Adult respiratory distress syndrome (ARDS) due to omeprazole-induced drug reaction with eosinophilia and systemic symptoms (DRESS): Case report and review of the literature.

Eosinophilia in not an uncommon findings in the intensive care unit (ICU); however, DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) syndrome, which is characterized by a hypersensitivity reaction to drugs and manifests as eosinophilia, systemic involvement and maculopapular erythematous rash 2-6 weeks after exposure to the offending drug, is an exceptional occurrence. We present the first case described in the literature of DRESS syndrome with pulmonary involvement in the form of interstitial pneumonitis and persistent adult respiratory distress syndrome (ARDS) secondary to proton pump inhibitors (PPI). The patient made a good recovery after withdrawal of the offending drug and long-term treatment with systemic corticosteroids. We also present a systematic review of all cases of DRESS with pulmonary involvement in the form of interstitial pneumonitis and cases of PPI-induced DRESS published to date; none of these describe pulmonary involvement.

Seguridad y eficacia de blinatumomab en el tratamiento de la leucemia linfoblástica aguda B en recaída/refractaria: a propósito de un caso / Safety and efficacy of blinatumomab in the treatment of relapsed/refractory acute lymphoblastic leukemia B: a case report

Blinatumomab es un anticuerpo biespecífico que está indicado en monoterapia en pacientes adultos con leucemia linfoblástica aguda B refractaria o en recaída con cromosoma filadelfia negativo como terapia puente al trasplante y se asocia a remisiones a largo plazo. Se presenta el caso de una paciente de 40 años diagnosticada de esta enfermedad, que respondió al tratamiento con blinatumomab y posteriormente fue trasplantada en condiciones óptimas, a pesar de no haber recibido los dos ciclos de tratamiento a dosis plenas por motivos de toxicidad pancreática y gastrointestinal. (AU)

Blinatumomab is a bispecific antibody that is indicated as monotherapy in adult patients with refractory or relapsed Philadelphia chromosome-negative acute lymphoblastic leukaemia B as bridge therapy to transplantation and is associated with long-term remissions. We present the case of a 40-year-old patient diagnosed with this disease, who responded to treatment with blinatumomab and was subsequently transplanted under optimal conditions, despite not having received the two cycles of full-dose treatment due to pancreatic and gastrointestinal toxicity. (AU)

CYB5R3 homozygous pathogenic variant as a rare cause of cyanosis in the newborn.

Detailed below is a very illustrative case of a rare pathology of recessive congenital methemoglobinemia. The patient, a newborn female, was homozygous for c.535G > A, p.(Ala179Thr) a pathogenic variant in the CYB5R3 gene. The reported population frequency of the allele is 0.853%, demonstrating why it is remarkable to find both parents are heterozygous carriers without consanguinity. A brief review of previously published cases is also presented.

GEIS-SEHOP clinical practice guidelines for the treatment of rhabdomyosarcoma

Rhabdomyosarcoma (RMS) is the most frequent soft tissue sarcoma (STS) in children and adolescents. In Spain the annual incidence is 4.4 cases per million children < 14 years. It is an uncommon neoplasm in adults, but 40% of RMS are diagnosed in patients over 20 years of age, representing 1% of all STS in this age group. RMS can appear anywhere in the body, with some sites more frequently affected including head and neck, genitourinary system and limbs. Assessment of a patient with suspicion of RMS includes imaging studies (MRI, CT, PET-CT) and biopsy. All patients with RMS should receive chemotherapy, either at diagnosis in advanced or metastatic stages, or after initial resection in early local stages. Local control includes surgery and/or radiotherapy depending on site, stage, histology and response to chemotherapy. This guide provides recommendations for diagnosis, staging and treatment of this neoplasm (AU)

GEIS-SEHOP clinical practice guidelines for the treatment of rhabdomyosarcoma.

