Synovial chondromatosis and chondrosarcoma. A study of the relationship between these two diseases

PURPOSE OF THE STUDY: This retrospective study concerns six patients in whom chondrosarcoma was suspected to develop in synovial chondromatosis. We discuss for these cases different diagnosis. The authors expose the clinical signs and radiological aspects which suggest malignant transformation. We report the indispensable criteria for established diagnosis of malignant transformation of synovial chondromatosis and appropriated forms of therapeutic management were suggested. MATERIALS AND METHODS: Six patients, 3 males and 3 females from 36 to 58 years of age were included in this study. Three patients presented 6 months, 3 years and 25 years history of synovial chondromatosis of the knee joint. When the malignant transformation appeared, a surgical biopsy was performed and the pathologist diagnosed a chondrosarcoma in all cases. For the other three patients, the chondrosarcoma and synovial chondromatosis were diagnosed at the same time. The localization was shoulder, hip and knee. A surgical biopsy was performed and the pathologist diagnosed chondrosarcoma. Synovial chondromatosis was diagnosed by histologic examination of the resection or amputation specimen. TREATMENT AND RESULTS: Four patients had thigh amputation, one patient had "en bloc resection" of the hip-joint and the last patient had resection of the shoulder joint. In all cases, the histologic examination diagnosed chondrosarcoma and synovial chondromatosis. All patients were free of disease. DISCUSSION: The malignant transformation of synovial chondromatosis is rare but this diagnosis must be established to perform appropriate treatment. Other possible diagnosis are: low grade synovial chondrosarcoma initially diagnosed as a synovial chondromatosis. Bertoni believes that all cases of malignant transformation of synovial chondromatosis are initially low grade chondrosarcoma. We believe that his criteria are too strict for diagnosed chondrosarcoma. coexistence of synovial chondromatosis and synovial chondrosarcoma. These 2 diagnosis are extremely rare and their coexistence are unlikely. secondary synovial chondromatosis developed into chondrosarcoma. We don't have histologic criteria to confirm this diagnosis in all our cases. The symptoms that should suggest a malignant transformation of synovial chondromatosis were: rapid late deterioration of clinical conditions, bone invasion diagnosed by X-ray films and medullar invasion discovered by MRI. According to us, the indispensable criteria to diagnose malignant transformation were: 1.) histologic diagnosis of synovial chondromatosis established before diagnosis of chondrosarcoma, 2.) histologic diagnosis of chondrosarcoma on the same anatomic site as the synovial chondromatosis, 3.) diagnosis of chondrosarcoma and synovial chondromatosis on the same resection specimen. Only the three first cases were in accordance with these criteria. The treatment must be a "en bloc resection" of the joint or an amputation. CONCLUSION: Malignant transformation is rare, but this diagnosis should be established to perform adequate treatment. This diagnosis should be suspected when a rapid deterioration of the clinical status appeared and when bone involvement was detected by MRI. However, the danger still lies in the misinterpretation of the synovial chondromatosis as chondrosarcoma. This diagnosis can be made with clinical, radiological and pathological criteria. The treatment must be a wide resection or an amputation.     

Socioeconomic differences in children's health: How and why do these relationships change with age?

The effects of socioeconomic status (SES) on health are well documented in adulthood, but far less is known about its effects in childhood. The authors reviewed the literature and found support for a childhood SES effect, whereby each decrease in SES was associated with an increased health risk. The authors explored how this relationship changed as children underwent normal developmental changes and proposed 3 models to describe the temporal patterns. The authors found that a model's capacity to explain SES-health relationships varied across health outcomes. Childhood injury showed stronger relationships with SES at younger ages, whereas smoking showed stronger relationships with SES in adolescence. Finally, the authors proposed a developmental approach to exploring mechanisms that link SES and child health. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Blood lead (Pb) levels: Further evidence for an environmental mechanism explaining the association between socioeconomic status and psychophysiological dysregulation in children.

