Temporal approach for resection of juvenile nasopharyngeal angiofibromas

OBJECTIVE: To describe a lateral preauricular temporal approach for resection of juvenile nasopharyngeal angiofibroma (JNA). STUDY DESIGN: A retrospective review of five patients with JNA tumors that were resected by a lateral preauricular temporal approach. METHODS: The medical records of five patients who underwent resection of JNA tumors via a lateral preauricular temporal approach were reviewed, and the following data collected: tumor extent, blood loss, hospital stay, and surgical complications. RESULTS: Five patients with JNA tumors had resection by a lateral preauricular temporal approach. These tumors ranged from relatively limited disease to more extensive intracranial, extradural tumors. Using the staging system advocated by Andrews et al., these tumors included stages II, IIIa, and IIIb. Four patients (stages II, IIIa, IIIa, and IIIb) who underwent primary surgical excision had minimal blood losses and were discharged on the first or third postoperative day with minimal transient complications (mild trismus, frontal branch paresis, serous effusion, and cheek hypesthesia). The remaining patient (stage IIIb) did well after surgery, despite having undergone preoperative radiation therapy and sustaining a significant intraoperative blood loss. There have been no permanent complications or tumor recurrences. CONCLUSIONS: A lateral preauricular temporal approach to the nasopharynx and infratemporal fossa provides effective exposure for resection of extradural JNA tumors. The advantages of this approach include a straightforward route to the site of origin, the absence of facial and palatal incisions, and avoidance of a permanent ipsilateral conductive hearing loss.     

Mast cells and T-lymphocytes in juvenile angiofibromas

Juvenile angiofibroma (JA) is regarded as a benign fibrovascular tumour of unknown aetiology. Due to its fibrovascular architecture the fibrous and vascular tumour component have been in the focus of most studies. This investigation aimed at characterizing inflammatory cells in JAs by immunohistochemical stainings and western blot analysis. Number and type of mast cells as well as T-lymphocytes were evaluated in a series of 10 JAs and 5 nasal mucosa (NM) specimens used as control tissue. A remarkable number of mast cells were found in JAs (14.6% of all cells). By using a combination of the mast cell markers tryptase and chymase three distinct mast cell populations could be identified: 12% expressed tryptase (T+) only, 3% stained for chymase (C+) only, and 85% were positive for both tryptase and chymase (TC+). Western blot analysis supported finding of remarkable expression of the mast cell markers tryptase and chymase in JAs and indicated for both proteins similar but also different molecular weights than being observed in NM. Furthermore an infiltration of the tumour by CD4- and CD8-positive T-lymphocytes (15.4% of all cells) was evident in immunofluorescent stainings. Compared to NM, a significantly higher number of TC+ (6.9% in JAs versus 2.7% in NM) and CD8-postive (9.7% in JAs versus 5.8% in NM) cells were found in the tumour tissue. Thus, mast cells and T-lymphocytes were identified as predominant cell types in JAs representing 30% of the cells in the tumour specimens analysed. Regarding these observations JAs are certainly not only built up by vascular cells and fibrous stroma cells. High rates of inflammatory cells like mast cells and T-lymphocytes have to be considered in this tumour.     

Elektronenmikroskopische Untersuchungen zur Zytogenese des juvenilen Nasenrachenfibroms

Summary After the electron microscopic examination of tissue samples of juvenile nasopharyngeal angiofibromas obtained from 9 male patients ranging in their age from 7–24 years the problems of cytogenesis and classification are discussed. Besides it is tried to correlate particular morphological findings to certain clinical phenomenons.The vascular component of juvenile nasopharyngeal angiofibromas shows a clear proliferation of the vascular wall cells. Particularly, proliferating pericytes, cells withous peculiar characteristics (undifferentiated cells) and cells in various stages of differentiation are to be emphasized. Obviously, vascular wall cells emigrate into the surrounding tissue and transform themselves into small fibroblasts. The second component of juvenile nasopharyngeal angiofibromas is represented by stromal fibroblasts with several cytological variations. Only activated classical fibroblasts and fibroblasts with histiocyte-like features reveal the nuclear pattern unique for these growths which is characterized by the combination of protrusions of nuclear membrane with formation of nuclear blebs and of dense intranuclear granules. Cells with these nuclear characteristics were considered as preexisting fibroblasts.Thus juvenile nasopharyngeal angiofibromas are formed by the proliferation of two tissue components, namely by the proliferation of vascular wall cells and stromal fibroblasts, and can be conceived as reactive hyperplasias. The swelling body-like and organoid appearance, cytological pecularities, characteristic topographic relations (localization and supplying vessels) and the sex-dependent occurrence speak for a tumor-like hyperplasia of a rudimentary organ unknown till now.

