Tumor-Induced Osteomalacia- Like Syndrome with Rickets and Infantile Hepatic Hemangioendothelioma.

Tumor-induced osteomalacia (TIO), a rare paraneoplastic syndrome is seen in association with the overproduction of fibroblast growth factor-23 (FGF-23) by certain mesenchymal tumors in adults. In children, these phosphaturic mesenchymal tumors produce features of rickets similar to TIO. This condition is characterized by elevated blood levels of FGF-23, low phosphate, low or normal active vitamin D, and high alkaline phosphatase. Though the removal of the tumor is curative; in cases where surgical resection is not possible, medical treatment is successful with phosphate and active vitamin D in the improvement of symptoms. The case of a child with features of rickets is presented here to illustrate the importance of identifying this rare condition and instituting appropriate intervention.

Clinico-Radiological Profile and Outcome of Airway Foreign Body Aspiration in Children: A Single-Center Experience From a Tertiary Care Center in Eastern India.

Background Foreign body aspiration is one of the leading causes of childhood morbidity and mortality among older infants and toddler age groups. Missed and delayed diagnosis of foreign body aspiration can lead to increased incidence of complications. Early diagnosis can prevent life-threatening complications and morbidity. In this study, we aimed to evaluate the clinical and radiological details, types, localization of foreign bodies, complications, and outcomes in pediatric patients who presented to our hospital with foreign body aspiration. Methodology We conducted a retrospective analysis of hospital case records of children aged one month to 14 years who were admitted to the Department of Pediatrics between June 2018 and May 2020, with clinical suspicion of foreign body aspiration. Results A total of 22 children with a diagnosis of airway foreign body were included. The mean age of presentation was three years (SD: ±2.22), with a boy-to-girl ratio of 3.4:1. Cough (81.8%) and tachypnea (72.7%) were the most common clinical symptoms. The median duration between symptom onset and diagnosis was three (interquartile range: 6) days. Unilateral reduced breath sound (81.8%) was the most common clinical examination finding. The common site of impaction was the right main bronchus in 59.1% of cases. The foreign bodies retrieved during bronchoscopy were organic substances in 63.6% of cases, with peanuts being the most common (31.8%). Chest radiographs were normal in 36.3% of cases, and common abnormalities included hyperinflation, collapse, consolidation, and mediastinal shift. Mechanical ventilation was required in 54.5% of cases. The mean duration of hospitalization was five (SD: ±2.84) days. Complications such as pneumothorax were seen in one (4.5%) case. Mortality was seen in 4.54% of cases during the bronchoscopic procedures. Conclusions Foreign body aspiration was common in young male children, with cough being the common symptom. Normal X-rays of the chest were seen in one-third of cases. The common site of impaction was the right main bronchus, and organic substances such as peanuts were common foreign bodies retrieved. Strong clinical suspicion of foreign body aspiration should be kept in cases with acute onset of cough in young children. Prompt medical attention is needed to reduce the morbidity and mortality associated with foreign body aspiration.

Eastern India Collaboration on Multisystem Inflammatory Syndrome in Children (EICOMISC): A Multicenter Observational Study of 134 Cases.

Background: Few single center studies from resource-poor settings have reported about the epidemiology, clinical feature and outcome of multisystem inflammatory syndrome in children (MIS-C). However, larger data from multi-center studies on the same is lacking including from Indian setting. Methods: This retrospective collaborative study constituted of data collected on MIS-C from five tertiary care teaching hospitals from Eastern India. Children ≤ 15 years of age with MIS-C as per the WHO criteria were included. Primary outcome was mortality. Results: A total of 134 MIS-C cases were included (median age, 84 months; males constituted 66.7%). Fever was a universal finding. Rash was present in 40%, and conjunctivitis in 71% cases. Gastro-intestinal and respiratory symptoms were observed in 50.7% and 39.6% cases, respectively. Co-morbidity was present in 23.9% cases. Shock at admission was noted in 35%, and 27.38% required mechanical ventilation. Fifteen (11.2%) children died. The coronary abnormalities got normalized during follow-up in all except in one child. Initial choice of immunomodulation had no effect on the outcomes. Presence of underlying co-morbidity, lymphopenia, thrombocytosis, hyponatremia, increased LDH (>300 U/L), and hypoalbuminemia were the factors significantly associated an increased mortality. Conclusions: MIS-C has myriad of manifestations. Underlying co-morbidity, lymphopenia, thrombocytosis, hyponatremia, increased LDH (>300 U/L), and hypoalbuminemia were associated with an increased mortality. No difference in outcome was noted with either steroid or IVIg or both. Coronary artery abnormalities resolved in nearly all cases.

Clinicopathological Profile and Outcome of Childhood Urticaria Vasculitis: An Observational Study.

Background Urticaria is a type III hypersensitivity reaction usually triggered by an infection, medication, or food item. It usually subsides within 24 hours without any residual lesion and does not have any systemic manifestation. Urticaria vasculitis (UV) is a clinicopathological condition defined by the presence of an urticarial lesion lasting for >24 hours or recurrent episodes of urticaria associated with histopathological features of leukocytoclastic vasculitis. Methods This retrospective study was conducted in a tertiary care teaching institute in Eastern India over a period of 2 and ½ years. Children presenting with urticaria lesions for a duration of > 24 hours that did not subside either spontaneously or with anti-histamines were admitted for further workup and management. Results During the study period (July 2015 to December 2017), a total of 20 children with urticaria needed admission for symptom control and further workup. There were 16 boys and 4 girls. The mean (SD) age of presentation was 6.5 years (±2.4). Besides urticaria in all, pain abdomen was present in 13 (65%) and fever in 6 (30%) children. Only one had arthritis. Skin biopsy performed in these children was suggestive of leukocytoclastic vasculitis. One child was ANA (anti-nuclear antibody) positive with low C3. All except three children required systemic steroid for symptom control along with other medications (anti-histamines). None had suffered any complication or relapse.  Conclusions Urticaria vasculitis (most common cause being idiopathic) remains underdiagnosed because of the need of confirmation by biopsy, which might not always be attempted in every case. Though anti-histamines remain the main stay of treatment, adding short course oral steroid shortens the course once infection is ruled out.