Rhabdomyosarcoma (RMS) is the most frequent soft tissue sarcoma (STS) in children and adolescents. In Spain the annual incidence is 4.4 cases per million children < 14 years. It is an uncommon neoplasm in adults, but 40% of RMS are diagnosed in patients over 20 years of age, representing 1% of all STS in this age group. RMS can appear anywhere in the body, with some sites more frequently affected including head and neck, genitourinary system and limbs. Assessment of a patient with suspicion of RMS includes imaging studies (MRI, CT, PET-CT) and biopsy. All patients with RMS should receive chemotherapy, either at diagnosis in advanced or metastatic stages, or after initial resection in early local stages. Local control includes surgery and/or radiotherapy depending on site, stage, histology and response to chemotherapy. This guide provides recommendations for diagnosis, staging and treatment of this neoplasm.

The paediatric cancer clinical research landscape in Spain: a 13-year multicentre experience of the new agents group of the Spanish Society of Paediatric Haematology and Oncology (SEHOP).

PURPOSE: Early phase trials are crucial in developing innovative effective agents for childhood malignancies. We report the activity in early phase paediatric oncology trials in Spain from its beginning to the present time and incorporate longitudinal data to evaluate the trends in trial characteristics and recruitment rates. METHODS: Members of SEHOP were contacted to obtain information about the open trials at their institutions. The study period was split into two equal periods for analysis: 2007-2013 and 2014-2020. RESULTS: Eighty-one trials and two molecular platforms have been initiated. The number of trials has increased over the time of the study for all tumour types, with a predominance of trials available for solid tumours (66%). The number of trials addressed to tumours harbouring specific molecular alterations has doubled during the second period. The proportion of industry-sponsored compared to academic trials has increased over the same years. A total of 565 children and adolescents were included, with an increasing trend over the study period. For international trials, the median time between the first country study approval and the Spanish competent authority approval was 2 months (IQR 0-6.5). Fourteen out of 81 trials were sponsored by Spanish academic institutions. CONCLUSIONS: The number of available trials, and the number of participating patients, has increased in Spain from 2007. Studies focused on molecular-specific targets are now being implemented. Barriers to accessing new drugs for all ranges of age and cancer diseases remain. Additionally, opportunities to improve academic research are still required in Spain.

An RCT of atraumatic restorative treatment for older adults: 5 year results.

OBJECTIVES: to compare the survival of ART and a conventional restorative technique (CT) for restoring carious lesions in older adults after 5 years. METHODS: In this parallel randomised controlled clinical trial, 219 independently-living adults were recruited from a dental hospital/community and a geriatric day hospital. Ninety-nine patients who met the inclusion criteria and presented with carious lesions were randomly allocated to receive either ART or conventional restorations (anaesthesia, rotary instruments and resin-modified glass ionomer). The status of restorations was assessed 6 months, 1, 2 and 5 years after restoration placement. Estimates of cumulative survival were calculated for each interval between assessments and a Cox Proportional Hazards (PH) model was fitted to the interval-censored survival time. RESULTS: Three hundred restorations (ART n=142; CT n=158) were placed on 99 patients, 46 males and 53 females, with a mean age of 73.2, SD: 6.8 (65-90 yrs). After 5 years, ART and CT presented cumulative probability of survival of 85% and 79% (p=0.8095), respectively. CONCLUSIONS: ART presents survival rates comparable to a conventional technique, when treating older adults after 5 years. The ART approach could be a useful tool to provide dental care for older adults particularly in the nonclinical setting. (Trial Registration number: ISRCTN 76299321). CLINICAL RELEVANCE: This study shows that ART presents survival rates comparable to conventional techniques to treat carious lesions in older patients after 5 years. It is well accepted by this age cohort, and therefore could be an alternative to treat the elderly, especially those who are homebound or cannot attend the dentist.

Insulin resistance in hidradenitis suppurativa: a case-control study.

