Diagnóstico e abordagem terapêutica da constipação idiopática crônica: uma revisão de especialistas brasileiros / Diagnosis and management of chronic idiopathic constipation: a narrative review from a brazilian expert task force

ABSTRACT Background Chronic idiopathic constipation (CIC) is a condition that widely affects the global population, represents relevant healthcare resource utilization and costs, and impacts the individual's well-being. Objective To review the consensus of expert societies and published guidelines on the diagnosis and treatment of CIC in adults, seeking to assist reasoning and decision-making for medical management of patients with CIC and provide a practical reference material. Methods A Brazilian medical task force searched the scientific literature in the following electronic databases: MEDLINE/PubMed, SciELO, EMBASE and Cochrane, using the following descriptors: chronic constipation, diagnosis, management of chronic constipation. In addition, a review of articles on the mechanism of action, safety, and efficacy of therapeutic options available in Brazil was carried out. Results The diagnostic approach and the understanding of the pathophysiology present in CIC are essential items to indicate the appropriate therapy and to understand the ecosystem of the patient's needs. Conclusion CIC is a common condition in adults, occurring more frequently in the elderly and in women. Proper management is defined by detailed medical history and physical examination, together with appropriate therapeutics, regardless pharmacological or not, and depending on the best moment of indication. This way, the impact on quality of life is also optimized.

RESUMO Contexto A constipação idiopática crônica (CIC) é uma condição que afeta amplamente a população global, representa um grande custo econômico, causa substancial utilização de recursos em saúde e impacta o bem-estar do indivíduo. Objetivo Revisar os consensos de Sociedades de especialistas e diretrizes publicados sobre o diagnóstico e tratamento da CIC em adultos, buscando auxiliar o raciocínio e a tomada de decisão para a conduta médica frente ao paciente e oferecer um material prático de referência. Métodos Uma força tarefa médica brasileira realizou uma busca na literatura científica nas bases de dados eletrônicos Medline/PubMed, SciELO, Embase e Cochrane, tendo sido utilizados os seguintes descritores: chronic constipation, diagnosis, management of chronic constipation. Adicionalmente, foi realizada uma revisão de artigos sobre o mecanismo de ação, segurança e eficácia das opções terapêuticas disponíveis no Brasil. Resultados A abordagem diagnóstica e o entendimento da fisiopatologia presente na CIC são itens fundamentais para que seja indicada a terapêutica apropriada e seja compreendido o ecossistema de necessidades do paciente. Conclusão A CIC é uma condição comum em adultos, ocorrendo com maior frequência em idosos e mulheres. O manejo correto é definido pela anamnese e exame físico detalhados, juntamente com a terapêutica apropriada, independentemente de ser farmacológica ou não, conforme o melhor momento de indicação. Desta forma, o impacto na qualidade de vida também é otimizado.

Cambios en el patrón epidemiológico de la colitis ulcerosa crónica idiopática en México en un hospital de tercer nivel de atención / Changes in chronic idiopathic ulcerative colitis epidemiological pattern in Mexico in a tertiary care hospital

Resumen Antecedentes: La colitis ulcerosa crónica idiopática (CUCI) es una enfermedad con inflamación crónica de la mucosa del colon de origen multifactorial. El objetivo de este trabajo es identificar posibles cambios en el comportamiento de la CUCI en un hospital de referencia. Métodos: Se incluyeron nuevos casos de CUCI confirmados por histopatología de enero del 2007 a diciembre del 2014. Resultados: Se incluyeron un total de 189 pacientes. La media de nuevos casos anuales de CUCI fue de 23.6. Este estudio incorpora 95 pacientes de sexo masculino (50 %) y 94 de sexo femenino (50 %), con una edad promedio al diagnóstico de 44.6 años. La frecuencia de pancolitis fue del 77 %, en comparación con el 59 % en el periodo anterior. Las manifestaciones extraintestinales (MEI) estuvieron presentes en el 55.8 % y las colectomías en el 5.2 %. Conclusión: Algunas características de la enfermedad han cambiado con el tiempo: aumento de la frecuencia de pancolitis y MEI, así como disminución de la tasa de colectomías.

