Kounis Syndrome or Allergic Coronary Vasospasm in a Two‐year‐old

Kounis syndrome is coronary vasospasm because of mast cell degranulation in the context of an allergic reaction. The syndrome has known associations with several drugs used during anesthesia, including rocuronium and isoflurane. In this case report, we discuss a 2‐year‐old patient who developed signs and symptoms of an acute coronary syndrome soon after anesthesia for atrial septal defect repair. A diagnostic angiography after the episode revealed diffusely small coronary arteries. Subsequent angiography after clinical improvement showed essentially normal coronary anatomy. We report the clinical course of this patient and postulate that Kounis syndrome was the explanation for his transient coronary vasospasm. To date, this is the youngest known patient with reported Kounis syndrome.     

Successful treatment of SAPHO syndrome with severe spinal disorder using entercept: a case study

SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, and osteitis) is a rare disease. Presently, there is no treatment guideline for this illness. Several studies suggested entercept, a novel biological agent against tumor necrosis factor-alpha, is effective in treating SAPHO syndrome. We report a case in which the clinical conditions of a middle-aged female patient diagnosed with SAPHO syndrome, with noted spinal disorder, improved significantly after receiving entercept treatment. The patient remained stable after 3-month follow-up.     

Immuno-allergic accidents with rifamipcin

We report 2 new cases of immuno-allergic side-effects of rifampicin (RMP), occurred in leprosy patients treated by multidrug therapy. These cases illustrate the various features of this type of complication. In one case, the patient exhibited few days after restarting of RMP (600 mg daily), a typical flu syndrome associated to a thrombopenia. Previously, the patient had received discontinued RMP (300 mg, 3/5 days) that had to be stopped after 11 months for "general malaise" that in fact corresponded to a flu syndrome. The second patient developed a flu syndrome associated with a diffuse eczematous eruption one year after the onset of daily RMP (600 mg). Anti-RMP antibodies were detected only in the first case. The pathogenic mechanisms and the clinico-biologic features are discussed.     

单肺移植治疗艾森曼格综合征(附1例报告)

目的总结单肺移植治疗艾森曼格综合征围手术期处理的经验教训。方法对1例52岁的房间隔缺损合并艾森曼格综合征女性患者在全麻体外循环下行同种异体右全肺移植术,同时行房间隔缺损修补术。供体为32岁男性,术前未作HLA配型,供肺保护采用4C改良低钾右旋糖酐液（LPD）灌洗,4CUW液保存。术后给予三联免疫抑制剂。结果术后心肺功能恢复良好。但术后12h发生超急性排斥反应,术后第5天导致急性肝肾功能衰竭,用人工透析维持。第9天出现口腔感染和泌尿系感染,给予口腔护理及氯霉素膀胱冲洗,第12天因全身感染死亡。结论术前供肺保护和手术技巧是单肺移植治疗艾森曼格综合征手术成功的关键;抗排斥和抗感染是手术后处理的重要环节。     

Severe acute hepatitis due to retroviral rebound syndrome after discontinuation of highly active antiretroviral therapy

A case of a 36-year-old female patient who presented a mononucleosis-like syndrome with severe acute hepatitis after discontinuation of antiretroviral therapy. Retroviral rebound syndrome is a relatively novel syndrome possible to occur after the discontinuation of antiretroviral therapy. This is the first case reported in the literature of severe acute hepatitis associated with this syndrome.     

Lethal vascular leak syndrome after denileukin diftitox administration to a patient with cutaneous gamma/delta T-cell lymphoma and occult cirrhosis

Denileukin diftitox (Ontak) is an immunotoxin used in the treatment of cutaneous T-cell lymphomas. Vascular leak syndrome is a known complication of this therapy, although the syndrome is most often self-limited. We report the case of a patient with cutaneous gamma/delta (gammadelta) T-cell lymphoma and previous undiagnosed liver disease treated with denileukin diftitox. Just 4 days after initiating drug therapy, the patient developed profound vascular leak syndrome characterized by a rapid fall in his previously normal serum albumin to levels below the limit of detection. The patient then quickly deteriorated into rhabdomyolysis and eventual death. To our knowledge, this is the first report of a death directly related to denileukin diftitox therapy. The purpose of this case is to increase awareness and improve management of patients who are treated with denileukin diftitox with resulting vascular leak syndrome leading to hypoalbuminemia.     

