Hematopoietic stem cell transplantation does not increase the risk of infection-related complications for pediatric patients with Hodgkin and non-Hodgkin lymphomas: A multicenter nationwide study.

BACKGROUND: Hodgkin (HL) and non-Hodgkin lymphoma (NHL) represent a spectrum of lymphoid malignancies that are often curable with currently applied treatment regimens; however, 15%-30% of lymphoma patients still suffer from relapsed or refractory (rel/ref) disease. Although hematopoietic stem cell transplantation (HSCT) improves outcomes of second-line therapy for lymphoma in childhood, the complication rates in this group of patients, especially infectious complications (IC), remain unclear. OBJECTIVE: The aim of this population-based cohort study was a retrospective analysis of incidence, epidemiology and profile of bacterial infections (BI), invasive fungal disease (IFD), and viral infections (VI) in primary or rel/ref lymphoma patients, both HL and NHL. PATIENTS AND METHODS: We subdivided lymphoma patients into three groups: patients with primary conventional chemotherapy/radiotherapy regimens (group A), patients with rel/ref lymphoma treated with second-line chemotherapy (group B), and rel/ref lymphoma patients who underwent HSCT (group C). The medical records of the patients were biannually reported by each pediatric oncology center, and the data were analyzed centrally. RESULTS: Within 637 patients with primary lymphoma, at least one IC was diagnosed in 255 (40.0%), among 52 patients with rel/ref lymphoma 24 (46.2%) ICs were observed, and in transplanted group, 28 (57.1%) out of 49 children were diagnosed with IC (P = .151). The distribution of etiology of IC differed between the patient groups (A, B, C), with a predominance of BI in group A (85.6% vs 72.0% and 47.9%, respectively), VI in group C (9% and 16.0% vs 46.6%, respectively), and IFD in group B (5.4% vs 12.0% vs 5.5%, respectively). Overall, 500 (68.0%) episodes of bacterial IC were diagnosed in the entire group. Apart from HL patients treated with chemotherapy, in all the other subgroups of patients Gram-positives were predominant. The rate of multidrug-resistant bacteria was high, especially for Gram-negatives (41.1% in group A, 62.5% in group B, and 84.6% in group C). The infection-related mortality was comparable for each group. CONCLUSIONS: The incidence of IC was comparable during first- and second-line chemotherapy and after HSCT, but their profile was different for primary or re/ref lymphoma and depended on the type of therapy.

Fibrinogen alpha and beta gene polymorphisms in pediatric stroke--case-control and family based study.

BACKGROUND/PURPOSE: Data on the role of the -455G > A polymorphism of the gene encoding ß fibrinogen subunit (FGB) and the Thr312Ala polymorphism of the gene for the α fibrinogen subunit (FGA) in childhood ischemic stroke are insufficient. Therefore the aim of the study was to evaluate a possible association between these two polymorphisms and arterial ischemic stroke. METHODS: The study group consisted of 85 children after ischemic stroke, 146 of their parents and 159 controls. Both polymorphisms were genotyped using the restriction fragment length polymorphism method. Two study designs were used: a case-control model and a family-based transmission-disequilibrium test. Statistica 7.1 and EpiInfo 6 softwares were used in all analyses. RESULTS: In the TDT test, a tendency to a higher transmission of the 312Ala allele of the FGA gene and the -455A allele of the FGB gene was observed, however, it was statistically non-significant. The frequencies of alleles and genotypes of both FGA and FGB genes polymorphisms did not differentiate children from both groups also in the case-control model. Additive or synergistic effects between FGA and FGB genes polymorphisms were not observed. CONCLUSION: An analysis of the results obtained in this study and a critical review of previously published data indicate that examined gene polymorphisms are not related to ischemic stroke in children.

The plasminogen activator inhibitor-1 gene polymorphism in determining the risk of pediatric ischemic stroke--case control and family-based study.

