Feasibility of a New Model of Care for Allogeneic Stem Cell Transplantation Recipients Facilitated by eHealth: The MY-Medula Pilot Study.

The use of allogeneic stem cell transplantation (allo-SCT) for the treatment of hematologic diseases is steadily increasing; however, allo-SCT has the downside of causing considerable treatment-related morbidity and mortality. Mobile technology applied to healthcare (mHealth) has proven to be a cost-effective strategy to improve care and offer new services to people with multimorbidity, but there are little data on its usefulness in allo-SCT recipients. Here we describe a new integrated healthcare model facilitated by an mHealth platform, EMMASalud-MY-Medula, and to report the results of a feasibility and usability pilot study. The MY-Medula platform was developed in 4 phases. First, patient and healthcare professional needs were identified, and technological development and pretesting tests were conducted (phases 1 to 3, January 2016 to March 2021). Then a nonrandomized, prospective, observational, single-center pilot study was conducted (October 2021 to January 2022) at the adult SCT unit of a tertiary university hospital. Twenty-eight volunteer allo-SCT recipients were included in the pilot study, of whom one-half were outpatients in the first-year post-SCT and one-half were affected by steroid-dependent graft-versus-host disease (SR-GVHD). All patients used the MY-Medula app during the 2-month follow-up period, with a median number of visits to the app of 143 (range, 6 to 477). A total of 2067 self-monitoring records were created, and 205 text messages were received, most of them related to symptoms description (47%) and doubts about medication (21%). In 3.4% of the cases, drug dosage was adjusted by the pharmacist because of dosing errors or interactions. At the end of the study, a 6-question Likert-type questionnaire for patients and a 22-question test for healthcare professionals showed a high degree of satisfaction (95% and 100%, respectively) with the new healthcare pathway. Reengineering the follow-up of allo-SCT recipients into an integrated, multidisciplinary model of care facilitated by mHealth tools is feasible and has been associated with high usability and a high degree of satisfaction by patients and healthcare professionals. A randomized trial aiming to determine the cost-effectiveness of MY-Medula-based follow-up post-SCT is currently enrolling participants.

Assessment of Confirmed Clinical Hypersensitivity to Rituximab in Patients Affected with B-Cell Neoplasia.

Rituximab hypersensitivity reactions are rare but are one of the main causes of rituximab elimination from antilymphoma immunochemotherapy treatments. While the clinical picture may be indistinguishable from other infusion-related reactions, hypersensitivity reactions (HSR) do not disappear and instead become more intense with subsequent administrations. Objective. To describe the use of the 12-step protocol for desensitization to intravenous rituximab in clinical practice and the complementary study of a possible IgE-mediated HSR in the context of B-cell lymphoma treatment. Methods. A 12-step rituximab desensitization protocol was performed prospectively within clinical practice in 10 patients with a history of severe infusion reactions or in patients who had a repeated reaction at subsequent doses despite taking more intense preventive measures. Skin prick tests were performed at the time of reaction and at a later time to eliminate false negatives due to possible drug interference. Results. Overall, with the desensitization protocol, 70% of patients were able to complete the scheduled immunochemotherapy. Two patients had to discontinue the therapy due to clinical persistence and the third due to lymphoma progression. Intradermal tests with 0.1% rituximab were positive in only 20% of cases, demonstrating a mechanism of hypersensitivity. Conclusions. The 12-step desensitization protocol is very effective and assumable within healthcare practice. There is a need to determine the mechanism underlying the infusion reaction in a large proportion of cases due to the risk of future drug exposure.

CD8-positive lymphocytes in graft-versus-host disease of humanized NOD.Cg-Prkdc(scid)Il2rg(tm1Wjl)/SzJ mice.

