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Showing papers by "Children's Memorial Hospital published in 1997"


Journal ArticleDOI
TL;DR: The National Cooperative Inner‐City Asthma Study (NCICAS) is a multicenter study designed to determine factors that contribute to asthma morbidity in children in the inner cities.
Abstract: Asthma morbidity has increased dramatically in the past decade, especially among poor and minority children in the inner cities. The National Cooperative Inner-City Asthma Study (NCICAS) is a multicenter study designed to determine factors that contribute to asthma morbidity in children in the inner cities. A total of 1,528 children with asthma, ages 4 to 9 years old, were enrolled in a broad-based epidemiologic investigation of factors which were thought to be related to asthma morbidity. Baseline assessment included morbidity, allergy evaluation, adherence and access to care, home visits, and pulmonary function. Interval assessments were conducted at 3, 6, and 9 months after the baseline evaluations. Over the one-year period, 83% of the children had no hospitalizations and 3.6% had two or more. The children averaged 3 to 3.5 days of wheeze for each of the four two-week recall periods. The pattern of skin test sensitivity differed from other populations in that positive reactions to cockroach were higher (35%) and positive reactions to house dust mite were lower (31%). Caretakers reported smoking in 39% of households of children with asthma, and cotinine/creatinine ratios exceeded 30 ng/mg in 48% of the sample. High exposure (> 40 ppb) to nitrogen dioxide was found in 24% of homes. Although the majority of children had insurance coverage, 53% of study participants found it difficult to get follow-up asthma care. The data demonstrate that symptoms are frequent but do not result in hospitalization in the majority of children. These data indicate a number of areas which are potential contributors to the asthma morbidity in this population, such as environmental factors, lack of access to care, and adherence to treatment. Interventions to reduce asthma morbidity are more likely to be successful if they address the many different asthma risks found in the inner cities.

367 citations



Journal ArticleDOI
TL;DR: It is concluded that 2 gm/kg IVGG combined with at least 30 to 50 mg/kg per day aspirin provides maximum protection against development of coronary abnormalities after KD.

312 citations


Journal ArticleDOI
TL;DR: Early surgical management of CAF is a safe and effective treatment resulting in 100% survival and 100% closure rate and transcatheter embolization is a reasonable alternative to standard surgical closure in only a very small, select group of patients.

310 citations


Journal ArticleDOI
TL;DR: Psychosocial characteristics in 1,528 4–9‐year‐old asthmatic urban children and their caretakers are presented.
Abstract: Previous research has demonstrated a significant reciprocal relationship between psychosocial factors and asthma morbidity in children. The National Cooperative Inner-City Asthma Study investigated both asthma-specific and non-specific psychosocial variables, including asthma knowledge beliefs and management behavior, caregiver and child adjustment, life stress, and social support. This article presents these psychosocial characteristics in 1,528 4–9-year-old asthmatic urban children and their caretakers. Caretakers demonstrated considerable asthma knowledge, averaging 84% correct responses on the Asthma Information Quiz. However, respondents provided less than one helpful response for each hypothetical problem situation involving asthma care, and most respondents had more than one undesirable response, indicating a potentially dangerous or maladaptive action. Both adults and children reported multiple caretakers responsible for asthma management (adult report: average 3.4, including the child); in addition, children rated their responsibility for self-care significantly higher than did adults. Scores on the Child Behavior Checklist indicated increased problems compared to normative samples (57.3 vs. 50, respectively), and 35% of children met the criteria for problems of clinical severity. On the Brief Symptom Inventory, adults reported elevated levels of psychological distress (56.02 vs norm of 50); 50% of caretakers had symptoms of clinical severity. Caretakers also experienced an average of 8.13 undesirable life events in the 12 months preceding the baseline interview. These findings suggest that limited asthma problem-solving skills, multiple asthma managers, child and adult adjustment problems, and high levels of life stress are significant concerns for this group and may place the inner-city children in this study population at increased risk for problems related to adherence to asthma management regimens and for asthma morbidity. Pediatr. Pulmonol. 1997;24:263–276. © 1997 Wiley-Liss, Inc.

