Introduction: Supraventricular tachycardia (SVT) is one of the arrhythmias that can occur in newborns. Most SVT incidents in the neonatal period are spontaneously resolved around the first year of life, but since tachycardia can frequently occur before complete resolution, appropriate medication use is required. However, no clear guidelines or consensus on the treatment of neonatal SVT have been established yet. Methods: From January 2011 to December 2021, demographic data and antiarrhythmic medications used were retrospectively analyzed for 18 newborns diagnosed with SVT at a single center. Results: A total of four medications (propranolol, amiodarone, flecainide, and atenolol) were used as maintenance therapy to prevent tachycardia recurrence, and propranolol was the most used, followed by amiodarone. Thirty-nine percent of the patients were controlled with monotherapy, but the remainder required two or more medications. The median period from medication initiation after diagnosis to the last tachycardia event was 15.5 days, and the median total duration of medication use was 362 days. None of the patients experienced any side effects of antiarrhythmic medications. The total duration of medication use was statistically significant according to the mechanism of SVT, and the usage time of the increased automaticity group was shorter than that of the re-entry group. Conclusion: Since most neonatal SVT resolves within 1 year, it is significant to provide prophylactic medication to prevent tachycardia recurrence at least until 1 year of age, and depending on the patient, the appropriate combination of medications should be identified.
31 July 2021 in Journal of Yeungnam Medical Science
Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a self-limiting lymphadenitis. It is a benign disease mainly characterized by high fever, lymph node swelling, and leukopenia. Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease with clinical symptoms similar to those of KFD, but it requires a significantly more aggressive treatment. A 19-year-old Korean male patient was hospitalized for fever and cervical lymphadenopathy. Variable-sized lymph node enlargements with slightly necrotic lesions were detected on computed tomography. Biopsy specimen from a cervical lymph node showed necrotizing lymphadenitis with HLH. Bone marrow aspiration showed hemophagocytic histiocytosis. The clinical symptoms and the results of the laboratory test and bone marrow aspiration met the diagnostic criteria for HLH. The patient was diagnosed with macrophage activation syndrome-HLH, a secondary HLH associated with KFD. He was treated with dexamethasone (10 mg/m 2 /day) without immunosuppressive therapy or etoposide-based chemotherapy. The fever disappeared within a day, and other symptoms such as lymphadenopathy, ascites, and pleural effusion improved. Dexamethasone was reduced from day 2 of hospitalization and was tapered over 8 weeks. The patient was discharged on day 6 with continuation of dexamethasone. The patient had no recurrence at the 18-month follow-up.
Background: The Act on Life-Sustaining Treatment (LST) for patients at the end of life (the Korean LST Decision Act), implemented in the Republic of Korea in February 2018, has led to changes in the end-of-life decision-making (EOLDM) process in children. This study aimed to investigate changes in pediatric EOLDM process and LST practices since the Korean LST Decision Act. Methods: This retrospective cohort study included 107 patients who died at Kyungpook National University Children’s Hospital from January 2015 to December 2020. Patients were divided into two groups: pre-law (January 2015–January 2018, n = 55) and post-law (February 2018–December 2020, n = 52). We analyzed medical records for EOLDM process, patient characteristics, intensive care unit (ICU) admission, documentation types, and LST withholding or withdrawal decisions. Results: After the Korean LST Decision Act, the median total hospitalization duration decreased significantly (14 days [IQR, 3–80] vs. 6 days [IQR, 2–18], p = 0.020), as did the median ICU length of stay (3 days [IQR 1–33] vs. 2.5 days [IQR 1–10.3], p = 0.002). The time from admission to end-of-life decision documentation was significantly shorter in group 2 (6 days [IQR 1–31] vs. 4 days [IQR 1–9], p = 0.027). The use of physician orders for life-sustaining treatment (POLST) documents increased (0% to 33.3%), while do-not-resuscitate (DNR) orders decreased (85.3% to 16.7%). Notably, LST withdrawal decisions increased from 0% to 27.8% (p = 0.001) in the post-legislation period. Conclusions: The Korean LST Decisions Act has led to significant changes in the EOLDM process for terminally ill children, including earlier decision-making, increased use of POLST documents, more frequent LST withdrawal decisions, and shorter hospital and ICU stays. These findings suggest a shift towards more structured and timely end-of-life care discussions in pediatric settings.
The first-line treatment for severe aplastic anemia (SAA) patients is hematopoietic stem cell transplantation (HSCT), with full-matched related donors considered the most suitable. We report a case of SAA in which the patient successfully underwent HSCT from a donor with β-thalassemia minor. The patient in this case underwent HSCT from a human leukocyte antigen (HLA)-matched younger brother with β-thalassemia minor. A 7-year-old girl was referred to our facility following a 6-month history of easy bruising and pallor. Laboratory examinations showed pancytopenia and hypocellular bone marrow with cellularity of <5%. She was diagnosed with acquired SAA, and HLA typing of her family members was performed. Her younger brother was an HLA-matched sibling but had β-thalassemia minor. Since his hemoglobin levels were maintained at 10–11 d/dL, he was considered a suitable HSCT donor. The conditioning regimen included fludarabine, cyclophosphamide, and anti-thymocyte globulin. The CD34+ and CD3+ cell counts were 6.6 × 106/kg and 0.48 × 108/kg, respectively. White blood cell engraftment was evident on day +11. Regimen-associated toxicities, such as anorexia and enteritis, were mild; no infections occurred, and no symptoms of acute graft-versus-host disease (GVHD) were observed. The 30-day follow-up bone marrow examination revealed normocellular marrow with 80%–90% cellularity. Acute or chronic GVHD has not been reported, and good performance status has been observed throughout the 5 years after HSCT. β-thalassemia minor patients can be considered as bone marrow donors for SAA patients.
30 April 2021 in Journal of Yeungnam Medical Science
We report the case of a 16-month-old patient with chronic immune thrombocytopenia (ITP) patient who experienced delayed treatment-free response (TFR) after romiplostim treatment. He received intravenous immunoglobulin every month to maintain a platelet count above 20,000/μL for 2 years. Thereafter, he received rituximab and cyclosporine as second-line therapy, with no response, followed by romiplostim. After 4 weeks of treatment, the platelet count was maintained above 50,000/μL. Following 7 months of treatment, he discontinued romiplostim, and the platelet count decreased. His platelet counts remained above 50,000/μL, without any bleeding symptoms, 2 years after romiplostim discontinuation. This is the first report of TFR after romiplostim treatment in pediatric chronic ITP.