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Biomedical subjects

F Launay

Publications and source records attributed to F Launay.

At least 19 recordsLinked to original sources

[Rituximab induced remission of pemphigus vulgaris: 2 cases].

INTRODUCTION: Pemphigus vulgaris frequently requires corticoids and immuno-suppressive drugs. The disease and the side effects of the drugs severely affect the quality of life, and sometime the vital prognosis of the patients. Other treatments than corticosteroids and immunosuppressive drugs are needed. EXEGESIS: We report 2 additional cases of pemphigus vulgaris uncontrolled by corticoids and immuno-suppressive drugs that responded spectacularly to rituximab. One patient had a recently onset disease, that was active despite 1,5 mg/kg/day prednisone and 1,5 g/day mycophenolate. She had a complete remission during 15 months after rituximab treatment. At relapse, another rituximab cycle led to a prompt remission. The other patient had longstanding pemphigus vulgaris complicated by cutaneous infections on prednisone (20 mg/d), immunosuppressive drugs and intravenous immune globulins. She had a prompt and complete remission after rituximab. CONCLUSION: Rituximab seems to be a promising drug for refractory pemphigus vulgaris. The benefit to risk ratio of this drug in this new indication must be precisely documented.

Antibodies, Monoclonal↗

[Necrotizing cellulitis complicating varicella in two children given nonsteroidal anti-inflammatory drugs].

We report two cases of fasciitis with necrotizing hypodermitis of the foot and arm which complicated varicella in immunocompetent children given nonsteroidal anti-inflammatory drugs. The skin barrier and immune function are weakened by the varicella zoster virus. Exposure to nonsteroidal anti-inflammatory drugs further favors necrotizing cutaneous infections caused by group A beta hemolytic streptococci. MRI can confirm the presence of superficial aponevrosis necrosis defining necrotizing fasciitis but should not retard surgical management which is always indicated for necrotizing fasciitis and sometimes for necrotizing dermo-hypodermitis.

Anti-Inflammatory Agents, Non-Steroidal↗

Pulmonary involvement in Sweet's syndrome: a case report and review of the literature.

Pulmonary involvement in Sweet's syndrome (SS) is rare. We report a case of SS with severe respiratory involvement responding to corticosteroid therapy. A 82-year-old man presented fever of 39 degrees C associated with cough and dyspnea, and crackles in the left lung. The infection work-up was negative. Chest X-ray showed cardiomegaly and left lower lobe pulmonary infiltrates. Pulmonary signs did not improve on treatment with antibiotics, and after 1 week maculopapular lesions appeared, localized on the knees, the periombilical area and the back. The antibiotics were changed without improvement. A skin biopsy revealed infiltration by neutrophilic granulocytes and marked edema in the dermis, consistent with SS. The patient's condition progressively worsened, requiring high oxygenotherapy, and he was transferred to an intensive care unit. Chest X-ray revealed an important alveolar and interstitial syndrome. Bronchoalveolar lavage found 170 leukocytes with 30% neutrophils (N < 5%), 7% lymphocytes and 63% macrophages. A search for bacteria, viruses or parasites in bronchoalveolar lavage was negative. The patient was treated with antibiotics, a high dose of furosemide and steroids for 4 days. Because the patient improved dramatically within 5 days, with a negative infection work-up and a dramatic decrease of C-reactive protein, the antibiotics were stopped. Steroids were secondarily tapered very slowly. A chest computed tomography (CT) scan showed a substantial improvement of pulmonary lesions. We also review the 22 cases of pulmonary involvement of SS reported in the literature.

Aged, 80 and over↗

Titanium elastic nailing of fractures of the femur in children. Predictors of complications and poor outcome.

Between 1996 and 2003 six institutions in the United States and France contributed a consecutive series of 234 fractures of the femur in 229 children which were treated by titanium elastic nailing. Minor or major complications occurred in 80 fractures. Full information was available concerning 230 fractures, of which the outcome was excellent in 150 (65%), satisfactory in 57 (25%), and poor in 23 (10%). Poor outcomes were due to leg-length discrepancy in five fractures, unacceptable angulation in 17, and failure of fixation in one. There was a statistically significant relationship (p = 0.003) between age and outcome, and the odds ratio for poor outcome was 3.86 for children aged 11 years and older compared with those below this age. The difference between the weight of children with a poor outcome and those with an excellent or satisfactory outcome was statistically significant (54 kg vs 39 kg; p = 0.003). A poor outcome was five times more likely in children who weighed more than 49 kg.

