Manifestaciones clínicas "no criterio" del Síndrome Antifosfolipídico. Presentación de una cohorte nacional

Introducción: El síndrome antifosfolipídico (SAF) es una afección de origen autoinmune caracterizada por trombosis, pérdidas fetales recurrentes y anticuerpos antifosfolipídicos (aFL). Existen manifestaciones clínicas no contempladas en los criterios clasificatorios, que se denominan manifestaciones no criterio. Objetivo: Analizar las manifestaciones clínicas del SAF, enfatizando las manifestaciones no criterio y su relación con el perfil de autoanticuerpos en un hospital general de Montevideo, Uruguay. Métodos: Se realizó un estudio retrospectivo de las historias clínicas de pacientes con diagnóstico definitivo o sospecha de SAF en un servicio de medicina ambulatoria de enfermedades autoinmunes, en el Hospital Maciel, asistidos entre el 2010 y 2019. Resultados: Se incluyeron 78 pacientes, con edad media de 50,3 ± 14,5 años, 69 (88,5%) correspondió a sexo femenino. Cuarenta y seis (59,0%) pacientes presentaron SAF secundario, de los cuales 28 (35,9%) asociaron LES. La trombosis venosa de miembros inferiores fue la manifestación más frecuente (51,3%). Dieciocho (24,0%) pacientes presentaron trombosis arteriales en forma de accidente cerebrovascular. Cincuenta y nueve (75.6%) casos presentaron, además de las manifestaciones clasificatorias, alguna de las manifestaciones "no criterio" y éstas se manifestaron de forma aislada en 10 (12.8%) pacientes. Las manifestaciones no clasificatorias más frecuentes fueron artralgias, livedo reticularis, migraña y trombocitopenia. Se observó una asociación significativa entre la presencia de anti-ß2GPI con manifestaciones cutáneas y de trombocitopenia con al menos una manifestación trombótica. Conclusiones: Las manifestaciones "no criterio" del SAF se presentaron en casi 3 de cada 4 casos, frecuencia similar a la observada en otras series. La presencia aislada de manifestaciones "no criterio" podrían hacer sospechar un SAF y en algunos casos, conducir a la solicitud de anticuerpos.

Introduction: Antiphospholipid syndrome (APS) is an autoimmune condition characterized by thrombosis, recurrent fetal loss, and antiphospholipid antibodies. There are clinical manifestations not contemplated in the classification criteria, which are called non-criterion manifestations. Objective: To analyze the clinical manifestations of APS, emphasizing the non-criterion manifestations and their relationship with the autoantibody profile in a general hospital in Montevideo, Uruguay. Methods: A retrospective analysis of the medical records of patients with a definitive or suspected diagnosis of APS in an outpatient medicine service for autoimmune diseases, at the Maciel Hospital, assisted between 2010 and 2019, was carried out. Results: 78 patients were included, with a mean age of 50.3 +/- 14.5 years, 69 (88.5%) were female. Forty-six (59.0%) patients presented secondary APS, of which 28 (35.9%) associated SLE. Venous thrombosis of the lower limbs was the most frequent manifestation (51.3%). Eighteen (24.0%) patients presented arterial thrombosis in the form of cerebrovascular accident. Fifty-nine (75.6%) cases presented, in addition to the classification manifestations, some of the "non-criterion" manifestations and these manifested in an isolated way in 10 (12.8%) patients. The most frequent non-classifying manifestations were arthralgia, livedo reticularis, migraine and thrombocytopenia. A significant association was observed between the presence of anti-ß2GPI with cutaneous manifestations and thrombocytopenia with at least one thrombotic manifestation. Conclusions: Non-criterion manifestations of APS occurred in almost 3 out of 4 cases, a frequency similar to that observed in other series. The isolated presence of "non-criterion" manifestations could lead to suspicion of APS and, in some cases, lead to the request for antibodies.

