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1.
We report a case of simultaneous involvement of the spleen and the left kidney in a marginal zone lymphoma with a monotypic lymphoplasmacytic cell component, which transformed into a diffuse large B-cell lymphoma of the immunoblastic type. PCR showed that the small and large B-cell populations carried the same type of immunoglobulin heavy chain gene rearrangement. This type of rearrangement was detected in the spleen, the latero-aortic lymphadenopathy and the kidney demonstrating that it is the same lymphoma that affected both organs and the lymph nodes. Primary renal lymphoma is very rare and only a few cases of renal marginal zone lymphoma, MALT type, have been reported. Involvement of simultaneous multiple sites has been described in MALT type lymphoma, but splenic involvement secondary to renal MALT lymphoma seems to have never been observed. Nevertheless, in our case the huge size of the spleen associated with splenic hilar node involvement is consistent with primary splenic marginal zone lymphoma. The extension into latero-aortic lymph nodes of this lymphoma can explain secondary kidney involvement. The nodal Kaposi’s sarcoma observed in this patient of Mediterranean origin was probably coincidental.  相似文献   

2.
Background Laparoscopic adrenalectomy is considered the gold standard for the surgical treatment of adrenal disorders in most centers. This study analyzes the authors’ experience using the lateral intraperitoneal approach with the first 100 patients. In addition to analyzing the authors’ experience, this article aims to contrast it with some published series as an internal quality control. Methods In a 10-year period, 138 laparoscopic adrenalectomies were performed for 100 patients. Demographics, surgical results, complications, and long-term outcomes were analyzed. Results The participants comprised 69 women and 31 men with a mean age of 37 years. The procedures included 24 right, 38 left, and 38 bilateral adrenalectomies. The indications for surgery were Cushing’s disease for 33 patients, pheochromocytoma (4 bilateral) for 23 patients, Cushing’s syndrome for 18 patients, Conn’s syndrome for 16 patients, and incidentaloma for 10 patients. Five procedures were converted to open surgery. Two patients with pheochromocytoma required intraoperative blood transfusion. The mean operative time was 174 min for unilateral adrenalectomies and 302 min for the bilateral procedures. The mean hospital stay was 5 days. Surgical morbidity included an abdominal wall hematoma, a small pneumothorax, and intraabominal bleeding in one patient that required reexploration. There were three operative mortalities not related to the technique. The long-term results showed control of hypercortisolism in all the patients with Cushing’s disease and 82% of the patients with pheochromocytoma. Most of the patients with Conn’s syndrome (91.4%) became normotensive after surgery. Conclusions Laparoscopic adrenalectomy is safe and effective. The complications are mild, and mortality is related more to the patient’s condition than to the surgical technique.  相似文献   

3.
Renal involvement is a common finding in non-Hodgkins lymphoma (NHL). Acute renal failure at initial presentation due to lymphomatous infiltration of the kidneys has been described infrequently. We report a 17-year-old male who presented with acute renal failure due to massive lymphomatous infiltration of the kidneys, which necessitated hemodialysis. The diagnosis of B-cell NHL was established by tru-cut biopsy of the kidneys and the patient had an excellent response to high-dose chemotherapy with no major complication. The presence of extrarenal involvement in the testes and the retroperitoneal lymph nodes made the diagnosis of primary renal lymphoma debatable. However, considering the delay in diagnosis and the high proliferative rate of B-cell NHL, we might postulate that the disease had originated primarily in the kidneys. We recommend that in NHL cases with severe renal involvement, full-dose chemotherapy should be instituted with meticulous clinical and laboratory follow-up in order to improve clinical and renal failure status rapidly and to avoid further dissemination of NHL.  相似文献   

