White blood cell differential counts in severely leukopenic samples: a comparative analysis of different solutions available in modern laboratory hematology

BACKGROUND: We evaluated the efficacy of white blood cell (WBC) differential counts in severely leukopenic samples by the Hematoflow method and by automated hematology analyzers and compared the results with manual counts. METHODS: EDTA-anticoagulated blood samples (175 samples) with WBC counts of 40-990/microL were selected. Hematoflow differential counts were performed in duplicates employing flow cytometry using the CytoDiff reagent and analysis software. Differential counts were also performed using the DxH 800 (Beckman Coulter) and XE-2100 (Sysmex) automated hematology analyzers. The sum of the manual counts by a hematology technician and a resident were used as the manual counts. RESULTS: The total analysis time and hands-on time required by the Hematoflow method were shorter than those required by manual counting. Hematoflow counts were reproducible, showed a good correlation with automated analyzers, and also showed strong correlation with manual counts (r > 0.8) in neutrophils, lymphocytes, and monocytes. None of the cases containing less than 4% blasts as analyzed by the Hematoflow method had blasts in the manual counts, but 8 cases of 21 cases (38.1%) with over 4% blasts by Hematoflow had blasts in manual counts. CONCLUSION: Hematoflow counts of severely leukopenic samples were reproducible and showed a good correlation with manual counts in terms of neutrophil, lymphocyte, and monocyte counts. The Hematoflow method also detected the presence of blasts. Manual slide review is recommended when over 4% blasts are found by Hematoflow.

Comparison of Group A, B and C Rotaviral Gastroenteritis among Children in Korea: Prevalence and Clinical Features / 소아감염

PURPOSE: The aim of this study is that the prevalence of rotavirus infection was evaluated by each group and clinical features of group A, B and C rotaviruses infections were described respectively to compare one with another. METHODS: Between January 2010 and December 2010, we enrolled a group of children below 10 years of age admitted for management of acute diarrhea at the Catholic University of Korea Bucheon St. Mary's Hospital. A total of 310 stool samples documented to be free of common bacterial pathogens were collected from children with diarrhea. The presence of group A, B or C rotavirus is indicated by amplification of DNA segments of the expected lengths after the first and second PCRs. RESULTS: In a total of 310 stool specimens, 40 (12.9%) specimens were positive for rotaviruses. These included 23 (7.4%) positive for group A, 5 (1.6%) for group B and 12 (3.9%) for group C rotaviruses. Group B rotavirus infected patients had significantly less diarrheas per day (group A: P=0.01, group C: P=0.01) and shorter duration of vomiting days (group A: P=0.03, group C: P=0.03) than those with group A and C rotaviruses infection respectively. All the group B rotaviruses had been isolated in March and October. Group C rotavirus infections were prevalent during late summer and early winter and peaked in October. CONCLUSION: These findings indicate that group B and C rotaviruses are notable causes or the contributing causes of diarrhea among infants and children in Korea.

Prevalence of Viruses with Diarrhea among Hospitalized Children West Gyeonggi Province / 소아감염

PURPOSE: This study was conducted to evaluate epidemiological data of the viral pathogens obtained from stool exams and provide information on the regional prevalence of infectious diarrheal disease west in Gyeonggi Province, Korea. METHODS: We enrolled a cohort of children <10 years of age admitted for treatment of acute diarrhea at Bucheon St. Mary's Hospital, College of Medicine, The Catholic University of Korea. In total, 310 fecal specimens, documented to be free of common bacterial pathogens, were collected from pediatric patients during a 12-month period from January to December 2009 and were tested for the presence of rotavirus, parechovirus, adenovirus, astrovirus, enterovirus, and norovirus using polymerase chain reaction (PCR) and reverse transcription polymerase chain reaction (RT-PCR) assay. RESULTS: The most common virus was parechovirus (16%), followed by adenovirus (15%), astrovirus (14%), rotavirus (13%), and enterovirus (5%). Interestingly, only one of the specimens was positive for norovirus. Single infection cases were detected in 173 (55.8%) of the 310 children, whereas mixed viral infections were detected in 10 (3.2%) of the same children. Viral gastroenteritis generally showed a double peak of incidence. Parechovirus, rotavirus, and adenovirus shared a similar pattern of peak incidence with overall viruses; however, astrovirus infections occurred more frequently in the spring. Eighty-five percent of the confirmed viral gastroenteritis cases developed in under 24 months. CONCLUSION: The results support the importance of parechovirus, adenovirus, astrovirus, and enterovirus as causative agents of diarrhea in children, which may be underestimated by current routine diagnostic testing.

