Perfil clínica y electromiográfico de las distrofias musculares en República Dominicana / Clinical and electromyographic profile of muscular dystrophies in the Dominican Republic

Introducción: Las distrofias musculares son trastornos miogénicos hereditarios caracterizados por una atrofia muscular progresiva y una debilidad de distribución y gravedad variable. La población de Republica Dominicana es fruto de una mezcla de etnias, haciéndola portadora de una herencia cromosómica y ADN diverso, siendo susceptibles a poder presentar cualquier desorden de carácter hereditario. Material y métodos: Con una muestra de 17 pacientes obtenidos entre septiembre 2019- marzo 2020, se realizó un estudio retrospectivo, descriptivo y transversal, en el cual se hizo una revisión de los expedientes de la clínica de miopatías en la consulta de neurología pediátrica del Hospital Infantil Doctor Robert Reid Cabral, para describir el perfil clínico de los pacientes con distrofia muscular y los hallazgos de electromiografía en los casos que la misma. Resultados: se encontró que la distribución de la edad correspondió a 5-9 años en un 53%, siendo el sexo masculino, el más frecuente. En el 70.59% presentaron antecedentes familiares de distrofia muscular. Los principales motivos de consulta fueron cansancio y caídas frecuentes. Conclusión: En los hallazgos de electromiografía, el porcentaje de pacientes que presentó esta prueba con alteraciones fue de 88.24% y sin alteraciones el 11.76%. Esto nos demuestra, la gran utilidad de dicho estudio en el diagnóstico de las distrofias musculares en países donde no se cuenta con estudio molecular, siendo una de las pruebas esenciales en el abordaje diagnóstico de los pacientes con sospecha clínica de dichas patologías.

Introduction: Muscular dystrophies are hereditary myogenic disorders characterized by progressive muscular atrophy and weakness of variable distribution and severity. The population of the Dominican Republic is the result of a mixture of ethnic groups, making it the bearer of a diverse chromosomal inheritance and DNA, being susceptible to presenting any hereditary disorder. Methods: With a sample of 17 patients obtained between September 2019-March 2020, a retrospective, descriptive and cross-sectional study, in which a review of the files of the myopathies clinic was made in the pediatric neurology consultation of the Children's Hospital Doctor Robert Reid Cabral, to describe the clinical profile of patients with muscular dystrophy and the electromyography findings in the cases with the same. Results: The age distribution corresponded to 5-9 years; 53%, being the masculines, the most frequent sex. In 70.59%, there was a family history of muscular dystrophy. The main reasons for consultation were fatigue and frequent falls. Conclusion: In the electromyography findings, the percentage of patients who presented this test with alterations was 88.24% and 11.76% without alterations. This result shows us the great utility of said study in the workup of muscular dystrophies in countries with no availabilities for molecular studies, being one of the essential tests in the diagnostic approach of patients with clinical suspicion of said pathologies.

Linfoma de células B de alto grado de malignidad con morfología blastoide durante el embarazo: reporte de un caso / High-grade malignant B-Cell lymphoma with blastoid during pregnancy: a case report

Resumen ANTECEDENTES: Durante el embarazo es más común el linfoma de Hodking que el no Hodking; afecta, en promedio, a mujeres de 30 años (18-44 años) y más. Suele diagnosticarse alrededor de las 28 semanas de embarazo y está documentado que puede llegarse al término. Los esquemas de tratamiento pueden iniciarse en el posparto inmediato o, incluso, antes. La incidencia mundial del linfoma no Hodking es de 0.8 por cada 100,000 mujeres; se desconoce la supervivencia durante el embarazo. CASO CLINICO: Paciente de 34 años, con antecedentes obstétricos de tres embarazos, una cesárea y un aborto y el embarazo actual en curso de las 29 semanas, referida de la ciudad de Colima debido a un reporte de BI-RADS 3 en el ultrasonido de mama y un nódulo mamario palpable, con evidencia de múltiples tumoraciones en la zona hepática, esplénica y peripancreática. La biopsia tomada de las zonas de la lesión reportó: linfoma de células B de alto grado de malignidad, con morfología blastoide y expresión de C-MYC y BCL2. Además, la paciente se encontró con: anemia, dolor abdominal, múltiples nódulos hepáticos y adenopatías abdominales. Se decidió la interrupción del embrazo a las 30 semanas, con la obtención de un recién nacido, sin complicaciones. Enseguida se inició el tratamiento con rituximab-etopósido-prednisolona-vincristina-ciclofosfamida-doxorrubicina (R-EPOCH) con adecuada adaptación por la paciente. CONCLUSION: Puesto que la información bibliográfica de linfoma y embarazo es escasa el caso aquí reportado es relevante por su aporte. La atención multidisciplinaria favorecerá siempre el pronóstico de las pacientes.

