Laboratory Markers in Incomplete Kawasaki Disease according to Coronary Artery Outcome

BACKGROUND AND OBJECTIVES: We defined laboratory marker profiles typical of incomplete Kawasaki disease (iKD) during illness, especially with respect to the presence of a coronary artery abnormality such as coronary artery dilation or aneurysm. METHODS: This retrospective study examined the clinical and laboratory markers of patients with iKD over time, along with those of patients with complete KD (cKD) and febrile controls. RESULTS: Of 795 patients, 178 had iKD, 504 had cKD and 113 were febrile controls. During the transition from the acute to subacute phase, the age-adjusted hemoglobin levels and platelet counts were significantly lower and higher, respectively, in the subacute phase than in the acute phase in both iKD and cKD patients, which differed from those of febrile controls. Lower levels of acute and subacute age-adjusted hemoglobin levels in iKD patients (odds ratio [OR], 0.538 and 0.583; p=0.006 and 0.018, respectively) and higher subacute platelet counts in cKD patients (OR, 1.004; p=0.014) were correlated with the risk of coronary dilation. A higher acute neutrophil-to-lymphocyte ratio was associated with aneurysm only in cKD patients (OR, 1.059; p=0.044). CONCLUSIONS: The iKD patients share KD-specific laboratory marker profiles in terms of complete blood cell counts and acute phase reactant levels with cKD patients. However, the factors predicting coronary dilation differ according to the phenotype; lower acute and subacute age-adjusted hemoglobin levels predict coronary dilation only in iKD patients.

Clinical and laboratory profiles of hospitalized children with acute respiratory virus infection / 소아과

PURPOSE: Despite the availability of molecular methods, identification of the causative virus in children with acute respiratory infections (ARIs) has proven difficult as the same viruses are often detected in asymptomatic children. METHODS: Multiplex reverse transcription polymerase chain reaction assays were performed to detect 15 common respiratory viruses in children under 15 years of age who were hospitalized with ARI between January 2013 and December 2015. Viral epidemiology and clinical profiles of single virus infections were evaluated. RESULTS: Of 3,505 patients, viruses were identified in 2,424 (69.1%), with the assay revealing a single virus in 1,747 cases (49.8%). While major pathogens in single virus-positive cases differed according to age, human rhinovirus (hRV) was common in patients of all ages. Respiratory syncytial virus (RSV), influenza virus (IF), and human metapneumovirus (hMPV) were found to be seasonal pathogens, appearing from fall through winter and spring, whereas hRV and adenovirus (AdV) were detected in every season. Patients with ARIs caused by RSV and hRV were frequently afebrile and more commonly had wheezing compared with patients with other viral ARIs. Neutrophil-dominant inflammation was observed in ARIs caused by IF, AdV, and hRV, whereas lymphocyte-dominant inflammation was observed with RSV A, parainfluenza virus, and hMPV. Monocytosis was common with RSV and AdV, whereas eosinophilia was observed with hRV. CONCLUSION: In combination with viral identification, recognition of virus-specific clinical and laboratory patterns will expand our understanding of the epidemiology of viral ARIs and help us to establish more efficient therapeutic and preventive strategies.

Laboratory Markers in Incomplete Kawasaki Disease according to Coronary Artery Outcome

BACKGROUND AND OBJECTIVES@#We defined laboratory marker profiles typical of incomplete Kawasaki disease (iKD) during illness, especially with respect to the presence of a coronary artery abnormality such as coronary artery dilation or aneurysm.@*METHODS@#This retrospective study examined the clinical and laboratory markers of patients with iKD over time, along with those of patients with complete KD (cKD) and febrile controls.@*RESULTS@#Of 795 patients, 178 had iKD, 504 had cKD and 113 were febrile controls. During the transition from the acute to subacute phase, the age-adjusted hemoglobin levels and platelet counts were significantly lower and higher, respectively, in the subacute phase than in the acute phase in both iKD and cKD patients, which differed from those of febrile controls. Lower levels of acute and subacute age-adjusted hemoglobin levels in iKD patients (odds ratio [OR], 0.538 and 0.583; p=0.006 and 0.018, respectively) and higher subacute platelet counts in cKD patients (OR, 1.004; p=0.014) were correlated with the risk of coronary dilation. A higher acute neutrophil-to-lymphocyte ratio was associated with aneurysm only in cKD patients (OR, 1.059; p=0.044).@*CONCLUSIONS@#The iKD patients share KD-specific laboratory marker profiles in terms of complete blood cell counts and acute phase reactant levels with cKD patients. However, the factors predicting coronary dilation differ according to the phenotype; lower acute and subacute age-adjusted hemoglobin levels predict coronary dilation only in iKD patients.

