[Covid-19: Organizational measures and ethical questions in rehabilitation center for persons with severe handicap and disability]. / COVID-19 : expériences, aspects organisationnels et éthiques dans un établissement de soins pour personnes polyhandicapées et multihandicapées.

This article is a focus on the organizational measures, decision-making and ethical questions during the different COVID-10 waves in specialized rehabilition center for persons with severe handicap and disability. The a posteriori analysis leads to a reflection on the responsability, decision-making and ethical questions.

Unusual long survival despite severe lung disease of a child with biallelic loss of function mutations in ABCA-3.

Homozygous or compound heterozygous for frameshift or nonsense mutations in the ATP-binding cassette transporter A3 (ABCA3) is associated with neonatal respiratory failure and death within the first year of life without lung transplantation. We report the case of a newborn baby girl who developed severe respiratory distress soon after birth. She was diagnosed with compound heterozygous frameshift mutation of the ABCA3 gene. Despite extensive treatment (intravenous corticosteroids pulse therapy, oral corticosteroids, azithromycin, and hydroxychloroquine), she developed chronic respiratory failure. As the parents refused cardio-pulmonary transplantation and couldn't resolve to an accompaniment of end of life, a tracheostomy was performed resulting in continuous mechanical ventilation. A neurodevelopmental delay and an overall muscular dystrophy were noted. At the age of 5 years, after 2 episodes of pneumothorax, the patient died from severe respiratory failure. To our knowledge, this was the first case of a child with compound heterozygous frameshift mutation who posed such an ethical dilemma with a patient surviving till the age of five years.

Lethal form of spinocerebellar ataxia type 7 with early onset in childhood.

Progressive cerebellar ataxias are well-known hereditary neurological disorders. Among them, spinocerebellar ataxia type 7 (SCA7) is inherited as an autosomal dominant trait and is ascribed to the expansion of a CAG trinucleotide repeat within the ATXN7 gene. An anticipation phenomenon can occur during paternal transmission and sometimes is responsible for a severe infantile form. The specificity of SCA7 is the retinal involvement with retinitis pigmentosa and cone rod dystrophy. We describe a familial form with two siblings who died of a severe infantile form. Diagnosis was made in their father, who had a recent history of macular atrophy and presented with gait disturbance thereafter. Retrospectively, substantial triplet repeat expansion was confirmed in the two affected infants. These infantile forms are rare and difficult to diagnose in the absence of suggestive family symptoms.

[Macrophage activation syndrome and Kawasaki disease: Four new cases]. / Syndrome d'activation macrophagique et maladie de Kawasaki : à propos de quatre observations.

Macrophage activation syndrome (MAS) is a rarely reported complication of Kawasaki disease (KD). It must be sought during KD with unusual clinical signs, such as enlargement of the liver or spleen, cytopenia (including thrombocytopenia), and elevated serum triglycerides. Here, we report four cases from a single center. The first is the description of a case of KD occurring in an 11-month-old child 15 days after the occurrence of infectious spondylitis. For the second, acute myocarditis heralded KD in a 5-year-old child. A 15-year-old boy had multiorgan failure with shock. The last case describes an infant with atypical KD. All had hepatosplenomegaly, thrombocytopenia, and increased serum ferritin. For all of them, hemophagocytosis was present on the bone marrow smears. The condition rapidly evolved favorably with intravenous immunoglobulins or steroids. The extensive microbiological work-up was unrevealing. None had any coronary sequelae after the episode. KD should be added to the list of inflammatory conditions that may be complicated by MAS that modifies the presentation mimicking toxic shock or infection-associated activation syndrome and makes the diagnosis difficult. This association does not appear to have a prognostic impact on the course of the disease in the literature, confirmed by the analysis of this small series.

[Infantile pyknocytosis: A cause of noenatal hemolytic anemia. Is recombinant erythropoietin an alternative to transfusion?]. / Pyknocytose infantile : une cause d'anémie hémolytique néonatale. Érythropoïétine recombinante, une alternative à la transfusion ?