Abstract BACKGROUND: Hodking's lymphoma is more common during pregnancy than non-Hodking's lymphoma; it affects, on average, women aged 30 years (18-44 years) and older. It is usually diagnosed around 28 weeks of pregnancy and is documented to be carried to term. Treatment regimens can be initiated in the immediate postpartum period or even earlier. The worldwide incidence of non-Hodking's lymphoma is 0.8 per 100,000 women; survival during pregnancy is unknown. CLINICAL CASE: 34-year-old patient, with obstetric history of three pregnancies, one cesarean section and one abortion and the current pregnancy in progress at 29 weeks, referred from the city of Colima due to a report of BI-RADS 3 on breast ultrasound and a palpable breast nodule, with evidence of multiple tumors in the hepatic, splenic and peripancreatic area. Biopsy taken from the lesion areas reported: high grade malignant B-cell lymphoma, with blastoid morphology and expression of C-MYC and BCL2. In addition, the patient was found to have: anemia, abdominal pain, multiple hepatic nodules and abdominal adenopathies. It was decided to terminate the pregnancy at 30 weeks, with the delivery of an uncomplicated newborn. Rituximab-Etoposide, Prednisone, Vincristine, Cyclophosphamide, and Doxorubicin (R-EPOCH) therapy was started immediately with adequate adaptation by the patient. CONCLUSION: Since bibliographic information on lymphoma and pregnancy is scarce, the case reported here is relevant for its contribution. Multidisciplinary care will always favor the prognosis of patients.

Effects of peripartum treatment on delivery outcomes in women with primary immune thrombocytopenia: a prospective cohort study / 中华围产医学杂志

Objective:To investigate the effects of peripartum administration of low-dose corticosteroids or intravenous immunoglobulin (IVIG) on delivery outcomes in pregnant patients with primary immune thrombocytopenia (ITP).Methods:This prospective cohort study involved pregnant women (≥34 gestational weeks) who were diagnosed with ITP in Peking University People's Hospital from January 2017 to December 2021. Their platelet counts were between 20×10 9/L to 50×10 9/L without bleeding and none of them had been treated with any medications. All patients were divided into medication group (prednisone or IVIG) and platelet transfusion group based on their preference. Differences in vaginal delivery rate, postpartum hemorrhage rate and platelet transfusion volume between the two groups were compared using t-test, Wilcoxon rank sum test and Chi-square test. Binary logistic regression was used to investigate the factors influencing the rates of vaginal delivery and postpartum hemorrhage. Multiple linear regression was used to analyze the factors influencing the platelet transfusion volume. Results:A total of 96 patients with ITP were recruited with 70 in the medication group and 26 in the platelet transfusion group. The vaginal delivery rate in the medication group was higher than that in the platelet transfusion group [60.0% (42/70) vs 30.8% (8/26), χ 2=6.49, P=0.013]. After adjusted by the proportion of multiparae and the gestational age at delivery, binary logistic regression showed that the increased vaginal delivery rate in patients undergoing the peripartum treatment ( OR=4.937, 95% CI: 1.511-16.136, P=0.008). The incidence of postpartum hemorrhage in the two groups was 22.9% (16/70) and 26.9% (7/26), respectively, but no significant difference was shown ( χ 2=0.17, P=0.789). The median platelet transfusion volume was lower in the medication group than in the platelet transfusion group [1 U(0-4 U) vs 1 U(1-3 U), Z=－2.18, P=0.029]. After adjustment of related factors including the platelet count at enrollment, obstetrical complications and anemia, multiple linear regression showed that the platelet transfusion volume was also lower in the medication group (95% CI:0.053-0.911, P=0.028). Ninety-six newborns were delivered without intracranial hemorrhage. The overall incidence of neonatal thrombocytopenia was 26.0% (25/96). There was no significant difference in birth weight, and incidence of neonatal asphyxia or thrombocytopenia between the two groups. Conclusion:Peripartum therapy in ITP patients may increase vaginal delivery rate and reduce platelet transfusion volume without causing more postpartum hemorrhage.

Effects of mycophenolate mofetil on T cell subsets, renal function, erythrocyte sedimentation rate and C-reactive protein in patients with systemic lupus erythematosus / 中国基层医药

