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[This corrects the article DOI: 10.7759/cureus.58941.].
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Hemophilia A (HA) is a genetic disorder of hemostasis associated with a deficiency or reduced activity of clotting factor VIII (FVIII). This disorder remains unacceptably underdiagnosed in India. Early diagnosis and appropriate management of HA can substantially prevent morbidity and mortality. Currently, HA is managed with regular replacement therapy using standard or extended half-life FVIII concentrates or non-factor drug products. The challenges associated with FVIII concentrates include plateauing of drug effect, issues with its administration and adherence to treatment, breakthrough bleeds, and the development of inhibiting antibodies against administered clotting factors. Emicizumab is a bispecific antibody, launched in India in April 2019, for managing patients with HA. To investigate the role of emicizumab in Indian patients with HA, opinions were sought from 13 eminent hematologists and experts from India on the effectiveness of emicizumab in preventing all bleeds, spontaneous bleeds, perioperative bleeds, and intracranial hemorrhage; resolving target joints; and reducing the rate of hospitalizations and fatality associated with HA in children and adults, with or without inhibitors. The benefits of emicizumab over traditional FVIII concentrates include the subcutaneous route of delivery, less frequent dosing, and a lack of inhibitor development, in addition to providing sustained hemostasis without in-depth monitoring. It is a safe and effective management option for all HA patients, especially for patients with certain archetypes, such as those with inhibitors, those with high annualized bleed rates, those living far away from hemophilia care centers, pediatric patients and infants with intravenous access challenges, and those with a history of life-threatening bleeding events.
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Diabetic striatopathy is a neurological condition in patients with diabetes characterized by hemichorea-hemiballismus due to vascular and metabolic derangements in basal ganglia. This is a known entity in type 2 diabetic adult patients; however, seen rarely in pediatric patients with type 1 diabetes. Diabetic striatopathy develops in patients with poor glycemic control in the absence of ketosis. The patient tolerates hyperglycemia for a long time, which results in metabolic injury.
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OBJECTIVES: The objective of this study was to estimate the prevalence of meningitis in cases with late onset septicemia (LOS). MATERIALS AND METHODS: A prospective study was carried out for a period of 1 year in a tertiary care hospital in North West India to estimate the prevalence of meningitis in cases of LOS. In all the admitted neonates with features of sepsis with a positive C-reactive protein, a lumbar puncture (LP) was carried out and results interpreted on the basis of cerebrospinal fluid (CSF) cytology and biochemistry. Simultaneous blood and CSF cultures were also taken. All other baseline investigations were performed and in those diagnosed as meningitis an ultrasound head was carried out prior to discharge. No urine cultures were obtained. RESULTS: The study showed the prevalence of meningitis as 22.5% in neonates with LOS with statistically significant implications of meningitis versus gestation, sex, acquired the place of infection, and outcome in terms of sequelae/mortality. CONCLUSIONS: Meningitis is commonly associated with late onset sepsis hence LP should be the standard of care in such neonates as the treatment protocol and the outcome is directly proportional to the diagnosis at initial presentation.