BACKGROUND: The association between chronic inflammatory diseases, such as rheumatoid arthritis and psoriasis, and insulin resistance (IR) has been well established. Hidradenitis suppurativa (HS) is a chronic inflammatory cutaneous disease that affects the apocrine gland-bearing areas of the body. OBJECTIVE: We aimed to determine the prevalence of IR in patients with HS. METHODS: This cross-sectional, case-control study enrolled 137 subjects, 76 patients with HS and 61 age- and gender-matched controls. Demographic data, clinical examination of HS patients, anthropometric measures, cardiovascular risk factors and laboratory studies were recorded. The homeostasis model assessment of IR (HOMA-IR) was calculated in all participants by measuring fasting plasma glucose and insulin levels. RESULTS: The median (IQR) HOMA-IR value in HS patients was significantly higher [2.0 (1.0-3.6)] than in controls [1.5 (0.9-2.3)] (P = 0.01). The prevalence of IR was significantly higher in cases (43.4%) compared with controls (16.4%) (P = 0.001). In the linear regression multivariable analysis after adjusting for age, sex and body mass index (BMI), HS remained as a significant factor for a higher HOMA-IR [2.51 (0.18) vs 1.92(0.21); P = 0.04]. The HOMA-IR value and the prevalence of IR did not differ significantly among HS patients grouped by severity of the disease. CONCLUSION: Our results show an increased frequency of IR in HS. Thus, we suggest HS patients to be evaluated for IR and managed accordingly.

Thyroid autoimmunity in patients with hidradenitis suppurativa: a case-control study.

The aetiopathogenesis of hidradenitis suppurativa (HS) is not fully understood; however, increasing evidence suggests that it may be an immune-mediated disorder. Autoimmune thyroid disease (AITD) has classically been considered as the 'paradigm' of autoimmunity, and it has been linked to a variety of skin disorders. To our knowledge, the prevalence of AITD has not been investigated in patients with HS. The aim of the present study was to assess and compare, for the first time, the prevalence of thyroid autoimmunity in 70 patients with HS and in 70 age- and sex-matched controls. In all participants, thyroid autoantibodies and thyroid function tests were analysed. No statistically significant difference was detected between patients with HS and controls, either for the prevalence of thyroid antibodies or for thyroid function parameters. This lack of an association between HS and thyroid autoimmunity suggests that conventional autoimmune mechanisms may not be implicated in the pathogenesis of HS.

Evaluation of the Cariogram for root caries prediction.

OBJECTIVES: The aim of this study was to evaluate complete and reduced Cariogram models in predicting root caries risk in independently living older adults by comparing the caries risk assessment of the programme to observed root caries increment over a two-year period. METHODS: A prospective study recording root caries incidence was conducted on 334 dentate older adults. Data were collected on participant's medical history, fluoride exposure, and diet. Saliva samples were collected to measure salivary flow rate, buffer capacity and bacterial counts. Clinical examination was completed to record decayed, missing and filled teeth (DMFT) and also exposed, filled and decayed root surfaces (RDFS). This was repeated after 12 and 24 months. Scores were entered into the Cariogram and baseline risk category was recorded. Reduced Cariogram models were generated by omitting individual salivary variables and all salivary variables. The performance of the complete and reduced Cariogram models in predicting root caries incidence were evaluated by receiver operating characteristic (ROC) analysis. RESULTS: 280 participants were examined at two year follow up. 55.6% of those in the highest risk group developed new caries compared to 3.8% in the lowest risk group. The mean root caries increment in the highest risk group was 2.00 (SD 3.20) compared to 0.04 (SD 0.20) in the lowest risk group. The area under the ROC curve for the complete Cariogram model was 0.77 (95% CI 0.70-0.83) indicating a fair performance in predicting root caries. Omitting individual or all salivary variables did not significantly alter the predictive ability of the Cariogram. CONCLUSION: Within the limitations of this study, the Cariogram was clinically useful in identifying individuals with a high risk of developing root caries. CLINICAL SIGNIFICANCE: Identification of a caries risk assessment tool which could reliably select high-risk individuals for root caries prevention strategies would maximise the cost effectiveness of professionally delivered prevention measures.

Five Years of Multidisciplinary Care in Hereditary Cancer: Our Experience in a Spanish University Hospital.

