Evaluation of Bone Mineral Density in Children With Hemophilia: An Observational Case-Control Study.

OBJECTIVES: The objectives of this study were to study bone mineral content (BMC), bone mineral density (BMD), vitamin D level, and bone turnover markers in children with hemophilia and compare it with their normal counterparts. DESIGN: This was an observational case-control study. SETTING: This study was conducted in our tertiary care institute during the period spanning from September 2016 to June 2018. MATERIALS AND METHODS: A total of 38 children with hemophilia 2 to 18 years of age and 38 age-matched and sex-matched healthy controls were included. Children with symptomatic hypocalcemia and those receiving drugs affecting BMC and BMD were excluded. Dual-energy x-ray absorptiometry was performed to estimate BMC and BMD. 25-Hydroxyvitamin D [25(OH)D], intact parathormone, osteocalcin, calcium, phosphate and alkaline phosphatase, and spot urine pyrilinks-D/creatinine ratio were estimated in them. RESULTS: BMC and BMD in cases was lower than that in controls (P<0.05). Prevalence of low BMC was seen in 22 (58%) and low BMD in 8 (21%) of cases. All controls had normal BMC and BMD for age. The prevalence of low vitamin D level (<20 ng/mL) was seen in 36 (95%) among cases and in 25 (65%) among controls (P<0.001). Serum phosphorus was lower, and serum alkaline phosphatase was higher in cases than in controls (P<0.05). CONCLUSION: BMC, BMD, and vitamin D in hemophiliacs were lower than in the general population.

Clinical Outcome and its Predictors in Children With Newly Diagnosed Immune Thrombocytopenia.

OBJECTIVE: To determine the predictors for chronic and/or persistent immune thrombocytopenia (ITP) among children with newly diagnosed ITP. METHODS: Ours was a mixed-design study (prospective: January 2020 to March 2022 and retrospective: January 2014 to December 2019), wherein we enrolled children, aged 1 month to 18 years presenting with newly diagnosed ITP. RESULTS: Of the 64 enrolled participants, 58 were followed up for atleast 1-year duration and 6 children were followed up for 3 to 12 months' duration. The median (IQR) age of the cohort was 8 (5, 11) years with a female preponderance (62.5%). Wet bleeding was seen in 56%; 6.25% developed intracranial bleeding. 67.2% (43/64) and 41.4% (24/58) children developed persistent and chronic ITP, respectively. Of the 34 children who achieved complete response at 12-months follow up, 21 (62%) achieved complete response by 3 months and the rest achieved complete response over the next 9 months. Development of overall response (complete or partial) at 3 and 12 months, was associated with a higher absolute lymphocyte count (ALC) at admission. The median ALC (×103/µL) at admission was 3.77 and 2.87 in children who had overall response and no response at 3 months, respectively (P = 0.03). The median ALC (×103/µL) at admission was 3.99 and 2.96 in children who had overall response and no response at 12 months, respectively (P = 0.04). Response rate was lesser in the treated group by approximately 10% compared to the non-treated group. CONCLUSION: The rate of chronicity and intracranial bleeding in our cohort is more than the reported rates in literature. Higher ALC was found to be associated with response.

Pediatric Thrombotic Events: Incidence, Clinical Risk Factors and Outcome.

OBJECTIVE: To study the incidence, clinical risk factors and outcome of thrombotic events in pediatric age group of 1 mo-12 y. METHODS: This prospective observational study was conducted in a tertiary care institute from September 2015 through October 2017. Forty nine children with thrombosis from 1 mo-12 y were enrolled. RESULTS: Out of 49 cases, 30 (61.2%) were due to venous thromboembolism (VTE) and 19 (38.8%) were of arterial thromboembolism (ATE). The cumulative average annual incidence for VTEs was found to be 38.2 (n = 30) and for ATEs it was found to be 24.2 (n = 19) per 10,000 hospital admissions over 2 y of study period. With total of 19 (38.7%), catheters were the leading cause of thrombosis followed by infection numbering to 10 cases (20.4%). Total 42.8% cases (n = 21) achieved complete resolution. Partial resolution was noted in 53.2% of cases (n = 26) and no resolution in 4% cases (n = 2). Total seven (14.3%) deaths were recorded during the study period. CONCLUSIONS: The present study showed that thrombosis is an emerging problem in tertiary care setting adding to both mortality and morbidity in children. Central venous catheters followed by infection were the leading cause of thrombosis in this study.

Antiphospholipid Syndrome Complicating Pneumococcal Meningitis.

BACKGROUND: Antiphospholipid syndrome is a multisystem auto-immune disorder characterized by arterial or venous thrombosis in children. CASE CHARACTERISTICS: 11-year-old child with pneumococcal meningitis also had cerebral sinus vein thrombosis and pulmonary artery segmental thrombosis. OBSERVATION: Pro-thrombotic evaluation showed positive lupus anticoagulant at baseline and after 12 weeks. Investigations for lupus were negative at admission and after one year of follow-up. MESSAGE: Antiphospholipid syndrome is a possibility even in thrombosis occurring in the setting of meningitis.

Atypical Presentation of Acute Lymphoblastic Leukemia in a 7 Year Old Child.

Seven year child, who had intussusception 4 month before, readmitted for fever and walking difficulty. On examination, child had mild pallor and kyphosis of the spine but no organomegaly, lymphadenopathy or bone pain . Further evaluation revealed hypercalcemia, diffuse osteoporosis with vertebral fracture. Peripheral smear showed no blasts or pancytopenia. Bone marrow aspiration turned out to be a pre B cell ALL. This child had many atypical presentations like intussusception, osteoporosis, vertebral collapse and hypercalcemia without any classical features of ALL.