合并脑静脉窦血栓形成的Evans综合征一例并文献复习

作者回顾性分析1例合并脑静脉窦血栓形成(CVST)的Evans综合征患者的临床资料。患者中年女性,因突发头痛、呕吐入诊首都医科大学宣武医院神经内科,既往Evans综合征病史9个月。患者入院即存在溶血和血小板减少,MR静脉血管成像和DSA示上矢状窦、直窦及右侧乙状窦静脉血栓形成。脑脊液压力增高,眼底示双侧视乳头水肿,确诊为Evans综合征合并CVST。给予抗凝、激素及降颅压等治疗,症状好转。Evans综合征是自身免疫性溶血性贫血和免疫性血小板减少症并发的综合征,可增加发生血栓的风险。当患者出现头痛、呕吐及视乳头水肿等症状时,需警惕合并CVST的可能。Evans综合征合并CVST的患者,需长期抗凝治疗,同时在激素治疗的减量过程中,应警惕Evans综合征的复发。     

Exacerbation of adult-onset Still's disease, possibly related to elevation of serum tumor necrosis factor-alpha after etanercept administration

Adult-onset Still's disease (AOSD) is a systemic inflammatory disease of unknown etiology. A 44-year-old male patient presented with AOSD complicated by macrophage activation syndrome after etanercept therapy. His serum tumor necrosis factor-α (TNF-α) level was increased dramatically after etanercept therapy. The clinical course of this case suggests that the increased TNF-α level by etanercept administration might cause macrophage activation syndrome in this case.     

HYPONATRÆMIA AND RENOVASCULAR HYPERTENSION

A case of traumatic renal artery thrombosis in which the patient developed the hyponatnemic hypertensive syndrome is described. Decreased vascular sensitivity to angiotensin, grossly elevated plasma renin levels, hypertension and hyponatræmia returned to normal after unilateral nephrectomy. Sequential renin and electrolyte studies indicated that this syndrome was caused by the pressor and natriuretic actions of high circulating levels of renin and angiotensin. No evidence could be found to support the suggestion that the high renin levels were secondary to sodium depletion. A positive result to a phentolamine test was recorded, leading to initial problems in diagnosis. The patient is normotensive and well three years after this episode.     

Cantrell综合征的诊断与治疗

目的 探讨Cantrell综合征的诊断、治疗与预后。方法 回顾性分析5例临床诊断为Cantrell综合征患者的临床、影像学、手术及随访材料,总结Cantrell综合征的临床特征。结果 5例患者年龄7 d～76岁。3例有Cantrell综合征的5种畸形,术前准确诊断并经手术证实。1例术后60个月死亡,2例存活（分别随访43个月和4个月）。2例仅有Cantrell综合征的部分畸形（左心室憩室形成）。1例非完全性Cantrell综合征经手术证实,另l例为影像学发现,分别随访75个月和64个月均健在。结论 随着现代影像学技术的发展,Cantrell综合征术前诊断不难,及时治疗可改善预后。     

2型糖尿病患者胰岛β细胞功能衰竭与脂联素和抵抗素的临床相关研究

目的 探讨T2DM患者胰岛β细胞功能衰竭所致血清APN和抵抗素的变化特点及临床意义。 方法 选取T2DM病程〈6个月者21例,6个月2年者18例及健康对照（NC）者21名。测量身高、体重、血压和WC,检测真胰岛素（TI）、胰岛素原（PI）、APN、抵抗素、FPG、FIns、TG、TC、HDL-C和LDL-C,计算胰岛素分泌指数（HOMA-β）。 结果 随着T2DM病程进展,胰岛β细胞功能进行性下降,TI分泌逐渐减少（P〈0.05）。T2DM病程较长组PI高于病程较短及NC组（P〈0.05）。T2DM各组APN、抵抗素、TC、LDL-C和TG与NC组比较差异有统计学意义（P〈0.05）。 结论 随着T2DM病程进展,胰岛β细胞功能逐渐衰竭,且伴随血浆APN和抵抗素水平改变,这可能是导致T2DM发病原因之一。     

Radiographic findings in ovarian hyperstimulation syndrome

The authors report a patient with ovarian hyperstimulation syndrome who had findings on chest radiographic. Bilateral pleural effusions and bibasilar partial atelectasis were the preeminent features. The pleural effusions resulted from accompanying ascites. Ovarian hyperstimulation syndrome occurs in the postovulatory or post-oocyte retrieval phase, 5 to 7 days after administration of human chorionic gonadotropin. Awareness of imaging findings should facilitate the diagnosis of this syndrome.     

Mesenteric vein thrombosis triggered by blunt abdominal trauma in a patient with the primary antiphospholipid syndrome

The antiphospholipid syndrome is defined by the presence of autoimmune antiphospholipid antibodies in serum together with venous, arterial or small-vessel thrombosis and/or morbidity with pregnancy. Superior mesenteric vein thrombosis represents a rare complication associated with this syndrome; triggering events such as surgical procedures, drug administration and anticoagulation withdrawal have been reported. We describe a case of superior mesenteric vein thrombosis triggered by blunt abdominal trauma in a 47-year-old man with the primary antiphospholipid syndrome. It confirms a previous report describing a patient suffering from the catastrophic antiphospholipid syndrome after a fall. This provides evidence, previously unreported, for the possible role of trauma as a precipitating factor leading to thrombosis, even in cases of 'simple' antiphospholipid syndromes. Our patient required extensive small-bowel resection but could be discharged after complete recovery.     