Objective: The authors recently reported that blood lead (Pb) was a significant mediator for the positive association between socioeconomic status (SES) and peripheral vascular responses to acute stress in children (B. B. Gump et al., 2007). The present study considers the possibility that Pb may also mediate an association between SES and cortisol responses to acute stress. Design: Early childhood Pb exposure was tested as a mediator for cross-sectional associations between SES and cortisol responses. Main Outcome Measures: The primary outcome was cortisol responses to acute stress in 9.5-year-old children (N = 108). Results: Lower family income was associated with significantly greater cortisol levels following an acute stress task. A mediational analysis confirmed that Pb was a significant mediator for this association. Conclusion: These results reaffirm the importance of considering the chemical environment as well as social and psychological environment when evaluating psychophysiological effects of low SES. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Binding of properdin to solid-phase immune complexes: critical role of the classical activation pathway of complement

OBJECTIVE: Our objectives were to assess the clinical and microbiological aspects of septic osteoarthritis in children admitted to our center from 1987 until July 1997 and to determine the sensitivity of ultrasound in this age group. PATIENTS AND METHODS: The medical records of 36 children diagnosed as having septic osteoarthritis of the hip were reviewed retrospectively. The diagnosis had been based on clinical criteria, along with synovectomy and drainage of purulent material from the affected joint. An X-ray and/or ultrasound had been performed when the diagnosis was suspected. RESULTS: Nineteen children were diagnosed during the neonatal period, 8 between the ages of 1 and 12 months and 9 older than one year of age. Mean age at diagnosis was 16.8 +/- 6.2 months (median 29 days, range 6 days to 13 years). The hip was involved in 32 children, the ankle in 3 and the elbow in 1. A microbiological diagnosis was achieved in 22 cases (61%) by culture from blood, CSF, and/or synovial fluid. The most common isolates were Gram positive cocci (S. aureus in 9 cases and coagulase negative Staphylococcus in 3). The diagnostic value of the X-rays was very low (18%). The ultrasound was initially considered abnormal in 64.5% of the patients, with a lower sensitivity in the neonatal period. After a mean follow-up period of 36 months, the outcome was good in 86% of the cases, although three children continue with sequelae. Two preterm infants died due to sepsis associated with the osteoarthritis. CONCLUSIONS: At the time of diagnosis of septic arthritis of the hip, the ultrasound is frequently normal. Due to the poor outcome when there is a delay in surgery, we suggest immediate synovectomy and drainage when there is clinical suspicion of septic arthritis despite an apparently normal ultrasound.     

Acute coronary occlusion and in-stent thrombosis in a patient with essential thrombocythemia

BACKGROUND: Physical activity in old age probably protects against hip fracture. However, very little is known about the impact of occupation during working life, in terms of its physical activity level and the socio-economic status (SES) it indicates, on the risk of hip fracture in later life. METHOD: A population-based case-control study was conducted on 416 subjects to investigate relationships between risk of hip fracture and occupational physical activity and occupation-related SES. Occupational physical activity was coded according to the proportion of the working day at ages 20 and 50 years that the subjects were likely to have spent sitting. The Australian Classification of Standard Occupations (ASCO) was used as the basis for the coding of SES, indicated by job titles. RESULTS: Compared with those in the intermediate category, women with a sedentary job at age 50 years (odds ratio (OR) 7.2, 95 per cent confidence interval (CI) 1.2-41.9) or a mainly weight-bearing job (OR 5.1, 95 per cent CI 1.1-23.2) had an increased risk of hip fracture. A decreasing hip fracture risk with increasing SES of the longest held job was also observed. CONCLUSION: The finding of a J-shaped relationship in women between occupational physical activity and risk of hip fracture supports the need for research into the most effective types of exercise for maintenance of bone strength. Furthermore, the protective effect of SES demonstrated in this study still need to be corroborated in other studies before establishing evidence of a causal relationship.     

Association between socioeconomic status and metabolic syndrome in women: Testing the reserve capacity model.

