伴有泪腺炎的淋巴细胞性垂体炎

淋巴细胞性垂体炎是一种少见的自身免疫性疾病，本例的临床症状包括眼睑肿胀，垂体前叶功能低下和中枢性尿崩症，MRI显示双侧眼睑肿胀，双泪腺增大，各眼外肌未见异常，垂体柄明显增粗，垂体后叶高信号消失，垂体前叶未见异常。泪腺活检显示淋巴细胞浸润。经大剂量甲基强的松龙冲击治疗后，垂体病变和眼睑肿胀均在短期内显著减轻，临床诊断为伴有泪腺炎的淋巴细胞性垂体炎。     

A case of lymphocytic panhypophysitis (LPH) during pregnancy

A 37-year-old pregnant woman developed continuous headache in the 10th week of pregnancy, followed by bilateral visual field defect and general malaise in the 24th week. The brain magnetic resonance imaging showed a pituitary mass. In laboratory examination, plasma concentration of free thyroxine, thyroid stimulating hormone (TSH), cortisol, and adrenocorticotropic hormone (ACTH) was low. General malaise vanished shortly after the replacement therapy of glucocorticoid and thyroid hormone, but partial central diabetes insipidus (CDI) appeared, which could be treated with desmopressin acetate (DDAVP). The visual field defect having enlarged, transsphenoidal surgery was performed in the 31st week of pregnancy. Adenohypophysis could be resected, and it showed infiltration of mature lymphocytes. After the surgery, the visual defect had improved, but hormone replacement was still necessary. She delivered a baby in the 38th week without any trouble. Provocative tests after delivery revealed a low response in TSH, prolactin (PRL), and follicle stimulating hormone (FSH). Hormone replacement and DDAVP administration was necessary in the same doses after delivery. The diagnosis was lymphocytic panhypophysitis (LPH). In the case of pregnant woman, LPH should be included in the differential diagnosis of pituitary mass for the fetomaternal safety.     

Lymphocytic Hypophysitis Masking a Suprasellar Germinoma in a 12-year-old Girl<197>A Case Report

Case history, light and electron microscopic findings of a case of a lymphocytic hypophysitis in coincidence with a suprasellar germinoma in a 12-year-old girl are reported. The girl presented with a long time case history of diabetes insipidus and subsequent panhypopituitarism. Two years after the diagnosis of diabetes insipidus magnetic resonance imaging (MRI) showed a tumorous enlargement of the sellar content and pituitary stalk. A transnasal exploration was initially performed and revealed a lymphocytic hypophysitis. Light microscopy showed a dense infiltration of mature lymphocytes and plasma cells in the interstitium of the anterior pituitary gland. The stalk area could not be exposed to exclude a germinoma. One year later the lesion relapsed despite dexamethason therapy and a second operation by another neurosurgeon had to be performed. Light microscopy showed lymphocytic infiltrates, fibrosis and necrosis. The diagnosis was a lymphocytic hypophysitis again. Though transcranially exposed only pituitary tissue was removed. No infundibular mass became visible at surgery as shown by MRI. The girl developed five months later multiple cerebral lesions, which revealed to be a germinoma. Lymphocytic hypophysitis in children is very rare and a coincedence with a germinoma has not been described from histopathological aspect until now. The origin of the pituitary infiltration is discussed.     

Lymphocytic hypophysitis: a rare cause of hypoglycaemia in a man with type 2 diabetes mellitus

Lymphocytic hypophysitis is a primary inflammatory disorder of the pituitary gland, which may cause panhypopituitarism. The majority of cases occur in women during the peripartum period and it is rare in men. We present a case, initially presenting with recurrent hypoglycaemic episodes, in a man with previously well-controlled type 2 diabetes mellitus.     

经面静脉入路栓塞治疗海绵窦区硬脑膜动静脉瘘

目的探讨经面静脉途径治疗海绵窦区硬脑膜动静脉瘘（arteriovenous fistula，AVF）的方法和疗效。方法回顾性分析经面静脉途径（导管置入股静脉-面静脉-角静脉-眼上静脉-海绵窦）治疗7例海绵窦区硬脑膜AVF的病例资料，其中5例患者岩下窦闭塞；1例患者岩下窦开放，但其AVF所在的海绵窦和岩下窦之间无交通；1例患者的瘘与岩下窦相通。对该患者先行经岩下窦栓塞海绵窦后部，然后再经面静脉途径栓塞海绵窦前部。其他6例患者只经面静脉途径行栓塞治疗。结果血管造影显示，7例患者的角静脉和眼上静脉的走行清晰，其中有1例眼上静脉闭塞的患者，由于导管无法置入闭塞的眼上静脉，导致经静脉途径治疗终止。其余6例患者经面静脉入路用弹簧圈栓塞海绵窦治疗后，4例达到完全栓塞，2例临床症状明显好转。结论相对其他静脉途径而言，经股静脉-面静脉入路是血管内治疗海绵窦区硬脑膜AVF的一种很有价值的方法，即使该入路对术者手术技能要求较高，但仍然不失为一种安全和有效的血管内治疗方法。     