Objective:To investigate the effects of mycophenolate mofetil combined with prednisone and hydroxychloroquine on immune function, renal function, erythrocyte sedimentation rate and C-reactive protein in patients with systemic lupus erythematosus.Methods:A total of 64 patients with systemic lupus erythematosus who received treatment in Zhoushan Hospital from March 2018 to March 2021 were included in this study. These patients were divided into control and observation groups according to different treatment drugs. The control group ( n = 33) was given prednisone and hydroxychloroquine treatment. The observation group ( n = 31) was given mycophenolate mofetil treatment based on prednisone and hydroxychloroquine treatment. Before and after treatment, the number of T cell subsets, renal function, erythrocyte sedimentation rate and C-reactive protein level were determined in each group. The Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) was compared between the two groups. Results:Before and after treatment, there was no significant difference in CD 3+ cells between the two groups [control group: (58.23 ± 9.74)%, (59.12 ± 10.59)%; observation group: (57.33 ± 10.27)%, (58.85 ± 9.74)%, t = -0.34, -0.60, both P > 0.05]. After treatment, CD 4+ cells and CD 4+/CD 8+ cells in each group were significantly increased compared with those before treatment (both P < 0.05). After treatment, CD 4+ cells and CD 4+/CD 8+ cells in the observation group were significantly higher than those in the control group [(34.11 ± 5.37)% vs. (30.95 ± 6.65)%, (1.42 ± 0.33) vs. (1.23 ± 0.33), t = -2.08, -2.30, both P < 0.05]. After treatment, serum creatinine, urea nitrogen and 24-hour urine protein in each group were significantly decreased compared with those before treatment (all P < 0.05). After treatment, serum creatinine, urea nitrogen and 24-hour urine protein in the observation group were significantly lower than those in the control group [(65.36 ± 16.28) μmol/L vs. (91.88 ± 18.74) μmol/L, (5.19 ± 0.94) mmol/L vs. (8.57 ± 1.27) mmol/L, (0.12 ± 0.04) g/L vs. (0.22 ± 0.06) g/L, t = -6.02, -12.03, -7.79, all P < 0.05]. After treatment, erythrocyte sedimentation rate in each group was significantly decreased compared with that before treatment (both P < 0.05). After treatment, erythrocyte sedimentation rate in the observation group was significantly lower than that in the control group [(26.36 ± 11.29) mm/h vs. (39.89 ± 13.74) mm/h, t = -4.28, P < 0.05]. After treatment, C-reactive protein level in each group was significantly decreased compared with that before treatment (both P < 0.05), and C-reactive protein level in the observation group was significantly lower than that in the control group [(7.52 ± 3.23) mg/L vs. (12.83 ± 5.72) mg/L, t = -4.53, P < 0.05]. After treatment, SLEDAI in each group was significantly decreased compared with that before treatment (both P < 0.05), and SLEDAI in the observation group was significantly lower than that in the control group [(5.52 ± 1.25) points vs. (8.25 ± 2.42) points, t = -5.61, P < 0.05]. Conclusion:Mycophenolate mofetil combined with prednisone and hydroxychloroquine for systemic lupus erythematosus can improve patient's immune function and renal function, reduce the body's inflammatory response, and better lower disease activity.

Fulminating herpes simplex hepatitis

ABSTRACT Herpes simplex virus (HSV) is a rare cause of acute hepatitis in patients with chronic immunosuppression, including Crohn's disease. HSV hepatitis has the propensity to cause acute liver failure and death. The presenting signs and symptoms can be nonspecific, thereby causing the diagnosis to go overlooked with inadequate management, leading to a high mortality rate. We report a case of a 31-year-old male on chronic prednisone treatment for Crohn's disease who unexpectedly died. Subsequently, an autopsy showed HSV hepatitis as the cause of death. Thus, although a rare complication, HSV hepatitis should always be kept in mind as a fatal complication in patients with acute hepatitis and chronic immunosuppression.

A case series on the impact of abiraterone acetate with prednisone in metastatic prostate adenocarcinoma: The real world experience

OBJECTIVE@#To evaluate the clinical benefit of Abiraterone acetate plus prednisone (AA + P) withandrogen deprivation therapy in patients with metastatic prostate cancer as a local experience in thePhilippines.@*MATERIALS AND METHODS@#The authors evaluated retrospectively a case series of seven patients receivingandrogen deprivation therapy with high-risk metastatic castration-sensitive prostate cancer (mCSPC)and metastatic castration-resistant prostate cancer (mCRPC) treated with AA + P in a tertiary hospitalfrom April 2019 to October 2020. Disease characteristics, biochemical trend, quality of life evaluationusing the European Organization for Research and Treatment of Cancer Questionnaire (EORTCQLQ-C30 v.3), and adverse events reporting using Common Terminology Criteria for Adverse Events(CTCAE) Version 5.0 were all retrieved from the medical records as outcome measures.@*RESULTS@#Analysis of 18 months period using chart review was done. Five patients showed clinicalimprovement on positive PSA response. Patients also presented with Grade 1-2 adverse events scorebased on CTCAE including hypertension, hepatotoxicity, gastrointestinal symptoms, and electrolyteimbalances. Using the EORTC QLQ-C30 v.3 showed that AA + P provided significant improvementon the overall quality of life, functioning in terms of role, emotional, cognitive and social aspectswith reasonable safety profile and minimal adverse events limited to worsening of gastrointestinalsymptoms from baseline.@*CONCLUSION@#The addition of AA + P to androgen deprivation therapy is a suitable option for bothhigh-risk mCSPC and mCRPC exhibiting a significant biochemical, functional and quality of lifeimprovement with reasonable safety profile and limited adverse events in the ‘real-world’ setting, whichis comparable with the findings in other similar studies.

