Nodular regenerative hyperplasia: azathioprine-induced hepatotoxicity in a patient with Crohn's disease

We report the case of a 53-year-old man with Crohn's disease who developed azathioprine-induced nodular regenerative hyperplasia of the liver. The diagnosis was suspected when abnormalities in liver function tests were observed and transabdominal ultrasonography and upper gastrointestinal endoscopy showed signs of portal hypertension. The final diagnosis was established by liver biopsy, showing the characteristic alterations in liver architecture. Outcome was favorable after treatment discontinuation, with complete normalization of liver function tests within a year.The present article describes the incidence and the main clinical characteristics of this adverse effect to thiopurines.     

Tratamiento mínimamente invasivo de los síntomas del tracto urinario inferior por hiperplasia benigna de próstata. Nuestra experiencia inicial con Urolift® bajo anestesia local y sedación

IntroductionThe treatment for lower urinary tract symptoms secondary to benign prostatic hyperplasia with the Urolift® system has been carried out since 2005 with good results in the medium term. In this work, we present our experience performing this technique under local anaesthesia and sedation in 2 Spanish centres.Material and methodsA prospective study was conducted with 20 patients treated with Urolift® under local anaesthesia and sedation between April 2017 and April 2018. The anaesthesia protocol consisted in the placement of 2 lubricants with cold lidocaine (the first one, 10 min before the intervention, and the second one, just before introducing the cystoscopy). A prostate block (similar to the one employed in prostate biopsies) was administered to one third of the patients and 1 mg of intravenous midazolam was added if required during the procedure. Our primary objective is to evaluate the tolerability of this procedure under local anaesthesia using the validated Visual Analogue Scale measurement instrument.ResultsThe procedure has been performed under the same anaesthetic protocol to 20 patients from 2 different centres. The average pain scores on the Visual Analogue Scale were 1.37 for the cystoscopy procedure and 1.19 for the placement of the implants. When asked whether the pain sensations had been higher, lower or the same during the procedure or at the preoperative cystoscopy, only 18% of the patients responded it was higher. In all cases there was a good tolerance to the procedure, and changes to the anaesthesia protocols were never required.ConclusionsWe consider that the Urolift® system under local anaesthesia and sedation is a well-tolerated, safe and effective method for the treatment of lower urinary tract symptoms secondary to benign prostatic hyperplasia.     

Evaluación morfológica endometrial histeroscópica

Objective

To determine the prevalence of endometrial hyperplasia and adenocarcinoma in distinct groups of endometrial morphology defined by hysteroscopy, and to study the validity of hysteroscopic diagnosis in identifying endometrial tumors.

Materials and methods

We performed a prospective study of 830 hysteroscopies carried out between June 1, 2004 and December 31, 2005 in the Gynecology Outpatient Clinic of Hospital Donostia in San Sebastian, northern Spain. Hysteroscopy was used to classify endometria into atrophic, hypotrophic, active, hypertrophic, suspicious for adenocarcinoma and adenocarcinoma, according to a series of morphological criteria. The findings were later correlated with the histopathological diagnoses obtained through endometrial biopsy.

Results

The sensitivity, specificity and negative predictive value (NPV) of hysteroscopic evaluation of endometrial morphology in diagnosing malignant and premalignant disease were extremely high. Sensitivity was 87.5% (95% CI 0.753-0.941), specificity was 94.8% (95% CI 0.925-0.965) and NPV was 98.7% (95% CI 0.971-0.994). The specificity of morphological diagnosis of adenocarcinoma was 99.9%, sensitivity was 74.3%, and the likelihood ratio for a positive result was 625,486. When the groups of adenocarcinoma and suspicious for adenocarcinoma were combined, sensitivity was 100% and specificity was 96.3%.

Conclusions

The prevalence of premalignant disease is very low in the hysteroscopic morphological groups of atrophic, hypotrophic and active endometria. Premalignant disease is slightly more prevalent in hypertrophic endometria and is significantly more prevalent in cases in which the hysteroscopic diagnosis is of suspicious adenocarcinoma and adenocarcinoma. In these cases, the prevalence of adenocarcinoma is very high. Hysteroscopic evaluation of endometrial morphology alone, without biopsy, is a valid tool to exclude or confirm endometrial disease in some groups.     

Hiperplasia nodular focal hepática y embarazo

Focal nodular hyperplasia of the liver in pregnancy may lead to doubts and inappropriate fears regarding the course of the pregnancy. There is a scarcity of literature addressing focal nodular hyperplasia during gestation, and there are even less data focused on delivery. We report the case of a pregnant woman previously diagnosed with an hepatic lesion and present a review of the literature.     

