Juvenile systemic lupus erythematosus in Turkey: demographic, clinical and laboratory features with disease activity and outcome.

Objectives This paper aims to assess in a retrospective fashion the clinical and laboratory features, severity and outcome of juvenile systemic lupus erythematosus (jSLE) from a referral center in Turkey. Methods We have included all jSLE patients ( n = 92) diagnosed according to the revised American College of Rheumatology 1997 criteria between January 2004 and January 2017. Results The most prevalent clinical feature in our cohort was mucocutaneous manifestations (97.8%), followed by constitutional (81.5%), hematological (59.8%) and musculoskeletal manifestations (56.5%). Renal involvement was observed in 38% ( n = 35) of the patients, whereas biopsy-proven lupus nephritis was detected in 29.3% ( n = 27) of the cohort. Neurologic involvement was seen in 15 (16.3%) individuals. Among the patients positive for anticardiolipin IgM and/or IgG ( n = 11, 12%), only three developed antiphospholipid antibody syndrome. The mean SLEDAI-2K scores at disease onset (10.5 ± 4.8) showed a substantial decrease at last visit (4.3 ± 4.6). One-quarter of the patients (26.1%, n = 24) had damage according to the PedSDI criteria with a mean score of 0.45 ± 1.0 (range 0-7). When the PedSDI damage items were evaluated individually, growth failure was the most frequent damage criterion ( n = 6), followed by seizure ( n = 5). Two patients died during the designated study period of end-stage renal disease. The five-year and 10-year survival rate of our cohort was 100% and 94.4%, respectively. Conclusions Given the lower frequency of nephritis and central nervous system disease and lower basal disease activity and damage scores, we could conclude that children with jSLE in Turkey have a more favorable course compared to Asian and African American children, as expected from Caucasian ethnicity.

Dissection of the abdominal aorta in a child with Takayasu's arteritis.

Takayasu's arteritis is a chronic inflammatory disease that primarily involves the aorta and its main branches. Varying degrees of narrowing, occlusion, or dilatation develop in the involved vessel segments. However, dissection of the aorta is quite rare in this disease, and it may develop particularly after angioplasty. We report a very rare case of Takayasu's arteritis with dissection of the abdominal aorta just distal to the origin of the inferior mesenteric artery in a 9-year-old girl. She was treated conservatively with close follow-up. At the end of 1 year's follow-up, the dissection of the aorta did not show progression, and new lesions were not identified. To our knowledge, this patient is the youngest child presented with arterial dissection as the initial manifestation of the disease.

Hepatitis B vaccination in children with juvenile idiopathic arthritis.

OBJECTIVES: To evaluate the responsiveness of children with juvenile idiopathic arthritis (JIA) to hepatitis B vaccination and to determine the most useful vaccination schedule. METHODS: 39 children with JIA were enrolled in the study; all were in remission and negative to serological testing for hepatitis B surface antigen (HbsAg). The control group consisted of 41 healthy children. There were two different vaccination schedules: group I was vaccinated at 0, 1, and 3 months; group II was vaccinated at 0, 1, and 6 months. Positive responsiveness to the vaccine was defined as an anti-hepatitis B antibody titre above 10 mIU/ml. RESULTS: All the children except one with systemic JIA developed an antibody response. None of the JIA patients experienced a flare up or clinical deterioration related to the vaccination. The antibody levels in children with JIA were significantly lower than in the healthy controls. Comparison of the antibody levels between the two vaccination schedules showed no statistical difference in the controls; in the JIA subjects the group II schedule resulted in a trend to a greater response than the group I schedule (p<0.07). Vaccine responsiveness was not influenced by either methotrexate or prednisolone treatment. CONCLUSIONS: Children with JIA had an adequate response to hepatitis B vaccination and the response was not affected by immunosuppressive treatment. A vaccination schedule at 0, 1, and 6 months seems to be preferable to 0, 1, and 3 months.

Patterns of first-year survival among infants with selected congenital anomalies in Texas, 1995-1997.

BACKGROUND: Few registry-based studies have investigated survival among infants with congenital anomalies. We conducted a registry-based study to examine patterns and probability of survival during the first year of life among infants with selected congenital anomalies. METHODS: Data from the Texas Birth Defects Monitoring Division were merged with linked birth-infant death files for 2,774 infants born January 1, 1995 to December 31, 1997, with at least 1 of 23 common anomalies. Deaths before the first birthday were assessed from infant death files. Kaplan-Meier was used to estimate first-year survival; first-year survival was assessed for specific anomalies and by the number of life-threatening anomalies. RESULTS: Overall, 80.8% of infants with these 23 anomalies survived the first year of life. We observed the highest survival rates for infants with gastroschisis (92.9%, 95% CI = 86.8, 96.3), trisomy 21 (92.3%, 95% CI = 89.5, 94.4) or cleft lip with or without cleft palate (87.6%, 95% CI = 84.0, 90.5). Infants with intermediate survival rates included those with microcephaly (79.7%; 95% CI = 73.6, 84.6), tetralogy of Fallot (75.0%; 95% CI = 65.5, 82.2), or with diaphragmatic hernia (72.8%; 95% CI = 61.8, 81.2). As expected, all infants with anencephaly and almost all infants with trisomy 13 or trisomy 18 died during the first year of life. First-year survival declined as the number of co-occurring life-threatening anomalies increased. CONCLUSIONS: Overall, first-year survival for infants with congenital anomalies was high. Additional population-based studies are needed to quantify improvements in first-year survival.

