Monitoring Anti-HPA-1a Platelet Antibody Levels during Pregnancy Using the MAIPA Test

Anti-HPA-1a platelet antibody levels in pregnant women with a history of fetomaternal alloimmune thrombocytopenia (FMAIT) were monitored longitudinally using the monoclonal antibody immobilisation of platelet antigens (MAIPA) assay, in order to examine any variation in optical density (OD) readings obtained over the course of pregnancy and after delivery. Seven women were selected; 4 were studied retrospectively and 3 prospectively (the latter being treated with intravenous gammaglobulin; IVGG). Levels of anti-HPA-1a were measured at various intervals after delivery of the first affected infant, to post delivery of the following affected infant. A decrease in MAIPA OD was demonstrated in all patients during the course of these pregnancies. This assay is a useful tool for monitoring anti-HPA-1a in women with a history of infants affected with FMAIT. A maternal antibody 'resting' level prior to, or early in the first trimester, must be established for comparison.     

Brief communication The primate MHC contains sequences related to the fibroblast growth factor receptor gene family

We have cloned and sequenced a genomic region centromeric of the HLA-B locus from different MHC ancestral haplotypes. These haplotypes are associated with several diseases. The sequences were analyzed for coding potential and their relevance to disease associations were assessed with respect to the level of polymorphism. Analysis of sequences located approximately 25kb centromeric of HLA-B reveals the existence of fibroblast growth factor receptor related sequences. These sequences designated PERB1 (FGFR6 ) reveal 80% homology, at both nucleic acid and amino acid level, to the immunoglobulin domain 1 (Ig-1) of the human fibroblast growth factor receptor 3 ( FGFR3 ) gene. Amino acid comparison of the Ig-1 domain of PERB1 to those of other FGFR molecules indicates that PERB1 is more closely related to FGFR3 and FGFR5 than to FGFR1 , FGFR2 or FGFR4 . Genomic sequence analysis, however, reveals no consensus splice sites and indicates the existence of inframe premature stop codons in the putative coding sequences. The results suggest that these sequences may represent FGFR gene fragments existing within the central MHC. Sequence analysis of the Mhc in 6 chimpanzee and one orangutan indicates that the existence of PERB 1 predates the spe-ciation of the three species. The fact that the MHC contains a mixture of functional and nonfunctional (pseudo) genes suggests that a functional copy of PERB1 (FGFR6 ) may exist within or in close proximity to the MHC.     

Proximal femoral focal deficiency: radiologic analysis of 49 cases

Proximal femoral focal deficiency, an uncommon congenital anomaly, necessitates early radiologic classification for surgical planning and treatment. Objective radiographic criteria, including femoral length index, acetabular depth index, acetabular angle index, and shape of the proximal femur were determined in 49 patients before cartilaginous ossification of the femoral capital epiphysis; final classification was based on follow-up radiographs or findings at arthrography or surgery. These parameters were analyzed to determine the accuracy and contributions of each in classification. Correct classification into one of three groups was possible in 86% of cases with use of three of the parameters: femoral length index, acetabular depth index, and shape of the proximal femur. The acetabular angle was found to contribute insignificantly to classification. Magnetic resonance imaging, used in only one case, depicted the nonossified cartilaginous femoral capital epiphysis, thus obviating the need for invasive diagnostic procedures and facilitating early classification.     

44Sacral extracorporeal magnetic stimulation is an effective treatment for pelvic floor dyssynergia; Comparative study with biofeedback therapy

Background: Recent development of extracorporeal magnetic stimulation (ECMS) which uses current‐changing magnetic fields allows the induction of electrical stimulation in the desired deep tissue. Recent study showed the sacral nerve stimulation reduces corticoanal excitability that may play a functional role in anal continence mechanisms. Preliminary study shows that ECMS of sacral nerve can modify pelvic floor function and expel rectal balloon in patients with pelvic floor dyssynergia (PFD). Aims: To evaluate the effect of ECMS compared with biofeedback therapy (BF) in patients with PFD. Methods and Materials: Thirty‐eight patients who fulfilled Rome II criteria for PFD by colon transit time and anorectal function tests, were randomly treated with 8 sessions of ECMS (2/weeks; n = 19) at prone position or BF (2/weeks; n = 19) at sitting position. Stimulation parameters were set at 50–80% of maximum intensity, 10 and 50 Hz frequency, 3 s burst length with 3 and 6 s off using arm‐typed stimulator (BioCom‐1000, Mcube Co., Korea). Symptom scores for constipation with/without anorectal function test were repeatedly measured after each treatment. Response was defined as 50% or more decreased symptom score after treatment (partial response: 30–50%, poor: <30%). Results: Fifteen patients (age 49.1 ± 13.4 years, mean ± SD; 4 men) completed 8 session of BF and 14 patients (54.5 ± 17.6 years, 3 men) completed 8 session of ECMS. Four patients of BF group discontinued treatment due to unsatisfactory therapeutic effect (n = 1) and withdrew consent (n = 3) and 5 patients of ECMS group discontinued treatment because of same reasons (n = 1, 4). Total symptom scores were significantly decreased after treatment of 8 session in both treatment groups (13.4 ± 6.6 vs. 4.3 ± 4.0 for BF, p = 0.009; 14.9 ± 5.6 vs. 3.4 ± 4.0 for ECMS, p < 0.001). Bowel movements per week were also significantly increased after treatment in both groups (median 2 vs. 7 for BF, p = 0.035; median 2 vs. 7 for ECMS, p = 0.008). Thirteen out of 15 patients showed response in BF group and 12 out of 14 showed good response in ECMS group. No adverse effects in both groups. Conclusions: ECMS is as effective as BF for the treatment of PFD. Long‐term effect of ECMS for the patients with pelvic floor dyssynergia need to be evaluated in the near future.     

Balloon dilatation of the aortic valve: limited success and early restenosis.

Balloon dilatation of the aortic valve was attempted 16 times in 15 patients with severe aortic stenosis. None died but one had a transient stroke after the procedure. At dilatation the gradient across the aortic valve was reduced by greater than 30% in 69% of patients and the Gorlin valve area (calculated in 7/15 patients) increased by 30% in half. But a comparison of Doppler gradients measured before and one to two days after dilatation in 11 patients showed a greater than 30% reduction in the simultaneously measured gradient in only four. Doppler gradient was the most accurate predictor of symptomatic benefit and a fall in Doppler gradient persisted mainly in patients whose peak to peak gradient fell by at least 40% at the time of the procedure. Balloon dilatation of the aortic valve is a relatively safe procedure but it is less successful than previous reports suggest, perhaps because of early restenosis. Some forms of aortic stenosis may be more amenable to this procedure than others.