Dega-Osteotomie bei Hüftgelenkdysplasie

Zusammenfassung Operationsziel überdachung des Femurkopfes bei Hüftgelenkdysplasie. Indikationen Dysplastisches Acetabulum bei Patienten mit neuromuskul?ren und nichtneuromuskul?ren Erkrankungen. übergro?es, flaches Acetabulum. Fehlen einer lateralen und kranialen überdachung. Kontraindikationen Y-Fuge geschlossen, Wachstum abgeschlossen. Stark verformter Femurkopf. Operationstechnik Modifizierter Zugang nach Salter/Smith-Petersen. Freilegung und Teilung der Apophyse des Beckenkammes mit einem Messer. Freilegung der Darmbeinschaufel. Osteotomie der ?u?eren Wand des Iliums; sie wird vorsichtig mit Hilfe eines gebogenen mei?els nach unten gebogen und in dieser Stellung durch Einsetzen von trikortikalen Beckenkammsp?nen gehalten. Eine Osteosynthese ist nicht notwendig. Becken-Bein-Gipsverband mit Einschlu? des gegenseitigen Oberschenkels für sechs Wochen. Ergebnisse Zwischen 1987 und 1997 wurden 26 Hüften von 23 Patienten operiert. überwiegend handelte es sich um Kinder mit spastischen Paresen. Folgende zus?tzliche Eingriffe wurden vorgenommen: Offene Reposition zehnmal, Femurosteotomie 18mal und Tenotomien sowie Muskelabl?sungen 15mal. Der Durchschnittswert des azetabul?ren Index verbesserte sich von 32° auf 22°, der durchschnittliche Wert des Kopfzentrum-Pfannenrand-Winkels von −25° auf 22° und der durchschnittliche Migrationsindex von 62% auf 69%. Als Komplikationen wurden beobachtet: eine erneute Subluxation, ein frühzeitiger Verschlu? des Y-Knorpels, eine Fraktur des anderen Femur, einmal heterotope Ossifikationen und einmal eine Infektion der Harnwege.     

Good outcome in prune-belly syndrome despite associated severe anomalies

A boy aged 4.5 years with prune-belly syndrome (PBS) and associated urethral stenosis, oligohydramnios, imperforate anus and vesicosigmoid fustula is described. In contrast to the anticipated poor prognosis, vesicostomy and divided transverse colostomy performed after birth followed by prophylaxis of infection and bicarbonate supplementation have resulted in a good outcome. The vesicosigmoid fistula might have served in utero as a natural diversion protecting from pressure-induced renal damage. It is suggested that the main determinant of prognosis in PBS is the presence and degree of kidney dysplasia at birth as reflected by the neonatal renal function after performance of an indicated urinary diversion procedure rather than the presence of severe associated anomalies.Supported in part by grants from the National Institute of Health AM 37223-01 and the Medical School and Graduate School Research Committees of the University of Wisconsin and a Research Career Development Award KO4 AM 00421 (RWC), by the Pearl M. Stetler Foundation (SD) and by a National Kidney Foundation fellowship (IZ)     

The role of imperatives in psychopathology: A reply to Ellis

In his recent formulations of rational emotive therapy (RET), Ellis (1985) has increasingly emphasized what he believes to be the central role of necessitous thinking or musturbation in the development of depression. In his most recent article (Ellis, 1987), he suggests that necessitous thinking is the primary cognitive component of depression and that RET stands alone in its recognition of the role of this cognitive element. The present study addressed three issues raised by Ellis's paper: (1) Clarify whether necessitous thinking has been neglected by other cognitive theorists, (2) test empirically the role of necessitou thinking in depression relative to other established cognitive constructs, e.g., the cognitive triad (Beck, 1967), and (3) determine whether necessitous thinking is particularly salient in depression as compared with other forms of psychopathology. It was predicted that necessitous thinking would be found in other forms of psychology in addition to depression, and this was confirmed. The results are discussed as highlighting the importance of empirically testing theoretical predictions.From the Foundation for Cognitive Therapy.     

Measurement of post-operative pain and narcotic administration in infants using a new clinical scoring system

We developed a clinical neurologic and behavioral scoring system composed of 10 items to measure the post-operative pain levels in infants: (1) sleep during preceeding hour, (2) facial expression of pain, (3) quality of cry, (4) spontaneous motor activity, (5) Spontaneous excitability, (6) flexion of fingers and toes, (7) sucking, (8) global evaluation of tone, (9) consolability and (10) sociability. Using this system, a group of infants ranging from one to seven months in age and undergoing minor surgical procedures was studied. The infants were randomly assigned to two groups: Group I received Fentanyl intravenously (3 g/kg) prior to surgery, and Group II received a placebo. The infants then were studied post-operatively in the recovery room at 30, 60, 90 and 120 min intervals. Over the entire post-operative observation period, 54% of the infants in Group I had satisfactory analgesia compared to 18% in Group II. There were no significant differences in Group I and Group II in oxygenation, carbon dioxide elimination, blood pressure, heart rate or temperature.     

