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1.

Background

Endothelial damage, hypertension and cytotoxic medications may serve as risk factors for the posterior reversible encephalopathy syndrome (PRES) in systemic lupus erythematosus. There have been few case reports of these findings in pediatric lupus patients.

Objective

We describe clinical and neuroimaging findings in children and adolescents with lupus and a PRES diagnosis.

Materials and methods

We identified all clinically acquired brain MRIs of lupus patients at a tertiary care pediatric hospital (2002–2008). We reviewed clinical features, conventional MRI and diffusion-weighted imaging (DWI) findings of patients with gray- and white-matter changes suggestive of vasogenic edema and PRES.

Results

Six pediatric lupus patients presenting with seizures and altered mental status had MRI findings suggestive of PRES. In five children clinical and imaging changes were seen in conjunction with hypertension and active renal disease. MRI abnormalities were diffuse and involved frontal regions in five children. DWI changes reflected increased apparent diffusivity coefficient (unrestricted diffusion in all patients). Clinical and imaging changes significantly improved with antihypertensive and fluid management.

Conclusion

MRI changes suggestive of vasogenic edema and PRES may be seen in children with active lupus and hypertension. The differential diagnosis of seizures and altered mental status should include PRES in children, as it does in adults.  相似文献   

2.

Background

Avascular necrosis (AVN) of the metacarpal head is a rare condition that can go unrecognized on radiography and progress to degenerative arthritis. Few reports describe the association with trauma, steroid use and systemic lupus erythematosus (SLE). Optimal treatment for this condition has not been established and long-term prognosis is unknown.

Objective

We present two cases of children with AVN of the fourth metacarpal head with a relatively recent history of indirect hand trauma.

Materials and methods

Both of our cases had radiographs and MRI of the hand.

Results

MRI was diagnostic in both cases; however, radiographs were reported as normal in the early stage in one case.

Conclusion

AVN of the metacarpal head is rare but can occur in children. Pediatric radiologists should be aware of this uncommon clinical entity, especially in light of increased involvement of children in sporting activities. Active children with hand trauma and children treated with steroids for a variety of conditions are particularly at risk.  相似文献   

3.

Background

North American pediatric rheumatologists have created an investigator-initiated research network (the Childhood Arthritis and Rheumatology Research Alliance – CARRA) to facilitate multi-centre studies. One of the first projects undertaken by this network was to define, by consensus, research priorities for the group, and if possible a first group-sponsored clinical trial in which all members could participate.

Methods

We determined consensus using the Delphi approach. This approach has been used extensively in health research to reach consensus in large groups. It uses several successive iterations of surveys eliciting ideas and opinions from specialists in the field. Three surveys were designed based on this method and were distributed to members of CARRA to elicit and rank-order research priorities.

Results

A response rate of 87.6% was achieved in the final survey. The most highly ranked research suggestion was to study infliximab treatment of uveitis unresponsive to methotrexate. Other highly ranked suggestions were to study i) the treatment of systemic arthritis with anakinra and ii) the treatment of pediatric systemic lupus erythematosus with mycophenolate mofetil.

Conclusion

The Delphi approach was an effective and practical method to define research priorities in this group. Ongoing discussion and cooperation among pediatric rheumatologists in CARRA and others world-wide will help in developing further research priorities and to facilitate the execution of clinical trials in the future.  相似文献   

4.

Objective

To describe the clinical features, treatment and prognosis of acquired thrombotic thrombocytopenic purpura (TTP) in children based on a single institution experience.

Methods

This study is a retrospective review of all 12 children with TTP seen at New York Medical College- Westchester Medical Center during a period of 15 y from 1993 to 2008.

