VMA Levels Research Articles

Confirmation of pain-related neuromodulation mechanism of Bushen Zhuangjin Decoction on knee osteoarthritis

Ethnopharmacological relevanceBushen Zhuangjin Decoction (BZD) are an herbal compound commonly used to treat osteoarthritis (OA) in China. Aim of the studyThis study aimed to verify the mechanism of Bushen Zhuangjin Decoction in relieving the pain of knee osteoarthritis. Materials and methodsNetwork pharmacology evaluation was used to discover the potential targets of BZD to relieve pain in KOA. The therapeutic effects of BZD treatment on KOA pain using histomorphology, behavioral assessments, suspension chip analysis, and ultra-high performance liquid chromatography/tandem mass spectrometry (UHPLC-MS/MS) assays. The functional magnetic resonance imaging was used to explore the effects of BZD treatment on brain function associated to KOA. ResultsNetwork pharmacological analysis revealed the association between the analgesic effect of BZD on KOA and the pain signaling neurotransmitter 5-HT. Subsequently, we conducted experiments to verify the therapeutic effect of BZD on pain in KOA animal models. Behavioral tests demonstrated that the pain threshold of knee osteoarthritis rats decreased in PWT and PWL, but BZD was able to increase the pain threshold. Histopathological staining indicated thinning of the cartilage layer and sparse trabeculae in the subchondral bone. Suspension chip analysis revealed a significant increase in pro-inflammatory factors of IL-1α, IL-5, IL-12, IL-17A, RANTES, TNF-α and M-CSF in KOA, along with a significant decrease in anti-inflammatory factor of IL-13. However, BZD treatment decreased the expression of pro-inflammatory factors and increased the content of anti-inflammatory factor. UHPLC-MS/MS analysis showed a significant decrease in the serum levels of GABA, E, GSH, Kyn, Met, and VMA in KOA, which were significantly increased by BZD. Conversely, the serum levels of TrpA, TyrA, Spd, and BALa were significantly increased in KOA and significantly decreased by BZD. ELISA and Western blot analysis showed increased expression of subchondral bone pain-related neuropeptides SP, CGRP, TH, NPY, VEGFA, 5-HT3 in KOA, which were decreased in BZD. Functional magnetic resonance imaging demonstrated that BZD exerts its therapeutic effect on KOA by modulating the activity and functional connections of the cortex, hypothalamus, and hippocampus. ConclusionsThis study confirmed the significant role of pain-related neuromodulation mechanisms in the analgesic therapy of BZD and provides a theoretical foundation for using BZD as a traditional Chinese medical treatment for KOA pain.

Changes in blood pressure, blood sugar and creatinine in patients undergoing pheochromocytoma surgery

Objective: To discover the variables that may predict changes in blood pressure and blood sugar levels and to pinpoint the time frame during which such changes occur in patient with pheochromocytoma. Materials and Methodology: Consecutive patients undergoing surgery for pheochromocytoma over a 20-month period were included in this ethics review board-approved, prospective cohort study. Blood pressure and sugar levels were serially monitored using a fixed protocol in the perioperative period and subsequently at 3 months after surgery. Changes were compared and assessed for the predictive factors. Result: Fifty patients undergoing surgical procedures were included in the study of whom 32% developed hypotension and 10% developed hypoglycaemias after surgery. All hypotension episodes occurred within 6 hours of surgery. However, while 7 of 8 patients who developed hypoglycaemia manifest in the first 4 h after surgery, one occurred after 12 h. Occurrence of hypotension correlated with preoperative 24-h urinary vanillylmandelic acid levels (P = 0.024). Out of 21 hypertensive patients, 15 had persistent hypertension at 3 months and this was associated with age (P = 0.04) and diabetes mellitus at presentation. Conclusion: 32% of patients receiving pheochromocytoma surgery experienced postoperative hypotension needing inotropic support. Patients who require greater dosages of alpha-blockers or those with higher 24-h urine VMA levels are more prone to experience this. This happens within the first six hours following surgery. Patients who are older or who have had DM for a long time are more likely to suffer chronic HTN following surgery. Keywords: Pheochromocytoma, hypertension, hypoglycaemia, creatinine.