Immunocompromised mice that can support a human immune system are an increasingly important model for the investigation of haemopoietic stem/progenitor cell (HSPC) development and human infectious disease. NOD-SCID IL-2Rγ(-/-) (NSG) mice engrafted with human fetal liver and thymus prior to HSPC engraftment, commonly known as NSG-bone marrow-liver-thymus (NSG-hu-BLT) mice, are one such model and have robust reconstitution of human leucocytes within the peripheral blood and tissues. Four NSG-hu-BLT mice were submitted for diagnostic necropsy examination following the development of alopecia, pruritus and lethargy after HSPC engraftment. Histopathology revealed multifocal to coalescing single keratinocyte cell death in the epidermis and follicles with dermatitis and mild dermal fibrosis. Single-cell hepatocyte cell death was present in three cases, with various degrees of portal fibrosis. In the skin and liver, cell death was associated with lymphocytes that reacted with anti-human CD45, CD3 and CD8 antibodies, consistent with a diagnosis of graft-versus-host disease (GvHD). This study expands on recently reported microscopical features of GvHD in NSG-hu-BLT mice and suggests a role for CD8(+) T lymphocytes in the progression of the disease. NSG-hu-BLT mice represent an excellent model of GvHD, but its prevalence may compromise their use in other fields of biomedical research.

[Radial head and neck fractures in children]. / Fracturas pediátricas de cabeza y cuello de radio.

OBJECTIVE: To analyse cases of radial head and neck fractures in children and compare them with the literature. METHOD: Retrospective and descriptive study of 21 children with radial head and neck fractures. The following parameters were collected: demographics, comorbidity, classification, treatment, need for rehabilitation, lack of range of motion (ROM), time for recovery and complications. RESULTS: The series included 11 males, and the mean age was 8.3 years. The right side was affected in 14 patients. Twelve cases had an associated ipsilateral elbow injury. According to the Chambers classification, 15 cases belonged to group A, while in the Steele-Graham classification, 12 cases were in group I. Eleven patients were treated with immobilization only, 4 percutaneously, and 6 by open reduction and internal fixation (ORIF). Eleven of them needed rehabilitation and despite this, 8 did not achieve full mobility. The mean time to obtain the greatest ROM was 4.71 months. Eight patients had complications, with the most common being neuroapraxia and valgus deformity of the elbow. DISCUSSION AND CONCLUSIONS: Treatment of paediatric radius head and neck fractures must be step-wise, from immobilization only, manual and/or percutaneous reduction, to ORIF, whichever is less indicated. In this respect, both the transcapital needle and/or removal the radius head should be avoided. The most common complication is lack of supination, especially in cases treated by ORIF. The posterior interosseous neuroapraxia was the most common of the rest of complications.

[Bone mineral density in a population with severe infantile cerebral palsy]. / Densidad mineral ósea en la población con afectación severa por parálisis cerebral infantil.

OBJECTIVE: To determine the bone mineral density (BMD) values in children and adolescents with moderate and severe infantile cerebral palsy (ICP) in our catchment area, and compare these values with a healthy population. MATERIAL AND METHOD: A prognostic study of cases and controls for the assessment of BMD in patients from 2 to 18 years old with infantile cerebral palsy belonging to the Gross Motor Function Classification System (GMFCS) Groups IV and V. The BMD measurements were performed at distal femur level, dividing this region into 3 areas following the forearm protocol. RESULTS: The BMD for each of the three areas studied results in the final sample of 69 patients were much lower than the reference levels. There was a statistically significant difference (P<.05) between the BMD values in the two sub-groups studied. DISCUSSION: The greater the involvement, from a neurological point of view, in patients classified as Group V shows a very low BMD compared to patients of similar sex and age. The acquisition of bone capital in patients with ICP does not follow the normal pattern of the healthy population.

Síndrome compartimental glúteo y rabdomiólisis tras sobredosis / Gluteal compartment syndrome and rhabdomyolysis after overdose

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Prevalence of M. tuberculosis infection and tuberculosis disease among HIV-infected people in Spain.

