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Hemophagocytic Lymphohistiocytosis can Mimic the Superior Vena Cava Syndrome
Park, Meerim,Choi, Jae-Wook,Park, Hyeon Jin,Kim, Hee Jin,Hi Son, Meong,Yoon, Jong-Hyung,Kim, Su-Jin,Park, Won Seo,Park, Byung-Kiu Lippincott Williams Wilkins, Inc. 2012 Journal of pediatric hematology/oncology Vol.34 No.4
We report a case of a 3-year-old boy with hemophagocytic lymphohistiocytosis (HLH), with enlarged cervical lymph nodes causing internal jugular vein compression, who initially presented a condition similar to the superior vena cava syndrome. Laboratory data along with neck node and bone marrow biopsies confirmed the HLH. Genetic analysis revealed the patient to be compound heterozygous for 2 variations of the perforin gene, c.1620 A>G and c.562C>G. This case featuring a rare initial manifestation of HLH that has not been previously reported, points to the necessity of considering this disease when symptoms similar to superior vena cava syndrome are encountered.
Lee, Jun Ah,Lee, Jae Min,Park, Hyeon Jin,Park, Meerim,Park, Byung Kiu,Ju, Hee Young,Kim, Ji Yoon,Park, Sang Kyu,Lee, Young Ho,Shim, Ye Jee,Kim, Heung Sik,Park, Kyung Duk,Lim, Yeon-Jung,Chueh, Hee Won The Korean Pediatric Society 2020 Clinical and Experimental Pediatrics (CEP) Vol.63 No.4
Background: For children and adolescents with cancer, going back to school is a key milestone in returning to "normal life." Purpose: To identify the support vital for a successful transition, we evaluated the parents' needs and the challenges they face when their children return to school. Methods: This multi-institutional study was conducted by the Korean Society of Pediatric Hematology and Oncology. The written survey comprised 24 questions and was completed by 210 parents without an interviewer. Results: Most parents (165 of 206) reported that their children experienced difficulties with physical status (n=60), peer relationships (n=30), academic performance (n=27), emotional/behavioral issues (n=11), and relationships with teachers (n=4) on reentering school. Parents wanted to be kept informed about and remain involved in their children's school lives and reported good parent-teacher communication (88 of 209, 42.1%). Parents reported that 83.1% and 44.9% of teachers and peers, respectively, displayed an adequate understanding of their children's condition. Most parents (197 of 208) answered that a special program is necessary to facilitate return to school after cancer therapy that offers emotional support (n=85), facilitates social adaptation (n=61), and provides tutoring to accelerate catch up (n=56), and continued health care by hospital outreach and school personnel (n=50). Conclusion: In addition to scholastic aptitude-oriented programs, emotional and psychosocial support is necessary for a successful return to school. Pediatric oncologists should actively improve oncology practices to better integrate individualized school plans and educate peers and teachers to improve health literacy to aid them in understanding the needs of children with cancer.
Myelodysplastic syndrome with genetic predisposition
Meerim Park 대한혈액학회 2021 Blood Research Vol.56 No.-
Myelodysplastic syndrome (MDS) refers to a heterogeneous group of clonal blood disorders characterized by ineffective hematopoiesis, cytopenia, dysplasia, and an increased risk of acute myeloid leukemia (AML). A growing number of inherited genetic loci that contribute to MDS/AML development are rapidly being identified. As genetic sequencing has become increasingly integrated into clinical practice, clearly defined syndromes have emerged, known as the MDS/AML predisposition syndrome. With more patients and families being identified with predisposing conditions, knowledge of the approach of evaluating and managing MDS with genetic predisposition is increasingly essential. This article reviews MDS with genetic predisposition and the practical aspects of management in patients with predisposition syndrome.