Neuroblastoma Presentation With Multiple Cranial Nerve Involvement

Objective NA. Background We report a case of neuroblastoma, a pediatric neuroendocrine tumor of the sympathetic nervous system, in a 3-year-old female with multiple cranial nerve involvement. Design/Methods A 3-year-old afebrile, lethargic female presented with bilateral eyelid droop, right head tilt, slurred speech, gagging, abnormal walking and no bowel movement. Neurological examination noted bilateral ptosis, dysarthria, left tongue deviation, proximal weakness in upper and lower extremities, areflexia in biceps and patellar tendons, dysmetria, and wide-based gait. MRI of the brain showed heterogeneous appearance of the clivus and MRI of the spine showed right adrenal mass and heterogeneous enhancement of multiple vertebrae, suggesting possible metastatic disease. Serum and cerebrospinal studies were unremarkable. Patient was treated with intravenous methylprednisolone and plasmapheresis for suspected paraneoplastic syndrome; however, she continued to clinically progress. Adrenal mass biopsy results and elevated urine VMA and HVA levels were consistent with the diagnosis of neuroblastoma. Nuclear imaging and meta-iodobenzylguanidine scan were negative. Paraneoplastic panels and Lambert Eaton panel were negative for autoantibodies. Total resection of the abdominal mass and right adrenal gland with continued steroid taper, resulted in reported near total symptom resolution. Results Our patient presented with antibody negative paraneoplastic polyneuropathy due to neuroblastoma. Cranial nerve involvement in neuroblastoma can result from tumor involvement of the sympathetic chain, paraneoplastic syndromes, or metastasis to the skull. Our patient's initial imaging suggested potential metastatic or inflammatory involvement of the clivus, suspected of causing her cranial nerve symptoms. However, nuclear studies were negative for metastatic disease and patient's symptoms resolved after resection of the main mass suggesting a paraneoplastic etiology. Although her paraneoplastic panel and Lambert-Eaton panel were negative for common associated antibodies, it is likely an unmeasured autoantibody, cytokine, hormones, or peptide was at play. Conclusions Neuroblastoma should be considered as a differential for a neurological presentation involving multiple cranial nerves in a child.

Mixed cortico-medullary adrenal carcinoma in children: looks are deceptive!!

BackgroundMixed cortico-medullary adrenal carcinoma (MCMAC) is an extremely rare entity with scarce literature on its cytomorphology.Case presentationA 2-year-old girl presented with abdominal pain for 3 days and a past history of fever with significant weight loss. On examination, a non-tender left hypochondrial firm mass and an enlarged left supraclavicular node were found. Twenty-four-hour urinary levels of VMA were marginally high. Contrast-enhanced computed tomography of the abdomen showed a suprarenal heterogeneous mass encasing major vessels. Aspiration cytology of both mass and node showed similar features comprising a predominant population of singly scattered large cells with moderate cytoplasm, eccentric nucleus and prominent nucleolus in a necrotic background. Tumour cells expressed Synaptophysin and Melan-A. In view of increasing respiratory distress, debulking surgery was performed, and histopathology of the specimen revealed the presence of both malignant medullary and cortical components supported by immunohistochemistry making a final diagnosis of MCMAC. The patient succumbed to death in the postoperative period. The cytology slides were reviewed and were seen to show a dual cell population.ConclusionCoexistent malignant cortical and medullary tumour of the adrenal gland is the first case reported in the paediatric age group in the literature with only three previous case reports in adults.

Vasoresponsiveness in patients with heart failure (VASOR): protocol for a prospective observational study

