Efficacy and Tolerability of Twice-Daily Dosing Schedule of Deferasirox in Transfusion-Dependent Paediatric Beta-Thalassaemia Patients: A Randomized Controlled Study.

Background: Deferasirox has proved good efficacy and acceptable safety for the management of thalassaemia patients. However, some patients are unresponsive or intolerant to once-daily administration of deferasirox even at a high dose. The current study evaluated the effectiveness and tolerability of twice-daily dosing of deferasirox among transfusion-dependent paediatric beta-thalassaemia patients. Methods: This prospective randomized single-blinded parallel study included all transfusion-dependent paediatric beta-thalassaemia patients prescribed with deferasirox, who visit the study site for their regular blood transfusions and follow-up. The enrolled patients were randomized into intervention and control groups by using a simple block randomization method. In the intervention group, the once-daily dosing of deferasirox was changed to twice-daily dosing with the same total daily dose. Whereas, in the control group, the patients continued with the once-daily deferasirox dosing. The serum ferritin levels of both groups were determined on the enrolment day and after 6 months of follow-up. Results: Forty-one patients were included for analysis. A statistically significant mean decrease in serum ferritin levels was detected in the intervention group, while the serum ferritin levels of the control group significantly increased from baseline. The twice-daily dosing of deferasirox was better tolerated by the thalassaemia patients when compared to once-daily dosing. Conclusion: This study concludes that twice-daily dosing of deferasirox with the same total daily dose significantly enhances the iron chelation efficacy and tolerability among transfusion-dependent paediatric beta-thalassaemia patients when compared to once-daily regimen.

A Rare Pediatric Case of Cefixime Induced Toxic Epidermal Necrolysis.

Cefixime is a third-generation cephalosporin that has been used for the treatment of a wide range of infections in children and adults. The incidence of cefixime induced toxic epidermal necrolysis (TEN) is less than 2% in adults, but it is infrequent among pediatric patients. We report a rare case of cefixime induced TEN in a 7-year-old boy. In this case, the child presented with symptoms of TEN after 2 days of administration of cefixime. This case highlights the need to select structurally different antibiotics in case of antibiotic-induced severe cutaneous adverse reaction (SCAR) to avoid recurrence of SCAR. Furthermore, concluded that irrational use of antibiotics could be disastrous as it can result in TEN as the incidence of antibiotics induced TEN ranges from 29% to 42%.

A Rare Case of Dapsone Hypersensitivity Syndrome and Leukemoid Reaction With Coexisting Hepatitis E in a Pediatric Patient.

Dapsone is extensively used for a variety of infectious, immunological, and hypersensitivity disorders. Dapsone can cause several adverse effects, the most serious being dapsone hypersensitivity syndrome (DHS), which is potentially fatal. DHS is characterized by triad of eruptions, fever, and organ involvement (including liver, kidney, hematological system, etc.). DHS can develop several weeks to as late as 6 months after treatment initiation with dapsone. Here, we report a case of DHS and leukemoid reaction with coexisting hepatitis E in a 10-year-old girl. Three weeks prior to the current admission, she was treated with dapsone (1 mg/kg/day in two divided doses) for 8 days by a local doctor for lichen nitidus. She was managed successfully for DHS with intravenous (IV) steroids followed by the oral steroid. This case is being reported to highlight the importance to ensure timely diagnosis of DHS and its appropriate management. Patients started on dapsone for various clinical conditions need to be observed carefully for the development of the DHS. If this occurs, DHS can be mistaken for the progression of the primary disease. If dapsone is not withdrawn, it could have deleterious and potentially fatal effects due to major organ dysfunction.

Drug Rash With Eosinophilia and Systemic Symptoms (DRESS) Syndrome Probably Related to Cefpodoxime: A Case Report.

Cefpodoxime is a common antibiotic with a favorable side effect profile. Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) syndrome has been described with several cephalosporins but not cefpodoxime. We report the probable first case of cefpodoxime-induced DRESS syndrome in a 52-year-old female patient. In our case, the patient presented with symptoms of DRESS syndrome 16 days after initiation of cefpodoxime. This case highlights the necessity of consideration of an iatrogenic reason for presenting signs and symptoms at all times. Reinforcing the importance of taking a thorough drug history and considering drug reactions even if onset of symptoms are delayed.

