Reconstructive surgery for patellofemoral joint incongruency.

PURPOSE: A retrospective analysis of a heterogeneous patient cohort was performed to determine the outcome and eligibility of a combined trochleaplasty and soft tissue-balancing technique for repair of patellofemoral joint disorders. METHODS: A strict surgical treatment algorithm including trochleaplasty and reconstruction of the medial patellofemoral ligament and vastus medialis oblique muscle was implemented to restore the patellofemoral joint. A heterogeneous patient cohort including 46 consecutively treated symptomatic knees was reviewed. The median follow-up period was 4.7 years (range 24-109 months). RESULTS: No patellar redislocation occurred post-operatively, and the median Kujala score improved from 62 (9-96) to 88 (47-100) points (p < 0.001) at follow-up. Radiological signs of trochlear dysplasia were corrected, and both patellar height and trochlear depth were significantly restored after surgery. In total, 16% of affected patients with pre-existing patellofemoral degenerative changes showed progression of osteoarthrosis according to the Kellgren and Lawrence classification. CONCLUSION: The surgical combination of trochleaplasty and reconstruction of the medial patellofemoral ligament and vastus medialis oblique muscle offers excellent clinical and radiological results. The overall results of the present study showed significant improvement of the Kujala score in patients with Dejour grades C and D dysplasia. These results outline the clinical relevance of trochleaplasty with additional soft tissue balancing as an effective joint-preserving method with satisfying results in patients with pre-existing degenerative changes. LEVEL OF EVIDENCE: IV.

[Eosinophilia: from neoplastic disease to anguillulosis]. / Eosinophilie: de la néoplasie à l'anguillulose.

Hypereosinophilia (HE), defined as eosinophil count above 0.5 G/l in the peripheral blood. Most cases are secondary to other diseases. Etiological diagnosis remains complex especially as causes of HE vary across the population. Allergic diseases and parasitic infections are the predominant causes. However up to 10% of HE are secondary to tumors. Here, we describe a case of HE initially attributed to a breast cancer that turn out to be due to an angillulosis acquired just before the diagnosis of the tumor.

Identification of a novel imatinib responsive KIF5B-PDGFRA fusion gene following screening for PDGFRA overexpression in patients with hypereosinophilia.

Idiopathic hypereosinophilic syndrome (IHES) is a disease that is difficult to classify, and diagnosis is one of exclusion. The identification of a cytogenetically invisible interstitial deletion resulting in the fusion of FIP1-Like-1 (FIP1L1) to platelet-derived growth factor receptor alpha (PDGFRA) has enabled many IHES cases to be reclassified as chronic eosinophilic leukemia. As it is likely that PDGFRA may fuse to other partner genes, we established a reverse transcriptase-PCR test to detect specific overexpression of the PDGFRA kinase domain as an indicator of the presence of a fusion gene. Overexpression was detected in 12/12 FIP1L1-PDGFRA-positive patients, plus 9/217 (4%) patients with hypereosinophilia who had tested negative for FIP1L1-PDGFRA. One of the positive cases was investigated in detail and found to have a complex karyotype involving chromosomes 3, 4 and 10. Amplification of the genomic breakpoint by bubble PCR revealed a novel fusion between KIF5B at 10p11 and PDGFRA at 4q12. Imatinib, a known inhibitor of PDGFRalpha, produced a complete cytogenetic response and disappearance of the KIF5B-PDGFRA fusion by PCR, from both genomic DNA and mRNA. This study demonstrates the utility of screening for PDGFRA kinase domain overexpression in patients with IHES and has identified a third PDGFRA fusion partner in chronic myeloproliferative disorders.

Cerebral amyloid induces aberrant axonal sprouting and ectopic terminal formation in amyloid precursor protein transgenic mice.