Infantile pyknocytosis is a neonatal hemolytic disorder which causes anemia and icterus and is characterized by the presence of an increased number of distorted red blood cells called pyknocytes. Resolution spontaneously occurs in the first semester of life. It has been generally described as a rare entity, with an occasional family history. We report seven cases of infantile pyknocytosis observed in our hospital in 3 years. Most of the infants presented with hemolytic icterus and profound anemia that was reaching its peak by the 3rd week of life. Three neonates received one to three red blood cell transfusions, according to former recommendations. However, the following four received a treatment with recombinant erythropoietin administered subcutaneously. Only one of these four cases required a transfusion. All of them were free of hematological disease 2-3 months after completion of treatment. Infantile pyknocytosis is a recognized cause of neonatal hemolytic anemia, which requires careful examination of red cell morphology on a peripheral blood smear. The cause of this transient disorder remains unknown. Our observations show that recombinant erythropoietin therapy is effective in treating infantile pyknocytosis and increases the reticulocyte response, thus improving the hemoglobin level.

The accuracy of renal tumor biopsy: analysis from a national prospective study.

OBJECTIVE: To assess preoperative renal tumor biopsy (RTB) accuracy. MATERIALS AND METHODS: As part of the prospective NEPHRON study, data from 1,237 renal tumors were collected, including the use and results of RTB and final histology following nephrectomy. During the 6 months period of inclusion, 130 preoperative biopsies were performed. We used the kappa coefficient of the McNemar test to determine the concordance between the biopsy and the nephrectomy specimen (NS) regarding four parameters: malignant/benign status, histological subtype, Fuhrman grade and microscopic necrosis. RESULTS: Preoperative biopsies were performed in 9.7 and 11.4 % of the 667 radical and 570 partial nephrectomies, respectively. Tumor biopsy was inconclusive in 7.7 % of the cases. In 117 cases, a comparison between RTB and NS was available. Benign tumors accounted for three (2.6 %) and five (4.3 %) of the RTB and NS, respectively (κ = 0.769, good). With seven (6 %) discordant results in terms of histological subtype characterization between RTB and final pathology, RTB accuracy was considered excellent (κ = 0.882). In 33 cases (31.7 %), Fuhrman grade was underestimated at biopsy resulting in an intermediate concordance level (κ = 0.498). Tumor microscopic necrosis was identified in 12 RTB (10.4 %) versus 33 NS (28.4 %) (κ = 0.357, poor). CONCLUSIONS: RTB provides good to excellent diagnostic performance for discriminating malignancy and tumor histological subtype. However, its performance is intermediate or even poor when considering prognostic criteria like Fuhrman grade or microscopic necrosis. Thus, this possible inaccuracy should be taken into consideration when using RTB for accurate guidance of treatment strategy.

Results of surgical treatment of calcaneus insertional tendinopathy in middle- and long-distance runners.

PURPOSE: Calcaneus insertional tendinopathy in runners is common and involves important therapeutic controversies. The object of this study was to determine the delay and level of return to sport after insertional surgery in runners, with and without tendon damage. METHODS: Eighteen runners underwent surgery for insertional calcaneus tendinopathy. Nine required an exostosectomy/bursectomy, and nine others required a tendon reinsertion/autograft. All patients were clinically assessed pre- and post-operatively with AOFAS scores and post-operatively with ATRS. This series included analysis of "pure conflicts" and "severe insertional lesion" scores. If the insertional tendon was free or the lesion was smaller than 50 %, the group was classified as "pure conflict/minor tendon damage". In the situation in which a loss of tendon occurred or the tendon lesion was greater than 50 %, the group was classified as "major tendon damage". Pre-operatively, the AOFAS "overall", "pure conflicts/minor tendon damage" and "major tendon damage" groups' scores were 58.5 ± 15, 68.2 ± 8.8 and 48.9 ± 13.9/100, respectively. RESULTS: Post-operatively, the AOFAS "overall", "pure conflicts/minor tendon" and "major tendon damage" groups' scores were 93.7 ± 8.2, 93.2 ± 10.2 and 95.2 ± 5.7/100, respectively. The AOFAS score gain for each group was, respectively, 35.2 ± 19, 24 ± 17 and 46.3 ± 14.1. The ATRS "overall", "pure conflicts/minor tendon damage" and "major tendon damage" groups' scores were 81.5 ± 14.9, 78.3 ± 20.1 and 84.7 ± 6.7/100, respectively. The global sport recovery delay was 9.3 ± 4.1 months; it was 6 ± 3.3 months for the pure conflict/minor tendon damage subgroup and 10 ± 4.6 months for the severe tendon damages subgroup. CONCLUSION: Achilles insertional tendinopathy surgery on this population results in few complications with good functional results if the surgical technique is adapted to the type of tendon injury. The clinical relevance of this study is that it highlights the various forms of calcaneus insertional tendinopathy and various treatment options. The authors show that in the case of major tendon damage, time to return to sport is longer.