Infecundity and consumption of polychlorinated biphenyl-contaminated fish.

Biologic capacity for reproduction, or fecundity, may be threatened by environmental contaminants, especially compounds capable of disrupting endocrine pathways. Telephone interviews that focused on reproductive events were conducted with female members of the New York State Angler Cohort Study who became pregnant between 1991 and 1993 and who reported known time to pregnancy (N = 895; 73%). Consumption of polychlorinated biphenyl-contaminated Lake Ontario sportfish and other factors were ascertained in 1991. The authors classified the women as follows: (a) fecund (time to pregnancy < or =12 cycles; n = 723); (b) having resolved infecundity (time to pregnancy > 12 cycles; n = 81); or (c) having unresolved infecundity (time to pregnancy > 12 cycles without pregnancy; n = 94). Adjusted odds ratios for duration of fish consumption for both resolved and unresolved infecundity were elevated (1.46 and 1.19, respectively), although confidence intervals included unity. Frequency of recent fish consumption was associated with an increased risk for select categories, although confidence intervals included one.

Growth and IGF-1 levels of children with familial Mediterranean fever on colchicine treatment.

OBJECTIVE: To evaluate growth process and insulin like growth factor-1 (IGF-1) levels in children with familial Mediterranean fever (FMF). METHODS: This prospective study group consisted of 51 children with FMF under colchicine therapy (20 boys, 31 girls) and 42 healthy children (22 boys, 20 girls). All children were prepubertal. Bone ages and IGF-1 levels were determined in all cases. Height velocity (HV), height standard deviation score (SDS), target height and target height SDS were calculated. RESULTS: There was no statistical difference in age, HSDS, target height SDS and bone ages between healthy and diseased subjects. HV of children with FMF did not differ significantly from the control group. There was no statistical difference in age, HSDS, target height SDS and bone ages between healthy and FMF subjects. HV of children with FMF did not differ significantly from the control group. There was no significant correlation between disease duration, number of attacks, erythrocyte sedimentation rate and HV, HSDS and IGF-1 levels of FMF patients. There was positive correlation between cumulative colchicine dose and HV (r = 0.29). CONCLUSION: Growth and IGF-1 levels of children with FMF do not differ from their healthy peers. However, there was positive correlation between HV and cumulative colchicine dose. This study suggests that colchicine not only has no adverse influence on growth, but more by suppressing disease activity and inflammation it has an enhancing role.

Early-stage breast cancer treatment among medically underserved women diagnosed in a national screening program, 1992-1995.

BACKGROUND: Little research has been conducted on the breast cancer treatment of low income, underserved women. This study was designed to describe initial treatment of breast cancer among low-income women diagnosed through federally funded screening programs in Detroit, Michigan, and the states of New Mexico and California; and to compare the treatment received by program women with early-stage breast cancer with that of all women diagnosed in those regions. METHODS: Data from the three screening programs were linked with cancer registry data from the corresponding geographic areas. All women diagnosed between 1992 and 1995 through the state-based screening programs and all women contemporaneously diagnosed with breast cancer in the three regions were studied. Descriptive analyses were done of the proportion of women with breast cancer receiving treatment; the proportion of early-stage breast cancer (stage I or II) cases treated with breast-conserving surgery, and the proportion treated with mastectomy; and among women with breast-conserving surgery, the proportion receiving radiation therapy. Logistic regression models controlled for age and stage at diagnosis, race or ethnicity and geographic region. RESULTS: Less than 2% of program women diagnosed with breast cancer received no treatment. More than two of five women with early-stage breast cancer underwent breast-conserving surgery, with 72% of these women receiving radiation therapy. Multivariate regression analysis revealed that women with stage IIA or IIB breast cancer had lower odds of undergoing breast-conserving surgery than women with stage I (0.51 [95% CI = 0.30-0.87] and 0.36 [95% CI = 0.19-0.70], respectively). Women over age 65 and those with incompletely staged cancer had the lowest odds for receiving radiation therapy after breast-conserving surgery (0.29 [95% CI = 0.09-0.99] and 0.14 [95% CI = 0.03-0.72], respectively). Women diagnosed through the screening programs had odds of undergoing breast-conserving surgery similar to those of all women in the regions (1.11 [95% CI= 0.89-1.39]). CONCLUSIONS: Treatment patterns for women diagnosed with early-stage breast cancer through three state-based screening programs appear to have been similar to those reported in the literature. In addition, their treatment appears to have been similar to that of other women during the same time period.