     

C-PEPTIDE IN JUVENILE DIABETICS BEYOND THE POSTINITIAL REMISSION PERIOD Relation to Clinical Manifestations at Onset of Diabetes, Remission and Diabetic Control

Abstract Ludvigsson, J., Heeling, L. G., Larsson, Y. and Leander, E. (Department of Paediatrics, Linköping University, Sweden, Novo Research Institute, Copenhagen, Denmark, Department of Mathematics, Linköping University, Sweden). C-peptide in juvenile diabetics beyond the postinitial remission period.—Relation to clinical manifestations at onset of diabetes, remission and diabetic control. Acta Paediatr Scand, 66:177, 1977.—A group of 58 diabetics, age 6–17 years and with a duration of diabetes of 3–14 years was studied in order to show whether the nature of the clinical manifestations and the treatment at the onset of the disease are related to the subsequent C-peptide production and also whether remaining C-peptide production is related to better diabetic control. The relations between a number of clinical and laboratory variables were analysed including the degree of ketosis and the insulin dose given at onset of diabetes, the incidence of postinitial remission period, the fasting C-peptide level after the remission period, the level of insulin antibodies and the actual diabetic control expressed as the degree of glucosuria in the patients' urine tests at home. Multiple regression analysis was the main method used. Postinitial remission was positively correlated to initial insulin dose and negatively correlated to duration of ketonuria at onset. C-peptide, which was found in 24.1% of the patients was positively correlated to age at onset and initial insulin dose, but negatively correlated to ketonuria at onset. Diabetic control was positively correlated to insulin dose at onset and to C-peptide level, but negatively correlated to insulin antibodies. It could further be shown that patients who had received a more vigorous treatment immediately at onset had both a higher incidence of postinitial remission and a better diabetic control. The results suggest that an early diagnosis followed by rapid normalization of the metabolism at the onset of juvenile diabetes increase the possibility of preservation of some of the endogenous insulin production, which seems to facilitate diabetic control.     

A randomized,placebo‐controlled,double‐masked clinical trial of etanercept for the treatment of uveitis associated with juvenile idiopathic arthritis

Objective

To investigate the safety and efficacy of etanercept in the treatment of uveitis associated with juvenile idiopathic arthritis (JIA).

Methods

Children who met the American College of Rheumatology diagnostic criteria for JIA with active uveitis, who had anterior chamber cells of ≥1+ or requiring topical corticosteroid ≥3 times daily, and who were on a stable regimen for arthritis treatment were eligible. Study participants received etanercept (0.4 mg/kg) or placebo administered subcutaneously twice weekly for 6 months. All participants received open‐label etanercept for an additional 6 months.

Results

Five patients received placebo and 7 received etanercept. Three of the 7 patients treated with etanercept and 2 of the 5 placebo‐treated patients were considered ophthalmic successes (P = 1.0). One patient in each treatment group was considered a treatment failure. Three of the 7 etanercept‐treated and 2 of the 5 placebo‐treated patients were neither successes nor failures by our definition. There were no serious adverse events for any patient during the entire study period. Reports of minor infections were comparable in each treatment group, 71% for etanercept and 60% for placebo (P = 0.58).

Conclusion

In this small pilot study there was no apparent difference in the anterior segment inflammation between patients treated with etanercept and placebo. The stringent criteria used to measure ophthalmic success of treatment and the small patient population limit the implications of our findings.

     

浙南地区11~14岁儿童血红蛋白参考范围的建立

目的建立浙南地区11~14岁少年儿童血红蛋白参考范围。方法使用Sysmex全自动血液分析仪检测4502例11~14岁健康体检儿童静脉血血红蛋白,根据平均红细胞体积<80fl或者运铁蛋白饱和度<0.15或者红细胞锌原卟啉>4.0μg/g血红蛋白(Hb)剔除可能为铁缺乏症的儿童487名,分性别组统计4015名儿童的血红蛋白参考范围。结果健康男性儿童的血红蛋白均数为137.46g/L,正常参考范围为122.56~152.36g/L;女性儿童的血红蛋白均数为134.81g/L,正常参考范围为122.41~147.31g/L,通过u检验比较,两者之间有显著性差异(P<0.01)。结论调查符合美国第二次全国卫生与营养调查要求,制定与少年儿童生理发育阶段特征符合本地区血红蛋白参考值,得到的参考范围与使用传统手工法测定的参考范围有所不同,尤其是女性儿童血红蛋白参考范围下限有所提高,为本地区少年儿童早期诊断贫血提供重要的实验室依据。     