Results

There were 7 females and 5 males with acquired TTP, with a median age of 13 (range, 6–17); and no cases of congenital TTP. The classic pentad of TTP (microangiopathic hemolytic anemia, thrombocytopenia, neurologic symptoms, renal dysfunction and fever) was seen in only three patients. Nine had renal involvement; eight had neurologic symptoms; and four had fever. All 12 patients had thrombocytopenia, anemia, and elevated LDH. Nine had idiopathic TTP. Three patients had one of the following underlying disorders: systemic lupus erythematosus, mixed connective tissue disorder, and aplastic anemia (post-bone marrow transplant on cyclosporine). ADAMTS13 level was decreased in 7 of 8 patients studied. Eight of 10 patients achieved remission with plasmapheresis alone. Two needed additional treatment before achieving remission. Two had one or more relapses, requiring immunosupressive treatment with vincrisine, prednisone, or rituximab. The patient with aplastic anemia died of pulmonary hypertension 5 y after bone marrow transplantation. All other 11 patients are alive and free of TTP for a median follow-up of 12 mo (range, 3–72 mo).

Conclusions

Acquired pediatric TTP responds well to plasmapheresis. However, many patients do require additional treatment because of refractoriness to plasmapheresis or relapse. The clinical features, response to treatment, and relapse rate of pediatric TTP appear similar to those of adult TTP.  相似文献   

5.
The presence and specificity of circulating autoantibodies in children are a guide to the diagnosis of autoimmune diseases that may occur during childhood. Antinuclear antibodies (ana), directed to ubiquitous nuclear antigens, are associated with a number of systemic autoimmune diseases. Juvenile idiopathic arthritis is the most common rheumatic disease in children; however, the combination of arthritis and thrombocytopenia or a Rayanaud phenomenon on the one hand and ana on the other hand, should alert the clinician for the possible presence of a much rarer autoimmune disease such as systemic lupus erythematosus or systemic sclerosis. Organ specific antibodies, directed against specific tissue antigens, are characteristic for some organ specific autoimmune diseases such as insulin-dependent diabetes mellitus and autoimmune thyroid disease. The presence of different organ specific antibodies with or without non-organ specific antibodies is of relevance, since it may precede the clinical evolution towards a combination of autoimmune diseases. Anti-neutrophil cytoplasmic antibodies (anca) are strongly associated with several types of primary systemic vasculitis, but they are rarely found in the most common types of vasculitis in children, i.e. Henoch Schönlein purpura and Kawasaki disease. Accordingly, when a child with purpura also has anca and a strong acute phase response, the possibility of a primary vasculitis such as Wegener granulomatosis (in a child with c-anca) and microscopic polyarteritis (in a child with p-anca) should be considered.  相似文献   

6.

Background

Rhombencephalosynapsis is a rare genetic aberration characterized by variable vermian hypoplasia/aplasia in conjunction with united cerebellar hemispheres. Genetic defects in the isthmic organizer at the mesencephalic–metencephalic junction are presumably responsible for the associated aqueductal stenosis.

Objective

We performed a retrospective review of 20 children with rhombencephalosynapsis to evaluate for and emphasize the association of aqueductal stenosis and hydrocephalus.

Materials and methods

We retrospectively reviewed the MR and CT images of 20 children (0–11 years old) with rhombencephalosynapsis encountered at two academic children’s hospitals. Rhombencephalosynapsis spectrum severity was graded based on pre-existing literature. We analyzed examinations for ventriculomegaly and degree of aqueductal stenosis. The collicular distances were measured from the collicular apices. Imaging studies were also analyzed for malformations of cortical and cerebellar development.

Results

Thirteen of the 20 children (65%) with rhombencephalosynapsis presented with clinical or imaging evidence of hydrocephalus and aqueductal stenosis, principally involving the caudal cerebral aqueduct. All children with aqueductal stenosis had collicular fusion. All six children with complete rhombencephalosynapsis had aqueductal stenosis. The cerebral aqueduct varied from normal to stenotic in children with incomplete rhombencephalosynapsis. Corpus callosum dysgenesis was present in four children.

Conclusion

Aqueductal stenosis in the setting of rhombencephalosynapsis is an under-recognized cause of noncommunicating hydrocephalus. Our findings support the hypothesis that a defect involving the common gene(s) responsible for the differentiation and development of both the roof plate and midline cerebellar primordium at the mesencephalon/first rhombomere junction may be responsible for the association of aqueductal stenosis and rhombencephalosynapsis.  相似文献   

7.