Parents make the difference: a randomized-controlled trial of a parenting intervention in Liberia.

BACKGROUND: The objective of this study was to evaluate the impact of a brief parenting intervention, 'Parents Make the Difference'(PMD), on parenting behaviors, quality of parent-child interactions, children's cognitive, emotional, and behavioral wellbeing, and malaria prevention behaviors in rural, post-conflict Liberia. METHODS: A sample of 270 caregivers of children ages 3-7 were randomized into an immediate treatment group that received a 10-session parent training intervention or a wait-list control condition (1:1 allocation). Interviewers administered baseline and 1-month post-intervention surveys and conducted child-caregiver observations. Intent-to-treat estimates of the average treatment effects were calculated using ordinary least squares regression. This study was pre-registered at ClinicalTrials.gov (NCT01829815). RESULTS: The program led to a 55.5% reduction in caregiver-reported use of harsh punishment practices (p < 0.001). The program also increased the use of positive behavior management strategies and improved caregiver-child interactions. The average caregiver in the treatment group reported a 4.4% increase in positive interactions (p < 0.05), while the average child of a caregiver assigned to the treatment group reported a 17.5% increase (p < 0.01). The program did not have a measurable impact on child wellbeing, cognitive skills, or household adoption of malaria prevention behaviors. CONCLUSIONS: PMD is a promising approach for preventing child abuse and promoting positive parent-child relationships in low-resource settings.

Treatment of chronic Achilles tendon rupture by shortening suture and free sural triceps aponeurosis graft.

INTRODUCTION: The Bosworth technique is old but still widely used. It involves problems of precisely determining the length of the Achilles tendon and of a volume effect in the turndown area. HYPOTHESIS: A new reconstruction technique is assessed, based on free sural triceps aponeurosis transfer without turndown, associated to tendon shortening suture. MATERIALS AND METHODS: Twenty-three patients were assessed by AOFAS score and clinical examination (plus MRI in 14 cases) at a mean 24.5 months' follow-up. Mean age was 52.1 years. Mean pre-operative AOFAS score was 63.6/100. RESULTS: Mean postoperative AOFAS score was 96.1. Mean graft length was 7.5 cm. Surgical revision was required for one case of postoperative infection. Twelve patients resumed leisure sports at their previous level by a mean 9.4 ± 2 months; three competitive sportsmen resumed sport at their previous level by a mean 7.6 months. None were dissatisfied or disappointed with their operation. MRI performed at 1 year found increased tendon volume without abnormality in 57% of cases; 43% showed abnormal images. DISCUSSION: Functional results were comparable to literature reports. It can be difficult to determine Achilles length for the Bosworth technique: this is made easier by conserving a fibrous support of a length determined with reference to the healthy side. The technique avoids aponeurosis turndown, and thus avoids the problem of plasty volume effect. The two cases of cutaneous complication occurred in the two most elderly patients, raising the question of the indications for reconstructive surgery in the elderly. The abnormalities found on MRI concerned scar tissue remodeling in patients with good or excellent clinical results. LEVEL OF EVIDENCE: Level IV, retrospective study.

Anterior cruciate ligament reconstruction with fascia lata using a minimally invasive arthroscopic harvesting technique.

Anterior cruciate ligament (ACL) reconstruction using the fascia lata has undergone a number of technical modifications since the work of Hey-Groves, MacIntosh, and Jaeger. Arthroscopy has simplified this technique, notably in the positioning of the tunnels. Minimally invasive harvesting through two lateral proximal and distal approaches considerably reduces cosmetic problems. The femoral tunnel is made from the outside to the inside using a specific targeting device, and the transplant harvest site is closed using the Jaeger procedure so as not to weaken lateral knee stabilizing structures. This procedure consists in opening the lateral intermuscular septum 1cm from the femur to let it shift laterally and allow the transplant harvesting area to be closed. This technique uses a fascia lata transplant, the harvesting of which has shown few iatrogenous complications but requires rigorous adherence to certain rules.