Norovirus Associated Cerebellitis in a Previous Healthy 2-year-old Girl

Norovirus causes acute gastroenteritis, occasional afebrile seizures, and rarely encephalitis. We describe a child with cerebellitis due to norovirus that has not been reported previously. A previously healthy 2-year-old girl with a recent history of acute gastroenteritis, who presented with acute cerebellar ataxia. She exhibited marked truncal ataxia, was barely able to walk, and was prone to sitting or lying down. Multiplex PCR using stool samples revealed norovirus (genogroup II) and magnetic resonance imaging showed increased T2 signal in the hemi-cerebellum. Norovirus may be a potential pathogenic cause of acute cerebellitis in children.

Familial Mediterranean fever presenting as fever of unknown origin in Korea / 소아과

Familial Mediterranean fever (FMF) is the most common Mendelian autoinflammatory disease, characterized by uncontrolled activation of the innate immune system that manifests as recurrent brief fever and polyserositis (e.g., peritonitis, pleuritic, and arthritis). FMF is caused by autosomal recessive mutations of the Mediterranean fever gene, MEFV which encodes the pyrin protein. Although FMF predominantly affects people from Mediterranean and Middle Eastern ethnic origins, 3 cases of FMF have been reported in Korea since 2012. We report another case of FMF in Korea in which the patient presented with a month-long fever without serositis. After treatment with colchicine was initiated, the patient’s symptoms quickly subsided. The response to colchicine was helpful for diagnosis. We compare the FMF genotypes in Korea with in other countries. Studying FMF cases in Korea will help establish the best MEFV exons to use for screening and diagnosis of Korean FMF.

Characteristics and Outcomes of Second Malignant Neoplasms after Childhood Cancer Treatment: Multi-Center Retrospective Survey

This retrospective study investigated the clinical characteristics and outcomes of second malignant neoplasms (SMNs) in survivors of childhood cancer from multiple institutions in Korea. A total of 102 patients from 11 institutions who developed SMN after childhood cancer treatment between 1998 and 2011 were retrospectively enrolled. The most common primary malignant neoplasms (PMNs) were central nervous system (CNS) tumors (n = 17), followed by acute lymphoblastic leukemia (n = 16), non-Hodgkin lymphoma (n = 13), and osteosarcoma (n = 12). The most common SMNs were therapy-related myeloid neoplasms (t-MNs; acute myeloid leukemia [AML], 29 cases; myelodysplastic syndrome [MDS], 12 cases), followed by thyroid carcinomas (n = 15) and CNS tumors (n = 10). The median latency period was 4.9 years (range, 0.5-18.5 years). Among 45 patients with solid tumors defined as an SMN, 15 (33%) developed the lesion in a field previously subjected to radiation. The 5-year overall survival (OS) rate of patients with an SMN was 45% with a median follow-up time of 8.6 years. Patients with AML, MDS, and CNS tumors exhibited the poorest outcomes with 5-year OS rates of 18%, 33%, and 32%, respectively, whereas those with second osteosarcoma showed comparable outcomes (64%) to patients with primary counterpart and those with second thyroid carcinoma had a 100% OS rate. Further therapeutic efforts are recommended to improve the survival outcomes in patients with SMNs, especially in cases with t-MNs and CNS tumors.