Abstract Background: Chronic idiopathic ulcerative colitis (CIUC) is a disease with multifactorial chronic inflammation of the colonic mucosa. In Mexico, there are studies that show an increase in the frequency of new cases. The purpose of this work was to identify possible changes in CIUC behavior in a referral hospital. Methods: New ulcerative colitis (UC) cases confirmed by histopathology from January 2007 to December 2014 were included. Results: A total of 189 patients were included. Mean number of UC annual new cases was 23.6. The study included 95 male patients (50 %) and 94 female patients (50 %), with an average age of 44.6 years at diagnosis. The frequency of pancolitis was 77 %, in comparison with 59 % in the previous period. Extra-intestinal manifestations (EIM) were present in 55.8 % and colectomies in 5.2 %. Conclusion: Some characteristics of the disease have changed over time: there is an increased frequency of pancolitis and EIM, as well as a decrease in the rate of colectomies.

Research advances in congenital non-hemolytic jaundice / 临床肝胆病杂志

Congenital non-hemolytic jaundice is an important type of jaundice diseases, and except breast milk jaundice, the other types of this disease are relatively rare in clinical practice. Most of them belong to genetic and metabolic liver diseases, including Gilbert syndrome, Crigler-Najjar syndrome, and Lucey-Driscoll syndrome with an increase in unconjugated bilirubin and Dubin-Johnson syndrome and Rotor syndrome with an increase in conjugated bilirubin. With reference to the recent literature in China and foreign countries, this article reviews the pathogenesis, genetic characteristics, diagnosis, treatment, and differential diagnosis of six types of hereditary congenital unconjugated jaundice.

Role of immunoglobulin E autoantibodies in autoimmune skin diseases / 中华皮肤科杂志

A large number of studies have shown that immunoglobulin E (IgE) not only participates in the occurrence and development of allergic reactions, but also induces and aggravates autoimmune reactions through various mechanisms. IgE autoantibodies have been confirmed to be present in a variety of autoimmune skin diseases, and may be involved in the occurrence and development of related diseases by affecting multiple immune cells such as dendritic cells, mast cells, and basophils via binding to autoantigens. This review summarizes the role and possible mechanism of action of IgE in the induction and exacerbation of autoimmune skin diseases such as systemic lupus erythematosus, bullous pemphigoid and chronic idiopathic urticaria, and provides a theoretical basis for clinical diagnosis and treatment.

Prucalopride: novel drug for chronic idiopathic constipation

Chronic Idiopathic Constipation (CIC), defined as constipation in which the underlying cause is unknown, is a common medical illness with a profound negative impact on health-related quality of life and increased propensity for life threatening complications. Current treatment for CIC includes lifestyle modifications, over-the-counter medications, and prescription medications. Presently, the only approved, prescription products for CIC in the US are prosecretory agents. However, the current knowledge that serotonin plays an important role in colonic motility has opened new horizons in the treatment of CIC promoting use of prokinetic agents with a different mechanism of action. Prucalopride is a highly selective 5-hydroxytryptamine type 4 (5-HT4) receptor agonist that enhances propulsive motor patterns in the large intestine due to a high affinity for 5-HT4 receptors in gastrointestinal (GI) tissues. The onset of action of Prucalopride is fast, shows rapid absorption, oral bioavailability of 93% and linear pharmacokinetics. Most common adverse reactions seen are headache, nausea, diarrhea, and abdominal pain. Clinical trials for Prucalopride have been positive, and results suggest that the drug may be a new safe and effective option for CIC treatment, especially for patient’s refractory to prosecretory agents. As a prescription drug for the management of constipation and given the virtual demise of other prokinetic agents for this indication, prucalopride competes primarily with another class of agents: those that stimulate secretion. With Shire Pharmaceuticals having already received US FDA approval in Dec 2018, Prucalopride may soon be a new addition to the mounting armoury of drugs against CIC.

