Israel Medical Association Journal

Background: A good physical exam is necessary to help pediatricians make the correct diagnosis and can save unnecessary imaging or invasive procedures. Distraction by medical clowns may create the optimal conditions for a proper physical examination.

Methods: Children aged 2–6 years who required physical examination in the pediatric emergency department were recruited and randomly assigned to one of two groups: physical exam by a pediatrician in the presence of caregivers vs. physical exam with the assistance of a medical clown. Outcome measures consisted of the level of child's discomfort, anxiety, and the quality of the physical examination.

Results: Ninety three children participated. Mean age was 3.3 ± 3.6 years (range 2–6). The duration of the physical exam was similar between the clown and control groups (4.6 ± 1.4 minutes vs. 4.5 ± 1.1 minutes (P = 0.64). The duration of discomfort was shorter in the clown group (0.2 ± 0.6 minutes) than the control group(1.6 ± 2.0 minutes, P = 0.001). In the medical clown group, 94% of pediatricians reported that the medical clown improved their ability to perform a complete physical examination. A trend of less hospitalization in the medical clown group was also noticed (11.3% in the medical clown group vs. 18.3% in the control group, P = 0.1); however, further study is required to verify this observation.

Conclusions: Integration of a medical clown in physical examination improves the overall experience of the child and the caregivers and helps the pediatrician to perform a complete physical examination.

Background: During the past decades, beta thalassemia major (TM) and beta thalassemia intermedia (TI) have transformed from a universally fatal disease at a young age into a chronic disease. This advancement is attributed to improved chelation therapy as well as enhanced management strategies, with focused attention on disease and treatment-related complications.

Objectives: To describe characteristics of adults with thalassemia as well as treatment modalities, disease and treatment-related complications, and socioeconomic information of the patients.

Methods: We preformed a retrospective analysis of 14 adult patients > 35 years of age with TM and TI who were treated at our institute, a single center specializing in the care of adult thalassemia patients living in Israel, between the years 2006 and 2016.

Results: The median age of patients was 37 years and most patients were transfusion-dependent. The median number of chelation therapeutic lines was three, and 85.7% of patients were treated at one point by combination chelation therapy. Most patients suffered from at least some form of endocrine dysfunction (n=12), and four patients developed overt heart failure. Of the patients, 85% had completed at least a high school education, 78% were employed, and 64.2% were married.

Conclusions: Prolonged survival of thalassemia patients in recent years has been accompanied by a new set of challenges for both the patients and the treating staff. Further research is warranted to improve both medical management and the socioeconomic well-being of this unique group of adult thalassemia patients.

A 47 year old man presented with a combination of dry mouth and lightheadedness while standing. His medical background was unremarkable except for cigarette smoking and hyperlipidemia. Sjögren’s syndrome was ruled out, and he was referred for evaluation of orthostatic hypotension, which by then included syncopal episodes and injuries. Additional symptoms included dry eyes, constipation, reduced sweating, and erectile dysfunction. After excluding medications and structural cardiac abnormalities as causes of orthostatic hypotension, a clinical autonomic evaluation was performed. The pattern of beat-to-beat blood pressure associated with performance of the Valsalva maneuver, and a low plasma norepinephrine level that did not increase in response to standing, established that the orthostatic hypotension was neurogenic. Treatment with an alpha-adrenoceptor agonist and fludrocortisone yielded partial improvement. After systemic diseases involving autonomic failure were excluded, cardiac sympathetic neuroimaging was performed by ¹²³I-metaliodobenzylguanidine (MIBG) scanning. The normal uptake seen in the heart indicated intact post ganglionic sympathetic innervation. There were no signs of central neurodegeneration or peripheral neuropathy. Because of symptoms and signs of both parasympathetic and sympathetic failure without denervation, an autonomic ganglionopathy was considered. A high titer of antibody to the neuronal nicotinic receptor, which mediates ganglionic neurotransmission, was obtained. The diagnosis of autoimmune autonomic ganglionopathy (AAG) was made, and the management strategy shifted to first lowering the antibody burden by plasma exchanges and then instituting chronic anti-autoimmune treatment with rituximab and a low dose of cortiosteroid. The patient showed remarkable improvement.

Background: Magnetic resonance imaging (MRI) is the most sensitive imaging modality for the detection of sacroiliitis. Diagnosing sacroiliitis on MRI is not always straightforward and can be challenging in some cases.

Objectives: To evaluate the prevalence of alternative diagnoses suggested by MRI and characterize the MR appearance of the most common ones.

