Clinical and histopathology characteristics of Castleman disease: a multicenter study of 51 Brazilian patients.

Castleman's disease (CD) is a rare and heterogeneous lymphoproliferative disorder, with limited available clinical information in Brazil. A retrospective study was carried out through information contained in the medical records of 51 patients, between July 1999 and June 2020. Seven patients were excluded, and 44 were analyzed in total. The average age of unicentric CD (UCD) patients was 35 years old and of multicentric CD (MCD) patients was 49 years old (p = 0.013). Regarding gender, there was a predominance of females among patients with UCD (68.4%) and males in patients with MCD (57.9%) (p = 0.103). The most common site of involvement in UCD was the cervical region (36.8%). A total of 73.7% of patients with UCD and 68.4% of patients with MCD presented the histological form hialyne-vascular (HV) (p = 0.499). Most patients with laboratory abnormalities had MCD. A total of 78% of the patients were asymptomatic, with the majority of symptomatic patients with MCD (p = 0.042). Only two of the 27 patients evaluated for the presence of human immunodeficiency virus (HIV) had positive serology. HHV-8 was evaluated in 14 cases, being positive in two. Of the patients with UCD, 94.7% underwent excisional biopsy, against only 41.2% of patients with MCD (p = 0.01). The mean follow-up was 61 months. We observed similarities in the clinical profile between patients in our study and patients described in the literature, such as gender, mean age, B symptoms, visceromegaly, fluid accumulation, and treatment. Unlike the literature, the cervical region was the most affected site, besides the greater association of the HV histological subtype among patients with MCD.

Maternal and perinatal outcomes in pregnant women with sickle cell disease: an update

ABSTRACT Introduction: The aim of this study was to describe maternal and perinatal outcomes in pregnant women with sickle cell disease (SCD) followed at Santa Casa de Sao Paulo over a 10-year period (between 2010 and 2019). Method: Fifty-five records of pregnancies were analyzed among 35 women with SCD. Results: Among 29 newborns, 19 (65.5%) were full-term and 10 pre-term; 24 (82.7%) caesareans and 5 (17.2%) natural births were observed. The mean gestational age at birth and mother's age were 36.6 weeks (30-40) and 26.7 years (17-39), respectively. No maternal death was observed. The main maternal obstetric and non-obstetric complications were: preeclampsia and gestational diabetes, and vaso-occlusive crisis, urinary tract infection and acute chest syndrome, respectively. Twenty-six (47.0%) fetal deaths were observed, 24 being intrauterine fetal (14 early abortions, 10 late abortions and 2 stillbirths). Regarding the red blood cell transfusion history, 40 (72.7%) out of 55 pregnancies received transfusion. Pregnant women who received 6 or more transfusions throughout pregnancy had a significantly lower number of abortions, i.e., no cases of early abortion and only 1 case of late abortion, versus 14 and 9 cases in pregnancies with 0-5 transfusions, respectively. Despite advances in the management of SCD, pregnant women with SCD (particularly those with HbSS) are at a high risk for maternal and fetal complications, even though they are followed in reference centers. Conclusion: The lower risk of intrauterine fetal death for those women who received more transfusions throughout pregnancy observed in the current study leads us once more to raise the need for prospective, multicenter, randomized trials to determine whether the potential benefits balance the risks of prophylactic transfusions.

Maternal and perinatal outcomes in pregnant women with sickle cell disease: an update.

INTRODUCTION: The aim of this study was to describe maternal and perinatal outcomes in pregnant women with sickle cell disease (SCD) followed at Santa Casa de Sao Paulo over a 10-year period (between 2010 and 2019). METHOD: Fifty-five records of pregnancies were analyzed among 35 women with SCD. RESULTS: Among 29 newborns, 19 (65.5%) were full-term and 10 pre-term; 24 (82.7%) caesareans and 5 (17.2%) natural births were observed. The mean gestational age at birth and mother`s age were 36.6 weeks (30-40) and 26.7 years (17-39), respectively. No maternal death was observed. The main maternal obstetric and non-obstetric complications were: pre-eclampsia and gestational diabetes, and vaso-occlusive crisis, urinary tract infection and acute chest syndrome, respectively. Twenty-six (47.0%) fetal deaths were observed, 24 being intrauterine fetal (14 early abortions, 10 late abortions and 2 stillbirths). Regarding the red blood cell transfusion history, 40 (72.7%) out of 55 pregnancies received transfusion. Pregnant women who received 6 or more transfusions throughout pregnancy had a significantly lower number of abortions, i.e., no cases of early abortion and only 1 case of late abortion, versus 14 and 9 cases in pregnancies with 0-5 transfusions, respectively. Despite advances in the management of SCD, pregnant women with SCD (particularly those with HbSS) are at a high risk for maternal and fetal complications, even though they are followed in reference centers. CONCLUSION: The lower risk of intrauterine fetal death for those women who received more transfusions throughout pregnancy observed in the current study leads us once more to raise the need for prospective, multicenter, randomized trials to determine whether the potential benefits balance the risks of prophylactic transfusions.

