Cladribine in myasthenia gravis: a pilot open-label study.

BACKGROUND AND PURPOSE: The aim of this study was to assess the effectiveness of cladribine treatments in a population of patients with refractory myasthenia gravis (MG). METHODS: In a prospective open-label study of cladribine in refractory MG, 13 patients received cladribine at baseline with repetitive cycles driven by clinical response. A Myasthenia Gravis Composite (MGC) score was obtained and a standard dose of steroids was administered. RESULTS: A total of 11 patients achieved significant clinical improvement in MGC score during their therapy. The mean MGC score declined from 15.1 to 6.3 points within 4 months of observation. The dosage of prednisolone declined from 9.5 to 1.9 mg. None of the patients required intravenous immunoglobulin or plasma exchange treatments and no adverse events occurred in the study period. CONCLUSION: Cladribine seems to be a safe and effective emergency therapy in a population of patients with refractory MG.

3D osteoarthritic changes in TMJ condylar morphology correlates with specific systemic and local biomarkers of disease.

OBJECTIVE: To assess 3D morphological variations and local and systemic biomarker profiles in subjects with a diagnosis of temporomandibular joint osteoarthritis (TMJ OA). DESIGN: Twenty-eight patients with long-term TMJ OA (39.9 ± 16 years), 12 patients at initial diagnosis of OA (47.4 ± 16.1 years), and 12 healthy controls (41.8 ± 12.2 years) were recruited. All patients were female and had cone beam CT scans taken. TMJ arthrocentesis and venipuncture were performed on 12 OA and 12 age-matched healthy controls. Serum and synovial fluid levels of 50 biomarkers of arthritic inflammation were quantified by protein microarrays. Shape Analysis MANCOVA tested statistical correlations between biomarker levels and variations in condylar morphology. RESULTS: Compared with healthy controls, the OA average condyle was significantly smaller in all dimensions except its anterior surface, with areas indicative of bone resorption along the articular surface, particularly in the lateral pole. Synovial fluid levels of ANG, GDF15, TIMP-1, CXCL16, MMP-3 and MMP-7 were significantly correlated with bone apposition of the condylar anterior surface. Serum levels of ENA-78, MMP-3, PAI-1, VE-Cadherin, VEGF, GM-CSF, TGFßb1, IFNγg, TNFαa, IL-1αa, and IL-6 were significantly correlated with flattening of the lateral pole. Expression levels of ANG were significantly correlated with the articular morphology in healthy controls. CONCLUSIONS: Bone resorption at the articular surface, particularly at the lateral pole was statistically significant at initial diagnosis of TMJ OA. Synovial fluid levels of ANG, GDF15, TIMP-1, CXCL16, MMP-3 and MMP-7 were correlated with bone apposition. Serum levels of ENA-78, MMP-3, PAI-1, VE-Cadherin, VEGF, GM-CSF, TGFß1, IFNγ, TNFα, IL-1α, and IL-6 were correlated with bone resorption.

Fluorescent tricyclic analogues of acyclovir and ganciclovir. A structure-antiviral activity study.

Of a series of new guanine base modified tricyclic analogues of acyclovir (ACV, 1) and ganciclovir (GCV, 2), derivatives of the 3,9-dihydro-9-oxo-5H-imidazo[1,2-a]purine system, evaluated for activity against herpes simplex virus type 1 and 2, several fluorescent analogues, 6-(4-MeOPh)-TACV (8), 7-Me-6-Ph-TACV (17), 6-(4-MeOPh)-TGCV (27), and 7-Me-6-Ph-TGCV (28), were obtained that showed similar potency and selectivity as the parent compounds. The activity was found to be strongly dependent on the nature and steric demands of the substituents in the 6 and/or 7 position.

[Angiosarcoma of the hand. Case report]. / Angiosarkom der Hand. Falldarstellung.

Angiosarcoma is a very rare malignant bone tumour. The case of a 45-year-old man is presented with a primary epithelioid hemangioendothelioma in the fourth metacarpus. After radical extirpation of the tumour radiation followed. The function of the hand was fully preserved. Five years after operation the patient is free of disease.