Adolescent↗

[Evaluation of balance in neurologic and geriatric disorders].

OBJECTIVE: To analyse the clinical usefulness and metrological properties of the main techniques and indices used to assess balance disorders. METHODS: More than 4000 abstracts referenced in MEDLINE and dealing with postural control and postural disorders (wide screening) were reviewed to determine the main postural techniques and indices used in a clinical context. We retained abstracts with a high citation frequency and those with interesting findings. Corresponding key words were identified for a specific search of articles that we analysed. RESULTS: Postural assessment tools can be classified as scales of ordinal items, tests based on metric or chronometric measurement, posturography, and verticality perception. These techniques are complementary, and their association is recommended in a clinical context. Regarding generic tools, the Falls-related Efficacy Scale (FES) and the Activities-specific Balance Confidence scale (ABC scale) would be enhanced if comparatively analysed and reworked to allow for a feasible and reliable assessment of the fear of falling. Despite a wide diffusion in numerous postural fields worldwide, the Berg Balance Scale (BBS) and the Functional Reach Test (FRT) do not have the required criteria to remain the gold standards they were in the 1990s. Static posturography should be normalised and yield more reliable indices. The clinical relevance of the subjective assessment of visual, haptic, and postural verticals are questionable, especially to explain postural disability. Regarding specific tools, the Tinetti test (TT) and the Time Up and Go test (TUG) are the most suited to assess postural capacities in very elderly people, in whom the predictive validity of the postural assessment of falls is still modest. In stroke patients, the Postural Assessment Scale for Stroke (PASS), posturography, lateropulsion assessment, and vertically perception are interesting and complementary techniques. Postural assessment relies mainly upon the 5 postural items of the Unified Parkinson Disease Rating Scale (UPDRS) in people with Parkinson disease and upon the Romberg test and posturography in patients with cerebellar or proprioceptive ataxia. Some novel postural scales for patients with multiple sclerosis or spinal cord injury are also emerging. CONCLUSION: Among numerous tools that contribute to the assessment of postural disorders, only the most recent ones (developed in the last 10 years) have undergone complete validation. It is now crucial to compare these tools, not only in terms of reproducibility and internal consistency, but also overall, in terms of feasibility, responsiveness, and predictive validity for a given population.

Aged↗

[Radiological index as a predictive factor of kyphosis in spina bifida].

PURPOSE OF THE STUDY: The aim of this work was to validate the pertinence of a radiological index (P/A) used as a predictive factor for risk of kyphosis in children with spina bifida. P/A is defined as the ratio between P, the distance between the posterior vertebral wall and the remnant of the posterior arch, over A, the distance between the anterior wall and the posterior vertebral wall, measured on the lateral view of the spine in the most dysplastic vertebra before one year of age. MATERIAL AND METHODS: The files of 163 children were reviewed. The value of the P/A ratio was measured on lateral views of the spine obtained before the age of one year. The bone level on the anteroposterior film was also noted. The presence or absence of kyphosis was determined from the lateral views obtained at ten years of age. Paired variable analysis was used to compare the mean differences in P/A at one year and at ten years in 31 patients with a bone level of L1 or higher. The exact Fisher test was used to determine the difference in the kyphosis distributions in patients with a bone level of L1 or higher between those with P/A < or = 1/2 and the others. In addition, the specificity, sensitivity, and positive predictive value of a positive test (bone level L1 or higher and P/A < or = 1/2) for development of kyphosis were calculated. Results There was no statistically significant difference in the P/A mean value before the age of one year and after ten years. The distribution of patients with kyphosis was different between patients with a bone level of L1 or higher and P/A < /2 and the other patients (p < 0.01). Considering having a bone level of L1 or higher and a P/A < or = 1/2 as a prognostic test, the specificity was 100%, the sensitivity 87.56%, and the positive predictive value 100%. Many patients with spina bifida will develop kyphosis in the course of life. This spinal deformation raises many specific problems for patient management. Use of a radiological index as presented here allows early detection of patients at risk and can also predict which patients will remain free of deformation. This allows a selection of patients who can benefit most from more regular surveillance of the spinal static and avoid unnecessary radiographic examinations in others.

