Hair selenium status in children with leukemia and lymphoma

Selenium (Se) is a trace element contributing to the structure of antioxidant system that saves cells from reactive oxygen species. Low serum Se levels have been reported in pediatric and adult patients with cancers. On the other hand, hair Se levels, predicting the long-term body Se status, have been reported in only adult patients with cancer. The aim of the study was to investigate the hair Se status in children with newly diagnosed lymphoid malignancies and the relation between malnutrition and Se deficiency. Thirty patients with leukemia (n=17) and lymphoma (n=13), and 25 healthy controls were enrolled to the study. Se was determined with atomic absorption spectrophotometrical method. Hair Se levels of the patients were significantly lower than those of control group [666.96+/-341.46 ng/g vs. 1019.22+/-371.83 ng/g (P<0.001)]. Children with lymphoma had lower Se than the children with acute lymphoblastic leukemia but not statistically significant [547.03+/-283.67 ng/g vs. 758.67+/-361.05 ng/g (P>0.05)]. Malnourished patients (11/30) had lower hair Se levels (483.51+/-235.55 ng/g) than those of the controls (P=0.036), whereas the Se levels of the patients who had no malnutrition (773.17+/-352.92 ng/g) were also lower than those of the controls but not statistically significant (P=0.053). There was no correlation between age, sex, and the hair Se levels. In this study, we found that hair Se levels of the children with leukemia and lymphoma, especially those of malnourished patients, were lower than those of controls. Additional studies are needed to determinate whether low levels of hair Se may play a role in carcinogenesis.     

Fulminant type 1 diabetes mellitus in Korean adolescents

Fulminant type 1 diabetes has recently been identified as a new subtype of idiopathic diabetes that is mostly found in Japanese adults. The aim of this study was to investigate the frequency as well as the clinical and laboratory characteristics of fulminant type 1 diabetes among Korean children with childhood-onset type 1 diabetes. One-hundred and fifty patients that had been newly diagnosed with type 1 diabetes over the past 10 years were included. These patients came from three hospitals. Out of the 150 patients, two female patients fulfilled the criteria for fulminant type 1 diabetes. They were negative for islet autoantibodies. The patients with fulminant type 1 diabetes had an older age of onset and a lower HbA1c than the patients with autoimmune or idiopathic type 1 diabetes. In addition, the patients with fulminant type 1 diabetes had increased serum aspartate aminotransferase, alanine aminotransferase and amylase levels, and decreased fasting serum C-peptide levels. The frequency of fulminant type 1 diabetes was 1.33% among all patients newly diagnosed with type 1 diabetes under the age of 16. Although this type of diabetes is more commonly an adult-onset disease, it is possible that fulminant type 1 diabetes has not yet been fully recognized in children and adolescence, and may be more common than initially thought.     

Serum zinc levels in celiac disease

This study was done to determine the zinc levels in 30 children with celiac disease. Serum zinc level was estimated at inclusion and zinc supplementation was given for 3 months. Zinc levels were repeated at 3 and 6 months after inclusion. The serum zinc levels of newly diagnosed CD cases (0.64+/-0.34 microg/mL) versus controls (0.94+/-0.14 microg/mL) were significantly lower (95% CI -0.44 to -1.4), whereas in the old cases this difference was non-significant. The serum zinc level among severely malnourished and stunted celiac cases was also significantly lower irrespective of their treatment status. We conclude that serum zinc levels are low in newly diagnosed and severely malnourished children with celiac disease.     

Low Levels of Serum Transport Proteins Indicate Catabolic Protein Status During Induction Therapy for Acute Lymphoblastic Leukemia

Twelve children with newly diagnosed acute lymphoblastic leukemia (ALL) were followed during the first 24 weeks of induction and consolidation therapy. Twelve additional patients with other types of cancer, receiving no prednisone medication, served as a reference group. The serum total protein, albumin, transferrin, and prealbumin concentrations were measured at 0, 2, 4, 6-10, 16, and 24 weeks and used as biochemical indices of protein nutritional status. In all patients studied, serum albumin and prealbumin concentrations were low at diagnosis. Decreasing serum total protein and transferrin concentrations, stable low serum albumin, and increasing prealbumin levels were observed during the ALL induction therapy. In contrast, these protein levels remained stable in the children with other malignancies. By week 8 the patients with ALL had lower serum total protein, albumin, and transferrin than the children with other types of cancer. We conclude that the low levels of the serum transport proteins indicate catabolic protein status in children with ALL during early weeks of therapy.     