OBJECTIVE: To analyse the evolution of a multidisciplinary heredofamilial cancer unit (HFCU) in a university hospital. METHODS: This was a retrospective analysis of the activity of our HFCU in its first 5 years of existence. RESULTS: Between July 2010 and July 2015, 1,518 patients from 1,318 families attended our HFCU. Genetic testing was offered to 862 patients. Of those, 833 (96.6%) accepted testing, with available results for 636 (76.4%). Pathogenic mutations in BRCA1 and BRCA2 were found in 175 patients. Lynch syndrome and adenomatous polyposis were the most frequent syndromes diagnosed (151/175, 86.3%) among 17 different syndromes studied. Of the 404 patients without a previous genetic diagnosis in the family, 62 (15.3%) were found to have mutations in disease-causing genes. Prophylactic surgery and follow-up (33.7%) or follow-up only (66.3%) was proposed for mutation carriers according to international guidelines and patients' preferences. CONCLUSION: We have a high mutation detection rate, genetic test acceptance, and compliance with risk reduction strategies. However, there is room for improvement, especially in genetic testing timing, considering that an increase in the indications for genetic testing is expected.

Evaluation of Biodentine in the Restoration of Root Caries: A Randomized Controlled Trial.

There is no "gold-standard" material for the operative management of root caries. The aim of this study was to determine if the clinical performance of Biodentine would be acceptable for the restoration of root caries in older adults. A randomized controlled clinical trial was conducted comparing a calcium silicate cement (Biodentine), a high-viscosity glass ionomer cement (Fuji IX GP Extra), and a resin-modified glass ionomer cement (Fuji II LC). Of the 334 volunteers assessed for eligibility, 249 were excluded. A total of 303 lesions in 85 participants were randomized, with 151 lesions allocated to receive Biodentine, 77 to Fuji IX GP Extra, and 77 to Fuji II LC. Patients were reviewed by a calibrated dentist who was not involved in restoration placement and who was blinded to material allocation. Restorations were assessed according to a modified US Public Health Service criteria. The cumulative survival percentages after 6 mo and 1 y were 58.6% and 47.2% in the Biodentine group, 89.6% and 83.8% in the Fuji IX GP Extra group, and 89.5% and 84.9% in the Fuji II LC group, respectively. There were statistically significant differences ( χ2 test, P < 0.001) in restoration failure rates between restoration groups. There was no difference between Fuji IX GP Extra and Fuji II LC, but differences ( P < 0.001) were shown between the Fuji II GP Extra group and the Biodentine group and also between the Fuji II LC group and the Biodentine group at both time points. Based on the results of this study, Biodentine cannot be recommended for the operative management of root caries. Fuji IX GP Extra and Fuji II LC displayed similar success rates, and high-viscosity glass ionomer cement and resin-modified glass ionomer cement continue to be the best available option for the restoration of root caries ( ClinicalTrials.gov NCT01866059). Knowledge Transfer Statement: The results of this study can assist dental practitioners when selecting a restorative material for the operative management of root caries. This randomized controlled trial compared the 1-y clinical performance of a calcium silicate-based material to that of a high-viscosity glass ionomer cement and a resin-modified glass ionomer cement in the operative management of root caries. The study concluded that high-viscosity glass ionomer cement and resin-modified glass ionomer cement continue to be the best available option to dental practitioners when restoring the root surface.

Cutaneous vasculitis associated with severe bacterial infections. A study of 27 patients from a series of 766 cutaneous vasculitis.

