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1.
J Intern Med ; 289(5): 688-699, 2021 05.
Artículo en Inglés | MEDLINE | ID: mdl-33210357

RESUMEN

BACKGROUND: COVID-19 is caused by the coronavirus SARS-CoV-2, which uses angiotensin-converting enzyme 2 (ACE-2) as a receptor for cellular entry. It is theorized that ACE inhibitors (ACE-Is) or angiotensin receptor blockers (ARBs) may increase vulnerability to SARS-CoV-2 by upregulating ACE-2 expression, but ACE-I/ARB discontinuation is associated with clinical deterioration. OBJECTIVE: To determine whether ACE-I and ARB use is associated with acute kidney injury (AKI), macrovascular thrombosis and in-hospital mortality. METHODS: A retrospective, single-centre study of 558 hospital inpatients with confirmed COVID-19 admitted from 1 March to 30 April 2020, followed up until 24 May 2020. AKI and macrovascular thrombosis were primary end-points, and in-hospital mortality was a secondary end-point. RESULTS: AKI occurred in 126 (23.1%) patients, 34 (6.1%) developed macrovascular thrombi, and 200 (35.9%) died. Overlap propensity score-weighted analysis showed no significant effect of ACE-I/ARB use on the risk of occurrence of the specified end-points. On exploratory analysis, severe chronic kidney disease (CKD) increases odds of macrovascular thrombi (OR: 8.237, 95% CI: 1.689-40.181, P = 0.009). The risk of AKI increased with advancing age (OR: 1.028, 95% CI: 1.011-1.044, P = 0.001) and diabetes (OR: 1.675, 95% CI: 1.065-2.633, P = 0.025). Immunosuppression was associated with lower risk of AKI (OR: 0.160, 95% CI: 0.029-0.886, P = 0.036). Advancing age, dependence on care, male gender and eGFR < 60 mL min-1 /1.73 m2 increased odds of in-hospital mortality. CONCLUSION: We did not identify an association between ACE-I/ARB use and AKI, macrovascular thrombi or mortality. This supports the recommendations of the European and American Societies of Cardiology that ACE-Is and ARBs should not be discontinued during the COVID-19 pandemic.


Asunto(s)
Lesión Renal Aguda , Antagonistas de Receptores de Angiotensina/uso terapéutico , Inhibidores de la Enzima Convertidora de Angiotensina/uso terapéutico , COVID-19 , Hipertensión , Insuficiencia Renal Crónica , Trombosis , Lesión Renal Aguda/diagnóstico , Lesión Renal Aguda/etiología , Factores de Edad , Anciano , COVID-19/diagnóstico , COVID-19/mortalidad , COVID-19/fisiopatología , Comorbilidad , Diabetes Mellitus/epidemiología , Femenino , Tasa de Filtración Glomerular , Mortalidad Hospitalaria , Humanos , Hipertensión/tratamiento farmacológico , Hipertensión/epidemiología , Masculino , Evaluación de Procesos y Resultados en Atención de Salud , Insuficiencia Renal Crónica/epidemiología , Insuficiencia Renal Crónica/terapia , Ajuste de Riesgo/métodos , SARS-CoV-2/aislamiento & purificación , Trombosis/diagnóstico , Trombosis/etiología , Reino Unido/epidemiología , Privación de Tratamiento/normas , Privación de Tratamiento/estadística & datos numéricos
2.
Semin Musculoskelet Radiol ; 23(5): 523-533, 2019 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-31556087

RESUMEN

Regarding the upper extremity, osteonecrosis can relate to the humeral head and to any carpal bone, most commonly the lunate (Kienböck's disease), scaphoid (Preiser's disease and nonunion), and capitate bone (osteonecrosis of the capitate head). In children and adolescents, osteochondrosis is an important differential diagnosis at the epiphyses. Appropriate imaging of osteonecrosis depends on knowledge about blood supply, biomechanical load, and bone repair mechanisms. Contrast-enhanced MRI (ceMRI) enables the differentiation of up to three mostly band-shaped zones: necrotic tissue (proximal), hypervascular repair tissue (intermediate), and viable bone (distal). To distinguish between necrotic and repair zones, intravenous gadolinium is recommended in MRI. Osteosclerosis and insufficiency fractures in early and intermediate stages as well as osteoarthritis in advanced stages are best depicted using high-resolution CT (HRCT). The combination of HRCT and ceMRI allows for exact classification of osteonecrosis regarding morphology and viability.


