Novel MRI techniques in the diagnosis of musculoskeletal infection in children

Abstract Introduction: Musculoskeletal infections in children are common and if not adequately treated can result in poor prognoses. Early diagnosis and rapid treatment are crucial. The clinical and laboratory results are often non-conclusive or misleading. Radiology has always played a part in the diagnosis of Paediatric musculoskeletal infections. MRI is the most sophisticated MSK imaging tool and advanced MRI protocols with tailored techniques can improve the diagnostic yield of MRI in Paediatric MSK infection. Methods: 99 children with acute MSK pain and signs of infection were prospectively recruited. Each patient had a MRI scan on the first day of admission. A tailored MRI protocol was utilised, consisting of structural and biodynamic (DWI & SCE) sequences. Two consultant MSK radiologists independently analysed the scans and completed evaluation forms consisting of qualitative and semi-quantitative questions. The quantitative data from DWI and SCE sequences were separately analysed.Results: 103 MRI scans were performed. 3 scans were excluded due to incomplete protocols. 5 quantitative DWI and 3 SCE analyses were excluded due to irregularities of the values. The most common clinical diagnosis was infection. The feasibility of the protocol was excellent. The accuracy of the MRI protocol was good-excellent. The most accurate sequence was SCE. The diagnostic accuracy of the readers was good compared to an agreed gold standard. The most valued sequence by the readers for the diagnostic process was SCE. There were no relevant statistical thresholds for ADC and most of permeability values except for one regarding MSK infection. Conclusion: The tailored MRI protocol of the study is a feasible diagnostic tool with high level of diagnostic accuracy for paediatric MSK infections. The advanced MRI techniques in this study are the strengths of the protocol, which have demonstrated high accuracy and subjective diagnostic confidence. The quantitative values of the biodynamic sequences are promising parameters which need further evaluation in future studies.Open Acces

Assessment of the Cytotoxic Activity of Triphala: A Semisolid Traditional Formulation on HepG2 Cancer Cell Line

Cancer chemotherapies may result in resistance, and therefore, contemporary treatments including natural products may find an increasing consideration. As per Persian medicine (PM), many natural products have been used for malignant and chronic diseases. Triphala, with a combination of Terminalia chebula Retz., Terminalia bellirica Retz., Phyllanthus emblica L., and honey, is a multi-ingredient traditional formulation attributed to anticancer activities in PM. This study is aimed at evaluating the cytotoxic activity of this preparation on HepG2, the human liver cancer cell line. Hydroalcoholic extracts were prepared from the formulation and its components. Compared with the control and Cisplatin, the extracts were tested using MTT assay at different concentrations. All concentrations of the preparation, as well as Cisplatin, were effective significantly against HepG2 cells. All extract preparations at multiple concentrations were significantly effective as evidenced by MTT assay when compared to the control group. The IC50 level for Triphala extract was 77.63±4.3 μg/ml. Based on the results, Triphala and its components have cytotoxic activity on the HepG2 cancer cell line and they can reduce the survival rate significantl

The clinical and genetic spectrum of autosomal-recessive TOR1A-related disorders.

In the field of rare diseases, progress in molecular diagnostics led to the recognition that variants linked to autosomal-dominant neurodegenerative diseases of later onset can, in the context of biallelic inheritance, cause devastating neurodevelopmental disorders and infantile or childhood-onset neurodegeneration. TOR1A-associated arthrogryposis multiplex congenita 5 (AMC5) is a rare neurodevelopmental disorder arising from biallelic variants in TOR1A, a gene that in the heterozygous state is associated to torsion dystonia-1 (DYT1 or DYT-TOR1A), an early-onset dystonia with reduced penetrance. While 15 individuals with TOR1A-AMC5 have been reported (less than 10 in detail), a systematic investigation of the full disease-associated spectrum has not been conducted. Here, we assess the clinical, radiological and molecular characteristics of 57 individuals from 40 families with biallelic variants in TOR1A. Median age at last follow-up was 3 years (0-24 years). Most individuals presented with severe congenital flexion contractures (95%) and variable developmental delay (79%). Motor symptoms were reported in 79% and included lower limb spasticity and pyramidal signs, as well as gait disturbances. Facial dysmorphism was an integral part of the phenotype, with key features being a broad/full nasal tip, narrowing of the forehead and full cheeks. Analysis of disease-associated manifestations delineated a phenotypic spectrum ranging from normal cognition and mild gait disturbance to congenital arthrogryposis, global developmental delay, intellectual disability, absent speech and inability to walk. In a subset, the presentation was consistent with fetal akinesia deformation sequence with severe intrauterine abnormalities. Survival was 71% with higher mortality in males. Death occurred at a median age of 1.2 months (1 week - 9 years) due to respiratory failure, cardiac arrest, or sepsis. Analysis of brain MRI studies identified non-specific neuroimaging features, including a hypoplastic corpus callosum (72%), foci of signal abnormality in the subcortical and periventricular white matter (55%), diffuse white matter volume loss (45%), mega cisterna magna (36%) and arachnoid cysts (27%). The molecular spectrum included 22 distinct variants, defining a mutational hotspot in the C-terminal domain of the Torsin-1A protein. Genotype-phenotype analysis revealed an association of missense variants in the 3-helix bundle domain to an attenuated phenotype, while missense variants near the Walker A/B motif as well as biallelic truncating variants were linked to early death. In summary, this systematic cross-sectional analysis of a large cohort of individuals with biallelic TOR1A variants across a wide age-range delineates the clinical and genetic spectrum of TOR1A-related autosomal-recessive disease and highlights potential predictors for disease severity and survival