经静脉入路采用Onyx或联合弹簧圈治疗海绵窦区硬脑膜动静脉瘘的疗效

目的探讨经静脉入路采用Onyx或联合可脱性弹簧圈栓塞治疗海绵窦区硬脑膜动静脉瘘的安全性和有效性。方法回顾性分析2007年2月—2010年12月,经静脉入路栓塞治疗的12例海绵窦区硬脑膜动静脉瘘患者的临床资料,其中经岩下窦入路10例,经面静脉-眼上静脉入路2例。单独应用Onyx 8例,Onyx联合可脱性弹簧圈4例。结果①治疗后即刻血管造影显示完全闭塞10例,少量残留2例。单独应用Onyx栓塞治愈6例,好转2例,Onyx联合可脱性弹簧圈栓塞治愈2例,好转2例;总有效率100%,治愈率66.7%,无加重或死亡病例。②2例术中出现心动过缓,3例术后出现眶部疼痛。③对所有患者进行临床随访,随访时间为6～32个月,无复发及加重者。结论静脉入路Onyx或联合可脱性弹簧圈栓塞海绵窦区硬脑膜动静脉瘘的疗效确实,安全可靠。     

Five years follow-up of invasive prolactinomas with special reference to the control of cavernous sinus invasion

Background Few data are presently available on the effective control of cavernous sinus (CS) invasion of invasive prolactinomas. The aim of this retrospective study, through a mean period of 5 years follow up, is to observe the tumor shrinkage of CS invasive prolactinomas, as well as PRL normalization with bromocriptine therapy. Methods 68 patients met the criteria of invasive prolactinomas (Grade III or IV in the classification scheme of Knosp and colleagues; serum PRL level greater than 200 ng/ml). 33 patients underwent bromocriptine therapy as the initial treatment, and 14 of these 33 had combined treatment with microsurgery and/or radiotherapy. The other 35 patients received microsurgery as the primary treatment, after which two patients had normal PRL without taking bromocriptine and other 33 patients received bromocriptine treatment after microsurgery. Results Tumor volume on magnetic resonance images had completely disappeared in 50 patients (74%), while all the other 18 patients had residual tumor in the parasellar areas, invading the CS, and 14 patients had a secondary empty sella due to tumor shrinkage. Of those 14 patients, seven still had elevated PRL levels; five had optic chiasmal herniation by different degrees (P < 0.05). There were 49 patients with normal PRL levels (72%); five patients with PRL levels more than 200 ng/ml. After the treatment, 14 patients with tumor volume disappearance on MR images and PRL normalization therefore withdrew from bromocriptine therapy. During a subsequent one-and-a-half-year follow-up, tumor recurrence and PRL increase were not found in those 14 patients. Twenty-seven patients maintained normal PRL levels with low-dose bromocriptine, of which 20 patients had their tumor disappear while seven patients had CS residual tumor. Conclusions About three-fourths of prolactinomas with CS invasion can be effectively controlled not only with regard to tumor volume disappearance but also in serum PRL normalization. Residual tumor in the CS areas with PRL normalization and no pressure symptoms can be treated with low-dose of bromocriptine so as to achieve long-term tumor volume control and endocrine control. Great attention should be paid to CS residual tumors accompanying the empty sella, especially in cases with optic chiasmal herniation.     

淋巴细胞性垂体炎三例并文献复习

目的提高临床医师对淋巴细胞性垂体炎的认识。方法分析3例淋巴细胞性垂体炎的临床特点和病理结果。结果3例患者均为青年女性,例1产后起病,例2和例3在非妊娠或产后期间发病;影像学特点:病变广泛,不仅局限于鞍区和垂体柄,并向下丘脑扩展呈“舌状”改变侵犯海绵窦;增强后病变均匀或环状强化;主要临床表现:(1)垂体功能受损和尿崩症;(2)有局部受压症状,如头痛,视交叉受压;(3)病变侵犯海绵窦,引起动眼、外展神经麻痹;2例经病理证实为淋巴细胞性垂体炎,垂体组织见大量淋巴细胞、浆细胞、组织细胞浸润;2例大剂量甲泼尼龙治疗后,症状明显好转。结论淋巴细胞性垂体炎亦可发生在非妊娠或产后期间的年轻女性,大剂量甲泼尼龙冲击治疗能有效缩小病变和改善垂体功能。     