Primary Sj?gren's syndrome preceding the presentation of systemic lupus erythematosus as a benign intracranial hypertension syndrome.

The case is reported of a 41 year old white woman who developed systemic lupus erythematosus (SLE) seven years after primary Sjögren''s syndrome and four years after the association of Sjögren''s syndrome with Jaccoud''s arthritis. The SLE was detected by a benign intracranial hypertension, which is a rare neuro-ophthalmic presentation of lupus. No associated conditions linked to benign intracranial hypertension syndrome were seen in this patient and the hypertension syndrome recurred one year later.     

Blue rubber bled nevus syndrome presenting with recurrences

Summary An unusual patient with blue rubber bleb nevus syndrome is presented. At birth a single abdominal wall hemangioma was noted, and over a 21-year follow-up the full syndrome developed, including many cutaneous and intestinal lesions and anemia. Our long-term follow-up of this patient allows an understanding of the natural history of this condition, especially regarding recurrence of lesions after excision.     

Hemolytic uremic syndrome in a patient with small cell lung cancer

Hemolytic uremic syndrome is a rare entity in patients with carcinoma and presents with a triad of renal insufficiency, microangiopathic hemolytic anemia, and thrombocytopenia. We report this syndrome for the first time in a patient with small cell lung carcinoma. Spontaneous platelet aggregation of washed normal platelets was demonstrated using patient plasma. Circulating immune complex levels were not elevated. The entity completely resolved after treatment with plasma, vincristine, aspirin, and dipyridamole.     

Concurrent long QT and Brugada syndrome in a single patient]

We present a 61-year-old patient with previous cardiac arrest and frequent syncopal spells. ECG showed a typical Brugada pattern and a QTc interval of 425 ms. During programmed ventricular stimulation a self-limited syncopal polymorphic ventricular tachycardia was induced. On diagnosis of the Brugada syndrome an implantable cardioverter defibrillator was implanted. Two days later two episodes of polimorphic ventricular arrhythmia were converted by the device. The ECG at this time showed a prolonged QTc of 500 ms in addition to a typical Brugada pattern. Atenolol was started and after a 36-month follow-up the patient has remained asymptomatic without arrhythmic events. In conclusion, this patient has the Brugada syndrome and also fulfills the clinical and ECG characteristics of the Long QT syndrome. These findings suggest a genetic link between the two syndromes.     

Guillain-Barré and Miller Fisher syndromes occurring with tumor necrosis factor alpha antagonist therapy

OBJECTIVE: Diverse neurologic syndromes have been described in association with tumor necrosis factor alpha (TNFalpha) antagonist therapy for inflammatory arthritides and Crohn's disease. The objective of this study was to review the occurrence and clinical features of Guillain-Barré syndrome and its variant, the Miller Fisher syndrome, during TNFalpha antagonist therapy. METHODS: The postmarketing database of the US Food and Drug Administration (FDA) was searched, following our experience with a patient with rheumatoid arthritis in whom the Miller Fisher syndrome variant of the Guillain-Barré syndrome developed while he was receiving infliximab therapy. RESULTS: Our index patient had a neurologic illness defined initially by ataxia and dysarthria, which fluctuated in relation to each subsequent infliximab infusion and, after 6 months, culminated in areflexic flaccid quadriplegia. In addition, 15 patients in whom Guillain-Barré syndrome developed following TNFalpha antagonist therapy were identified from the FDA database. Guillain-Barré syndrome developed following infliximab therapy in 9 patients, following etanercept therapy in 5 patients, and following adalimumab therapy in 1 patient. Among the 13 patients for whom followup data were available, 1 patient experienced no resolution, 9 patients had partial resolution, and 3 patients had complete resolution of Guillain-Barré syndrome following therapy. CONCLUSION: An association of Guillain-Barré syndrome with TNFalpha antagonist therapy is supported by the worsening of neurologic symptoms that occurred in our index patient following each infusion of infliximab, and by the temporal association of this syndrome with TNFalpha antagonist therapy in 15 other patients. An acute or subacute demyelinating polyneuropathy should be considered a potential adverse effect of TNFalpha antagonist therapy.     

Pathophysiology of auricular flutter and fibrillation in patients with Wolff-Parkinson-White syndrome. A case successfully treated by fulguration of the bundle of His

Atrial flutter and fibrillation are common in patients with Wolff-Parkinson-White syndrome. Pathophysiology of these arrhythmias is poorly understood. Treatment is empirical and oriented to symptomatology. Here we describe a patient with Wolff-Parkinson-White syndrome with paroxysmal reentrant orthodromic AV tachycardias and atrial flutter episodes that disappeared after His Bundle fulguration. This suggests that a complete AV reentry circuit favored the occurrence of atrial flutter in this patient.