Vascular lesions in Beh?et's disease

Vascular lesions in Beh?et's disease are generally localized in the venous system: up to 24% of the cases reported in the literature are accompanied by thrombosis/thrombophlebitis. Reports of arterial involvement are rare but have increased during the last decade. Surgical treatments of arterial aneurysms and occlusions are often followed by recurrences due to progression or relapses of the inflammatory vessel wall lesions. Aneurysms of the pulmonary artery are rare. They may be complicated by thrombosis within the aneurysm and massive hemoptysis due to bronchial erosion and formation of an arterio-bronchial fistula. 27 cases of Beh?et's disease are reported.     

Cognitive impairment after unilateral hemispheric injury of congenital or adult origin.

OBJECTIVE: The purpose of this study was to assess and compare cognitive functioning in adults with unilateral hemispheric injury due to either congenital damage or an ischemic event in young adulthood. METHOD: Adults with cerebral palsy resulting from left hemispheric brain damage were compared with adults who had a unilateral stroke in either the left or the right hemisphere. Our primary interest was to determine the impact on hemispheric dominance as revealed by dichotic listening, a task that assesses the bias for preferential listening and processing of sounds. Performance also was determined on a language-related task (word finding) and a spatial task (dot localization). RESULTS: Scores on the Quick Neurological Screening Test indicated that all participants demonstrated significant neuromotor deficits, whereas scores on the Barthel Index indicated that the participants were functional in basic activities of daily living. On cognitive assessments, healthy control participants demonstrated a pronounced left-hemisphere dominance and right-ear advantage; participants with injury to the left hemisphere showed a strong shift toward a right-hemisphere and left-ear dominance. In particular, injury of congenital origin appeared to foster this neural reorganization and localization of language-related functions into the healthy hemisphere. This shift was associated with a deterioration of performance on both the language and the spatial tasks. CONCLUSION: The importance of appreciating subtle deficits after unilateral injury is important in therapy. The dichotic listening test may provide a simple and useful means for evaluating persistent unilateral brain dysfunction in the clinical setting.     

Long-Term Clinical Outcomes Following Treatment of Actinic Keratosis with Imiquimod 5% Cream

BACKGROUND: The results from four phase III, randomized, vehicle-controlled studies showed that imiquimod 5% cream (imiquimod) was safe and effective in the treatment of actinic keratosis (AK). Patients applied imiquimod or vehicle cream to AK lesions on the face or balding scalp, dosing three times per week or two times per week for 16 weeks. OBJECTIVE: To obtain long-term safety follow-up data and estimate AK recurrence in patients who completely cleared their AK lesions in the treatment area at the 8-week post-treatment visit in the phase III studies. METHODS: One hundred forty-six patients from 30 study centers in the United States were evaluated for clinical evidence of AK, and safety data were collected. RESULTS: After a median follow-up period of 16 months, 24.7% (19 of 77) of the patients administered imiquimod three times per week and 42.6% (23 of 54) of the patients administered imiquimod two times per week had a recurrence of AK (the appearance of at least one AK lesion) in the original treatment area. The median number of AK lesions present was one lesion for both patients receiving imiquimod three times and those receiving imiquimod two times per week compared with a median of six lesions at baseline in the combined three times per week and two times per week phase III studies. There were no long-term safety issues, and the skin quality seen in the imiquimod-treated patients at the end of the phase III studies was maintained. CONCLUSION: One and a half years following treatment, imiquimod continued to provide a long-term clinical benefit in a majority of patients who experienced complete clearance of their AK lesions.     

Noonan syndrome associated with central giant cell granuloma

We report a case of Noonan syndrome associated with central giant cell granuloma. The patient was a 101/2-year-old boy with the chief complaint of proptosis of the right eye. He also had various malformations such as short stature, webbed neck, pectus excavatum, cubitus valgus, pulmonary valve stenosis and patent foramen ovale, a characteristic face appearance and cryptorchidism and so on. Chromosome analysis showed a 46, XY karyotype. A computed tomographic scan and magnetic resonance imaging showed a mass originated from the lateral wall of the right maxillary sinus. The patient underwent Caldwell-Luc operation. Histological examination of the mass showed the characteristics of central giant cell granuloma. This case report describes a patient with the features of the recently described Noonan-like/multiple giant cell lesion syndrome.     