Asunto(s)
Imagen por Resonancia Magnética/métodos , Osteonecrosis/diagnóstico por imagen , Extremidad Superior/diagnóstico por imagen , Huesos/diagnóstico por imagen , Huesos/patología , Diagnóstico Diferencial , Humanos , Osteonecrosis/patología , Extremidad Superior/patología
3.
Unfallchirurg ; 122(3): 200-210, 2019 Mar.
Artículo en Alemán | MEDLINE | ID: mdl-30725118

RESUMEN

The most important goals of scaphoid reconstruction in pseudarthrosis are correction of the humpback deformity, the realignment of the proximal carpal row and the bony union of the scaphoid. Therefore, in most cases bone grafting is required. To increase the healing rate and to improve vascularization, several kinds of vascularized bone grafts have been developed. Pedicled grafts are preferably harvested from the dorsal or palmar side of the distal radius with fusion rates between 27% and 100%. Free microvascular grafts can be obtained from the iliac crest and the medial or lateral femoral condyle with fusion rates between 60% and 100%. For their application microsurgical equipment and skills are required. Up to now osteochondral grafts from the femoral condyle offer the only chance for joint surface replacement by transferring part of the surface of the femoropatellar joint. The use of vascularized grafts is still a matter of controversy, since their superiority is still unproven compared to nonvascularized grafts, which also achieved 100% fusion rates in several series. They are indicated in secondary procedures after failed reconstruction and nonunion with small avascular proximal pole fragments. Since no evidence-based guidelines exist, this article provides an experience-based treatment algorithm for scaphoid nonunion with special consideration to vascularized bone grafts.


Asunto(s)
Seudoartrosis/terapia , Hueso Escafoides , Trasplante Óseo , Humanos , Radio (Anatomía)
4.
Oper Orthop Traumatol ; 31(5): 393-407, 2019 Oct.
Artículo en Alemán | MEDLINE | ID: mdl-30218133

RESUMEN

OBJECTIVE: Restoration of proximal interphalangeal joint stability with preservation of mobility by reconstruction of the middle phalanx base using an osteochondral graft from the carpometacarpal joint surface of the hamate. INDICATIONS: Acute and older isolated destruction of the palmar middle phalanx base >25%. CONTRAINDICATIONS: Destruction of the head of the proximal phalanx, advanced chondropathia of the head of the proximal phalanx, extensive soft tissue injury with loss of skin coverage at the proximal interphalangeal joint. SURGICAL TECHNIQUE: The fractured middle phalangeal base is debrided and the defect is replaced by a size-matched autograft from the dorsal carpometacarpal hamate osteoarticular surface that is secured in place with miniscrews. POSTOPERATIVE MANAGEMENT: Immobilization for 2 weeks in a below-elbow cast in intrinsic plus position. Subsequent immobilization by a splint including the distal and proximal interphalangeal joint. RESULTS: Healing was achieved in 100% with restoration of joint congruity in 12 of 13 cases and slight subluxation in 1 case. Follow up was possible in 9 cases after 22 ± 16 (5-51) months. The average range of motion in the reconstructed joint achieved 0/9/73°, grip strength 82% of the unaffected side. Of the 9 patients, 5 developed a mild flexion contracture of the proximal interphalangeal joint. The DASH score achieved 4 ± 3 (0-8) points, pain at rest was 1 ± 2 (0-5), pain at exercise 2 ± 2 (0-5) on a visual analogue scale (0-10). All patients were satisfied and willing to undergo the procedure again. According to the literature, reconstruction of the base of the middle phalanx by using an osteochondral graft from the hamate is a reliable procedure to restore stability and mobility of the joint.


Asunto(s)
Traumatismos de los Dedos , Falanges de los Dedos de la Mano , Hueso Ganchoso , Autoinjertos , Traumatismos de los Dedos/cirugía , Articulaciones de los Dedos , Falanges de los Dedos de la Mano/lesiones , Falanges de los Dedos de la Mano/cirugía , Hueso Ganchoso/trasplante , Humanos , Rango del Movimiento Articular , Resultado del Tratamiento
5.
Unfallchirurg ; 121(5): 381-390, 2018 May.
Artículo en Alemán | MEDLINE | ID: mdl-29549407

RESUMEN

BACKGROUND: There still is no standard therapy that predictably results in healing of avascular necrosis of the lunate bone. Nevertheless, there exists a wide spectrum of operative treatment options for different stages. OBJECTIVE: This article reviews the treatment options for necrosis of the lunate bone and proposes algorithms based on the age of the patient and condition of the lunate bone and the wrist. METHODS: Surgical treatment options for necrosis of the lunate bone can be divided into relieving or revascularization procedures and salvage procedures. RECOMMENDATIONS: For patients under 20 years old the treatment of choice is prolonged immobilization, in cases of non-response or progression, minimally invasive and relieving procedures are used. In adult patients with limited affection of the lunate bone the first therapeutic approach should also be immobilization. If in progressive disease or advanced stages only the lunate bone is compromised but reconstructable, restoration should be considered. In progressive collapse of a non-reconstructable lunate bone the therapeutic efforts shift to mobility-preserving procedures utilizing still functional articulations of the wrist. If all functional articulations are lost only classical salvage procedures are feasible. CONCLUSION: According to the presented algorithms a stage-dependent therapy of necrosis of the lunate bone is possible. It should not be ignored that there are still no scientific and evidence-based arguments for some of these treatment options. This is also true for maximally invasive procedures, where superiority to more simple procedures have not been proven. Therefore, their application should be restricted and based on an individual decision.


