Tissue Disease In Children Research Articles

Overlap syndrome: Juvenile dermatomyositis with systemic lupus erythematosus and lupus hepatitis in a child: An uncommon presentation

Autoimmune connective tissue disease in children carries a unique management challenge to the treating physician due to the potential years of disease burden and resultant complications. Early diagnosis and treatment positively impact disease course. Overlap syndrome in children is a rare entity. We report a case of juvenile dermatomyositis with systemic lupus erythematosus who on follow-up developed lupus hepatitis. Lupus hepatitis in children with overlap syndrome is not a well-known entity and there is paucity of literature describing this association.

Incidence of the musculoskeletal system diseases in children and the organization of specialized care in Saint Petersburg

BACKGROUND: The high prevalence of musculoskeletal system and connective tissue diseases, effects on the quality of life of children, and disability level are among the main medical and social problems.
 AIM: To assess the morbidity rates and provision of medical care to St. Petersburg children with musculoskeletal diseases between 2017 and 2021.
 MATERIALS AND METHODS: Data on the incidence of musculoskeletal diseases in children of St. Petersburg were analyzed based on the Federal statistical observation forms submitted by the St. Petersburg State Budgetary Institution Medical Center for Information and Analysis, collections of N.N. Priorov National Medical Research Center for Traumatology and Orthopedics of the Ministry of Health of the Russian Federation “Trauma, orthopedic morbidity, the state of trauma and orthopedic care for the population,” edited by Acad. S.P. Mironov, RAS, and data from the Federal State Statistics Service.
 RESULTS: In Saint Petersburg, the morbidity rate of musculoskeletal and connective tissue diseases from 2017 to 2021 was 13%, excluding 2020, which was most likely due to the introduction of measures aimed at counteracting the spread of COVID-19 and the suspension of planned outpatient specialized care. The number of orthopedic beds for children decreased by 58, whereas the availability of beds for children remained at the same level and amounted to 4.3 beds per 10,000 children, which exceeded the indicator for the Russian Federation (1.0). The number of children with these disabilities increased 1.4-fold. Deaths of children due to musculoskeletal and connective tissue diseases were recorded in 2019 and 2020.
 CONCLUSIONS: The results of the 5-year observation period of musculoskeletal and connective tissue diseases in children in St. Petersburg revealed increasing incidence because of the increase in the number of children newly diagnosed with these diseases.

Особенности заболеваемости детей болезнями костно-мышечной системы и соединительной ткани в Чеченской республике.

Pathology of the musculoskeletal system is of great medical and social importance and is one of the ten most common diseases. The high prevalence of diseases is due to a variety of risk factors. Polyethics, Cultural, Territorial and Demographic Features of the Chechen Republic (high birth rate, high proportion of the child population, predominance of rural residents, shortage of medical personnel), are reflected in the specifics of the provision of medical care to the population, and the analysis of the incidence of diseases of the musculoskeletal system and connective tissue in children is of interest and the need for detailed consideration. The purpose of the study: to determine the peculiarities of primary and general morbidity of diseases of the musculoskeletal system and connective tissue in children in the Chechen Republic. Materials and methods. Primary and general morbidity rates of children with musculoskeletal and connective tissue diseases were assessed and analyzed according to official statistical reports and publications of the Federal State Statistics Service and the Ministry of Health of Russia for the Chechen Republic in dynamics for 2016–2022. Statistical processing was performed in MS Office‑2016 program (Word, Excel). Results. The study showed a low incidence of musculoskeletal and connective tissue diseases in children in the Chechen Republic. The incidence of children in the groups of 0–14 and 15–17 years for the last 7 years has generally had a decreasing dynamics, with a change of trend: a decrease in 2016–2019 and an increase in cases of most nosologies in 2020–2022. Against the background of the COVID‑19 pandemic, there was a significant increase in deforming dorsopathies and arthropathies in general, including reactive arthropathies and juvenile arthritis. At the same time, no such correlation was found in rheumatoid arthritis: against the background of the pandemic, the downward trend in the registration of cases was continued. Conclusions. When organizing medical care for children with diseases of the musculoskeletal system and connective tissue, the prevalence of pathology, the level of medical staffing and the age composition of the child population should be taken into account. During 2016–2022, a decrease in primary morbidity prevailed in children 0–14 years of age, and in adolescents – in total morbidity. The COVID‑19 pandemic (the virus itself, decreased physical activity, and conditions limiting the availability of ambulatory care) contributed to the increase in most arthropathies and deforming dorsopathies. On the contrary, the incidence of rheumatoid arthritis in children has been steadily decreasing for 7 years.