OBJECTIVE: To study the prevalence of Mycobacterium tuberculosis infection (MTBI) and past/current tuberculosis (TB) among human immunodeficiency virus (HIV) infected persons in Spain. DESIGN: Longitudinal study conducted between 2000 and 2003 at 10 HIV hospital-based clinics. Data were drawn from clinical records. Associations were measured using odds ratios (ORs) and their 95% confidence intervals (95%CI). RESULTS: Of the 1242 persons who met the eligibility criteria, most were male (75%), aged <40 years (75%) and unemployed (40%). HIV infection occurred through intravenous drug use (53%), heterosexual sex (29%) and sex between men (16%). In the initial evaluation, 315 subjects had evidence of MTBI: 84 (6.8%) had a history of TB, 23 (1.8%) current TB and 208 (16.8%) latent tuberculosis infection (LTBI). MTBI was associated with male sex, age 30-49 years, contact with a TB case, homelessness, poor education, and negatively with CD4 <100 cells/mm(3). Among subjects with MTBI, past/current TB was associated with retirement/disability (OR 6, 95%CI 1.6-22.5), CD4 <200 cells/mm(3) (OR 9.7, 95%CI 3.8-24.6), viral load >55,000 copies (OR 5.3, 95%CI 1.4-20.0), and negatively, with skilled work (OR 0.4, 95%CI 0.1-1.0) or administrative/managerial/professional work (OR 0.05, 95%CI 0.01-0.4). CONCLUSION: Social context has an impact on the effectiveness of HIV and TB control programmes even in industrialised countries with free access to health care.

Continuous peripheral nerve blockade in lower extremity surgery.

Peripheral nerve blocks afford numerous benefits for lower extremity surgery. There is growing interest in continuous peripheral nerve blocks, mainly for treatment of postoperative pain, a field that represents a challenge to the anaesthesiologist. This paper seeks to review the efficacy of continuous lower limb blocks for postoperative pain relief. Not only do continuous peripheral nerve blocks afford specificity of analgesic area but current research has shown that they enhance postoperative analgesia and patient satisfaction. New techniques and devices are increasingly appearing, and catheters are constantly being developed and improved; an example being the stimulating catheter, which represents one of the newest advances in this area. The above techniques show that continuous postoperative analgesia with catheters in the lower extremities is not only possible, but indeed provides sustained effective postoperative analgesia, reduces use of opioids, and improves rehabilitation and patient well-being with minimal side-effects. These techniques could prove an alternative to postoperative pain treatment following ambulatory surgery.

Alteraciones del trazado de la frecuencia cardíaca fetal durante el parto en gestantes con oligoamnios causado por rotura prematura de membranas / Foetal heart rate changes dirung labour in pregnant women with oligohydramnios due to premature rupture of the membranes

El objetivo del estudio fue poner de manifiesto cuáles son las alteraciones del registro cardiotocográfico intraparto más características y frecuentes, así como los resultados neonatales, en una población seleccionada por el hecho de presentar oligoamnios debido a rotura prematura de membranas. El estudio llevado a cabo por el Hospital Universitario Virgen de las Nieves de Granada incluye casos consecutivos de gestantes que ingresaron con el diagnóstico de RPM en las 24 h previas para inducción del parto en el área de dilatación. La población elegida estaba formada por gestantes con ILA normal en la semana previa a la rotura de las membranas ovulares. En total fueron incluidas 130 gestantes que se asignaron en función del ILA inicial a 2 grupos, siendo el grupo 2 considerado el control con respecto a los casos de oligoamnios. El grupo I estaba formado por gestantes con ILA inicial 5 (n = 82). No se han encontrado diferencias estadísticamente significativas entre ambos grupos respecto de las alteraciones de la frecuencia cardíaca fetal intraparto. Como conclusión, se puede afirmar que, basándonos en estos resultados, no se puede sustentar la hipótesis de que el oligoamnios al inicio del parto causado por RPM aumenta la frecuencia de deceleraciones variables cuando se compara con gestantes que inician el parto con bolsa rota e ILA > 5. Posiblemente, la justificación se encuentre en el hecho de la pérdida progresiva de LA a lo largo del parto que tiende a igualar el ILA en ambos grupos (AU)

