Ultrasound in the diagnosis of calcium pyrophosphate dihydrate deposition disease. A systematic literature review and a meta-analysis.

OBJECTIVE: Ultrasonography (US) demonstrated to be a promising tool for the diagnosis of calcium pyrophosphate dihydrate deposition disease (CPPD). The aim of this systematic literature review (SLR) was to collect the definitions for the US elementary lesions and to summarize the available data about US diagnostic accuracy in CPPD. METHODS: We systematically reviewed all the studies that considered US as the index test for CPPD diagnosis without restrictions about the reference test or that provided definitions about US identification of CPPD. Sensitivity and specificity were calculated for each study and definitions were extrapolated. Subgroup analyses were planned by anatomical site included in the index text and different reference standards. RESULTS: Thirty-seven studies were included in this review. All the studies were eligible for the collection of US findings and all definitions were summarized. US description of elementary lesions appeared heterogeneous among the studies. Regarding US accuracy, 13 articles entered in the meta-analysis. Considering each joint structure, the sensitivity ranged between 0.77 (0.63-0.87) and 0.34 (0.16-0.58) while the specificity varies between 1.00 (0.89-1.00) and 0.92 (0.16-1.00). Considering the reference standards used, the sensibility ranged between 0.34 (0.02-0.65) and 0.87 (0.76-0.99) while specificity ranged between 0.84 (0.52-1.00) and 1.00 (0.99-1.00). CONCLUSION: US is potentially a useful tool for the diagnosis of CPPD but universally accepted definitions and further testing are necessary in order to assess the role of the technique in the diagnostic process.

Understanding bisphosphonates and osteonecrosis of the jaw: uses and risks.

OBJECTIVE: Bisphosphonates are chemically stable analogs of pyrophosphate compounds, which have been used to treat multiple disorders of calcium metabolism. Although bisphosphonates have been employed for many years and have demonstrated an excellent safety profile, severe osteonecrosis of the jaw (ONJ) has been described in patients with bone metastases who have been treated with bisphosphonates. METHODS: In this review we describe the reasons for ONJ and discuss the varying effects of different bisphosphonates on the development of ONJ. Bisphosphonates tend to accumulate in bone, subject to remodeling (such as the jaw) and can affect osteoclast-mediated bone resorption and osteoclast formation, leading to the osteonecrosistic phenomenon. RESULTS: Risk factors for previously -treated patients include the type of bisphosphonates (amino or non-amino), length of treatment and route of administration, the presence of co-morbidities and/or treatment with immune-suppressing drugs, and the presence of other risk factors in addition to the type of intervention required. In oncological patients currently in treatment with receiving intravenous bisphosphonates, greater consideration must be taken depending on the length of treatment already undertaken and concomitant therapies. In these patients, a preventive dental surgery visit and examination of the case would be advisable prior to beginning treatment with bisphosphonates. CONCLUSIONS: Practical approaches in the prevention of ONJ include thorough pre-treatment evaluation and performing any preventative procedures (treat periodontal conditions, extract loose teeth, provide protective and endodontic therapies); initiating amino-bisphosphonates only after any gum tissue damage has healed; establishing a regimented check-up schedule and hygieneic precautions the patient can take; and during bisphosphonate treatment conduct any dental procedures in the least invasive manner during bisphosphonate treatment.

Change perspective to increase diagnostic accuracy of ultrasonography in calcium pyrophosphate dehydrate deposition disease! A new approach: the axial scan of the meniscus.

Ultrasonography (US) is a relevant tool in the study of calcium pyrophosphate dihydrate (CPP) deposition disease. However, differential diagnosis of hyperechoic deposits within the fibrocartilage can be difficult; moreover, US study is limited by the need of an adequate acoustic window. We describe a US scanning technique that offers a new viewpoint in the study of knee meniscal structure: a longitudinal scan performed according to the long axis of meniscus. This technique proves to be particularly useful for the identification of CPP deposition, but could also improve the US diagnostic utility and accuracy in other meniscal pathologies.