Pediatric ischemic stroke, though relatively rare, remains an important medical problem since 20-40% of patients have recurrent strokes and 50-85% of them suffer from long-term neurological deficits. Approximately 20-50% of the affected children have prothrombotic disorders, therefore upon looking for possible genetic causes of the disease we focused on the plasminogen activator inhibitor (PAI-1)--the major inhibitor of fibrinolysis. The aim of the present study was to investigate a possible association between the -675_-674insG PAI-1 gene polymorphism and pediatric ischemic stroke. The study population consisted of 343 individuals: 70 children with ischemic stroke, 140 their biological parents and 133 control children. The PAI-1 gene polymorphism was genotyped using the restriction fragment length polymorphism and was visualized by AgNO3 staining. The transmission/disequilibrium test showed exactly the same transmission of alleles from parents to the affected children (37:37). The case-control model also did not reveal any statistical significance in alleles and genotypes distribution between patients and control children. The obtained results suggest that the 4 G/5 G polymorphism of the PAI-I gene is not a risk factor of ischemic stroke in Polish children.

Failure to develop multiple sclerosis in patients with neurologic symptoms without objective evidence.

BACKGROUND: Many patients referred to multiple sclerosis (MS) centers with symptoms suggestive of MS are found to have normal neurologic examinations, normal or non-specific brain magnetic resonance imaging (MRI) scan findings, and normal cerebrospinal fluid (CSF). Persistent symptoms often lead to multiple consultations and repeated diagnostic investigations. We performed a study to evaluate the diagnostic utility of repeated evaluations in patients with normal initial assessments and persistent neurologic symptoms. METHODS: 143 patients were evaluated initially and 109 returned for a second evaluation after a mean interval of 4.4 years. RESULTS: All 143 patients had normal initial examinations, brain MRI scans, screening blood tests, and CSF studies. Spinal cord imaging was normal in all patients tested (cervical cord, n = 126; 88.1%; thoracic cord, n = 58; 40.6%). Evoked potential studies were abnormal in a small percentage of patients: visual evoked potentials, VEP (8.1%), somatosensory evoked potentials, SSEP (4.9%), and brainstem auditory evoked potentials, BAEP (2.8%). All follow-up patients (n = 109) had normal examinations and MRI scans. Repeat CSF studies (n = 35; 32.1%) and spinal cord imaging (cervical cord n = 57; 52.3%; thoracic cord n = 32; 29.4%) were normal in all follow-up patients tested. No patients at initial presentation or at follow-up fulfilled diagnostic criteria for MS. PATIENTS: and clinicians may be reassured that persistent neurologic symptoms in the absence of objective clinical evidence do not lead to the development of MS. Costly serial investigations should be carefully considered, particularly in the presence of normal neurologic examination at follow-up.

Xanthoma disseminatum of the central nervous system and cranium.

Xanthoma disseminatum is a rare normolipemic histiocytic disorder of non-Langerhans cell origin. It is a chronic systemic disease with a benign course, characterized by disseminated, yellow-orange-colored papules on the face, flexures, and mucosal membranes. We report 3 patients with xanthoma disseminatum, who presented primarily with central nervous system disease and a multitude of imaging findings throughout the craniospinal axis.

Diagnosis of normocalcemic hyperparathyroidism by oral calcium loading test.

We evaluated the oral calcium-loading test (OCLT) in diagnosing normocalcemic primary hyperparathyroidism. Calcium and PTH levels were measured before, 60, 120 and 180 min after oral 1 g of calcium gluconolactate administration in 102 consecutive females with high circulating PTH levels, and 25 controls. Patients were classified as follows: Group A, patients with a parathyroid adenoma identified by two imaging modalities. Sub-Group AO, hyperparathyroid patients [no.=13, mean age 59 yr (SD=10)] evaluated prior to parathyroidectomy. Sub-Group AH, non-operated hypercalcemic patients [no.=29, age 63 yr (SD=11)]. Sub-Group AN, normocalcemic non-operated women [no.=14, age 59 yr (SD=8)]. Group B, normocalcemic individuals [no.=46, age 58 yr (SD=11)] with negative parathyroid imaging. Group C, control patients [no.= 25, age 56 yr (SD=12)]. The concentrations of calcium and PTH overlapped in the normocalcemic groups during the OCLT. Product P, defined as circulating PTH nadir (pg/ml) x peak calcium concentration (mg/dl), better discriminated Sub-Group AN from Group B, AUC=0.98 (95% CI 0.95, 1.00) than did Ratio R, defined as relative PTH decline/relative calcium increment, AUC= 0.86 (95%CI 0.73, 0.99). Assuming normal threshold of Product P and Ratio R at 260 and 17 respectively, the combined parameters diagnose normocalcemic hyperparathyroid patients with 100% sensitivity and 87% specificity.