Asunto(s)
Hueso Semilunar , Osteonecrosis , Adulto , Algoritmos , Artrodesis , Humanos , Osteonecrosis/terapia , Articulación de la Muñeca , Adulto Joven
6.
J Hand Surg Eur Vol ; 42(3): 253-259, 2017 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-28196441

RESUMEN

We carried out a retrospective study to analyse the long-term outcome of 36 patients after radial shortening osteotomy for treatment of Lichtman Stage IIIA Kienböck disease at a mean follow-up of 12.1 years (range 5.4-17.5). At review, seven wrists had progressed to Stage IIIB, eight wrists to Stage IV and 21 remained in Stage IIIA. Motion and grip strength were significantly improved. The mean Disabilities of the Arm, Shoulder, and Hand (DASH) score at review was 12 points (range 0-52), and patient satisfaction was high. Apart from plate removals in 14 patients and one wrist denervation, no subsequent surgical procedures were done. Radial shortening yields good long-term clinical results, but does not prevent radiographic progression of disease in some patients. LEVEL OF EVIDENCE: Therapeutic IV.


Asunto(s)
Osteonecrosis/cirugía , Osteotomía , Radio (Anatomía)/cirugía , Articulación de la Muñeca , Adolescente , Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Osteonecrosis/diagnóstico por imagen , Rango del Movimiento Articular , Estudios Retrospectivos , Resultado del Tratamiento , Adulto Joven
7.
Orthopade ; 45(11): 966-973, 2016 Nov.
Artículo en Alemán | MEDLINE | ID: mdl-27650446

RESUMEN

BACKGROUND: Avascularity of fragments is a common problem in treatment of scaphoid nonunion. Therefore, vascularised bone grafts have gained increasing importance. Especially the free vascularised femoral trochlea flap has become the subject of special interest because of its particular characteristics in the last few years. OBJECTIVES: Indications for and the technique of free vascularised corticocancellous as well as osteocartilagineous femoral trochlea flap are reported according to the author's practice combined with an evaluation of these methods considering current literature. RESULTS: Indications for the free vasscularised corticocancellous femoral trochlea flap include scaphoid nonunion with avascular proximal fragment combined with humpback deformity, previous operations, extensive bone defect, and long standing nonunion. The free vascularised osteocartilagineous medial trochlea flap may replace a destroyed proximal scaphoid pole. The literature reports only a few patients with healing rates between 80 and 100 %. CONCLUSIONS: Both corticocancellous and osteocartilagineous free vascularised medial trochlea flap improve therapeutic options in treatment of difficult scaphoid nonunion. Until now only the results of a few patients have been published. Therefore, a final evaluation will be possible when long-term results in a reasonable number of patients are available.


Asunto(s)
Trasplante Óseo/métodos , Fracturas Óseas/diagnóstico por imagen , Fracturas Óseas/cirugía , Fracturas no Consolidadas/cirugía , Hueso Escafoides/lesiones , Hueso Escafoides/cirugía , Colgajos Quirúrgicos/trasplante , Medicina Basada en la Evidencia , Fracturas no Consolidadas/diagnóstico por imagen , Humanos , Resultado del Tratamiento
8.
Handchir Mikrochir Plast Chir ; 48(1): 33-40, 2016 Feb.
Artículo en Alemán | MEDLINE | ID: mdl-26895518

RESUMEN

BACKGROUND: Trigger finger in children is a rare condition with relevant differences to the more frequent trigger thumb in children and the very frequent trigger finger in adults. PATIENTS AND METHODS: In a retrospective analysis of children who underwent surgery for trigger fingers in 2 specialised centres, we evaluated 42 children with a total of 63 trigger fingers. Due to persistent triggering after release of the A1 pulley based on intraoperative findings in 12 children with a mean age of 3.7 years ranging from 0.6 to 10.2 years and a total of 17 trigger fingers, additional measures after the release of the A1 pulley were required, mainly the resection of one limb of the sublimis tendon. In one of these children another simultaneously operated finger required the release of the A1 pulley alone. We performed a retrospective chart review of these patients and evaluated the results after a mean follow-up time of 4.8 years ranging from 1.2 to 11.8 years by phone call. Functional impairments, persistent pain, satisfaction and complications were monitored. RESULTS: All patients treated with A1 pulley release and resection of one limb of the sublimis tendon had excellent results. One patient had a recurrence and another patient developed a contracture of the PIP joint. In both patients, the widening of the tendon sheath had been done by A2 pulley enlargement in addition to the A1 pulley release. CONCLUSIONS: Persistent triggering of a finger following the release of the A1 pulley in a child can be successfully treated by resection of one limb of the sublimis tendon.