Hiperbilirrubinemias hereditarias: un diagnóstico diferencial a considerar en ictericia / Hereditary hiperbilirrubinemias: a differential diagnosis to considerer in icterus

Las hiperbilirrubinemias hereditarias (HBH) son patologías originadas por defectos en las enzimas y proteínas que participan del metabolismo de la bilirrubina. El clearence de bilirrubina incluye captación y almacenamiento en hepatocitos, conjugación, excreción hacia la bilis y recaptura de su forma conjugada por hepatocitos. Las HBH varían de acuerdo a su patogenia, presentación clínica, niveles de bilirrubinemia y tratamientos disponibles. En general son poco frecuentes, a excepción del Síndrome de Gilbert. Están las que son de predominio indirecto, como el Síndrome de Gilbert y el de Crigler-Najjar, y las de predominio directo, como el Síndrome de Dubin-Johnson y el de Rotor. En general no requieren tratamiento específico y tienen curso benigno, a excepción del Síndrome de Crigler-Najjar para el cual existen medidas terapéuticas específicas a considerar, teniendo un pronóstico reservado para algunas de sus formas de presentación. Es importante el conocimiento de estos síndromes dado el alto índice de sospecha requerido para su diagnóstico y para su diferenciación de otras patologías hepatobiliares de mayor riesgo y severidad.

Hereditary hiperbilirrubinemias (HBH) are pathologies originated from the defect of the enzymes and proteins involved in the metabolism of bilirubin. The bilirubin clearance includes uptake and storage in hepatocytes, conjugation, excretion into bile and recapture of its conjugated form by hepatocytes. HBH vary according to their pathogenesis, clinical presentation, levels of bilirubin and available treatments. Generally they are infrequent, except for Gilbert Syndrome. There are those with indirect bilirubin predominance, such as Gilbert and Crigler-Najjar syndromes, and those with direct bilirubin predominance, including Dubin-Johnson and Rotor syndromes. In general, they do not require specific treatment and have a benign course, with the exception of the Crigler-Najjar Syndrome, for which there are specific therapeutic measures to consider, as well as a reserved prognosis for some of their forms of presentation. The knowledge of these syndromes is important 2 given the high index of suspicion required for its diagnosis and for its differentiation from other hepatobiliary pathologies of greater risk and severity.

A study of Allergy pattern in Chronic Idiopathic Urticaria

Introduction: Urticaria is a common disease with many different clinical presentations. Urticaria is traditionally classified into acute and chronic urticaria with acute off time of 6 weeks. The aim of study is to find the common allergens involved in chronic idiopathic urticaria. Aim of the study: To find common allergens involved in patients of Chronic Idiopathic Urticaria. Materials and methods: A total of 100 patients with chronic idiopathic urticaria were included in the study and allergy test was done using chemiluminescence method. Conclusion: Allergy test are useful diagnostics test in the management of chronic idiopathic urticaria.

System Evaluation of Glucoside Tripterygium Total Combined with Antihistamine Medicine for Chronic Idiopathic Urticaria / 中国中医药信息杂志

Objective To systematically evaluate the effectiveness and safety of glucoside tripterygium total (GTT) combined with antihistamine medicine for chronic idiopathic urticaria (CIU). Methods All randomized or semi-randomized controlled trials (RCTs or semi-RCTs) of GTT in treating CIU were collected from CNKI, VIP, WanFang Data, CBM, Embase, PubMed and Cochrane Library clinical controlled trials database. After two researchers conducted screening and data extraction independently, the quality of the included literature research was evaluated according to the risk of bias tool described in the Cochrane Handbook version 5.1.0, and then RevMan 5.3 was used to undertake Meta analysis. Results A total of 21 articles about RCTs and semi-RCTs were obtained, including 2194 patients. Meta analysis indicated that GTT combined with antihistamine medicine treatment showed higher effective rates compared with the control group [RR=1.33, 95%CI (1.26, 1.40), P<0.000 01], with obviously lower recurrence rate [RR=0.45, 95%CI (0.33, 0.62), P<0.000 01]. There was no statistical significance in adverse reactions. Conclusion GTT combined with antihistamine medicine for CIU has definite efficacy, and is relatively safe. Whether apply GTT combined with antihistamine medicine for CIU should be verified through randomized controlled trial with large-scale samples, multiple centers and high quality.