Combined CNS and pituitary involvement as a primary manifestation of Wegener granulomatosis

Wegener granulomatosis (WG) is a systemic vasculitis of small and medium vessels. It predominantly affects the upper and/or lower respiratory airway and kidneys. Its pathogenesis is not fully understood. WG relatively frequently affects the nervous system (in 30–50% according to the different studies). Most frequently, it manifests as necrotizing vasculitis that leads to the peripheral neuropathies or to the cranial nerves palsy. Impairment of the central nervous system (CNS) is less frequent and occurs in 2–8% of patients. Three major pathogenetic mechanisms were described: CNS vasculitis, spreading of granulomas from the adjacent anatomical areas (paranasal cavities, orbit etc.), and new formation of granulomas in brain tissue. This case report describes patients in whom WG manifested in the form of localized skin involvement and combined CNS involvement that included pituitary gland. Atypical presentation of WG impedes and slows down the process of diagnosis and emphasizes the need for collaboration between medical specialists.     

Pilonidal sinus involving the breast in a man: A case report and literature review

Rationale:Pilonidal sinus disease (PSD) involving the breast is extremely rare and has not been described in man.Patient concerns:This current case report presents a case of a pilonidal cyst in a 46-year-old man which was surgically treated. He had intermittent pain in his left breast for 2 months and came for local rupture and discharge for 1 week.Diagnosis:The initial diagnosis is male mastitis, on the basis of the histological features of H&E-stained specimens and immunohistochemistry of the resected lump, this case was diagnosed as PSD.Interventions:The patient underwent “enlarged resection of the left breast lesion” under local anesthesia.Outcomes:The patient''s surgical area healed well, without any signs of recurrence.Conclusion:PSD involving the breast is extremely rare in man, with no typically clinical manifestations, and could be easily ignored. This disease requires great attentions from clinicians.     

颅内外高流量搭桥治疗海绵窦内动脉瘤

目的 探讨利用大隐静脉进行颅内外搭桥，治疗海绵窦内动脉瘤的疗效，并对手术指征及手术方法进行讨论。方法 利用大隐静脉进行颅内外搭桥手术治疗海绵窦内动脉瘤13例。手术中首先准备大隐静脉20～30cm，并用等渗盐水加肝素充盈。根据手术前血管造影显示的侧支循环及手术中试验性夹闭颈内动脉时的脑电图监测情况，选用颈部颈内动脉或颈外动脉，利用大隐静脉与大脑中动脉搭桥。手术后随访3～63个月，平均23个月。结果 除1例患者因术后基底核血肿造成一侧肢体轻度偏瘫外，其余12例患者均恢复良好，眼球后疼痛及三叉神经痛均消失，眼球运动功能完全恢复，也无新的脑出血或脑缺血发生。结论 在严格选择病例的前提下，利用大隐静脉行颅内外高流量搭桥，仍然为治疗海绵窦内动脉瘤的有效方法。     

Infiltrative cardiac lymphoma with tricuspid valve involvement in a young man

Cardiac metastases are among the topics with limited systematic reviews.Theoretically,the heart can be infiltrated by any malignancy with the ability to spread to distant structures.Thus far,no specific tumors are known to have a predilection for the heart,but some do metastasize more often than others,for example,melanoma and primary mediastinal tumors.We report a case of cardiac metastasis from a diffuse large B cell lymphoma in a young man.The peculiarity of this case is that besides the involvement of right ventricle and atrium,the tricuspid valve was also infiltrated.Valvular metastasis is rarely reported in the medical literature.     

淋巴细胞性胃炎的内镜和病理特点及随访分析

目的 :探讨淋巴细胞性胃炎 (LCG)的内镜、病理特点 ,以及根除幽门螺杆菌 (Hp)后对其的影响。方法 :分析 50例LCG的电子胃镜表现和病理所见 ,应用分级法评价淋巴细胞的浸润程度 ,应用PPI三联疗法根除Hp。结果 :LCG多见于中年男性 ,Hp感染率为84%。内镜特点 :90 %以上的病变位于胃窦和胃体的下段 ,特征性表现为多发性或弥漫性隆起样糜烂或脐样糜烂 ,共占 70 % ,可并发十二指肠球部病变 ,占 40 %。病理特点 :全部组织均有淋巴细胞浸润 ,淋巴滤泡形成占 58%。Hp根除后 ,41 .7%患者的胃镜表现胃黏膜大致正常 ;33 .3 %患者表现为黏膜稍充血水肿和花斑样改变 (P <0 .0 5) ;2 5%患者的糜烂灶明显减少 ,主要为散在的平坦性糜烂 (P <0 .0 5) ,而隆起样糜烂消失 ,淋巴滤泡完全消失 ;66.7%患者无淋巴细胞浸润 ,33 .3 %患者为 1级改变。根除Hp后 ,淋巴细胞分级为 0 .33± 0 .49级 (P <0 .0 0 1 )。结论 :LCG有一定的内镜和病理特点。根除Hp后 ,可明显减少淋巴细胞的浸润 ,且可治愈LCG。     