Background

Elevated intracranial pressure (ICP) resulting from impaired drainage of cerebrospinal fluid (CSF) causes hydrocephalus with damage to the central nervous system. Clinical symptoms of elevated intracranial pressure (ICP) in infants may be difficult to diagnose, leading to delayed treatment by shunt placement. Until now, no biochemical marker of elevated ICP has been available for clinical diagnosis and monitoring. In experimental animal models, nerve growth factor (NGF) and neurotrophin-3 (NT-3) have been shown to be produced by glial cells as an adaptive response to hypoxia. We investigated whether concentrations of NGF and NT-3 are increased in the CSF of children with hydrocephalus.

Methods

NGF was determined in CSF samples collected from 42 hydrocephalic children on 65 occasions (taps or shunt placement surgery). CSF samples obtained by lumbar puncture from 22 children with suspected, but unconfirmed bacterial infection served as controls. Analysis was performed using ELISA techniques.

Results

NGF concentrations in hydrocephalic children were over 50-fold increased compared to controls (median 225 vs 4 pg/mL, p < 0.0001). NT-3 was detectable (> 1 pg/mL) in 14/31 hydrocephalus samples at 2–51 pg/mL but in none of 11 control samples (p = 0.007).

Conclusion

NGF and NT-3 concentrations are increased in children with hydrocephalus. This may represent an adaptive response of the brain to elevated ICP.  相似文献   

8.

Background

Children represent 10-20% of all systemic lupus erythematosus (SLE) patients. Their clinical manifestations and outcomes vary with age. We aim to clarify the relationship between pubescent status and the clinical manifestations of pediatric SLE.

Methods

In this study, pediatric SLE patients were divided into three groups, based on age at disease onset (?Q8, 8?C13 &; 13?C18?years), defined as prepubescent, pubescent and postpubescent, respectively. Initial clinical manifestations and laboratory characteristics at diagnosis were analyzed.

Results

Ninety-six patients were entered into the study: 8 had disease onset before age 8, while 49 were between 8?C13 and 39 of them were 13?C18. Female predominance was noted in all three groups (2.5-7.0:1). Postpubescents showed significantly more renal involvement and lymphopenia, along with lower levels of C3 and C4, when compared with prepubescents. They also showed significantly more lymphopenia when compared with pubescents. Pubescents showed significantly more renal involvement, leukopenia and lupus anticoagulant (LAC) positivity, along with lower C3 and C4 levels, when compared with prepubescents. Pubescents also showed significantly higher anti-Sm antibody positivity when compared with postpubescents. Prepubescents showed significantly more splenomegaly and anti-Jo-1 antibody positivity when compared with those of pubescents. The results showed that the disease activity (SLEDAI-2K score) correlated positively with age at disease onset and negatively with disease duration before diagnosis (p?=?0.011).

Conclusions

Age at disease onset is related to initial manifestations in pediatric SLE patients at our center. Certain parameters such as renal involvement, splenomegaly, low C3 level, low C4 level, lymphopenia, leukopenia, and anti-Sm &; anti-Jo-1 antibody were found to be significantly different among the age groups. Renal involvement might be the key symptom that varies with age.  相似文献   

9.

Background

The pathologic diagnosis of isolated myocarditis without pericardial involvement is uncommonly encountered in systemic onset Juvenile Idiopathic Arthritis (soJIA).

Case

An eleven year-old boy with soJIA died suddenly while being treated with the interleukin 1 (IL-1) receptor inhibitor, anakinra. His autopsy revealed an enlarged heart and microscopic findings were consistent with myocarditis, but not pericarditis. Viral PCR testing performed on his myocardial tissue was negative.

Conclusion

This case illustrates myocarditis as a fatal complication of soJIA, potentially enabled by anakinra.  相似文献   

10.

Background

Eosinophilic colitis (EC) is a gastrointestinal disease of undetermined etiology whose clinical features overlap with those of the inflammatory bowel diseases. To the best of our knowledge, the CT imaging features of EC have not been described in children.

Objective

To report and analyze the clinical, imaging and histological findings in seven children with EC.

Materials and methods

Children with EC were identified in a pediatric pathology database, and those with CT imaging within 2 months of diagnosis were included, totaling seven children. Clinical, imaging and pathological features were reviewed and analyzed.