Adolescent↗

[Shoulder arthrodesis with vascularized fibular graft after tumor resection of the proximal humerus].

PURPOSE OF THE STUDY: Resection of malignant tumors of the proximal humerus often requires dissection of the rotator cuffs and the deltoid muscle. There is no consensus on the ideal method for shoulder reconstruction. We report the functional outcome in a homogeneous series of eight patients treated by arthrodesis using a vascularized free fibular flap. MATERIAL AND METHODS: Eight patients were included in this study. All had an aggressive tumor of the upper humerus. Tumor resection was associated with a rotator cuff and deltoid muscle resection in all patients. All patients then underwent shoulder arthrodesis using a free vascularized fibular flap fixed with a plate. Clinical and radiological evaluation was available for six patients at mean 28 months. The Musculoskeletal Tumor Society function score was used to assess overall function of the upper limb. The cosmetic outcome and radiographic bone healing as well as hypertrophy of the fibular graft were noted. RESULTS: Active abduction and active flexion were 82 degrees on average. All patients could bring their hand to the mouth. Circumduction was possible but limited in amplitude. The mean function score was 26.5/30 with an excellent functional outcome in all patients. The cosmetic outcome was considered poor by all patients. Radiographically, bone healing was achieved at last follow-up in all patients but there was one case of failed fusion between the fibular graft and the scapula which required secondary iliac grafting. Mean fibular graft hypertrophy was 32.8% at last follow-up. DISCUSSION AND CONCLUSION: Two reconstruction methods have been described for patients who require tumor resection of the upper humerus: reconstruction with preservation of glenohumeral joint function and shoulder arthrodesis. Many techniques have been described for each method. It is however difficult to compare the different series reported in the literature because rotator cuff and deltoid muscle resection was not systematically performed and reconstruction methods varied between patients. An analysis of the literature shows that preservation of motion of the scapular glenoid joint can give good functional results when the rotator cuff and deltoid muscle can be preserved. If they cannot, results favor shoulder arthrodesis which provides the patient with very satisfactory upper limb function. Use of a vascularized fibular flap has provided very good arthrodesis results. The patient must however be informed of the probable poor final cosmetic result.

Adolescent↗

[Results at skeletal maturity after double-approach hemivertebral resection].

PURPOSE OF THE STUDY: The natural history of congenital scoliosis or kypho-scoliosis resulting from a hemivertebra is well documented. The spinal deformation generally worsens in children with a free or semi-segmented hemivertebra situated in the thoraco-lumbar, lumbar or lumbosacral region. MATERIAL AND METHODS: From 1982 to 1997, fifteen pediatric patients with 15 hemivertebrae causing progressive scoliosis or kypho-scolisosis underwent hemivertebral resection via a double posterior and anterior approach associated with convex fusion. Mean age at surgery was 4.4 years. The fifteen patients were reviewed at bone maturity (Risser 4 or 5) to assess outcome. RESULTS: Genitourinary tract anomalies were associated in five of the 15 patients and intrathecal anomalies in two. Mean follow-up was 12.1 years. Segmental scoliosis was 30.2 degrees preoperatively, 12.3 degrees postoperatively and 8.9 degrees at last follow-up. The values for total scoliosis were 30.5 degrees, 12.3 degrees and 12.6 degrees respectively. This was a 70.5% improvement at last follow-up for segmental scoliosis and 58.7% for total scoliosis. The real trunk imbalance improved from 31% preoperatively to 9% at last follow-up. All these differences were significant. DISCUSSION: The main objective of surgical treatment for congenital scoliosis due to hemivertebra is to prevent the development of severe deformation which would necessitate a dangerous and difficult procedure to achieve correction. Resection of the hemivertebra via a double approach is the ideal method for early correction. The procedure not only corrects the spinal deformation but also prevents later deterioration. Correction should be performed as early as possible. The result is sustained over time if there is no other associated spinal anomaly. The procedure is safe and the technique well controlled in experimented hands. Patients need to wear a corset for only six months after resection of the hemivertebra.