Neutrophilic dermatosis of the dorsal hands related to cocaine abuse

Abstract Neutrophilic dermatoses encompass a wide spectrum of diseases characterized by a dense infiltration mainly composed of neutrophils. Neutrophilic dermatosis of the dorsal hands is currently considered a localized variant of Sweet syndrome. Cocaine abuse has been related to a wide range of mucocutaneous manifestations, including neutrophilic dermatoses such as pyoderma gangrenosum. The authors of this study present a patient with neutrophilic dermatosis of the dorsal hands, in which cocaine abuse was identified as a probable trigger.

Switch from abiraterone plus prednisone to abiraterone plus dexamethasone in patients with metastatic castration-resistant prostate cancer: a case report / 中华泌尿外科杂志

To explore the therapeutic effect of abiterone combined with dexamethasone by reporting a case of metastatic castration-resistant prostate cancer (mCRPC) who was treated with abiterone combined with prednisone and then changed to abiterone combined with dexamethasone. A 61-year-old man was admitted to Sir Run Run Shaw Hospital in November 2017 due to elevated prostate-specific antigen (PSA) at 11 ng/ml. Prostate MRI showed that abnormal signal intensities in the periphery of the prostate which was considered as prostate cancer. Consideration of metastases in the right iliac crest; partial signal changes in the medial seminal vesicles of both sides which were considered involved. The prostate needle biopsy demonstrated that left prostate adenocarcinoma while Gleason score: 5+ 4=9, diagnosed as metastatic hormone-sensitive prostate cancer (mHSPC). In December 2017, the patient underwent robot assisted laparoscopic radical prostatectomy. Prostatic adenocarcinoma was confirmed by postoperative pathology, Gleason score: 5+ 4=9. The bilateral seminal vesicles and nerves were invaded. The level of PSA was monitored during the continuous postoperative treatment using bicalutamide and goserelin.The PSA was 0.32, 0.15, and 1.72 ng/ml in February, June and October of 2018. In October 2018, the testosterone was 40 ng/dl, considering the biochemical progress. In November 2018, the PSA was 2.51ng/ml, considering entering mCRPC. The treatment plan was switched to abiraterone, prednisone and goserelin, and PSA was monitored. In January, March, and June of 2019, the PSA was 0.1, 0.03, and 1.1 ng/ml. By March 2020, it had reached 8.9 ng/ml. The treatment plan was changed to abiraterone and dexamethasone. In April, July and September of 2020, PSA was 7.9, 3.98, and 2.58 ng/ml respectively. The treatment is still ongoing.Abiraterone combined with prednisone is still effective after asymptomatic PSA progression in mCRPC.

The clinical exploration of corticosteroid switch of abiraterone acetate in patients with metastatic castration-resistant prostate cancer / 中华泌尿外科杂志

The terminal stage of prostate cancer is metastatic castration-resistant prostate cancer (mCRPC) with poor prognosis and high mortality. Abiraterone acetate (AA), as a new generation drug for endocrine therapy, has been demonstrated to prolonged overall survival signicantly in mCRPC patients. In addition, a corticosteroid drug must be administered during treatment to avoid side effects of abiraterone. It has been found that a corticosteroid switch from prednisone to dexamethasone could delay the development of resistance, significantly prolong the progression-free survival rate of patients, with well tolerance. But the specific mechanism and long-term clinical benefit still need further study. This emerging treatment paradigm provides new ideas for treatment options for patients with mCRPC, however, caution is still needed in clinical practice, and it is recommended to determine the treatment plan after considering all aspects of the patients.

The efficacy of low dose short-term prednisone therapy for remission induction in newly diagnosed rheumatoid arthritis patients