Juvenile dermatomyositis

Myositis in childhood is characterized by elevated serum levels of muscle-derived enzymes, proximal symmetrical muscle weakness, abnormal EMG findings, and a muscle biopsy, which frequently documents an inflammatory process. In the pediatric age group, JDMS, which has characteristic cutaneous involvement in addition to myositis, is much more common than PM and is more common among female patients. With the use of steroids, mortality has been reduced from 33 per cent to 7 per cent. The development of calcifications can be the most debilitating consequence of JDMS. It is our premise that JDMS is a distinct disease entity and that the increase in HLA-B8 and DR3 in JDMS suggests that genetic background may predispose to disease development. There are conflicting data concerning immunologic abnormalities in JDMS, but there appears to be impairment of natural killing and evidence of complement activation. Results of tests for ANA frequently are positive in JDMS, but Jo-1 antibody, found in some adults with PM, has not been found in JDMS. Most newly diagnosed JDMS patients have antibodies to coxsackie B that may be related to the pathogenesis of this disease. Specific pathologic findings of endothelial cells containing reticulotubular inclusions are associated with small vessel occlusion, subsequent obliteration, and increased factor VIII levels in clinically active disease. In addition to physical therapy, steroids are used most frequently, but other immunosuppressive agents and plasmapheresis have been tried in severely ill children. Rigorous evaluation of the efficacy of these modalities is needed.     

Diagnostic yield of parathyroid hormone testing in children evaluated for hypercalciuria

Hypercalciuria is a frequent cause of non-glomerular hematuria in pediatric patients. Because hypercalciuria can be secondary to primary hyperparathyroidism, measurement of serum parathyroid hormone (PTH) levels is often performed in children with this urinary abnormality. A retrospective chart review was performed to determine the diagnostic yield of PTH measurements when performed under these clinical circumstances. Over a 30-month period (January 1, 2001 to September 30, 2003), among 31 children who had a PTH determination, the level was elevated in 1 (3%) patient. Based on these findings and the serious nature of untreated primary hyperparathyroidism, serum PTH level should be measured in pediatric patients with newly diagnosed hypercalciuria.     

Epidemiologic and clinical characteristics of children with newly diagnosed inflammatory bowel disease in Wisconsin: a statewide population-based study

OBJECTIVE: To define epidemiologic and clinical characteristics of newly diagnosed pediatric inflammatory bowel disease (IBD) in a large population-based model. STUDY DESIGN: All pediatric gastroenterologists providing care for Wisconsin children voluntarily identified all new cases of IBD during a 2-year period. Demographic and clinical data were sent to a central registry prospectively for analysis. RESULTS: The incidence of IBD in Wisconsin children was 7.05 per 100,000, whereas the incidence for Crohn's disease was 4.56, more than twice the rate of ulcerative colitis (2.14). An equal IBD incidence occurred among all ethnic groups, and children from sparsely and densely populated counties were equally affected. The majority (89%) of new IBD diagnoses were nonfamilial. CONCLUSIONS: This study provides novel, prospective, and comprehensive information on pediatric IBD incidence within the United States. The surprisingly high incidence of pediatric IBD, the predominance of Crohn's disease over ulcerative colitis, the low frequency of patients with a family history, the equal distribution of IBD among all racial and ethnic groups, and the lack of a modulatory effect of urbanization on IBD incidence collectively suggest that the clinical spectrum of IBD is still evolving and point to environmental factors contributing to the pathogenesis.     

