Intra-arterial vasodilators infusion for management of reversible cerebral vasoconstriction syndrome in a 12-year-old girl: A case report.

Reversible cerebral vasoconstriction syndrome (RCVS) is a vascular disease characterized by diffuse transient vasoconstriction and vasodilatation of the cerebral arteries. It is commonly associated with recurrent severe acute headaches with or without focal neurological deficits due to hemorrhages, infarcts, and even posterior reversible encephalopathy syndrome. The optimal management of acute neurologic deficits caused by RCVS is still uncertain. Calcium channel blockers (CCBs) such as nimodipine or verapamil have been reported to be effective in adult series. Intra-arterial injection of nimodipine, verapamil, and milrinone has recently been demonstrated to be safe and effective for treating severe segmental vasoconstriction in adults. CCBs are the most used treatment in the available pediatric literature. Intra-arterial vasodilators have been reported in some rare pediatric reports with more severe diseases, but their utility is still under investigation. We report a case of a 12-year-old girl who underwent a severe course of RCVS complicated by multiple cerebral infarcts, treated by several sessions of intra-arterial vasodilators infusion

Comparison of Pheochromocytoma-Specific Morbidity and Mortality among Adults with Bilateral Pheochromocytomas Undergoing Total Adrenalectomy vs Cortical-Sparing Adrenalectomy

Importance: Large studies investigating long-term outcomes of patients with bilateral pheochromocytomas treated with either total or cortical-sparing adrenalectomies are needed to inform clinical management. Objective: To determine the association of total vs cortical-sparing adrenalectomy with pheochromocytoma-specific mortality, the burden of primary adrenal insufficiency after bilateral adrenalectomy, and the risk of pheochromocytoma recurrence. Design, Setting, and Participants: This cohort study used data from a multicenter consortium-based registry for 625 patients treated for bilateral pheochromocytomas between 1950 and 2018. Data were analyzed from September 1, 2018, to June 1, 2019. Exposures: Total or cortical-sparing adrenalectomy. Main Outcomes and Measures: Primary adrenal insufficiency, recurrent pheochromocytoma, and mortality. Results: Of 625 patients (300 [48%] female) with a median (interquartile range [IQR]) age of 30 (22-40) years at diagnosis, 401 (64%) were diagnosed with synchronous bilateral pheochromocytomas and 224 (36%) were diagnosed with metachronous pheochromocytomas (median [IQR] interval to second adrenalectomy, 6 [1-13] years). In 505 of 526 tested patients (96%), germline mutations were detected in the genes RET (282 patients [54%]), VHL (184 patients [35%]), and other genes (39 patients [7%]). Of 849 adrenalectomies performed in 625 patients, 324 (52%) were planned as cortical sparing and were successful in 248 of 324 patients (76.5%). Primary adrenal insufficiency occurred in all patients treated with total adrenalectomy but only in 23.5% of patients treated with attempted cortical-sparing adrenalectomy. A third of patients with adrenal insufficiency developed complications, such as adrenal crisis or iatrogenic Cushing syndrome. Of 377 patients who became steroid dependent, 67 (18%) developed at least 1 adrenal crisis and 50 (13%) developed iatrogenic Cushing syndrome during median (IQR) follow-up of 8 (3-25) years. Two patients developed recurrent pheochromocytoma in the adrenal bed despite total adrenalectomy. In contrast, 33 patients (13%) treated with successful cortical-sparing adrenalectomy developed another pheochromocytoma within the remnant adrenal after a median (IQR) of 8 (4-13) years, all of which were successfully treated with another surgery. Cortical-sparing surgery was not associated with survival. Overall survival was associated with comorbidities unrelated to pheochromocytoma: of 63 patients who died, only 3 (5%) died of metastatic pheochromocytoma. Conclusions and Relevance: Patients undergoing cortical-sparing adrenalectomy did not demonstrate decreased survival, despite development of recurrent pheochromocytoma in 13%. Cortical-sparing adrenalectomy should be considered in all patients with hereditary pheochromocytoma

Utilizing the prognostic impact of minimal residual disease in treatment decisions for pediatric acute lymphoblastic leukemia.