Introdução: A síndrome antifosfolipídica (SAF) é uma doença de origem auto-imune caracterizada por trombose, perdas fetais recorrentes e anticorpos antifosfolípidos (aFL). Existem manifestações clínicas não abrangidas pelos critérios de classificação, que são designadas por manifestações não-critério. Objetivo: Analisar as manifestações clínicas da SAF, enfatizando as manifestações não-critério e sua relação com o perfil de auto-anticorpos em um hospital geral de Montevidéu, Uruguai. Métodos: Foi realizado um estudo retrospectivo dos prontuários de pacientes com diagnóstico definitivo ou suspeita de SAF em um serviço ambulatorial de doenças autoimunes do Hospital Maciel, atendidos entre 2010 e 2019. Resultados: Foram incluídos 78 pacientes, com idade média de 50,3 +/- 14,5 anos, sendo 69 (88,5%) do sexo feminino. Quarenta e seis (59,0%) pacientes apresentavam PFS secundária, dos quais 28 (35,9%) tinham LES associado. A trombose venosa dos membros inferiores foi a manifestação mais frequente (51,3%). Dezoito (24,0%) doentes apresentaram trombose arterial sob a forma de acidente vascular cerebral. Cinquenta e nove (75,6%) casos apresentaram, para além das manifestações classificatórias, algumas das manifestações "não-critério" e estas manifestações foram isoladas em 10 (12,8%) doentes. As manifestações não classificatórias mais frequentes foram artralgias, livedo reticularis, enxaqueca e trombocitopenia. Foi observada uma associação significativa entre a presença de anti-ß2GPI com manifestações cutâneas e trombocitopenia com pelo menos uma manifestação trombótica. Conclusões: As manifestações "não-critério" de SAF ocorreram em quase 3 de cada 4 casos, frequência semelhante à observada noutras séries. A presença isolada de manifestações "não-critério" pode levantar a suspeita de SAF e, nalguns casos, levar à pesquisa de anticorpos.

Clinical study advance of orthostatic hypertension in children / 中华实用儿科临床杂志

Orthostatic hypertension (OHT)in children was first proposed by Chinese scholars in 2012.The diagnostic criterion was revised in 2015.OHT in children was not rare in clinical practice.Children and adolescents are susceptible to OHT when they suddenly change posture or standing for a long time.The mechanisms of OHT were not clear.Dizziness,headache,nausea and vomiting were the main manifestations of OHT.Severe symptom in childhood OHT includes syncope.The prognosis of OHT was good,but the abnormal blood pressure will last to adults.Further study in OHT will help to predict the risk of cardiovascular and cerebrovascular diseases and help to evaluate target or-gan injury.The treatment of OHT has been explored,and non -medical treatment is the main option.Study in medical treatment for OHT has not been conducted.Now,the diagnostic criteria in children,epidemiologic status,clinical fea-tures and treatment of OHT were focused to deepen the understanding of OHT in children and improve the clinical standard of the disease.

An Iranian scoring system for diagnosing Buerger's disease

Buerger's disease or thromboangiitis obliterans [TAO] seems to be common in IR Iran, The present study aimed to evaluate an Iranian population with Buerger's disease in order to suggest a diagnostic criterion for Buerger's disease based on the most frequent findings and to compare it with Papa diagnostic criteria. In a cross-sectional study, all patients with resting limb pain, limb ischemic ulcers, intermittent claudication and limb ischemia who referred to the Vascular Clinic of Sina Hospital during 2009-2011 were evaluated. The patients were allocated to Buerger's and non-Buerger's groups; Evaluating 122 patients [61 in each group], according to the model each clinical manifestations and risk factors in the patients with Buerger's disease obtained a score. Absent pulsation, abnormal distal Doppler sonography and ischemic ulcer were respectively present in 58 [95.1%], 58 [95.1%] and 49 [80.3%] individuals with Buerger's disease. Multivariate linear regression analysis and multivariate logistic regression analysis were used for modeling. Considering the model finding findings, diagnostic criteria including age, sex, smoking, Raynaud's phenomenon, abnormal proximal Doppler, diabetes mellitus and hyperlipidemia were suggested [R[2]=0.582]; the sensitivity and specificity of the criteria was respectively 95.1% and 78.7%. Compared with Papa criteria, Kappa coefficient was measured at 0.66 with a P-value<0.001. It seems that the recommended criteria have an acceptable accuracy in diagnosing Buerger's disease, especially in the Iranian population; however, it is necessary to conduct more studies with larger sample sizes to evaluate the criteria, especially in other populations