Fetale Chirurgie

Zusammenfassung Die fetale Chirurgie ist ein neuer interdisziplinärer Fachbereich, der sich in spezialisierten Zentren mit der Therapie von wenigen, stark selektierten Patienten beschäftigt. Ziel dieser Bemühungen ist es, Fehlbildungen während der fetalen Entwicklung operativ zu korrigieren. Es soll verhindert werden, dass am Ende der Gestationsphase irreparable Schäden der betroffenen Organe und Systeme entstehen, die eine hohe Morbidität und Letalität beim Neugeborenen verursachen. Beispiele sind die Therapie der obstruktiven Uropathie, die unbehandelt zu einer Niereninsuffizienz führen würde, die Therapie der Lungenhypoplasie bei angeborener Zwerchfellhernie, großer kongenitaler zystischer adenomatoider Malformationen der Lunge und großer sakrokokzygealer Teratome. Die vorliegende Übersicht beschreibt grundsätzliche Prinzipien der fetalen Chirurgie, die pathophysiologischen Prozesse einiger fetalchirurgisch korrigierbarer Fehlbildungen sowie die diagnostischen und therapeutischen Ansätze. Die bisher veröffentlichten Ergebnisse werden dargestellt und kritisch diskutiert.     

Displasia adenomatoide quística del pulmón,recurrente, y agenesia renal bilateral asociada

Congenital cystic adenomatoid malformation of the lung is an infrequent anomaly that is frequently diagnosed by prenatal ultrasonography. Outcome varies from hydrops and fetal death to resolution after birth. The association of this entity with bilateral renal agenesis is rare. We report a recurrent case in a second pregnancy, which is an exceptional finding.     

Clinical and molecular profile of newborns with confirmed or suspicious congenital adrenal hyperplasia detected after a public screening program implementation

ObjectiveTo describe the results obtained in a neonatal screening program after its implementation and to assess the clinical and molecular profiles of confirmed and suspicious congenital adrenal hyperplasia cases.MethodsA cross-sectional study was conducted. Newborns with suspected disease due to high 17-hydroxyprogesterone levels and adjusted for birth weight were selected. Classical congenital adrenal hyperplasia (salt-wasting and simple virilizing forms) was diagnosed by an increase in 17-hydroxyprogesterone levels as confirmed in the retest, clinical evaluation, and genotype determined by SNaPshot and multiplex ligation-dependent probe amplification.ResultsAfter 24 months, 15 classic congenital adrenal hyperplasia cases were diagnosed in a total of 217,965 newborns, with an estimated incidence of 1:14,531. From 132 patients, seven non-classical and 14 heterozygous patients were screened for CYP21A2 mutations, and 96 patients presented false positives with wild type CYP21A2. On retest, increased 17-hydroxyprogesterone levels were found in classical congenital adrenal hyperplasia patients and showed significant correlation with genotype-related classical genital adrenal hyperplasia. The most frequent mutations were IVS2-13A/C>G followed by gene deletion or rearrangement events in the classical form. In non-classical and heterozygous diseases, p.Val282Leu was the most common mutation.ConclusionsThe results underscore the effectiveness of congenital adrenal hyperplasia neonatal screening in the public health system and indicate that the adopted strategy was appropriate. The second sample collection along with genotyping of suspected cases helped to properly diagnose both severe and milder cases and delineate them from false positive patients.     

Clasificación inmunofenotípica de 3 casos de adenomas hepatocelulares. Diagnóstico diferencial con hiperplasia nodular focal

Interest in adenomas has been renewed by the discovery of the molecular changes in these tumors. The latest World Health Organization publication on gastrointestinal tract tumors (2010) includes four types of hepatic adenomas, which are well characterized immunohistochemically, genotypically and phenotypically. In these tumors, medical history and morphological behavior play an important role in determining the risk of malignancy, mainly in adenomas with a b-catenin mutation.     

Incidence of reactive hyperplastic lesions in the oral cavity: a 10 year retrospective study in Santa Catarina,Brazil

IntroductionReactive hyperplastic lesions develop in response to a chronic injury simulating an exuberant tissue repair response. They represent some of the most common oral lesions including inflammatory fibrous hyperplasia, oral pyogenic granuloma, giant cell fibroma, peripheral ossifying fibroma, and peripheral giant cell lesions.ObjectiveThe incidence of those lesions was investigated in an oral pathology service, and the clinical characteristics, associated etiological factors, concordance between the clinical and histopathological diagnostic was determined.MethodsA total of 2400 patient records were screened from 2006 to 2016. Clinical features were recorded from biopsy reports and patients’ files.ResultsA total of 534 cases of reactive hyperplastic lesions were retrieved and retrospectively studied, representing 22.25% of all diagnoses. The most frequent lesion was inflammatory fibrous hyperplasia (72.09%), followed by oral pyogenic granuloma (11.79%), giant cell fibroma (7.30%), peripheral ossifying fibroma (5.24%), and peripheral giant cell lesions (3.55%). Females were predominantly affected (74.19%), the gingiva and alveolar ridge were the predominant anatomical site (32.89%), and chronic traumatism was presented as the main etiological factor. The age widely ranges from the 1st decade of life to the 7th. Clinically, the reactive hyperplastic lesions consisted of small lesions (0.5–2 cm) and shared a strong likeness in color to the oral mucosa. The concordance between the clinical and histopathological diagnostic was high (82.5%).ConclusionReactive hyperplastic lesions had a high incidence among oral pathologies. The understanding of their clinical features helps to achieve a clearer clinical and etiological diagnosis, and the knowledge of factors related to their development. This may contribute to adequate treatment and positive prognosis.