Abstract Background: The ACR/EULAR recommendations endorse the use of glucocorticoids (GCs) for rheumatoid arthritis (RA) patients' flares and as a bridge to a DMARD. However, the recommendation of low dose short-term monotherapy with (GCs) remains open to the discretion of the clinician. The aim of this study was to assess whether a short-term use of low dose prednisone monotherapy was effective in inducing remission in newly diagnosed RA patients. Methods: A retrospective analysis of patients newly diagnosed with RA at a Community Health Center in North Dakota was performed based on the ACR/EULAR RA classification criteria. Demographic and clinical data were abstracted from patients' medical charts. Patients treated with (≤ 10 mg/day) of prednisone up to 6 months were included. Response to prednisone was analyzed according to pre- and post-treatment DAS28-ESR score and EULAR response criteria. Results: Data on 201 patients were analyzed. The mean prednisone dose was 8 mg/day (range: 5-10; SD = 1.2) and the mean treatment duration was 42.2 days (12-177; 16.9). Disease severity significantly improved from baseline to follow-up for: tender joint count (8.6 ± 4.8 vs. 1.5 ± 3.3; P < 0.001), swollen joint count (6.2 ± 5.0 vs. 1.4 ± 3.0; P < 0.001), and visual analog pain score (4.8 ± 2.6 vs. 2.1 ± 2.5; P < 0.001). DAS28-ESR disease severity significantly improved from baseline to follow-up: (5.1 ± 1.2 vs. 2.7 ± 1.3; P < 0.001). Per EULAR response criteria, 69.7% of patients showed good response to treatment and 20.4% showed moderate response. 54.2% of patients reached remission. Conclusion: Short-term use of low dose prednisone monotherapy induced disease remission and improved clinical severity of RA in the majority of newly diagnosed patients.

Effects of salbutamol, montelukast and prednisone on orthodontic tooth movement in rats / Efeitos do salbutamol, montelucaste e prednisona no movimento dentário ortodôntico em ratos

Objective: To evaluate the effect of salbutamol, montelukast, and prednisone on orthodontic tooth movement in rats. Material and Methods: In vivo experimental preclinical study. The sample consisted of 48 rats randomly distributed in four study groups. Group A was given saline solution; to group B, salbutamol 4 mg/Kg; to group C, montelukast 2.5 mg/Kg and to group D, prednisone 2.5 mg/Kg. All were fitted with orthodontic devices and the medications were administered intraperitoneally every 12 hours for 5 days. The clinical evaluation (variation in the interincisal distance) was performed at one, three, five, and seven days and the histopathological analysis (cell count) at five and seven days. Results: In the clinical evaluation of the variation in the interincisal distance, a significant difference was found in all the evaluations (p <0.05). It was found that the salbutamol group presented higher variation values in the interincisal distance on all the days evaluated. In the histopathological analysis at five and seven days, it was found that the osteoblast and osteocyte count was significantly higher in the salbutamol group compared to the other groups (p <0.05). However, in the subgroup analysis, it was found that there was no significant difference in the osteoblast and osteocyte count between the prednisone, montelukast, and control group (p> 0.05). Conclusion: The administration of salbutamol increased the magnitude of orthodontic tooth movement; nonetheless, the administration of montelukast and prednisone did not modify the magnitude of orthodontic tooth movement in rats. (AU)

Objetivo: Avaliar o efeito do salbutamol, montelucaste e prednisona no movimento dentário ortodôntico em ratos. Material e métodos: Estudo pré-clínico experimental in vivo. A amostra foi composta por 48 ratos distribuídos aleatoriamente em quatro grupos de estudo. O grupo A recebeu solução salina; para o grupo B, salbutamol 4 mg/kg; ao grupo C, montelucaste 2,5 mg/kg e ao grupo D, prednisona 2,5 mg/kg. Todos foram equipados com dispositivos ortodônticos e os medicamentos foram administrados por via intraperitoneal a cada 12 horas por 5 dias. A avaliação clínica (variação da distância interincisal) foi realizada em um, três, cinco e sete dias e a análise histopatológica (contagem de células) em cinco e sete dias. Resultados: Na avaliação clínica da variação da distância interincisal, houve diferença significativa em todas as avaliações (p <0,05). Verificou-se que o grupo salbutamol apresentou maiores valores de variação na distância interincisal em todos os dias avaliados. Na análise histopatológica aos cinco e sete dias, verificou-se que a contagem de osteoblastos e osteócitos foi significativamente maior no grupo salbutamol em comparação aos demais grupos (p<0,05). No entanto, na análise de subgrupos, verificou-se que não houve diferença significativa na contagem de osteoblastos e osteócitos entre os grupos prednisona, montelucaste e controle (p>0,05). Conclusão: A administração de salbutamol aumentou a magnitude do movimento dentário ortodôntico; no entanto, a administração de montelucaste e prednisona não modificou a magnitude do movimento dos dentes ortodônticos em ratos. (AU)

Nefritis lúpica: experiencia con dosis reducidas de glucocorticoides en una unidad de enfermedades autoinmunes sistémicas / Lupus nephritis: low-dose glucocorticoids in a systemic autoimmune diseases unit / Nefritis lúpica: experiência con doses reduzidas de glicocorticoides em uma unidade de doenças autoinmunes sistémicas