BackgroundVasoplegia is a severe complication which may occur after cardiac surgery, particularly in patients with heart failure. It is a result of activation of vasodilator pathways, inactivation of vasoconstrictor pathways and the resistance to vasopressors. However, the precise etiology remains unclear. The aim of the Vasoresponsiveness in patients with heart failure (VASOR) study is to objectify and characterize the altered vasoresponsiveness in patients with heart failure, before, during and after heart failure surgery and to identify the etiological factors involved.MethodsThis is a prospective, observational study conducted at Leiden University Medical Center. Patients with and patients without heart failure undergoing cardiac surgery on cardiopulmonary bypass are enrolled. The study is divided in two inclusion phases. During phase 1, 18 patients with and 18 patients without heart failure are enrolled. The vascular reactivity in response to a vasoconstrictor (phenylephrine) and a vasodilator (nitroglycerin) is assessed in vivo on different timepoints. The response to phenylephrine is assessed on t1 (before induction), t2 (before induction, after start of cardiotropic drugs and/or vasopressors), t3 (after induction), t4 (15 min after cessation of cardiopulmonary bypass) and t5 (1 day post-operatively). The response to nitroglycerin is assessed on t1 and t5. Furthermore, a sample of pre-pericardial fat tissue, containing resistance arteries, is collected intraoperatively. The ex vivo vascular reactivity is assessed by constructing concentrations response curves to various vasoactive substances using isolated resistance arteries. Next, expression of signaling proteins and receptors is assessed using immunohistochemistry and mRNA analysis. Furthermore, the groups are compared with respect to levels of organic compounds that can influence the cardiovascular system (e.g. copeptin, (nor)epinephrine, ANP, BNP, NTproBNP, angiotensin II, cortisol, aldosterone, renin and VMA levels). During inclusion phase 2, only the ex vivo vascular reactivity test is performed in patients with (N = 12) and without heart failure (N = 12).DiscussionUnderstanding the difference in vascular responsiveness between patients with and without heart failure in detail, might yield therapeutic options or development of preventive strategies for vasoplegia, leading to safer surgical interventions and improvement in outcome.Trial registrationThe Netherlands Trial Register (NTR), NTR5647. Registered 26 January 2016.

MP03-19 BLOOD PRESSURE AND SUGAR TRENDS AND QUALITY OF LIFE IN PATIENTS UNDERGOING PHEOCHROMOCYTOMA SURGERY

You have accessJournal of UrologyAdrenal1 Apr 2018MP03-19 BLOOD PRESSURE AND SUGAR TRENDS AND QUALITY OF LIFE IN PATIENTS UNDERGOING PHEOCHROMOCYTOMA SURGERY Pradeep Prakash, Rashmi Ramachandran, Nikhil Tandon, and Rajeev Kumar Pradeep PrakashPradeep Prakash More articles by this author , Rashmi RamachandranRashmi Ramachandran More articles by this author , Nikhil TandonNikhil Tandon More articles by this author , and Rajeev KumarRajeev Kumar More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2018.02.3062AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookTwitterLinked InEmail INTRODUCTION AND OBJECTIVES Hemodynamic, sugar and metabolic fluctuations occur after surgery for pheochromocytoma and some patients may have complete resolution of hypertension (HTN) and diabetes (DM) with changes in body-mass index (BMI), all of which could impact their quality of life (QoL). We evaluated the trends of such changes in a cohort of patients and assessed factor that could predict them. METHODS Consecutive patients undergoing surgery for pheochromocytoma over a 20 months period were included in this ethics review board approved, prospective cohort study. Blood pressure and sugar levels were serially monitored using a fixed protocol in the perioperative period and at 3 months after surgery. BMI and QoL (using WHOQOL-BREF questionnaire) were recorded at baseline and 3 months. Changes were compared and assessed for predictive factors. RESULTS Out of 26 patients, 8 (30%) developed hypotension and 4 (15%) developed hypoglycemia after surgery. Occurrence of hypotension correlated with 24 hour urinary VMA levels (p = 0.02) and total daily dose of prazosin (p = 0.04; table 1). Out of 21 hypertensive patients, 7 (33%) had persistent HTN at 3 months. Persistence was associated with age (p = 0.04) and DM at presentation (p = 0.04; table 2). Among 6 diabetic patients, 1 (16%) had persistent DM. There was significant increase in the BMI (p < 0.0001) and in WHOQOL-BREF scores postoperatively (table 3). CONCLUSIONS Hypotension occurs in 30% patients after pheochromocytoma surgery and is associated with urinary VMA levels and dose of alpha blockers. Older, diabetic patients are more likely to have persistent hypertension. Surgery causes increase in BMI and improvement in QOL. © 2018FiguresReferencesRelatedDetails Volume 199Issue 4SApril 2018Page: e30-e31 Advertisement Copyright & Permissions© 2018MetricsAuthor Information Pradeep Prakash More articles by this author Rashmi Ramachandran More articles by this author Nikhil Tandon More articles by this author Rajeev Kumar More articles by this author Expand All Advertisement Advertisement PDF downloadLoading ...

The role of N-Myc gene amplification in neuroblastoma childhood tumour - single-centre experience.