Results

The most common presenting symptoms were abdominal pain, bloody diarrhea and rectal bleeding. EC was characterized as a dense and predominantly eosinophilic inflammatory infiltrate in the lamina propria or epithelium without granulomas. CT scans were abnormal in six children (86%), demonstrating colonic wall thickening, predominantly cecal, in five (71%), mild to moderate terminal ileal thickening in two (29%), and pneumatosis in one (14%). Right colonic involvement was greater than terminal ileal involvement.

Conclusion

CT imaging findings in children with EC include right colonic wall thickening of variable extent downstream and absent or mild involvement of the terminal ileum. EC should be considered in the differential diagnosis in children presenting with abdominal pain and bloody diarrhea.  相似文献   

11.
Schütteltrauma     

Background

Shaking babies causes severe cerebral lesions, resulting in death or lasting neurological deficits, without external signs of violence. The identification of shaken babies is of outstanding forensic importance to avoid further assaults by their guardians.

Methods

CT and MRI scans of four patients with probable or proven non-accidental shaking injuries according to Duhaimes classification scheme were evaluated retrospectively.

Results

All patients had extensive cortical and subcortical lesions with a parietal and occipital predominance. The basal ganglia were spared. In those three patients with diffusion-weighted MRI shortly after the onset of symptoms, the lesions showed restricted diffusion of protons. On follow-up, all patients developed extensive cortical and subcortical necroses, cerebral atrophy and hemorrhagic cortical transformation. The diffusion abnormalities did not persist longer than 3 weeks.

Conclusions

Imaging features of the shaken baby syndrome are dominated by ischemic and hypoxic cerebral lesions. The best imaging modality for these patients is MRI including diffusion-weighted images, because it is most sensitive for the typical lesion pattern in this form of non-accidental trauma.  相似文献   

12.

Background

Nephrogenic systemic fibrosis is a fibrosing disorder associated with exposure to gadolinium-based contrast agents in people with severely compromised renal function.

Objective

The purpose of this study was to determine the reported number of cases of nephrogenic systemic fibrosis in children using three distinct publicly available data sources.

Materials and methods

We conducted systematic searches of the U.S. Food and Drug Administration Adverse Event Reporting System (FAERS), the International Center for Nephrogenic Systemic Fibrosis Research (ICNSFR) registry and published literature from January 1997 through September 2012. We contacted authors of individual published cases to obtain follow-up data. Data sets were cross-referenced to eliminate duplicate reporting.

Results

We identified 23 children with nephrogenic systemic fibrosis. Seventeen had documented exposure to gadolinium-based contrast agents. Six children had been reported in both the FAERS and the literature, four in the FAERS and the ICNSFR registry and five in all three data sources.

Conclusion

Nephrogenic systemic fibrosis has been rarely reported in children. Although rules related to confidentiality limit the ability to reconcile reports, active pharmaco-vigilance using RADAR (Research on Adverse Drug events And Reports) methodology helped in establishing the number of individual pediatric cases within the three major data sources.  相似文献   

13.

Background

Vaginal reflux is a functional voiding disorder seen in prepubertal girls without anatomical or neurological abnormality. When not associated with urinary tract infections (UTI), asymptomatic bacteriuria, post-void dribbling or daytime enuresis it may be considered a normal finding.

Objective

To review the radiographic features of vesicovaginal reflux based on multiple imaging modalities.

Materials and methods

Three girls aged 11, 13 and 5 years were referred for pelvic US for daytime incontinence, post-void dribbling, frequency and urgency. One girl also had recurrent UTIs treated with antibiotics and was investigated for vesicoureteric reflux with US and voiding cystourethrogram (VCUG). All three were examined with MRI.

Results

Imaging appearance common to all three girls was a fluid-filled mass posterior to the bladder that disappeared after voiding. A previous VCUG in one girl had shown contrast medium refluxing into the vagina which disappeared after bladder emptying. Pelvic MRI confirmed the findings in all three girls.

Conclusion

US examination of a distended bladder followed by a post-void study easily provides the correct diagnosis of vesicovaginal reflux by identifying the vagina as the fluid-filled mass. Treatment involves behavioural modifications. Though well known to urologists, this may be a perplexing pathology for the inexperienced trainee radiologist.  相似文献   

14.