Cervical lymph node metastasis in adenoid cystic carcinoma of the larynx: a collective international review

Adenoid cystic carcinoma (AdCC) of the head and neck is a well-recognized pathologic entity that rarely occurs in the larynx. Although the 5-year locoregional control rates are high, distant metastasis has a tendency to appear more than 5 years post treatment. Because AdCC of the larynx is uncommon, it is difficult to standardize a treatment protocol. One of the controversial points is the decision whether or not to perform an elective neck dissection on these patients. Because there is contradictory information about this issue, we have critically reviewed the literature from 1912 to 2015 on all reported cases of AdCC of the larynx in order to clarify this issue. During the most recent period of our review (1991-2015) with a more exact diagnosis of the tumor histology, 142 cases were observed of AdCC of the larynx, of which 91 patients had data pertaining to lymph node status. Eleven of the 91 patients (12.1%) had nodal metastasis and, based on this low proportion of patients, routine elective neck dissection is therefore not recommended

Pemphigoid Gestationis: A Retrospective Study in Southwest Iran

Pemphigoid gestationis (PG) is a rare autoimmune bullous dermatosis of pregnancy usually presents in the second or third trimester. It is characterized by pruritic, urticarial plaques with the development of tense vesicles and bullae within the lesions. Pathogenesis of PG is not fully established, however, most patients develop circulating autoantibodies targeting the bullous pemphigoid (BP) 180 antigen. The aim of this work is to draw a profile of the epidemiology, clinical aspects, treatment and evolution of the disease by studying hospital series. We retrospectively investigated the 13 patients who were diagnosed with PG based on hospital data at the Referral Center of Southwest Iran located in Ahvaz city between March 2002 and March 2011. The age of onset was 21 to 40 years (mean age: 27.5 years). The onset of the disease occurred in the second trimester of pregnancy in 6 patients and in the third trimester of pregnancy in 4 patients. One patient had a flare up of disease during the first trimester and two out of cases in puerperium period. In all cases, pruritus was the first symptom, followed by an erythematous vesiculobullous eruption.  The diagnosis of PG was confirmed by skin biopsy. Ten out of the patients treated with oral corticosteroids (0.5-1 mg/kg/day), one of the patients underwent oral corticosteroids plus topical glucocorticoid and the last patient treated with topical glucocorticoid. PG remains a rare dermatosis of pregnancy. Our series had two particularities compared to other studies: high frequency in primigravida and the frequent involvement of the face. Additionally our study demonstrated that improvement could occur faster and provide acceptable management if the treatment of the patients would be implemented sooner

A Study of 13315 Papanicolau Smear Diagnoses in Shohada Hospital

Objective: This study aimed to review Papanicolaou (Pap) smears in patients of Shohada hospital in Tehran. Materials and methods:  A cross-sectional descriptive study was carried out and a database search of all Pap smear reports from 1995 to 2005 at cytopathology department was reviewed. The abnormal Pap smear reports based on the Bethesda System were assessed. Results: A total of 13315 Pap smear reports were reviewed from patients aging 20 to 79 years old. Totally 6065 (45.55%) cases had diagnosis of ‘negative', 293(2.21%) smears were labeled as ‘inadequate', and 6799 (51.06%) reports had ‘Benign cellular changes'. There were 158(1.18%) abnormal Pap smears consisting of 84 (53.18%) SIL with undetermined grade (ASC-US), 28(17.73%) low grade SIL (LSIL), 17(10.75%) high grade SIL (HSIL), 27(17.08%) carcinoma and 2(1.26%) atypical glandular cells of undetermined significance (AGUS). Overall incidence of the cervical cancer among these samples was 0.2%. Benign cellular changes numbered 6799(51.06%), of which 5365 (78.9%) smears demonstrated infectious processes. Conclusion: The number of abnormal pap smears in this study is less than western countries, but is comparable with Middle East and Islamic regions. More prospective studies are recommended

Modified unilateral periureteral injection technique in the treatment of patients with high-grade vesicoureteral reflux: A study of primary findings

Objective: Although endoscopic treatment has been used by many pediatric urologists for the treatment of vesicoureteral reflux (VUR), it has no considerable success in high-grade VUR. We aimed to describe the primary outcomes of unilateral periureteral injection technique (PIT), as well as bilateral PIT in high-grade VUR. Methods: In this prospective study, we examined 92 ureters in 45 boys and 40 girls from February 2010 to May 2018. Bilateral PIT and unilateral PIT were applied in 67 and 25 refluxing units, respectively. In the unilateral PIT, the subureteral injection site was only at the 5- or 7-o'clock position. However, in the bilateral PIT, the subureteral injection sites were at 5- and 7-o'clock position. Pre- and post-operative reflux grades were evaluated by voiding cystourethrography 6 months after surgery. Results: Seven patients had bilateral reflux. Overall, 75 (81.5%) ureters showed Grade IV VUR, while 17 (18.5%) had primary Grade V VUR. The mean age of the subjects was 39.2 months. In unilateral PIT ureters, VUR was resolved in 23 (92.0%) refluxing units. It was downgraded to Grade III in one ureter (4.0%) and to Grade II in another ureter (4.0%). In addition, in bilateral PIT cases, VUR was resolved in 60 (89.6%) ureters; it downgraded to Grades II and III in 3 (4.5%) and 4 (6.0%) refluxing units, respectively. Conclusion: Unilateral PIT can be highly effective in the treatment of selected ureters of high-grade VUR. However, further studies are needed to confirm our results