Articles

Toxic epidermal necrolysis -the most serious variant of Steven Johnson Syndrome -arises as the result of cell-mediated cytotoxic reaction against keratinocytes. Most common inciting factors include drugs, and infections. On the other hand, Hemophagocytic lymphohistiocytosis (HLH), is a syndrome characterized by enormous immune response in the absence of down-regulation of activated immune cells resulting in cytokine storm causing severe tissue damage. Up to date, several cases of concomitance of Toxic Epidermal Necrolysis (TEN) and Hemophagocytic Lympohystiocytosis (HLH) in pediatric patients have been reported. Both situations can be fatal and pediatricians should be aware that these two clinical entities are not mutually exclusive, to the contrary they may coexist. We herein describe a case of Toxic Epidermal Necrolysis, complicated with Hemophagocytic Lymphohistiocytosis with Central Nervous System involvement due to EBV infection.

Dopamine agonists should usually be the first treatment for patients with prolactinomas of all sizes, because these drugs decrease serum prolactin concentrations and decrease the size of most lactotroph adenomas. Cabergoline is preferred first. When cabergoline develops side effects bromokriptin is switched. Cabergoline is more efficient than bromocriptine. Its side effect profile more favorable than bromocriptine. Cabergoline is an ergot dopamine agonist that is administered once or twice a week. Cabergoline can have rarely serious psychiatric adverse effects, including psychosis, impulse control disorders, dyskinesia, pulmonery fibrosis and valvular heart disease. Prolactinomas are the most common pituitary tumors, 93%-95% of the cases are microadenomas. Macroprolactinomas larger than 40 mm, known as giant prolactinomas, are exceptionally rare, accounting for 0.5% - 4% of all prolactin-hypersecreting adenomas. In our case, after the 7 x 6 cm giant macroprolactinoma operated we report the manic episode occuring during the second week of cabergoline treatment. In the treatment of patients with prolactinoma, cabergoline is a first choice drug because it has a better tolerance profile and is more effective, however, bromocriptine can be switched to when drug resistance or side effects develop to cabergoline.

Zoon’s vulvitis or plasma cell vulvitis (PCV) is a rare, benign inflammatory condition of the vulvar mucosa [1]. PCV can present with an asymptomatic lesion, or can cause discomfort, dyspareunia and pruritus [2]. In this way, PCV can mimic other lesions of the vulval mucosa, such as lichen planus.

Lung cancer is a major cause of morbidity and mortality worldwide. Metastasis can be seen in many organs in advanced-stage disease. Acral metastasis rate in cancer is quite low. However, because of the direct opening of the arterial circulation, the risk of acral metastasis stem from lung cancer is higher than any other cancers. Although the mechanism is not known exactly, acral metastases occur in dominant extremities. Here, we present a case with lung adenocarcinoma metastasis of the left hand in the second phalanx. We presented this case which is rarely seen in the literature to emphasize acral metastases.

In the cardiovascular field, tachycardia and increment of catecholamine plasma levels (sympathetic hyperactivity) have been reported by hypertensive patients that use L-type Ca2+ channel blockers (CCBs) since 70´s. Our discovery of the involvement of interaction between the intracellular signalling pathways mediated by Ca2+ and cAMP (Ca2+/cAMP interaction) revealed that this phenomenon (sympathetic hyperactivity) was resulting of increase of transmitter release from sympathetic neurons stimulated by CCBs due to its interference on the Ca2+/cAMP interaction. In the neuroscience field, this discovery has produced new paths for the understanding of the cellular and molecular mechanisms involved in the pathogenesis of neurodegenerative diseases, such as Alzheimer´s and Parkinson’s diseases. In this way, novel journeys for the development of new pharmacological strategies more effective for the treatment of neurodegenerative diseases may be initiated.

Priapism is currently defined as a prolonged painful erection without sexual stimuli, if priapism persists > 36 hours, conservative treatment does not lead to detumescence. The most common etiologies are: medications into corpora cavernosa, anti-psychotics, anti-hypertensives, hematological disorders (chronic myeloid leukemia). The Penile prosthesis is the choice treatment for restoring the erectile function after the failure of the conservative treatment. But the corporal fibrosis makes it very difficult, with high risk of complications [1].

Objective: We evaluated the 30-day outcomes of early (≤ 14 days) or delayed (15 days to three months) carotid revascularization (CR) performed in patients who presented acute brain infarction secondary to internal carotid artery (ICA) stenosis. Methods: We included all patients with a recent cerebral infarction from July 2010 to June 2014 who underwent CR in our center within three months after the onset of symptoms for ICA stenosis. Data were retrospectively collected. Two groups were identifi ed: Group A included patients who underwent early CR within the first fourteen days after symptom onset, and Group B, patients who underwent delayed CR, from the fifteenth day up to the third month after symptom onset. Death, stroke and major adverse cardiac events (MACE) were analyzed. Results: Seventy-one patients underwent CR (73.2% men, with a median age of 71). Nineteen patients underwent early CR and 52 underwent delayed CR. The mean interval from initial examination to surgery was 9.5 days (range, 3-14 days) in Group A and 42 days (range, 15-92 days) in Group B. No complication occurred in Group A within the 30 postoperative days. In Group B, no MACE or death was observed and two patients presented with post-operative stroke (3.8%). Conclusion: This retrospective study confi rmed the satisfactory outcomes of early CR after acute brain infarct. Accurate clinical and radiological selection of patients prevents early neurological complications.

A case study on Jakob Erdheim-Chester disease. Jakob Erdheim, pathologist, collector, scientist and educator was born in 1874 in Galicia and received his medical degree from the University of Vienna in 1900. He became interested in pathology and joined the Pathology Institute of the Municipal Hospital (Lainz) of Vienna

Sexual activity during healthy pregnancy is safe. There are little data on how coital activity affects outcome of the high-risk pregnancies. Hereby we report a case demonstrating that orgasmic coitus triggered placental abruption resulting in preterm stillbirth. A 38-year-old 8-para, 12-gravida woman lived unmarriedly with a constant partner in low socioeconomic conditions. Her previous pregnancies included 7 deliveries without complication, two early miscarriages and two pregnancy terminations. Her present pregnancy was complicated with gestational hypertension successfully treated with nifedipine. She had coitus 2 to 4 times a month, mostly without orgasm. The last coitus which happened in side-by-side position was accompanied by orgasm which continued in uterine hypertonicity and massive vaginal bleeding at 29 weeks gestation. Two hours subsequently, on admission to hospital, placental abruption and fetal demise were diagnosed. At the emergency cesarean section, a dead female infant weighing 1,510 g was born. Fetal pathology was not discovered. Placental histopathology showed retroplacental hematoma, intervillous and decidual hemorrhages, focal distal villous hypoplasia and avascular villuses. Patient’s recovery rapidly occurred after intensive care. Placental abruption complicates 0.4% - 1.0% of deliveries. It is known that most cases of abruption cannot be predicted and prevented. Our report suggests that orgasmic coitus may be a trigger for placental abruption in those women who have gestational hypertension and multiple risks for placental abruption. We infer from the above case that sexual intercourse is advised to avoid during pregnancy of such women in order to prevent placental abruption.

Experience with allogeneic hematopoietic stem cell transplantation (HSCT) in mycosis fungoides/Sezary syndrome (MF/SS) is limited to a small number of case reports and case series [1,2]. The advantage of allogeneic HSCT has been indicated in progressive disease in the review of CIBMTR study groups [3]. A consensus is still not available about the intensity and the content of the conditioning regimen due to the rarity of the disease and heterogeneous patient groups.