Clinical effect of autohemotherapy combined loratadine on 120 cases of ASST positive chronic idiopathic urticaria / 重庆医学

Objective To investigate the efficacy and safety of autohemotherapy therapy in ASST positive chronic idiopathic ur‐ticaria .Methods One hundred and twenty cases of chronic idiopathic urticaria treated in our department from April ,2012 to Janu‐ary ,2013 were divided into treatment group and control group ,60 cases in each group .Patients in treatment group were given oral ioratadine (10 mg/d) ,supplemented by autohemotherapy ;patients in control group were only given oral ioratadine (10 mg/d) .Both of the two groups were treated with twelve weeks ,then observed the effectiveness and safety of two kinds of treatment .Results The effective rate of treatment group and control group were 68 .33% and 48 .33% respectively ,and there was significant difference between the two groups (P<0 .01) .Conclusion Autohemotherapy combined antihistamine was of high efficiency and safety in the treatment of ASST positive chronic idiopathic urticaria ,while the long‐term curative effect remains to be observed .

A randomized placebo-controlled double-blind pilot study of methotrexate in the treatment of H1 antihistamine-resistant chronic spontaneous urticaria.

Background: Chronic urticaria not responsive to antihistamines is a diffi cult disease to manage. Methotrexate has been used in diffi cult chronic urticarias with some benefi t. Objective: To evaluate the effi cacy of methotrexate in the treatment of chronic spontaneous urticaria poorly responsive to H1 antihistaminics. Methods: In a randomized double-blind trial at the Department of Dermatology and Venereology of a tertiary care centre, 29 patients with chronic spontaneous urticaria not responding well to H1 antihistaminics were recruited. Patients were randomly allocated to receive either a weekly dose of oral methotrexate 15 mg or placebo (calcium carbonate) for a total duration of 12 weeks, after which treatment was stopped and patients were followed up for relapse of urticaria. Each group also received levocetrizine 5 mg once daily for symptom control. Primary outcome measured was a reduction by >2/3rd of baseline urticaria scores after 12 week therapy. Secondary outcome was a reduction in antihistamine requirement after stopping therapy. Results: Fourteen patients were randomized to the methotrexate group and fi fteen patients to the placebo group. Out of 17 patients who completed therapy, the primary outcome was achieved by 3.5 ± 1.9 (out of 10) patients in the methotrexate group and by 3.67 ± 1.03 (out of 7) patients in the placebo group (P > 0.05). Ten patients followed up, after stopping therapy, for a mean period of 3.5 ± 2.4 months; 3 remained in remission and 7 had relapsed. One patient had uncontrollable nausea and vomiting after taking methotrexate and was withdrawn from the study. The placebo group did not experience any side effects. Conclusions: Methotrexate 15 mg weekly for 3 months did not provide any additional benefi t over H1 antihistamines in this study but an adequately powered study with longer follow up is required to assess its utility.

Study of serum interleukin (IL) 18 and IL-6 levels in relation with the clinical disease severity in chronic idiopathic urticaria patients of Kashmir (North India)

BACKGROUND: Chronic urticaria is termed as idiopathic if there is an absence of any identifiable causes of mast cell and basophil degranulation. Various cytokines have been found to be involved in inflammatory processes associated with chronic idiopathic urticaria, including interleukin (IL) 18 and IL-6. OBJECTIVE: To evaluate any possible correlation of IL-18 and IL-6 cytokines with the clinical disease severity in chronic idiopathic urticaria (CIU). METHODS: IL-18 and IL-6 levels of CIU patients (n = 62) and healthy controls (n = 27) were assessed by commercially available enzyme linked immunosorbent assay kits following the manufacturer's protocols. RESULTS: Serum IL-18 concentration (mean ± standard deviation [SD], 62.95 ± 36.09 pg/mL) in CIU patients and in healthy controls (54.35 ± 18.45 pg/mL) showed no statistical significance (p > 0.05). No statistically significant difference (p > 0.05) was observed between autologous serum skin test (ASST) positive and ASST negative patients with regard to the serum IL-18 levels either. Similarly, serum IL-6 concentration (0.82 ± 4.6 pg/mL) in CIU patients and in healthy controls (0.12 ± 1.7 pg/mL), showed no statistical significance (p > 0.05). Also, comparison between positive and ASST negative patients with regard to the serum IL-6 levels was statistically nonsignificant (p > 0.05). However, statistical significance was found both in IL-18 and IL-6 concentrations in certain grades with regard to the clinical disease severity of urticaria. CONCLUSION: There is no significant association as such found between IL-18 and IL-6 levels with CIU, however, these cytokines may help in predicting the clinical disease severity in CIU. Hence, these cytokines may indicate a potential role as a biomarker to assess the disease severity in CIU.