Adult post-traumatic radioulnar synostosis.

Post-traumatic radioulnar synostosis is a rare complication of forearm fracture. Resulting in loss of forearm axial rotation, it is functionally very disabling. The surgical indication, timing of operation, surgical technique, interest and type of adjuvant treatment are all issues with which physicians managing radioulnar synostosis must deal. No therapeutic consensus yet exists, but a wide variety of surgical techniques and adjuvant treatments are suggested. A literature review sought to identify risk factors for synostosis, with a view to prevention and determining a suitable therapeutic attitude in the light of existing data.

High independent prognostic and predictive value of circulating tumor cells compared with serum tumor markers in a large prospective trial in first-line chemotherapy for metastatic breast cancer patients.

BACKGROUND: Circulating tumor cells (CTCs) are a prognostic marker in metastatic breast cancer, but comparisons with serum tumor markers (CA 15-3, carcinoembryonic antigen and lactate dehydrogenase) variations are needed. PATIENTS AND METHODS: CTCs were counted with CellSearch® at baseline, before cycle 2 (C2) and cycle 3 or 4 (C3/4) in 267 metastatic breast cancer patients on first-line chemotherapy with/without targeted therapy. RESULTS: Baseline CTC detection rate was 65% with ≥1 CTC/7.5 ml threshold and 44% with ≥5 CTC/7.5 ml and was independent of subtypes (luminal, triple negative, human epithelial growth factor receptor 2 (HER2)+). CTCs were associated with tumor markers, bone/liver involvement, tumor burden and performance status. CTC detection ≥1 CTC/7.5 ml was a strong prognostic factor for progression-free survival (PFS), P < 0.0001. Threshold of CTC ≥5 was statistically significant for PFS and overall survival (OS), P = 0.03 on multivariate analysis. Among patients with ≥5 CTC/7.5 ml at baseline, 50% had <5 CTC/7.5 ml at C2. Changes were correlated with both PFS and OS (P < 0.0001). All patients receiving anti-HER2 therapy had <5 CTC/7.5 ml after three cycles of treatment. CONCLUSION: This is the largest prospective series validating the prognostic value of CTC independently from serum tumor marker. Elevated CTCs before C2 are an early predictive marker of poor PFS and OS, which could be used to monitor treatment benefit. CTC decrease under treatment seems stronger with targeted therapy.

Spatial and temporal distribution of zooplankton related to the environmental conditions in the coral reef lagoon of New Caledonia, Southwest Pacific.

The distribution of zooplanktonic prey of fish larvae was examined in three bays and two lagoonal stations in the Southwest lagoon of New Caledonia. Water column conditions were characterized by increasing chlorophyll a and particulate organic matter (POM) concentrations from the lagoon to the estuarine bay. The mean zooplankton settled volume and total density were significantly higher in the estuarine bay, reaching 35.1 mL m(-3) and 3.5 x 10(5) individuals m(-3), respectively. The total zooplankton density also progressively increased along the sampling period. The composition of assemblages differed between the lagoon and the bays, and was similar in the three bays. Wind speed, surface temperature, chlorophyll a and POM explained these variations, as revealed by a co-inertia analysis (COIA). The prey preferred by fish larvae, i.e. small crustaceans and small copepods, were more abundant in bays. Sheltered bays, most influenced by terrigenous inputs, are likely to provide the best feeding conditions.

[Should temperature be monitorized during kidney allograft preservation?]. / Faut-il monitorer la température pendant la phase de préservation en transplantation rénale ?