4.
A 48-year-old woman who had bilateral swelling in the eyelids and submandibular region was admitted. Clinical findings suggested that her renal function had deteriorated. Laboratory data showed renal insufficiency (2.52 mg/dl), hypergammaglobulinemia (IgG 3,729 mg/dl, IgA 124 mg/dl, IgM 73 mg/dl). Gallium-67 scintigram indicated abnormal uptake in bilateral lacrimal glands, submandibular glands, and kidneys. A diagnosis of Mikulicz’s disease and interstitial nephritis was made, since biopsy specimens of her lacrimal gland and minor salivary gland showed diffuse infiltration of lymphocytes. In addition, renal biopsy specimens showed diffuse severe interstitial infiltration of IgG4-positive mononuclear cells. Symptoms and laboratory data normalized in response to methylprednisolone semi-pulse therapy and prednisolone 50 mg/day. Mikulicz’s disease was recently reported to be IgG4 associated disease. In our case, Mikulicz’s disease complicated with diffuse severe interstitial nephritis was successfully treated by corticosteroid. The present case supports the hypothesis that IgG4-related autoimmune disease could be causes of Mikulicz’s disease and interstitial nephritis.  相似文献   

5.
Freiberg’s disease usually affects the second and, less commonly, the third metatarsal heads [Helal and Gibb in Foot Ankle 8(2):94–102, 1987]. It was mainly observed unilaterally in adolescent females (F/M: 3/1–4/1) (Hay and Smith in The Foot 2:176–178, 1992). Bilateral occurrence and adjacent metatarsal involvement has been previously reported separately [Maresca et al. in Arthroscopy 12(1):103–108, 1996; Rafee et al. in Foot Ankle Surg 12:153–155, 2006]. The cases presented here involve two female patients aged 42 and 72 with bilateral Freiberg’s disease in both the second and third metatarsal heads. To our knowledge, this is the first report of such involvement of both the second and third metatarsal heads.  相似文献   

6.
We report three autopsy cases of patients with renal symptoms related to intravascular malignant lymphomatosis (IML) exclusively involving both kidneys. The patients were one woman and two men, aged 64, 65, and 82 years, respectively. Their presenting symptoms included fever, anemia, thrombocytopenia, icterus, dyspnea, weight loss, and enlarged adrenal glands. All patients presented with acute renal dysfunction, with creatinine ranging from 2.0 to 6.5 mg/dl, or with modest proteinuria. One patient had an underlying bilateral disease, chronic pyelonephritis. Another patient also had an autoimmune disease. None of the three patients were aggressively treated. All died of progressive disease 2 weeks to 2 months after the onset of their renal symptoms. The diagnosis of the lymphomas was confirmed by morphological and immunohistochemical studies of the postmortem material. The lymphomas were subclassified as large-cell, characterized by angiotropism. Immunophenotyping revealed that all were B-lineage neoplasms. Renal involvement by IML was characterized histologically by a unique pattern of multifocal neoplastic disseminations within the glomerular and peritubular capillaries, with relative sparing of the interstitum. In one patient, glomerular mesangiolysis in association with embolic proliferations of lymphoma cells was a striking feature. All patients had bilateral renal involvement by IML which occurred in a setting of widespread disease. Our studies indicate that IML is an unusual subset of secondary renal lymphomas with bilateral diseases, and, rarely, may produce renal symptomatology early in the course. Received: June 10, 1998 / Accepted; December 24, 1998  相似文献   

7.
A 76-year-old man underwent endovascular stent-grafting therapy for chronic aortic dissection. The preoperative multi detector row helical computed tomography (CT) revealed that Adamkiewicz’s artery originated from the level of thoracic 9 intercostal artery. Intraoperative monitoring of motor evoked potentials showed no reduction of amplitude in the anterior tibialis muscle after deployment retrievable stent-graft. Postoperative CT showed that stent-graft blocked the ostium of intercostal arteries including Adamkiewicz’s artery. On the other hand, the distal portion of the Adamkiewicz’s artery was clearly enhanced. Our result showed that there is a collateral network supplying to Adamkiewicz’s artery. This study is partially supported by a grant from Japan Foundation of Cardiovascular Research.  相似文献   