Characteristics and Outcomes of Second Malignant Neoplasms after Childhood Cancer Treatment: Multi-Center Retrospective Survey

This retrospective study investigated the clinical characteristics and outcomes of second malignant neoplasms (SMNs) in survivors of childhood cancer from multiple institutions in Korea. A total of 102 patients from 11 institutions who developed SMN after childhood cancer treatment between 1998 and 2011 were retrospectively enrolled. The most common primary malignant neoplasms (PMNs) were central nervous system (CNS) tumors (n = 17), followed by acute lymphoblastic leukemia (n = 16), non-Hodgkin lymphoma (n = 13), and osteosarcoma (n = 12). The most common SMNs were therapy-related myeloid neoplasms (t-MNs; acute myeloid leukemia [AML], 29 cases; myelodysplastic syndrome [MDS], 12 cases), followed by thyroid carcinomas (n = 15) and CNS tumors (n = 10). The median latency period was 4.9 years (range, 0.5-18.5 years). Among 45 patients with solid tumors defined as an SMN, 15 (33%) developed the lesion in a field previously subjected to radiation. The 5-year overall survival (OS) rate of patients with an SMN was 45% with a median follow-up time of 8.6 years. Patients with AML, MDS, and CNS tumors exhibited the poorest outcomes with 5-year OS rates of 18%, 33%, and 32%, respectively, whereas those with second osteosarcoma showed comparable outcomes (64%) to patients with primary counterpart and those with second thyroid carcinoma had a 100% OS rate. Further therapeutic efforts are recommended to improve the survival outcomes in patients with SMNs, especially in cases with t-MNs and CNS tumors.

The Association between Influenza Treatment and Hospitalization-Associated Outcomes among Korean Children with Laboratory-Confirmed Influenza

There are limited data evaluating the relationship between influenza treatment and hospitalization duration. Our purpose assessed the association between different treatments and hospital stay among Korean pediatric influenza patients. Total 770 children < or = 15 yr-of-age hospitalized with community-acquired laboratory-confirmed influenza at three large urban tertiary care hospitals were identified through a retrospective medical chart review. Demographic, clinical, and cost data were extracted and a multivariable linear regression model was used to assess the associations between influenza treatment types and hospital stay. Overall, there were 81% of the patients hospitalized with laboratory-confirmed influenza who received antibiotic monotherapy whereas only 4% of the patients received oseltamivir monotherapy. The mean treatment-related charges for hospitalizations treated with antibiotics, alone or with oseltamivir, were significantly higher than those treated with oseltamivir-only (P < 0.001). Influenza patients treated with antibiotics-only and antibiotics/oseltamivir combination therapy showed 44.9% and 28.2%, respectively, longer duration of hospitalization compared to those treated with oseltamivir-only. Patients treated with antibiotics, alone or combined with oseltamivir, were associated with longer hospitalization and significantly higher medical charges, compared to patients treated with oseltamivir alone. In Korea, there is a need for more judicious use of antibiotics, appropriate use of influenza rapid testing.

An Adolescent Case of Castleman Disease of the Plasma Cell Type Treated with Radiotherapy / 임상소아혈액종양

Castleman disease (CD) is a disorder with distinct lymphoid hyperplasia of unknown origin, and it is rare in children. There are two major forms of CD: unicentric (localized) and multicentric. Pathologically, it can be categorized into three subtypes: hyaline- vascular, plasma cell, and mixed types. We experienced a 15-year-old boy with histopathologically proven mediastinal CD of the plasma cell type. He was referred to our hospital because of mediastinal widening in a chest x-ray found by chance. Surgery was performed to resect two right paratracheal masses and subtotal excision was done. As complete surgical resection was not possible, the patient subsequently received three-dimensional conformal radiation therapy (total 5,400 cGy in 27 fractions). The size of the mass decreased, and his clinical symptoms regressed without any side effects from radiotherapy. Here we report a case of unicentric plasma cell type CD, treated with radiotherapy due to incomplete surgical resection.