Status and Needs for the Return to School of Childhood and Adolescent Cancer Survivors
Eun Sil Park,Eun Young Ju,Jae Young Lim,Hyun Sik Kang,Jin Kyung Suh,Meerim Park,Jun Ah Lee,Bo-Yoon Jeong,Young Ae Kim,Hyeon Jin Park 대한소아혈액종양학회 2023 Clinical Pediatric Hematology-Oncology Vol.30 No.2
Background: As the treatment effects for childhood and adolescent cancer continue to improve, the survivor group is gradually growing and their age is increasing. This study aimed to determine the perceptions toward childhood and adolescent cancer survivors and assess the current situation regarding their return to school, to under-take policy development to help them grow into healthy members of society.Methods: From December 2019, for about 4 months, a structured questionnaire was used to conduct one-on-one interviews with childhood and adolescent cancer survi-vors and their parents, as well as with parents of healthy children and adolescents.Results: The participants included 79 survivors of childhood and adolescent cancer, 186 parents of cancer survivors, and 661 general parents; their mean age was 21.8, 13.9, and 12.5 years, respectively. After completing their cancer treatment, 77.2% of the cancer survivors returned to school, with the majority returning to regular schools in the same grade as their peers. Reasons for not returning to school (20.3%) included concerns about health management (43.8%), concerns about psychological and emo-tional adjustment (12.5%), and poor school attendance (12.5%). Among the parents of cancer survivors, 48.9% stated that they were “satisfied” with their children’s school life; a better health status in children was associated with a higher level of satisfaction (P=0.0071). In addition, they stated that national-level support was need-ed in the following areas for a successful return to school: a continuous health man-agement system (36.1%) and understanding homeroom teachers who enable flexible participation in classes and school events (29.5%).Conclusion: For survivors of childhood and adolescent cancer to successfully return to school and society, nationwide awareness-raising activities should be expanded, in addition to services that are tailored to the survivors’ needs and characteristics such as management of physical and mental health and educating homeroom teach-ers and peers.
Intraventricular Vancomycin Therapy for Intractable Bacillus cereus Ventriculitis
Hahn, Jong Woo,Ju, Hee young,Park, Meerim,Yi, Eun Sang,Park, Byung-Kiu,Shin, Sang-Hoon,Lee, Sang-Hyun,Park, Hyeon Jin,Kang, Ji-Man The Korean Society of Pediatric Infectious Disease 2019 Pediatric Infection and Vaccine Vol.26 No.2
Bacillus cereus causes serious central nervous system infections, especially in immunocompromised patients. Successful treatment requires adequate antimicrobial concentrations in the cerebrospinal fluid; however, in some cases, achieving this with systemic treatment alone is difficult. We treated intractable B. cereus ventriculitis with intraventricular vancomycin, with no major adverse events.
Jun Ah Lee,Bin Cho,Sun Ah Shin,Seog Yun Park,Meerim Park,Hyeon Jin Park 대한소아혈액종양학회 2021 Clinical Pediatric Hematology-Oncology Vol.28 No.1
Synovial sarcoma occurring in the pleura and lung is extremely rare. We report a case of pleuropulmonary synovial sarcoma as a second malignant neoplasm. The patient had been diagnosed with acute myelomonocytic leukemia at 5 years of age, and received matched sibling donor allogeneic bone marrow transplantation, with total body i rradiation and cyclophosphamide conditioning. At 22 years of age, he complained of worsening chest discomfort and exertional dyspnea. Chest CT revealed a huge mass in the right middle lobe, pleura, and diaphragm. The patient was initially diagnosed as sarcomatoid malignant mesothelioma, without any environmental or occupational a sbestos exposure. Five months later, the patient p resented with soft tissue metastasis and underwent needle biopsy. Pathological examination including SYT-SSX RT-PCR revealed synovial sarcoma, which led to a review of the original tumor findings and confirmed the diagnosis of pleuropulmonary synovial sarcoma. To the best of our knowledge, our patient is the first case of pleuropulmonary synovial sarcoma developed after allogeneic hematopoietic stem cell transplantation.