Pielonefritis aguda recurrente en mujeres / Recurrent acute pyelonephritis in women

Se realizó un estudio descriptivo transversal para describir el comportamiento de la pielonefritis aguda recurrente en mujeres de 18 y más años de edad, que ingresaron en el Hospital Mártires del 9 de Abril de Sagua la Grande, de enero de 2004 a junio de 2007. El universo estuvo constituido por las 593 pacientes ingresadas con ese diagnóstico. Se realizó un muestreo no probabilístico por criterios y la muestra quedó constituida por 67 pacientes. Las edades fluctuaron entre 18 y 85 años. Las manifestaciones clínicas más comunes fueron: dolor lumbar, fiebre y síntomas urinarios bajos. En el examen físico se halló dolor a la palpación profunda de las fosas lumbares, prácticamente, en todos los casos. El diagnóstico de certeza se realizó por el urocultivo, en 104 ocasiones. Los gérmenes que con mayor frecuencia infectaron las vías urinarias altas fueron bacilos gramnegativos y E. Coli fue el más común. La mayor sensibilidad fue a la amikacina y la mayor resistencia, a ampicillina, ciprofloxacilo y ácido nalidíxico. Muchas de las pacientes con mayor cantidad de ingresos tenían anomalías estructurales de las vías urinarias. La enfermedad subyacente más frecuentemente asociada fue la diabetes mellitus

A descriptive cross-sectional study was conducted to describe the behavior of recurrent acute pyelonephritis in women of 18 years of age and older who were admitted to Mártires del 9 de Abril Hospital in Sagua La Grande, from January 2004 to June 2007. The universe was composed of the 593 patients who were admitted with this diagnosis. A non-probabilistic sampling criterion was performed and then, the sample was composed of 67 patients. The ages ranged from 18 to 85 years. The most common clinical manifestations were low back pain, fever and low urinary symptoms. On physical examination, pain on deep palpation of the lumbar fosses was found, practically, in all cases. The diagnosis of certainty was made by urine culture in 104 occasions. The most common germs that infected the upper urinary tracts were gram-negative bacilli, and E. coli was the most common. The greatest sensitivity was seen for Amikacin and the resistance was greater to Ampicillin, Ciprofloxacin and Nalidixic Acid. Many of the patients with the majority of admissions presented structural anomalies of the urinary tract. The most frequently associated underlying disease was diabetes mellitus

Clinical Manifestations and Diagnosis of Psoriatic Arthritis / 대한내과학회지

Psoriatic arthritis (PsA) is an autoimmune arthritis related to psoriasis and one of seronegative spondyloarthropathies. PsA provokes joint pain and morning stiffness more than 30 minutes, which is relieved by exercise. PsA usually affects distal small joints and exhibits asymmetry, which is one of the typical characteristics of PsA and gives clues to make a differential diagnosis between PsA and rheumatoid arthritis. Thirty to forty patients with PsA experience arthritis in one large joint or asymmetric multiple joints. Arthritis in distal joints and arthritis mutilans often develop concurrently and patterns of PsA change along with disease progression. Spondylitis is observed in 20-30% of PsA patients. In contrast to ankylosing spondylitis, spondylitis in PsA present with mild clinical symptoms despite radiological progression, inflammation limited to one spinal tract, cervical spine dominance, non-marginal syndesmophytosis. Enthesitis is also one of the typical characteristics of PsA and it frequently affects Achilles tendon, plantar fascia and tendons inserting pelvic bones. Tenosynovitis can develop accompanied by enthesitis. Typical dactylitis (sausage digit), pitting edema and nail lesions, including nail pits, onycholysis, hyperkeratosis and splinter hemorrhage, also contribute to a differential diagnosis of PsA. Anterior uveitis, SAPHO syndrome, amyloidosis and IgA nephropathy are well-known extra-articular manifestation of PsA. In 2006, a new classification-criterion for PsA was suggested by the CASPAR study. The CASPAR criteria included 5 categories with a certain number of points; 1) skin psoriasis, 2) nail lesions, 3) dactylitis, 4) negative RF and 5) bone formation around joints. The CASPAR criteria should be applied to PsA patients having at least one of three (peripheral arthritis, spondylitis and enthesitis).