Asunto(s)
Trastorno del Dedo en Gatillo/diagnóstico , Trastorno del Dedo en Gatillo/cirugía , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/cirugía , Recurrencia , Reoperación , Estudios Retrospectivos , Técnicas de Sutura , Tenotomía/métodos
9.
Unfallchirurg ; 118(6): 515-9, 2015 Jun.
Artículo en Alemán | MEDLINE | ID: mdl-25986769

RESUMEN

BACKGROUND: Apart from clean cut finger amputations, every kind of hand injury can be seen in mountain and winter sports but only skier's thumb and injuries of the pulley system in sport climbers are seen in a greater number of cases. Nevertheless, these two common injuries as well as the rare frostbite of the fingers are often underdiagnosed or overdiagnosed as well as undertreated or overtreated. PURPOSE: This paper describes the diagnostics and treatment of skier's thumb, injuries of the pulley system in sport climbers and frostbite of the fingers. RESULTS: Before checking the metacarpophalangeal (MP) joint of the thumb for stability, radiographs should be taken to exclude a bony avulsion of the ulnar collateral ligament in skier's thumb. If there is no bony ligament avulsion further diagnostic procedures, e.g. ultrasound, are recommended to prove or exclude a Stener lesion, which is an absolute indication for operative treatment together with a dislocated bony ligament avulsion. To quantify the severity of a lesion of the pulley system ultrasound and magnetic resonance imaging (MRI) are needed. Most lesions of the pulley system can be treated conservatively. Only multiple pulley ruptures or isolated ruptures associated with a lesion of the lumbrical muscles or collateral ligaments require operative treatment. As long as there is no infection amputation should be done as late as possible in frostbite of the fingers because the extent of the frostbite can rarely be correctly estimated. CONCLUSION: Most cases of skier's thumb as well as lesions of the pulley system can be treated non-operatively but precise diagnostics are needed.


Asunto(s)
Traumatismos de los Dedos/diagnóstico , Traumatismos de los Dedos/terapia , Montañismo/lesiones , Traumatismos de los Tejidos Blandos/diagnóstico , Traumatismos de los Tejidos Blandos/terapia , Pulgar/lesiones , Diagnóstico Diferencial , Traumatismos de la Mano , Humanos , Traumatismo Múltiple/diagnóstico , Traumatismo Múltiple/terapia , Traumatismos de los Tendones/diagnóstico , Traumatismos de los Tendones/terapia
10.
Oper Orthop Traumatol ; 26(6): 556-63, 2014 Dec.
Artículo en Alemán | MEDLINE | ID: mdl-25452091

RESUMEN

OBJECTIVE: Trapeziometacarpal arthroscopy improves diagnostic and therapeutic options in pathologies of the carpometacarpal (CMC) I joint. INDICATIONS: Pathologic conditions of the CMC I joint which are not sufficiently diagnosed by X-rays or MRI. CONTRAINDICATIONS: Pathologic conditions of the CMC I joint in which trapeziometacarpal arthroscopy is not expected to make a contribution to diagnosis or therapy. SURGICAL TECHNIQUE: Trapeziometacarpal arthroscopy using a 1.9 mm arthroscope after instillation of physiological solution. POSTOPERATIVE MANAGEMENT: Thumb cast sparing the interphalangeal joint for 1 week, then functional therapy. RESULTS: Trapeziometacarpal arthroscopy improves diagnosis of CMC I pathologies and gives new therapeutic options, a field which is still rapidly expanding.