8.
Background Most patients requiring bilateral adrenalectomy have adrenocorticotropin hormone (ACTH)-dependent Cushing’s syndrome. Some of these patients are severely debilitated from the chronic effects of cortisol overproduction. This study aimed to analyze the indications, safety, efficacy, and outcomes for laparoscopic bilateral adrenalectomy from the authors’ experience. Methods A retrospective review was conducted at a university tertiary referral center. Between March 1996 and August 2006, 30 consecutive patients underwent simultaneous laparoscopic bilateral adrenalectomy. The patient records were analyzed to obtain patient demographics, disease etiology, surgical approach, operating room information, postoperative complications (30 days), hospital length of stay (LOS), and follow-up information. Results The 30 participants (22 women and 8 men) had a mean age of 44 years. The indications for bilateral adrenalectomy were refractory Cushing’s disease (n = 16), occult ectopic ACTH syndrome (n = 9), and bilateral pheochromocytoma (n = 5). A mean of 53 months elapsed between onset of symptoms and adrenalectomy. Laparoscopic bilateral adrenalectomy was completed for all the patients with no intraoperative complications. Four patients (13%) experienced six complications. The mean postoperative LOS was 3.5 days (range, 1–12 days). Seven patients required a preoperative LOS, for a mean of 7.1 days (range, 1–20 days), and a postoperative LOS, for a mean of 5 days (range, 2–12 days). The 23 patients who did not require preoperative hospitalization had a mean postoperative LOS of 3 days (range, 1–7 days). All the patients received postoperative steroid replacement and appropriate follow-up assessment with an endocrinologist. At this writing, the patients with Cushing’s syndrome available for follow-up evaluation continue to receive steroid replacement, and all the pheochromocytoma patients have experienced a documented postoperative biochemical cure. Conclusions Laparoscopic bilateral adrenalectomy is safe and effective for this high-risk patient population. Although patients should be monitored closely in the postoperative period, most are discharged with glucocorticoid and mineralocorticoid replacement in a short time without complications.  相似文献   

9.
Antireflux surgery is supposed to prevent the development of adenocarcinoma in patients with Barrett’s esophagus. The purpose of this study was to determine the prevalence of adenocarcinoma late after antireflux surgery. A total of 161 patients with long-segment Barrett’s esophagus had antireflux surgery and were followed for a mean of 148 months (range 54 to 268 months) Clinical, endoscopic, histologic, and functional studies were performed. Of the original 161 patients, 147(91.3%) completed long-term follow-up. Six patients (4.1%) developed adenocarcinoma 4,5,6,9,17, and 18 years, respectively, after surgery. Five were men. Two of them were asymptomatic for 12 and 17 years. Three of them had extralong-segment Barrett’s esophagus. Five underwent manometric evaluation with only one showing an incompetent lower esophageal sphincter. In two cases, 24-hour pH studies showed massive acid reflux. Two patients had early adenocarcinoma, whereas four had advanced carcinoma. Adenocarcinoma in long-segment Barrett’s esophagus seems to develop mainly in patients with recurrence of pathologic reflux, especially among men. A review of the English language literature during the last 2 3 years found 25 articles dealing with Barrett’s esophagus and antireflux surgery. Most of these reports had only a few patients with short-term follow-up (<60 months). To determine the true prevalence of this complication, a long-term objective follow-up is necessary.  相似文献   

10.
Summary Background. We investigated retrospectively the short and long-term motor and cognitive functioning of staged bilateral pallidotomy using motor testing and a comprehensive neuropsychological battery before and after each procedure. Methods. Fifteen patients with idiopathic Parkinson’s disease were assessed at baseline and at least 3 months after each of their two staged surgeries. Motor and neuropsychological results were compared to 15 non-surgical Parkinson’s disease patients matched for disease stage and mental status. In addition, nine bilateral pallidotomy patients were evaluated for long-term cognitive changes (>2 years). Findings. Bilateral pallidotomy patients demonstrated significant improvements in motor functioning in the “on” and “off” states and with dyskinesias after the first surgery, with an additional improvement reported for dyskinesias after the second procedure. On long-term follow-up, dyskinesia improvements were maintained. Bilateral pallidotomy patients did not show significant cognitive declines following both procedures on the short-term follow-up and when compared to the Parkinson’s disease group. However, significant cognitive declines were found on the long-term follow-up evaluation. Conclusions. Parkinson’s disease patients received significant short- and long-term motor benefits, particularly reduced dyskinesias, following staged bilateral pallidotomy without significant short-term cognitive consequences. Two years following the second procedure, bilateral pallidotomy patients tended to show an increase in both motor and non-motor symptoms of Parkinson’s disease, particularly cognitive decline.  相似文献   

