Deferiprone Related Arthropathy in 11-Year-Old Boy with Hereditary Spherocytosis

Regular transfusion therapy in children with intractable hematologic disorder results in hemosiderosis. It causes several organ damages such as liver, heart, and endocrine system and increases risk of infection. Thus, iron-chelating therapy is essential to promote normal development, prevent excess iron overload and death in children. In abroad, deferiprone has been approved for use in thalassemic children with transfusion induced iron overload. Side effects associated with deferiprone include agranulocytosis, arthropathy, gastrointestinal symptoms, increased alanine aminotransferase levels, and zinc deficiency. Arthropathy has been reported to occur approximately 9%–38% of patients. We present an 11-year-old boy patient with hereditary spherocytosis, who experienced arthropathy while under deferiprone therapy.

Ecthyma gangrenosum and agranulocytosis in a previously healthy 12-month-old girl: Report of 1 case with a literature review

Ecthyma gangrenosum (EG) is a rare skin manifestation which starts with a maculopapular eruption and followed by a necrotic ulcer covered with black eschar. EG usually occurs in immunosuppressed patients with Pseudomonas aeruginosa sepsis. We present a previously healthy 12-month-old girl with EG by P. aeruginosa and agranulocytosis due to influenza A and then rhinovirus infection, without bacteremia. It is important for allergists to culture wound and differentiate EG from other skin disorders including Tsutsugamushi disease and initiate appropriate empiric antipseudomonal antibiotic treatment, and to evaluate for possible immunodeficiency, even in a healthy child.

A Concomitant Hemato-Oncologic Disorder of Patient with Down Syndrome

A patient with Down syndrome has hematologic and oncologic disorders of medical complication. Down syndrome shows that characteristic hematologic and oncologic abnormalities and developments of disorders are different at ages. Many hematologic disorders were related to Down syndrome. There are diseases of red blood cells, white blood cell disorders, platelet and bleeding disorders, aplastic anemia, and transient myeloproliferative disease. Acute myeloid leukemia, acute lymphoblastic leukemia, and rarely solid tumors (Neuroblastoma, Wilm's tumor, Hodgkin's lymphoma) are also associated with Down syndrome. We checked clinical manifestations of each disorder and we should make standard hematologic index of different age groups. In addition, the relations between chromosome 21 and hemato-oncologic disorders should be found. We need to investigate potential therapeutic interventions that can improve quality of life and life expectancy in patients with Down syndrome.

Achromobacter xylosoxidans Bacteremia in a Child with Neutropenia

Achromobacter xylosoxidans is a non-fermentative, aerobic, oxidase, and catalase-positive Gram-negative rod similar to Pseudomonas species. This organism colonizes aquatic environments and can cause nosocomial infections, especially in patients with immune deficiency such as human immunodeficiency virus infection, cancer, cystic fibrosis, neutropenia, and immunoglobulin M deficiency. Infections are found as bacteremia, pneumonia, meningitis, urinary tract infection, abscess formation, and osteomyelitis. It is known that most effective antibiotics are piperacillin/tazobactam, meropenem, and trimethoprim/sulfamethoxazol. But there is no optimal antibiotic therapy so far. We present a case of Achromobacter xylosoxidans bacteremia in a 13-month-old Korean girl who had past history of neutropenia.

Rosai-Dorfman Disease of 4-Year-Old Girl

Rosai-Dorfman disease also known as sinus histiocytosis with massive lymphadenopathy is a benign, rare systemic disease characterized by a histiocyte proliferation which presents with lymphadenopathy. We report a case of a 4-year-old girl who presented with recurrent cervical lymphadenopathy with tenderness, without any other symptoms. After 1 month of medical treatment, her lymphadenopathy still remained, so we performed complete excision and biopsy. She was diagnosed on cytology as a case of Rosai-Dorfman disease. She responded well to become asymptomatic without recurrence by 1 month.