A rise and fall in AST and ALT level in nonspecific reactive hepatitis / 소아과

PURPOSE: The study was performed to assess the distribution of the diseases associated with nonspecific reactive hepatitis (NRH) for the past 10 years and to evaluate the change in the level of AST and ALT, and the difference by several variable factors. METHODS: From January 1997 to December 2006, 646 patients had elevated liver enzymes among 22,763 patients admitted to the Holy Family Hospital. We analyzed the difference in the age distribution, the period of elevated levels of AST and ALT, the resolution period, the peak value, the daily resolution value of AST and ALT, the sexual differences and the difference in several disease entities. One hundred and ninety-seven patients not confirmed as NRH or lost during follow-up were excluded. RESULTS: The prevalence rate of NRH was 2.84%. When compared to AST, ALT showed longer period of morbidity and resolution and the peak value was also higher in ALT. The male and female ratio showed significant value of 1.63:1. The morbid and resolution periods of AST and ALT between males and females were longer periods in males. The most prevalent disease entities were respiratory and gastrointestinal infections. Between the respiratory and the gastrointestinal diseases, the highest level of AST and ALT was observed in the respiratory disease. CONCLUSION: NRH is a common disease that occurs in 2.84% of the admitted pediatric patients. However, the pathogenesis and the progress of the disease have not been well known due to the lack of generalized information. Further research is necessary in the future.

Stroke Associated With Protein C and S deficiency in Neonate: Report of two Cases

Recently, cases of stroke with inherited disorders of blood coagulation have been reported in children. Deficiency of protein C or S predisposing to a hypercoagulable state has been associated with cerebrovascular disease. We describe here two cases of cerebral infarction in neonates presenting with respiratory distress and seizure respectively. One had decreased protein C and the other was found to have decreased protein C and S. One case had asphyxia as an additional risk factor for stroke. No specific therapy was administered in both cases. They are currently being observed in the outpatient clinic without specific events.

Normal antistreptolysin O titers of children by age group in the Gyeonggi-Incheon region / 소아과

PURPOSE: Measurement of antistreptolysin O (ASO) is often necessary to confirm a clinical diagnosis of recent streptococcal infection, especially in patients suspected of rheumatic fever and acute glomerulonephritis. Standard normal ranges for ASO should be established locally for each age group. We analyzed ASO to determine the upper limit of normal (ULN) ASO in children in the Gyeonggi-Incheon area. METHODS: ASO in normal individual concentrations were measured quantitatively by nephelometry on sera from 753 children (Male:381, Female:372). ULN were determined by separating the upper 20% from the lower 80% of the group (80 percentile). RESULTS: The mean ASO concentration calculated in a total cases was 149.9+/-7.2 IU/mL. The ASO concentration in neonates was 83.4+/-10.7 IU/mL, and lowest in the 1 year of age group, 26.7+/-6.6 IU/ mL, and increased to 318.0+/-33.2 IU/mL gradually in the 9 years of age group. Thereafter, ASO concentration decreased. The ULN for neonates was 122 IU/mL, for 0-3 years, 40 IU/mL; for 4-6 years, 113 IU/ mL; for 7-9 years, 489 IU/mL; for 10-19 years, 433 IU/mL; for 20-29 years, 122 IU/mL. CONCLUSION: The age-specific ULN for children in the Gyeonggi-Incheon area were determined. The distribution of ASO concentration according to age groups was different from previous reports. These results should be of clinical value to physicians to interprete the ASO results of their patients.

A case of multicystic dysplastic kidney and cystic adenomatoid malformation of the lung identified as incidental findings / 소아과

Multicystic dysplastic kidney and congenital cystic adenomatoid malformation of the lung are independent disorders, but both result from abnormal morphogenesis during embryogenesis. Congenital cystic adenomatoid malformation of the lung is associated with renal anomalies as well as other extrapulmonary anomalies and almost all cases with these anomalies are stillborn. We report a case of a 21-month-old male who was admitted with the impression of acute infectious gastroenteritis; multicystic dysplastic kidney with congenital cystic adenomatoid malformation of the lung was detected incidentally during evaluation.