Paget's disease.

Paget's disease of bone is the most common metabolic bone disease after osteoporosis and affects 2-4% of adults over 55 years of age. Its etiology is only partly understood and includes both genetic and environmental factors. The disease may be asymptomatic and can be uncovered incidentally on x-ray or in biochemical tests performed for another condition. It can also manifest itself with bone pain, deformity, fracture or other complications. Paget's disease is diagnosed by x-rays and in general has very typical radiological features, but occasionally the clinical picture may be unusual and a differential diagnosis of sclerotic or lytic metastases needs to be considered. Plasma total alkaline phosphatase activity is the most clinically useful indicator of disease activity. It is elevated in most untreated patients, but may be within the normal range in patients with monostotic or limited disease. Bisphosphonate therapy is indicated for patients with symptoms and should also be considered in patients with disease sites that suggest a risk of complications, such as long bones, vertebrae or base of the skull. Orthopedic surgery in Paget's disease patients includes almost exclusively the correction of fractures and arthroplasty.

Prevalence of Baker's cyst in patients with knee pain: an ultrasonographic study.

The objectives of this study are to investigate the prevalence of Baker's cyst (BC) in patients with knee pain, and to assess the correlation between BC and severity of osteophytes and joint effusion. A retrospective study was conducted on a group of patients with knee pain referred to our outpatient clinic for ultrasonography of the knee between January 2010 and February 2011. Patients underwent an ultrasonographic exam of the knees to assess the presence of marginal femorotibial osteophytosis, joint effusion and BC. A dichotomous score was assigned to each item (1 present, 0 absent) and severity of US signs of osteoarthritis and joint effusion were also graded semiquantitatively. Collected data were processed using logistic regression analysis to evaluate the correlation between degree of osteophytosis and joint effusion and BC. Patients affected by inflammatory joint conditions or with history of joint surgery or recent trauma were excluded. A total of 399 patients with knee pain were studied (299 women), in the age range 18-89 years (mean 56.2, SD 16.3 years). 293 patients (73.4%) showed sonographic features of osteoarthritis and 251 (62.9%) joint effusion. BC was found in 102 patients (25.8%) together with a positive association with sonographic features of osteoarthritis and joint effusion. Our data show a prevalence of BC of 25.8% in a population of patients with knee pain, and suggest that BC is positively related to osteoarthritis and joint effusion. Ultrasonographic examination of knee is worthwhile in patients with painful osteoarthritis or evidence of effusion.

Ultrasonography vs. clinical examination in children with suspected arthritis. Does it make sense to use poliarticular ultrasonographic screening?

OBJECTIVES: Juvenile idiopathic arthritis (JIA) is a term that encompasses all forms of arthritis that begin before the age of 16 years old, persist for more than 6 weeks and are of unknown cause. The ILAR criteria for JIA classification are based on the number of joints involved. The aim of our study was to compare clinical evaluation and ultrasonography (US) in the assessment of joint synovitis in children with suspected JIA. METHODS: We enrolled in our study all children who presented at our outpatient clinic of Paediatric Rheumatology with suspected JIA. All the children underwent a clinical examination for joint swelling (40 joints), a tender joint count (42 joints) and US examination (42 joints) on the same day. They all returned to the clinic after approximately 2 weeks with the results of the tests prescribed at the first visit and a diagnosis was formulated. RESULTS: Thirty-one children were enrolled. More synovitis was identified by US than by than clinical examination (42 joints vs. 27). Clinical examination classified as swollen 13 joints that did not result affected at US. Of the 94 painful joints, 24 were affected by synovitis at US. The final diagnoses were: 9 children with JIA (any form), 9 were classified as healthy and 13 with other diseases. One child was reclassified and 2 were diagnosed with JIA thanks to US. CONCLUSIONS: US detected more synovitis than clinical examination in children with suspected JIA, therefore, US should be included in the screening procedure of children with suspected JIA.