Case Report of Inferior Vena Cava Agenesis Accompanying Deep Vein Thrombosis / 임상소아혈액종양

A 17-year-old male patient was admitted to the author's hospital complaining of pain in both flanks. He had a history of being admitted to a tertiary-level hospital a year before, for swelling of the left limb. Deep vein thrombosis (DVT) was observed in the left common iliac vein, and the patient received anticoagulation therapy for six months. Prior to revisiting the hospital, he was symptom free and claimed to be healthy. An abdominal CT scan showed findings of congenital absence throughout the entire length of the IVC. To prevent the obstruction of the multiple collateral vessels developed around the IVC, and to improve the renal venous drainage, the patient was immediately started on with anticoagulation therapy. Reported herein is a case of an incidentally discovered congenital anomaly of the IVC in a patient with DVT.

Case Report of Inferior Vena Cava Agenesis Accompanying Deep Vein Thrombosis / 임상소아혈액종양

A 17-year-old male patient was admitted to the author's hospital complaining of pain in both flanks. He had a history of being admitted to a tertiary-level hospital a year before, for swelling of the left limb. Deep vein thrombosis (DVT) was observed in the left common iliac vein, and the patient received anticoagulation therapy for six months. Prior to revisiting the hospital, he was symptom free and claimed to be healthy. An abdominal CT scan showed findings of congenital absence throughout the entire length of the IVC. To prevent the obstruction of the multiple collateral vessels developed around the IVC, and to improve the renal venous drainage, the patient was immediately started on with anticoagulation therapy. Reported herein is a case of an incidentally discovered congenital anomaly of the IVC in a patient with DVT.

Risk Factors for Acute Hepatitis A Infection in Korea in 2007 and 2009: A Case-Control Study

This study aimed to identify the risk factors associated with acute hepatitis A virus (HAV) infection in the Korean population. Participants were recruited from five referral hospitals across the country in 2007 and from 11 hospitals in 2009. Patients with positive anti-HAV IgM antibody tests became the case group, while patients treated for non-contagious diseases at the same hospitals were recruited as controls. A total of 222 and 548 case-control pairs were studied in the 2007 and 2009 surveys, respectively. Data from the surveys were analyzed jointly. In a multivariate analysis, sharing the household with HAV-infected family members (OR, 6.32; 95% CI, 1.4-29.6), contact with other HAV-infected individuals (OR, 4.73; 95% CI, 2.4-9.4), overseas travel in 2007 (OR, 19.93; 95% CI, 2.3-174.4), consumption of raw shellfish (OR, 2.51; 95% CI, 1.8-3.5), drinking bottled water (OR, 1.64; 95% CI, 1.3-8.4), and occupation that involve handling food (OR, 3.30; 95% CI, 1.3-8.4) increased the risk of HAV infection. Avoiding contact with HAV-infected individuals and avoiding raw foods eating could help minimize the risk of hepatitis A infection. Immunization must be beneficial to individuals who handle food ingredients occupationally or travel overseas to HAV-endemic areas.

Efficacy of Intravenous Immunoglobulin Therapy in Childhood Atopic Dermatitis

OBJECTIVE: Atopic dermatitis (AD) is a chronic inflammatory skin disease with significant morbidity, and for which there is a need for safe and effective alternative therapies. Although a few observations on the efficacy of intravenous immunoglobulin (IVIG) in AD have been reported, clinical evidence of effectiveness from controlled trials is lacking. Therefore, the purpose of this study was to clarify whether IVIG therapy (1.0 g/kg body weight at each monthly visit for 6 months) is effective in childhood atopic dermatitis and to analyze the clinical characteristics of IVIG responses in this disease. METHODS: Forty three atopic dermatitis patients who had characteristic clinical features of atopic dermatitis were included in this study. The patients received an injection of IVIG at 1.0 g/kg body weight at each monthly visit for 6 months. Laboratory tests were performed for blood chemistry, total immunoglobulin E, immunoglobulin G/immunoglobulin A/immunoglobulin M, blood eosinophil count, and C-reactive protein. RESULTS: In total forty three atopic dermatitis patients, only 14 patients completely underwent 6 cycles, but other 29 patients incompletely (1-5 cycles). In the 14 patients, there were just 13 records of scoring atopic dermatitis (SCORAD) index. The mean SCORAD score in the 13 patients was 39.6+/-24.4. SCORAD score decreased significantly (initial SCORAD, 39.6+/-24.4; final SCORAD, 21.3+/-15.6; P=0.016). CONCLUSION: IVIG therapy may be recommended in the treatment of recalcitrant atopic dermatitis. In addition, further investigation on predictive markers for responses of IVIG therapy in atopic dermatitis may be needed.

