Diagnosing Single and Multiple Drug Hypersensitivity in Children: A Tertiary Care Center Retrospective Study.

Drug hypersensitivity reactions (DHRs) are a type of adverse drug reactions with heterogeneous pathophysiological mechanisms and a broad spectrum of clinical manifestations. Since over-diagnosing is common in children, a complete allergy work-up is needed. A cross-sectional study was conducted at a tertiary care institution, covering the five-year period. Five hundred and four patients of both sexes, mean age 7.5 and with a medical history suggestive of DHR were evaluated. ENDA/EAACI guidelines were used for a diagnostic algorithm. Single drug hypersensitivity was registered in 375 patients and multiple drug hypersensitivity in 129. The main culprits in medical history were antibiotics (83%), non-steroidal anti-inflammatory drugs (NSAIDs) (8.4%) and analgoantipyretics (3.8%). Skin involvement was registered in 96.2%. DHRs were confirmed in 4.4% patients-six patients had positive skin tests and 13 had a positive drug provocation test. In the proven DHRs group, the main agents were antibiotics (72.7%), followed by NSAIDs (8.3%), and of all the skin manifestations, urticaria was most common (78.2%), followed by exanthema (10.5%) and angioedema (5.3%). Considering the above, anticipating DHRs and a proper referral of children to an allergologist is a key step in the assessment of drug hypersensitivity. A complete allergy work-up prevents unnecessary drug exclusion and allows most children to safely continue the use of first-line medications when needed.

PRISM III Score Predicts Short-Term Outcome in Children with ARDS on Conventional and High-Frequency Oscillatory Ventilation.

Therapeutic recommendations for pediatric acute respiratory distress syndrome (PARDS) include conventional (CMV) and rescue high-frequency oscillatory mode (HFOV) of mechanical ventilation (MV). The pediatric risk of mortality (PRISM) is a frequently used mortality score for critically ill patients. In search of methods to recognize those patients, we analyzed the PRISM III score as a potential predictor of the short-term outcome in MV subjects with PARDS. A retrospective five-year study of PARDS in children on MV was conducted in the Pediatric ICU. Seventy patients were divided into two groups (age group <1 year and age group 1−7 years). The PRISM III score was used to assess the 28-day outcome and possible development of complications. The most common causes of PARDS were pneumonia and sepsis. Male sex, malnourishment, sepsis, and shock were significant indicators of poor outcome. The PRISM III score values were significantly higher in those who died, as well as in subjects requiring HFOV. The score had a significant prognostic value for short-term mortality. There was no significant difference in outcome based on the comparison of two modes of ventilation. A significantly higher score was noted in subjects who developed sepsis and cardiovascular insufficiency. The PRISM III score is a fair outcome predictor during the 28-day follow-up in MV subjects with PARDS, regardless of the ventilation mode.

Chronic inflammatory demyelinating polyneuropathy associated with diabetes: a European multicentre comparative reappraisal.

INTRODUCTION: The association between chronic inflammatory demyelinating polyneuropathy (CIDP) and diabetes is uncertain despite important diagnostic and management implications. METHODS: We retrospectively analysed two European cohorts, totaling 257 patients with 'definite' or 'probable' CIDP, from Serbia and Birmingham, UK. RESULTS: Diabetes was present at CIDP diagnosis in 25/139 (18%) subjects in the Serbian cohort and in 23/118 (19.5%) in the UK cohort. In both cohorts, diabetes prevalence was higher than local general population prevalence rates (RR: 2.09; 95% CI 1.39 to 2.95 and RR: 2.22; 95% CI 1.46 to 3.17, respectively). Considering typical CIDP only, diabetes prevalence was greater than expected in both cohorts (RR: 2.58; 95% CI 1.60 to 3.82 and RR: 2.68; 95% CI 1.71 to 3.87, respectively). CIDP with diabetes occurred later in life than CIDP without diabetes (58.96 years, SD: 11.09 vs 51.71 years, SD: 16.02; p=0.003) and presented more frequently in the typical form than in patients without diabetes (79.2% vs 61.2%; p=0.02). Baseline Inflammatory Neuropathy Cause and Treatment disability scores were similar in patients with and without diabetes (p=0.90). Proportions of treatment responders were similar in both groups (70% vs 74.9%; p=0.65), as were response amplitudes (p=0.87). DISCUSSION: Our results, both for all CIDP and typical CIDP presentations, support a twofold increased relative risk of diabetes compared with the general population. CIDP with diabetes appears to present older and more frequently in the typical form, as compared with CIDP without diabetes. CIDP with diabetes appears similar to CIDP without diabetes in disability levels at diagnosis and probability, as well as amplitude of treatment response.