A Case of Osteosarcoma after Treatment of Endodermal Sinus Tumor / 임상소아혈액종양

Endodermal sinus tumor is a type of germ cell tumor that is relatively common in children. An important problem concerns secondary neoplasms after treatment. We report a case of osteosarcoma that developed five years after treatment of intrapelvic endodermal sinus tumor. The patient was a seven-year-old girl who presented with right thigh and knee joint pain. The patient had been diagnosed with endodermal sinus tumor five years previously and treated with a regimen consisting of high dose cisplatin, etoposide, bleomycin and surgery but without radiotherapy. We detected a mass shadow on the right distal femur that proved to be osteoblastic osteosarcoma by incisional biopsy. The patient received surgical treatment after chemotherapy that included high dose methotrexate. The follow-up bone scan revealed no abnormal uptakes. There has been no evidence of recurrence eighteen months of follow-up after chemotherapy.

An Adolescent Case of Castleman Disease of the Plasma Cell Type Treated with Radiotherapy / 임상소아혈액종양

Castleman disease (CD) is a disorder with distinct lymphoid hyperplasia of unknown origin, and it is rare in children. There are two major forms of CD: unicentric (localized) and multicentric. Pathologically, it can be categorized into three subtypes: hyaline- vascular, plasma cell, and mixed types. We experienced a 15-year-old boy with histopathologically proven mediastinal CD of the plasma cell type. He was referred to our hospital because of mediastinal widening in a chest x-ray found by chance. Surgery was performed to resect two right paratracheal masses and subtotal excision was done. As complete surgical resection was not possible, the patient subsequently received three-dimensional conformal radiation therapy (total 5,400 cGy in 27 fractions). The size of the mass decreased, and his clinical symptoms regressed without any side effects from radiotherapy. Here we report a case of unicentric plasma cell type CD, treated with radiotherapy due to incomplete surgical resection.

A Case of Osteosarcoma after Treatment of Endodermal Sinus Tumor / 임상소아혈액종양

Endodermal sinus tumor is a type of germ cell tumor that is relatively common in children. An important problem concerns secondary neoplasms after treatment. We report a case of osteosarcoma that developed five years after treatment of intrapelvic endodermal sinus tumor. The patient was a seven-year-old girl who presented with right thigh and knee joint pain. The patient had been diagnosed with endodermal sinus tumor five years previously and treated with a regimen consisting of high dose cisplatin, etoposide, bleomycin and surgery but without radiotherapy. We detected a mass shadow on the right distal femur that proved to be osteoblastic osteosarcoma by incisional biopsy. The patient received surgical treatment after chemotherapy that included high dose methotrexate. The follow-up bone scan revealed no abnormal uptakes. There has been no evidence of recurrence eighteen months of follow-up after chemotherapy.

Rapid Growing Superficial Cutaneous Leiomyosarcoma of the Face

Leiomyosarcomas are uncommon malignant smooth muscle tumors, mainly derived from vessels or viscera. Superficial leiomyosarcomas are a rare soft tissue sarcoma arising from the dermis or subcutaneous tissue in the skin. According to tumor origin and location, they are divided into cutaneous and subcutaneous leiomyosarcoma. They have distinctly different histologic and prognostic features from each other. Superficial leiomyosarcomas show a predilection for the proximal extremities and tend to be slow growing. We report one rare case of superficial cutaneous leiomyosarcoma on the right temporal area of face, which showed an extremely rapid growing mass within 3 months.

Idiopathic acute eosinophilic pneumonia in a 14-month-old girl / 소아과

Idiopathic acute eosinophilic pneumonia (IAEP), characterized by acute febrile respiratory failure associated with diffuse radiographic infiltrates and pulmonary eosinophilia, is rarely reported in children. Diagnosis is based on an association of characteristic features including acute respiratory failure with fever, bilateral infiltrates on the chest X-ray, severe hypoxemia and bronchoalveolar lavage fluid >25% eosinophils or a predominant eosinophilic infiltrate in lung biopsies in the absence of any identifiable etiology. We present a 14-month-old girl who was admitted to our pediatric intensive care unit because of acute respiratory distress. She had a fever, dry cough, and progressive dyspnea for 1 day. Chest X-ray showed multifocal consolidations, increased interstitial markings, parenchymal emphysema and pneumothorax. IAEP was confirmed by marked pulmonary infiltrates of eosinophils in the lung biopsy specimen. Most known causes of acute eosinophilic pneumonia, such as exposure to causative drugs, toxins, second-hand smoking and infections were excluded. Her symptoms were resolved quickly after corticosteroid therapy.