Asunto(s)
Artroscopía/métodos , Articulaciones Carpometacarpianas/lesiones , Articulaciones Carpometacarpianas/cirugía , Fracturas Óseas/patología , Fracturas Óseas/cirugía , Artropatías/patología , Artropatías/cirugía , Articulaciones Carpometacarpianas/patología , Humanos , Aumento de la Imagen/métodos , Posicionamiento del Paciente/métodos , Hueso Trapezoide/lesiones , Hueso Trapezoide/patología , Hueso Trapezoide/cirugía
11.
Unfallchirurg ; 117(8): 723-37; quiz 738-9, 2014 Aug.
Artículo en Alemán | MEDLINE | ID: mdl-25116013

RESUMEN

Injuries to the scapholunate ligament are the most frequent cause of carpal instability. Therefore, if a scapholunate lesion is not diagnosed, it may result in a severe dysfunction of the wrist. This review describes the anatomy, and the kinematics of the wrist with an intact as well as a disrupted scapholunate ligament. The diagnostic of an isolated ligament lesion and a ligament injury associated with a fracture of the distal radius is presented. Finally, an algorithm for treatment based on the stage of injury is proposed.


Asunto(s)
Ligamentos/lesiones , Hueso Semilunar/lesiones , Hueso Escafoides/lesiones , Traumatismos de los Tejidos Blandos/cirugía , Traumatismos de la Muñeca/cirugía , Humanos , Ligamentos/diagnóstico por imagen , Ligamentos/cirugía , Hueso Semilunar/diagnóstico por imagen , Hueso Semilunar/cirugía , Radiografía , Hueso Escafoides/diagnóstico por imagen , Hueso Escafoides/cirugía , Traumatismos de los Tejidos Blandos/diagnóstico por imagen , Traumatismos de la Muñeca/diagnóstico por imagen
12.
Oper Orthop Traumatol ; 25(4): 350-59, 2013 Aug.
Artículo en Alemán | MEDLINE | ID: mdl-23934299

RESUMEN

OBJECTIVE: Improvement of active forearm supination or pronation. Reduction of paralytic pronation or supination posture. INDICATIONS: Disability or impairment of active supination or pronation due to cerebral palsy, obstetric palsy or traumatic brachial plexus palsy, quadriplegia or paralysis from other causes. CONTRAINDICATIONS: Inadequate passive range of motion of forearm supination or pronation. Insufficient power of brachioradialis muscle < M4. Insufficient rehabilitation after conservative treatment or neurosurgical intervention with possible improvement of supination or pronation. Lack of patient's cooperation and compliance. SURGICAL TECHNIQUE: Exposure and mobilisation of brachioradialis muscle. Division of brachioradialis tendon distally with Z-plasty. Passing distal tendon through the interosseus space in dorsal to palmar direction for restoration of supination respectively in palmar to dorsal direction for restoration of pronation. Suturing both tendon ends. POSTOPERATIVE MANAGEMENT: Management includes an above elbow cast with the elbow in 70° flexion for 4 weeks. Then active physiotherapy to learn new brachioradialis muscle function for supination or pronation over 1-1.5 years. If needed dynamic orthesis. RESULTS: Özkan et al. performed brachioradialis rerouting to restore supination in 5 children between 4 and 14 years with pronation deformity and to restore pronation in 4 children aged 5-9 years with supination deformity. Mean active gain for supination was 81° (40-140°). Active pronation improved from 28 to 49° (30-75°; Özkan et al., J Hand Surg Br 29:263-268, 2004; Özkan et al., J Hand Surg Am 29:22-27, 2004). Between April 2006 and January 2011 we used this technique in 4 patients aged 7-26 years (mean 14 years). Three patients could be followed up. One patient had preoperative a fixed pronation deformity of the forearm in 80° pronation. In this case active range of motion could be improved to 80/30/0° pronation/supination. One patient improved from preoperative 0/0/90° pronation/supination to 30/0/90° postoperatively. In one case no functional improvement of forearm rotation could be achieved in long-term follow-up. No functional loss in forearm rotation to the opposite direction or of the elbow function was observed. Mean follow-up time was 51 months (21-77 months).


Asunto(s)
Antebrazo/cirugía , Artropatías/cirugía , Trastornos del Movimiento/cirugía , Procedimientos de Cirugía Plástica/métodos , Transferencia Tendinosa/métodos , Tendones/cirugía , Adolescente , Adulto , Niño , Femenino , Humanos , Masculino , Procedimientos de Cirugía Plástica/instrumentación , Transferencia Tendinosa/instrumentación , Resultado del Tratamiento , Adulto Joven
13.
Unfallchirurg ; 115(4): 343-52, 2012 Apr.
Artículo en Alemán | MEDLINE | ID: mdl-22367515