Serum levels of mannose-binding lectin and the risk of fever in neutropenia pediatric cancer patients

BACKGROUND: Fever in neutropenia (FN) is a frequent complication in pediatric oncology. Deficiency of mannose-binding lectin (MBL), an important component of innate immunity, is common due to genetic polymorphisms, but its impact on infections in oncologic patients is controversial. This study investigated whether MBL serum levels at cancer diagnosis are associated with the development of FN in pediatric cancer patients. PROCEDURE: Serum MBL was measured using ELISA. Frequency, duration, and cause of FN were assessed retrospectively. Association with MBL level was analyzed using uni- and multivariate Poisson regression taking into account both intensity and duration of chemotherapy. RESULTS: In 94 children, with a cumulative follow-up time of 81.7 years, 177 FN episodes were recorded. Patients with both very low MBL levels (<100 microg/L; risk ratio (RR), 1.93; 95% CI, 1.14-3.28; P = 0.014) and normal MBL levels (>or=1,000 microg/L; RR, P = 0.011) had significantly more frequent FN episodes than patients with low MBL levels (100-999 microg/L). Patients with very low MBL levels had significantly more episodes of FN with severe bacterial infection (bacteremia or pneumonia; RR, 4.49; 1.69 = 11.8; P = 0.003), while those with normal MBL levels had more FN episodes with no microbial etiology identified (RR, 1.85; 1.14 = 3.03; P = 0.014). CONCLUSIONS: Very low MBL levels are associated with more frequent FN episodes, mainly due to severe bacterial infections. The surprising finding that children with normal MBL levels had more frequent FN episodes than those with low MBL levels needs testing in prospective studies.     

Assessment of Nutritional Status in Children With Cancer and Effectiveness of Oral Nutritional Supplements

Malnutrition is a common consequence of cancer in children, but the most effective methods of nutrition intervention are under debate. We aimed to evaluate the nutritional status of children diagnosed with cancer, and to investigate the effect of oral nutritional supplements on anthropometric measurements, biochemical parameters, and outcome. A randomized clinical study of 45 newly diagnosed cancer patients was performed. Anthropometric and biochemical data and related factors were assessed at 0, 3, and 6 months after diagnosis. On initial anthropometric assessment, prevalence of malnutrition by weight or height was found to be lower as compared with body mass index (BMI), or weight for height (WFH), or arm anthropometry. Twenty-six of the patients (55%) received oral nutritional supplement. During the second 3 months after diagnosis, there was a statistically significant decrease in number of the patients with WFH <90th percentile and BMI <5th percentile (P = .003 and P = .04, respectively). Infectious complications occurred more frequently in malnourished patients during first 3 months, and survival of children who were malnourished at the 6th month was significantly lower than that of well-nourished children (P = .003). On laboratory assessment, serum prealbumin levels of the all subjects were below normal ranges, but no relation was found for serum prealbumin or albumin levels in patients who were malnourished or not at diagnosis. Nutritional intervention is necessary to promote normal development and increase functional status as a child receives intensive treatment. Protein- and energy-dense oral nutritional supplements are effective for preventing weight loss in malnourished children.     

39例儿童白血病合并败血症临床特点分析

目的探讨儿童急性白血病(AL)化疗后合并败血症的临床特点及降钙素原(PCT)在判断细菌类别中的作用。方法 2014年6月-2016年6月入住安徽医科大学第二附属医院儿科AL患儿39例,回顾性分析患儿化疗期间合并败血症的的发病因素、感染部位、病原学及不同PCT水平时的细菌类别。结果 39例患儿中急性淋巴细胞白血病28例,急性髓细胞白血病11例。15例患儿有明确感染部位,依次为呼吸道、口腔、肛周及皮肤。病原菌中革兰阳性球菌占53.8%(21/39),革兰阴性杆菌占43.6%(17/39),真菌占2.6%(1/39)。中性粒细胞计数0.5×109/L时败血症发病率显著提高(P0.05)。PCT高水平组革兰阴性杆菌检出率高,PCT低水平组革兰阳性球菌检出率高,不同菌种之间PCT水平存在显著性差异(P0.01)。结论 AL患儿合并败血症与粒细胞计数密切相关;PCT的结果对不同的细菌感染有提示作用,高水平的PCT提示革兰阴性杆菌感染可能性大。     

