10.1007/s12098-025-05656-x","url":null,"abstract":"","PeriodicalId":13320,"journal":{"name":"Indian Journal of Pediatrics","volume":" ","pages":"1047-1048"},"PeriodicalIF":2.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144583812","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}