Usefulness of Spot Urine beta2-microglobulin in Pediatric Acute Pyelonephritis

OBJECTIVE: It is difficult to make a distinction between acute pyelonephritis and lower urinary tract infection due to nonspecific clinical symptoms and laboratory findings. We measured the spot urine beta2-microglobulin in children with urinary tract infection (UTI) to distinguish between acute pyelonephritis and lower UTI. We compared the accuracy of urine beta2-microglobulin measurement with other inflammatory markers. METHODS: We studied 83 children (mean, 86+/-44.9 months) who suspected of having UTI. Leukocyte counts, erythrocyte sedimentation rates (ESR) and C-reactive protein (CRP), beta2-microglobulin were measured. Renal parenchymal involvement was evaluated by 99mTc dimercaptosuccinic acid scintigraphy in the first 7 days after admission. beta2-microglobulin was measured by radioimmunoassay. RESULTS: Urine beta2-microglobulin values were correlated with the presence of renal defects in children with UTI (n=22) (0.98+/-0.24 microg/mL, P<0.05). Using a cutoff of 0.4 microg/mL for spot urine beta2-microglobulin and 20 mm/hr for ESR, 2.6 mg/L for CRP, sensitivity and specificity between UTI with and without renal involvement were 78.7% and 90.1% for spot urine beta2-microglobulin, 77.2% and 90.1% for spot urine beta2-microglobulin/creatinine (Cr), 77.2%, 68.8% for ESR, 86.3%, 68.8% for CRP, respectively. Positive and negative predictive values were 72.7%, 90.1% for spot urine beta2-microglobulin, 73.9% and 91.6% for spot urine beta2-microglobulin/Cr, and 57.5%, 94% for CRP, respectively. CONCLUSION: In febrile UTI, spot urine beta2-microglobulin and beta2-microglobulin/Cr values were more specific than CRP, ESR, and leukocyte count for determine the renal defects.

A Case of Basaloid Squamous Cell Lung Carcinoma in an 11-year-old Boy / 소아과

Primary lung cancer is unusual in children; the squamous cell variant is extremely rare. Lung cancer is classified by histologic types into small-cell lung cancer, non-small cell lung caner, carcinoid, mucoepidermoid carcinoma, and adenoid cystic carcinoma. Furthermore, non-small cell lung cancer is subclassified into adenocarcinoma, large-cell carcinoma, and squamous cell carcinoma. The incidence of lung cancer is influenced by smoking, especially in squamous cell carcinoma, and large cell carcinoma. The present treatments for these tumors are chemotherapy, radiation therapy, and surgical resection depending on their histologic types or stages, but yield very poor survival rates. In this article, we report a case of basaloid squamous cell lung carcinoma in an 11-year-old boy who had symptoms of both leg weakness and back pain radiating to both legs. We confirmed the primary lung carcinoma cells by percutaneous transthoracic needle biopsy. The metastatic carcinoma cells were identified at the bone marrow and lumbar spine. We treated with a combination chemotherapy and radiation therapy. However, he expired 4 months after the onset of disease.

A Case of Kawasaki Disease in a 16-year-old Girl Associated with Congestive Heart Failure

Kawasaki disease is an acute febrile vasculitis of childhood and leading cause of acquired heart disease in children. The illness occurs predominantly in young children and only occasionally occurs in teenagers and adults. So we report a case of adolescent-onset Kawasaki disease associated with congestive heart failure and development of coronary dilatation. A 16-year-old girl presented with fever, truncal rash, congestive heart failure due to myocarditis, bilateral conjunctival injection, strawberry tongue, redness of hands and feet, and cervical adenopathy. After Kawasaki disease was diagnosed, intravenous gamma globulin(2 g/kg) and aspirin(70 mg/kg/day) were administered. On the 20th hospital day, echocardiography showed moderate dilatation of right coronary artery. Four months after the discharge, follow up echocardiography showed sustained dilatation of right coronary artery.