Objective: Low socioeconomic status (SES) environments may impede the development of a bank of resources, labeled reserve capacity, and may also be stressful, thereby depleting available reserves. In consequence, lower SES persons may experience more negative emotions, leading to adverse health consequences. The authors tested the reserve capacity model in relation to the metabolic syndrome. Design: There were 401 initially healthy women who followed longitudinally for 12 years. Self-reported characteristics, stressors, and cardiovascular risk factors were measured repeatedly. Structural equation modeling was used to evaluate hypothesized relationships. Main Outcome Measure: Metabolic syndrome factor. Results: Confirmatory factor analysis verified reserve capacity as the aggregate of optimism, self-esteem, and social support, and negative emotion as the aggregate of depressive symptoms, anger, and tension. Structural equation modeling showed two pathways to the metabolic syndrome factor, (χ2(59) = 111.729, p ldf = 1.894; CFI = .956; RMSEA = .047): direct from low SES to the metabolic syndrome factor (B = -0.19, t = -3.24, p = .001); and indirect, from low SES to low reserve capacity to high negative emotions to the metabolic syndrome factor (B = -0.024, t = -2.05, p = .04). Conclusion: Low SES may increase risk for metabolic syndrome, in part, through reserve capacity and negative emotions. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Children with Asperger syndrome

In 1994 Hans Asperger (1906-80), an Austrian physician, described a group of children with impaired social interaction and communication abilities. The name of this disorder today is Asperger's syndrome, and it is currently defined under the category of pervasive developmental disorder in DSM-IV and ICD-10. In this article the following aspects of Asperger's syndrome are focused on: personality, epidemiology, etiology, examination, differential diagnosis, management and prognosis. The article is based on a literature study. Asperger's syndrome seems to be considerably more common than "classic" autism. The syndrome is much more common in boys than in girls. The clinical characteristics of Asperger's syndrome are probably influenced by many factors, including organic and genetic factors. Asperger's syndrome is the term applied to the highest functioning end of the autism scale. There are several commonalities between Asperger's syndrome and autism, namely impairment of social interaction and communication abilities, and range of interests and activities. Differences exist primarily in the degree of impairment in language and cognitive development. Differential diagnosis, examination and management are discussed. There is a need for further research. It is important that the diagnostic criteria for Asperger's syndrome are as uniform as possible, and that they do not overlap with infantile autism.     

Symptom-giving pelvic girdle relaxation of pregnancy, postnatal pelvic joint syndrome and developmental dysplasia of the hip. The Norwegian Association for Women with Pelvic Girdle Relaxation (Landforeningen for Kvinner Med Bekkenl?sningsplager)

OBJECTIVES: To describe the clinical characteristics and outcomes of a large group of women with symptom-giving pelvic girdle relaxation of pregnancy and postnatal pelvic joint syndrome. To determine if there is an increased incidence of developmental dysplasia of the hip in the children of women with such pelvic problems. METHODS: A postal survey of 1,609 Norwegian women registered as having pregnancy-initiated pelvic joint pain. The response rate was 79% and from the answers 1,115 women were defined as having had symptom-giving pelvic joint syndrome of pregnancy and/or postnatal pelvic joint syndrome. RESULTS: Pelvic pains began in the first pregnancy in 74% of the respondents usually beginning in the first trimester. Pelvic pain worsened with subsequent pregnancies and persisted for a mean of 6.25 years, often causing major incapacity and lifestyle changes. Rest and physical supports brought temporary relief only. Sacroiliac joints and the symphysis pubis were the commonest sites of pain but peripheral joints were also often affected. There was a strong family history of both pelvic joint syndrome and developmental dysplasia of the hip. The incidence of hip dysplasia in the children of women surveyed was 45/1,000 which is 5 times the Norwegian incidence. CONCLUSION: Pelvic joint syndrome nearly always follows pelvic girdle relaxation of pregnancy and may have prolonged debilitating effects which do not respond long term to current therapies. The incidence of developmental dysplasia of the hip in the children of these women was high. A genetic susceptibility to joint dysfunction in both mother and fetus, possibly due to an aberration of relaxin physiology, is surmized. Identification of possible relaxin receptor changes in affected joints is a hypothesis worthy of testing with a view to the design of selective relaxin receptor modulators in pregnancy.     