11.
Wegener’s granulomatosis (WG) is a systemic necrotizing vasculitis of unknown etiology characterized mainly by the involvement of the upper airways, lungs, and kidneys. Although most organ systems can be involved, gastrointestinal involvement in WG is notably uncommon. We herein present the case of a WG patient who developed two massive gastrointestinal hemorrhages treated respectively by surgery and angiographic embolization of the bleeding artery. The present case indicates that gastrointestinal manifestations might thus be considered in the natural history of WG.  相似文献   

12.
Gastroeosphageal reflux disease has been associated with long segments of Barrett’s esophagus (≥3 cm), but little is known about its association with shorter segments. The aim of this study was to evaluate anatomic and physiologic alterations of the cardia and esophageal exposure to gastric and duodenal juice in patients with short and long segments of Barrett’s esophagus. Furthermore, these patients were compared to each other and to patients with erosive esophagitis and those with no mucosal injury. Two hundred sixty-two consecutive patients with foregut symptoms were divided into the following four groups based on endoscopic and histologic findings: group 1, no mucosal injury; group 2, erosive esophagitis; group 3, short-segment Barrett’s esophagus; and group 4, long-segment Barrett’s esophagus. Esophageal exposure time to acid and bilirubin, lower esophageal sphincter characteristics, and endoscopie anatomy of the cardia were compared between the groups. Patients with short-segment Barrett’s esophagus had elevated esophageal acid and bilirubin exposure, decreased lower esophageal sphincter pressure and length, and a high incidence of hiatal hernia. These abnormalities were similar to those in patients with esophagitis and in general less profound than those found in patients with long-segment Barrett’s esophagus. The length of intestinal metaplasia was higher in patients with a defective lower esophageal sphincter. Short-segment Barrett’s esophagus is a complication of severe gastroesophageal reflux disease and is associated with the reflux of both gastric and duodenal juice similar to that seen in patients with long-segment Barrett’s esophagus. Presented at the Thirty-Eighth Annual Meeting of The Society for Surgery of the Alimentary Tract, Washington, D.C., May 11–14,1997.  相似文献   

13.
Noh SJ  Ahn JY  Lee KS  Kim SH 《Acta neurochirurgica》2007,149(12):1223-1228
Summary Although pituitary adenomas and Rathke’s cleft cysts have a shared ancestry, they rarely occur simultaneously. Only 32 reports involving a pituitary adenoma and a concomitant Rathke’s cleft cyst were identified upon review of the literature. Most initial presenting complaints include hormonal symptoms, visual disturbances, and headache. Next to growth hormone, Prolactin was the most commonly hypersecreted pituitary hormone. Rathke’s cleft cysts show variable position, size, and signal intensity on magnetic resonance imaging (MRI). Here, we report a patient with a growth hormone- secreting pituitary adenoma associated with a Rathke’s cleft cyst. The mass contained two different signal intensities on MRI. The lesion was successfully removed assisted by intraoperative MRI, when the presence of both lesions was confirmed. When a non-enhancing cyst-like structure is demonstrated on imaging in a patient with a pituitary adenoma, the possibility of a coexisting Rathke’s cleft cyst should be considered.  相似文献   

14.
Endoscopic surveillance is recommended for patients with Barrett’s esophagus to detect high-grade dys-plasia (HGD) or cancer. We studied the outcome of esophagectomy in a cohort of patients who devel-oped HGD or cancer between 1995 and 2003 while under surveillance for Barrett’s. Outcomes were measured by analysis of clinical records, symptom questionnaire, and SF-36 (version 2). In 34 patients, mean surveillance time was 48 months (range, 4–132); the mean number of endoscopies was 10 (range, 3–30). Preoperative diagnosis was HGD in 9 patients (26.5%), carcinoma in situ in 16 (47%), and ad-enocarcinoma in 9 (26.5%). There was no esophagectomy-related mortality; 10 patients (29%) had com-plications. At mean follow-up of 46 months (range, 13–108), SF-36 (version 2) results showed quality of life scores equal to or better than those of healthy individuals. Incidence and severity scores (VAS 1–10) for postoperative symptoms were reflux, 59% (2.8); dysphagia, 28% (3.7); bloating, 45% (2.6); nausea, 28% (2.1); and diarrhea, 55% (2.5). Twenty-nine patients (85%) have no clinical, radiographic, or en-doscopic evidence of recurrent esophageal cancer or metastasis. One patient has metastatic disease. En-doscopic surveillance in Barrett’s patients yields malignant lesions at an early, generally curable, stage. Esophagectomy is curative in the great majority and can be accomplished with minimal mortality and excellent quality of life. This work was supported in part by the Mary and Dennis Wise Fund and in part by an educational grant from United States Surgical Corpo-ration, Tyco Healthcare.  相似文献   