A characteristic feature of Alzheimer's disease (AD) is the formation of amyloid plaques in the brain. Although this hallmark pathology has been well described, the biological effects of plaques are poorly understood. To study the effect of amyloid plaques on axons and neuronal connectivity, we have examined the axonal projections from the entorhinal cortex in aged amyloid precursor protein (APP) transgenic mice that exhibit cerebral amyloid deposition in plaques and vessels (APP23 mice). Here we report that entorhinal axons form dystrophic boutons around amyloid plaques in the entorhinal termination zone of the hippocampus. More importantly, entorhinal boutons were found associated with amyloid in ectopic locations within the hippocampus, the thalamus, white matter tracts, as well as surrounding vascular amyloid. Many of these ectopic entorhinal boutons were immunopositive for the growth-associated protein GAP-43 and showed light and electron microscopic characteristics of axonal terminals. Our findings suggest that (1) cerebral amyloid deposition has neurotropic effects and is the main cause of aberrant sprouting in AD brain; (2) the magnitude and significance of sprouting in AD have been underestimated; and (3) cerebral amyloid leads to the disruption of neuronal connectivity which, in turn, may significantly contribute to AD dementia.

Use of echocardiography to assess function of hDAF-transgenic pig cardiac xenografts.

The current standard of hand palpation may not be a sensitive method to detect rejection in heterotopic heart xenotransplants (HHTx). We sought to assess the use of echocardiography to detect rejection of pig heart xenografts. Four cynomolgus monkeys received HHTx from hDAF-transgenic pigs. Immunosuppression was cyclophosphamide induction, cyclosporine, steroids, sodium mycophenolate, alphaGal trisaccharide polymer, +/-soluble complement receptor type 1. Echocardiography was performed immediately after HHTx and three times a week postoperatively. Contractility on echo was scored as 1(none), 2(severely impaired), 3(moderate to severely impaired), 4(moderately impaired), 5(mild to moderately impaired), 6(mildly impaired), or 7(normal). Left ventricle wall thickness (LVWT) was measured in the anterior, inferior, posterior, lateral, and septal walls, the average was calculated. Impaired contractility or increase in LVWT were considered rejection and treated with steroids (solumedrol 15 mg/kg IV for 3-5 days). Palpation score (4-strong to 1-none) was recorded daily. Myocardial biopsies were obtained infrequently. At the time of first rejection, all four monkeys had an increase in LVWT and a decrease in contractility on echocardiography. Steroid treatment enhanced contractility in four monkeys and decreased LVWT in three monkeys. Palpation score remained at four of four during initial rejection episodes. Decrease in contractility and increase in LVWT on echocardiography appear to signify graft injury because steroid treatment results in improvement. Compared to palpation, echocardiography is more sensitive for assessing function of heterotopic pig heart xenografts. Echocardiography has, therefore, the potential to detect and treat early rejection episodes of heterotopic heart xenografts in nonhuman primates. This may help to achieve longer graft survival.

3D-Reconstruction of microglia and amyloid in APP23 transgenic mice: no evidence of intracellular amyloid.

Microglia cells are closely associated with compact amyloid plaques in Alzheimer's disease (AD) brains. Although activated microglia seem to play a central role in the pathogenesis of AD, mechanisms of microglial activation by beta-amyloid as well as the nature of interaction between amyloid and microglia remain poorly understood. We previously reported a close morphological association between activated microglia and congophilic amyloid plaques in the brains of APP23 transgenic mice at both the light and electron microscopic levels [25]. In the present study, we have further examined the structural relationship between microglia and amyloid deposits by using postembedding immunogold labeling, serial ultrathin sectioning, and 3-dimensional reconstruction. Although bundles of immunogold-labeled amyloid fibrils were completely engulfed by microglial cytoplasm on single sections, serial ultrathin sectioning and three-dimensional reconstruction revealed that these amyloid fibrils are connected to extracellular amyloid deposits. These data demonstrate that extracellular amyloid fibrils form a myriad of finger-like channels with the widely branched microglial cytoplasm. We conclude that in APP23 mice a role of microglia in amyloid phagocytosis and intracellular production of amyloid is unlikely.

[Lupus erythematosus, circulating anticoagulant and obstetric complications. Apropos of 2 cases]. / Lupus érythémateux, anticoagulant circulant et complications obstétricales. A propos de deux cas.