RESUMEN

BACKGROUND: This retrospective study examines long-term follow-up results after complete denervation of the wrist. PATIENTS AND METHODS: Between 1994 and 2000 a total of 61 complete wrist denervations of 59 patients (median age at operation 46 years) were performed. In 2009 29 patients with 30 complete wrist denervations took part in a follow-up examination after an average of 10 years. The mobility of the wrist and the grip force were examined. Pain and satisfaction with the operation were determined by means of a visual analogue scale (VAS) (0-100). The patients were asked about pain reduction and how long it lasted. Further the DASH and the Mayo Wrist Score were evaluated. Radiographs of the denervated wrist were performed and the degree of the degenerative osteoarthritis was determined according to Knirk and Jupiter. RESULTS: In 7 of the 36 patients examined, a partial or total wrist arthrodesis was performed. These patients were excluded from the study. Pain was improved in 28 of the 30 denervated wrists examined, in 22 the improvement lasted until the follow-up examination, whereas in 6 the pain increased after a median of 90 months. The median pain intensity was 10 at rest and 50 with activity; the satisfaction was 90. The median of the extension/flexion was 81% and the grip force 82% in comparison to the opposite hand. The DASH Score was 25 and the Mayo Wrist Score 73. CONCLUSION: Complete denervation of the wrist according to Wilhelm is a treatment option for the chronically painful wrist and can lead to good grip force, mobility, sufficient pain reduction and satisfaction in the long term.


Asunto(s)
Artralgia/cirugía , Desnervación/métodos , Recuperación de la Función , Articulación de la Muñeca/inervación , Articulación de la Muñeca/cirugía , Artralgia/diagnóstico , Femenino , Humanos , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Dimensión del Dolor , Resultado del Tratamiento
14.
J Clin Apher ; 27(2): 99-105, 2012.
Artículo en Inglés | MEDLINE | ID: mdl-22267052

RESUMEN

Refsum's disease is a rare autosomal recessive disorder of fatty acid metabolism. Poorly metabolized phytanic acid accumulates in fatty tissues, including myelin sheaths and internal organs, leading to retinitis pigmentosa, peripheral polyneuropathy, cerebellar ataxia, and renal, cardiac or liver impairment. Dietary restriction of phytanic acid in some cases is not sufficient to prevent acute attacks and stabilize the progressive course. Phytanic acid bound to large low density lipoproteins (LDL) and very low density lipoproteins (VLDL) molecules offers the possibility of extracorporeal elimination by lipid apheresis. We report on the long-term lipid apheresis treatment of four patients with severe Refsum's disease. Retinitis pigmentosa, peripheral polyneuropathy, cerebellar ataxia, anosmia, and sensorineural hearing loss were major symptoms exhibiting a progressive course. Lipid apheresis was performed for 5-13 years without severe complications. Maximum levels of phytanic acid before commencing chronic lipid apheresis were >300 mg/l. During steady state with lipid apheresis, mean phytanic acid before treatments was 87 mg/l and was reduced to 36 mg/l. Mean reduction rate was 59% per treatment. In all patients, abnormal motor nerve conduction velocity with signs of chronic denervation improved, morphological and functional stabilization of eye involvement was observed. Lipid apheresis prevented the extension of the disease to previously unaffected organs in three patients. Extracorporeal elimination of lipoprotein-phytanic acid complexes by lipid apheresis represents a pathophysiologically guided therapeutic approach, resulting in long-term improvement or stabilization of overall rehabilitation in patients with progressive Refsum's disease.


Asunto(s)
Eliminación de Componentes Sanguíneos/métodos , Enfermedad de Refsum/sangre , Enfermedad de Refsum/terapia , Anciano , Femenino , Humanos , Lípidos/química , Lipoproteínas/química , Masculino , Persona de Mediana Edad , Ácido Fitánico/química , Ácido Fitánico/metabolismo , Polineuropatías/metabolismo , Retinitis Pigmentosa/metabolismo , Estudios Retrospectivos
15.
Handchir Mikrochir Plast Chir ; 42(3): 204-11, 2010 Jun.
Artículo en Alemán | MEDLINE | ID: mdl-20535655