Correlations between Anti-Mast Cell Autoantibodies and Chronic Idiopathic Urticaria

BACKGROUND: The etiology of chronic idiopathic urticaria (CIU) is not completely clear. There are a few antibodies were reported to correlate with CIU. OBJECTIVE: To investigate the correlation these antibodies and CIU. METHODS: The autologous serum skin test (ASST) and allergens were performed. Serum levels of immunoglobulin E (IgE), anti-FcepsilonRI and anti-IgE, anti-Helicobacter pylori (HP) antibodies and anti-thyroglobulin antibody (TGAb) were measured in 100 patients with CIU, acute urticaria (AU) and normal controls respectively. RESULTS: Eighty-six percent food or inhalant allergens were detected in AU patients, but no allergens were detected in CIU patients and normal controls. Serum anti-FcepsilonRI antibody and anti-IgE antibody levels were higher in the CIU than that in the AU patients and normal controls (p<0.05, respectively). IgE level was lower in the CIU patients (T=190.00, p< 0.05), but increased in the AU patients (T=226.00, p<0.05) compared with the normal controls. The ASST positive rates in the CIU and the AU patients were 53.4% and 12.6% respectively, but all normal controls were negative. The anti-FcepsilonRI antibody level was higher in the ASST-positive CIU patients than those negative ones (T=101.73, p<0.05). In anti-HP antibody positive and TGAb positive CIU patients, anti-FcepsilonRI antibody positive rate was higher than AU patients (p<0.01) and normal controls (p<0.01). CONCLUSION: The anti-FcepsilonRI and anti-IgE antibodies play a key role in CIU, but anti-HP antibody and TGAb have an indirect correlation with CIU.

Usefulness of the Autologous Serum Test for the Diagnosis of Chronic Idiopathic Urticaria

BACKGROUND: The majority of chronic urticaria cases are chronic idiopathic urticaria (CIU) with no specific identifiable etiology. The role of autoantibodies in such cases remains controversial. OBJECTIVE: This study determined the positivity rate of autologous serum tests in CIU patients. METHODS: This study was performed on 30 patients with CIU and 30 individuals without any systemic or dermatologic disease. After the volar parts of right and left forearms were cleansed, 0.05 ml serum physiologic and 0.05 ml autologous serum were injected intradermally on the right forearm 5 cm apart from each other, resulting in the formation of small papules; meanwhile, 0.05 ml histamine alone was injected to the left forearm. The test results were evaluated after 30 minutes as positive in positive cases. RESULTS: The autologous serum test produced significant and non-significant results in patients with CIU and controls, respectively. The positivity rates of the autologous serum test in the CIU and control groups were 53.3% and 26.6%, respectively. There was no relationship between autologous serum test positivity and sex in either group. In male patients with CIU, positive results ranged widely with age, while in female patients, positive results were mainly observed at younger ages with a narrow age range. CONCLUSION: The autologous serum test is a useful test in the diagnosis and treatment of CIU as well as the selection of immunotherapy, especially in patients refractory to classic therapy.