The alteration of the positive rate of cytomegalovirus IgG antibody among preschool period children / 소아과

PURPOSE: In order to evaluate the time of disappearance of cytomegalovirus(CMV) IgG antibodies from mothers, and the alteration of the positive rate of CMV IgG antibodies among preschool period children, we investigated the positive rate of CMV antibodies among preschool children. METHODS: We studied 391 children who visited the Department of Pediatrics from March, 2001 to February, 2004. We measured the serum CMV IgG of 217 children and the serum CMV IgM of 358 children. RESULTS: The positive rate of CMV IgG antibodies is 83.9 percent(the number of positive IgG children is 182 out of 217). The alteration of the positive rate is 92.9 percent in 0-3 months, 75.0 percent in 4-6 months and the nadir was 20.0 percent in 7-9 months. Then, the positive rate increased to 83.9 percent in 22-24 months. After 22 months, the positive rate was 92.1 percent(the number of positive IgG children was 105 out of 114). The positive rate of CMV IgM antibody by age is 3.3 percent in 0-1 months, 3.6 percent in 1-2 months, 10.5 percent in 2-3 months, 14.3 percent in 3-4 months, 14.3 percent in 4-5 months, and then the results of five children among 148 children were positive. The distribution was one in 22-23 months, one in 25-26 months, one in 27-28 months, one in 28-29 months, one in 40-41 months. We discovered IgM positive children succesively from birth to 5 months, but sporadically after 5 months. CONCLUSION: The CMV IgG from mothers has decreased since birth and the time of nadir is 7-9 months. But it increases to a mean value of 83.9 percent at 22-24 months because of perinatal or postnatal infections.

A Case of Young-Simpson Syndrome / 소아과

Young Simpson syndrome is a rare malformation syndrome characterized by congenital hypothyroidism, dysmorphic face, mental retardation, severe postnatal growth retardation, hypotonia and congenital heart abnormalities. In the present study, we report a case of 4-year-old girl with Young Simpson syndrome for the first case in Korea.

A Case of G-6-PD Guadalajara / 소아과

Glucose-6-phosphate dehydrogenase(G-6-PD) deficiency is a disease that shows hemolytic anemia and jaundice caused by injury of erythrocytes. The gene of G-6-PD has 13 exons and locates in Xq28, and over 150 mutations of this gene have been reported. We experienced a G-6-PD deficienct male patient who was suffering hemolytic anemia and jaundice confirmed by measuring low G-6-PD activity in the erythrocytes. We found point mutation at 1159th nucleotide in 10th exon, cytosine was changed to thymidine, and was confirmed as G-6-PD Guadalajara.

Comparison of Cytokine Expressions among Kawasaki Disease and Its Symptom-related Diseases / 소아과

PURPOSE: Kawasaki disease(KD) is an important acute febrile systemic vasculitis disease. Various cytokines have been studied to evaluate the pathogenesis of vascular injury. Some cytokines have been implicated for vascular injury, but there has been no direct evidence. We compared cytokine profiles of KD with Henoch-Schonlein purpura(HSP), and febrile infections. METHODS: We investigated the serum concentrations of six cytokines and intracellular TNF-alpha and IL-6 levels in T cells and monocytes with acute KD, HSP and infections. We determined serum cytokine concentrations using the cytometric bead array method and detected intracellular TNF-alpha and IL-6 levels using QuantiBRITE and dual color flow cytometry. RESULTS: Serum IFN-gamma concentrations were increased more in KD and infections than in HSP and normal controls. Serum TNF-alpha concentrations were higher in KD and infections than in HSP and normal controls. And TNF-alpha concentrations in KD were lower than in infections. Serum IL-10 concentrations were higher in KD and in infections than in HSP and normal controls. And IL-10 concentrations in KD were higher than in infections. Serum IL-6 concentrations were higher in KD and in infections than in HSP and normal controls. Serum IL-4 and IL-2 concentrations were lower and similar to those of normal controls, respectively. However, intracellular IL-6 levels in T cells were higher in KD than in infections and intracellular IL-6 levels in monocytes were higher in KD than in infections. CONCLUSION: These results suggest that the alterations of various cytokines in KD are related to fever rather than to vasculitis. KD showed higher IL-10 levels in serum and intracellular IL-6 levels in T cell and monocyte than HSP. This reveals that the pathogenesis of KD is different from that of HSP.