Resumen: Introducción: las recomendaciones actuales del tratamiento de la nefritis lúpica (NL) apuntan a dosis de glucocorticoides más bajas para lograr el control de la enfermedad y evitar el daño acumulado. Objetivo: conocer y comparar la respuesta al tratamiento de pacientes con NL proliferativa en su etapa de inducción con dos pautas de tratamiento con prednisona (PDN): dosis iniciales reducidas 30 mg/d. Método: se compararon variables clínicas, analíticas y terapéuticas de pacientes con NL proliferativa categorizados en dos grupos según la dosis inicial de prednisona (PDNi) estándar o reducida. Resultados: se estudiaron 21 pacientes con NL proliferativa (n=12 PDNi reducida vs. n=9 PDNi estándar). No hubo diferencias significativas en las variables clínicas y analíticas. Se observó una diferencia estadísticamente significativa en el número de pulsos de metilprednisolona (5 ± 2,95 PDNi 30 mg/d, p = 0,041) y en la dosis de prednisona acumulada a 6 meses (12,8 mg ± 4,9 PDNi 30 mg/d, p =0,008). No hubo diferencias significativas en la proporción de pacientes que alcanzaron la respuesta completa, en el tiempo hasta alcanzarla ni en los efectos adversos entre ambos grupos. Conclusiones: el esquema terapéutico del grupo PDNi <30 mg/d se asoció a una menor dosis acumulada de prednisona y una respuesta al tratamiento comparable, lo que hace presumir menor daño acumulado relacionado al uso de glucocorticoides.

Abstract: Introduction: current recommendations to treat lupus nephritis (LN) point to low-dose glucocorticoids to control the disease and avoid cumulativedamage. Objective: to learn about and compare the response of patients with proliferative LN who are treated following two prednisone therapy guidelines: reduced initial doses 30 mg/d during the induction stage. Method: clinical, analytical and therapeutic guidelines of patients with proliferative LN were compared and classified into two groups according to the standard or low-dose initial prednisone dose. Results: 21 patients with proliferative LN were studied (n=12 low-dose initial prednisonevs. n=9 standard initial prednisone). No significant differences were found between clinical and analytical variables, although a significantly different statistic difference was observed in the number of methylprednisone pulses (5 ± 2.95 initial prednisone 30 mg/d, p = 0.041) and in the prednisone dose accumulated in 6 months (12.8 mg ± 4.9 initial prednisone 30 mg/d, p =0.008). No significant differences were seen between both groups in the proportion of patients who achieved complete response, neither in terms of the time it took to achieve it or in the side effects. Conclusions: the treatment plan for the initial prednisone <30 mg/d was associated to a lower cumulative dose of response prednisone considering the comparable treatment, what suggests there being smaller cumulative harm as a consequence of the use of glucocorticoids.

Resumo: Introdução: as recomendações atuais para o tratamento da nefrite lúpica (NL) estão orientadas a doses de glicocorticoides mais baixas para controlar a enfermidade e evitar o dano acumulado. Objetivo: conhecer e comparar a resposta ao tratamento de pacientes com NL proliferativa na etapa de indução com duas pautas de tratamento com prednisona (PDN): doses iniciais reduzidas 30 mg/d. Método: foram comparadas variáveis clínicas, analíticas e terapêuticas de pacientes com NL proliferativa divididos em dois grupos segundo a dose inicial de prednisona (PDNi) padrão ou reduzida. Resultados: 21 pacientes com NL proliferativa (n=12 PDNi reduzida vs. n=9 PDNi estândar) foram estudados. Não foram observadas diferenças significativas nas variáveis clínicas e analíticas. Observou-se uma diferença estatisticamente significativa no número de pulsos de metilprednisolona (5 ± 2,95 PDNi 30 mg/d, p = 0,041) e nas doses de prednisona acumulada aos 6 meses (12,8 mg ± 4,9 PDNi 30 mg/d, p =0,008). Não foram observadas diferenças significativas na proporção de pacientes que alcançaram a resposta completa, no tempo até alcançá-la nem nos efeitos adversos entre ambos grupos. Conclusões: o esquema terapêutico do grupo PDNi <30 mg/d foi associado a uma menor dose acumulada de prednisona em resposta ao tratamento comparável, o que sugere menos dano cumulativo relacionado ao uso de glicocorticoides.

A case of facial atrophic sarcoidosis in an adolescent, successfully treated with the combination of prednisone and hydroxychloroquine

Abstract Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology. Cutaneous involvement occurs in up to 30% of patients and skin findings are often the initial presenting symptom. The facial atrophic form of sarcoidosis without associated ulceration in adolescents has rarely been described in the literature. We report a case of 13-year-old male patient with a facial atrophic sarcoidosis who was successfully treated with the combination of prednisone and hydroxychloroquine.