Aim of the studyNeuroblastoma (NBL) is one of the most common extracranial tumours occurring in children with N-Myc gene amplification, acknowledged as a marker of poor prognosis. We assessed the frequency of N-Myc amplification and its impact on NBL markers and on the treatment outcome.Material and methodsAmong 160 children with NBL treated from 1991 to 2015 in one centre 140 patients had known N-Myc gene status, and they were enrolled in the study. The analysed group was divided into two subgroups: with and without N-Myc amplification (25 and 115 children, respectively). Association of N-Myc amplification with stage of the disease, levels of biochemical parameters, overall survival (OS) and failure-free survival (FFS) were analysed.ResultsThe frequency of N-Myc amplification was 17.9%. Most children with N-Myc amplification (64%) were classified to stage 4 NBL. The levels of biochemical markers of NBL: ferritin, dopamine, NSE, and LDH were significantly higher in the group with N-Myc amplification, whereas the levels of VMA and HVA were lower. OS and FFS were significantly lower in children with N-Myc amplification in comparison to children from the control group (OS 53% vs. 76%, p = 0.03; FFS 50% vs. 72%, p = 0.03). The impact of N-Myc amplification on the treatment outcome was significant in patients with stage 4 NBL and children under one year of age.ConclusionsN-Myc amplification is a crucial prognostic factor in neuroblastoma, which is associated with almost all features related with poor prognosis and a higher probability of unfavourable outcome.

胎児超音波検査にて発見され尿中 VMA・HVA 高値を示した嚢胞状神経芽腫の 1 例

胎児超音波検査にて発見され尿中 VMA・HVA 高値を示した嚢胞状神経芽腫の 1 例

Evaluation of the SIOPEN semi-quantitative scoring system in planar simpatico-adrenal MIBG scintigraphy in children with neuroblastoma.

Neuroblastoma is the most common malignancy in children comprising 7.6% of all infantile cancers. MIBG scintigraphy is amandatory neuroblastoma diagnostic test, which is among others methods, semi-quantified by the SIOPEN method. The aim of this study was to test both the skeletal and the soft tissue segments of the SIOPEN scoring method in the diagnostic milieu and to correlate them with the Curie score. Since there is little knowledge of their diagnostic power, the following variables were tested: VMA, HVA, LDH, and MYCN, ferritin, bone marrow infiltration, the INSS and the INPC classification. The cross-sectional study with repeated measurements of 143 scintigrams was performed on 76 pediatric patients with suspected or proven neuroblastoma, who had been referred to the Center for Nuclear Medicine of the Clinical Center of Serbia in the period 2007-2012. The range of the SIOPEN soft tissue scores was 0-5. The range of the SIOPEN skeletal scores was 0-57. The range of the Curie scores was 0-26. The skeletal SIOPEN scores were significantly higher in bone marrow positive children, in children with pathologically elevated urinary VMA levels and in children having amore advanced clinical stage. There was no difference in the SIOPEN soft tissue score due to higher VMA levels, or depending on the clinical stage and positive bone marrow assessment. There was no difference between the SIOPEN skeletal and soft tissue scores on one hand and the histological grade of the tumor; elevated or normal levels of HVA, LDH, NSE and ferritin, or the presence or absence of MYNC amplification in the neuroblastoma cell line, on the other hand. The results of both SIOPEN scores showed ahigh linear correlation with the Curie score. The conclusion is that the soft tissue segment of the SIOPEN score needs further elucidation in amore controlled milieu. Excellent correlation between all segments of the two semi-quantitative scoring methods speaks in favor of the application of the complete SIOPEN scoring system in every day mIBG scanning.