GOAL: It is generally considered that kidney grafts should be preserved at 4 degrees C during cold storage. However, actual temperature conditions are not known. We decided to study the temperature levels during preservation with the Biotainer storage can and Vitalpack transport pack. MATERIAL: Temperature was monitored using the Thermobouton probe during preservation of pig kidneys, in the same conditions used with human grafts. The probe recorded the temperature level every 10 minutes during four days. We compared the results found with the new storage can with results obtained in the same conditions with the storage can formerly used by our team. We also studied the best position of the probe for temperature monitoring and the influence of the amount of ice within the transport pack on the temperature level. We then monitored the temperature during the conservation of actual human kidney grafts harvested at our institution from August 2007 to May 2008. RESULTS: The temperature levels were the same regardless of the position of the probe within the transport pack. The lowest temperature was maintained during 15 hours, and the temperature level stayed below 5 degrees C for 57 hours with the new storage can. The former storage can maintained the lowest temperature level for 80 minutes, and temperature reached 5 degrees C after 10 hours 40 minutes. Temperature levels were similar when 2 or 4 kg of crushed ice were used. We observed similar results when monitoring the conservation of human grafts. CONCLUSION: The new storage can affords more stable temperature levels when compared to the formerly used can. Since temperature is stable during conservation, continuous monitoring in everyday practice does not seem warranted.

[Early failure of anterior bone block in multidirectional shoulder instability: a case report]. / Echec précoce d'une butée antérieure d'épaule dans le traitement d'une instabilité multidirectionnelle.

We report the case of a 47-year-old female patient who developed predominantly anterior multidirectional glenohumeral instability. After rehabilitation, this patient was treated surgically with an anterior coracoid bone block using the Latarjet technique. The second postoperative day, the control scan revealed posterior glenohumeral dislocation. The patient underwent a second operation for a posterior iliac block. At last clinical follow-up (six years), the patient had normal shoulder motion; shoulder function was considered excellent using the clinical scores. We recall the pathogenic hypotheses explaining multidirectional shoulder instability and discuss the proposed treatments. This case illustrates the difficult diagnosis and therapeutic management of multidirectional instability. Under general anesthesia, examination showed that posterior laxity predominated while paradoxically, the patient presented predominantly anteromedial dislocations. This case is also exceptional by the development of posterior dislocation, an unusual postoperative complication after an anterior bone block; we describe the therapeutic options. In the event of multidirectional instability with bone injuries, we recommend a block in the direction of the instability. Preoperative examination under general anesthesia can be helpful in understanding the mechanism of residual postoperative instability, which would require complementary surgical stabilization.

[Neuromuscular and architectural alterations of the vastus lateralis muscle in elderly patients with unilateral knee osteoarthritis]. / Altérations architecturales et neuromusculaires du muscle vastus lateralis chez des patients âgés atteints de gonarthrose unilatérale.

AIM: The aim of this study was to determine the mechanisms involved in muscle weakness in elderly patients with unilateral knee osteoarthritis. SUBJECTS: We investigated 7 patients with unilateral knee osteoarthritis. METHOD: We measured knee position sense and isometric maximal voluntary contraction (MVC) of the knee extensors. Electromyographic (EMG) measurement of biceps femoris (BF), rectus femoris (RF), vastus lateralis (VL) and vastus medialis (VM) involved different levels of contraction (25, 50, 75 and 100% MVC). Neuromuscular efficiency of quadriceps was also calculated (MVC/EMG). Ultrasonography was used to investigate the VL architectural parameters at the median part of the VL for different levels of contraction (25, 50, and 75% MVC). All tests were performed on the osteoarthritic and healthy knees. RESULTS: The quadriceps MVC of the affected knee was reduced by 30%. The VL thickness of the affected knee was 10% smaller than that of the unaffected knee. VL activity seen on EMG did not differ between knees, but RF, VM and BF activity was greater in the unaffected than affected knee. Neuromuscular efficiency was higher (26%) in the unaffected knee. Knee position sense was reduced by 33% in the affected knee. CONCLUSION: Quadriceps weakness associated with knee osteoarthritis seems to be related to changes in muscle rather than pennation angle and changes in fascicule length.

[Surgical treatment of ureteral stenosis after kidney transplantation]. / Traitement chirurgical des sténoses urétérales après transplantation rénale.

Ureteral stenosis is the most frequent complication after kidney transplantation. The diagnosis is based on an elevated creatinine level and a dilatation of the urinary cavity. The first step of the management is a urinary diversion with endo-ureteral prosthesis or a nephrostomy placement. The surgical gold standard is the pyelo-ureterostomy. This is a safe and efficient technique in the treatment of this complication. We describe the different surgical alternatives and the endo-urologic management of the stenosis.

Use of the SCOT solution in kidney transplantation: preliminary report.