15.
We report a case of non-Hodgkin's lymphoma (NHL) presenting with acute renal failure. A-56-year-old male was admitted to our hospital on October, 1997 with fever and renal dysfunction. Physical examination showed no abnormality except for hepatomegaly. Body surface lymphadenopathy was not observed. Computed tomography (CT) of the abdomen showed markedly enlarged kidneys bilaterally and a mass of soft tissue density, which was considered as a swelling lymph node, around the aortic artery. The renal biopsy revealed parenchymal involvement of the NHL cells without normal tubulo-interstitial structure, but the glomeruli were almost intact. Our case rapidly fell into oliguria and acute renal failure, hence needed hemodialysis. After chemotherapy was performed, his renal function gradually improved and the kidney became smaller on subsequent CT. Unfortunately, the patient happened to suffer from methicillin-resistant staphylococcus aureus (MRSA) infection in a neutropenic state and died. Necropsy revealed recovery of the renal interstitium without residual NHL cells. Renal lymphoma without any other organ or nodal involvement is a rare type of NHL, which considered primary renal lymphoma (PRL). However, we believe this case to have been a result of lymphomatous infiltration of the kidneys in disseminated lymphoma.  相似文献   

16.
The A3243G mutation of the mitochondrial tRNA(Leu) gene has been recently reported in rare patients with focal and segmental glomerulosclerosis (FSGS). However, the full spectrum of systemic and kidney manifestations in adults presenting with this mutation remains poorly defined. Assessment of renal and nonrenal manifestations was performed in nine patients with A3243G mutation and prominent kidney disease diagnosed in adulthood. At first renal evaluation, median age was 35 years. Renal lesions consisted of FSGS (n = 2), tubulointerstitial nephropathy (n = 3), or bilateral enlarged cystic kidneys (n = 1). All but one patient exhibited extrarenal manifestations: deafness (8 of 9) requiring hearing aid in half the cases, diabetes mellitus (3 of 9), neuromuscular involvement (2 of 9), hypertrophic cardiomyopathy (1 of 9), and macular dystrophy (1 of 9). After a median follow-up of 5 yr, five patients progressed to end-stage renal disease between the ages of 15 and 51 years, four being successfully transplanted. Similarly, extrarenal manifestations progressed since all patients had deafness and diabetes (including three posttransplants), while half had neuromuscular, cardiac, or retinal involvement. In the adult patients with A3243G mutation and renal involvement, preexisting deafness is almost consistently found. While FSGS remains the most typical lesion, tubulointerstitial nephropathy or bilateral, enlarged cystic kidneys may also be encountered. In most cases, diabetes mellitus, macular dystrophy, hypertrophic cardiomyopathy, or neuromuscular features occur later in the course of the disease. The severity of the clinical course is heterogeneous, with end-stage renal failure being reached between the second and sixth decades. Renal transplantation may be offered to these patients, despite a high incidence of steroid-induced diabetes mellitus.  相似文献   

17.
The aim of the study was an evaluation of Furlow’s method in correction of velopharyngeal insufficiency. A prospective study was conducted by a speech pathologist and a plastic surgeon. Rating of hypernasality and speech intelligibility were completed prior to and after surgical intervention. Nasometric measurements were also performed before and after surgical intervention. From May 2003 through September 2006, the first author performed 44 Furlow surgeries for the treatment of velopharyngeal insufficiency in patients with cleft palate. Patients with short, repaired cleft palates but with preserved normal function of pharyngeal sphincter qualified for the operation. The surgery was performed using double-opposing Z-plasty. The method was used in 24 males and 20 females aged from 6 to 25 years (mean age, 12 years). One patient (2%) developed wound dehiscence, and two further patients (4.5%) developed maceration of the wound margins, which delayed the healing process by only several days. Very good, good, or moderate results were obtained in 41 patients (91%). Poor outcome of treatment—excessive hypernasality or poor speech quality after the operation—was still present in four patients (9%). In our opinion, Furlow’s method is a very useful treatment method in patients with velopharyngeal insufficiency especially with sagittal orientation of levator veli palatini muscles.  相似文献   