注射用依那西普联合骨痹汤治疗幼年脊柱关节病的临床观察

目的观察注射用依那西普联合骨痹汤治疗幼年脊柱关节病(JSp A)的临床疗效。方法将77例JSp A患者随机分为2组,治疗组40例应用注射用依那西普联合骨痹汤治疗,对照组37例单纯应用柳氮磺吡啶肠溶片治疗。2组均治疗12周,观察2组治疗前、治疗2周后及治疗12周后红细胞沉降率(ESR)、C反应蛋白(CRP)、血小板计数(PLT)及视觉模拟评分(VAS)变化,比较2组疗效。结果 2组治疗过程中ESR、CRP、PLT及VAS评分均逐步降低,治疗2周后比较差异无统计学意义(P0.05),但治疗12周后,2组均较本组治疗前降低(P0.05),且治疗组各项观察指标较对照组降低更明显(P0.05)。治疗组缓解率55.0%、总有效率97.5%,对照组缓解率27.0%、总有效率81.1%,2组缓解率、总有效率比较差异均有统计学意义(P0.05),治疗组疗效优于对照组。结论注射用依那西普联合骨痹汤治疗JSp A,疗效显著,安全性好。     

Practical Management Issues for Idiopathic Generalized Epilepsies

Summary:  The idiopathic generalized epilepsies (IGE) are a group of epilepsies that are genetically determined, have no structural or anatomic cause, and usually begin early in life. Neurologic examination, intelligence, and imaging studies are normal, and EEG shows only epileptiform abnormalities (i.e., no abnormal slow activity or evidence for diffuse encephalopathy). In some IGE, the genetic substrate has been identified, whereas in most, it remains unknown. Depending on the age at onset and predominant seizure type, individual subtypes of IGE (syndromes) are defined. However, overall, there are more similarities than there are differences among the various subtypes, and the IGE are best viewed as a spectrum or continuum of conditions. In general, IGE respond to treatment, with 80–90% becoming fully controlled. However, not all antiepileptic drugs (AED) are equally effective in IGE. Some AED are ill advised because they either do not work or exacerbate seizure types other than generalized tonic–clonic (GTC) seizures, that is, absence and myoclonic seizures. These include carbamazepine, oxcarbazepine, phenytoin, gabapentin, and tiagabine. Their use is the main cause of "pseudo-intractability," and at least in the United States where PHT and CBZ are the most commonly used AEDs, patients with IGE are often on inadequate medications. For patients with clear IGE, the drug of choice is generally valproic acid because it effectively controls absence myoclonic seizures and GTC seizures. Second-line drugs (when first-line drugs fail or are not tolerated) may include benzodiazepines, but the use of second-line drugs is evolving rapidly. Some of the newer AEDs are considered broad spectrum, meaning that they work in IGE and focal epilepsies, although the evidence is largely preliminary at this point. These newer AEDs include lamotrigine, topiramate, levetiracetam, and zonisamide.     

Juvenile Myoclonic Epilepsy: Factors of Error Involved in the Diagnosis and Treatment

Juvenile myoclonic epilepsy (JME), a common form of idiopathic generalized epilepsy, has a distinct clinical and electroencephalographic profile. Often JME is not recognized, with serious consequences on the sufferers. We examined factors contributing to the missed diagnosis even in an epilepsy clinic. Of 70 JME patients, 66 (91.4%) were not diagnosed on referral and 22 (33%) were not initially recognized in the epilepsy clinic. The correct diagnosis was established after a mean of 8.3 +/- 5.5 years from disease onset and an interval of 17.7 +/- 10.4 months from first evaluation in the epilepsy clinic. Myoclonic jerks, the hallmark of the disease, were not usually reported by patients. Similarly, relevant questioning may not be included in the history. Absence seizures antedating jerks by many years, myoclonic jerks reported as unilateral, generalized tonic-clonic seizures occurring during sleep and focal EEG abnormalities are other factors contributing to not recognizing JME. Our study reemphasizes the need to have not only a correct seizure diagnosis but also a correct epilepsy-disease diagnosis.