组蛋白去乙酰化酶7基因表达与儿童急性淋巴细胞白血病临床生物学特征及预后的相关性

目的：探讨组蛋白去乙酰化酶7（HDAC7）基因在急性淋巴细胞白血病（ALL）患儿骨髓标本中的表达及其与临床特征、早期治疗反应和长期预后的关系。 方法：纳入2010年1月1日至2012年12月30日首都医科大学附属北京儿童医院（我院）血液肿瘤中心收治的ALL患儿,排除入院前曾不规则使用激素和初诊骨髓样本中幼稚细胞比例＜70%的患儿。采用实时定量PCR（RT-qPCR）检测ALL患儿初诊骨髓标本中的HDAC7基因表达。以3例ALL患儿停药3年以上处于缓解状态的骨髓样本中的HDAC7表达水平作为对照,将ALL患儿分为高表达（≥1.0）组和低表达（＜1.0）组,分析组间临床生物学特征、微小残留病和无事件生存率（EFS）。 结果：共纳入236例ALL患儿,初诊骨髓标本中HDAC7基因表达水平为0.046~10.581,高表达组124例,低表达组112例。单因素分析显示HDAC7基因表达与初诊外周血WBC＜50×109·L-1 、免疫表型和融合基因类型相关,P均＜0.05;多因素分析显示WBC＜50×109·L-1 和融合基因类型是HDAC7表达的影响因素,P均＜0.05。中危ALL患儿中,HDAC7高表达组的预后好于低表达组,5年EFS分别为（91.0±3.5）%和（75.5±4.9）%,P=0.013,HDAC7表达是中危患儿的独立预后因素（P=0.013）,OR值和95%置信区间为1.26（1.31~9.51）。 结论：ALL患儿骨髓中HDAC7基因表达水平与临床和生物学特征相关;HDAC7基因低表达是中危患儿预后不良的独立危险因素。     

Living donor lobar lung transplantation: the pediatric experience

Living donor (LD) lobar lung transplantation is now an accepted alternative to cadaveric lung transplantation in selected patients with end-stage lung disease. This study reviews the Childrens Hospital Los Angeles LD experience of 17 patients (mean 13.2 +/- 2.7 yrs; range 9.3-18.5 yrs). 12 LD patients had end-stage cystic fibrosis, 4 had primary pulmonary hypertension, and 1 child had bronchiolitis obliterans. LD candidates must meet the same criteria as for cadaveric lung transplant candidates. Donor candidates are rigorously screened (physically and psychologically) prior to acceptance for lobectomy. LD patients receive the same triple immunosuppression regimen as our cadaveric recipients (prednisone, cyclosporine/FK506, and azathioprine/mycophenolate). Comparison of rejection episodes, incidence of bronchiolitis obliterans, pulmonary function tests, exercise stress tests, and cardiac catheterization data was made between LD and cadaveric lung transplantation (CL) pediatric recipients. Donor outcomes were also reviewed. In our pediatric program, the 1-year survival rate for LD recipients is currently 81%, which compares favorably with the ISHLT average of 70% for pediatric transplant patients. The incidence of rejection is about the same for LD and CL recipients, but the episodes are less severe for pediatric LD patients. There have been no histological cases of bronchiolitis obliterans syndrome in our LD recipients. Although there have been questions as to whether transplanted lobes can supply comparable pulmonary reserve to whole cadaveric lungs, the lung volumes (TLC and VC), expiratory flow rates, maximal exercise stress tests, and pulmonary artery pressures (no evidence of pulmonary hypertension) in LD patients are not significantly different to CL recipients in our institution. Besides pain from the thoracotomy, the donors have a decrease of 16% (right lower lobe donor) and 18% (left lower lobe donor) in their vital capacity. Otherwise, there have been no major complications to the donors and most have resumed their usual activities. Based on outcomes, pulmonary function tests, exercise stress tests, and hemodynamic studies as well as low donor morbidity, living donor double lobar lung transplantation is a viable alternative to cadaveric lung transplantation in selected pediatric patients with end-stage lung disease.     