Systemic lupus erythematosus (SLE) is a disease occurring mostly in women in the childbearing age. We present two cases of pregnancy complicated one by SLE, the other by a "lupus-like" syndrome. In the first case, the fetus suffered from a complete heart block; in the second one, a lupus anticoagulant was discovered in the mother. These two cases are discussed by the light of the literature.

[History of gynecologic treatment of infertility]. / Zur Geschichte der gynäkologischen Behandlung der Infertilität.

Within less than 20 years this branch of the infertility treatment by new reproductive medical technologies has left behind the original concept of bypassing blocked tubes. It has started to bypass nature. As the extracorporal way, however of shortest duration within the human prenatal development, exposes the human being to external influences, the discussion about the pros and cons of the new technologies is already abundant and still rising. The field named infertility-treatment was once in the middle and in the heart of the gynecologic discipline. It gave rise to the most intellectual branch of gynecology, the gynecologic endocrinology. Undoubtedly, there are tendencies at present to make "Reproductive Medicine" an independent discipline splitting from gynecology and obstetrics, with only the core remaining gynecology, but with the addition of essential constituents coming from genetics, human biology, andrology, medical ethics, even from sociology and law. Gynecology would tremendously suffer from this loss. The field "Reproductive Medicine" has gained the most attentive audience far beyond the scope of our gynecologic profession. One thinks it should have calm and peace to grow further in the same way other fields of medicine flourish, guided by science, concience and compassion. The juxta-medical expansion of the field could as well not have been foreseen one-hundred years ago.

Miniaturization (0.2 g) and evaluation of attachment techniques of telemetry transmitters.

We have developed a miniaturized very high frequency (VHF) transmitter design for radio-tagging small animals. The average mass of the circuitry is 0.084 g (range 0.081-0.087), hence, with the smallest power cell, complete tags weigh 0.2 g and have a life of 18-22 days. We further demonstrate that with such small tags the technique of attaching the device to the animal's body strongly affects the effective radiated power, and thus transmission ranges. Ideally the attachment couples the electronics to the animal's body as a ground plane for improved radiation. The transmitter allows the application of radio-tracking to be expanded to new taxa whose spatial behaviour and population dynamics are largely unknown, for example arthropods, reptiles or amphibians. The new design is also suitable for miniature implants and signal modulation with sensors.

Electrically switchable diffractive optical element for image processing.

An electrically switchable diffractive optical element has been built based on a computer-generated phase hologram and a liquid-crystal layer. The design of the diffractive optical element and the fabrication and performance of the device are discussed. The application of the device in a machine vision system for optical surface inspection is shown.

Linearly polarized light with axial symmetry generated by liquid-crystal polarization converters.

Novel liquid-crystal devices are described that generate linearly polarized light with axial symmetry; the beam propagation axis is the symmetry axis. Such light fields can be characterized by a polarization order number P. For example, P = 1 fields represent radially or azimuthally polarized light. The reorientation of the polarization orientation in these polarization converters is due to the twisted nematic effect and the effect of lambda/2 wave plates. A single polarization converter can generate fields of orders 1 and 2. It is shown that one can in principle generate fields of any integral order P by cascading such elements. Devices that generate P = 1 fields are achromatic and can be used as polarization axis finders or as versatile tools for studying birefringent or polarizing materials.

[The value of anticardiolipin antibody assessment]. / Intérêt du dosage des anticorps anticardiolipine.

Anticardiolipin antibodies (ACL) are associated with the presence of several clinical conditions. To determine the clinical interest of their evaluation, we reviewed all the results of ACL determinations performed in our laboratory during 1990 and 1991. 266 analyses (232 patients) were carried out. Determinations of IgG and IgM type ACL were performed by ELISA. ACL values were positive or slightly positive in 40% of the analyzed samples. Diagnosis was mentioned as the grounds for 212 requests (186 patients). ACL were positive (> m + 3SD) in about 40% of patients with autoimmune disease or with at least two of the following pathologies: autoimmune disease, venous or arterial thromboembolism, recurrent abortion.

