Human epididymis protein 4 (HE4) levels inversely correlate with lung function improvement (delta FEV1) in cystic fibrosis patients receiving ivacaftor treatment

Background

We have recently shown that human epididymis protein 4 (HE4) levels correlate with the severity of cystic fibrosis (CF) lung disease. However, there are no data on how HE4 levels alter in patients receiving CFTR modulating therapy.

EQUAL FREQUENCY OF TEL/AML1 ACUTE LYMPHOBLASTIC LEUKEMIA IN CHILDREN WITH AND WITHOUT DOWN SYNDROME

Constitutional trisomy 21 is the most prominent predisposing factor to childhood leukemia, whereas the t(12;21)(p13;q22) with its molecular genetic counterpart, the TEL/AML1 fusion gene, is the most common acquired chromosomal rearrangement in childhood B-cell precursor (BCP) acute lymphoblastic leukemia (ALL). Thus, it was somewhat surprising that according to the currently available literature the incidence of TEL/AML1+ BCP ALL is extremely low in patients with Down syndrome (DS). To further investigate this issue in a population-based fashion, the authors retrospectively assessed the number of DS patients with a TEL/AML1+ ALL in two consecutive Austrian ALL multicenter trials. Accordingly, they were able to analyze 8 of 10 individuals with DS and a BCP ALL, including 2 who suffered from a TEL/AML1+ leukemia. Based on this observation we concluded that individuals with a constitutional trisomy 21 may have the similar likelihood to develop a TEL/AML1+ leukemia as BCP ALL patients without this specific predisposing factor.     

Results of Transplanting Developing Third Molars as Part of Orthodontic Space Management

PATIENTS AND METHOD: The aim of the present study was to contribute to a more accurate indication and better integration of developing third molar transplantation into orthodontic space management, using the results gleaned from the authors' own pool of patients. For this purpose, a clinical and radiographic examination of 61 transplanted developing molars in 57 patients was carried out after an average of 3.3 years. RESULTS: The overall success rate was 85%. Failure was due in 10% of cases to impaired periodontal healing (inflammatory root resorption, ankylosis, or increased pocket probing depths) and in 5% of cases to insufficient or arrested postoperative root development accompanied by increased transplant mobility. The incidence of postoperative cessation of further root development was significantly higher (p = 0.011) in transplants at early developmental stages, which also had a negative impact on the final root length, the crown-root ratio and the mobility values of these transplants. CONCLUSIONS: The transplantation of developing third molars should be given stronger consideration in treatment plans for orthodontic space management although it represents a real alternative to orthodontic space closure only in exceptional cases. However, unlike implantological or prosthodontic treatment, it offers an opportunity to replace a missing or non-retainable tooth with a patient's own vital and fully functional tooth, provided the appropriate transplant is selected.     

Mechanical function of intermediate filaments in arteries of different size examined using desmin deficient mice

Protein composition and mechanical function of intermediate filaments were examined in arteries of different sizes using desmin deficient mice (Des−/−) and their wild-type controls (Des+/+). Using SDS-PAGE gels and Western blots we found a gradient in desmin expression in the arterial tree; the desmin content increased from the elastic artery aorta, via the muscular mesenteric artery to the resistance-sized mesenteric microarteries ∼150 μm in diameter in Des+/+ mice. Mechanical experiments were performed on the aorta, the mesenteric artery and resistance-sized arteries using wire myographs. For aorta and mesenteric artery, no differences in passive or active circumference- stress relations were found between Des−/− and Des+/+ mice. In microarteries, both passive and active stress were lower in the Des−/− group. In conclusion, large elastic and muscular arteries contain a relatively low amount of desmin, and the desmin intermediate filaments do not seem to play a major role in the mechanical properties of these larger arterial vessels. In the microarteries, where expression of desmin is high, desmin plays a role in supporting both passive and active tension.     

Intradiscal Thermal Annuloplasty for Discogenic Pain: An Outcome Study

Objectives: Published studies of intradiscal thermal annuloplasty (IDTA) have shown at most 50% pain relief as an improved outcome with little focus on functional improvement in the treatment of discogenic pain. Previous studies have used a number of criteria for patient selection including low back pain unresponsive to conservative care, no compressive radiculopathy, positive provocative discography and absence of previous surgery at the same symptomatic level. The purpose of present study is to examine the hypothesis that additional inclusion criteria for patient selection such as disc height, absence of degenerative disc disease (DDD) in untreated discs, absence of herniated nucleus pulposus or lumbar canal stenosis may improve the outcome of treatment. Methods: In this prospective case‐series study additional criteria of patient selection were introduced, namely disc height of at least 50%, no lumbar canal stenosis, one or two levels of DDD, no evidence of nucleus pulposus herniation on magnetic resonance image. Thirty‐four patients were enrolled in the study and 32 of them were followed over a period of 12 months. The visual analog scale (VAS) pain score and seven activities of daily living (ADLs) were followed and reported on a scale from 0 to 10. Results: Sustained decrease of the VAS pain scores was observed from 3 to 12 months following IDTA. ADLs improved in all patients between 3 and 12 months post‐treatment. Patients in the Bureau of Workers Compensation (BWC) group had a higher VAS score but showed the same level of improvement in ADLs as compared to commercial insurance or self‐pay patients. In the non‐BWC patient group an average VAS pain score decrease of more than 6 points on a 10‐point scale was reported at 6 to 12 months following IDTA. Conclusions: We found dramatic improvement of pain scores and ADLs following IDTA when strict patient selection was applied. We believe that IDTA is an effective, minimally invasive treatment for discogenic pain in properly selected patients.     

股骨干骨折--扩髓和不扩髓髓内钉比较

治疗股骨干骨折，采用不扩髓髓内钉比扩髓髓内钉手术时间明显缩短，而且失血也少。但是，扩髓钉的骨愈合更快，延迟愈合更少。两者都没有显著增加包括肺损害等其它并发症的风险。功能结果不能确定。     

Familial hemiplegic migraine and autosomal dominant arteriopathy with leukoencephalopathy (CADASIL)

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a recently described familial cerebrovascular disorder shown to map to chromosome 19q12. Familial hemiplegic migraine has also been shown in some families to map close to the CADASIL locus. The fully developed CADASIL phenotype consists of recurrent strokes developing in the fourth decade, progressing to a pseudobulbar palsy, spastic quadriparesis, and subcortical dementia. In an Irish family 15 members were fully investigated by magnetic resonance scanning; 10 had typical magnetic resonance features of CADASIL. Five members of this family had familial hemiplegic migraine and 4 of these had magnetic resonance evidence of CADASIL. Two other members had migraine with and without aura as a presenting clinical symptom of CADASIL. This disorder has been shown by linkage analysis to map to the CADASIL locus at chromosome 19. The phenotype at presentation of CADASIL in this family was variable and age related and included familial hemiplegic migraine, migraine with and without aura, transient ischemic attacks, strokes, and spinal cord infarction. This family study increases our understanding of the spectrum of clinical manifestations of this underrecognized familial cerebrovascular disorder.     

Palliative treatment of advanced colorectal carcinoma with the YAG laser.

The Nd-YAG laser was used to provide palliative treatment for eight patients (two women and six men), ranging in age from 53 to 76 years, who had locally advanced colorectal cancer. The main indications for treatment were obstruction, bleeding and copious rectal mucus discharge. Only one complication, a Staphylococcus aureus infection, followed the treatment; the infection was easily controlled. The laser treatment was considered to ease the symptoms in five patients, but survival was not influenced by the treatment. This form of palliation must be evaluated in larger numbers of patients to assess its real value in advanced colorectal cancer.