经动脉入路Onyx栓塞海绵窦区硬脑膜动静脉瘘

目的经动脉入路以Onyx栓寨海绵窦区硬脑膜动静脉瘘（CSDAVF）的疗效观察。方法同顾性分析2006年5月-2010年5月在第二军医大学长海医院神经外科经动脉入路栓塞治疗的CSDAVF患者10例,其中女8例,粥2例,Borden分型I型2例,Ⅱ型8例？均在全身麻醉下以Onyx-18栓塞治疗。结果①10例患者共进行11次介入治疗,8次术中即刻被完全栓塞,3次被部分栓塞,其中1例第2次治疗时被完全栓塞,2例被部分栓塞的患者随访造影显示完全栓寒：共经14支供血动脉栓塞,其中咽升动脉5次,脑膜中动脉3j欠,脑膜副动脉5次,眼动脉脑膜返动脉1次。平均注胶时间为32．9min（10～63min）,注胶量为1．8ml（0．8～3．1ml）：②平均随访27个月（4～51个月）。患者症状均有好转：被完全栓塞的患者术后即刻颐内杂昔消失,球结膜充血、眼球突出在术后1周内消失,复视和视力下降症状在术后1～3个月内好转;被部分栓塞的3例患者术后即刻杂音减轻,3个月后完全消失,眼部症状在3个月后完全消失。⑧1例BordenⅡ型患哲在被部分柃塞后3．5年发生颅内出血,第2次术后随访7个月,未见再出血和眼部症状;2例术中Onyx弥散至颈内动脉系统,1例出现永久性脑神经麻痹。结论采用Onyx经动咏入路栓塞可以有效地治疗CSDAVF,但应注意避免危险吻合栓塞带来的并发症。     

Glu^47缺失造成的常染色体显性遗传垂体性尿崩症的家系分析

目的寻找常染色体显性遗传垂体性尿崩症（ADNDI）的基因突变位点。方法在一临床确诊的ADNDI家系，提取外周血的基因组DNA，PCR扩增精氨酸加压素前体基因（AVP－NPⅡ），并进行全长测序，亚克隆测序确认突变位点。结果本家系中所有患者在AVP－NPⅡ基因2号外显子的1824－1829位AGGAGG存在一个AGG密码子的缺失，而其他表型正常的成员测序结果正常。结论AVP－NPⅡ基因1824-1829位AGGAGG中的一个AGG的缺失导致NPⅡ第47位谷氨酸（Glu）的缺失，可能影响NPⅡ蛋白对AVP的运输，进一步造成AVP降解增加，发生尿崩症。     

一个先天性肾性尿崩症家系精氨酸血管升压素受体2基因的突变检测

采集临床诊断为X-连锁肾性尿崩症的一家系3例患者及其12名亲属的血液样本，抽提基因组DNA，通过PCR扩增精氨酸血管升压素2型受体（arginine vasopressin receptor 2，AVPR2）基因的全部编码区，并直接测序。在3例患者中发现AVPR2基因的突变：g1236T→C（L292P），他们的母亲在该位点均为杂合突变。     

Glu~(47)缺失造成的常染色体显性遗传垂体性尿崩症的家系分析

目的寻找常染色体显性遗传垂体性尿崩症(ADNDI)的基因突变位点。方法在一临床确诊的ADNDI家系,提取外周血的基因组DNA,PCR扩增精氨酸加压素前体基因(AVP-NPⅡ),并进行全长测序,亚克隆测序确认突变位点。结果本家系中所有患者在AVP-NPⅡ基因2号外显子的1824-1829位AGGAGG存在一个AGG密码子的缺失,而其他表型正常的成员测序结果正常。结论AVP-NPⅡ基因1824-1829位AGGAGG中的一个AGG的缺失导致NPⅡ第47位谷氨酸(Glu)的缺失,可能影响NPⅡ蛋白对AVP的运输,进一步造成AVP降解增加,发生尿崩症。     

一例持续高钠血症伴渴感减退患者的临床研究

采用禁水和水负荷试验对一例高钠血症伴渴感减退患者进行临床研究,观察血钠、血尿渗透压等变化。显示其仍有调节ADH释放机制,但其阈值增高,符合原发性高钠血症。口服双氢克尿噻和醋酸去氨加压素可部分改善上述异常。