PREVALENCE OF PERIODONTAL TISSUE DISEASES IN CHILDREN WITH AUTISM

It is known that among the many factors that cause dental diseases in children, disorders of mental development and disorders of the nervous system, including autism, are important. The study of dental incidence in children with autism is relevant in view of the need to develop and implement effective methods of treatment and prevention of the main dental diseases in these children.
 The aim of the study was to study the prevalence and features of the course of periodontal tissue diseases in children with autism.
 Methods and Material. 38 children with autism and 49 practically healthy children aged 13-18 were ex-amined. Assessment of periodontal tissue condition was carried out on the basis of clinical data. For an objective assessment of periodontal status, the papillary-marginal-alveolar index (PMA) was determined (S. Parma, 1960). The prevalence of periodontal diseases was determined using WHO criteria.
 Results. When analyzing the obtained results, periodontal tissue pathology was diagnosed in children of the main group in 68.42±6.67%, which is 1.4 times more than among children in the control group (48.86±5.89%, p<0.05 ). Basically, chronic catarrhal gingivitis (CCG) was diagnosed, the prevalence of which was 60.72±4.67%. In addition, 14.99±2.46% of the children of the main group had generalized periodontitis of mild severity. It was established that, on average, children with autism have a PMA index of 39.01±3.76%, which corresponds to the average severity of gingivitis. So, among the children of the main group, a mild degree of severity of CCG was diagnosed, on average, in 41.96±3.56%, an average degree in 44.92±7.64% of children with autism, severe in 5.34±2, 33%.
 Conclusions. Summary, children with autism have a high prevalence of periodontal diseases, in particular, chronic catarrhal gingivitis and generalized periodontitis. This requires the creation of special educational programs for children with autism in order to develop algorithms for providing individualized dental care to these children.

Capillary microscopy is a potential screening method for connective tissue disease in children with Raynaud\u2019s phenomenon

BackgroundNailfold capillary microscopy (NCM) is a cornerstone in the diagnosis of Systemic Sclerosis (SSc) in adulthood. Although Raynaud’s phenomenon (RP) is very common in childhood, studies on diagnostic methods to differentiate between primary RP (PRP) and secondary RP (SRP) at a young age are scarce. The aim of this study was to determine the value of NCM in differentiating between PRP and SRP in children and adolescents with RP.MethodsIn this nested case–control study, 83 patients diagnosed with RP and having underwent NCM in childhood were retrospectively included. Based on whether they were diagnosed with a connective tissue disease (CTD) during follow-up, patients were classified as PRP or SRP. NCM was performed by a vascular technician. PRP and SRP patients were compared on demographics, NCM and serology. Variables associated with SRP were included in a multivariate logistic regression model. Predictive values were calculated for NCM, ANA positivity and the combination of NCM and ANA positivity.ResultsAt the time of the NCM, the mean age of the RP patients was 15.4 ± 2.3 years. Of these patients, 78.3% were classified as PRP and 21.7% as SRP at mean follow-up of 6.4 ± 3.20 years. CTDs were miscellaneous, with only one patient having developed SSc. Of the NCM parameters, only capillary loss was associated with SRP (p = 0.01). In a multivariate logistic regression model including ANA, capillary loss was not a predictor of SRP. In a model without ANAs, capillary loss was an independent predictor (OR = 3.98, CI 95% 1.22–12.99). Capillary loss had a sensitivity of 44.4% and a specificity of 84.4% for SRP. ANA combined with capillary loss had a sensitivity of 66.7% and a specificity of 85.7%.ConclusionWhereas RP in adulthood is most strongly associated with SSc, children with RP seem to be at risk for developing other CTDs with less apparent NCM abnormalities. Of all NCM findings, only capillary loss was predictive for SRP. NCM did not add to the predictive value of ANA screening. However, with a specificity of 84.4% and being non-invasive, NCM shows potential as a screening method for SRP. More research with a larger study population is required before drawing conclusions.