Advances in pathogenesis of chronic idiopathic thrombocytopenic purpura / 国际儿科学杂志

Idiopathic thrombocytopenic purpura (ITP),as a heterogeneous autoimmune disease,is the most common bleeding disorder,of which etiology and pathogenesis are still unclear. Health related quality of life are severely affected,especally in chronic patients . Studys on chronic idiopathic thrombocytopenic purpura pathogenesis ,both in China and abroad,have found that genetic factors may be involved in the occurrence and prognosis of the disease. The paper gives a review from the follow aspects,such as its risk factors,relationship between genetic polymorphisms or expression levels and chronic ITP susceptibility and seriousness,the role of epigenetic,researchs about chronic and refractory ITP,et al.

Clinical Study on Treatment of Chronic Idiopathic Thrombocytopenic Purpura by Expelling Wind, Cooling Blood and Invigorating Kidney Prescription / 中国中医药信息杂志

Objective To observe the effect of expelling wind, cooling blood and invigorating kidney prescription on the marrow megakaryocyte differentiation maturity and TGF-β1 in patients with chronic idiopathic thrombocytopenic purpura (CITP). Methods Fifty CITP patients were randomly divided to three groups, including 20 patients administrated with the expelling wind, cooling blood and invigorating kidney prescription as TCM group, 15 patients with the same decoction and prednisone as combine group, and 15 patients with prednisone as western medicine group, 4 weeks as one treatment course. After 3 courses, the change of platelet counts, marrow megakaryocyte amount, platelet-produced megakaryocyte proportion and TGF-β1 level in the marrow were observed. Results The fine efficiency of TCM group (55.0%, 11/20) excelled western medicine group (20.0%, 3/15) significantly and close to that of combine group (60.0%, 9/15). Platelet counts of TCM group was raised obviously after treatment (P<0.01), better than that of western medicine group (P<0.05), and was similar to that of combine group. The marrow megakaryocyte amount of TCM group was effectively decreased and the platelet-produced megakaryocyte proportion was increased greatly (P<0.01), similar to that of combine group, which of western medicine group had no significant difference between pre-therapy and post-therapy. TGF-β1 of TCM group and combine group degraded obviously (P<0.05) compared with that before treatment, and was significant deviation compared with that of western medicine group (P<0.05). Conclusion The expelling wind, cooling blood and invigorating kidney prescription can effectively degrade the value of TGF-β1 of CITP patients, facilitate the maturity of the marrow megakaryocyte amount and raise the platelet counts.

Clinical Study of Itch in Korean Patients with Chronic Idiopathic Urticaria / 대한피부과학회지

BACKGROUND: Although itch is a cardinal symptom of urticaria, its clinical characteristics are not well elucidated in Korean urticaria patients. OBJECTIVE: To characterize the clinical patterns and sensory, affective and therapeutic dimensions of itch in Korean patients with chronic idiopathic urticaria (CIU). METHODS: A face-to-face structured questionnaire based on the McGill pain questionnaire was given to 83 patients with CIU. RESULTS: All of the 83 patients recruited in the study have fully filled-out the questionnaire. In 71.1 percent of the total CIU patients, itch appeared on a daily basis. Patients experienced exacerbation of itch during winter (41.0%) and at night (37.3%). The buttock (50.6%) and front of the thigh (50.6%) were major sites of involvement with itchiness. Patients perceived itch as burning (62.7%), stinging (44.6%) and crawling sensations (42.2%), rather than stabbing (25.3%), tickling (24.1%) and pinching (10.8%). The main associated symptom was heating sensation (60.2%). The majority of the patients answered that itch was annoying (84.3%), worrisome (80.7%) and bothersome (78.3%). Korean CIU patients answered that stress (36.1%) and hot climate (33.7%) aggravated the itch, and medications (55.4%) alleviated it. Among antihistamines, systemic corticosteroids and topical corticosteroids, antihistamines were the most effective treatment for management of itch in patients with CIU. CONCLUSION: This study highlights the detailed description and characteristics of itch in Korean CIU patients. Moreover, we tried to emphasize the clinical differences of itch in CIU patients in comparison with that in atopic dermatitis patients.