Acute lymphoblastic leukemia (ALL) is the first pediatric cancer where the assessment of early response to therapy by minimal residual disease (MRD) monitoring has demonstrated its importance to improve risk-based treatment approaches. The most standardized tools to study MRD in ALL are multiparametric flow cytometry and realtime-quantitative polymerase chain reaction amplification-based methods. In recent years, MRD measurement has reached greater levels of sensitivity and standardization through international laboratory networks collaboration. We herewith describe how to assess and apply the prognostic impact of MRD in treatment decisions, with specific focus on pediatric ALL. We also highlight the role of MRD monitoring in the context of genetically homogeneous subgroups of pediatric ALL. However, some queries remain to be addressed and emerging technologies hold the promise of improving MRD detection in ALL patients. Emerging technologies, like next generation flow cytometry, droplet digital PCR, and next generation sequencing appear to be important methods for assessing MRD in pediatric ALL. These more specific and/or sensitive MRD monitoring methods may help to predict relapse with greater accuracy, and are currently being used in clinical trials to improve pediatric ALL outcome by optimizing patient stratification and earlier MRD-based interventional therapy

Caracterização e controle das doenças de maçãs em pós-colheita.

Controle das podridões de pós-colheita em maçãs com produtos biológicos As podridões são responsáveis por grande parte das perdas de maçãs durante o período de pós-colheita. Estas doenças são causadas por um grupo de patógenos conhecidos como característicos de pós-colheita e incluem principalmente Alternaria, Botrytis cinerea e Penicillium, e por outro que é associado às doenças de verão. Os organismos que causam estas doenças são C. gloeosporioides e C. acutatum (podridão amarga), Botryosphaeria dothidea (podridão branca) e Cryptosporiopsis perennans (olho-de-boi) e manchas das maçãs conhecidas como fuligem e sujeira-de-mosca (VALDEBENITO SANHUEZA et.al., 2002)

A rare case of ileo-ileal intussusception due to a bleeding lipomatous mass treated by laparoscopic ileal resection

INTRODUCTION: Adult intussusception is a rare cause of bowel obstruction with atypical presentation. It can be associated with primary or secondary intestinal tumors and, rarely, with lipomatous masses. CASE REPORT: We report the case of a 69-year old man presenting with a history of gastrointestinal bleeding and anemia. Upper and lower endoscopies were negative for bleeding. On abdominal contrast enhanced computerized tomography (CT) scan, a trans-omental hernia in the right lower abdominal quadrant was diagnosed with no active bleeding or evidence of tumor. On exploratory laparoscopy we detected an ileo-ileal intussusception caused by a submucosal mass in the distal ileum, which was reduced and we then performed a segmental resection of the involved small bowel tract. The patient fully recovered by postoperative day 3 when he was discharged home. Final pathology confirmed an ileal lipoma. CONCLUSION: Ileal intussusception caused by lipoma is a rare condition, which can be diagnosed with endoscopy, barium enema, and abdominal ultrasound or CT scanning, but preoperative diagnosis may be difficult. The treatment of choice is the reduction of the intussusception and the resection (laparoscopic or open) of the involved tract

Herniation of cerebellar tonsils in acromegaly: prevalence, pathogenesis and clinical impact.

Herniation of cerebellar tonsils (CTH) might occur in acromegaly patients and improve after acromegaly treatment. Our study investigated CTH prevalence in acromegaly, its relationship with clinical, laboratory and neuroimaging findings and its possible pathogenesis and clinical impact. 150 acromegaly patients (median-age 56 years, age-range 21-88, 83 females) underwent brain magnetic resonance imaging (MRI). Clinical data, laboratory and pituitary adenoma imaging findings were recorded. CTH, posterior cranial fossa area, tentorial angle, clivus, supraocciput and Twining's line length were measured in acromegaly patients and controls, who included MRI of 115 consecutive subjects with headache or transient neurological deficits (control group-1) and 24 symptomatic classic Chiari 1 malformation patients (control group-2). Acromegaly patients were interviewed for symptoms known to be related with CTH. 22/150 acromegaly patients (15 %) and 8/115 control group-1 subjects presented with CTH (p = 0.04). In acromegaly patients, CTH correlated positively with younger age and inversely with GH-receptor antagonist treatment. Control group-2 had a shorter clivus than CTH acromegaly patients (40.4 \ub1 3.2 mm vs 42.5 \ub1 3.3 mm, p < 0.05), while posterior fossa measures did not differ among acromegaly subgroups (with and without CTH) and control group-1. Headache and vision problems were more frequent in CTH acromegaly patients (p < 0.05); two acromegaly patients presented with imaging and neurological signs of syringomyelia. Despite no signs of posterior fossa underdevelopment or cranial constriction, CTH is more frequent in acromegaly patients and seems to contribute to some disabling neurological symptoms