Mechanical Intestinal Obstruction after Appendectomy for Perforated Appendicitis in Children

Intestinal obstruction secondary to intraabdominal adhesion is a well-known postoperative complication occurring after appendectomy. The aim of this study was to measure the incidence and clinical manifestations of mechanical intestinal obstruction after appendectomy for perforated appendicitis. We reviewed all of the children (age <16 years) who had been treated for appendicitis at Asan Medical Center between January 1996 and December 2001. Inclusion criterion included either gross or microscopic evidence of appendiceal perforation. Exclusion criteria were interval appendectomy, and patients immune compromised by chemotherapy. Associations of intestinal obstruction with age, sex, operation time, and use of peritoneal drains were analyzed. Four hundred and sixty two open appendectomies for appendicitis were performed at our department. One hundred and seventeen children were treated for perforated appendicitis (78 boys, 39 girls). The mean age was 8.9 years (range 1.5 to 14.8 years). There were no deaths. Eight patients were readmitted due to intestinal obstruction, but there was no readmission due to intestinal obstruction in patients with non-perforated appendicitis. The interval between appendectomy and intestinal obstruction varied from 12 days to 2 year 7 months. Four patients needed laparotomies. In three of four, only adhesiolysis was performed. One child needed small bowel resection combined with adhesiolysis. There was no significant association between age or sex and the development of intestinal obstruction. This was no association with operative time or use of peritoneal drain. Patients who required appendectomy for perforated appendicitis have a higher incidence of postoperative intestinal obstruction than those with nonperforated appendicitis. For the patients with perforated appendicitis, careful operative procedures as well as pre and postoperative managements are required to reduce adhesions and subsequent bowel obstruction.

Construcción y validación de un instrumento de medición de síntomas de enfermedad por reflujo gastroesofágico: informe preliminar / Construction and validation of a measurement instrument of gastroesophageal reflux symptom: preliminary report

Background: Gastroesophageal reflux symptoms are frequent in the general population, but there is a lack of information about its prevalence and valid and reliable scales to determine it. Aim: To design a valid and reliable scale to determine gastroesophageal reflux disease. Subjects and methods: A cross-sectional study with patients and controls was conducted. The diagnosis of gastroesophageal reflux was based of clinical plus radiological, endoscopic or pathologic criteria. A structured questionnaire was built, containing items covering different clinical manifestations of gastroesophageal reflux disease. All subjects were subjected to 24 h esophageal pH monitoring. Internal consistency of items, interobserver reliability, criterion and construct validity, sensitivity, specificity and predicted values were obtained. Results: One hundred thirteen subjects (74 female and 39 male with a mean age of 46 years old) were studied. Seventy three had gastroesophageal reflux and 40 were controls. Internal consistency of the score was 0.82. Inter observer reliability was greater than 0.80 and a significant association was observed between the score and 24 h esophageal pH monitoring (p <0.001). Instrument sensitivity was 97 percent and specificity 83 percent. Conclusions: The designed scale proved to be a valid and reliable instrument to measure gastroesophageal reflux symptoms, that can be used for future prevalence studies

Clinical Observations on Cerebral Sparganosis

We have met 5 cases of cerebral sparganosis since 1984, including one which was reported by Hong et al. In 1985. We reviewed their medical records and clinical manifestations, possible etiologic histories, findings of brain CT and micro-ELISA done in all cases, and findings of brain biopsy done in 2 cases were discussed. 1. Initial manifestations were seizures (in 4 of 5) or limb weak-nesses (in 4 of 5), which were followed by symptoms and signs due to space occupying effects. 2. Raw snakes or raw frogs were ingested in 3 cases and estimated incubation periods in 2 cases were 2 and 5 years respectively. 3. Brain CT's showed widespread low density, usually in white matter area, ipsilateral ventricular dilatation and/or enhaneing nodules. 4. Micro-EILSA's showed that initial titers of sparganum-specific Ab were above criterion (0.22) except for serum of case 5, and F/U micro-ELISA'S done in 3 cases revealed negative conversion (decrease of Ab titer below criterion) only for CSF of case 5, in which surgical removal was successfully done, and otherwise variable changes of titers. 5. Brain biopsies done in 2 cases showed such findin-gs as degenerating worm, granuloma, tunnel formation, and calcospherules.