A Case of Kawasaki Disease in a 16-year-old Girl Associated with Congestive Heart Failure

Kawasaki disease is an acute febrile vasculitis of childhood and leading cause of acquired heart disease in children. The illness occurs predominantly in young children and only occasionally occurs in teenagers and adults. So we report a case of adolescent-onset Kawasaki disease associated with congestive heart failure and development of coronary dilatation. A 16-year-old girl presented with fever, truncal rash, congestive heart failure due to myocarditis, bilateral conjunctival injection, strawberry tongue, redness of hands and feet, and cervical adenopathy. After Kawasaki disease was diagnosed, intravenous gamma globulin(2 g/kg) and aspirin(70 mg/kg/day) were administered. On the 20th hospital day, echocardiography showed moderate dilatation of right coronary artery. Four months after the discharge, follow up echocardiography showed sustained dilatation of right coronary artery.

Epidemiology and Clinical Outcomes in Children with Aplastic Anemia in Korea: Retrospective Study / 대한소아혈액종양학회지

PURPOSE: The annual incidence of aplastic anemia (AA) in Asian countries is higher than in Western countries. The pathogenesis in AA has been investigated in hematopoiesis and immunology. Recently, the survival rate and the quality of life of the patients with AA have been steadily improved by the development of a variety of treatments such as the immunosuppressive therapy (IST), and hematopoietic stem cell transplantation (HSCT). The Korean Society of Pediatric Hematology-oncology retrospectively investigated the incidence, treatment strategies, survival rate, and time to become independent from transfusion in patients with AA, who were diagnosed from January 1st, 1991 to December 31st, 2000 in Korea. METHODS: All the questionnaires were sent to a group of training hospitals, and we collected about 600 questionnaire forms from 27 hospitals. However, 493 reports were available for data analysis. RESULTS: The male and female ratio in AA is 1.1 (259 males vs. 234 female). The median age at diagnosis is 9 years old (range; 0.8~16 years old). The annual incidence of children with AA in Korea is 4.5 per million on the basis of Korean pediatric population. In etiology, there are 20 cases (4.1%) in congenital and others in acquired AA. In acquired AA, the cause of illness was not identifiable for most of the patients, but 1 patient had hepatitis-associated AA, and 3 patients developed the illness after medication. According to the initial laboratory data at diagnosis, the peripheral blood findings showed that hemoglobin is 7.1+/-2.4 g/dL, white blood cell 3, 200/microL (200~16, 550), absolute neutrophil counts 670/microL (0~12, 487), platelets 19, 000/microL (1, 000~500, 000), and corrected reticulocytes 0.18% (0.0~4.7). The bone marrow examination revealed that cellularity was below 25% in 348 patients, and over 25% in 105 patients. In the available data, 269 patients (54.6%) were diagnosed of severe aplastic anemia (SAA) and 224 patients of non-SAA (NSAA). HSCT were done for 96 patients (19.5%) and others received another treatments such as the IST. The anti-thymocyte globulin (ATG) or anti-lymphocyte globulin (ALG) treatment was done for 263 cases, corticosteroids for 259, cyclosporine A (CSA) for 215, and anabolic steroids for 138. The combination IST including ATG or ALG plus corticosteroid plus CSA were applied to 154 children with AA, and transfusion only in 37, as conservative care. In case of those patients with HSCT, the time from diagnosis to transplantation was 12 months (1~144 months) and the sources of stem cells were bone marrow in 82 cases, growth factor mobilized peripheral blood in five, and cord blood in six. There were 57 patients transfused below 40 units of blood products before HSC transplantation. Graft rejection was identified from 16 patients, and booster transplantations were done for 12 patients among them. In complications of HSCT, the graft versus host disease was developed in 20 patients and viral diseases in 12 cases including the CMV, herpetic infection, and hepatitis. Also, one patient suffered from veno-occlusive disease. The overall survival rate in children with AA is 64.3%. The survival rate in HSCT is better than that IST (76.9% vs. 62.6%, P< 0.05). In IST, overall survival rate in very SAA showed lower than SAA and NSAA, and in SAA lower than NSAA in case of absolute neutrophil count below 200/microL (P< 0.05). There was no significant difference in terms of the sex, age at beginning of treatment. In HSCT, transfusion was not related to the survival rate. However, overall survival rate is better in short interval between diagnosis and HSCT than in long interval (P< 0.05). There was no significant difference in the probability of transfusion independence according to treatment strategies, even though it was 71.0% in HSCT and 12.8% in immunosuppressive therapy at the end point of survey (P=0.47). The response pattern was as follows. There were 155 cases of complete response, 110 of partial response and 120 of no response in spite of various treatments. The relapse after treatment was found in 11 patients after IST, of which 6 patients experienced more than 2nd relapse. The median time between the end of treatment and relapse was 16 months (6~84 months). Only three cases developed into other diseases (1 case into acute myeloid leukemia and 2 cases into myelodysplastic syndrome). The median time from diagnosis to the end of treatment was 62 months (0.5~174 months). In fatal cases, the median time between diagnosis and death was 29 months (0~144 months) despite several therapeutic strategies. CONCLUSION: In Korea, the annual incidence of children with AA is 4.5 per million. This result is similar to the ones reported in other Asian countries, but higher than those in Western countries. Although a lot of children with AA received various therapies including IST or HSCT, new treatment strategies have to be developed to improve the survival rate and the quality of life of children with AA