Methods: Consecutive MRI examinations of the sacroiliac joints (SIJ) performed between 2005 and 2012 were retrospectively evaluated for the presence of structural and active sacroiliitis findings according to the Assessment of SpondyloArthritis International Society guidelines. Alternative diagnoses, including degenerative changes, diffuse idiopathic skeletal hyperostosis (DISH), Osteitis condensans ilii (OCI), septic sacroiliitis/discitis, stress reaction as well as anatomic variants, were registered

Results: We evaluated 281 MRI examinations, 116 males, 165 females, average age 44 ± 15 years. Sacroiliitis was found in 71 examinations (25%) and alternative diagnoses were suggested in 87 (31%) (OCI 8.9%, anatomic variants 5.3%, septic sacroiliitis 5.3%, degenerative findings 4.3%, diffuse idiopathic skeletal hyperostosis [DISH] 1.5%, stress reaction 0.7%, tumor 0.3%). A normal examination was found in the remaining 123 examinations. Patients with alternative diagnoses were older than those with sacroiliitis (62 vs. 47 years of age, respectively, P > 0.05). Alternative pathologies in the SIJ were significantly more common in females (66) than males (21), P < 0.05.

Conclusions: A substantial proportion of patients with suspected sacroiliitis had normal SIJ while the rest were more commonly diagnosed with other pathologies. A referral by an experienced rheumatologist may improve the sensitivity and specificity of this important examination.

Background: Metal-on-metal total hip prostheses (MoM-THR) have been shown to produce hypersensitivity reactions and fluid collection (pseudotumor) by the hip as well as high blood metal ions levels (BMILs).

Objectives: To evaluate the magnetic resonance imaging (MRI) signal-to-noise ratio (S/N) in selected body tissues around the hip of patients who underwent MoM hip replacement and to correlate to BMILs.

Methods: Sixty-one MRI hip examinations in 54 post-MoM-THR patients (18 males, 36 females, mean age 65 years) were retrospectively evaluated independently by two readers. The mean S/N ratio in a region of interest was calculated for periprosthetic pseudotumor collection (PPC), the bladder, fat, and muscle on axial T1w, FSE-T2w, and short tau inversion recovery (STIR) sequences on the same location. BMILs were retrieved from patient files.

Results: PPC was detected in 32 patients (52%) with an average volume of 82.48 mm³. BMIL did not correlate with the presence of PPCs but positively correlated with the PPC's volume. A trend for positive correlation was found between BMILs and S/N levels of STIR images for muscle and bladder as well as for PPC and cobalt levels. A trend for correlation was also seen between BMIL with PPC's T1 w S/N.

Conclusions: Alteration of MRI S/N for different hip tissues showed a tendency for correlation with BMILs, possibly suggesting that metal deposition occurs in the PPC as well as in the surrounding tissues and bladder.

Background: Magnetic resonance imaging (MRI), which has recently become the leading imaging modality in the study of ankylosing spondylitis (AS), has not been evaluated in the assessment of disease-specific changes at the craniocervical junction (CCJ) in patients with AS.

Objectives: To describe the spectrum of active inflammatory lesions at the CCJ using MRI in a cohort of patients with AS and neck pain.

Methods: The study included 18 patients with AS presenting with neck pain and a control group of 9 fibromyalgia patients matched for age and levels of neck pain. All patients underwent a focused rheumatologic examination, X-ray of the cervical spine, and a 3T MRI study, which included STIR, CUBE T2, FSE and FSE FAT SAT sequences before and after administration of gadolinium.

Results: The median age of AS patients was 43 years with a median disease duration of 7 years. Fifteen of 18 patients were under biologic treatment. Seven of 18 AS patients had evidence of cervical syndesmophytes on X-ray films. Active inflammatory lesions of atlanto-occipital joints and apical and alar ligaments were detected in MRIs in 2 out of the 18 patients with AS and in none of the patients with fibromyalgia. Both AS patients with active inflammation of CCJ detected on MRI received treatment with biological agents prior to and during the study.

Conclusions: Active inflammation of both entheses and joints of the CCJ can be demonstrated by MRI in patients with AS.

Background: Blogs have become a major venue of information sharing and emotional release for people worldwide. Illness blogs are a specific type of blog in which patients describe their experience with illness and coping with disease. Illness blog research has been conducted on several disorders; however, blogs by Parkinson's disease (PD) patients have been neglected.

Objectives: To categorize the characteristics of PD patients from blogs and explore whether we can learn about the medical issues with which they are most concerned, as conveyed through their blog posts.

Methods: Using located PD patients' blogs, we analyzed the contents of 78 blogs, and employed thematic analysis of eight arbitrarily selected blogs.