181 citations


Journal ArticleDOI
TL;DR: Measurements of serum mullerian inhibiting substance can be used to determine testicular status in prepubertal children with nonpalpable gonads, thus differentiating anorchia from undescended testes in boys with bilateral cryptorchidism and serving as a measure of testicular integrity in children with intersexual anomalies.
Abstract: Background Mullerian inhibiting substance, produced constitutively by the prepubertal testes, promotes involution of the mullerian ducts during normal male sexual differentiation. In children with virilization and nonpalpable gonads, only those with testicular tissue should have detectable serum concentrations of mullerian inhibiting substance. Methods We measured serum mullerian inhibiting substance in 65 children with virilization at birth and nonpalpable gonads (age at diagnosis, 2 days to 11 years) and serum testosterone in 54 of them either after the administration of human chorionic gonadotropin or during the physiologic rise in testosterone that occurs in normal infants. Results The mean (±SD) serum mullerian inhibiting substance concentration in the 17 children with no testicular tissue was 0.7±0.5 ng per milliliter, as compared with 37.5±39.6 ng per milliliter in the 48 children with testes (P<0.001). In the latter group, the mean values in the 14 children with abnormal testes and the 34 with nor...

180 citations


Journal ArticleDOI
TL;DR: The authors extended the drug sampling period to more clearly define acetaminophen pharmacokinetics in children having surgery and recommend that the initial dose should be approximately 40 mg/kg.
Abstract: Background:Rectal acetaminophen is often administered during operation to provide supplemental analgesia or antipyresis in children. Recent studies examining current dose guidelines are limited by short sampling times. The authors extended the drug sampling period to more clearly define acetaminophe

173 citations


Journal Article
TL;DR: The prominence of IgA plasma cells in the vascular infiltrate in the early, acute, and subacute stages of KS indicates an Ag-driven immune response to an etiologic agent with a respiratory or gastrointestinal portal of entry and it is speculated that this unusual immune response is integral to the pathogenesis of the illness.
Abstract: The etiology and pathogenesis of Kawasaki syndrome (KS) remain unknown. Clinical and epidemiologic features of KS are consistent with an infectious cause. To search for an etiologic agent of KS, a phage cDNA expression library was constructed from the aorto-iliac junction of a patient with fatal acute KS and screened with convalescent KS serum followed by anti-human Ig. Unexpectedly, 0.1% of the clones in the library react with anti-human Ig, indicating the presence of many Ig-producing B lymphocytes in the vasculitic tissue. To confirm this finding and to determine the isotypes produced, frozen vascular tissue sections from the patient and paraffin sections from coronary arteries from six additional patients with fatal acute or subacute KS were incubated with Abs to Ig isotypes. Histopathology of the tissues revealed the presence of many plasma cells in the inflammatory infiltrate. IgA was the predominant isotype produced in vascular tissue in all seven KS patients. IgM- and IgG-producing cells were less often detected. We conclude that there is a marked plasma cell response within the vasculitic tissue in KS, with unusual IgA production locally in this nonlymphoid, nonmucosal tissue. We suggest that the prominence of IgA plasma cells in the vascular infiltrate in the early, acute, and subacute stages of KS indicates an Ag-driven immune response to an etiologic agent with a respiratory or gastrointestinal portal of entry and speculate that this unusual immune response is integral to the pathogenesis of the illness.

162 citations


Journal ArticleDOI
TL;DR: The design and methods of the broad‐based initial Phase I epidemiologic investigation of the National Cooperative Inner‐City Asthma Study, established to identify and then intervene on those factors which are related to asthma morbidity among children in the inner‐city, are described.
Abstract: The National Cooperative Inner-City Asthma Study (NCICAS) was established to identify and then intervene on those factors which are related to asthma morbidity among children in the inner-city. This paper describes the design and methods of the broad-based initial Phase I epidemiologic investigation. Eight research centers enrolled 1,528 children, 4 to 9 years of age, from English- or Spanish-speaking families, all of whom resided in major metropolitan inner-city areas. The protocol included an eligibility assessment and an extensive baseline visit, during which symptom data, such as wheezing, lost sleep, changes in activities of daily living, inpatient admissions, and emergency department and clinic visits were collected. A comprehensive medical history for each child was taken and adherence to the medical regimen was assessed. Access, as well as barriers, to the medical system were addressed by a series of questions including the location, availability, and consistency of treatment for asthma attacks, follow-up care, and primary care. The psychological health of the caretaker and of the child was also measured. Asthma knowledge of the child and caretaker was determined. Sensitization to allergens was assessed by skin-prick allergen testing and exposure to cigarette smoke and the home environment were assessed by questionnaire. For more than a third of the families, in-home visits were conducted with dust sample allergen collection and documentation of the home environment, such as the presence of pets and evidence of smoking, mildew, and roaches. Urine specimens were collected to measure passive smoke exposure by cotinine assays, blood samples were drawn for banking, and children age 6 to 9 years were given spirometric lung function assessment. At 3, 6 and 9 months following the baseline assessment, telephone interviews were conducted to ask about the child's symptoms, unscheduled emergency department or clinic visits, and hospitalizations. At this time, peak flow measurements with 2-week diary symptom records were collected.