Entrapment neuropathies

Relative frequency of entrapment neuropathies was studied from amongst the patients referred to an electrodiagnostic medicine laboratory for electrophysiological studies. During the study period electrophysiological procedures were done on 650 patients with various peripheral nerve disorders. The entrapment neuropathies constituted 8.5%. Carpal tunnel syndrome (CTS) was the commonest entrapment neuropathy (83.6%). Diagnosis of CTS was established in 84 Patients referred with the diagnosis of CTS. Electrophysiological tests confirmed the diagnosis of thoracic outlet syndrome in 4 (15.4%) of the 26 patients referred with this diagnosis and in 5 (19.3%) of them the diagnosis turned out to be CTS. Diagnosis of cubital tunnel syndrome was not suspected clinically in all the 3 patients, they were referred with the diagnosis of ulnar neuropathy. In both the patients with tarsal tunnel syndrome the initial diagnosis was peripheral neuropathy.     

Randomized study of chemotherapy and surgery versus radiotherapy for stage IIIA non-small-cell lung cancer: a National Cancer Institute of Canada Clinical Trials Group Study

We treated two children with the unusual complication of ulnar nerve palsy after closed both-bone forearm fractures. Both patients developed an ulnar claw-hand deformity within 7 weeks of injury that resolved spontaneously by 20 weeks postinjury with nonoperative treatment. No patient showed any signs or symptoms of an ischemic compartment syndrome. Both nerve injuries were identified immediately at the time of fracture by a careful neurologic examination. This avoids confusion with a postreduction nerve entrapment injury or ischemic injury after a localized compartment syndrome, which may have considerably different treatments and outcomes. We recommend that a careful neurologic examination be recorded before any manipulative reduction of forearm fractures in children. If an ulnar nerve palsy is detected, it is probably a result of nerve contusion and should resolve without the need for surgical exploration.     

Entrapment neuropathy of the deep peroneal nerve associated with the extensor hallucis brevis

The authors report a case of entrapment neuropathy of the deep peroneal nerve associated with the extensor hallucis brevis. This entrapment neuropathy was found distal to the inferior retinaculum that causes the anterior tarsal tunnel syndrome. Surgical decompression of the deep peroneal nerve that was entrapped by the extensor hallucis brevis relieved the symptoms. This condition, like the anterior tarsal tunnel syndrome, deserves attention.     

Labrum lesions and residual dysplasia of the hip joint. Definition and prospectives

Labral lesions are a sign of biomechanical decompensation of the hip joint and often represent the first clinical symptom of residual hip dysplasia (RHD) in the adult. Provocation tests (impingement, apprehension) are typical but not specific. Labral lesions and concomitant findings (intra- and extraosseous ganglia, stress bone marrow oedema) can be detected by magnetic resonance arthrography (MRA) with an accuracy of 91%. Primary therapeutic goal is the normalization of the underlying pathomorphology and instability by a redirectional acetabular osteotomy. There are several concepts concerning simultaneous arthrotomy at the time of osteotomy: no arthrotomy at all, selective arthrotomy, routine arthrotomy in every case. There are more clinical studies necessary before one of these concepts can be widely accepted and recommended. Based on preliminary results, palliative arthrotomy with partial labral resection but without corrective osteotomy in osteoarthritis secondary to residual hip dysplasia gives poor results; we therefore urgently dissuade from palliative labral surgery via arthrotomy. Whether labral surgery via arthroscopy might be a useful concept in symptomatic residual hip dysplasia, is still an open question. In this review article, the "state of the art" presented at the "Vienna Labral Symposium 1997" is reflected and summarized. At the end of this article, a "common statement" of the experts is published in English and German language.     