Clinical laboratory assessment and predictability of the periodontal inflammation development in children with undifferentiated connective tissue disease

Relevance. The development of multiple organ lesions in undifferentiated connective tissue disease leads to secondary immunodeficiency, which triggers oral homeostasis disruption and activates periodontal pathogens, which produce anti-inflammatory cytokines, which trigger the mechanisms of periodontal destruction. Purpose – to establish the relationship between undifferentiated connective tissue disease in children and their predisposition to periodontal inflammation and destruction.Materials and methods. The study examined the patients, aged 15 to 17 years old, of the endocrinological department of the Children's Clinical Hospital of N. N. Burdenko Voronezh State Medical University. All examined children had the same diagnosis of undifferentiated connective tissue disease. The control group consisted of 15 children with healthy periodontium. Silness-Loe plaque index (Loe H., Silness J., 1962) at the gingival margin assessed the children periodontal status. Mühlemann bleeding index (Mühlemann H.R., Son S., 1971) [19] evaluated the bleeding. The study measured the intensity and extension of the inflammatory reaction by the cytological changes in the periodontium according to the Page and Schroeder model (Page R.C. and Schroeder M. E., 1976). The enzyme immunoassay kits from eBioscince determined the level of pro-inflammatory cytokines: interleukin (IL-1β), interferon-gamma (IFN-γ) and transforming growth factor (TGF-β1) in the oral fluid; and the anti-inflammatory cytokine, receptor antagonist interleukin IL-1 (IL-1ra), was measured using Invitrogen kit in strict accordance with Multiskan FC microplate photometer instructions (Thermo Scientific).Results. Children periodontal status evaluation did not reveal any pronounced clinical manifestations of the inflammation that could cause concern and complaints of bleeding gums. Thus, the Silness-Loe plaque index at the gingival margin was 1.70 ± 0.07 (control group 1.10 ± 0.03), the Mühlemann gingival sulcus bleeding index in children with undifferentiated connective tissue disease was 2.10 ± 0.05 (control group 0). The results of the oral fluid cytokine count in patients with undifferentiated connective tissue disease demonstrated a tendency for pro-inflammatory cytokine increase and anti-inflammatory cytokine decrease, in contrast to the control group.Conclusions. Thus, the qualitative composition of pro-inflammatory cytokines – interleukin (IL-1β), interferongamma (IFN-γ) and transforming growth factor (TGF-β1), interleukin IL-1 receptor antagonist (IL-1ra) in the oral fluid, in combination with clinical diagnostic methods in periodontal practice, can reliably predict the predisposition of people with undifferentiated connective tissue disease to periodontal inflammation and destruction. Medical checkup in children with undifferentiated connective tissue disease mainly aims to carry out comprehensive treatment and preventive measures to preserve the functions of the dental system. As children periodontal service is not allocated in the register of medical specialties in the Russian Federation, pediatric periodontal patients are followed-up in the periodontally healthy groups. However, it is evident today that periodontal passports are necessary, which indicate a genetic predisposition to inflammatory periodontal diseases.

Expanding the phenotypic spectrum of Mendelian connective tissue disorders to include prominent kidney phenotypes.