A Case of Sustained Cholestasis Caused by Acute A Viral Hepatitis in Dubin-Johnson Syndrome / 대한소화기학회지

Dubin-Johnson syndrome is a rare clinical entity. It shows intermittent symptoms such as chronic or intermittent jaundice, abdominal pain, weakness, nausea, vomiting, anorexia and diarrhea. Symptoms are precipitated or aggravated by pregnancy, alcoholism, surgical procedures and intercurrent disease. Chronic idiopathic jaundice is typical of Dubin-Johnson syndrome and its prognosis is good. We describe a case of prolonged cholestasis for more than 10 months caused by acute A viral hepatitis in a patient with Dubin-Johnson syndrome. It is a first report of cholestasis complicated by acute A viral hepatitis in a patient with Dubin-Johnson syndrome.

Paquimeningitis craneal hipertrófica y estenosis traqueal idiopáticas: coincidencia o asociación? / Idiopathic hypertrophic cranial paquimeningitis and tracheal stenosis: coincidence or association?

La paquimeningitis hipertrófica idiopática crónica y la estenosis traqueal subglótica idiopática son raras condiciones de origen desconocido. En ambas existen un proceso inflamatorio fibrótico que afecta respectivamente, la duramadre y la traquea. La paquimeningitis hipertrófica idiopática crónica en forma típica, causa parálisis progresiva de nervios craneales, cefaleas, hipertensión intracraneal o disfunción cerebelosa. En la estenosis traqueal subglótica idiopática, usualmente limitada a la región subglótica y los dos primeros anillos traqueales, los sintomas son variables incluyendo disnea progresiva, sibilancias y estribor notables sobre el cuello. La paquimeningitis hipertrófica idiopática crónica ocurre en pacientes de todas las edades y el examen de elección para detectarla es la resonancia magnética cerebral, en tanto que la estenosis traqueal subglótica idiopática suele afectar mujeres jóvenes o maduras y es mejor evidenciada mediante tomografía computarizada. El diagnóstico es uno de exclusión. Deben descartarse enfermedades infecciosas como tuberculosis y lúes, y otras como sarcoidosis, carcinomatosis meníngea o vasculitis. Suelen responder inicialmente a los corticosteroides pero puede haber recurrencia al suspenderlos. La evolución a largo plazo es incierta. Este trabajo informa acerca de una paciente en quien ambas condiciones se dieron cita, discutiéndose sus manifestaciones clínicas, radiológicas y patológicas. Aunque considerada esencial para el diagnóstico, en nuestro caso no se realizó una biopsia meníngea. Planteamos la posibilidad de una asociación entre ambas condiciones, por su inicio simultáneo con recaídas, por su condición de inflamación crónica y recurrente y su respuesta a los corticosteroides.

Chronic idiopathic hypertrophic paquimeningitis and chronic subglottic tracheal stenosis are rare conditions of unknown origin. In both there is a fibrotic inflammatory process affecting, respectively the dura mater and the trachea. The chronic idiopathic hypertrophic paquimeningitis in its typical presentation causes progressive paralysis of cranial nerves, headaches, intracranial hypertensión or cerebellar dysfunction. In the chronic subglottic tracheal stenosis, usually limited to the subglottic region and the two first traqueal rings, its symptoms are variable including progressive shortness of breath, wheezing and stridor notable on the neck. The chronic idiopathic hypertrophic paquimeningitis occurs in patients of all ages and the test of choice for diagnosis it is the brain magnetic resonance image, while the chronic subglottic tracheal stenosis usually affect young or mature women and is best evidenced by computerizide tomography scan. The diagnósis is one of exclusion. Infectious diseases such as tuberculosis and lues, and others as sarcoidosis, meningeal carcinomatosis or vasculitides should be discarded. Often initially respond to corticosteroids but may have recurrences when stopped. The evolution in the long term is uncertain. This paper reports on a patient with both conditions, discussing their clinical, radiological and pathological manifestation. Although considered essential for the diagnosis, in our case was not performed a meningeal biopsy. We postulate the possibility of association between these two conditions, based in their simultaneous onset, the occurence of relapses in both diseases, its condition of recurrent and chronic inflammation, and its response to corticosteroids.