Paracoccidiodomicosis suprarrenal / Adrenal paracoccidioidomycosis

La insuficiencia suprarrenal primaria es un defecto en la producción de glucocorticoides, mineralocorticoides y andrógenos sexuales. Los pacientes afectados por esta condición se caracterizan por concentraciones bajas de cortisol y deficiencia de aldosterona con hiponatremia e hiperpotasemia concomitantes. La etiología más común es el desarrollo de anticuerpos contra la enzima 21 hidroxilasa. Otra causa importante de la insuficiencia suprarrenal primaria son las enfermedades infecciosas, en especial en los países de bajos ingresos. Entre las causas infecciosas que se han descrito se encuentran: Mycobacterium tuberculosis, el complejo de Mycobacterium avium, Neisseria meningitidis, Pseudomonas aeruginosa, Haemophilus influenzae, citomegalovirus, Pneumocystis jirovecii, Histoplasma capsulatum, Blastomyces dermatiditis, Cryptococcus neoformans, Cocciodiodes immitis, Nocardia spp. y Paracoccidioides brasiliensis. En este artículo se presenta la imagen de la tomografía de un paciente que presentó falla suprarrenal, con masas en las glándulas suprarrenales, cuya biopsia permitió establecer el diagnóstico final de paracoccidioidomicosis.

Primary adrenal insufficiency is a defect in glucocorticoid, mineralocorticoid and sexual androgens production. Patients with this disorder have low cortisol levels and aldosterone deficiency with concomitant hyponatremia and hyperkalemia. The most common etiology of this disease is the production of antibodies against the enzyme 21 hydroxylase. Another common cause, particularly in low income countries, are infectious diseases. Several micro-organisms have been reported as a causal agent in adrenal insufficiency including Mycobacterium tuberculosis, Mycobacterium avium complex, Neisseria meningitidis, Pseudomonas aeruginosa, Haemophilus influenzae, cytomegalovirus, Pneumocystis jirovecii, Histoplasma capsulatum, Blastomyces dermatiditis, Cryptococcus neoformans, Cocciodiodes immitis, Nocardia spp. and Paracoccidioides brasiliensis. In this article, we present the computerized tomography and the adrenal biopsy of a patient with adrenal insufficiency. The final diagnosis was paracoccidioidomycosis.

Efficacy of medicinal penetration on acupoint as adjunctive therapy on histiocytic necrotizing lymphadenitis of heat-toxin syndrome: a randomized controlled trial / 中国针灸

OBJECTIVE@#To evaluate the clinical efficacy of medicinal penetration on acupoint combined with medication for histiocytic necrotizing lymphadenitis (HNL) of heat-toxin syndrome, and to explore the methods of improving the clinical effect.@*METHODS@#A total of 72 cases with HNL with heat-toxin syndrome were randomly divided into an observation group and a control group, 36 cases in each group. The patients in the control group were treated with oral administration of prednisone tablets for 40 days (first 5 days: 10 mg, three times a day; since then, reduced by 5 mg every 7 days). In the observation group, on the basis of the medication in the control group, the patients were treated with acupoint application and ultrasonic drug penetration therapy, once a day for 14 days. The acupoints of Waiguan (TE 5), Fengchi (GB 20) of affected side and points were selected. The changes of target lymph node swelling, visual analogue score (VAS), axillary temperature and total score of symptoms and signs were evaluated before treatment and 7, 14, 28 and 40 d into treatment; the changes of white blood cell (WBC), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and lactic dehydrogenase (LDH) were evaluated on 14 d and 40 d into treatment; the patients were followed-up for half a year.@*RESULTS@#① Fourteen days into treatment, the clinical cured rate in the observation group was 38.9% (14/36), which was superior to 16.7% (6/36) in the control group (0.05). ④ The recurrence rate in the observation group was 5.6% (2/36), which was similar to 16.7% (6/36) in the control group (>0.05).@*CONCLUSION@#The medicinal penetration on acupoint as adjunctive treatment could effectively relieve the discomfort symptoms of HNL patients with syndrome of heat and toxin, improve the clinical cured rate, and provide the research direction for shortening the course of medication.

Hypercalcemic Crisis and Severe Renal Failure Due to Sarcoidosis Followed by Autoimmune Thrombocytopenia on Remission: A Case of Drug-Refractory and Chronic Disease

A 70-year-old woman, with a history of type II diabetesmellitus, presented with a 1-month history of progressiveweakness, disorientation and decrease appetite. She wasfound to have high serum calcium at 3.4 mmol/L and severerenal failure with serum creatinine at 700 umol/L. She hadnormal kidney ultrasound, vitamin D2, parathyroid hormoneand negative Quantiferon and Brucella tests. PET scanshowed increase uptake in the paratracheal lymph nodes.Mediastinoscopic lymph node biopsy disclosed non-caseatinggranulomata. Serum calcium and subsequently her acuterenal failure had improved with corticosteroids. She could nottolerate Azathioprine, Cyclophosphamide, andMycophenolate for severe gastrointestinal side effects.Moreover, her disease did not respond to Cyclosporine A,Methotrexate, Hydroxychloroquine and Rituximab.Ultimately, she was kept on Prednisone 20 mg daily as along-term maintenance therapy. Eight months afterremission, she developed severe thrombocytopenic purpura.Fortunately, the latter was controlled with temporary increasein her corticosteroid dose for 6 weeks. After 2 years oftreatment, she had spontaneous remission and Prednisonewas discontinued. No relapse was reported 1 year later. Inconclusion: Sarcoidosis can induce hypercalcemic crisis,acute renal failure and thrombocytopenia with activity up to 2years and treatment limited only to Corticosteroids.