RESUMEN

PURPOSE/BACKGROUND: The integrity of the articular surfaces is of major importance for the prognosis and treatment of lunate necrosis (Kienböck's disease). Though arthroscopy is the most reliable method in the diagnosis of intraarticular pathology it is rarely applied in this condition. The purpose of this study was to evaluate the value of arthroscopy in Kienböck's disease. PATIENTS/MATERIAL AND METHODS: 20 prospectively evaluated patients underwent arthroscopy for Kienböck's disease. Initial diagnosis was performed by enhanced magnet resonance images, standard radiographies and computed tomography. Preoperative staging was performed according to the MRI-adapted classification of Lichtman and Ross . The arthroscopical measures followed standard procedures. Cartilage lesions were rated by an own modified classification. The wrists were further staged according to an arthroscopical classification system for Kienböck's disease (Bain and Begg ). The posterior and anterior interosseus nerves were resected in all patients and, if necessary, an arthroscopical debridement of the lunate performed. RESULTS: No clinical or statistical correlation was found between the Lichtman-stages and the distribution and severity of the cartilage lesions. In selected cases severe cartilage lesions (3 degrees and 4 degrees ) were already found in Lichtman-stage IIIA and IIIB, while less severe lesions than expected were observed in Lichtman-stage IV. There was no correlation between the Lichtman-stages and the classification according to Bain and Begg. In selected cases without cartilage lesions carpal collapse (Lichtman stage IIIB) was found. The individual response was unequivocal: Four patients reported minimal, five patients remarkable improvement but all did not demand further procedures. Four patients reported complete relieve. Seven patients demanded further operations (3 proximal row carpectomies, 4 STT fusions). In three of these seven cases the arthroscopical evaluation guided the further operative therapy to appropriate procedures. CONCLUSIONS: In Kienböck's disease arthroscopy more precisely detected alterations of the articular surfaces than radiological diagnostics. Radiological staging over- but also underestimated the cartilage damage. Arthroscopic results strongly influenced the further surgical treatment. Therefore in Kienböck's disease arthroscopy is of high value and recommended.


Asunto(s)
Artroscopía , Hueso Semilunar/patología , Osteonecrosis/diagnóstico , Osteonecrosis/cirugía , Adulto , Desnervación , Femenino , Estudios de Seguimiento , Humanos , Hueso Semilunar/cirugía , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Osteonecrosis/clasificación , Estudios Prospectivos , Sensibilidad y Especificidad , Tomografía Computarizada por Rayos X
16.
Handchir Mikrochir Plast Chir ; 42(3): 162-70, 2010 Jun.
Artículo en Alemán | MEDLINE | ID: mdl-20552545

RESUMEN

In recent years, our increased knowledge about the biomechanics of the wrist and reparative mechanisms in osteonecrotic bones has significantly influenced the imaging procedures in Kienböck's disease as well as its differential diagnosis. In addition to projection radiography, computed tomography (CT) is used for evaluating the osseous microstructure, and contrast-enhanced magnetic resonance imaging (MRI) for determining the viability of bone marrow. CT is essential in the morphological staging of Kienböck's disease due to its high sensitivity in detecting proximally located fractures (stage IIIa) and in visualising osteoarthritic lesions (stage IV). Bone marrow oedema of the lunate bone as well as the zonal layers including the proximal necrotic zone, the middle reparative zone, and the distal viability zone can be depicted only with the use of MRI. Furthermore, intravenous application of gadolinium is mandatory in MRI, because vascular differentiation of the osteonecrotic zone from the reparative zone is reliable only in MRI perfusion studies. By evaluating clinical, biomechanical and imaging findings synoptically, a differentiation of Kienböck's disease from ulnocarpal impaction syndromes, intraosseous ganglion cysts, the fibrocartilageous type of lunotriquetral coalition, and post-traumatic and inflammatory lesions is possible in over 80% of all cases.


Asunto(s)
Hueso Semilunar/patología , Imagen por Resonancia Magnética , Osteonecrosis/diagnóstico , Tomografía Computarizada por Rayos X , Médula Ósea/patología , Medios de Contraste/administración & dosificación , Diagnóstico Diferencial , Fracturas Óseas/clasificación , Fracturas Óseas/diagnóstico , Fracturas Óseas/patología , Gadolinio , Humanos , Infusiones Intravenosas , Isquemia/clasificación , Isquemia/diagnóstico , Isquemia/patología , Hueso Semilunar/irrigación sanguínea , Hueso Semilunar/lesiones , Angiografía por Resonancia Magnética , Osteonecrosis/clasificación , Osteonecrosis/patología , Flujo Sanguíneo Regional/fisiología , Supervivencia Tisular
17.
Handchir Mikrochir Plast Chir ; 42(3): 187-97, 2010 Jun.
Artículo en Alemán | MEDLINE | ID: mdl-20425692