OBJECTIVES: To assess the clinical spectrum of severe bacterial infections presenting as cutaneous vasculitis (CV) in a defined population. METHODS: Unselected series of 766 patients with CV diagnosed at a single university referral center. RESULTS: An underlying severe bacterial infection was diagnosed in 27 (22 men/5 women; mean age ± standard deviation [SD]: 53 ± 18 years) of 766 cases presenting with CV (3.5%). These infections were: pneumonia (n=8), endocarditis (n=6), meningitis (n=4), intra-abdominal infections (n=3), septic arthritis (n=2), septicaemia (n=2), septic bursitis (n=1), and urinary tract infection (n=1). All the patients were admitted for suspected CV. The median delay from admission to the diagnosis of infection was 4 days. A typical palpable purpura without relevant visceral vasculitic involvement was the main clinical manifestation. Patients with severe bacterial infections were older, with male predominance, had more frequently fever, constitutional symptoms, focal infectious features, and leukocytosis with left shift and anaemia than the remaining patients with CV. Although antibiotics were prescribed in all the patients, seven also required the use of low-dose corticosteroids to achieve complete resolution of the cutaneous lesions. Most patients experienced full recovery but two of them underwent prosthetic cardiac valve replacement, and another two died due to infection-related complications. CONCLUSIONS: CV may be the presenting manifestation of a severe underlying bacterial infection. Physicians should keep in mind this fact to make an early diagnosis of infection and, consequently, prevent life-threatening complications.

A study of primary teeth restored by intracoronal restorations in children participating in an undergraduate teaching programme at Cork University Dental School and Hospital, Ireland.

AIM: To study the outcomes for restored primary molar teeth; to examine outcomes in relation to tooth type involved, intracoronal restoration complexity and to the material used. DESIGN: Retrospective study of primary molar teeth restored by intracoronal restorations. A series of restored primary molar teeth for children aged 6-12 years was studied. The principal outcome measure was failure of initial restoration (re-restoration or extraction). Three hundred patient records were studied to include three equal groups of primary molar teeth restored with amalgam, composite or glass ionomer, respectively. Restorative materials, the restoration type, simple (single surface) or complex (multi-surface) restoration, and tooth notation were recorded. Subsequent interventions were examined. Data were coded and entered into a Microsoft Excel database and analysis undertaken using SPSS v.18. Statistical differences were tested using the Chi square test of statistical significance. RESULTS: Of the 300 teeth studied, 61 restoration failures were recorded with 11 of those extracted. No significant differences were found between outcomes for upper first, upper second, lower first or lower second primary molars. Outcomes for simple primary teeth restored by intracoronal restorations were significantly better than those for complex intracoronal restorations (P = 0.042). Teeth originally restored with amalgam accounted for 19.7% of the 61 failures, composite for 29.5%, while teeth restored with glass ionomer represented 50.8% of all restoration failures. The differences were significant (P = 0.012). CONCLUSIONS: The majority (79.7%) of the 300 restored primary teeth studied were successful, and 3.7% teeth were extracted. Restorations involving more than one surface had almost twice the failure rate of single surface restorations. The difference was significant. Significant differences in failure rates for the three dental materials studied were recorded. Amalgam had the lowest failure rate while the failure rate with glass ionomer was the highest.

Non-infectious aortitis: a report of 32 cases from a single tertiary centre in a 4-year period and literature review.

OBJECTIVES: Non-infectious aortitis often presents with non-specific symptoms leading to inappropriate diagnostic delay. We intend to describe the clinical spectrum and outcome of patients with aortitis diagnosed at a single centre. METHODS: We reviewed the clinical charts of patients diagnosed with non-infectious aortitis between January 2010 and December 2013 at the Rheumatology Division from a 1.000-bed tertiary teaching hospital from Northern Spain. The diagnosis of aortitis was usually based on FDG-PET-CT scan, and also occasionally on CT or MRI angiography or helical CT-scan. RESULTS: During the period of assessment 32 patients (22 women and 10 men; mean age 68 years [range, 45-87]) were diagnosed with aortitis. The median interval from the onset of symptoms to the diagnosis was 21 months. FDG-PET CT scan was the most common tool used for the diagnosis of aortitis. The underlying conditions were the following: giant cell arteritis (n=13 cases); isolated polymyalgia rheumatica (PMR) (n=11); Sjögren's syndrome (n=2), Takayasu arteritis (n= 1); sarcoidosis (n=1), ulcerative colitis (n=1), psoriatic arthritis (n=1), and large-vessel vasculitis that also involved the aorta (n=2). The most common clinical manifestations at diagnosis were: PMR features, often with atypical clinical presentation (n=23 patients, 72%); diffuse lower limb pain (n=16 patients, 50%); constitutional symptoms (n=12 patients, 37%), inflammatory low back pain (n=9 patients, 28%) and fever (n=7 patients, 22%). Acute phase reactants were increased in most cases (median erythrocyte sedimentation rate 46 mm/1st hour, and a median serum C-reactive protein 1.5 mg/dL). CONCLUSIONS: Aortitis is not an uncommon condition. The diagnosis is often delayed. Atypical PMR features, unexplained low back or limb pain, constitutional symptoms along with increased acute phase reactants should be considered 'red flags' to suspect the presence of aortitis.