Immunocytochemical profile of neurofibrillary tangles in Down's syndrome patients of different ages

Brains were obtained at autopsy from 24 patients with Down's syndrome, ranging in age from 13 to 71 years. Neurofibrillary tangle containing neurones of the hippocampus were stained using a Palmgren silver method and immunocytochemically (PAP) using antisera to paired helical filament protein, human tau protein and ubiquitin, as primary antibody. Counts of cells stained by each method were compared. In patients under 50 years of age, in whom only a limited number of tangle bearing cells were present, the number of profiles visualized with silver, anti-paired helical filament and anti-tau methods were similar. However, in patients over 50 years of age (and in certain of those under 50), in whom numerous tangles were present, the number of cell profiles visualized with silver and anti-paired helical filament methods were still similar though anti-tau detected fewer positive cells. This was because of the increased presence, in such patients, of extracellular tangles which had "lost" anti-tau immunoreactivity. Such data suggest that although tau protein forms a major antigenic determinant of neurofibrillary tangles in Down's syndrome (as it does in Alzheimer's disease) this protein may only decorate the basic paired helical filament protein skeleton, and is removed by macrophagic activity upon neuronal death. In all patients, anti-ubiquitin revealed fewer tangles than any other method. It is possible that ubiquitin may be present only transiently, within tangles perhaps following initial formation and lasting only as long as the normal protein degradation processes remain viable within the diseased neurone.     

The effects of 4-aminopyridine on focal nerve conduction block

In order to test whether 4-aminopyridine (4-AP), a potassium channel blocker, may be of therapeutic value in demyelinating neuropathies, a focal tibial nerve conduction block with demyelination was produced in adult rats by an intraneural microinjection of potassium tellurite. Onset and recovery of the lesion were monitored by evoked compound muscle action potentials (CMAP) activated from the proximal and distal nerve one day before and 1, 4, 7, 14, 21 and 28 days after the injection. Intraperitoneal 4-AP (2 mg/kg) or buffered saline were injected prior to the potassium tellurite and 6 days per week for 28 days. The data show that 4-AP is tolerated, it does not prevent conduction block, and only has a modest effect on increasing its recovery from day 4 to 7 (91 % increase in CMAP ratio compared with control of 35%). Recovery is similar by day 28 in 4-AP treated or untreated animals. These results suggest that 4-AP will have limited use in the therapy of subacute demyelinating neuropathies.     

Focus assay for varicella-zoster virus in human embryo cells stained with immunoperoxidase method

Rapid titration of varicella-zoster virus (VZV) in human embryonic fibroblasts (HEF) based on staining of virus-infected cells by indirect immunoperoxidase technique (IPA) is described. Cell monolayers were grown in wells of plastic plates (two different diameters). Foci of virus-infected cells as revealed by IPA could be counted either 48 hr post-infection, if cell-associated virus (VZV infected cells) was used as inoculum, or 72 hr p. i. if cell-free virus was used. A linear relationship was observed between virus dilution and number of foci. The first virus was detected 12 hr p. i., the highest titre at 36 hr, when cytopathic effect (CPE) involved about 50% of the monolayer.     

Microanatomical study of the arterial blood supply of the facial nerve in the ponto-cerebellar angle

Summary The blood supply of 50 facial nerves was examined during a micro-anatomical study of the ponto-cerebellar angle using injections of colored latex or Chinese ink.The results indicate the presence of a double arterial blood supply: the proximal supply, rising at the level of the brain stem, results from the confluence of three to five arterioles originating from the anterior inferior cerebellar artery or from the basilar artery itself; the distal supply at the level of the internal acoustic meatus arises from the labyrinthine artery.These two arterial systems do not anastomose directly but by way of capillaries of less than 200 microns in diameter.From these findings the authors conclude that certain postoperative facial paralyses, which may arise in spite of the conservation of the nerve, are in fact ischemic in nature.

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Etude micro-anatomique de la vascularisation artérielle du nerf facial dans l'angle ponto-cérébelleux

Résumé Dans le cadre d'une étude micro-anatomique de l'angle ponto-cérébelleux, la vascularisation de 50 nerfs faciaux est examinée sous microscope opératoire après injection au latex coloré ou à l'encre de Chine.Cette étude permet aux auteurs de décrire un double apport artériel: l'un proximal, né au niveau du tronc cérébral, résulte de la confluence de 3 à 5 artérioles issues de l'ACiA ou de l'artère basilaire ellemême; l'autre distal, au niveau du méat acoustique interne, provient de l'artère labyrinthique. Ces deux systèmes artériels ne s'anastomosent pas entre eux à plein canal, mais par des capillaires d'un diamètre inférieur à 200 microns.Les auteurs en tirent argument pour estimer que certaines paralysies faciales post-opératoires survenant malgré la conservation anatomique du nerf, sont de nature ischémique.

     

The Turner syndrome research registry: Creating equipoise between investigators and participants

To address knowledge gaps about Turner syndrome (TS) associated disease mechanisms, the Turner Syndrome Society of the United States created the Turner Syndrome Research Registry (TSRR), a patient‐powered registry for girls and women with TS. More than 600 participants, parents or guardians completed a 33‐item foundational survey that included questions about demographics, medical conditions, psychological conditions, sexuality, hormonal therapy, patient and provider knowledge about TS, and patient satisfaction. The TSRR platform is engineered to allow individuals living with rare conditions and investigators to work side‐by‐side. The purpose of this article is to introduce the concept, architecture, and currently available content of the TSRR, in anticipation of inviting proposals to utilize registry resources.