Hodgkin Disease / 소아과

No abstract available.

Pathophysiology of Proteinuria / 소아과

No abstract available.

Arsenic Trioxide (As2O3) Induced Apoptosis in NB4 Cell lines / 대한혈액학회지

BACKGROUND: Recently, inorganic arsenic trioxide (As2O3) was reported to induce complete remission in a high proportion of patients with refractory acute promyelocytic leukemia (APL). To illustrate cellular and molecular mechanisms of As2O3 in the treatment of APL, many experimental studies were performed on APL-derived cell lines in vitro. Previous studies showed that As2O3 inhibited proliferation and induced apoptosis in the APL-derived cell lines. This study was done to clarify the in vitro mechanisms of As2O3-induced apoptosis in APL-derived NB4 cell lines. METHODS: To determine the effects of As2O3 in the various concentrations, NB4 cells were cultured with 0.1 to 2micro M/L of As2O3. To assay the apoptosis in NB4 cell lines, DNA fragmentation assay and TUNEL were performed. To find out the molecular change of As2O3- induced apoptotic NB4 cell lines, RT-PCR and Western blot analysis for PML-RARalpha chimeric protein expression and flow cytometry for bcl- 2/bax expression were performed. To clarify the caspase activation pathway, Western blot analysis and flow cytometry for procaspase expression were performed. RESULTS: As2O3 induces apoptosis on NB4 cells in relatively high concentration (0.5 to 2 micro M/L) for 2 days. After 2 days of culture the PML-RARalpha chimeric protein expression decreased rapidly by Western blot and RT-PCR analysis and bcl-2 expression also decreased by flow cytometry. The expression of bax by flow cytometry showed a marked increase in high concentration (2micro M/L) but there was no change in low concentration (0.5micro M/L). In the Western blot analysis, the amount of pro`enzyme of caspase-3 was significantly decreased in the cells with high concentration (2micro M/L) compared with that in the cells with low concentration (0.5micro M/L). As2O3 induces proteolytic processing of pro-caspase 7 but not pro-caspase 9 and 8. CONCLUSION: Apoptosis of APL-derived NB4 cell lines was induced by As2O3 and progressed rapidly in higher concentrations. During apoptosis, activation of caspase-7 pathway and degradation of PML-RARalpha chimeric protein, decrease in bcl-2 and increase in bax were shown.