Macronutrient intake of black and white adolescent girls over 10 years: the NHLBI Growth and Health Study.

OBJECTIVE: To compare age-related changes in macronutrient and cholesterol intake between black and white girls, compare intakes with National Cholesterol Education Program (NCEP) recommendations, and examine sociodemographic associations with macronutrient intake. DESIGN: Cohort study with 3-day food records collected over 10 years. SUBJECTS: 2,379 girls, 1,166 white and 1,213 black, age 9 to 10 years at baseline, recruited from three geographic locations. Statistical Analysis Longitudinal generalized estimating equation (GEE) regression models examined the relationships of age, ethnicity, and sociodemographic factors with macronutrient and cholesterol intake and with percentage of girls meeting NCEP recommendations. RESULTS: Total and saturated fat intakes decreased with age, more in white girls than black girls, from 35.1% and 13.6% kcal at age 9 to 29.3% and 10.4% at age 19 for white girls and from 36.5% and 13.4% kcal at age 9 to 35.1% and 11.7% kcal at age 19 for black girls. Dietary cholesterol decreased with age, but decreased more in white girls than black girls (range 95 to 119 mg/1,000 kcal for white girls and 119 to 132 mg/1,000 kcal for black girls). Depending on age, 7% to 51% of white girls and 8% to 26% of black girls met NCEP recommendations for total fat (

Obesity development during adolescence in a biracial cohort: the NHLBI Growth and Health Study.

OBJECTIVE: The National Heart, Lung, and Blood Institute Growth and Health Study (NGHS) is a 10-year study to investigate the development of obesity in black and white girls during adolescence and its environmental and psychosocial correlates. The purpose of this report was to examine changes in the annual prevalence rates of overweight and obesity in the NGHS cohort from ages 9 to 19 years. PARTICIPANTS AND SETTING: A total of 2379 black and white girls, aged 9 to 10 years, were recruited from schools in Richmond, California, and Cincinnati, Ohio, and from families enrolled in a health maintenance organization in the Washington, DC area. Participant eligibility was limited to girls and their parents who declared themselves as being either black or white and who lived in racially concordant households. DESIGN AND STATISTICAL ANALYSIS: The NGHS is a multicenter prospective study of a biracial cohort followed annually from ages 9 to 10 years through 18 to 19 years. The prevalence of overweight and obesity was based on age-specific > or =85th and > or =95th percentile values, respectively, for body mass index based on the 1960-1965 National Health Examination Survey reference population. MAIN OUTCOME MEASURES: The main outcome measures were body mass index (weight in kilograms divided by height in meters, squared) and proportions of girls who were "overweight" and "obese" by age and race. RESULTS: The prevalence of overweight was 37% higher in blacks as compared with whites (30.6% vs 22.4%) even by age 9. The rate of overweight almost doubled in both groups during the 10-year period. By age 19, the rate of overweight was 56.9% in black and 41.3%, in white girls. The prevalence of obesity was 17.7% in black and 7.7% in white girls at 9 years old, and the rates also doubled during the study period. CONCLUSIONS: The doubling in the prevalence of overweight and obesity during adolescence in black and white NGHS girls was surprising. By age 19, more than half of black girls were overweight and more than one third were obese. Almost half of white girls were overweight and almost 1 of 5 girls were obese. These findings should sound an alarm for all primary care physicians and public health professionals to take heed of what is happening to our youth.