A Case of Bilateral Congenital Nasolacrimal Duct Cysts Presenting as Neonatal Respiratory Distress / 대한주산의학회잡지

Congenital nasolacrimal duct cyst is an uncommon anomaly of nasolacrimal duct obstruction in the newborn. It is cystic dilation of the lower end of the unperforated nasolacrimal duct with intranasal extension. In such cases, the bluish-gray cyst arises beneath the inferior turbinate on nasal endoscopy. A large nasolacrimal duct cyst can fill the nasal cavity and lead to nasal obstruction. Neonates are obligate nasal breathers at birth and require several weeks to learn to breathe through the mouth. Consequently this nasal obstruction can cause significant respiratory distress. We experienced a case of bilateral nasolacrimal duct cyst presenting as neonatal respiratory distress. Diagnosis was confirmed by nasal endoscopy and CT scan. After endoscopic marsupialization of the cysts , symptom and sign of respiratory distress were rapidly resolved.

Plasma G-CSF and GM-CSF Concentration and Amount of Their Receptors on the Granulocyte in Kawasaki Disease

PURPOSE: This study aimed to demonstrate the possible pathogenesis of granulopoiesis in patients of Kawasaki disease(KD) using quantitative analysis of G-CSF, GM-CSF and their CSFr. METHODS: The plasma levels of G-CSF, GM-CSF, G-CSFr and GM-CSFr were studied in 14 patients in the acute phase of KD; 13 children with normal peripheral white blood cell counts were used as the normal control group. The plasma concentration of G-CSF, GM-CSF were analyzed by ELISA. The G-CSFr and GM-CSFr on the peripheral granulocytes were analyzed by a quantitative flow cytometric assay and QuantiBRITE, and the quantitative changes of receptors which did not combine with G-CSF and GM-CSF were measured. RESULTS: The total number of leukocytes in KD was similar to normal control group, but the leukocytes increased according to the number of neutrophils. The plasma concentration of G-CSF were decreased similar to normal control group(P=0.133), but that of GM-CSF decreased more than the normal control group(P=0.227). The quantity of G-CSFr, GM-CSFr were revealed to be no less than the normal control(P=0.721, P=0.912). After incubation with excessive G-CSF, the expressed G-CSFr on the neutrophils were decreased in both groups(P=0.554). The quantities of expressions of GM- CSFr on the neutrophil after incubation with the excessive GM-CSF were always increased in both groups(P=0.255). The amount of GM-CSFr of neutrophils are in proportion to total white blood cells (r=0.788, P=0.035), but it wasn't in the case of KD(P=0.644). CONCLUSION: The leukocytosis in KD that mediated by increasing neutrophil was not correlated with the plasma concentrations of G-CSF and GM-CSF, and the amount of expression of G-CSFr and GM-CSFr on granulocyte. It is possible that the reduction of concentration of GM-CSF results by increasing the active GM-CSFr.

Plasma G-CSF and GM-CSF Concentrations and Expression of their Receptors on the Granulocyte in Children with Leukocytosis

PURPOSE: Granulocyte-colony stimulating factor(G-CSF) and granulocyte macrophage-colony stimulating factor(GM-CSF) are principal cytokines in granulopoiesis and their physiologic effects are mediated through binding to specific cell surface receptors. Although it is known that the level of serum G-CSF and GM-CSF, and presentation of the receptors are increased in infectious diseases, there have been no studies to find the correlation between the granulopoiesis and leukocytosis. This study was designed to measure G-CSF and GM-CSF in leukocytosis and in control and to demonstrate the possible pathogenesis of granulopoiesis in leukocytosis using quantitative analysis of G- CSF, GM-CSF and their CSFr. METHODS: The plasma levels of G-CSF, GM-CSF of 13 children without leukocytosis and 14 children with leukocytosis were measured. Counts of cell surface G-CSFr and GM-CSFr were measured by combining anti G-CSFr and anti GM-CSFr monoclonal antibodies to their respective receptors by using quantitative flow cytometric assay. RESULTS: There was no significant difference betweeen the plasma concentration of G-CSF and GM-CSF in acute leukocytosis and in the control group. However, levels of G-CSFr in acute leukocytosis decreased significantly compared to the control(P=0.012) and the levels of GM-CSFr in both groups revealed no significant difference. CONCLUSION: Increase in the number of leukocyte in leukocytosis was mediated by increasing the number of neutrophil, and increased plasma concentration of G-CSF may be the cause of neutrophilia. But GM-CSF did not have any influence on leukocytosis.