The Detection of Chlamydia trachomatis Using Nested PCR in Prematurity and Delivery of Low Birth Weight Infant

PURPOSE: Polymerase chain reaction(PCR) has generally been more sensitive than traditional tests for the detection of Chlamydia trachomatis which has been a leading cause of sexually transmitted disease. We performed this study to find out if there is an association between C. trachomatis infection and low birth weight(LBW) or prematurity. METHODS: The study included 98 premature or LBW infants admitted to the neonatal intensive care unit of Soonchunhyang Chunan hospital between January and July of 1999. Nasopharyngeal swab specimens were obtained from 98 neonates with a LBW or prematurity. Endotracheal secretions were obtained from 28 LBW or premature infants. Vaginal swabs were collected from 47 mothers who had given birth to LBW or premature infants. We performed the nested PCR with Chlamydia-CR kit(Bioneer, Korea). PCR products were resolved by electrophoresis on 2% agarose gel. These products were observed by their size which was 345 bp. RESULTS: C. trachomatis was not detected by the nested PCR from 98 nasopharyngeal swabs nor 28 endotracheal secretion specimens. But, C. trachomatis was detected in 2(4.2%) out of 47 vaginal swab specimens from mothers who had given birth to LBW or premature infants. CONCLUSION: The nested PCR is a relatively simple, fast and practical tool for the detection of C. trachomatis, but the positive rates of C. trachomatis were low in LBW or premature infants and also in mothers who had given birth to LBW or premature infants. We suggest two possible conclusions from our study. The first is that nasopharyngeal swabs and endotracheal secretions are improper specimens for C. trachomatis. The second is that C. trachomatis infection is lower in our study.

Ultrasonographic and CT findings of Cervical Ectopic Thymus of an Infant

We report a case of ectopic thymus in the left submandibular area of a two-month-old boy. On US and CT scans, a well-marginated, 3 x 2cm-sized solid mass along the left carotid sheath, anteromedial to the sternocleidomastoid muscle and posterior to the submandibular gland, was seen. CT attenuation of the mass showed that it was similar to that of normal thymus in the anterior mediastinum. Although a rare disease, ectopic thymus should be included in the differential diagnosis of cervical masses along the carotid sheath in infants.

A Study on Mean Blood Lead and ZPP Levels of Children around Cheonan-city, Chungcheongnam-do

PURPOSE: Recent years have witness a dramatic increase in public awareness of the health risks associated with lead exposure-particularly concerning young children. The lead poisoning in children was considered a rarity, usually resulting from unique circumstances such as in appropriate use of lead-based body cosmetics or direct administration of lead-containing folk medicine or contaminated atmosphere. The increasing concern about children with lower levels of lead exposure has developed, but there are no data regarding the mean blood lead levels and the incidence of lead poisoning in Korean children. So, this study was conducted to measure the blood lead and ZPP(zinc protoporphyrin) levels of 125 children around Cheonan city. METHODS: 125 children around Cheonan city (from 1 month to 15 year-old, 68 males and 57 females) were studied. We analyzed the mean blood Zinc Protoporphyrin(ZPP) and lead values and excluded children with hematologic disorders in this study. RESULTS: 1) The mean blood lead level was 4.15 microgram/dl (95% CI: 3.82-4.488) and the mean ZPP level was 53.48 microgram/dl (95% CI: 48.44-58.52). Both were within normal range. 2) The mean blood lead level of 68 males was 4.23 microgram/dl and 4.06 microgram/dl in 57 females. The mean blood ZPP level was 49.88 microgram/dl in males and 57.77 microgram/dl in females. There was no significant difference in males and females. 3) There was no siginificant correlation between blood lead and ZPP concentration. CONCLUSIONS: We concluded that the mean blood lead level of 125 children around Cheonan city was 4.15 microgram/dl. That was within normal range and the same values with United States. It is important that there must be a national counter plan and that pediatricians continue to pay attention to lead poisoning in children.