Ping-pong ball plombage for right postpneumonectomy syndrome in children

BACKGROUND/PURPOSE: Right postpneumonectomy syndrome is a rare complication of pneumonectomy characterized by exertional dyspnea resulting from mediastinal shift into the empty hemithorax. Historically, this problem has been treated with thoracoplasty, muscle flap transposition, pericardial fixation, and plombage using a variety of materials. Significant postoperative complications have been reported with each of these methods. In an effort to avoid the problems known to be associated with other plombage materials, we have used sterilized ping-pong balls as plombage to treat the postpneumonectomy syndrome. METHODS: Two children with pneumonectomy syndrome were treated with ping-pong ball plombage. RESULTS: Symptoms improved immediately in both patients and postoperative chest x-rays in each showed a normal cardiac position. Neither child had a postoperative complication. Six-year follow-up chest films in both children have demonstrated some fluid filling of the ping-pong balls, but no clinical problems attributable to them have been noted. CONCLUSION: The authors conclude that ping-pong ball plombage appears to be a viable management option in children with right postpneumonectomy syndrome.     

Why attend school? Chinese immigrant and European American preschoolers' views and outcomes.

The developing views of the purposes of school learning (PSLs) and related achievement among immigrant Chinese preschoolers and their European American (EA) age-mates were examined. Both culture and socioeconomic status (SES) were considered simultaneously, an often neglected research approach to studying Asian children. One hundred and fifty 4-year-olds—50 each of middle-class Chinese (CHM), low-income Chinese (CHL), and EA children—completed 2 story beginnings about school and were also tested for their language and math achievement. Results showed that 4-year-olds held sophisticated PSLs, ranging from intellectual to social and affect benefits. Large cultural and SES differences also emerged. CHM children mentioned more adult expectation and seriousness of learning than EA children who expressed more positive affect for self and compliance with adults. CHL children mentioned fewest PSLs. Achievement scores for oral expression of both immigrant groups were significantly lower than those of EA children despite similar reading and math achievement. Controlling for culture and SES, the authors found that children's articulated intellectual, but not other purposes, uniquely predicted their achievement in all tested domains. Cultural and SES influences on immigrant children are discussed. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Complications of femoral and acetabular modularity

The versatility of modular total hip arthroplasties have rapidly extended their applications. However, these new interfaces can lead to complications that were not observed with monolithic components. These problems have been noted with modular femoral and acetabular components and have been associated with the generation of particulate debris. This article reviews the authors' clinical observations and histologic, biomechanic, and spectophotometric evaluations of modular total hip arthroplasties. New data comparing both synovial fluid metal levels in well-fixed and loose monolithic and modular prosthetic hip implants are presented. In modular total hip components, synovial fluid cobalt levels correlated positively with patient weight and length of implantation. The generation of particulate debris in modular total hip components may induce periprosthetic osteolysis. Taper locks for femoral components and locking mechanisms for the polyethylene liner and metallic cup must be designed to avoid the production of particulate debris.     

An update on the diagnosis and treatment of attention-deficit/hyperactivity disorder in children.

What are the most appropriate empirically supported diagnostic and treatment approaches to children with attention-deficit/hyperactivity disorder (ADHD)? This article summarizes the nomenclature, prevalence and course, comorbidity, etiology, assessment, and federal laws associated with ADHD. The authors then review clinical research and consensus guidelines for the treatment of ADHD, including the largest randomized treatment study completed on ADHD (MTA Cooperative Group, 1999a). The empirical evidence supports either a behavioral-psychosocial or a combined behavioral-psychosocial and medication intervention in the treatment of children with ADHD. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Children's event-related potentials of auditory selective attention vary with their socioeconomic status.