The aim of the study was to outline the most common and characteristic intrapartum cardiotocographic changes and neonatal outcome in a selected population with oligohydramnios due to premature rupture of membranes. The study was carried out at the Virgen de las Nieves University Hospital of Granada (Spain), with consecutive cases of pregnant women admitted to the delivery ward for induction of labour due to premature rupture of the membranes during the previous 24 hours. All pregnant women had a normal amniotic fluid index (AFI) the week previous to rupture of the membranes. There were 130 pregnant women inc1uded in the study, and they were divided into 2 groups based on initial AFI value. Group I was pregnant women with an initial AFI value of up to 5 (n = 48), and group II, the control group, of more than 5 (n = 82). No statistically significant differences were found between the 2 groups in intrapartum foetal heart rate pattems. In conclusion, these results do not support the hypothesis that oligohydramnios before the onset of labour due to premature rupture of the membranes increases the incidence of variable decelerations compared to pregnant women with premature rupture of the membranes at the onset of labour and AFI value of greater than 5. This may possibly be because amniotic fluid leakage during labour tends to equalize AFI in both groups (AU)

Neumonía varicelosa en un paciente inmunocomprometido / Varicella pneumonia in an immunocompromised patient

La varicela es una enfermedad contagiosa y con carácter habitualmente benigno. La mayoría de los casos aparecen en la edad infantil y, en pocas ocasiones, cursan con complicaciones. Las más frecuentes son la sobreinfección de las lesiones, la laringitis y la neumonía, así como la encefalitis con cerebelitis. Los neonatos, adultos y los pacientes inmunocomprometidos constituyen el grupo de mayor riesgo. Se presenta el caso de un niño de cuatro años afectado por el síndrome de Cockayne que desarrolló una neumonía varicelosa de evolución fatal. El paciente había recibido tratamiento con corticoides y ciclosporina A durante el año previo a contraer la enfermedad para control de un síndrome nefrótico secundario a mínimos cambios. La neumonía por virus de la varicela es raramente descrita en la bibliografía pediátrica. Este paciente no había recibido vacunación previa. La instauración de un programa de vacunación contra la varicela en la población pediátrica beneficiaría la gran morbilidad de esta enfermedad, así como sus complicaciones derivadas de ella

Varicella is a highly contagious disease that is usually benign in nature. It generally occurs during childhood and complications seldom develop. The most common complications are superinfection of the cutaneous lesions, laryngitis and pneumonia, as well as encephalitis with cerebellitis. New-borns, adults and immunocompromised children present the groups at highest risk. We present the case of a 4-year-old boy with Cockayne syndrome who developed varicella pneumonia that resulted in death. The patient had been receiving corticosteroids and cyclosporine A for one year prior to contagion to treat minimal change nephrotic syndrome. Varicella pneumonia is rarely reported in the pediatric literature. Our patient had not been vaccinated. The establishment of an immunization program against varicella for the pediatric population would help to forestall the morbidity and complications associated with this disease

Hipertensión renovascular en la infancia / Renovascular hypertension in children

El objetivo de este trabajo es conocer los datos epidemiológicos, etiología, clínica al diagnóstico, tratamiento y evolución de la hipertensión arterial renovascular en nuestro medio. Se realizó una revisión retrospectiva de los niños con esta enfermedad diagnosticados en el Hospital Universitario Materno-Infantil de Canarias, de enero de 1993 a diciembre de 2001.Seis pacientes, con edades comprendidas entre los cuatro meses y los siete años, fueron diagnosticados de hipertensión renovascular. La clínica fue inespecífica en cinco pacientes (83,3%). Se demostraron complicaciones cardiacas en todos los pacientes. Los diagnósticos establecidos fueron: 4 pacientes con displasia fibromuscular, 1 con neurofibromatosis, y 1 con síndrome de aorta abdominal. Se efectuaron seis angioplastias percutáneas trasluminales (APT): cuatro como primera elección y dos en segunda alternativa. Tres pacientes recibieron tratamiento médico. Dos niños precisaron nefrectomía para control de la hipertensión arterial. El tiempo medio de seguimiento en consulta fue de 5,8 años (2,5-8 años). Se perdió el seguimiento de un paciente. Dos pacientes (20%) precisan tratamiento farmacológico para control de la presión arterial. Se recomienda valorar la APT como tratamiento de elección de la hipertensión renovascular. Se produjo una alta incidencia de complicaciones neurológicas y cardiacas. Una tercera parte de los pacientes quedaron monorrenos, aunque todos mantienen una función renal dentro de la normalidad