RESUMEN

PURPOSE/BACKGROUND: Kienböck's disease is a rare condition in children or juvenile patients with unique features. This article presents a review of the literature on this topic and an evaluation of our own patients to help to establish therapeutic guidelines. PATIENTS/MATERIAL AND METHOD: From 1994-2005 we treated 23 patients. The 2 paediatric patients could be followed - one for 102 months after vascularised transposition of the pisiform and the other one for 24 months after conservative treatment. 15 out of the 21 juvenile patients could be followed: 9 patients on average for 47 months after radial shortening osteotomy, 5 patients on average for 94 months after partial wrist fusion and one for 24 months after arthroscopic debridement. Beginning in 2006 our therapeutic strategy has been changed. Since then one paediatric and one juvenile patient could be followed after temporary transfixation of the scaphotrapezoidal joint as well as one juvenile patient after spontaneous healing of the lunate bone. We measured motion, grip strength and pain level in rest and under load using the visual analogue scale, as well as the DASH score. Furthermore, standard X-rays of the wrist were performed. RESULTS: The 3 paediatric patients demonstrated good clinical and radiological results with healing of the lunate bone. The juvenile patients with radial shortening osteotomy had a better motion than those with partial wrist fusion. Reduction of pain level was similar in both groups. Radiologically we found no progression of lunate disease in these patients. In spite of a temporary ST transfixation the 17-year-old boy with advanced Kienböck's disease developed symptomatic progression of the disease. CONCLUSION: In paediatric and juvenile patients with Kienböck's disease we find an age-related potential for spontaneous remodelling and revascularisation. Therefore in paediatric and in juvenile patients not older than 14 years our method of choice is an immobilisation with regular gadolinium-enhanced MRI studies. In those who do not improve and in older juvenile patients we prefer temporary transfixation of the scaphotrapezoidal joint. Alternatively, in cases of the ulnar minus variant, radial shortening osteotomy or a combination of these 2 methods is indicated as the primary approach. In our opinion there is no longer an indication for partial wrist fusions as a primary procedure in these patients.


Asunto(s)
Hueso Semilunar , Osteonecrosis/diagnóstico , Osteonecrosis/cirugía , Adolescente , Artroscopía , Remodelación Ósea/fisiología , Hilos Ortopédicos , Niño , Progresión de la Enfermedad , Femenino , Estudios de Seguimiento , Humanos , Hueso Semilunar/patología , Hueso Semilunar/cirugía , Imagen por Resonancia Magnética , Masculino , Osteotomía , Dimensión del Dolor , Hueso Pisiforme/trasplante , Complicaciones Posoperatorias/diagnóstico , Remisión Espontánea , Tomografía Computarizada por Rayos X
18.
J Hand Surg Eur Vol ; 35(7): 544-8, 2010 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-20237187

RESUMEN

Lunatomalacia or avascular necrosis of the lunate in children is a rare disease not described by Kienböck. It is somewhat surprising that the term Kienböck's disease has been used to describe this disease of the lunate in children. We report our experience in 13 cases and review the literature. In children under 12, the prognosis is very good after immobilization alone. Persistent symptoms may indicate surgery, probably radial shortening, in some older teenagers although the outcome appears better than in adults. We suggest the term lunatomalacia to describe this disorder, in recognition of the term originally used by Kienböck.


Asunto(s)
Hueso Semilunar , Osteonecrosis/diagnóstico por imagen , Osteonecrosis/cirugía , Adolescente , Niño , Preescolar , Humanos , Lactante , Osteonecrosis/etiología , Radiografía
19.
Persoonia ; 23: 35-40, 2009 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-20198159

RESUMEN

Developing powerful phylogenetic markers is a key concern in fungal phylogenetics. Here we report degenerate primers that amplify the single-copy genes Mcm7 (MS456) and Tsr1 (MS277) across a wide range of Pezizomycotina (Ascomycota). Phylogenetic analyses of 59 taxa belonging to the Eurotiomycetes, Lecanoromycetes, Leotiomycetes, Lichinomycetes and Sordariomycetes, indicate the utility of these loci for fungal phylogenetics at taxonomic levels ranging from genus to class. We also tested the new primers in silico using sequences of Saccharomycotina, Taphrinomycotina and Basidiomycota to predict their potential of amplifying widely across the Fungi. The analyses suggest that the new primers will need no, or only minor sequence modifications to amplify Saccharomycotina, Taphrinomycotina and Basidiomycota.

20.
Unfallchirurg ; 111(10): 804-8, 810-1, 2008 Oct.
Artículo en Alemán | MEDLINE | ID: mdl-18806976

RESUMEN

Ischemic muscle contracture after a compartment syndrome of the forearm and hand may result in severe loss of function. In addition to the established muscle contracture, a loss of nerve and vessel function can often be found. The clinical appearance depends on the involved muscles respectively compartments. Even though each case requires individual analysis of the clinical situation, the combination of Tsuge's classification with Holden's classification provides a more or less systematic approach to treatment that can be adapted to each case according to the severity of the contracture of the joints and muscles, the degree of nerve and vessel damage, the function of the remaining muscles and nerves, and the availability of other functioning muscles for reconstruction.


Asunto(s)
Síndromes Compartimentales/cirugía , Traumatismos del Antebrazo/cirugía , Antebrazo/cirugía , Traumatismos de la Mano/cirugía , Mano/cirugía , Procedimientos de Cirugía Plástica/instrumentación , Procedimientos de Cirugía Plástica/métodos , Humanos
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