Pheochromocytoma Is A Rare Tumor; Single Center Experience

Ozet Abstract Amac: Noroektodermal orijinli nadir bir tumor olan Feokromasitomali olgulardaki klinik deneyimlerimizi gozden gecirmektir. Yontem: 2005 ve 2012 yillari arasinda klinigimizde feokromasitoma tanisi konmus 14 hastanin klinik, laboratuar, radyolojik ve cerrahi verileri geriye donuk olarak degerlendirilmistir. Bulgular: Klinigimizde feokromasitoma tanisi ile tedavi goren 14 hastanin 8’i erkek, 6’si kadin olup yaslari 18 ve 60 arasinda degismekte idi. Hastalarin 10 (%71)’unda hipertansiyon mevcuttu. En sik semptom karin agrisi (%57) idi. Yerlesim yerleri acisindan sekizi (%57) solda, besi (%35) sag ve birinde (%7) tumor bilateral adrenal yerlesimliydi. Tanida kullanilan goruntuleme yontemi ultrasonografisi ve bilgisayarli tomografiydi. Hastalarimizin hic birisinde ailesel feokromasitoma sendromu ile iliskili degildi. Butun hipertansif hastalara preoperatif fenoksibenzamin ve propranolol verildi. Cerrahi islem dokuz olguda laparoskopik, besinde ise laparotomi ile gerceklestirildi. Hipertansif hastalarin % 80’inde cerrahi sonrasinda remisyon gozlendi. Iki hastada malign feokromasitoma tespit edildi. Tum hastalarin takibi 24 saatlik idrarda VMA duzeyi ve batin BT ile duzenli olarak yapildi. Takiplerde olgularin birinde karaciger metastazi tespit edildi. Sonuc: Bu calisma ile feokromasitomanin nonspesifik ve degisken bir kliniginin oldugu gosterilmek istendi. Siklikla tumor rastlantisal olarak bulunmaktadir. Feokromasitoma nadir bir tumor olmasina ragmen, dogru bir degerlendirme, iyi bir preoperatif hazirlik ve tam cerrahi eksizyon ile tedavi edilebilir. Objective: The aim of our study is to review our clinical experiences with pheochromocytoma cases which are rare tumors with neuroectodermal origins. Method: The clinical, lab, radiological, and surgical data of a total of 14 patients diagnosed with pheochromocytoma between 2005 and 2012 at our clinic were evaluated retrospectively. Results: Eight out of 14 patients who received treatment at our clinic having been diagnosed with pheochromocytoma were male, while 6 were female and their ages ranged between 18 and 60. 10 (71%) of the patients had hypertension. The most frequent symptom was abdominal pain (57%). Eight tumors were located on the left (57%) and five were located on the right (35%), while one tumor (7%) had bilateral adrenal location. The imaging techniques used in diagnosis were ultrasonography and computerized tomography. None of our patients had a history of familial pheochromocytoma. All the hypertensive patients were administered pre-op phenoxybenzamine and propranolol. The surgical procedures were performed by laparoscopy in nine cases, and by laparotomy in five. Post-op remission was observed in 80% of the hypertensive patients. Malign pheochromocytoma was detected in two patients. The follow-up of all the patients was conducted through testing the VMA level in 24-hour urine and through abdominal CT regularly. The follow-ups revealed that one case had liver metastasis. Conclusion: This study aims at demonstrating that pheochromocytoma has a non-specific and unstable clinic. The tumor is frequently found randomly. Although pheochromocytoma is a rare tumor, it can be treated through a right evaluation, good pre-op preparation, and complete surgical excision.

Development of a new biochemical test to diagnose and monitor neuroblastoma in Vietnam: Homovanillic and vanillylmandelic acid by gas chromatography–mass spectrometry

ObjectivesThe aim of this study was to develop an accurate robust testing method to simultaneously measure urine levels of HVA and VMA using gas chromatography mass spectrometry (GCMS) and to establish age-specific reference intervals of HVA and VMA in random urines for Vietnamese children. Design and methodsThe assay for urinary HVA and VMA was developed based on a classical urinary organic acid profiling method. Briefly, this incorporated 3-phenyl butyric acid as the internal standard and liquid–liquid extraction with ethyl acetate followed by derivatization with BSTFA. The Agilent 7890A GC and 5975C Mass Selective Detector in single ion monitoring mode was used for analysis.Reference intervals were developed from random urine samples collected from 634 disease free Vietnamese children and compared to 50 known neuroblastoma patient samples. Results were reported relative to creatinine concentration. Age related 95% reference intervals for urinary HVA and VMA were estimated from sample quantiles. The analytes (expressed as analyte/creatinine ratios) diagnostic values were determined by calculating the related sensitivity, specificity and likelihood ratios. ResultsHVA and VMA were linear to at least 193 and 221μmol/L, respectively. The limit of quantitation for both analytes was 0.9μmol/L. Using the bi-level control (n=15), the within-batch coefficients of variations (CVs) were less than 3% for both analytes across the assay range. The between-batch CVs (n=20 over three months), were 3.6% at 11μmol/L and 2.1% at 88μmol/L for HVA, 6.6% at 18.2μmol/L and 2.6% at 90.6μmol/L for VMA.Vietnamese age related reference intervals were established for urinary HVA and VMA per creatinine. HVA for children <6months (n=91) was 5.3–37.0μmol/mmol; 6months to <1year (n=141) was 2.7–27.7μmol/mmol; 1 to 5years (n=139) was 3.4–17.9μmol/mmol; 6 to 10years (n=136) was 2.7–8.8μmol/mmol; and 11 to 15years (n=127) was 1.1–9.4μmol/mmol. VMA for children <6months was 1.8–12.2μmol/mmol; 6months to <1year was 1.5–9.3μmol/mmol; 1 to 5years was 1.9–7.8μmol/mmol; 6 to 10years was 1.6–5.1μmol/mmol; and 11 to 15years was <0.9–6.3μmol/mmol. ConclusionsA robust testing method for simultaneous quantitation of urinary HVA and VMA by GCMS was developed. This method is accurate, precise and fit for its clinical purpose and suitable for developing countries. Age-related reference intervals of urinary HVA and VMA were established for Vietnamese children and the intervals declined progressively with increasing age for each analyte.