The aim of this study was to assess the epidemiological data, etiology, diagnosis, treatment and course of arterial renovascular hypertension in our pediatric population. For this purpose, we carried out a retrospective review of the children diagnosed as having renovascular hypertension in our hospital between January 1993 and December 2001.Six patients, ranging in age between 4 months and 7 years old, were diagnosed as having renovascular hypertension. The clinical findings were nonspecific in 5 patients (83.3%). All six patients had cardiac complications. The diagnosis was fibromuscular dysplasia in four cases, neurofibromatosis in one and abdominal aorta syndrome in one. All the patients underwent percutaneous transluminal angioplasty (PTA), as the treatment of first choice in four and as a second option in the other two. Three patients received medical treatment. Two of the children required nephrectomy to control arterial hypertension. The mean length of outpatient follow-up is 5.8 years (range: from 2.5 to 8 years). One patient was lost to follow-up. Two patients required pharmacological treatment in order to control their blood pressure. We recommend that PTA be considered as the possible treatment of choice for renovascular hypertension. We report a high incidence of neurological and cardiac complications. One third (n= 2) of our patients have only one kidney, although all of them present normal renal function

Endocarditis bacteriana secundaria a sepsis de origenurológico / Bacterial endocarditis due to urologycal sepsis

Presentamos el caso de una niña de 18 días diagnosticada de endocarditis bacteriana de la válvula aórtica, en el contexto de una pielonefritis con sepsis secundaria por Klebsiella Pneumoniae. El diagnóstico de sospecha se confirmó por ecocardiografía. La evolución de la paciente fue favorable con tratamiento médico y posteriormente se realizó tratamiento quirúrgico de la maltormación urológica subyacente. (AU)

Complementary DNA sequence of the HLA-B*3924 allele.

We have isolated the complete coding region of HLA-B*39 from a Spanish Caucasoid, using a new PCR primer for its 5' untranslated region. The cDNA matched partial genomic sequences of B*3924, an allele whose distribution appears to be restricted to Mediterranean and Arabian Caucasoids. A single amino acid change exclusive to B*3924 (threonine-98) distinguishes it from B*3903.

Pielonefritis xantogranulomatosa: dos casos nuevos / Childhood xanthogranulomatous pyelonephritis: two new cases

Presentamos dos casos clínicos de pielonefritis xantogranulomatosa (PNX) en la infancia. En los dos casos la clínica fue inespecífica y se demostró infección urinaria asociada. En uno de ellos el diagnóstico fue preoperatorio, según las nuevas técnicas de imagen (AU)

We report two cases of xanthogranulomatous pyelonephritis in children. In both cases, the symptoms were nonspecific, but the presence of urinary tract infection was demonstrated. One case coud be diagnosed preoperatively on the basis of new imaging techniques (AU)

[Myasthenia and thymoma in a seven year old girl]. / Miastenia y timoma en una niña de 7 años.

INTRODUCTION: Myasthenia is an autoimmune disease, being generalized muscular weakness, with important participation of facial muscles, a prominent feature. Signs of muscular fatigue arise, worsened by exercise and alleviated by rest. Clinical symptoms are less marked before noon, and get worse as the day advances, through the afternoon and evening. A clear relationship between myasthenia and thymic abnormalities does exist, being glandular hyperplasia and tumours the commonest underlying pathologic findings. Initial treatment is based on anticholinesterase drugs and steroids. Non respondents should be treated with immunoglobulins, immunosuppresses, plasmapheresis and surgical removal of the thymus, according to the symptoms control. CASE REPORT: We present the case of a seven years old girl with generalized muscular weakness, worsening through the day, being the diagnosis of myasthenia confirmed by the high level of acetylcholine antireceptors antibodies and the neurophysiologic study. Imaging study of the mediastinum showed a thymic mass located in the right lobe. CONCLUSION: It is therefore most important to rule out these conditions when myasthenia is suspected.

