Clinical course and prognostic value of disease activity in the first two years in different subtypes of juvenile idiopathic arthritis

Objective

Juvenile idiopathic arthritis (JIA) is a heterogeneous disease involving chronic arthritis. The clinical course is characterized by a fluctuating pattern of active and inactive disease. We have described in detail the clinical course in different JIA subtypes during the first 2 years after diagnosis and studied its relationship to disease activity in the following years.

Methods

Detailed clinical data on different parameters describing the disease activity in sequential time periods covering the first 2 years after diagnosis were retrieved from the charts of 311 patients with JIA and compared between subtypes. In a cohort of 146 patients, the relation of these different clinical variables to the course of disease in the following 3 years was evaluated.

Results

The percentage of time with active disease in the first 2 years differed significantly between subtypes. In all subtypes, a broad spectrum of activity was observed. The time with active disease in the first 2 years was the most significant factor associated with the duration of active disease in the following years.

Conclusion

Different percentages of time with active disease have been observed between JIA subtypes in the first 2 years. The cumulative duration of activity varied widely within each subtype. Regarding the prognosis of the individual patient, the clinical course in the first 2 years appears to be predictive of the clinical course in the following years. Patients that have less time with active disease in the first 2 years are not likely to develop an unremitting clinical course later on.     

Breast feeding and the dietary habits of children in rural Somalia

Breast feeding and dietary habits were studied prospectively in a cohort of children under the age of five years in a rural Somali community. The median duration of breast feeding was 19.5 months. However, all the children also received cow's milk by cup from the first day of life and onwards. Energy supplements (mainly sugar and oil) as well as additional water were given daily from early infancy. Staples, protein-rich foods (beans and meat), vegetables and fruits were usually introduced when the children reached the age of 12-18 months. There was a seasonal variation with the lowest intake of protein-rich and vitamin-rich foods during the rains in May to June. Thus, there was a complete absence of exclusive breast feeding. Energy-reinforced cow's milk and human milk dominated the diet up to the age of one year. Staples were mixed with oil and supplemented with milk, thereby leading to a much higher energy density in the complementary food than is usually the case in African communities.     

High-dose 5-fluorouracil and leucovorin as second-line chemotherapy in patients with platinum-resistant epithelial ovarian cancer

A total of 14 patients with platinum-resistant advanced epithelial ovarian cancer were treated with a continuous infusion of high-dose 5-fluorouracil (5-FU, 1200 mg/m² per day) for 2 consecutive days weekly for 4 weeks and, thereafter, every 2 weeks in combination with a push injection of folinic acid (20 mg/m²) given just before 5-FU and after 24 h. No objective response was documented, and only five patients showed stable disease. The median survival was 6.5 months. There was minimal toxicity. This schedule of 5-FU in combination with folinic acid is not effective as second-line chemotherapy in advanced ovarian cancer.     

Intravitreal injection of adeno-associated viral vectors results in the transduction of different types of retinal neurons in neonatal and adult rats: a comparison with lentiviral vectors

Replication-deficient viral vectors encoding the marker gene green fluorescent protein (GFP) were injected into the vitreous of newborn, juvenile (P14), and adult rats. We tested two different types of modified virus: adeno-associated viral-2-GFP (AAV-GFP) and lentiviral-GFP vectors (LV-GFP). The extent of retinal cell transduction in different-aged animals was compared 7, 21, and 70 days after eye injections. At all postinjection times, LV-GFP transduction was mostly limited to pigment epithelium and cells in sclera and choroid. In contrast, transduction of large numbers of neural retinal cells was seen 21 and 70 days after AAV-GFP injections. AAV-GFP predominantly transduced neurons, although GFP-positive Müller cells were seen. All neuronal classes were labeled, but the extent of transduction for a given class varied depending on injection age. After P0 injections about 50% of transduced cells were photoreceptors and 30-40% were amacrine or bipolar cells. After adult injections 60-70% of transduced cells were retinal ganglion cells. In adults many GFP-positive retinal axons were traced through the optic nerve/tract and terminal arbors were visualized in central targets.     

A New South Wales Population-based Study of Stillbirths Weighing 2,500 g or More

Summary: This paper describes factors associated with singleton stillbirths weighing 2,500 g or more, born in 1987 and reported to the NSW Midwives' Data Collection (MDC), a statewide perinatal data collection system. A total of 136 singleton stillbirths in this weight range were notified to the MDC, representing one-quarter of all singleton stillbirths in NSW. MDC records on these stillbirths were linked with perinatal death registrations for 125 of the 136. The death registrations indicated that fetal death occurred during labour in 20 cases, prior to the onset of labour in 98, and at an unknown time in the remaining 7 cases. Placental complications, including haemorrhage and functional abnormalities of the placenta, were the most frequent group of conditions associated with the stillbirths, being recorded as the underlying cause of death in 42 (34%) of the 125 cases. Cord complications (such as cord compression or cord around the neck) were given as the underlying cause of death in 30 cases (24%). The death certificate diagnosis was confirmed by autopsy in only 27 of the 125 cases (22%), although autopsies may have been done on a further 45 cases (36%). An adequate explanation of the cause appeared to be lacking for many of the fetal deaths. This highlights the importance of a thorough and systematic investigation of stillbirths. A list of standard investigations to be carried out following a stillbirth is proposed.     

Comparison of Cytology and Cervkography in Screening a High Risk Australian Population for Cervical Human Papillomavirus and Cervical intraepithelial Neoplasia

Summary: Two different screening methods, the Papanicolaou (Pap) smear and cervigram were compared in screening 245 Sydney women over a 6-month period in 1988 at a city sexually transmitted diseases (STD) centre, for cervical human papillomavirus (HPV), cervical intraepithelial neoplasia (CIN) and cervical cancer. The Pap smear through the identification of cytologically abnormal cells correctly detected 54% of cases of histologically proven CIN and 39.2% of cases of HPV. The cervigram through the identification of acetowhite epithelium and/or abnormal vessels on the cervix correctly detected 64% of cases of histologically proven CIN and 70.6% of cases of HPV. However, when both tests were used together, 92% of CIN lesions and 82.4% of HPV lesions were correctly identified. Histology of a colposcopically directed biopsy was used as the 'gold standard'. The sensitivity and specificity of the Pap smear after correction for verification bias was 46% and 78% respectively, and for the cervigram was 49% and 60% respectively. Hence neither screening test appears adequate on its own, at least in an STD population.     

Clinical significance of anismus in encopresis

Treatments designed to relieve paradoxical contraction of the anal sphincters during defecation (anismus) have had limited success in children with encopresis. This has raised doubts as to the clinical relevance of this diagnosis in childhood as anorectal dysfunction. Our aim was to determine whether, in patients who had treatment-resistant encopresis, the presence of electromyographic anismus was associated with increased faecal retention. Sixty-eight children with soiling (mean age 8.7 ± 2.06 years) were assessed by clinical examination, abdominal radiography and then with anorectal manometry. Patients with electromyographic anismus (n= 32; 47%) had significantly increased radiographic rectal faecal retention and were significantly less likely to be able to defecate water-filled balloons. There were no significant differences in response to prior therapy, history of primary encopresis, behavioural adjustment or in sociodemographic data. Our results suggest that electromyographic anismus is associated with obstructed defecation and faecal retention.