颈部异位嗜铬细胞瘤一例报告

颈部异位嗜铬细胞瘤十分罕见。作者报道了一例57岁的男性患者，因“发现左颈部肿块6年，头痛眩晕、胸闷胸痛9天”入院。入院时血压大幅度波动，每隔数分钟波动在200/100 mmHg~80/50 mmHg的范围。左颈部肿块进行性增大5年余。血浆17-羟皮质类固醇和17-酮类固醇正常，24小时尿VMA升高(82.7 umol/day (。颈部的CT平扫和增强显示左侧颈部一个4.6 × 3.2 cm大小的肿块。充分的术前准备后行左颈部肿块切除术。手术顺利，无术后并发症。病理和免疫组化显示为左侧颈部异位嗜铬细胞瘤。 Ectopic pheochromocytoma in neck is rare. We reported a 57-year-old male patient who was admitted to hospital because of a mass in left neck for 6 years with headache, dizziness, chest tightness and chest pain for 9 days. He had an alternation of fluctuation of blood pressure between 200/100 - 80/50 mmHg in every several minutes. The mass progressively enlarges for more than 5 years in his left side of neck. His plasma 17-hydroxy corticosteroid and 17-ketosteroid levels were normal. The 24 hours urine VMA level was high (82.7 umol/day (

Pheochromocytoma associated with neurofibromatosis type 1: concepts and current trends

BackgroundNeurofibromatosis Type 1(NF-1) has autosomal dominant inheritance with complete penetrance, variable expression and a high rate of new mutation. Pheochromocytoma occurs in 0.1%-5.7% of patients with NF-1.Case presentationWe present the case of a 37-year-old patient with laparoscopically resected pheochromocytoma. He was investigated for hypertension, flushing and ectopic heart beat. Abdominal CT and MRI revealed a mass measuring 8 × 4 cm in the right adrenal gland. The diagnosis of pheochromocytoma was confirmed by elevated 24-hour urine levels of VMA, metanephrines and catecholamines as well as positive MIBG scan. The patient presented with classic clinical features of NF-1, which was confirmed by pathologic evaluation of an excised skin nodule. The patient underwent laparoscopic right adrenalectomy through a transabdominal approach and was discharged on the second postoperative day, being normotensive. The patient is normotensive without antihypertensive therapy 11 years after the procedure.ConclusionNowadays in the era of laparoscopy, patients with pheochromocytoma reach the operating theatre easier than in the past. Despite, the feasibility and oncological efficacy of the laparoscopic approach to the adrenals, continued long term follow-up is needed to establish the minimally invasive technique as the preferred approach. Furthermore, these patients should be further investigated for other neoplasias and stigmata of other neurocutaneous syndromes, taking into account the association of the familial pheochromo-cytoma with other familial basis inherited diseases.

