Fehlstellungen der Hüfte

Monatsschrift Kinderheilkunde - Fehlstellungen der Hüfte sind bei Kindern und Jugendlichen gut an einem hinkenden Gang, an der Rumpfasymmetrie im Stand sowie an einem seitendifferenten aktiven...     

Digital subtraction angiography system evaluation with phantoms

Advances in digital subtraction angiography imaging demonstrate the need for critical evaluation of the performance of digital subtraction equipment. The design of a phantom set for noninvasive assessment of the imaging quality of digital subtraction equipment is described; components include a remotely controlled transport system and individual patterns to evaluate the contrast and detail properties of the image intensifier, low-contrast sensitivity and resolution of the system, geometric distortion of image, linearity, mechanical and electronic stability of equipment, and effects of bone and bowel gas on iodine perception. The performance of an add-on digital radiographic system is presented, along with radiation exposure levels at the image intensifier for a range of radiographic techniques.     

A founder mutation in the gamma-sarcoglycan gene of gypsies possibly predating their migration out of India

We investigated the molecular basis of a severe form of early onset autosomal recessive muscular dystrophy with sarcoglycan (SG) deficiency in seven large Gypsy families living in different parts of Western Europe and apparently not closely related. They were linked to the LGMD2C locus (13q12) suggesting a primary defect in the gamma-SG gene coding for the 35 kDa dystrophin-associated glycoprotein. All of the 18 investigated patients were homozygous for the same G-->A transition in codon 283 producing the replacement of a conserved cysteine of the extra-cellular domain of the protein by a tyrosine. All affected chromosomes in homozygous and heterozygous relatives carried the same allele 5 of the intragenic marker D13S232. Flanking markers were studied to delineate a common ancestral haplotype, the size of which was used to compute the date of the founding mutation. We found evidence that the mutation occurred between 60 and 200 generations ago, therefore possibly predating the commonly accepted date of migration of the Gypsy ancestors out of India.      

HPLC测定睾酮乳膏中睾酮的含量

采用ＨＰＬＣ测定睾酮乳膏中睾酮的含量，以甲醇－水（７０：３０）为流动相，波长２４０ｎｍ，在４０－３６０μｇ／ｍｌ范围内线性关系良好ｒ＝０．９９９９，本法操作简便，回收率高。     

Tracheal agenesis with unique anatomy

A premature infant with a unique form of tracheal agenesis is described. The combination of difficulty in intubation, abnormal course of the nasogastric tube on plain x-ray, and gastric perforation raised the suspicion of an upper airway malformation.Tracheal agenesis is an extremely rare, typically fatal, congenital anomaly with scattered case reports of its successful management. On many occasions, the diagnosis is a retrospective one at postmortem examination. The possibility of surgical correction rests on early diagnosis, anatomy, birth weight, and associated anomalies. Early recognition is mandatory, but in our case, the low birth weight precluded consideration for reconstructive surgery.     

Predicting speech perception outcomes following cochlear implantation using Nottingham children's implant profile (NChIP)

BACKGROUND-OBJECTIVE: Nottingham children's implant profile (NChIP) is a systematic framework to assess deaf children before implantation taking into account not only the well-known factors (age, duration of deafness) but also family and support services, expectations, children's cognitive abilities and learning style. The aim of the present paper is to assess the predictive value of the NChIP in the long-term outcomes of implanted children. PATIENTS: This prospective and longitudinal study involved 51 profoundly deaf children, implanted within the same paediatric cochlear implant programme. All children were pre-lingually deaf with age at onset of deafness <2 years and age at implantation <6 years. Three and 4 years following implantation all children were assessed using two measures of speech perception. No child was lost to follow-up and no child was excluded from the study for any reason. RESULTS: The most constant predictor of the outcomes was children's learning style explaining upto 29% of the variance. Other significant predictors of the outcome were shorter duration of deafness, young age at implantation and family structure/support. CONCLUSIONS: Intrinsic factors in children and how well they communicate with their environment are of paramount importance to the outcome following implantation. The predictive value of NChIP has been demonstrated and the most important predictors of the outcome were children's learning style, short duration of deafness, young age at implantation, and family structure/support.     

Infections following cochlear implantation

Cochlear implantation has become routine in the management of children and adults with profound sensorineural hearing loss. In rare cases postoperative infections necessitate removal of the implant. We present six such cases that have been managed within our programme. Extensive infected granulation tissue was found around the implant at exploration despite prolonged intravenous treatment with appropriate antimicrobial agents. All devices were explanted and three have been reimplanted at our unit. We discuss our management of these cases, the need for explantation, consideration for reimplantation and their functional outcome following reimplantation. We also highlight how systemic inflammatory markers can be unhelpful in detecting significant infection surrounding a cochlear implant.     

Cochlear implantation--United Kingdom and Ireland surgical survey

Cochlear implantation has now become a routine procedure in the management of severe to profound deafness. An initial survey was carried out in late 1997 of the surgeons carrying out implantation in the United Kingdom and Ireland, requesting details of medical and surgical aspects of cochlear implantation. A follow-up survey was conducted in early 2002 to evaluate any changes in clinical practice. The reported results show a low surgical complication rate in both children and adults. Reasons for this are discussed.