Aglossia-adactylia syndrome (special emphasis on the inheritance pattern)

Three cases, one with aglossia-adactylia and two with aglossia, are presented, all of whom were born to consanguineous families. Although none of the cases had similarly affected sibs, the possibility of the autosomal recessive mode of inheritance might be taken into account in this syndrome. The dermatoglyphic findings in one previously reported patient showed great similarity to those of one of our cases.     

Fertility of ejaculated and testicular megalohead spermatozoa with intracytoplasmic sperm injection

In this study the fertility and outcome of intracytoplasmic sperm injection (ICSI) using megalohead spermatozoa from the ejaculates and testicles was evaluated. Seventeen males with megalohead and pinhead sperm forms in their ejaculate were studied in 22 cycles. A high number of sperm heads without tails and abundant round spermatid forms were commonly observed. Round-headed spermatozoa were seldom accompanied by these severely abnormal spermatozoa. The majority of megalohead spermatozoa were observed to have multiple tails, were predominant in the sample, and were used for ICSI. Ejaculated megalohead spermatozoa were used for ICSI in 15 cycles, while testicular spermatozoa were used in seven cycles where there were no vital spermatozoa or spermatozoa of low vitality in the ejaculate. The same abnormal morphology was observed in the testicles as in the ejaculated spermatozoa in the same males. Mean (+/- SD) low motility 4.7 +/- 5.6% and sperm count (3.8 +/- 4.19 x 10(6)) were common findings in these severely teratozoospermic patients. A low fertilization rate (43.2%) was achieved by using megalohead sperm forms (group I, n = 17) in comparison with the control group (60.2%) which had zero normal sperm morphology according to strict criteria (group II, n = 30) (P <0.01). Furthermore, a low pregnancy rate (9.1%) was obtained in the megalohead sperm group in comparison with the control group (40%) (P <0.05). Low fertilization and pregnancy rates may be due to a high incidence of chromosomal abnormalities from severely defective spermatozoa in the ejaculate. Couples should be counselled and warned about possible low fertilization and pregnancy rates with ICSI when only pinhead and megalohead forms with a high number of sperm heads without tails are present in the ejaculate.     

A scoping Review of tools used to assess patient Complexity in rheumatic disease

ObjectivePatients with rheumatic diseases often have multiple comorbidities which may impact well‐being leading to high psychosocial complexity. This scoping review was undertaken to identify complexity measures/tools used in rheumatology that could help in planning and coordinating care.MethodsMEDLINE, EMBASE and CINAHL were searched from database inception to 14 December 2019 using keywords and Medical Subject Headings for “care coordination”, “complexity” and selected rheumatic diseases and known complexity measures/tools. Articles describing the development or use of complexity measures/tools in patients with adult rheumatologic diagnoses were included regardless of study design. Included articles were evaluated for risk of bias where applicable.ResultsThe search yielded 407 articles, 37 underwent full‐text review and 2 were identified during a hand search with 9 included articles. Only 2 complexity tools used in populations of adult patients with rheumatic disease were identified: the SLENQ and the INTERMED. The SLENQ is a 97‐item patient needs questionnaire developed for patients with systemic lupus (n = 1 study describing tool development) and applied in 5 cross‐sectional studies. Three studies (a practice article, trial and a cross‐sectional study) applied the INTERMED, a clinical interview to ascertain complexity and support coordinated care, in patients with rheumatologic diagnoses.ConclusionsThere is limited information on the use of patient complexity measures/tools in rheumatology. Such tools could be applied to coordinate multidisciplinary care and improve patient experience and outcomes.Patient contributionThis scoping review will be presented to patient research partners involved in co‐designing a future study on patient complexity in rheumatic disease.     

Agreement between proxy- and self-report scores on PROMIS health-related quality of life domains in pediatric burn survivors: a National Institute on Disability,Independent Living,and Rehabilitation Research Burn Model System Study

Quality of Life Research - To examine agreement between pediatric burn survivor self- and caregiver proxy-report on multiple PROMIS domains and examine factors associated with differences between...