18.
Periosteal Ewing’s sarcoma is very rare. In this report, we describe three cases of periosteal Ewing’s sarcoma, illustrating the principal clinical, radiographic, and histologic features. The patients were aged 15, 16, and 20 years old, with an average of 17 years. The tumor occurred in the humerus in two and in the femur in one. Plain radiography showed a laminated periosteal reaction with a Codman’s triangle over a subperiosteal soft-tissue mass or saucerization. The unusual site of Ewing’s sarcoma was clearly visualized by computed tomography (CT) and magnetic resonance imaging (MRI) and confirmed by histological examination. Chemotherapy and/or irradiation was employed before and after wide excision. The patients have survived an average of 6 years after the primary surgery. Received: 27 November 2000  相似文献   

19.
Plasmablastic lymphoma (PBL) is a rare acquired immunodeficiency syndrome-associated non-Hodgkin’s lymphoma (AIDS-NHL), with predilection for the mucosa of oral cavity. It usually has a plasmablastic morphology, expressing plasma cell-associated antigens with weak or absent expression of B-cell-associated markers. To further define the immunophenotypic and molecular genetics of these tumors, we investigated two cases of plasmablastic lymphomas of the head and neck for c-myc gene rearrangement and immunoglobulin heavy chain (IgVH) hypermutation status. For the first time we report a case of AIDS-related PBL that, by fluorescence in situ hybridization (FISH), shows a c-myc gene rearrangement. Although current literature suggests that most cases of c-myc gene rearranged AIDS-NHL are Burkitt’s lymphoma, our case has an immunophenotype characteristic for PBL. In this case, IgVH hypermutation analysis showed a somatic hypermutation, indicative of germinal center transit. The concurrent B-cell immunophenotype of BCL-6/CD138+/MUM-1+ also suggests a post-germinal center B-cell origin of this lymphoma. The immunophenotype of our second case (BCL-6/CD138+/MUM-1+) also suggests a post-germinal center B-cell origin. However, IgVH hypermutation analysis was not possible in this case.  相似文献   

20.
PURPOSE: We evaluated contrast enhanced spiral computerized tomography (CT) as a single session for the anatomical and functional assessment of patients with chronic obstructive uropathy and normal serum creatinine. MATERIALS AND METHODS: The study included 65 patients with unilateral or bilateral chronic renal obstruction and normal serum creatinine. Five patients had bilateral obstruction and the remaining 60 had unilateral obstruction and a normal contralateral kidney. Therefore, the total number of renal units was 130, that is 70 obstructed and 60 normal. All patients underwent contrast enhanced spiral CT together with excretory urography (IVP) and Tc-mercaptoacetyltriglycine renal scan. CT was used to identify the cause of obstruction and selectively determine the glomerular filtration rate (GFR) of the 2 kidneys. The diagnostic accuracy of CT for identifying the cause of hydronephrosis was compared with that of IVP. Moreover, a correlation was made between CT GFR and isotope GFR. RESULTS: Obstruction was caused by ureteropelvic junction narrowing in 25 cases, ureteral stones in 21, ureteral stricture in 20 and extrinsic ureteral obstruction in 4. Contrast enhanced spiral CT identified the cause of hydronephrosis in all obstructed kidneys (100% sensitivity), while IVP identified the cause in 52 (74% sensitivity), which was a significant difference (p <0.05). CT and IVP excluded obstruction in all normal kidneys (100% specificity). A comparison between the isotope GFR of obstructed kidneys with the corresponding CT GFR showed a perfect correlation (r = 0.78, p <0.0001). Moreover, a similar comparison between isotope GFR and CT GFR of normal kidneys showed an excellent correlation (r = 0.73, p <0.0001). In obstructed and normal kidneys mean isotope clearance was not significantly different from that of mean CT clearance. CONCLUSIONS: Contrast enhanced spiral CT is more sensitive than IVP for identifying the cause of chronic obstructive uropathy. Moreover, it is as accurate as radioisotope renal scan for calculating the total and separate kidney function. We recommend spiral CT with contrast medium as a single radiological diagnostic modality for the assessment of patients with chronic renal obstruction and normal serum creatinine.  相似文献   

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