One-year follow-up study of neuropathic pain in chronic inflammatory demyelinating polyradiculoneuropathy.

We sought to gather information about frequency and features of neuropathic pain (NeP) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients and to investigate course of NeP during 1-year follow-up. Study included 105 patients diagnosed with CIDP. Patients with diabetes (N = 26) were excluded. NeP was diagnosed by the official guidelines and painDETECT questionnaire (PD-Q). Medical Research Council Sum Score (MRC-SS), INCAT disability and sensory scores, and Beck Depression Inventory were also measured. PD-Q showed presence of NeP in 16 (20%) of 79 CIDP patients and their mean pain was moderate (5.1 ± 3.0 of 10). Diagnostic delay in CIDP patients with NeP was prolonged compared to CIDP patients without NeP (21 ± 28 vs 9 ± 12 months, P < .05). Slowly progressive course of the disease was more frequent in patients with NeP (81% vs 52%, P < .05). Patients with NeP had worse INCAT sensory score (P < .01), INCAT disability score (P < .05), MRC-SS, as well as worse disease outcome at time of testing (P < .05). Depression was more common in patients with NeP (69% vs 17%, P < .01). During 1-year follow-up, majority of our CIDP patients had good control of NeP with gabapentinoids or amitriptyline. NeP was common in our cohort of non-diabetic CIDP patients. It was associated with worse functional disability, worse sensory deficit, and depression. Special attention should be paid to CIDP patients with NeP because they request additional symptomatic therapy that appeared efficacious in our cohort.

Three-Year Follow-Up Study in Patients with Guillain-Barré Syndrome.

A majority of patients with Guillain-Barré syndrome (GBS) have tendency of a good recovery. Our aim was to evaluate the outcome of the disease 1 and 3 years after GBS symptom onset. METHODS: During 2014, GBS was diagnosed in 82 patients in seven tertiary healthcare centers. Neurological follow-up was conducted in 57 (70%) patients after 1 year, and in 54 (66%) after 3 years. Functional disability was estimated according to the GBS disability scale (GDS), with a score of 0-3 indicating mild disability and a score of 4-6 indicating severe disability during acute phase, whereas a score >1 indicated poor recovery on follow-ups. Visual analog scale was used to assess sensory symptoms and musculoskelatal pain, and Krupp's Fatigue Severity Scale was used to asses fatigue. RESULTS: Poor functional outcome was found in 39% of GBS patients at year 1 and 30% at year 3. Paresthesias/dysesthesias were detected in 60% of patients after 1 year and 43% after 3 years. Musculoskeletal pain was present in 40% of patients at year 1 and 33% at year 3. Significant fatigue after 1 year was found in 21% of subjects and after 3 years in 7%. Parameters associated with poor functional outcome after 1 year were age >55 years (p=0.05), severe disability at admission (p1 indique une récupération difficile au moment des suivis. L'échelle visuelle analogue (EVA) a aussi été utilisée pour évaluer leurs symptômes sensoriels et leurs douleurs musculo-squelettiques. Enfin, l'échelle de gravité de la fatigue de Krupp a été utilisée pour évaluer leur degré de fatigue. Résultats: La première année, on a observé une piètre amélioration des capacités fonctionnelles chez 39% des patients atteints du SGB; pour la troisième année, cette proportion était de 30%. Au bout d'un an, on a aussi détecté la présence de paresthésie/dysesthésie chez 60% des patients; pour la troisième année, cette proportion était de 43%. Des douleurs musculo-squelettiques ont été rapportées chez 40% des patients après un an; deux ans plus tard, ce pourcentage chutait à 33%. Enfin, un état de fatigue important a été noté chez 21% des patients au bout d'un an; ce pourcentage n'était plus que de 7% au bout de trois ans. Les paramètres associés à une piètre amélioration des capacités fonctionnelles au bout d'un an étaient l'âge (>55 ans; p=0,05) ainsi qu'une incapacité sévère au moment de leur admission (p<0,05) et de leur congé (p<0,01). Au bout de trois ans, une piètre amélioration des capacités fonctionnelles était associée au sexe masculin (p<0,05) et à une incapacité sévère au moment d'obtenir un congé (p=0,06). CONCLUSIONS: Un an et trois ans après l'apparition des premiers symptômes du SGB, un nombre important de patients donnaient à voir des séquelles neurologiques, ce qui incluait une forme ou une autre d'incapacité fonctionnelle, des symptômes sensoriels, des douleurs et un état de fatigue.