Heritable connective tissue disorders are a group of diseases, each rare, characterized by various combinations of skin, joint, musculoskeletal, organ, and vascular involvement. Although kidney abnormalities have been reported in some connective tissue disorders, they are rarely a presenting feature. Here we present three patients with prominent kidney phenotypes who were found by whole exome sequencing to have variants in established connective tissue genes associated with Loeys-Dietz syndrome and congenital contractural arachnodactyly. These cases highlight the importance of considering connective tissue disease in children presenting with structural kidney disease and also serves to expand the phenotype of Loeys-Dietz syndrome and possibly congenital contractural arachnodactyly to include cystic kidney disease and cystic kidney dysplasia, respectively.

Alterations of Extracellular Matrix Components in the Course of Juvenile Idiopathic Arthritis.

Juvenile idiopathic arthritis (JIA) is the most common group of chronic connective tissue diseases in children that is accompanied by joint structure and function disorders. Inflammation underlying the pathogenic changes in JIA, caused by hypersecretion of proinflammatory cytokines, leads to the destruction of articular cartilage. The degradation which progresses with the duration of JIA is not compensated by the extent of repair processes. These disorders are attributed in particular to changes in homeostasis of extracellular matrix (ECM) components, including proteoglycans, that forms articular cartilage. Changes in metabolism of matrix components, associated with the disturbance of their degradation and biosynthesis processes, are the basis of the progressive wear of joint structures observed in the course of JIA. Clinical evaluation and radiographic imaging are current methods to identify the destruction. The aim of this paper is to review enzymatic and non-enzymatic factors involved in catabolism of matrix components and molecules stimulating their biosynthesis. Therefore, we discuss the changes in these factors in body fluids of children with JIA and their potential diagnostic use in the assessment of disease activity. Understanding the changes in ECM components in the course of the child-hood arthritis may provide the introduction of both new diagnostic tools and new therapeutic strategies in children with JIA.

Allergic rhinitis and dental-supporting tissue diseases in children.

The etiology of dental-supporting tissue diseases in children is multi factorial and not merely related to oral hygiene. Therefore, in the present study, we investigated the relationship between children <18 years old with allergic rhinitis (AR) and the risk of dental-supporting tissue diseases.Data from the National Health Insurance Research Database (NHIRD) of Taiwan were used to conduct a retrospective longitudinal cohort study. The study cohort comprised 378,160 patients with AR (AR group) and 378,160 patients without AR (non-AR group), who were selected through frequency matching based on age, sex, and the index year. The study patients were followed until dental-supporting tissue diseases occurrence, withdrawal from the National Health Insurance program, or December 31, 2013. Cox proportional hazards regression analysis was conducted to calculate the risk of dental-supporting tissue diseases in the AR group after adjustment for age, sex, and relative comorbidities.The adjusted HRs of periodontal, pulp, and periapical diseases in AR children were higher than those in the non-AR controls (1.51, 95% CI: 1.50 to 1.53; 1.06, 95% CI: 1.05 to 1.07, respectively). The AR to non-AR HRs of these inflammatory dental diseases were particularly higher in children <6 years old and in boys. The HRs of periodontal, pulp, and periapical diseases were greatest in those with >5 AR-related medical visits/year (5.57, 95% CI: 5.50 to 5.56; 4.06, 95% CI: 4.00 to 4.12, respectively).Children with AR had a greater risk of inflammatory dental-supporting tissue diseases, particularly those <6 years old with primary teeth, boys, and those with severe persistent AR.

Клинико-прогностическое значение определения эластина и ламинина при недифференцированной дисплазии соединительной ткани у детей