Two Cases of Kawasaki Disease with Hidden Neuroblastoma

Neuroblastoma is the third most common pediatric cancer and can mimic many other diseases. However, clinicians need to be aware about the possibility of neuroblastoma, when other more obvious clinical conditions such as the Kawasaki disease (KD) are present. KD is an acute febrile vasculitis of childhood with coronary complications. There werea few reports for autopsy of KD patients with neuroblastoma. A 2-y-old girl was admitted for definite KD with five clinical signs. Her fever did not respond to retreatment with intravenous high dose immunoglobulin. Later a chest computerized tomography scan revealed a mass lesion in the right lower lung field, and ultimately diagnosed as neuroblastoma on the paravertebral area of thoracic spine levels T4 to T10. The second patient was a 3-y-old boy who was diagnosed as incomplete KD with left coronary ectasia. His fever also did not respond to retreatment with immunoglobulin, and the abdominal computerized tomography scan with biopsy confirmed neuroblastoma of the right adrenal gland. We report two patients with KD who were additionally diagnosed as having neuroblastoma.     

De novo malignances in pediatric organ transplant recipients

A study of 10813 types of cancer that occurred in 10151 organ transplant recipients showed that the pattern of malignancies that occurred in pediatric recipients was very different from the general pediatric population and from adult recipients. Tumors (527) occurred in 512 pediatric patients (aged 18 years or less), and 9639 adults developed 10286 neoplasms. Post-transplant lymphoproliferative disease (PTLD) was the predominant neoplasm in pediatric recipients and comprised 52% of all tumors compared with 15% in adult recipients. Eighty-four percent of PTLD in the former patients presented during childhood. There was a disproportionately high incidence among nonrenal allograft recipients compared with renal recipients (81% vs. 31% of all tumors). The second most common malignancy in pediatric patients was skin cancer (19% of tumors), but this was less frequent than in adult recipients, in whom it comprised 39% of neoplasms. Only 16 pediatric patients (16%) with skin cancers developed their tumors during childhood (6 had malignant melanomas), with an average time of appearance after transplantation of 126 months (range 5.5-292). Malignant melanomas were more common in pediatric than adult recipients (12% vs. 5% of skin cancers), as were lip cancers (23% vs. 12%). Spread to lymph nodes was also more common in pediatric than in adult recipients (9% vs. 6%). Sarcomas comprised 4% of tumors compared with 1% in adults. Carcinomas of the vulva and perineum also comprised 4% of tumors. Females outnumbered males in a ratio of 8.5:1. These tumors appeared beyond childhood at an average of 142 months (range 42-262 months) post-transplantation. Other cancers observed in recipients transplanted during childhood were thyroid carcinomas (15), Kaposi's sarcomas (15), carcinomas of the liver (13), leukemias (13), carcinomas of the cervix (10), brain tumors (7), renal carcinomas (7), ovarian carcinomas (5), and miscellaneous tumors (19). Of all 527 malignancies, 314 (60%) appeared during childhood and 213 (40%) manifested themselves between the ages of 19 and 40 years. By far the most common tumor diagnosed during childhood was PTLD, which comprised 230 of the 314 (73%) malignancies.     

Role of wireless capsule endoscopy in reclassifying inflammatory bowel disease in children

ObjectiveTo evaluate the role of wireless capsule endoscopy in identifying small bowel lesions in pediatric patients with newly diagnosed colonic inflammatory bowel disease (IBD) type unclassified (IBDU), and to assess whether capsule endoscopy findings result in altered patient management.MethodsTen pediatric patients recently diagnosed with IBDU through standard investigations were recruited from the pediatric gastroenterology clinic at McMaster Children's Hospital to undergo capsule endoscopy using the Pillcam SB^TM (Given Imaging) capsule. Findings consistent with a diagnosis of Crohn's disease required the identification of at least three ulcerations.ResultsThree out of ten patients had newly identified findings on capsule endoscopy that met criteria for Crohn's disease. Three more patients had findings suspicious for Crohn's disease, but failed to meet the diagnostic criteria. Three additional patients had findings most consistent with ulcerative colitis, and one had possible gastritis with a normal intestine. Findings from capsule endoscopy allowed for changes in the medical management of three patients. In all ten cases, capsule endoscopy allowed for a better characterization of the type and extent of disease. No adverse outcomes occurred in the present cohort.ConclusionThis prospective study reveals that wireless capsule endoscopy is feasible, valuable, and non-invasive, offering the ability to potentially better characterize newly diagnosed pediatric IBDU cases by identifying lesions in the small bowel and reclassifying these as Crohn's disease.     

