Ehlers-Danlos syndrome – vascular type (ecchymotic variant): cutaneous and dermatopathologic features

Ehlers-Danlos syndrome – vascular type, the only lethal form, is rarely reported in dermatology literature. It is characterized by translucent, atrophic skin, easy bruising, arterial, intestinal and/or uterine fragility manifesting as varicose veins, aneurysms and vascular/visceral/uterine rupture. As its dermatopathologic features are not well elucidated, diagnosis is often made after a catastrophic complication or at autopsy. This 36 year-old non-consanguineous male had brown-black plaques with atrophy and frequent ulceration over legs and dorsal feet and tortuous varicose veins around ankles for the past 15 years. Perivenous skin was translucent and hypopigmented. He had multiple and ecchymotic keloids and small atrophic, pityriasis versicolor-like lesions over trunk. He did not have hypermobile/hyperextensible skin and joints and showed no systemic or investigative abnormality. Histopathologic features of atrophic lesion included blood extravasation in atrophic epidermis/dermis, focal clustering and dilatation of blood vessels, malformed vessel walls, abundant hemosiderin in the dermis and homogenously stained/whorled patterned collagen especially around blood vessels. Pathology of keloidal lesion showed new collagen and vascular fragility. These histopathologic features appear of diagnostic value especially in patients who have compatible clinical findings but cannot afford confirmation by biochemical testing for abnormal synthesis of type III procollagen or identification of mutations in the COL3A1 gene.     

Practical management of patients with non-small-cell lung cancer treated with gefitinib.

PURPOSE: The use of gefitinib, the first drug approved to inhibit the epidermal growth factor receptor tyrosine kinase, is indicated in patients with non-small-cell lung cancer with tumors progressive after chemotherapy. The unique mechanism of action of this agent leads to distinctive patterns of response and toxicity in persons with lung cancer. Many of the principles of management relevant to gefitinib are distinct from those with conventional cytotoxic drugs. To meet this need, we present practical guidelines on the use of gefitinib in patients with non-small-cell lung cancer. METHODS: This article reviews gefitinib's indications, dosing, response phenomena, and patterns of relapse in individuals with radiographic response. RESULTS: We present our recommendations for the management of rash and diarrhea caused by this agent. CONCLUSION: This information can guide practitioners and help them inform their patients about what to expect when they receive gefitinib.     

Intracranial Angioplasty and Stenting in the Awake Patient

BACKGROUND AND PURPOSE: Endovascular treatment for intracranial atherosclerosis is evolving, but complications remain an issue. Most interventions are performed under general anesthesia, preventing intraprocedural clinical evaluations. We describe our approach to intracranial angioplasty and stenting, using local rather than general anesthesia, and intraprocedural neurological assessment. METHODS: We prospectively collected procedural and outcome information on all patients undergoing intracranial angioplasty and stenting. Patients underwent interventions under local anesthesia with mild intravenous sedation or analgesia only if needed. Intraoperative neurological evaluations were performed, and symptomatology was used to guide the interventional technique. RESULTS: Forty-eight arteries in 40 patients with a mean age of 65.2 years were treated. Thirty-two anterior and 16 posterior circulation segments were treated. Technical success was achieved in 100% of patients with reduction of the mean pretreatment stenosis from 85 +/- 8.6% to 7 +/- 10.1%. Stents were deployed in 40 segments; five patients were treated with drug-eluting stents. The cobalt-chromium coronary stents were the easiest to deliver. Thirty-seven patients were treated under local anesthesia and, of those, 61.4% experienced intraprocedural symptoms that led to some alteration of the interventional technique. Headache was the most common symptom, and, when persistent, it heralded the occurrence of subarachnoid hemorrhage. There were seven total neurological complications, but only five (10.5%) led to permanent morbidity (4 strokes) or mortality (1 death). CONCLUSIONS: Intracranial angioplasty and stenting can be successfully performed using coronary techniques and equipment including drug-eluting stents. Local anesthesia permits neurological evaluations and often leads to the adjustment of the interventional technique, potentially making the procedure safer.     

Medical management of chronic pancreatitis

慢性胰腺炎的临床表现包括疼痛、脂肪泻和糖尿病。在西方国家，慢性胰腺炎最常见的病因是酗酒。70％以上的病人在就诊时有疼痛的临床表现，而且，这些患者中又有75％以上会在几年之后出现疼痛减轻或完全消失。对于所有的慢性胰腺炎的病人来说，均应排除非胰源性疼痛和胆道梗阻、胰腺假性囊肿等胰腺局部并发症。应建议所有慢性胰腺炎病人戒烟、戒酒。阿片类镇痛剂仅应用于治疗疼痛严重的病人。尽管有报道认为胰酶替代治疗有助于止痛，但是，对于已经确诊的慢性胰腺炎病人来说，该疗法无效。激素类药物进行腹腔神经丛阻滞术可能有助于病人度过剧烈疼痛期。顽固性疼痛是进行胰液引流或胰腺切除的适应证。建议应用适量胰酶替代联合(或不联合)制酸剂治疗营养不良。慢性胰腺炎导致的糖尿病与原发性糖尿病的治疗原则相似。     

Pancreas-sparing duodenectomy for trauma.

The application of pancreas sparing duodenectomy (PSD) in extensive duodenal trauma has not been fully explored. We report 3 caes of duodenal trauma in whom PSD was performed successfully and with good results.