Aim: to determine clinical prognostic relevance of elastin and laminin in children with undifferentiated connective tissue disease (UCTD). Patients and Methods: 47 children with UCTD aged 3–6 were enrolled. The diagnosis was verified by ruling out genetic disorders via analyzing family tree, genetic consultation, etc. Phenotypic and visceral signs were interpreted based on "Congenital and multifactorial connective tissue diseases in children" guidelines (2016). The results of cardiovascular and urinary tract ultrasound were analyzed. Serum levels of elastin and laminin were measured by the sandwich ELISA using high-sensitive kits. All tests were performed at admission and after two years. Results: prognostic relevance (prognostic coefficient) of individual phenotypic and visceral signs and lab tests and prognostic threshold value (the sum of prognostic coefficients indicating high, moderate, or low risk of UCTD progression) were determined. It was demonstrated that measuring elastin and laminin levels improves the efficacy of UCTD progression risk assessment. This phenomenon was supported by the values of their prognostic coefficient, which were comparable to the values of significant phenotypic and visceral signs. Furthermore, the accuracy of prognosis based on the use of a set of prognostic indicators (phenotypic, visceral, and biochemical) was supported by the escalation of dysplasia signs. After two years, disease progression was reported in 36 out of 38 children with a moderate and high risk of UCTD progression as identified by the initial evaluation. Conclusion: given the relevance of elastin and laminin in dysplasia pathogenesis, these parameters are considered informative to assess the risk of dysplasia progression in children aged 3–6. It is reasonable to use elastin and laminin levels as additional criteria to evaluate the risk of UCTD progression. KEYWORDS: undifferentiated connective tissue disease, prediction, children, phenotypic signs, laminin, elastin, risk of progression, cardiovascular system, urinary system. FOR CITATION: Cherkasov N.S., Doronina T.N., Prakhov A.V., Lutsenko Yu.A. Clinical prognostic relevance of elastin and laminin in children with undifferentiated connective tissue disease. Russian Journal of Woman and Child Health. 2021;4(4):351–354 (in Russ.). DOI: 10.32364/2618-8430-2021-4-4-351-354.

Prevalence and structure of periodontic tissue diseases in children with insulin dependent diabetes mellitus

Objective: to determine the spread and structure of periodontal diseases in children with insulin-dependent diabetes mellitus (DM).Material and methods. To evaluate the state of the periodontal tissue in 170 children aged from 12 to 16 were examined including 130 of them suffering from DM, and 40 – without comorbid somatic pathology. The children were divided into the groups according to the level of glycemic control: those with optimal glycemic control (OGC) – 4 persons, with suboptimal glycemic control (SOGC) - 74 individuals, with glycemic control and high risk for life (HRFL) – 52 individuals, as well as duration of the disease: children suffering from DM less than 5 years – 74 individuals, children suffering from DM more than 5 years – 56 individuals.Results. Results of the stomatological examination are indicative of the fact that the number of children suffering from DM with intact periodontal tissue is considerably less (8,46±2,44)% in comparison with somatically healthy children (55,0±7,87)%. The signs of inflammatory process in the periodontal tissue in the form of CCG on an average were found in (83,85±3,23)% of DM children, which is practically twice as often concerning those without comorbid pathology – (45,0±7,87)%. CCG complications in DM children were found to be diagnosed in (2,32±1,32)% of cases, hypertrophic gingivitis – in (2,32±1,32)%, and periodontitis – in (3,08±1,52)% contrary to somatically healthy children who do not develop these forms of periodontal diseases.Conclusions. The obtained results indicate the need for further in-depth study of the pathogenetic mechanisms of periodontal diseases in children with diabetes to develop preventive and treatment measures.

Поширеність захворювань тканин пародонта в дітей з розладами аутистичного спектру

Поширеність захворювань тканин пародонта в дітей з розладами аутистичного спектру

Incidence of skin and subcutaneous tissue diseases in children at age of 15—17 years in the Udmurt Republic

Incidence of skin and subcutaneous tissue diseases in children at age of 15—17 years in the Udmurt Republic

Imaging findings of mixed connective tissue disease in children and adolescents: a case series.

Mixed connective tissue disease (MCTD) is a rare disease in children and adolescents which overlaps features of juvenile idiopathic arthritis, polymyositis/dermatomyositis, systemic lupus erythematosus, and systemic sclerosis. We have provided an image-based approach for evaluation of MCTD in children and adolescents, outlying the most frequent imaging findings. This approach would aid imagers and clinicians to consider the diagnosis of this rare entity and be able to make an accurate list of differential diagnosis for complex rheumatologic diseases such as MCTD, thus facilitating the ultimate goal of early diagnosis and optimal management of affected children.