A Case of An Incidentally Diagnosed Adrenal Venous Malformation in A Child

Adrenal venous malformation is an uncommon disease. We report a case of an incidentally diagnosed adrenal cystic mass during an evaluation for gastritis in a child. A 14-year-old girl admitted to our pediatric department for epigastric pain. After the gastroscopy, she was diagnosed with hemorrhagic gastritis. A 5.5 cm-sized cystic mass was incidentally found adjacent to her left adrenal gland during an ultrasound examination for evaluating her abdominal symptoms. She underwent laparoscopic surgery for the diagnosis and treatment of this cystic mass, which was confirmed to be venous malformation at pathologic diagnosis.

Clinical and hematologic manifestations in patients with Diamond Blackfan anemia in Korea / 대한혈액학회지

BACKGROUND: Diamond Blackfan anemia (DBA), characterized by impaired red cell production, is a rare condition that is usually symptomatic in early infancy. The purpose of this study was to assess nationwide experiences of DBA encountered over a period of 20 years. METHODS: The medical records of 56 patients diagnosed with DBA were retrospectively reviewed from November 1984 to July 2010. Fifteen institutions, including 13 university hospitals, participated in this study. RESULTS: The male-to-female ratio of patients with DBA was 1.67:1. The median age of diagnosis was 4 months, and 74.1% were diagnosed before 1 year of age. From 2000 to 2009, annual incidence was 6.6 cases per million. Excluding growth retardation, 38.2% showed congenital defects: thumb deformities, ptosis, coarctation of aorta, ventricular septal defect, strabismus, etc. The mean hemoglobin concentration was 5.1+/-1.9 g/dL, mean corpuscular volume was 93.4+/-11.6 fL, and mean number of reticulocytes was 19,700/mm3. The mean cellularity of bone marrow was 75%, with myeloid:erythroid ratio of 20.4:1. After remission, 48.9% of patients did not need further steroids. Five patients with DBA who received hematopoietic transplantation have survived. Cancer developed in 2 cases (3.6%). CONCLUSION: The incidence of DBA is similar to data already published, but our study had a male predilection. Although all patients responded to initial treatment with steroids, about half needed further steroids after remission. It is necessary to collect further data, including information regarding management pathways, from nationwide DBA registries, along with data on molecular analyses.

Pneumocystis jiroveci Pneumonia in a 5-month-old Boy with Agammaglobulinemia: A Case Report / 대한구급학회지

Pneumocystis jiroveci (P. jiroveci) pneumonia is known as a common opportunistic infection in patients with impaired immunity. Underlying disease or conditions related to the development of P. jiroveci pneumonia include acquired immunodeficiency syndromes, as well as malignancies and congenital immune deficiency disorders. We describe a 5-month-old boy without significant medical history who was admitted at our hospital because of fever, tachypnea, vomiting, diarrhea, and lethargy whose condition became worse within several hours after admission. A chest X-ray showed bilateral diffuse infiltration and high resolution computed tomography showed diffuse bilateral ground-glass opacity. The patient was diagnosed with P. jiroveci pneumonia by direct immunofluorescent antibody staining from lung biopsy and he was later diagnosed with agammaglobulinemia. Although the boy was treated with antibiotics, high-dose corticosteroids and mechanical ventilation, he expired on the 5th hospital day. Here, we report the case of P. jiroveci pneumonia in a boy with agammaglobulinemia.