Miastenia y timoma en una niña de 7 años / Myasthenia and thymoma in a seven year old girl

Introducción. La miastenia es una enfermedad autoinmune, caracterizada por debilidad muscular generalizada, con importante participación de la musculatura facial. Aparecen signos de fatiga muscular, que empeora con el ejercicio y mejora tras el reposo. Las manifestaciones clínicas son menos llamativas por la mañana y empeoran a medida que avanza el día o tras el ejercicio. Existe una relación importante entre la miastenia y la patología del timo, y se asocia con frecuencia a hiperplasias glandulares y a tumores tímicos. El tratamiento inicial se compone de anticolinesterásicos y corticoides, y, si la respuesta no es buena, se utilizan inmunoglobulinas, inmunosupresores, plasmaféresis y la extirpación quirúrgica del timo, todo ello en función del control de los síntomas. Caso clínico. Presentamos una niña de 7 años, con debilidad muscular generalizada, que empeoraba en el transcurso del día, y en la que el estudio neurofisiológico y los títulos de anticuerpos antirreceptores de acetilcolina confirman el diagnóstico de miastenia. Se practica estudio de imagen de mediastino y se demuestra la existencia de una masa tumoral que depende del lóbulo tímico derecho. Conclusiones. Es obligado investigar la patología tímica ante todo paciente sospechoso de la enfermedad (AU)

Complementary DNA sequence of the novel HLA-B*3704 allele.

The novel HLA-B*3704 allele was identified by polymerase chain reaction with sequence specific oligoneucletides (PCR-SSO) in a Spanish Caucasoid individual whose T-lymphocytes showed an ambiguous HLA-B phenotype. The nucleotide sequence of B*3704 was determined after reverse transcription-polymerase chain reaction (RT-PCR) amplification and molecular cloning of its complete coding region. B*3704 differs from B*3701 by a single nucleotide replacement that induces the substitution of histidine for tyrosine 171. Residue 171 is located in the alpha-helix of the alpha-2 domain, lining the A pocket of the peptide-binding site. Therefore, the His171 substitution seen in HLA-B*3704 is likely to affect its antigen-presenting properties and is probably responsible for the differentiated serological phenotype of this allele in comparison with B*3701.

SCH-C (SCH 351125), an orally bioavailable, small molecule antagonist of the chemokine receptor CCR5, is a potent inhibitor of HIV-1 infection in vitro and in vivo.

We describe here the identification and properties of SCH-C (SCH 351125), a small molecule inhibitor of HIV-1 entry via the CCR5 coreceptor. SCH-C, an oxime-piperidine compound, is a specific CCR5 antagonist as determined in multiple receptor binding and signal transduction assays. This compound specifically inhibits HIV-1 infection mediated by CCR5 in U-87 astroglioma cells but has no effect on infection of CXCR4-expressing cells. SCH-C has broad and potent antiviral activity in vitro against primary HIV-1 isolates that use CCR5 as their entry coreceptor, with mean 50% inhibitory concentrations ranging between 0.4 and 9 nM. Moreover, SCH-C strongly inhibits the replication of an R5-using HIV-1 isolate in SCID-hu Thy/Liv mice. SCH-C has a favorable pharmacokinetic profile in rodents and primates with an oral bioavailability of 50-60% and a serum half-life of 5-6 h. On the basis of its novel mechanism of action, potent antiviral activity, and in vivo pharmacokinetic profile, SCH-C is a promising new candidate for therapeutic intervention of HIV infection.

Coinfection of SCID-hu Thy/Liv mice with human herpesvirus 6 and human immunodeficiency virus type 1.

Human herpesvirus 6 (HHV-6) has been proposed as a potential cofactor in the progression of human immunodeficiency virus type 1 (HIV-1) disease. We used the SCID-hu Thy/Liv mouse model to evaluate the in vivo interactions between HHV-6 and HIV-1. Our results demonstrate that HHV-6 and HIV-1 can simultaneously replicate in the human thymus in vivo. In this model, however, the presence of one virus appears not to modify the replication or cytopathicity of the other.