Past research suggests a link between socioeconomic status (SES) and brain processes in children, but direct evidence from neuroimaging is scarce. The authors investigated the relationships among SES, performance, and the neural correlates of auditory selective attention, by comparing event-related potentials (ERPs) in lower- and higher-SES preadolescent children during a task in which they attended to two types of pure tones but ignored two other types. Our hypothesis was that, at comparable performance levels, higher-SES children ignore distracters (the unattended, irrelevant tones) while lower-SES children attend equally to distracters and to targets (the attended, relevant tones). The authors found that ERP waveform differences between attended and unattended tones (Nd, difference negativity) were significant in the higher-SES but not in the lower-SES group. However, the groups did not differ in reaction times or accuracy. Electroencephalographic power analysis revealed a differential pattern of theta activity concomitant with irrelevant tones for the two groups, indicating that although they performed similarly the children from these groups recruited different neural processes. Lower-SES children, the authors suggest, deployed supplementary resources to also attend to irrelevant information. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Radiolabelled monoclonal antibodies (McAb): an alternate approach to the conventional methods for the assessment of cardiomyocyte damage in an experimental brain-death pig model

BACKGROUND: According to the Ministry of Health and Welfare AIDS Surveillance Committee's report on vertically transmitted human immunodeficiency virus (HIV) infection, there have been eight children with acquired immune deficiency syndrome (AIDS) and 18 children with HIV infection in Japan, totalling 26 in all as of February 1997. A search of the literature fails to reveal any report that deals with many cases of vertically transmitted HIV infection in Japan. METHODS: A primary questionnaire survey was taken of the main medical institutions across the country, followed by a secondary questionnaire survey of physicians and pediatricians who treated the disease. A clinical review was made of 19 children with vertically transmitted HIV infection (including eight AIDS children) according to the 1994 Revised Classification System for HIV Infection in Children. RESULTS: The mean age at diagnosis was 14.5 months and the diagnosis was made at less than 18 months of life in approximately 70% of infected children. In the mean observation period of 16 months, six of eight AIDS children (75%), and one child of group B died. The mean period of observation for the seven dead children was 7 months, and six of seven children died by 36 months of life. The survival period after the diagnosis of AIDS was 15 months. The diagnosis of HIV infection was made based on the clinical symptoms of all children with AIDS. Of 11 children, six (45%) presented with symptoms of HIV infection by 6 months of life, and 10 of 11 children (91%) presented with symptoms by 26 months of life. The noteworthy clinical findings included hepatomegaly, splenomegaly, recurrent respiratory tract infection, lymph node swelling, oral candidiasis, hepatitis, wasting syndrome, HIV encephalopathy and severe pneumonia. The favored age for the start of complications and the magnitude of decrease in the HIV helper cell count varied with each case of complications of HIV infection (wasting syndrome, HIV encephalopathy) or opportunistic infections (cytomegalovirus infection, Mycobacterium avium complex infection). Anti-HIV drugs (mainly zidovudine) had been used in five of eight children with AIDS and were effective in two long survivors alone. CONCLUSIONS: Children who are diagnosed with HIV infection, based on their clinical symptoms, carry a poor prognosis. In this respect, early diagnosis and progress in anti-HIV therapy are necessary.     

Randomized comparison of two schedules of fluorouracil and leucovorin in the treatment of advanced colorectal cancer

The authors report on a case of trisomy 9 mosaicism syndrome, a rare chromosome abnormality. The common features of this syndrome are growth and mental retardation, low-set malformed ears, wide sutures and fontanelles, bulbous nose, short palpebral fissures, micrognathia, microphthalmia and enophthalmos, abnormal hands and feet, hip dislocation, joint limitation, cardiovascular defects and urogenital abnormalities. Our patient presented some unusual characteristics, such as 13 pairs of ribs, a vertebral malformation, a hemivertebra and a Dandy-Walker syndrome. They compare their clinical findings with the few cases previously described and they try to contribute to the further clinical definition of the syndrome. It is possible that there is a correlation between the variability of the phenotype and the percentage of trisomic cells in the patient.