Decline in physical activity in black girls and white girls during adolescence.

BACKGROUND: Physical activity declines during adolescence, but the underlying reasons remain unknown. METHODS: We prospectively followed 1213 black girls and 1166 white girls enrolled in the National Heart, Lung, and Blood Institute Growth and Health Study from the ages of 9 or 10 to the ages of 18 or 19 years. We used a validated questionnaire to measure leisure-time physical activity on the basis of metabolic equivalents (MET) for reported activities and their frequency in MET-times per week; a higher score indicated greater activity. RESULTS: The respective median activity scores for black girls and white girls were 27.3 and 30.8 MET-times per week at base line and declined to 0 and 11.0 by year 10 of the study (a 100 percent decline for black girls and a 64 percent decline for white girls, P<0.001). By the age of 16 or 17 years, 56 percent of the black girls and 31 percent of the white girls reported no habitual leisure-time activity. Lower levels of parental education were associated with greater decline in activity for white girls at both younger ages (P<0.001) and older ages (P=0.005); for black girls, this association was seen only at the older ages (P=0.04). Pregnancy was associated with decline in activity among black girls (P<0.001) but not among white girls, whereas cigarette smoking was associated with decline in activity among white girls (P<0.001). A higher body-mass index was associated with greater decline in activity among girls of both races (P< or =0.05). CONCLUSIONS: Substantial declines in physical activity occur during adolescence in girls and are greater in black girls than in white girls. Some determinants of this decline, such as higher body-mass index, pregnancy, and smoking, may be modifiable.

Selectin glycoprotein ligands.

Lectin selectins and their counter-receptors participate in discontinuous cell-cell interactions concurrent with leukocyte tethering and rolling on endothelium, which, in consequence, leads to leukocyte penetration to lymphatic organs and generation of inflammation sites. Counter-receptors are glycoproteins in which carbohydrate units, the direct selectin ligands, are built into the polypeptide framework. In this review, the distribution, structure and function of the main ligands and counter-receptors for P-, L- and E-selectins known so far, have been discussed. The common biosynthetic pathway of sialyl-Lewis x and sulpho-sialyl-Lewis x determinants of selectin ligands has been described.

Tc-99m MIBI scintigraphic detection of metastatic insular thyroid carcinoma.

Thallium-201 and, recently, Tc-99m MIBI have been used in conjunction with I-131 scintigraphy for follow-up of patients with well-differentiated thyroid cancer. Insular carcinoma of the thyroid is a fairly aggressive thyroid neoplasm that is believed to arise from follicular cells and usually concentrates I-131. The authors report a patient with recurrent insular thyroid carcinoma in whom bilateral adrenal and lung metastatic lesions developed 3 years after ablative I-131 therapy for cervical lymph node and skeletal metastases. Tc-99m MIBI planar and SPECT images demonstrated these new lesions better than pretherapy I-131 scintigraphy and affords an imaging technique for post-I-131 therapy follow-up that does not require withholding thyroid hormone suppression.

Composition of proteoglycans from rabbit aorta in the experimentally induced atherosclerosis.

Proteoglycans (PG) from normal and atherosclerotic rabbits aortas were extracted with 4 M guanidine hydrochloride and digested with collagenase in the presence of protease inhibitors. The contents of uronic acid and hexosamine from PG fractions purified by isopycnic CsCl gradient ultracentrifugation under associative and dissociative conditions were significantly higher in the atherosclerotic aortas (up to 40%) than in the control tissue. The uronic acid/protein ratio increased from 0.7 to 1.3 in the monomers PG fraction of atherosclerotic aortas. Chromatographic separation and electrophoretic analysis of PG monomers indicated the presence of three different subfractions PGI, PGII and PGIII in both groups of animals. The uronic acid/protein ratio in PGI from experimental aorta was increased whereas this ratio in PGIII was decreased compared to contrast tissue. The observed increase of sugar component in the core proteins suggests their over glycosylation.