Distúrbios evacuatórios em primigestas após parto normal: estudo clínico / Evacuatory disorders in primigravidae after vaginal delivery: clinical study

Introdução: Os distúrbios evacuatórios são descritos na literatura relacionados ao parto normal e episiotomia. Objetivo: Estudar a incidência de distúrbios evacuatórios em primíparas submetidas à episiotomia, durante o parto normal. Métodos: Estudo prospectivo em 32 pacientes primíparas, submetidas à episotomia durante parto vaginal. Para realização do estudo as pacientes reSponderam os questionários com o score de constipação de Agachan e score de incontinência de Jorge e Wexner, em relação ao período pré-gestacional e 90 dias após o parto. Resultados: Não foram encontradas diferenças estatisticamente significantes quando comparados os resultados dos questionários aplicados, em relação ao período pré-gestacional e 90 dias pós-parto. Conclusões: Não observamos alterações da constipação e continência fecal em primigestas após parto normal com episiotomia.

Introduction: Evacuatories disturbances are described in the literature related to vaginal delivery and episiotomy. Objective: To study the incidence of evacuatories disturbances in primiparous undergoing episiotomy during vaginal delivery. Methods: Prospective study in 32 primiparous patients submitted to episotomy during vaginal delivery. For the study the patients answered in relation to the period before pregnancy and 90 days postpartum questionnaires with the score of Agachan constipation and incontinence score of Jorge and Wexner. Results: No statistically significant differences were found when comparing the results from the questionnaires in relation to the period before pregnancy and 90 days postpartum. Conclusions: Episiotomy in vaginal delivery in primiparous women with no prior history of intestinal symptoms, caused no disorders of anal continence postpartum in the pacients of this study.

Técnica de delorme como opção para o tratamento da procidência retal recidivada: relato de caso / Delorme's technique as an option for the treatment of the rectal prolapse recurrence: case report

O prolapso retal é uma protrusão de todas as camadas do reto no sentido anal caracterizando um aspecto clínico de um tumor anal, inicialmente ele resulta de um esforço intenso com uma redução espontânea e posteriormente ele resulta de um esforço menor com dificuldade na redução. A incidência é maior em mulheres. O diagnóstico é essencialmente clínico e o tratamento é cirúrgico. Relato de caso: NM, 65 anos, diabético e hipertenso, submetido a sacropromontofixação associada a técnica de Moscowicz com recidiva após um mês, então a técnica de Delorme foi utilizada com sucesso. Discussão: os procedimentos abdominais são indicados para pacientes com menor risco cirúrgico devido a grande complexidade e menor taxa de recidiva. Dentre os procedimentos perineais, a técnica de Delorme possui uma aplicação simples, contudo com uma alta taxa de recidiva. A técnica de Delorme possui uma menor taxa de mortalidade e foi a melhor conduta para este paciente.

The rectal prolapse is a protusion of all the layers of the rectum through the anus, characterizing a clinical picture of anal tumor, initially it results of great efforts with spontaneous reduction and subsequently it results of small efforts with difficult reduction. The incidence is bigger in women. The diagnosis is essentially clinical and the treatment is surgical. Case Report: NM, 65 years, diabetic and hypertensive, subjected to sacropromontoryfixation associated to Moscowicz's technique with recurrence after one month, so the technique of Delorme was used successfully. Discussion: the abdominal procedures are indicated for patients with less surgical risk due to its bigger complexity and smaller tax of recurrence. Among the perineal proceedings perineais, the technique of Delorme has simpler execution, however with a bigger rate of recurrence. The technique of Delorme has a smaller rate of mortality and it was the best conduct for this patient.