Background

Childhood-onset eczema is a common condition associated with pruritus, sleep disturbance and disrupted quality of life. The mainstay of treatment is usage of emollients and topical corticosteroid (CS). Nevertheless, many steroid-phobic parents are very skeptical about western medicine that may contain CS. Furthermore, complementary and alternative medicine (CAM) is popular among Chinese patients in Asia and many citizens idolize CAM and believe that traditional Chinese medicine and herbs are without any side effects. Pressed by public??s quest for effi cacious and safe treatment, and lucrative profits, CAM practitioners may take the risks of prescribing steroids and ??western medicine?? in the name of traditional Chinese herbal medicine.

Methods

We report a series of illustrative cases of uninformed systemic and topical corticosteroid usage for eczema by steroid-phobic parents to alert the public of this risk. The drugs were detected by high-performance liquid chromatography with diode-array detection, liquid chromatography-tandem mass spectrometry, gas chromatography mass spectrometry, or liquid chromatography ion trap time-of-flight mass spectrometry.

Results

Five cases of uninformed corticosteroid usage for moderate-to-severe eczema by steroid-phobic parents were reported.

Conclusions

The physician caring for children with skin disease should also be aware that even steroid-phobic parents might indeed be using potent CS without awareness. The patient usually suffers chronic relapsing eczema of moderate-to-severe degree. The steroid-phobic parent is usually non-compliant in following advice on usage of emollient, topical CS, and avoidance of triggers in accordance with western doctors. The CAM practitioner, when confronted by an anxious steroidophobic parent who demands efficacious topical and/or systemic treatment, may knowingly or unknowingly be forced into prescribing potent albeit illegal products containing corticosteroids in the name of traditional Chinese herbal medicine.  相似文献   

15.

Introduction

By the end of 2009 all panel physician practices in Germany had to implement an internal quality management (QM) system. The present study investigates the implementation status in pediatric practices by autumn 2008.

Sample

A total of 457 (75%) out of 611 randomly selected pediatric practices participated in this study.

Method

In a cross-sectional study 50 quality features were studied by means of a questionnaire and analyses of variance were conducted with ??2 tests.

Results

Of the 457 pediatric practices 420 (92%) have commenced implementation of an internal QM. The most commonly applied quality measure for implementing QM was the practice self-assessment regarding quality targets and topics and the least common were patient surveys. The highest degree of realization was shown on quality features regarding practice safety and the lowest on patient responsiveness. The type of practice and years of practice showed some differences in the realization of quality features and the type of QM system chosen showed only few differences in this respect. However, practices without implemented QM systems have a significantly lower degree of realization in almost half of all quality features compared to practices with fully implemented QM systems.

Conclusion

Pediatric practices in Germany have implemented an internal QM system. An implemented internal QM enhances the realization of process based quality features.  相似文献   

16.

Introduction

Sodium phosphate-containing laxatives are commonly used as first-line treatment option for constipation in children and adolescents. Hyperphosphatemia is an infrequent, but potentially life-threatening complication of laxative application.

Methods

We report a case series of three children exhibiting severe hyperphosphatemia and hypocalcemia after utilization of sodium phosphate-containing laxatives, necessitating intensive care services in two of three cases. Additionally, we reviewed 32 case reports of similar occurrences.

Results

We identified 28 publications on the subject dating from 1968 to 2010. Mean age of all children in reports was 2.83 years; sex was approximately equally distributed. While 18 patients suffered from either pre-existing gastrointestinal comorbidity or other major systemic disease, no or only unrelated, minor conditions were present in ten children. One-third of patients received laxatives repeatedly before the incident. Findings associated with hyperphosphatemia include lethargy, dizziness, stiffness, tachypnea, tachycardia and severe dehydration in almost all cases, and tetany, carpopedal spasm, and prolonged QT interval in a subset. While about 80% of children recovered without residual findings, three deceased and one incurred persistent hypoxic brain damage.

Conclusion

Physicians should be alerted to the possibility of phosphate toxicity in children and adolescents treated with laxatives.  相似文献   

17.