149 citations


Journal ArticleDOI
TL;DR: Persistent neurologic deficits are characteristic for children with neuroblastoma and opsoclonus-myoclonu and treatment with chemotherapy may improve the neurologic outcome.
Abstract: A retrospective data collection was performed on 29 children diagnosed with neuroblastoma and opsoclonus-myoclonus between 1983-1993 from Pediatric Oncology Group institutions. The aim was to describe neurologic outcome in children with neuroblastoma and opsoclonus-myoclonus. Age at diagnosis ranged from one month to 4 years (median age, 18 months). The duration of opsoclonus-myoclonus symptoms prior to the diagnosis of neuroblastoma ranged from 6 days to 17 months (median duration, 6 weeks). There was a prevalence of low stage disease according to the POG staging system: stage A (n = 18), stage B (n = 3), stage C (n = 7), stage D (n = 1). There was a predominance of paraspinal primary tumors. There was no case of Nmyc amplification (0/17), and 2/8 cases were diploid. Treatment for neuroblastoma consisted of surgery alone in 19/29 (18 stage A, 1 stage C in thorax), and surgery plus chemotherapy in 10/ 29. No patient received radiotherapy. Treatment for opsoclonus-myoclonus ranged varied. Six children received no treatment for opsoclonus-myoclonus. The following agents were used ACTH (n = 14), prednisone (n = 12), IV IgG (n = 6), immuran (n = 2), depakote (n = 1), and inderal (n = 1). Eighteen of 29 children (62%) had resolution of opsoclonus-myoclonus symptoms. The range of time for recovery was a few days to 3 years. However the majority recovered over several months. Twenty of 29 children (69%) had persistent neurologic deficits including speech delay, cognitive deficits, motor delay, and behavioral problems. Of the 9 children who had complete recovery of opsoclonus-myoclonus without neurologic sequelae, age at diagnosis and duration of symptoms were not different from the entire group. Interestingly, 6/9 children with complete recovery received chemotherapy as part of their treatment. In conclusion, persistent neurologic deficits are characteristic for children with neuroblastoma and opsoclonus-myoclonus. Treatment with chemotherapy may improve the neurologic outcome.

148 citations


Journal ArticleDOI
TL;DR: Familiarity with the embryology of duplication and an awareness of the potential pitfalls of excretory urography and voiding cystourethrographY will foster an understanding of the varied appearances and associated complications of both incomplete and complete duplication.
Abstract: Duplication of the ureters is a common anomaly and is frequently encountered by radiologists. Duplication may be either complete or incomplete and is often accompanied by various complications. Incomplete duplication is most often associated with ureteroureteral reflux or ureteropelvic junction obstruction of the lower pole of the kidney. Complete duplication is most often associated with vesicoureteral reflux, ectopic ureterocele, or ectopic ureteral insertion, all of which are more common in girls than in boys. Vesicoureteral reflux affects the lower pole and can be outgrown, as in nonduplicated systems. Ectopic ureterocele and ectopic ureteral insertion affect the upper pole. The ectopic ureterocele produces a filling defect of variable size in the bladder; it can be identified with contrast material studies or ultrasound. Ectopic ureters may function poorly, be difficult to detect, and cause enuresis in girls. A fourth complication, ureteropelvic junction obstruction, occurs only in the lower pole and is seen in more boys than girls. Anatomic variants or anomalies as well as suboptimal imaging techniques can either simulate or obscure duplication, making diagnosis difficult. However, familiarity with the embryology of duplication and an awareness of the potential pitfalls of excretory urography and voiding cystourethrography will foster an understanding of the varied appearances and associated complications of both incomplete and complete duplication.