Mass screening in low-income populations: the challenges of securing diagnostic and treatment services in a national cancer screening program.

Funding for many mass screening programs for low-income and uninsured populations provides resources for screening tests, yet only rarely does it provide coverage for necessary follow-up diagnostic and treatment services. The National Breast and Cervical Cancer Early Detection Program (NBCCEDP), a federally funded initiative that provides cancer screening to low-income uninsured and underinsured women, covers some diagnostic follow-up tests and no treatment services. We conducted in-depth case studies of seven state programs participating in the NBCCEDP to investigate the strategies and approaches being used to secure diagnostic and treatment services. The results suggest that the program relies on a patchwork of resources--at state and local levels--to provide diagnostic and treatment services. This includes a number of components of local safety nets, all of which are unstable and have uncertain futures. Public health disease-screening initiatives need to reconsider the feasibility of continued reliance on case-by-case appeals to the local safety net for diagnostic follow-up and treatment services.

Ethics, society and occupational reproductive hazards: fetal consequences?

Occupational exposures to agents such as lead can adversely affect fetal outcome. With the US Supreme Court decision in the Johnson Controls case regarding the legality of employer's fetal protection policies, increasing responsibility lies with women to make decisions regarding exposure to potentially hazardous agents in the workplace. What are some of the ethical issues that society needs to address with respect to this responsibility? This paper considers the ethical principles of respect for autonomy, nonmaleficence, beneficence, justice, and self interest from the perspectives of the potential mother and those concerned with protecting the fetus. Since the fetus cannot make decisions, the potential role of society in protecting fetal rights in the context of workplace exposures is explored.

Parental consumption of contaminated sport fish from Lake Ontario and predicted fecundability.

Wildlife studies suggest that consumption of contaminated fish from the Great Lakes may expose humans to polychlorinated biphenyls and persistent chlorinated pesticides. To assess whether time to pregnancy or fecundability is affected, we conducted a telephone survey in 1993 with female members of the New York State Angler Cohort Study who were considering pregnancy between 1991 and 1994 (N = 2,445). Among the 1,234 (50%) women who became pregnant, 895 (73%) had a known time to pregnancy. Upon enrollment into the cohort in 1991, both partners reported duration and frequency of Lake Ontario sport fish consumption. We estimated lifetime exposure to polychlorinated biphenyls from recent consumption and used a discrete-time analog of Cox proportional hazards analysis to estimate conditional fecundability ratios and 95% confidence intervals (CIs) for fish consumption among couples with complete exposure data who discontinued birth control to become pregnant (N = 575). Maternal consumption of fish for 3-6 years was associated with reduced fecundability (fecundability ratio = 0.75; 95% CI = 0.59-0.91), as was more than a monthly fish meal in 1991 (fecundability ratio = 0.73; 95% CI = 0.54-0.98). Our findings suggest that maternal but not paternal consumption of contaminated fish may reduce fecundability among couples attempting pregnancy.

Paternal Lake Ontario fish consumption and risk of conception delay, New York State Angler Cohort.

The aquatic ecosystems of the Great Lakes are contaminated with a variety of compounds, some of which are considered reproductive toxicants. Few studies of paternal fish consumption and reproductive endpoints have been undertaken and serve as the impetus for study. Standardized telephone interviews were conducted with 2445 female members of the New York State Angler Cohort (82% response) to update reproductive profiles and to ascertain specific information on time-to-pregnancy (TTP). The study sample includes women with a known TTP and paternal fish consumption data (n=785). Conception delay was defined as more than 12 cycles of unprotected intercourse to achieve pregnancy. Paternal fish consumption was assessed by three measures: frequency of Lake Ontario sport fish meals in 1991, numbers of years eating fish, and estimated PCB exposure from fish consumption. Adjusted ORs for number of fish meals, based on logistic regression, ranged from 0.69 to 0.80; from 0.61 to 0.82 for number of years eating fish; and from 0.44 to 1.14 for quartiles of estimated PCB exposure from fish consumption. All confidence intervals included one. These findings suggest that, based on paternal self-reports, Lake Ontario fish consumption does not increase the risk of conception delay.

Strategies for follow-up and treatment services in state breast and cervical cancer screening programs.

This report describes strategies used to provide diagnostic follow-up and treatment services to low-income women screened through the National Breast and Cervical Cancer Early Detection Program.