Background

Tuberous sclerosis complex (TSC), an autosomal dominant genetic disorder, can lead to the development of hamartomas in various organs, including the heart, lungs, kidneys, skin and brain. The management of subependymal giant cell tumors (SGCTs) is still controversial, and peri- and/or intraventricular neoplasms may lead to life-threatening hydrocephalus. In the last years, many progresses have been made in research into the tumorigenesis and behaviors of SGCTs. This review aims to clarify the specific role of neurosurgeons in the multidisciplinary management of SGCTs in children with TSC.

Data sources

Based on the recent scientific literature and personal experience, we reviewed the up-to-date data and discussed the trends in the management of SGCTs in children with TSC. The data were collected after a bibliography made using PubMed/Medline with these terms: subependymal, subependymal giant cell astrocytoma, subependymal giant cell tumor, and tuberous sclerosis complex.

Results

SGCTs are shown to be generated from a glioneuronal lineage, but their filiation with subependymal nodules (SENs) is still under debate. While SENs may develop anywhere in the ventricular walls, SGCTs arise almost exclusively around the Monro foramina. In children with TSC, precise clinical and/or imaging criteria are mandatory to differentiate SENs that are always asymptomatic and riskless from SGCTs that have the potential to grow and therefore to obstruct cerebrospinal fluid pathways leading to hydrocephalus.

Conclusions

An earlier diagnosis of SGCT in neurologically asymptomatic children with TSC may allow a precocious surgical removal of the tumor before the installation of increased intracranial pressure signs, an attitude that is being progressively adopted to lessen the morbimortality rate.  相似文献   

18.
19.

Background

Pancreatic tumors are rare in children, resulting in limited information regarding their frequency and imaging appearances.

Objective

To review all pancreatic tumors seen in children over a decade at a large tertiary pediatric institution and to describe multimodality imaging findings.

Materials and methods

We conducted a retrospective review of imaging studies performed in children with pancreatic tumors between January 2000 and December 2012, analyzing imaging features on available US, CT and MR examinations. We reviewed patient charts for clinical features, management and final diagnosis.

Results

We included 23 children in this study. Of these, 12 had solid and papillary epithelial neoplasms (SPEN), 3 had neuroendocrine tumors, 3 had lymphoma, and 1 each had hemangioendothelioma, benign congenital cysts with adipose tissue, dendritic cell sarcoma, metastatic rhabdomyosarcoma, and lipoma. All children with SPEN were teenage girls. SPEN showed characteristic imaging features including well-defined margins with surrounding capsule, solid-cystic components and hemorrhage.

Conclusion

Pancreatic tumors are uncommon in children. SPEN is the most common tumor and is seen predominantly in teenage girls and shows characteristic imaging features.  相似文献   

20.

Objective

To test the Trier Social Stress Test for children (TSSTC) in a cohort of Indian adolescents.

Design

Cohort study

Setting

Holdsworth Memorial Hospital, Mysore, India.

Participants

Adolescent children (N=273, 134 males; mean age 13.6 yrs) selected from an ongoing birth cohort; 269 completed the test.

Intervention

Performance of 5-minutes each of public- speaking and mental arithmetic tasks in front of two unfamiliar ‘evaluators’.

Outcome measures

Salivary cortisol concentrations were measured at baseline and at regular intervals after the TSST-C. Continuous measurements of heart rate, finger blood pressure, stroke volume, cardiac output and systemic vascular resistance were carried out before, during and for 10 minutes after the TSSTC using a finger cuff.

Results

Cortisol concentrations [mean increment (SD): 6.1 (6.9) ng/mL], heart rate [4.6 (10.1) bpm], systolic [24.2 (11.6) mmHg] and diastolic blood pressure [16.5 (7.3) mmHg], cardiac output [0.6 (0.7) L/min], stroke volume [4.0 (5.6) mL] and systemic vascular resistance [225 (282) dyn.s/cm5] increased significantly (P<0.001) from baseline after inducing stress.

Conclusions

The TSST-C produces stress-responses in Indian adolescents of a sufficient magnitude to be a useful tool for examining stress physiology and its relationships to disease outcomes in this population.  相似文献   

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