Journal ArticleDOI
TL;DR: Percutaneous injection of sodium tetradecyl sulfate, either alone or before surgery, is a safe and effective method of managing symptomatic hemangiomas and venous malformations in infants, children, and young adults.
Abstract: The purpose of this study was to evaluate the efficacy and safety of percutaneous sclerosis therapy using sodium tetradecyl sulfate for treatment of symptomatic hemangiomas and venous malformations in infants, children, and young adults.Fifty-two sclerosis procedures were performed in 21 patients who were 13 months to 24 years old. Six of these patients had hemangiomas, and the remaining 15 patients had venous malformations. Sodium tetradecyl sulfate was injected solely percutaneously in 49 procedures, by both percutaneous and arterial routes in two procedures, and intraarterially only in one procedure. Thirteen patients had sclerosis therapy alone, and eight patients had sclerosis therapy followed by surgery within 48 hr.Sclerosis therapy alone or sclerosis therapy followed by surgery was judged beneficial in 18 (86%) of 21 patients, including five of the six patients who had hemangiomas and 13 of the 15 patients who had venous malformations. Two patients had an equivocal response to the therapy, and one...


Journal ArticleDOI
19 Mar 1997-JAMA
TL;DR: The results of this comprehensive office-based investigation suggest that with adequately trained personnel, negative OIA test results may not always need to be routinely confirmed with BAP cultures.
Abstract: Objective. —To compare the accuracy of an optical immunoassay (OIA) for the rapid diagnosis of group A streptococcal pharyngitis with blood agar plate (BAP) culture. Design. —Blinded comparison with criterion standard. Setting. —A total of 6 private pediatricians' offices, 3 in Connecticut and 3 in Chicago, III. Patients. —A total of 2113 consecutive patients with acute pharyngitis, 983 in Connecticut and 1130 in Chicago. Main Outcome Measures. —The sensitivities and specificities of OIA and BAP culture (both performed and interpreted in the office) were determined using a research laboratory's interpretation of a combination of BAP culture and Todd-Hewitt broth (THB) culture of transport tube pledget as criterion standard. Results. —Among patients in Connecticut, the sensitivities of the OIA and BAP culture were 94% and 89%, respectively ( P =.004), while the specificities were 96% and 99%, respectively ( P =.001). Among patients in Chicago, the sensitivities of the OIA and BAP culture were 79% and 72%, respectively ( P P P P Conclusions. —The results of this comprehensive office-based investigation suggest that with adequately trained personnel, negative OIA test results may not always need to be routinely confirmed with BAP cultures.

Journal ArticleDOI
TL;DR: It is concluded that parental birth weights are important risk factors for LBW in both African Americans and whites.
Abstract: The authors used a transgenerational data set of Illinois vital records to ascertain the relation between parental birth weights and infant birth weight. The infant generation consisted of all African Americans and whites born in Illinois during 1989-1991. The parent generation included the mothers and fathers who were also born in Illinois between 1956 and 1975. In the infant cohort, the rate of low birth weight (LBW) (<2,500 g) was 11.7% for African Americans (n = 15,287) versus 5.0% for whites (n = 117,708) (relative risk (RR) = 2.3, 95% confidence interval (CI) 2.2-2.5). For African Americans, the LBW rate was 17.9% among those born to LBW mothers (n = 1,943) compared with 10.8% among those born to non-LBW mothers (n = 13,344) (RR = 1.8, 95% CI 1.6-1.9). For whites, the LBW rate was 8.5% among those born to LBW mothers (n = 2,174) compared with 4.8% among those born to non-LBW mothers (n = 115,534) (RR = 1.7, 95% CI 1.6-2.0). A weaker association was observed between paternal birth weight and infant birth weight. The authors conclude that parental birth weights are important risk factors for LBW in both African Americans and whites.