A prospective study of a long‐term follow‐up of an observation program for neuroblastoma detected by mass screening

A nationwide mass screening for neuroblastoma (NBL) in 6-month-old infants (MS6M) was performed in Japan from 1985 to 2003. Favorable biological features were identified for most of the detected tumors; consequently, we began an observation program for selected screened patients in 1993. Here, we report the clinicopathological findings and present status of patients enrolled in our observation program, with the goal of evaluating its usefulness. Between 1993 and 2003, 53 of 101 patients with NBL detected by MS6M were enrolled. The patients were divided into four groups according to changes in urinary VMA and HVA levels and tumor size. Urinary VMA and HVA levels decreased in 39 of 53 patients. In 17 of these 39 patients, the tumor became undetectable (Group A); in 22 patients the tumor was detectable (Group B). In seven patients, tumor marker levels varied, and tumor volume gradually increased (Group C). In six patients, tumor marker levels and tumor volume increased in the short term (Group D). One patient had multiple tumors (1M according to International Neuroblastoma Staging System). All tumors in Groups C and D, four tumors in Group B, and one tumor in the 1M patient were removed. No unfavorable biologic factors were noted in any excised tumor. The observation program of the present study, one of the largest series for MS6M, confirmed that over 70% of patients who fulfilled the criteria could be observed without surgery.

Laparoscopic adrenalectomy: A single center experience

AIMS:To evaluate the efficacy and safety of laparoscopic adrenalectomy in benign adrenal disorders.METHODS AND MATERIAL:Since July 2007, twenty patients have undergone laparoscopic adrenalectomy for various benign adrenal disorders at our institution. Every patient underwent contrast enhanced CT-abdomen. Serum corticosteroid levels were conducted in all, and urinary metanephrines, normetanephrines and VMA levels were performed in suspected pheochromocytoma. All the patients underwent laparoscopic adrenalectomy via the transperitoneal approach.RESULTS:The patients were in the age range of 18-57 years, eleven males and nine females, seven right, eleven left, two bilateral. The mean operative time was 150 minutes (120-180), mean hospital stay four days (3-5), mean intraoperative blood loss 150 ml and mean post-operative analgesic need was for 36 (24-72) hours. One out of twenty-two laparoscopic operations had to be converted into open adrenalectomy due to intra-operative complications.CONCLUSIONS:Laparoscopic adrenalectomy is a safe, effective and useful procedure without any major post-operative complication and is the gold standard for all benign adrenal disorders.

Pheochromocytoma in children and adolescents

Eleven subjects aged <20 yr with histologically proven pheochromocytoma between 1987 and 2006 were analyzed. Family history was present in 18%. In 2 patients, pheochromocytoma was part of VHL and in one it was associated with MEN 2. Twenty four hour urine VMA level was elevated in 100% and metanephrine level in 73%. CT/ MRI were showing the tumor in all. Prazosin extended release tablets (maximum 30 mg/day) were used in 73% and doxazosin (maximum 12 mg/ day) in 27%. Intraoperative BP fluctuations were seen in 27%. All were biochemically cured after surgery. Preoperative á blockade with extended release prazosin and doxazosin were effective in controlling perioperative BP fluctuations. Hence these drugs can be used in children and adolescents without fear of postoperative hypotension.

Phaeochromocytoma comb?ned with subclinical Cushing's syndrome and pituitary microadenoma

Phaeochromocytoma (PHEO) occasionally associates with pathological lesions of the adrenal cortex. The coexistence of PHEO and pre-clinical Cushing's syndrome (PCS) of the same adrenal gland has rarely been reported. We report a case of PHEO and PCS originating from the same adrenal gland and discuss the peculiar diagnostic aspects of this entity. A 64 yr old man was hospitalized to evaluate the right adrenal mass which was discovered incidentally by ultrasonography. He had a history of type 2 diabetes mellitus and hyperlipidemia. Blood pressure measurements were all normal during his hospital stay. Laboratory examination showed: urinary catecholamines were markedly increased. HbA1C of 14.3 %, midnight cortisol of 11(microg/dL), cortisol was not suppressed after the overnight 1 mg oral dexamethasone suppression test (DST): 3.42(microg/dL), 24 hr free cortisol in the urine : 213 microg/day (10-100), cortisol levels were suppressed more than 50% with 8 mg of dexamethasone. CT scan of the adrenal glands showed a 6 cm well encapsulated right adrenal mass together with a clearly normal left adrenal gland. MRI investigation of the sella turcica revealed a pituitary microadenoma on the right side of the adenohypophysis He was treated with alpha and subsequent beta blockers after the diagnosis of PHEO and PCS was made. Right adrenalectomy was performed. The pathology showed typical PHEO with adrenocortical hyperplasia. VMA, metanefrin and free cortisol levels were normalized one month after surgery. The present report is a rare case of PHEO combined with PCS in the same adrenal gland.