Neuromuscular disease-specific questionnaire to assess quality of life in patients with chronic inflammatory demyelinating polyradiculoneuropathy.

To date, generic questionnaires have been used to investigate quality of life (QoL) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients. Although these measures are very useful, they are not usually precise enough to measure all specific characteristics of the disease. Our aim was to investigate QoL using the neuromuscular disease-specific questionnaire (individualized neuromuscular quality of life, INQoL) in a large cohort of patients with CIDP. Our study comprised 106 patients diagnosed with CIDP. INQoL questionnaire, Medical Research Council (MRC) sum score, Inflammatory Neuropathy Cause and Treatment (INCAT) disability score, Visual Analogue Pain Scale, Beck Depression Inventory, and Krupp's Fatigue Severity Scale were used in our study. Physical domains of INQoL were more affected than mental, and the overall score was 57 ± 25. Significant predictors of higher INQoL score in our patients with CIDP were severe fatigue (ß = 0.35, p < 0.01), higher INCAT disability score at time of testing (ß = 0.29, p < 0.01), and being unemployed/retired (ß = 0.22, p < 0.05). QoL was reduced in our cohort of CIDP patients, which was more pronounced in physical segments. Patients with fatigue, more severe disability, and unemployed/retired need special attention of neurologists because they could be at greater risk to have worse QoL.

Quality of life predictors in patients with chronic inflammatory demyelinating polyradiculoneuropathy.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic disease which can lead to many functional impairments, and like most other chronic disorders it might significantly affect quality of life (QoL). Information about QoL in patients with CIDP from developing countries is still lacking. We, therefore, sought to complete these data mosaic by investigating QoL in patients with CIDP from Serbia and surrounding countries. Our study comprised 106 patients diagnosed with CIDP. QoL was investigated using the Serbian version of the SF-36 questionnaire. The Medical Research Council 0-5 point scale, INCAT motor and sensory scores, Krupp's Fatigue Severity Scale, and Beck Depression Inventory were also used. Factors that significantly correlated with SF-36 total score in univariate analysis were included in the multiple linear regression analysis. Physical domains of the SF-36 were more affected than mental, and the overall score was 56.6 ± 25.4. Significant predictors of worse SF-36 score in our patients with CIDP were severe fatigue (ß = - 0.331, p < 0.01), higher INCAT motor score (ß = - 0.301, p < 0.01), depression (ß = - 0.281, p < 0.01), being unemployed/retired (ß = - 0.188, p < 0.05), and shorter duration of CIDP (ß = + 0.133, p < 0.01). QoL was reduced in CIDP patients, especially in physical domains. Patients with presence of fatigue and depression, with more severe motor disability, unemployed/retired ones, and those with shorter duration of the disease need special attention of clinicians since they could be at higher risk to have worse QoL.