[Efficacy of intramedullary stabilization of pathologic femoral shaft fractures]. / Effizienz der intramedullären Stabilisierung pathologischer Femurschaftfrakturen.

We report here on the treatment with intramedullary nailing or gamma nailing of 23 pathological femoral shaft fractures, 14 patients having tumors or metastases. Thirteen (13/14) tumor patients were treated palliatively and one patient radically. The results were that 10 patients were able to walk after the operation, all patients gained considerable relief from pain and nursing was facilitated. Intramedullary nailing or gamma nailing offers, in the given indications, a good alternative to the other osteosynthesis procedures.

[Revision operations in carpal tunnel syndrome]. / Revisions-Operationen beim Karpaltunnelsyndrom.

From 1975 to 1990, 1133 carpal tunnel releases were performed. Of these operations, 81 were secondary procedures, suggesting that repeat surgery for carpal tunnel syndrome is not uncommon. Of the 50 repeat procedures during the last six years, 37 were operated on due to persistent or early recurrent symptoms within fifteen months of their first operation. Technical errors and complications due to traumatic technique were found to be the underlying causes. The most frequent finding was an incompletely divided retinaculum flexorum. Twelve out of thirteen patients with an operation interval longer than fifteen months had been relieved of their complaints for months after the first operation. Some of these cases are considered to be true recurrences. In cases of persistent or recurrent symptoms after carpal tunnel release, operative revision is indicated without hesitation.

[The treatment concept and results of peri-/sub-prosthetic fractures]. / Behandlungskonzept und Ergebnisse peri-/subprothetischer Frakturen.

The treatment of choice in ipsilateral femoral shaft fractures in patients with hip prosthesis depends on the location of the fracture lines and the observation of loosening of the prosthesis. Either a change of prosthesis, if necessary in combination with refixation of the greater trochanter or simple open reduction with internal fixation (ASIF) along with autologous and/or homologus spongiosa are the used techniques. The rate of complications in peri- or subprothetic fractures is high. Lack of motion in hip and knee joint occurs frequently.

[Bundle nailing of forearm fractures. Indications and results]. / Bündelnagelung bei Unterarmfrakturen. Indikation und Ergebnisse.

Hackethal developed the nailing procedure named after him in 1959. The rationale of Hackethal nailing is an elastic jamming, which can only be achieved by obeying four rules: jamming of the nails in the cortical window, jamming then in the waist of the medullary cavity, spreading the bunch of nails in the metaphysis and filling up the conus of the medullary cavity with short nails. We confined Hackethal nailing to closed and first-degree open fractures of the 2nd-5th sixth of the shaft of the forearm. If closed reduction and nailing were impossible, we performed a plate fixation (AO), in second- or third-degree open fractures we treated with fixators (Orthofix). In a 13-year period we performed Hackethal bundle nailing in 65 patients with 115 fractures. In 54% patients surgery was performed within the first 8 hours following admission. We used two or three nails passing the fracture and one short nail. Except for 1 case, in which we needed a plaster of Paris, we achieved rational stability. On average, the nails were removed after 11.5 months. The healing and complication rates were assessed by follow-up examination of 49 patients. The results were excellent and good in 71.5% patients, satisfactory 11.4% and poor in 17.1%. Complications consisted of a 2.1% infection rate (osteitis), 3.1% non-union, 2.1% with a synostosis, 1% refracture (during removal of the nails) and 3.1% migration of nails, combined with tendon rupture. We have seen 1 case with metallosis. In conclusion with our confined spectrum of indications Hackethal nailing is a low-risk method, which leads to a rational stability and early bone healing.

[The radiologic evaluation of osteosyntheses]. / Radiologische Beurteilung von Osteosynthesen.

Surgeons, orthopedic surgeons, and neurosurgeons employ a wide variety of different osteosynthetic devices in the treatment of fractures and in spinal surgery. In order to assess these instruments correctly, the radiologist should be aware of their purpose and normal appearance. The complications should be identified, such as delayed union, pseudarthrosis, dislocation, device loosening, fracture of the device, osteomyelitis, and re-fracture. To evaluate the fixation device adequately two plain radiographs are mandatory. Normal fracture healing and the most commonly used fixation devices, the abnormalities of fracture healing and their complications are discussed.