Ulnar neuropathy at the elbow: how frequent is the idiopathic form? An ultrasonographic study in a cohort of patients.

OBJECTIVE: Ulnar neuropathy at the elbow (UNE) is the second most frequent focal neuropathy of the arm. The aim of our study was to establish the frequency of anatomical changes of the cubital tunnel capable of causing UNE. METHODS: Ninety-one consecutive patients affected by UNE, as established by neurophysiological studies, were enrolled in the study. All patients underwent ultrasonographic examination of the elbow, paying particular attention to the cubital tunnel, which was studied with either static or dynamic scans. RESULT: Fifty-four of the 91 patients (59.3%) had at least one anatomical alteration of the cubital tunnel. The changes observed in our patients were: subluxation of the ulnar nerve (18.7%), luxation of the ulnar nerve (9.9%), presence of osteophytes (6.6%), presence of accessory muscle (8.8%), articular ganglion (1.1%), post-traumatic lesions (3.3%), presence of osseous fragment (1.1%). CONCLUSIONS: A possible cause of ulnar nerve entrapment at the elbow was found in more than half of the patients. Joint ultrasonography is indispensable for the identification of such alterations as it allows for both static and dynamic evaluation of the ulnar nerve.

Occurrence of autoimmunity in a long-term survivor with metastatic colon carcinoma treated with a new chemo-immunotherapy regimen.

GOLFIG-1 chemo-immunotherapy is a new translational anticancer regimen based on the combined use of gemcitabine, oxalipatin, levofolinic acid and infusional 5-fluorouracil together with the subcutaneous administration immunoadjuvant cytokines (GM-CSF and ultra low dose IL-2). This regimen, tested in a phase II trial, was safe and very active in patients with metastatic colorectal carcinoma and it has been shown to have powerful immunobiological activity. Treatment with the GOLFIG regimen resulted in the induction of a colon cancer specific cell mediated immune response associated with a significant reduction in the percentage of peripheral regulatory T (T(reg)) cells, a very immunosuppressive lymphocyte subset which is commonly over-represented in cancer patients. These cells are able to prevent the occurrence of autoimmunity in response to immunological stimuli, thus their malfunctioning has been associated with the occurrence of auto-immune diseases but may also be responsible for more efficient anticancer immune reaction. In this manuscript we describe a clinical case concerning a patient with metastatic colon carcinoma who responded to the GOLFIG regimen, showed symptoms of autoimmunity [Discoid Lupus Erythematosus (DLE)] and had a very long survival.

Placement of two overlapping uncovered metallic stents for malignant gastric outlet and duodenal obstruction due to colon cancer.

Malignant obstruction of the gastric outlet and duodenum is frequently due to extrinsic involvement by tumors from contiguous organs, in particular from pancreas and gallbladder. The treatment of malignant gastroduodenal stenoses is difficult. Many patients have advanced malignant disease and are too ill to undergo surgical approach. Surgical gastrojejunostomy has been considered the palliative treatment of choice. Metallic stents can be useful in this condition with adequate palliation obtained in most cases. We report a case in which self-expanding metallic stents were placed for stenoses of the gastric outlet and duodenum due to a colon cancer.

Evolution of surgery for liver hydatidosis from 1950 to today: analysis of a personal experience.