Особливості стоматолоґічного статусу в дітей з надмірною масою тіла

Aim. To a number of factors that have a significant influence on the processes of formation of teeth and periodontal tissues is excessive body weight. In the XXI century, overweight and obesity became one of the most serious public health problems in the European region. At the same time, high rates of caries and periodontal disease, which is especially unfavorable, remain in childhood. Therefore, the purpose of the work is to assess the dental status of children with excessive body weight. Material and Methods. 648 children were examined in Lviv, age 7, 12, 15 years. The prevalence of caries (%) and intensity (DMF) was determined. The Schiller-Pisarev test was used, the PMA index (Parma, 1960), the index of gum bleeding (M.Muhleman, 1971), and the CPI index were used. The quality of hygiene of the oral cavity was estimated using the OHI-S Hygiene Index (Green-Vermillion, 1964). The assessment of the physical development of the examined children was carried out according to anthropometric measurements. The body mass index was estimated using percentile tables developed by the WHO for children aged 5 to 19. The results of the study were worked out using the methods of variation statistics. Results and Discussion. The analysis of the data showed that, on average, the caries intensity in children with excess body weight is 5.53±0.52, which significantly exceeds the mean values in children with normal body mass (DMF=3.34±0.53 tooth, p<0.05) and corresponds to a high intensity level according to WHO recommendations. It was also found that the prevalence of periodontal tissue diseases in children with overweight is, on average, 67.9±2.8%, which is much more frequent than in children with normal weight body (46,92±2,92%, p<0,01). The analysis of the structure of the diseases of periodontal tissues showed that chronic catarrhal gingivitis was most often diagnosed in children with excessive body weight in 64,03±2,88% of cases, which is 1,4 times more than in children with normal body weight (45.21±2.91%), (p<0.05). Thus, in children with overweight, the positive Schiller-Pisarev test was found to be 1.44 times more frequent than in children with normal body weight (52.16±2.94%, p<0.001). It was found that in children with overweight the index of PMA was 33.71±2.84%, whereas in children with normal body mass (24.95±2.52). The value of index of gum bleeding in children with overweight was 1.48 times higher than in children with normal body mass. Most of children with excess body weight (36.06%) had satisfactory oral hygiene. Conclusions. The results of assessing the dental status in overweight children indicate higher rates of caries and periodontal disease than children with normal body weight. Particularly significant increasing of caries and periodontal disease is observed in the age from 12 to 15 years.

Поширеність та структура захворювань тканин пародонта у дітей із надмірною масою тіла

Epidemiological studies in recent years have shown a high prevalence of periodontal tissue diseases in children and adolescents in the world and in Ukraine in particular. One of the main general factors in the development of periodontal tissue diseases is the presence of concomitant pathology, one of which is an excessive body weight.The aim of the study – to learn the prevalence, structure and peculiarities of the clinical course of periodontal tissue diseases in overweight children.Materials and Methods. 278 children with overweight (the main group) were examined. The comparison group was made of 292 children with normal body weight. The study was conducted in age groups of 7, 12, 15 years old. The state of the periodontal tissues of the examined persons was objectified according to the classification of Danylevskyi (1994) and by using of the periodontal indices of PMA, CPI and hygienic index OHI-S. On the basis of the anthropometric examinations, the body mass index (BMI) was calculated.Results and Discussion. The results of the dental examination showed that the number of overweight children with intact periodontium was significantly lower (32.01 ± 2.8 %) compared with children with normal body mass (53.08 ± 2.92 %) (p &lt;0.01). On the other hand, the inflammatory process in the tissues of periodontium in overweight children, on average, was 67.99 ± 2.80 %, which was much higher in relation to children with normal body mass – 46.92 ± 2.92 % (p &lt;0.01).Conclusions. The results of the examination of periodontal tissues in overweight children indicate a high incidence and severity of inflammatory processes in periodontal tissues compared with normal weight children.