[Modified dorsal approach to supracondylar humerus fractures]. / Modifizierter dorsaler Zugang bei suprakondylären Humerusfrakturen.

The fracture line in children's supracondylar fractures of the humerus always runs through the olecranon fossa and the radial and ulnar metaphyseal columns. As the articular capsule covers the fossa, all these fractures are intracapsular fractures with haemarthrosis. They require an anatomical reduction and a safe stabilisation in order to avoid a varus deformity. The results after conservative or operative treatment of extension type fractures are only fair, and Magerl found, that 47% of the operated cases suffered a varus deformity. We modified a dorsal approach to the elbow joint, which allows us to overlook the whole joint and the fracture site. Then anatomical reduction is easy. For stabilisation we use two K-wires for the radial and another two for the ulnar column. It is no problem to use twice the number of wires compared with other operative procedures, because the whole fracture region is exposed. Thus we get better stability and avoid secondary dislocation. We used the modified dorsal approach in 13 cases during the last two years. We have obtained excellent results, but a follow-up will be necessary in three to five years.

[Complementary osteosynthesis and dynamic axial fixation of the distal tibia]. / Komplementärosteosynthese und dynamisch-axiale Fixation an der distalen Tibia.

In view of the problems encountered with fractures of the distal tibial shaft, with or without damage to the ankle joint, we have developed a regimen of our own. We reconstruct the fracture by means of simple techniques (K-wires or one to three screws), taking the greatest possible care to avoid damage to the soft tissues. The length and axis of the lower leg are maintained by means of a unilateral fixator, which can also be used for decompression of the ankle joint in a joint-bridging modification. The two components of our regimen complement each other, and we have therefore coined the phrase "complementary osteosynthesis". Evaluation of data in 15 patients who had suffered trauma at least 1 year earlier and in whom the follow-up period was a mean of 86.8 weeks showed that results classified as good or fair can be achieved even in these problematic fractures.

[Current indications for intramedullary bundle nailing]. / Aktuelle Indikationen zur Bündelnagelung.

Hackethal developed the nailing procedure named after him in 1959. The original method consisted in two steps: a non-sterile step in which mechanical reduction was performed with a traction device and a sterile step, i.e. nailing. The rationale of Hackethal nailing is an elastic jamming, which can only be achieved by obeying four rules: jamming of the nails in the cortical window, jamming then in the waist of the medullary cavity, spreading the bunch of nails in the metaphysis and filling up the conus of the medullary cavity with short nails. Hackethal believed it was possible to achieve an elastic jamming in all transverse and short oblique fractures of the shaft. He used his technique in fractures and non-unions of femur, tibia, humerus, radius and ulna. Extensive indications led to overuse of the method and resulted in a major rate of complications. Meanwhile, plating of shaft fractures has proved to involve an unexpectedly high rate of complications, and locking nails and new types of fixators have enlarged the surgeon's armamentarium for stabilization of fractures. Therefore, it seemed important to determine the present indications for Hackethal nailing. We confined Hackethal nailing to fractures of the middle third of the shaft in the upper extremity. All second- and third-degree open fractures were treated with fixators. In a 13-year period we performed Hackethal nailing of the humerus in 53 cases, of the radius in 20, of the ulna in 17, and in both of the forearm simultaneously in 12 cases. Plating and Hackethal nailing were combined for the treatment of forearm fractures in 10 cases.(ABSTRACT TRUNCATED AT 250 WORDS)

Genu recurvatum due to partial growth arrest of the proximal tibial physis: correction by callus distraction. Case report.

The formation of a genu recurvatum after partial growth arrest of the proximal tibial physis is uncommon. This contribution deals with the case of a 16 years old male patient who after a direct injury to the proximal tibia four years ago showed a genu recurvatum of 18 degrees. An incomplete upper tibial corticotomy was performed and a hinge type de Bastiani fixator applied. The deformity was corrected at a distraction rate of one millimetre a day. The corticotomy gap filled with callus during the distraction process. The advantages of this concept are omision of iliac crest grafts, maintainance of a full range of knee motion with partial weight bearing during distraction and determination of the final degree of correction with the aid of proper radiographs.