Adalimumb Induced Systemic Vasculitis Following Treatment of Hidradenitis Suppurativa

A 52-year-old man, with diabetes mellitus and obesity, hadreceived Adalimumb for extensive Hidradenitis suppurativa.The latter was difficult to control with local antiseptics,antibiotics and surgery. Two weeks later, he developed fever,palpable maculopapular rash and progressive renal failure. Hisskin biopsy showed leucocytoclastic vasculitis with negativeimmune deposits and his kidney biopsy showed crescenticglomerulonephritis. Adalimumb was discontinued and thepatient was treated with Prednisone 1 mg/kg/day. His kidneyfailure improved. However, his skin lesions progressed tonecrotizing fasciitis despite aggressive surgical care.Ultimately, he died from disseminated sepses 2 months later

Rituximab Therapy for Idiopathic Chronic Urticaria with Recurrent Angioedema

We report on 4 patients with severe and idiopathic chronicurticaria with recurrent attacks of angioedema. Initially, theyresponded initially to high dose corticosteroids yet relapsed onhigh-maintenance dose. Subsequently, trial to use steroidsparing drugs viz. intravenous immunoglobulin thenmycophenolate mofetil with hydroxychloroquine and tacrolimushad failed to maintain a remission. Hence, Rituximab wasgiven. One month after Rituximab infusions, Prednisone dosewas successfully tapered down and was discontinued.Subsequently, all patients remained in complete remission for14-24 months of follow up.

Rationality evaluation of predisone dosage in the treatement of SLE with different clinical scoring systems / 中华疾病控制杂志

Objective To evaluate the rationality of predisone dosage in the treatement of SLE with different clinical scoring systems. Methods The clinical data of 51 newly diagnosed patients with systemic lupus erythematosus(SLE) were collected, and disease activity was assessed by SLEDAI- 2000, BILAG- 2004 and SLAM-R scoring systems. The correlations between SLEDAI- 2000, BILAG- 2004 , SLAM-R scores and prednisone dosage were analyzed. Results BILAG-2004 score showed the best correlation with the dosage of prednision (r=0.827, P<0.001) .SLAM score showed the worst correlation with the dosage of prednision (r=0.512, P<0.001). Different treatment choice was associated with different organ/system involvement. Conclusions BILAG- 2004 , SLEDAI- 2000 and SLAM-R scoring systems all can be used as references for the prednisone dosage slection.It is suggested that the BILAG- 2004 scoring system is more reliable and comprehensive in clinical practice．

Efficacy of oral prednisone acetate for prevention of esophageal stenosis after endoscopic submucosal dissection / 中华消化内镜杂志

Objective To investigate efficacy and safety of oral prednisone acetate for prevention of esophageal stenosis after endoscopic submucosal dissection ( ESD) for patients with esophageal precancerous lesions or early esophageal carcinoma. Methods A retrospective analysis was performed on data of 56 patients who underwent circumferential or semi-circumferential ( more than three quarters but not a complete circular) ESD for esophageal precancerous lesions or early cancer in Nanjing Drum Tower Hospital from October 2014 to October 2017. The patients were divided into the study group ( n=26, prednisolone oral administration after ESD ) and the control group ( n=30, without prednisolone oral administration after ESD) . Endoscopic dilatation was performed whenever patients experienced persistent dysphagia to solids. Clinical data, stricture rate, numbers of endoscopic dilatation, and adverse events were compared between the two groups. Results There were no differences in age, gender, location and length of lesions, endoscopic findings, depths of tumor invasion, and pathological subtypes between the two groups ( all P>0. 05) . The proportion of circumferential esophageal lesions in the study group was higher than that in the control group[53. 85％ (14/26) VS 23. 33％ (7/30), χ2=5. 53, P=0. 02]. The rata of post-procedural esophageal stricture in the study group was significantly lower than that in the control group[ 30. 77％ ( 8/26) VS 60. 00％ (18/30), χ2=4. 78, P=0. 03], and the number of endoscopic dilatation was lesser in the study group than the control group (3. 85±2. 57 VS 9. 83±5. 82, t =7. 22, P =0. 00). There were no adverse events related to oral prednisone, and no treatment-related mortality. Conclusion Prednisone acetate oral administration is safe and effective to prevent esophageal stenosis after complete or semi-circular ESD for patients with esophageal precancerous lesions or early esophageal carcinoma.