Adolescent↗

[Predicting the use of Emla cream by the triage nurse in a paediatric emergency department].

INTRODUCTION: The current issue of paediatric emergency care is to decrease the length of stay of the child and his/her family in the emergency department while improving health care quality. Up to now, the paediatric emergency department's triage nurses of the children's Timone hospital apply two EMLA patches when they think that a blood test will be ordered by the medical doctor in order to decrease the waiting time between the clinical exam and the blood test. PURPOSE: The objective of this study was to check if this attitude allowed to efficiently select children who will actually get a blood test. MATERIAL AND METHODS: During four weeks, the triage nurses were asked to write down for each patient after the reception time whether or not a blood test would be ordered by the medical doctor after clinical exam. We compared these data to the number of blood tests actually done. RESULTS: Out of the 2758 children admitted in the emergency department during this period, 1973 nurse prediction forms were filled out. The triage nurses predicted a blood test for 253 patients. Out of these 253 patients, only 132 of them had a blood test done. The study of various studied criteria did not allow to reveal any good agreement between the nurse and the medical doctor. CONCLUSION: The literature study shows that some medical criteria allow to improve this agreement rate. The assessment of blood test necessity have to be carried on and needs further studies in order to precise the most discriminant medical criteria.

Blood Chemical Analysis↗

[Sneddon-Wilkinson disease. Four cases report].

INTRODUCTION: We report four cases of subcorneal pustular dermatosis or Sneddon-Wilkinson disease. Clinical and histological lesions and immunofluorescence data were presented. This disease is classified among neutrophilic dermatitis. PATIENTS AND METHODS: All of four patients presented with clinical and histological lesions compatible with the diagnosis of Sneddon-Wilkinson disease. Indeed, direct and indirect immuno-testing were negative. We noted an association with a benign IgA monoclonal gammapathy in one case and with a seronegative polyarthritis in one other case. Three patients correctly responded to dapsone. One of them after transient improvement was resistant to dapsone and then dramatically responded to etretinate. CONCLUSION: Subcorneal pustular dermatosis is a chronic disease, rarely described in literature. It's a pustular eruption, involving the trunck, axillae and inguinal holds. It's often associated with monoclonal gammapathy, particulary IgA. Its nosological situation is still contested, especially with IgA pemphigus sharing with it the association with IgA monoclonal gammapathy and the same efficacy of dapsone. We discuss relationships between both diseases.

Aged↗

[Fingertip injuries in children: 81 cases with at least one year follow-up].

PURPOSE OF THE STUDY: Fingertip crush injuries are frequent in young children. Rigorous care is required to avoid esthetic and functional sequelae, but treatment is often difficult. We report our experience focusing on simple therapeutic principles useful for everyday practice in the emergency room. MATERIAL AND METHODS: We reviewed retrospectively 91 fingertip injuries in 81 children followed for at least one year. Mean age of the children was 4.5 years. Crush injuries predominated (82%) and mainly involved the middle finger. We recorded the initial lesions, therapeutic interventions, and early complications. At last follow-up, we recorded sensitivity, aspect of the pulp and nail, and the subjective assessment of the parents. RESULTS: There were four infectious including three periungual and subungual felons and two necrotic complications. One child developed a bone infection that required surgery and antibiotics. At last follow-up, seven patients (8%) had sensorial disorders, six (7%) an inesthetic pulp and 23 (25%) dystrophic nails (minor=19, major=4). The parents felt the result was very good for 80% of the children,. Pulp sensitivity disorders were more frequent when the nail bed was injured. Nail dystrophy was more frequent after initial pulp amputation. DISCUSSION: The nail plays an important role in finger function, increasing tactile sensitivity and facilitating prehension. Fingertip injuries can be considered benign but require careful initial evaluation and rigorous management. If a sub-ungual hematoma covers more than half of an intact nail, puncture with a cold lancet appears to be indicated to avoid infection and reduce pain. Perforation with a heated instrument (paper clip) is easy to perform but the hematoma may reform rapidly. In the event of fracture, perforation should always be performed. Detached nails should be removed to examine the nail bed which must be sutured carefully if injured. Pulp lesions should also be sutured.