Neuroblastoma;Variable Symptoms of A Neurogenic Tumor; A Report From Iran

Objective to current data available, neuroblastoma is the most frequent extracranial solid tumor in infants and children; because of its relationship to the primitive sympathetic ganglia, it may progress or regress spontaneously to more malignant or benign forms of tumors, respectively. It is also an important cause of the Opsoclonus Myoclonus Ataxia Syndrome (OMAS), or the “dancing eyes” syndrome. The risk factor of neuroblastoma includes patient’s age at diagnosis; degree of tumor spread, and selected biologic variables such as serum LDH, urinary and serum catecholamines such as VMA and HVA, ploidy and MYCN copy numbers. So, detection of risk factors and risk directed therapy are the mainstay of patient management. Materials & Methods For this study the records of 43 out of 46 patients, aged less than 14 years, admitted over 8 years (1996-2004), with the confirmed diagnosis of neuroblastoma or ganglioneuroblastoma were evaluated for full course of therapy and follow up. Results patient group, 60% were male and 40% female. The most frequent clinical stage was stage 3 (34.7%), followed with stage 4 (32.6%) and 2 (26%). Less than 2% of patients presented with pure neurologic symptoms and these responded well to treatment. Relapse was seen more frequently in stage 4 patients and less in stage 4S. The OPEC protocol, started in 81% of patients, achieved a 54% response; on the other hand, in 15%, N6 was used, with the response rate of 40%. Patient data was analyzed and interpreted using SPSS software to reveal which clinical and biologic factors improve neuroblastoma outcome. Conclusion and patients’ age at the time of diagnosis are the most important clinical factors to predict outcome, while primary tumor site and some biologic findings such as urinary VMA and serum LDH levels have a less important value

Effectiveness of green tea on weight reduction in obese Thais: A randomized, controlled trial

This study was undertaken to investigate the effects of green tea on weight reduction in obese Thais. A randomized, controlled trial involving 60 obese subjects (body mass index, BMI > 25kg/m 2) was conducted. All subjects consumed a Thai diet containing 3 meals (8373.6kJ/day) for 12weeks, prepared by the Nutritional Unit at Srinagarind Hospital. The diet contained 65% carbohydrates, 15% protein, and 20% fat. Body weight, BMI, body composition, resting energy expenditure, and substrate oxidation were measured at baseline, and during weeks 4, 8, and 12 of the study. Serum levels of leptin and urine VMA were measured at baseline and during the 12th week. Differences over time and between the treatments (green tea or placebo) over time were determined using two-factor ANOVA with repeated measures. In comparing the two groups, differences in weight loss were 2.70, 5.10, and 3.3kg during the 4th, 8th, and 12th weeks of the study, respectively. At the 8th and 12th weeks of the study, body weight loss was significantly different ( P < 0.05). At the 8th week, the difference in resting energy expenditure was 183.38kJ/day ( P < 0.001), the difference in the respiratory quotient was 0.02 ( P < 0.05), and no significant differences existed in satiety score, food intake, or physical activity. Urine VMA was significantly different in the 12th week of the study ( P < 0.05). We conclude that green tea can reduce body weight in obese Thai subjects by increasing energy expenditure and fat oxidation.

Fronto-limbic functioning in children and adolescents with and without autism

We used neuropsychological tasks to investigate integrity of brain circuits linking orbitofrontal cortex and amygdala (orbitofrontal–amygdala), and dorsolateral prefrontal cortex and hippocampus (dorsolateral prefrontal–hippocampus), in 138 individuals aged 7–18 years, with and without autism. We predicted that performance on orbitofrontal–amygdala tasks would be poorer in the Autism group compared to the Non-Autism group regardless of intellectual level (verbal mental age, VMA) and that performance on dorsolateral prefrontal–hippocampus tasks would be associated primarily with intellectual level. Predicted differences between Autism and Non-Autism groups on orbitofrontal–amygdala tasks were present but greater in individuals with higher VMA. On dorsolateral prefrontal–hippocampus tasks, poorer performance by the Autism compared to the Non-Autism group was found at all VMA levels. Group differences suggest both brain circuits are impaired in autism, but performance on all tasks is also associated with intellectual level.