Tratamiento de la mielofibrosis idiopática crónica con bajas dosis de talidomida. Comunicación de 2 casos / Treatment of chronic idiopathic myelofibrosis with talidomide low doses: Report of 2 cases

La mielofibrosis idiopática crónica (MIC), también conocida como metaplasia mieloide agnogénica, mielofibrosis primaria, mieloesclerosis con metaplasia mieloide, y mielofibrosis idiopática, se caracteriza por esplenomegalia, hematopoyesis extramedular, anemia progresiva, reacción leucoeritroblástica, hematíes en lágrimas en sangre periférica y fibrosis en médula ósea. Se han obtenido beneficios modestos con las terapias para la anemia (eritropoyetina y andrógenos) o la esplenomegalia (hidroxiurea, interferón-alfa). Ninguno de estos regímenes confiere un beneficio de supervivencia o cambio demostrable en la fibrosis intramedular. La ausencia de tratamiento eficaz para la enfermedad ha llevado al estudio de sus mecanismos patogénicos y el uso de nuevas alternativas terapéuticas. Se describen 2 pacientes con diagnóstico de MIC de 9 y 5 años de evolución que debido a los altos requerimientos transfusionales y la gran esplenomegalia, se les administró tratamiento con talidomida y prednisona. El tratamiento combinado logró aumento de las cifras de hemoglobina y de los conteos de plaquetas y una reducción y eliminacin de los requerimientos transfusionales

Chronic idiopathic myelofibrosis (CIM) also known as agnogenic myeloid metaplasia, primary myelosclerosis with myeloid metaplasia and idiopathic myelofibrosis is characterized by splenomegalia, extramedullary hematopoiesis, progressive anemia, leukoerythroblastosis reaction, tears white cells in peripheral blood and bone marrow fibrosis. There have been modest benefits with anemia therapies (erythropoietin and androgens) or the splenomegalia (hydroyurea, alpha-interferon). Neither of these regimes confers survival benefit or a demonstrable change in extramedullary fibrosis. The lack of an effectiveness treatment for this disease has leads us to study its pathogenic mechanisms and the use of new therapeutical alternatives. Two cases are described diagnosed with CIM with a course of 9 and 5 years and due to the high transfusion requirements and a significant splenomegalia it was necessary to administer a treatment with thalidomide and prednisone. Combination treatment achieved an increase in hemoglobin figures and of platelet counts and a decrease and elimination of transfusion requirements

The Efficacy, Safety and Long-term Effect of Cyclosporine for Treating Chronic Idiopathic Urticaria / 대한피부과학회지

BACKGROUND: The efficacy and safety of cyclosporine for the treatment of chronic idiopathic urticaria (CIU) have been studied in numerous trials, but there have been few studies on the long-term effect of cyclosporine. OBJECTIVE: This study was aimed to assess the efficacy and safety of low-dose cyclosporine for treating CIU. Furthermore, its long-term effect on the natural course of CIU was investigated. METHODS: Thirty patients who suffered from persistent CIU despite conventional treatments received 2~3 mg/kg/day of cyclosporine for 12 weeks. The severity score was assessed by means of the urticaria activity score and the visual analogue score at baseline and at weeks 2, 4, 8 and 12. The safety assessments consisted of reporting the side effects and monitoring the laboratory parameters. After a follow-up period of at least 1 year, the patients were asked whether they had any remaining or new symptoms and whether they still used antihistamines or any other drugs. RESULTS: Twenty seven patients completed the trial medication, and the respective symptom scores significantly improved after 12 weeks. Four (14.8%) patients reported adverse events during the trial period, but the events were not severe enough to require withdrawal from the study. Of the twenty one patients who were followed for at least 1 year (range: 12~45 months) after the completion of cyclosporine administration, eight patients (38.1%) were symptom free, and seven patients (33.3%) used only antihistamines. CONCLUSION: This study shows that low-dose cyclosporine is an efficacious and safe treatment option for treating CIU. In addition, the preliminary results suggest that low-dose cyclosporine might be helpful for the long-term control of this disease.