Una nueva mutación en el gen GLDC en la hiperglicinemia no cetósica / A new mutation in the GLDC gene in non-ketotic hyperglycinaemia

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Henoch-Schönlein purpura nephritis and IgA nephropathy: a comparative clinical study.

OBJECTIVES: Henoch-Schönlein purpura nephritis (HSPN) and IgA nephropathy (IgAN) are related syndromes. In the present study we aimed to compare the clinical characteristics and outcome of a large and unselected series of patients diagnosed as having HSPN and IgAN. METHODS: Comparative study of a wide and unselected population of HSPN (142 patient) and IgAN (61 patients) from a teaching hospital of Northern Spain. RESULTS: All of the following comparisons were expressed between HSPN vs. IgAN, respectively. HSPN patients were younger (30.6±26.4 vs. 37.1±16.5 years, p<0.001). Precipitating events, usually an upper respiratory tract infection and/or drug intake, were more frequently observed in HSPN (38% vs. 23%, p=0.03). Extra-renal manifestations were also more common in HSPN than in IgAN; skin lesions (100% vs. 1.8%; p<0.001), gastrointestinal (62% vs. 7.4%; p<0.001), and joint involvement (61.3% vs. 3.6%; p<0.001). However, nephritis was less severe in HSPN, renal insufficiency (25% in HSPN vs. 63.4% in IgAN; p<0.001), nephrotic syndrome (12.5%, vs. 43.7%; p<0.001), and nephritic syndrome (6.8% vs. 10.7%; NS). Leukocytosis was more frequent in HSPN (22.5% vs. 8.2%; p=0.015) and anaemia in IgAN (12.7% in HSPN vs. 36% in IgAN, p<0.001). The frequency of corticosteroid (79.6% vs. 69%; NS) and cytotoxic drug (19% vs. 16.5%, NS) use was similar. The frequency of relapses was similar (38.6% in HSPN vs. 36.3% in IgAN). After a median follow-up of 120.8 (IQR; 110-132) months in HSPN and 138.6 (IQR; 117-156) in IgAN, requirement for dialysis (2.9% vs. 43.5%; p<0.001), renal transplant (0% vs. 36%, p<0.001) and residual chronic renal insufficiency (4.9% vs. 63.8%; p<0.001) was more frequently observed in patients with in IgAN. CONCLUSIONS: HSPN and IgAN represent different syndromes. IgAN has more severe renal involvement while HSPN is associated with more extra-renal manifestations.

Association of bla(DHA-1) and qnrB genes carried by broad-host-range plasmids among isolates of Enterobacteriaceae at a Spanish hospital.

A collection of 30 DHA-1-Enterobacteriaceae producers was examined for the presence of qnr genes. PCR-based replicon typing, plasmid profile and Southern hybridisation analyses revealed that all isolates co-harboured bla(DHA-1) and qnrB genes on the same plasmid. All but one of these plasmids belonged to the L/M group. Genetic organization analyses of a randomly selected isolate revealed the co-localization of both genes on an IS26-composite transposon. As plasmids carrying both genes seem to have a high prevalence and a worldwide distribution, care should be taken when quinolones are used to treat infections caused by DHA-1 producers.