Results: The majority of blog authors in our sample (N=78) were from the United States (42%). The number of blogs written by male and female authors was fairly similar: 49% and 44%, respectively, while gender was unknown for 7%. Blogs were written by both early- and late-onset PD patients. The thematic analysis revealed five major themes: diagnosis, symptoms, treatment, coping mechanisms, and information.

Conclusions: Thematic analysis of blogs by PD patients provided considerable information and insight regarding the experience of these patients, which is significant to other patients and their families, as well as to medical professionals. Further qualitative studies of larger blog samples utilizing data mining techniques are needed to further explore the subjective experiences of patients.

Background: Neuroblastoma is the most common non-central nervous system (CNS) solid malignant tumor in children. The surgical treatment of high-risk neuroblastoma presents a challenge, and the benefits of aggressive surgical resection have been called into question.

Objectives: To examine our experience with surgical resection of neuroblastoma.

Methods: We report on a retrospective chart review of our preliminary surgical experience in 25 patients with neuroblastoma who underwent surgery performed by a single surgeon at two institutions over a 3 year period. Demographic data, including stage of tumor and risk stratification, were recorded. Primary outcome was total gross resection. Patients were followed for 3 years after surgery.

Results: We found that 80% of the patients, including those with high-risk neuroblastoma tumors, had total gross resection of their tumor with minimal operative morbidity and no mortality; 88% had greater than 90% resection of their tumor. Overall, 3 year survival was 84% (21/25).

Conclusions: Resection of neuroblastoma, even large, high-risk, bilateral tumors, was possible when performed by surgical teams with considerable experience.

Enthesitis is a term that refers to inflammation at tendon, ligament, or joint capsule insertions. The entheses are increasinlgly considered to be the primary site of joint inflammation in the spondyloarthropathies including psoriatic arthritis (PsA). Great advances have occurred in the understanding of enthesopathy, which has resulted in a better understanding of the etiopathogenesis of PsA. Enthesitis is difficult to assess on both clinical examination and on imaging because of the overlap in features between mechanical, degenerative, and inflammatory pathologies. Ultrasonography frequently detects entheseal abnormalities in patients with psoriasis, despite the absence of clinical symptoms of arthropathy and the longitudinal value of such lesions for PsA prediction remains unknown. The role of magnetic resonance imaging (MRI) in the assessment and monitoring of enthesitis is not fully agreed on but it is clearly superior for the assessment of spinal polyenthesitis and for diffuse peri-enthseal osteitis that can occur anywhere in the skeleton. Nuclear medicine, including conventional positron-emission tomography (PET) and high-resolution PET scan (hrPET), is more of a research tool for enthesitis and can, for example, help distinguish between PsA and osteoarthritis. Entheseal abnormalities are common in osteoarthritis, which creates diagnostic difficulty from PsA. Entheseal changes, especially on imaging, may also occur in rheumatoid arthritis (RA) and likely reflect the extension of the inflammatory process from the adjacent synovium. The nail is anatomically anchored to the skeleton via a mini-enthesis network. An association between ultrasonography determined distal interphalageal joint (DIP) extensor tendon enthesopathy and clinical nail disease was found, which highlights the pivotal role of the enthesis in this PsA risk factor. This review summarizes the relevant insights and implication of imaging for enthesitis, primarily in PsA but also in other arthropathies.

Axial spondyloarthritis (axSpA) covers the stage of non-radiographic axial spondyloarthritis (nr-axSpA) and classic ankylosing spondylitis. The pathognomonic findings of axSpA are mainly inflammatory and osteoproliferative changes in the sacroiliac joints (SIJ) and the spine. Various imaging techniques are being used in daily practice for assessment of disease-specific changes, such as periarticular bone marrow edema, erosions, sclerosis, fat metaplasia and ankylosis in the SIJ or spondylitis, spondylodiscitis, facet joint involvement, or syndesmophytes in the spine of patients with axSpA. Conventional radiographs are still considered the gold standard for assessment of structural changes, while the method of for detection of inflammatory changes is magnetic resonance imaging (MRI).

A result for an MRI in the SIJ is considered positive for axSpA when more than one lesion is present on one MRI slice, If there is one lesion only, this should be present on at least two consecutive slices. For the spine, inflammatory lesions should preferably be located in the corner of the vertebral bodies, while occurrence of spondylitis in three or more vertebral corners is considered highly suggestive of axSpA.

This review gives a detailed overview about the benefits and limitations of all available imaging techniques in patients with axSpA, explains the usage of imaging techniques in the context of diagnosis and differential diagnosis of the disease, and reports on the potential future trends in the area of imaging of the axial skeleton in patients who are suspicious for this diagnosis.