Journal ArticleDOI
TL;DR: Use of a prognostic staging system based on tumor cell ploidy, augmented with the NMYC gene copy number and serum level of lactate dehydrogenase, would very likely improve the treatment of infants with unresectable or metastatic neuroblastoma.
Abstract: Background: Current staging systems for unresectable or metastatic neuroblastoma do not reliably predict responses to chemotherapy in infants under 1 year of age. Previous studies have indicated that the DNA content, or ploidy, of malignant neuroblasts can discriminate between good and poor responders in this group of patients, but the clinical utility of ploidy assessment has remained in question. Purpose: We tested, in a prospective nonrandomized study, the hypothesis that neuroblast ploidy could be used as the sole guide for treatment selection in infants with unresectable or metastatic tumors and could differentiate between those who would respond to our previous standard regimen and those who would benefit from an immediate switch to another therapy. Methods: One hundred seventy-seven infants were enrolled in this trial. Five of these infants were subsequently excluded (two ineligible, two lacking ploidy information, and one protocol violation); therefore, 172 patients were included in the study. One hundred thirty infants with hyperdiploid tumors (DNA index >1.0; better prognosis in retrospective studies) were treated with a well-tolerated regimen of cyclophosphamide (150 mg/m 2 per day orally or intravenously on days 1-7) and doxorubicin (35 mg/m 2 intravenously on day 8). Forty-two infants with diploid tumors (DNA index =1.0; worse prognosis in retrospective studies) received cisplatin (90 mg/m 2 intravenously on day 1) and teniposide (100 mg/ m 2 intravenously on day 3) after an initial course of cyclophosphamide plus doxorubicin. Statistical end points were response and long-term survival. In addition, we assessed within each ploidy group (i.e., patients with hyperdiploid tumors and those with diploid tumors) the prognostic significance of NMYC gene copy number, tumor stage, and other variables commonly measured in this disease. Results: Of the 127 assessable infants with hyperdiploid tumors, 115 (91%) had complete responses—85 after receiving five courses of cyclophosphamide plus doxorubicin and 30 after receiving further therapy including cisplatin plus teniposide. The 3-year survival estimate for the entire hyperdiploid group was 94% (95% confidence interval [CI] = 89%-98%). Nineteen (46%) of 41 assessable infants with diploid tumors were complete responders. The overall 3-year survival estimate for this group was 55% (95% CI = 39%-70%). Prognostic factor analysis indicated that NMYC gene amplification and an elevated serum lactate dehydrogenase level were statistically significant markers of higher risk disease within the diploid group (two-sided P values of .005 and .003, respectively). Only NMYC was predictive in the hyperdiploid group (P = .003). Conclusion: Use of a prognostic staging system based on tumor cell ploidy, augmented with the NMYC gene copy number and serum level of lactate dehydrogenase, would very likely improve the treatment of infants with unresectable or metastatic neuroblastoma. Patients with diploid tumors characterized by an amplified NMYC locus represent a particularly unfavorable risk group that may benefit from innovative new therapies. [J Natl Cancer Inst 1997;89:373-80]

Journal ArticleDOI
TL;DR: The uniform distribution and striking amount of EBV in the tumor cells demonstrates that EBV is capable of infecting smooth muscle cells and that these cells support EBV replication, and suggests thatEBV infection occurs at an early stage of tumor development.
Abstract: Smooth muscle tumors (leiomyosarcomas) are the second most prevalent malignancy of children with the acquired immunodeficiency syndrome (AIDS). We have investigated the tumors, plasma, and peripheral white blood cells of eight children with AIDS with smooth muscle tumors for evidence of tumor association with human immunodeficiency virus (HIV) and Epstein-Barr virus (EBV). Very low levels of HIV were found in the tumors of the AIDS patients, probably resulting from blood-borne carriage of virus. These smooth muscle tumors had very high quantities of EBV in all the tumor cells by in situ hybridization, with an average of 4.5 EBV genomes per cell by quantitative polymerase chain reaction amplification. Increased amounts of EBV were found in the peripheral blood cells of two AIDS patients before the time of tumor diagnosis. EBV clonality studies demonstrated different monoclonal EBV infection of two separate colonic tumors from one patient, and dual or mixed monoclonal EBV infection in another patient. The muscle cells of leiomyomas and leiomyosarcomas of patients with AIDS demonstrated prominent staining with antibodies to the EBV receptor. The uniform distribution and striking amount of EBV in the tumor cells demonstrates that EBV is capable of infecting smooth muscle cells and that these cells support EBV replication. Clonal EBV proliferation suggests that EBV infection occurs at an early stage of tumor development. These findings indicate that EBV has a causal role in the oncogenesis of leiomyosarcomas of patients with AIDS.

Journal ArticleDOI
TL;DR: It is demonstrated that changes in EGF receptor glycosylation by GnT-III transfection reduces the number of the active receptors in U373 MG cells and that this change results in change in the cellular response to EGF.