A Case of Oligodendroglioma after Treatment of Acute Lymphoblastic Leukemia in Childhood / 임상소아혈액종양

Secondary brain tumors after cranial irradiation have been reported in survivors of childhood acute lymphoblastic leukemia (ALL). We report a case of secondary oligodendroglioma, occurring in a 19-year-old male, 12 years after the diagnosis of ALL. He was treated with vincristine, asparaginase, doxorubicin, methotrexate, prednisone, 6-mercaptopurine and cytarabine followed by prophylactic cranial irradiation at the age of 7 years. After treatment of ALL was completed at the age of 10 years, he showed symptoms of seizure, headache and decreased visual acuity at the age of 19 years. Magnetic resonance imaging (MRI) of the brain showed a mass lesion which was revealed as low-grade oligodendroglioma by stereotactic biopsy. He was treated with cranial radiotherapy, followed by 4 cycles of chemotherapy composed of PCV (procarbazine, methyl-1-(2-chloroethyl)-1-nitrosourea (CCNU), and vincristine) for oligodendroglioma, and sodium valproate for seizure control. The patient showed stable disease and no recurrence of seizure 8 months after treatment.

A Case of Oligodendroglioma after Treatment of Acute Lymphoblastic Leukemia in Childhood / 임상소아혈액종양

Secondary brain tumors after cranial irradiation have been reported in survivors of childhood acute lymphoblastic leukemia (ALL). We report a case of secondary oligodendroglioma, occurring in a 19-year-old male, 12 years after the diagnosis of ALL. He was treated with vincristine, asparaginase, doxorubicin, methotrexate, prednisone, 6-mercaptopurine and cytarabine followed by prophylactic cranial irradiation at the age of 7 years. After treatment of ALL was completed at the age of 10 years, he showed symptoms of seizure, headache and decreased visual acuity at the age of 19 years. Magnetic resonance imaging (MRI) of the brain showed a mass lesion which was revealed as low-grade oligodendroglioma by stereotactic biopsy. He was treated with cranial radiotherapy, followed by 4 cycles of chemotherapy composed of PCV (procarbazine, methyl-1-(2-chloroethyl)-1-nitrosourea (CCNU), and vincristine) for oligodendroglioma, and sodium valproate for seizure control. The patient showed stable disease and no recurrence of seizure 8 months after treatment.

Alteration of CD4+CD25+Foxp3+ T cell level in Kawasaki disease / 소아과

PURPOSE: Exaggerated pro-inflammatory reactions during the acute phase of Kawasaki disease (KD) suggest the role of immune dysregulation in the pathogenesis of KD. We investigated the profiles of T regulatory cells and their correlation with the clinical course of KD. METHODS: Peripheral blood mononuclear cells were collected from 17 KD patients during acute febrile and subacute afebrile phases. T cells expressing CD4, CD25, and Foxp3 were analyzed using flow cytometry, and the results were correlated with the clinical course of KD. RESULTS: The percentage of circulating CD4+CD25highFoxp3+ T cells among CD4+ T cells was significantly higher during the subacute afebrile phase than during the acute febrile phase (1.10%+/-1.22% vs. 0.55%+/-0.53%, P=0.049). Although levels of CD4+CD25lowFoxp3+ T cells and CD4+CD25-Foxp3+ T cells were only slightly altered, the percentage of CD4+CD25+Foxp3- T cells among CD4+ T cells was significantly lower during the subacute afebrile phase than during the acute febrile phase (2.96%+/-1.95% vs. 5.64%+/-5.69%, P=0.036). Consequently, the ratio of CD25highFoxp3+ T cells to CD25+Foxp3- T cells was higher during the subacute afebrile phase than during the acute febrile phase (0.45%+/-0.57% vs. 0.13%+/-0.13%, P=0.038). CONCLUSION: Decreased CD4+CD25highFoxp3+ T cells and/or an imbalanced ratio of CD4+CD25highFoxp3+ T cells to CD4+CD25+Foxp3- T cells might play a role in KD development. Considering that all KD patients were treated with intravenous immunoglobulin (IVIG), recovery of CD4+CD25highFoxp3+ T cells during the subacute afebrile phase could be a mechanism of IVIG.