Effectiveness and tolerability of twice daily dosing of deferasirox in unresponsive and intolerant transfusion-dependent beta-thalassemia patients: A narrative review.

Chronic iron overload in beta-thalassemia patients after continuous blood transfusions has caused notable morbidity and mortality in these patients. The once-a-day oral iron chelator, deferasirox has established efficacy and bearable safety in adults and pediatric thalassemia patients. It is now extensively used for the management of transfusional hemosiderosis. However, a number of studies have revealed a few patients continued to be none respondent or intolerant toward the once-a-day regimen of deferasirox even after the administration of maximum dose recommended by the World Health Organization. In the literature, there were three studies showing the boon of twice in a day dosing of deferasirox among transfusional-dependent beta thalassemia patients. Therefore, a nonsystematic review was conducted on above three studies to ascertain the enhanced effectiveness and tolerability of twice per day regimen of deferasirox with the same total dose as that of once daily regimen of deferasirox in unresponsive or intolerant transfusion-dependent beta-thalassemia (TDT) patients. All the above studies concluded that the twice per day regimen of deferasirox was more efficacious and tolerable among TDT patients when compared to the once-a-day regimen with the same total daily dose. Although there was a significant good results from these studies, there is a need to conduct either muticenter study or randomized control study in a larger number of patients for the better confirmation of the results as all the above studies were conducted in the small number of TDT patients.

Probable blueberry-induced haemolysis in a G6PD deficient child: A case report.

BACKGROUND: Blueberry is a North American native fruit increasingly popular as a source of health-promoting bioactive compounds. However, there is evidence in the literature stating that blueberries should be avoided in individuals with glucose-6-phosphate dehydrogenase (G6PD) deficiency. CASE PRESENTATION: We report the first case of a G6PD deficient child who developed haemolysis approximately after half a day following the intake of fresh blueberry. He presented with the complaints of the passage of orange coloured urine and hurried breathing. The child had haemoglobin of 7.9 g/dl on admission day and the laboratory results were indicative of haemolytic process. OUTCOMES AND IMPLICATIONS: During the stay in the hospital, the child was managed conventionally, and he improved both clinically and symptomatically. The child was discharged after four days of treatment and diet counselling was provided to his parents. RECOMMENDATIONS: Our report should encourage further research on this fruit regarding the mechanism behind the development of haemolytic anaemia. Also, it is important in the case of G6PD deficient patients to take lifelong precautions to avoid the foods that can trigger haemolysis.

A pediatric case report of fixed drug eruption related to carmoisine colorant present in paracetamol syrup.

Various adverse drug reactions, including hypersensitivity skin reaction after the exposure to carmoisine colorant, have already been reported in the literature. This case report gives the details of a 5-year-old male child with a recurrent fixed drug eruption (FDE) over the neck and trunk after the use of paracetamol syrup containing carmoisine as a colorant. Patch test to the carmoisine colorant in the paracetamol syrup was positive. This case report adds an increased awareness of the probability of allergic skin reaction to carmoisine in a previously sensitized person. The report also emphasizes the necessity to consider artificial dyes in pharmaceutical products as a potential cause for FDE.

Estimation of serum neopterin level as an early marker for detecting severe dengue infection.

BACKGROUND: This study aimed to determine the role of neopterin in the early assessment of dengue severity in children of age one month to 18 years admitted for fever and confirmed by dengue serology. METHOD: Two sets of samples were collected from 77 confirmed dengue patients aged one month to 18 years for serum neopterin determination. The first sample was collected at 2-3 days of fever onset, and the second sample was collected at 24-36 h after the first sample was collected and followed up cases and correlated serum neopterin levels. RESULTS: Among the 77 patients enrolled, 19 were diagnosed with severe dengue, 15 patients as having dengue with warning signs, and 43 patients as having dengue fever. CONCLUSION: In this study, we found that the serum neopterin levels were significantly higher in the severe dengue category (mean: 58.75(±4.91) nmol/l) than in the non-severe dengue category (mean: 48.11(±8.80) nmol/l). Furthermore, a positive correlation was observed between neopterin concentration and duration of fever hence, higher serum neopterin levels may indicate dengue severity. The determination of serum neopterin concentration may be used for the early assessment of severe dengue.