Journal ArticleDOI
TL;DR: The bone-scintigraphy classification preceded the radiographic head-at-risk signs by an average of 3 months, allowing earlier treatment and correlated with subsequent femoral head involvement.
Abstract: We reviewed 44 consecutive patients treated for Legg-Calve-Perthes disease who underwent serial technetium 99m diphosphonate bone scintigraphy. The average follow-up was 4.4 years. The bone-scintigraphy classification characterizes the A pathway by early lateral column formation not seen in the B pathway. Pathway A had 20 hips. The average age at presentation was 6.1 years. At last follow-up, this group had an average Mose classification of 1.2 and Catterall score of 2.4, without any patient having "head-at-risk" signs or requiring operative treatment. Pathway B had 20 hips. The average age at presentation was 5.8 years. At last follow-up, this group had an average Mose classification of 5.2, a Catterall score of 3.5, and 18 patients had head-at-risk signs, with 11 requiring operative treatment. Our bone-scintigraphy classification preceded the radiographic head-at-risk signs by an average of 3 months, allowing earlier treatment and correlated with subsequent femoral head involvement.

Journal ArticleDOI
TL;DR: In pubertal children, sexual maturation, BMI, dietary intervention (in girls), and dietary cholesterol (in boys) were significant in determining LDL-C, and Sexual maturation was the factor associated with the greatest difference in HDL-C.
Abstract: Background The Dietary Intervention Study in Children (DISC) is a multicenter, randomized, controlled clinical trial designed to examine the efficacy and safety of a dietary intervention to reduce serum LDL cholesterol (LDL-C) in children with elevated LDL-C. Methods and Results The effects of dietary intake of fat and cholesterol and of sexual maturation and body mass index (BMI) on LDL-C were examined in a 3-year longitudinal study of 663 boys and girls (age 8 to 10 years at baseline) with elevated LDL-C levels. Multiple linear regression was used to predict LDL-C at 3 years. For boys, LDL-C decreased by 0.018 mmol/L for each 10 mg/4.2 MJ decrease in dietary cholesterol ( P <.05). For girls, no single nutrient was significant in the model, but a treatment group effect was evident ( P <.05). In both sexes, BMI at 3 years and LDL-C at baseline were significant and positive predictors of LDL-C levels. In boys, the average LDL-C level was 0.603 mmol/L lower at Tanner stage 4+ than at Tanner stage 1 ( P <.01). In girls, the average LDL-C level was 0.274 mmol/L lower at Tanner stage 4+ than at Tanner stage 1 ( P <.05). Conclusions In pubertal children, sexual maturation, BMI, dietary intervention (in girls), and dietary cholesterol (in boys) were significant in determining LDL-C. Sexual maturation was the factor associated with the greatest difference in LDL-C. Clinicians screening for dyslipidemia or following dyslipidemic children should be aware of the powerful effects of pubertal change on measurements of lipoproteins.

Journal Article
TL;DR: It is concluded that a community's violent crime rate is associated with intrauterine growth retardation among infants born to African-American women.
Abstract: OBJECTIVES: To ascertain the extent to which residence in violent communities is an independent risk factor for adverse pregnancy outcomes among impoverished (census tract median family income Language: en

Journal ArticleDOI
TL;DR: These results identify important amino acids in the central core domain of transglutaminases and show that the C-terminal end influences the structural and functional integrity of TGK.

Journal ArticleDOI
TL;DR: Factors found to be statistically significantly associated with an increased risk of LCH included: maternal urinary tract infection during the index pregnancy, feeding problems during infancy, and blood transfusions during infancy.
Abstract: There is little information available regarding epidemiologic risk factors for Langerhans cell histiocytosis (LCH). An exploratory investigation was conducted using information obtained from parents of 177 cases of LCH diagnosed before 21 years of age (median 2 years). Utilizing data available from the Children's Cancer Group, LCH cases were compared to two matched control groups including 614 patients diagnosed with a variety of childhood cancers and 318 community controls. Questionnaire data included information on demographics, prenatal and perinatal factors, complications in the neonatal period, environmental exposures, family medical history, and childhood exposures. Factors found to be statistically significantly associated with an increased risk of LCH included: maternal urinary tract infection during the index pregnancy, feeding problems during infancy, and blood transfusions during infancy. Use of supplemental vitamins was associated with a significantly decreased risk of LCH. Results from this exploratory study provide a basis for speculation on potential etiologic risk factors for LCH. Future epidemiologic investigations of LCH need to consider the presenting disease characteristics in assessing possible etiologic factors.

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TL;DR: The data suggest that this 50 kDa glycosylated protein from the membrane fraction of the small intestine is a putative receptor for ITF in the gastrointestinal tract.