Adolescent↗

Sweet's syndrome revealing relapsing polychondritis.

Sweet's syndrome is extremely rare in relapsing polychondritis. We report a rare case of Sweet's syndrome revealing relapsing polychondritis. A 77-year-old man presented with fever, associated with respiratory symptoms and non pruritic, painful, erythematous, papules and plaques mainly on the arms and legs. Skin biopsy of the arm found a neutrophilic dermal infiltrate consistent with Sweet's syndrome. A month and a half after admission, the patient presented with ear chondritis and dysphonia. Biopsy of the ear cartilage found a significant perichondral inflammatory cells infiltrate. The diagnosis of relapsing polychondritis was established and a steroid treatment was instituted with a complete regression of the cutaneous signs and chondritis.

Aged↗

[Multifocal osteomyelitis as the first manifestation of chronic granulomatous disease].

Chronic granulomatous disease is a rare immune disease related to an anomaly in phagocytes NADPH oxidase. The characteristic clinical feature is early recurrent and sometimes serious infection. We report the case of a 22-month-old child who developed multifocal osteomyelitis, an unusual inaugural manifestation of chronic granulomatous disease. Septic chronic granulomatous disease is an uncommon differential diagnosis in children who develop recurrent infections. Diagnosis is established with specific blood tests: reduction of tetrazolium nitroblue, chemoluminescence test, molecular analysis. Therapeutic management must be undertaken as early as possible in order to preserve the long-term prognosis. No curative treatment is currently available. Aggressive treatment of each infectious focus with an adapted antibiotic regimen and in certain cases surgical debridement is required in addition to long-term antibiotic prophylaxis.

Consanguinity↗

[Total hip arthroplasty without cement in children and adolescents: 17 cases].

PURPOSE OF THE STUDY: Hip disease can produce major pain and functional disorders in children who should benefit from total hip arthroplasty. We report our experience with total hip prostheses implanted without cement in children. MATERIAL AND METHODS: We performed 17 total hip arthroplasties in 13 children who had various conditions, mainly chronic juvenile osteoarthritis and aseptic osteonecrosis secondary to sickle cell anemia. The acetabular inserts were not cemented. All the femoral stems were custom-made using computer-assisted preoperative planning based on standard x-rays and computed tomography findings. Outcome was assessed on the basis of patient satisfaction, pain, and function. Radiographically, we assessed stem implantation, stability and integration. Results were classed with the Harris score and also with the Steinbrocker classification in order to take into account the child's overall functional handicap. RESULTS: Mean follow-up was 36.4 months. There were three cases of superficial hematoma, one case of superficial sepsis and one acetabular loosening. All patients were satisfied. Unsatisfactory function, observed in 80% of the children preoperatively, was found in only 17% postoperatively. The Harris score improved from 23.8 preoperatively to 87.7 at last follow-up. There were no cases of stem loosening and integration was achieved in 85.3% of the cases proximally in the area with hydroxyapatite surfacing. DISCUSSION: The problem with these children is to determine when total hip arthroplasty should be proposed. We retain three important indications: uncontrollable chronic pain, normal school attendance impossible, no other possibility for conservative surgery. Several studies have reported only mediocre results with cemented stems. We opted for custom-made stems without cement for three reasons: preservation of bone stock, better adaptation to bone whose quality and morphology had been remodeled by the underlying condition and repeated osteotomies which also affect the gluteus medius, and finally, better chance of success for future revisions in these active young patients. CONCLUSION: The question on whether or not total hip arthroplasty should be performed early in these children to avoid osteotomies which could alter the longevity of a future prosthesis remains open. Custom-made stems inserted without cement have provided satisfactory results in our experience. To date, follow-up is too short to compare our results with those obtained by others using cemented stems.

Activities of Daily Living↗

[Pediatric surgical emergencies].

A quarter of pediatric surgical emergencies are non trauma and are for most of them limping child, acute abdominal pain, and acute scrotum. All of the child's lower limb pathology can be described as a limp, and the same is true for abdominal pain and acute scrotum. We will describe the important inquiring and clinical tools as well as a few simple paraclinics that allow to recognize the few cases requiring emergency care.

Abdominal Pain↗