Reduced intracellular T-helper 1 interferon-gamma in blood of newly diagnosed children with Crohn's disease and age-related changes in Th1/Th2 cytokine profiles

Abnormal cytokine production by T-helper 1 (Th1)/T-helper 2 (Th2) lymphocytes has been implicated in the pathogenesis of inflammatory bowel disease (IBD). Few studies have examined Th1/Th2 cytokine status in pediatric IBD patients, and results have been inconsistent. We used flow cytometric detection of intracellular IFN-gamma/IL-4 cytokine production to investigate CD4+, Th1, and Th2 cells in the peripheral blood of children with untreated, newly diagnosed Crohn's disease (CD) (n = 23) and matched healthy controls (n = 49). Th1 cytokine levels were lower in CD patients compared with controls (p = 0.006) and strongly correlated with levels of albumin and hematocrit (r = 0.51, p = 0.007 and r = 0.35, p = 0.052, respectively). An age-dependent increase in Th1 cells was observed (p < 0.0005); however, no correlation was found between age, clinical end points, %CD4+, or Th2 cell numbers. In conclusion, the Th1 cytokine levels in blood are lower in early onset CD patients than in healthy children and are directly associated with disease-related clinical parameters. In future studies of pediatric IBD patients, it will be critical to consider the effect of age and disease progression on cytokine status in intestinal mucosa and peripheral blood.     

Coagulation abnormalities in type 1 Gaucher disease in children

Gaucher disease is the most prevalent inherited lysosomal storage disorder caused by deficiency of β-glucocerebrosidase enzyme. Clinically, 3 forms of Gaucher disease are recognized, of which type 1 is the mild to moderately severe, slowly progressive, nonneuropathic form. Bleeding disorders in Gaucher disease are believed to be due to thrombocytopenia but there may be additional factors that influence coagulation and fibrinolysis in Gaucher disease patients. The aim of the present work was to study some coagulation parameters in the Egyptian children with type 1 Gaucher disease. Five newly diagnosed patients and another 5 patients on enzyme replacement therapy (ERT) were enrolled in the study. Their coagulation profile, including coagulation factors, was evaluated. The results showed that in newly diagnosed cases factors II and VII were deficient in 40%, factor V was deficient in 20%, and all the cases had low levels of serum fibrinogen. In patients on ERT, factors VII and VIII were deficient in 60%, factor XI was deficient in 40% and factors V, X, and XII were deficient in 20% of cases. In conclusion, Egyptian patients with type 1 Gaucher disease, whether newly diagnosed or receiving enzyme replacement therapy, experience coagulation factor abnormalities regardless the clinical expression of bleeding diathesis. This should be taken into consideration before these patients are subjected to surgery for, e.g., splenectomy, which is common in these patients.     

Serum vascular endothelial growth factor as a significant marker of treatment response in pediatric malignancies