Hydatid disease (echinococcosis) remains a complex worldwide problem in rural areas. Medical treatment does not definitively cure hydatid cysts, and surgical management aims to eliminate the parasite, favoring rapid disappearance of the residual cavity, preventing complications and recurrence, and shortening the hospital stay. In this consecutive series, 298 patients with liver hydatidosis were treated surgically by the authors from 1950 to 1987 in various hospitals in Rome, Italy. Surgery comprised conservative methods (marsupialization and partial cystopericystectomy) and radical methods (total pericystectomy and liver resection). Liver resection was used only if unavoidable. Complications were exudate from the residual cystic cavity, cholerrhagia, and nonspecific incisional fistula. In the entire series, morbidity was 8.7% of which 12.6% was seen with conservative methods and 5.5% with radical methods (p < 0.05); mortality was 3.6%, of which 5.9% was seen with conservative methods and 1.8% with radical methods (p = NS). Over the 37 years, as medical science and complementary therapies progressed, the use of conservative operations diminished and radical treatments increased. Long-term follow-up showed that hydatid disease relapsed more frequently in patients who underwent conservative or subtotally radical treatment than in those who underwent radical surgery: eight patients (11.2%) versus one (0.9%) (p < 0.01). Hence surgical treatment that removes all of the pericyst and preservation of the nonpathologic liver are important.

Malignant metastatic eccrine poroma. Proposal for a new therapeutic protocol.

BACKGROUND: Malignant metastatic eccrine poroma is a very rare cutaneous neoplasm, and consequently the references in the literature regarding the treatment of this tumor, known also as porocarcinoma, are very poor. OBJECTIVE: To call attention to a new therapeutic protocol in the treatment of metastatic porocarcinoma, as well as to underline an antineoplastic efficacy of vitamin A analogues. METHODS: The results are presented on the basis of the clinical case of a malignant eccrine poroma with metastatic regional lymph nodes. RESULTS: With our new chemotherapeutic protocol, arrest of the metastatic progression was achieved after 3 months and the remission was maintained until the 10th month of therapy. CONCLUSIONS: A new chemotherapy protocol consisting of isotretinoin and interferon alpha has confirmed the advantages of polychemotherapy in the treatment of metastatic malignant eccrine poroma. On the basis of the considerably long, although incomplete, remission with good drug tolerance in spite of the high doses used as well as the undoubtedly major antineoplastic strength of the latest generation of synthetic retinoids, we feel that these findings could be a good starting point for further experimental verifications of the therapy of this aggressive cutaneous neoplasm.

Sleep organization in children at risk for sudden infant death syndrome.

The organization of sleep was studied in four groups of 6-month-old babies: monitored near-miss babies (NM), monitored siblings (HR), nonmonitored siblings (LR), and siblings considered at low risk but monitored because of marked parental anxiety (PA). It was studied using the method of cumulated occurrences and orthogonal polynomial fitting introduced for the analysis of sleep by Gaillard and Martinoli in 1976. No monitoring effect was found. We also found no difference between the groups when the usual sleep scores were used. However, differences were found with the polynomial adjustment method: there was more quiet sleep stage 3 in NM than in any other group. There was less waking state and more paradoxical sleep at the end of the night in PA and NM than in HR or LR babies. This suggests that the differences may not be related so much to real risk as to parental comportment.

[Pirenzepine parenterally administered in high doses in the treatment of upper digestive hemorrhage]. / La pirenzepina per via parenterale ad alto dosaggio nel trattamento delle emorragie digestive alte.

22 patients with moderate and severe haemorrhages of the UAC were given endovenous doses of Pirenzepine (100 mg/48 hrs). The drug was effective in 86% of the cases and its effect on pH values was demonstrated by testing before and after administration (p less than 0.01). Better results were observed in the treatment of patients with moderate and severe DUS.

[Spectral analysis of fast EEG rates in normal 6-to-16-year-old children]. / Analyse spectrale des fréquences rapides EEG chez des enfants normaux âgés de 6 à 16 ans.

Spectral analysis of EEGs from 208 school children aged from 6 to 16 shows two types of beta activity: anterior beta which is low in amplitude but increases in energy in response to eye opening and posterior beta which, like alpha, decreases in response to eye opening. After reviewing the role of the FFT in frequency analysis we conclude that anterior beta activity corresponds to the classic beta rhythm of visual analysis, while posterior beta activity probably represents harmonics of alpha or even theta activity. In any case, beta activity in the normal child is very low in amplitude and energy.