Journal ArticleDOI
TL;DR: Although delayed release of the spinal cord, following the onset of a neurological deficit, may reverse some lost function, it is unlikely to restore bladder and bowel function.

Journal ArticleDOI
TL;DR: Twenty patients underwent craniomaxillofacial fixation procedures with the use of the LactoSorb (Walter Lorenz Surgical, Jacksonville, FL) resorbable plating system.
Abstract: Twenty patients underwent craniomaxillofacial fixation procedures with the use of the LactoSorb (Walter Lorenz Surgical, Jacksonville, FL) resorbable plating system. The patients ranged in age from 4 months to 67 years. No significant problems from the use of this plating system were noted. The resorbable plating system offers several distinct advantages over traditional metallic systems.

Journal ArticleDOI
TL;DR: A preferential PAF-R expression in the ileum is found, suggesting a role for PAF in intestinal inflammation and a complex regulation of PAR-R gene expression in vivo involving a network of various pro-inflammatory mediators.
Abstract: A competitive PCR assay was developed to quantify platelet-activating factor (PAF) receptor (PAF-R) transcripts in rat tissues using a synthetic RNA as a competitor. We found PAF-R mRNA constitutively expressed in the eight organs tested, with the ileum containing the highest concentration [(3.49+/-0.15) x 10(7) molecules/microg of RNA]. Significant but lower levels were also detected in the jejunum, spleen, lungs, kidneys, heart, stomach and liver. Furthermore we defined the regulatory role of inflammatory mediators in ileal PAF-R gene expression using a rat model of intestinal injury induced by PAF or lipopolysaccharide (LPS). Injection of LPS or low-dose PAF resulted in a marked increase in ileal PAF-R mRNA within 30 min. The up-regulation on PAF-R elicited by PAF was biphasic, peaking first at 90 min, then again at 6 h. In contrast, LPS elicited a weak monophasic response. The second phase of PAF-R mRNA increase after PAF administration was completely abolished by WEB 2170, a PAF antagonist, and partially inhibited by antitumour necrosis factor (TNF) antibody. These observations indicate the involvement of endogenous PAF and TNF in this event. In conclusion, we found: (a) preferential PAF-R expression in the ileum, suggesting a role for PAF in intestinal inflammation; (b) induction of PAF-R expression in vivo by its own agonist; (c) a complex regulation of PAR-R gene expression in vivo involving a network of various pro-inflammatory mediators.

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TL;DR: Calcium oxalate saturation in infancy and childhood is established to allow a more specific prediction of the risk of (recurrent) stone disease and is recommended for diagnostic purposes as well as for therapy control.

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TL;DR: Familiarity of familial autoimmune disease, exposure to environmental factors, or elevated antibody titers to T gondii, HSV, or CVB are not increased in juvenile DM, but young patients have elevated enteroviral titers, as do young geographic controls.
Abstract: Objective. To determine, in a case-control study, if patients with new-onset juvenile dermatomyositis (juvenile DM) have increased symptoms prior to onset, exposure to certain environmental conditions, frequency of familial autoimmune diseases, or antibody titers, compared with 2 control groups. Methods. A structured interview with the families of 80 children with juvenile DM, 40 children with juvenile rheumatoid arthritis (JRA), or 23 healthy children, from the same geographic area as the children with juvenile DM, was conducted. All children's sera were tested for antibody to Toxoplasma gondii, herpes simplex virus (HSV), or coxsackievirus B (CVB). Results. A high proportion of children with juvenile DM had constitutional symptoms 3 months before the disease-onset date (P = 0.013 versus control children). Children with JRA had more relatives with rheumatoid arthritis (P = 0.0001) and pernicious anemia (P = 0.003) than did children with juvenile DM or healthy children. Among children ⩽7 years of age, elevated enteroviral titers were more frequent in those with juvenile DM (81%) and in healthy controls (90%) than in those with JRA (64%), suggesting a common environmental exposure. Titers to T gondii, HSV, or CVB 1-6 were normal. Conclusion. Frequencies of familial autoimmune disease, exposure to environmental factors, or elevated antibody titers to T gondii, HSV, or CVB are not increased in juvenile DM. Children with juvenile DM do have symptoms of illness 3 months before the disease-onset date, and young patients have elevated enteroviral titers, as do young geographic controls.

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TL;DR: This is the first report of a whole blood procedure adapted for routine clinical use which is able to detect abnormal CD40L expression in XHIM patients and carriers.