The aim of this pilot study was to determine VEGF serum levels (S-VEGF) at diagnosis and at restaging in children diagnosed with cancer, and to investigate whether this parameter provides prognostic information for remission after induction therapy and response to treatment. S-VEGF levels of 35 consecutive pediatric patients with various types of cancer were assayed at diagnosis and at restaging. Levels of VEGF were determined using a commercially available ELISA anti-human VEGF immunoassay kit. Thirty-one children went into complete remission or had a very good partial response to first-line therapy; 4 patients developed tumor progression. At diagnosis average S-VEGF level was 495 pg/mL (range, 0.89--2220 pg/mL) and at restaging it decreased to 118.36 pg/mL (range, 7.44--487 pg/mL). (p=.0039). The 4 patients with tumor progression had increased S-VEGF levels at restaging. The comparison between the levels of S-VEGF at diagnosis and at restaging showed a significant difference for the patients who responded to treatment with decreased S-VEGF and the patients who developed tumor progression with increased S-VEGF (p=.0019). One child with metastatic Ewing sarcoma developed progressive disease after several weeks, with significantly progressively higher S-VEGF levels. One child with Hodgkin disease, who had a higher level at first restaging and developed progressive disease, responded to reinduction therapy and had a significantly lower level at the second restaging. The child with metastatic hepatoblastoma responded to first-line chemotherapy with concomitant decrease in S-VEGF and alpha-fetoprotein levels, but developed local recurrence with elevation in both parameters. Changes in S-VEGF levels correlated with response to treatment for most of the children diagnosed with cancer. This provides a rationale for exploring clinical interest in S-VEGF measurements of a larger group of children with malignancies, and using the test for clinical trials of antiangiogenic therapies.     

Serum C-reactive protein levels in patients with Kawasaki disease: from the results of nation-wide surveys of Kawasaki disease in Japan

Thirteen nation-wide epidemiological surveys of Kawasaki disease have been carried out successively since 1970 in Japan. In the latest survey, questionnaires on serum C-reactive protein (CRP) levels of the patients were included to clarify whether serum CRP levels could be available for the diagnosis and prediction of prognosis. A questionnaire form and diagnostic guidelines for Kawasaki disease were sent to all paediatric departments of hospitals with 100 or more beds throughout Japan, and information including maximal serum CRP levels was obtained on patients with Kawasaki disease diagnosed during the 2-year period from January 1993 to December 1994. Of the 11 458 patients diagnosed during the 2-year period, maximal serum CRP levels were reported in 11040 patients (96.4%). The values of maximal serum CRP were higher in the age groups < 6 months and >2 years. The mean value and the distribution of serum CRP levels in suspected cases were lowest among the three diagnostic categories and this difference among diagnostic categories was highly significant in the age groups 6M-1Y and 1-2Y. The proportion of patients with cardiac sequelae increased with serum CRP levels in each age group. The mean value and the distribution of serum CRP levels of the patients with cardiac sequelae was higher than those without it and this difference between cardiac prognoses was outstanding in the age groups 6M-1Y and <6M. The Receiver/Response Operating Characteristic (ROC) curve for maximal serum CRP levels in Kawasaki disease revealed that accuracy of maximal serum CRP levels for prediction of cardiac sequelae was highest in the age group 6M-1Y. A large-scale observation and analysis of serum CRP levels of the patients with Kawasaki disease revealed age-dependent relationships among maximal serum CRP levels, diagnostic categories and prognosis. Serum CRP levels may be helpful for the prediction of prognosis with the consideration of age.     

Pediatric common variable immunodeficiency: Immunologic and phenotypic associations with switched memory B cells

Yong PL, Orange JS, Sullivan KE. Pediatric common variable immunodeficiency: Immunologic and phenotypic associations with switched memory B cells.
Pediatr Allergy Immunol 2010: 21: 852–858.
© 2010 John Wiley & Sons A/S Recent studies suggest that patients with common variable immunodeficiency (CVID) and low numbers of switched memory B cells have lower IgG levels and higher rates of autoimmune disease, splenomegaly, and granulomatous disease; however, no prior literature has focused exclusively on pediatric cases. We examined the relationship between switched memory B cells and clinical and immunologic manifestations of CVID in a pediatric population. Forty‐five patients were evaluated. Patients were categorized as Group I (<5 switched memory B cells/ml, n = 24) or Group II (≥5 switched memory B cells/mL, n = 21). CD3⁺ T‐cell counts and CD19⁺ B‐cell levels were lower among Group I patients. Only those in Group I had meningitis, sepsis, bronchiectasis, granulomatous lung disease, autoimmune cytopenias, or hematologic malignancies. Segregation of pediatric patients into high risk (Group I) and average risk (Group II) may assist in targeting surveillance appropriately.