SUBJECT: This article reports preliminary findings relating to the use of a new preservation solution, the Solution de Conservation des Organes et des Tissus (SCOT), in renal transplantation. This fourth-generation solution combines an extracellular-like composition with 20 kDa polyethylene-glycol, known for its cell-protection capacity and immunocamouflage properties. METHODS: We have reported preliminary data obtained in 29 transplantations (25 cadaveric donors and 4 living related donors) between December 2004 and November 2005. The SCOT solution was used for both in situ flush and static preservation. We compared primary organ nonfunction and delayed graft function rates as well as blood creatinine levels in recipients of grafts processed with SCOT solution, versus University of Wisconsin solution (paired for age with the previous group) and with EuroCollins solution (historic data). RESULTS: We observed delayed graft-function in 10% of the SCOT-processed group, 14% in the University of Wisconsin solution group, and 23% of the EuroCollins group. No case of primary organ nonfunction was reported. Creatinine levels in both SCOT and University of Wisconsin solution groups were similar. We did not observe any humoral or cellular graft rejection in the first 3 months after transplantation. In light of these preliminary results, the use of SCOT is safe for kidney preservation in the human setting. Further experience is warranted to assess the clinical consequences of its immunocamouflage properties as described in animal models.

[Sequelae of intra-articular calcaneal fractures: patterns and management]. / Chirurgie des séquelles des fractures du calcanéum: à propos de 24 cas.

PURPOSE OF THE STUDY: We reviewed retrospectively 24 feet with sequelae of transtalar process fractures of the calcaneum in order to identify the lesion pattern and determine optimal management options, both for acute and sequelar lesions. MATERIAL AND METHODS: There were fourteen men and nine women, mean age 42 years (19-73). Twenty-three had subtalar osteoarthritis, eight had calcaneocuboid osteoarthritis, and fifteen had lateral submalleolar conflicts. There were twelve fibular tendon dislocations or fissurations, three tarsal tunnel syndromes, and two plantar splinters. Prior to treatment, all patients complained of pain. Preoperatively, walking distance was less than 500 m for thirteen patients, 2000-3000 m for four, and greater than 3000 m for five. Mean subtalar joint motion was 30% (0-100%) compared with the healthy side and mean frontal misalignment of the rear foot was 6 degrees valgus. Physical examination, podoscopy and x-rays were obtained in all patients. The Kitaoka score was noted. RESULT: Mean follow-up was 36 months (24-72). Sequelae were treated with a single procedure or with combinations: subtalar arthrodesis (n = 23) including one in association with calcaneocuboid arthrodesis, tension on fibular tendons (n = 7), neurolysis of the posterior tibial nerve (n = 3), resection of plantar splinters (n = 2), resection of the lateral shell (n = 14), and osteotomy (n = 2) to lower the greater tubercle of the calcaneum because of pain when wearing shoes. The mean Kitaoka function score was 31.7/100 (14-79) preoperatively. After treatment, the mean score was 81.7/100 (31-94), giving a 73.2% gain. The outcome was considered good in sixteen feet, fair in six, and poor in two. Mean walking distance was greater than 3000 m for 18 patients. Mean frontal misalignment of the rear foot under loading was 4.5 degrees valgus and the podoscopy demonstrated flat foot in thirteen patients. Three subtalar arthrodesis required revision for nonhealing. DISCUSSION: Initial treatment of a fracture, particularly an articular fracture, of the calcaneum must avoid disabling postoperative pain and shoe wearing problems. These sequelae basically concern: subtalar and calcaneocuboid arthritis, lateral submalleolar conflict, fibular tendon injury, plantar splinters, tarsal tunnel syndrome, loss of height, and misalignment of the rear foot. At the sequelar stage, the physical examination is primordial to confirm the lesion and search for any complication which could develop later postoperatively when walking distance becomes longer. For nine patients with residual pain, four resulted from lesions which were missed at the preoperative physical examination. Arthrodesis of the subtalar joint should be preferred over realignment of the rear foot and can be associated with the treatment of conflicts. This management scheme allows treating during a single operative time all sequelae, thus limiting recovery time. A scan of the ankle and foot with or without opacification of the fibular tendons is needed to confirm the physical examination which, for us, remains the key to successful surgery.