[Poor fibrinolytic response to venous occlusion: defect of endothelial cells or plasma interference?]. / Mauvaise réponse fibrinolytique à l'occlusion veineuse: défaut des cellules endothéliales ou interférence plasmatique?

To better characterize the plasminogen activators (v-PA) before and after venous occlusion, the euglobulins obtained from the plasma of 24 volunteers have been separated on SDS-PAGE and revealed zymographically. All the subjects with an excellent fibrinolytic response on fibrin plates (greater than 1.3 TAU/ml) exhibited activity of 68 kdaltons, which corresponds to free v-PA. The poor responders on fibrin plates (less than or equal to 0.3 TAU/ml) only showed increase of a lysis zone at 110 kdaltons, corresponding to a complex of v-PA and a fast-acting inhibitor. Hence poor fibrinolytic activity on fibrin plates after venous occlusion could be due to high plasma concentration of this inhibitor and not necessarily to defective production or release of v-PA.

Relationship between plasma insulin levels and high density lipoprotein cholesterol levels in healthy men.

Insulin and high density lipoproteins are considered to play a role in the development of atherosclerosis. In order to study whether there was a relationship between endogenous plasma insulin response and high density lipoproteins, an acute intravenous glucose tolerance test (0.5 g glucose/kg body weight) was performed in 94 healthy men, aged 20-49 years. Cholesterol and triglyceride levels were measured in very low density lipoproteins, low density lipoproteins and high density lipoproteins isolated from fasting serum by preparative ultracentrifugation. The subjects were divided into quartiles according to their fasting and post-glucose load plasma insulin and high density lipoprotein cholesterol levels. The results obtained in the subjects of the upper quartiles were compared with the results obtained in the subjects of the lower quartiles. The mean glucose disappearance rates were within the normal range and did not differ between the upper and lower quartiles. Subjects with high fasting plasma insulin had lower high density lipoprotein cholesterol levels (1.11 +/- 0.34 mmol/l, p = 0.01) than men with low fasting plasma insulin (1.40 +/- 0.37 mmol/l). Higher mean post-glucose plasma insulin was associated with lower high density lipoprotein cholesterol levels (1.18 +/- 0.32 mmol/l, p less than 0.05) and increased high density lipoprotein triglyceride levels (0.14 +/- 0.07) mmol/l, p less than 0.01) when compared with the men with low post-glucose plasma insulin (1.40 +/- 0.36 mmol/l and 0.09 +/- 0.03 mmol/l respectively). These observations reflect the close relationship between endogenous insulin and lipoprotein metabolism.

Resistant pure red cell aplasia after allogeneic stem cell transplantation with major ABO mismatch treated by escalating dose donor leukocyte infusion.

We report a case of pure red cell aplasia (PRCA) following allogeneic stem cell transplantation (SCT) with major ABO mismatch which proved resistant to all standard treatment options such as change in immunosuppressive treatment, high-dose erythropoietin (EPO) or plasma exchange. We therefore proceeded to administer five cycles of Rituximab therapy, without success. Finally, escalating doses of donor-derived leukocyte infusion (DLI) resolved the PRCA of our patient 415 d after bone-marrow transplantation (BMT) and 140 d after the first infusion of donor leukocytes. A review of the literature shows the efficacy of various treatments; the role of DLI and other treatment options are discussed. Furthermore, the underlying pathophysiological mechanisms especially with regard to the role of NK cells in alloreactivity after allogeneic SCT are explained.

Peripheral neuropathy associated with high-dose Ara-C therapy.

Central nervous system toxicity associated with high-dose cytosine arabinoside (Ara-C) therapy (HD Ara-C) is well known. The authors report the case of a severe isolated peripheral polyneuropathy due to HD Ara-C. Electrophysiologic changes and histologic observations were consistent with axonal degeneration and scattered destruction of myelin sheaths. This observation emphasizes the need for careful complete neurologic evaluation for patients receiving HD Ara-C treatment.