Koh, Kyung Nam,Park, Meerim,Kim, Bo Eun,Im, Ho Joon,Seo, Jong Jin Korean Society of Hematology; Korean Society of Bl 2010 Blood Research Vol.45 No.3
<P><B>Background</B></P><P>Central nervous system (CNS) complications after allogeneic hematopoietic stem cell transplantation (HSCT) have not been well characterized in the pediatric population.</P><P><B>Methods</B></P><P>We retrospectively analyzed data of 202 consecutive children who underwent allogeneic HSCT (60 from matched related donors, 9 from mismatched related donors, and 133 from unrelated donors) at Asan Medical Center between 1998 and 2009.</P><P><B>Results</B></P><P>Twenty-seven children (13.5%) developed CNS complications within 6 months after HSCT. Calcineurin inhibitor (CNI)-associated neurotoxicity was the most common CNS complication (n=16), followed by CNS infection (n=2), cerebrovascular events (n=2), thrombotic microangiopathy-associated events (n=2), metabolic encephalopathy (n=2), irradiation/chemotherapy injury (n=1), and encephalopathy/myelopathy of unknown causes (n=2). Univariate analysis showed that a transplant from an alternative donor and the occurrence of acute graft-versus-host disease (GVHD) (>grade 2) were associated with a significantly increased risk of CNS complications. In the multivariate analysis, acute GVHD >grade 2 was identified as an independent risk factor for early CNS complications. The 5-year overall survival rate was significantly lower in patients with CNS complications (52.1% vs. 64.9%, <I>P</I>=0.014), whereas CNI-associated neurotoxicity did not affect the survival outcome.</P><P><B>Conclusion</B></P><P>CNS complications are frequent among children undergoing HSCT, contributing to early death after transplant. More attention should be paid to the development of CNS complications for recipients of alternative donor transplants and patients with severe acute GVHD who are at increased risk for CNS complications.</P>
Jae Yeob Jung,Arum Oh,Je Keong Kim,Meerim Park 대한소아청소년과학회 2016 Clinical and Experimental Pediatrics (CEP) Vol.59 No.8
Purpose: This study aimed to evaluate the clinical course of childhood immune thrombocytopenia (ITP) and to assess the risk factors for developing chronic ITP Methods: The records of 64 children diagnosed with ITP from November 2005 and December 2014 at single center were retrospectively analyzed. Results: The median age at diagnosis and the median platelet count were 1 year (range, 1 month to 15 years) and 9×109/L (range, 0–84×109/L), respectively. No patient experienced severe bleeding. Nineteen children (29.7%) spontaneously recovered their platelet count to ≥100×109/L at a median of 10 days. In total 45 patients (70.3%) received intravenous immunoglobulin (IVIG) as first-line therapy, and showed platelet recovery at 1 week. The final diagnosis of 55 (85.9%) and 9 patients (14.1%) was acute and chronic ITP, respectively. Older age, absence of prior infection and insidious onset of symptoms were significantly associated with the development of chronic ITP. Among the patients who received IVIG, those with platelet count <45×109/L at 1 month after IVIG showed a significantly higher incidence of chronic ITP compared to those with platelet count ≥45×109/L (88.8% vs. 44.4%, P<0.01). Conclusion: In most patients, ITP runs a benign course and approximately 86% of them recover within 1 year of their initial diagnosis. The potential impact of the risk factors of chronic ITP on clinical practice needs to be explored and further studies are warranted to determine whether IVIG influences the course of ITP.
Yun, Yeong Ju,Gyon, Yun Hee,Yang, Sohyoung,Lee, Youn Kyung,Park, Joohyun,Park, Meerim The Korean Pediatric Society 2015 Clinical and Experimental Pediatrics (CEP) Vol.58 No.12
Purpose: To determine the efficacy and safety of oral propranolol as a first-line treatment for superficially located infantile hemangioma (IH) and propose an assessment tool to measure treatment response. Methods: Patients with superficial IH under 1 year of age were prospectively recruited between May 2012 and December 2013 at the Department of Pediatrics of Chungbuk National University Hospital. Propranolol was administered to 12 infants (median age, 3.8 months) while monitoring cardiovascular and adverse metabolic effects. If a patient showed no adverse events, the dosage was gradually increased up to 3 mg/kg/day and maintained for 1 year. We used our own scoring system to assess treatment response using parameters like change in color, and longest diameter, and thickness of the IH. Results: Eleven out of 12 patients completed the protocol with consistent improvement of hemangiomas during therapy. Patients on propranolol showed a more than 50% involution in the first 3 months, with additional steady involution until 1 year. Patients with the highest scores at 1 month maintained their score and showed better responses until treatment termination. The patient with the lowest score at 1 month did not show any further regression and stopped propranolol treatment 4 months after initiation. In two children with recurrences after successful therapeutic regression, propranolol was effective after being reintroduced. Propranolol treatment was not interrupted in any patient due to adverse events. Conclusion: Oral propranolol at 3 mg/kg/day showed a consistent, rapid, and therapeutic effect on superficial IHs without significant adverse events.