Screening workers for genetic hypersusceptibility: potential ethical, legal, and social implications from the Human Genome Project.

One of the potential outcomes of the Human Genome Project will be the ability to identify individuals who are at increased risk of adverse health effects following exposure to hazardous substances in the workplace because of genetic hypersusceptibility. The ability to identify such individuals is likely to lead to the inclusion of genetic screening in worker protection programs. This technology and its applications will have a number of potential ethical, legal, and social implications. In this commentary, the authors examine five broad topics relating to the use of screening for genetic hypersusceptibility in the workplace: (1) issues of risk; (2) the rationale and legal basis for screening; (3) the privacy concerns of workers; (4) the confidentiality of test results; and (5) potential discrimination. The authors close by suggesting some guidelines for developing policies regarding genetic screening.

Pesticide exposures and fetal death: a review of the epidemiologic literature.

Despite considerable concern regarding the effects on reproductive outcome of exposures to pesticides, convincing evidence for the developmental toxicity of occupational and environmental pesticide exposure in humans is lacking. In this comprehensive review of the English language epidemiologic literature, we summarize studies that have examined potential associations between fetal deaths (both spontaneous abortions and stillbirths) and specific pesticides, as well as maternal and paternal employment in occupations with potential for exposure. While many of the epidemiologic studies to date suffer from methodologic problems, the data are suggestive of increased risks of fetal deaths associated with pesticides in general and maternal employment in the agricultural industry. There is a clear need for epidemiologic research that focuses on specific pesticide products or chemical families, with improved exposure assessment. The potential role of solvents in developmental toxicity associated with pesticide use by both males and females should also be considered.

Hypercalciuria and hematuria in juvenile rheumatoid arthritis.

OBJECTIVE: To investigate the frequency of hypercalciuria and the relationship between hypercalciuria and hematuria in patients with juvenile rheumatoid arthritis (JRA). METHODS: Twenty-eight children with JRA were studied, as well as 10 patients with acute arthritis unrelated to JRA and 14 healthy children as control groups. Cases with urinary calcium excretion (UCE) >4 mg/kg/day were considered hypercalciuric. Urinalysis was performed for detecting hematuria in all cases. RESULTS: UCE was 4.19 +/- 2.9 mg/kg/day in patients with JRA, 1.94 +/- 1.57 mg/kg/day in children with acute arthritis, and 2.0 +/- 1.45 mg/kg/day in healthy children. UCE was significantly higher in JRA compared with the other study groups. Of the 28 patients with JRA, 13 (46.4%) had hypercalciuria and 6 (21.4%) had hematuria. UCE was significantly higher in hematuric patients with JRA than in those with no hematuria (p<0.05). UCE in patients with JRA without hematuria was also higher than the UCE values detected in the disease and healthy control groups (p<0.05). CONCLUSION: Hypercalciuria is a frequent finding in patients with JRA [13/28 (46.4%)] and should be considered during the investigation of hematuria in patients with JRA.

Erythromelalgia associated with hypertension and leukocytoclastic vasculitis in a child.

Erythromelalgia is an acrocyanotic rheumatic disease presenting with erythema, and pain and a burning sensation in the hands and feet; it is rarely encountered during childhood. Hot or warm conditions may precipitate pain and erythema in the extremities and the symptoms may regress upon the application of cold water. The disease is usually secondary to other systemic diseases in adults. On the other hand, it is idiopathic in children. This article describes a case of erythromelalgia presenting with leukocytoclastic vasculitis and hypertension in a 7-year-old child who responded to therapy with prednisolone and phenoxybenzamine.

Consumption of contaminated sport fish from Lake Ontario and time-to-pregnancy. New York State Angler Cohort.

Sport fish from the Great Lakes are contaminated with halogenated organics, heavy metals, and pesticides, thus serving as a route of exposure for fish-consuming populations. These contaminants are recognized reproductive toxicants in animals; few human studies are available. The purpose of this study was to assess consumption of contaminated fish in relation to time-to-pregnancy (TTP) among women in the New York State Angler Cohort. In 1993, structured telephone interviews were conducted with 2,445 of 2,977 (82%) female cohort members aged 18-40 years who stated upon enrollment in the cohort in 1991 that they were considering pregnancy over the next 3 years. Among the 1,234 women who reported being pregnant, 874 (71%) had a known TTP and comprise the study sample. After descriptive analyses, log transformations of the number of years of fish consumption (duration) and TTP were performed and entered into multiple regression models that also included other covariates. Duration of fish consumption and maternal age accounted for only a small percentage of the explained variance in TTP (R2 = 0.005), even after the analysis was restricted to women who reported eating fish (R2 = 0.006). All beta coefficients were positive. These preliminary findings do not support an adverse effect of contaminated fish consumption on TTP.