Down Syndrome

No abstract available.

A Case of Glucose-6-phosphate dehydrogenase Riley Causing Hemolytic Anemia / 대한혈액학회지

A glucose-6-phoshate dehydrogenase variant called G6PD Riley was detected in an Korean boy with nonspherocytic hemolytic anemia. Using polymerase chain reaction based single-strand conformation polymorphism (PCR-SSCP) followed by DNA sequence analysis, we found mutation T to C at nucleotide 1139 in exon 10, resulting in a substitution of 380th amino acid isoleucine to threonine. The patient's mother was confirmed to be a heterozygote.

Antineutrophil Cytoplasmic Antibody in Kawasaki Disease

PURPOSE: Antineutrophil cytoplasmic antibody (ANCA) has been identified in various disorders including Wegener's granulomatosis, microscopic polyarteritis and Kawasaki disease. Measuring this antibody has a diagnostic role. It facilitates monitoring disease activity and may also help understand the pathogenesis of the diseases in which it is found. We investigated the correlation between the hematologic findings and ANCA in acute Kawasaki disease and the diagnostic potential of ANCA to predict coronary artery involvement. METHODS: Thirty-eight patients who met the diagnostic criteria for Kawasaki disease were enrolled in this study. We sampled and investigated the hematologic findings and the assay of ANCA before intravenous immunoglobulin treatment and weekly echocardiographs weekly. RESULTS: There was no sexual difference between ANCA positive and negative group. The age in ANCA positive group was significantly lower than in ANCA negative group. Duration of fever before treatment in ANCA positive group were not significantly different from those in ANCA negative group. In ANCA positive group, the mean WBC count and the mean ESRs were higher than in ANCA negative group. There was no relation between ANCA and coronary artery involvement. CONCLUSION: The assays of ANCA in acute Kawasaki disease does not help to predict disease activity and coronary artery involvement.

A Case of Glucose-6-Phosphate Dethydrogenase Deficiency with Chronic Hepatitis B / 대한소아내분비학회지

Glucose-6-phosphate dehydrogenase(G6PD) deficiency is the most common X- linked inherited disorder and is estimated to affect 400 million people worldwide. But the incidence of this disease is very rare in far-east Asia, especially in Korea. Many drugs and infections cause hemolytic anemia in patients with G6PD deficiency. We experienced a case of G6PD deficiency with chronic hepatitis B. The diagnosis was made by clinical symptoms, laboratory data including serologic test and bone marrow findings. We report a case of G6PD with chronic hepatitis with a brief review of related literatures.

Interphase cytogenetics of non-Hodgkin's lymphoma using non-fluorescent in situ hybridization in paraffin embedded tissue

Paraffin-embedded tissue samples from 30 cases of non-Hodgkin's lymphoma(NHL) and 10 of reactive hyperplasia, were processed for interphase cytogenetic chromosomal study. We performed non-fluorescent in situ hybridization(NFISH) using the enzymatic method with digoxigenin-labeled DNA centromeric probes for chromosome 7,12,18 and X, and a painting probe for chromosome 18. Chromosomal aberrations were observed in 27(90%) out of 30 cases of NHL. The most commonly observed numerical aberration was extracopy of X chromosome. There were some characteristic aberrations corresponding to each grade and group of NHL by International Working Formulation: In low grade NHL(9 cases), a third were associated with extracopy of chromosome 12, and disomy X was frequently found in small lymphocytic lymphoma(75%). With intermediate grade(16 cases), tetraploidy(25%), translocation of chromosome 18(25%), and extracopy of chromosome 18(19%) were characteristically associated. These results suggest that interphase NFISH is an easily performable method in retrograde cytogenetic study of archival materials. Some specifically correlated chromosomal aberrations corresponding to the histopathologic grades and groups could provide us more valuable information for determining pathologic diagnosis and assessing the clinical outcome of NHL.

Use of Fluorescent in Situ Hybridization to Evaluate the Number of Chromosomes in Buccal Smear Cells in Normal Newborn Infants

No abstract available.