Стан стоматологічного здоров’я дітей, які хворіють на дисметаболічні нефропатії

The urgency of this work is conditioned with the necessity to study dental health of children with dismetabolic nephropathy influenced by toxic environmental factors. The purpose of the study was to explore the prevalence and intensity of dental caries and periodontal tissue diseases in children suffering from dismetabolic nephropathy and living in different environmental conditions. To clarify the goals and objectives of the study there were examined 356 children aged 6–15 years with dismetabolic nephropathy living in different environmental conditions. Analysis of the survey results showed that in children with dismetabolic nephropathy who live in ecologically unfavorable region there is a high prevalence (91.56±2.15%) and intensity (7.36±0.29 carious teeth) of the caries process, compared to the examined children with dismetabolic nephropathy from the clean region (81.48±3.34% and 6.37±0.25 carious tooth? respectively) and compared to physically healthy children (73.60±3.96% and 4.87±0.22 carious tooth, respectively), and was recorded also a higher incidence of complicated forms of caries (18.69±1.25 17.16±1.21 against and 13.84±1.22%, respectively). The prevalence of the periodontal tissues diseases in children with dismetabolic nephropathy from the ecologically adverse region was 1.6 times higher than in children with dismetabolic diseases of kidneys who live in ecologically clean regions.

Mixed connective tissue disease in children – case series

Mixed connective tissue disease in children – case series

Clinical and electrophysiological study of peripheral and central neuromuscular changes in connective tissue diseases in children

IntroductionChildren with juvenile connective tissue diseases (JCTDs) may have a wide variety of clinical features ranging from fever or a simple arthritis to complex multisystem autoimmune diseases. Aim of the workTo study clinical and electrophysiological peripheral and central neuromuscular changes in children with connective tissue diseases. Patients and methodsThirty children with different JCTDs were enrolled. Clinical and neurological examination and laboratory investigations were done. Electrophysiological evaluation was performed and included: peripheral nerve conduction studies, late responses, somatosensory evoked potential and electromyography. ResultsTwenty patients had juvenile idiopathic arthritis (JIA) (66.7%), 8 patients had juvenile systemic lupus erythematosus (JSLE) (26.7%), one patient had juvenile systemic sclerosis (JSScl), and one patient had juvenile overlap syndrome (JSScl and polymyositis). Clinical neurologic abnormalities were present in 3 patients (ulnar neuropathy, median neuropathy and polymyositis). Electrophysiological abnormalities were detected in 18 patients (clinical in 3 and subclinical in 15 patients) and included ulnar entrapment neuropathy, median axonal neuropathy, demyelinating sensory motor polyneuropathy, deep peroneal nerve entrapment at the ankle (anterior tarsal tunnel syndrome), prolonged posterior tibial somatosensory evoked potential latency and prolonged H reflex latency not explained by peripheral neuropathy, increased H/M ratio and myopathic motor units. The most common electrophysiological abnormalities were present in patients with JSLE. ConclusionClinical neurological abnormalities are not common in JCTDs whereas subclinical neurological abnormalities are common findings. Juvenile systemic lupus erythematosus had the most common abnormalities among JCTDs. Polyneuropathy in JIA is commonly of demyelinating type. Entrapment neuropathy is less frequent than in adults.

PReS-FINAL-2126: How to follow up children with Raynaud syndrome - recommendations based on the Hamburg consensus meeting 2012

Raynauds phenomenon (RP) can be the first symptom of a connective tissue disease in children, in particular juvenile systemic scleroderma (JSSC) or systemic lupus erythematosis (SLE). However, the prevalence of RP in healthy school children has been shown to be as high as 15%[1]. There are currently no guidelines or agreed management strategies amongst Paediatric Rheumatologists on how to differentiate primary from secondary RP or how often patients require evaluation.