Multiple Segmental Lesions Research Articles

Magnetic resonance imaging in enterovirus-71, myelin oligodendrocyte glycoprotein antibody, aquaporin-4 antibody, and multiple sclerosis-associated myelitis in children.

We used magnetic resonance imaging (MRI) to compare the neuroimaging of children with their first episode of clinical enterovirus 71-associated transverse myelitis (EV71-TM), myelin oligodendrocyte glycoprotein antibody positive transverse myelitis (MOG-TM), aquaporin-4 antibody positive transverse myelitis (AQP4-TM), transverse myelitis in multiple sclerosis (MS-TM), and unclassified transverse myelitis (UNC-TM). We performed a retrospective blinded radiological assessment and compared the neuroimaging of 52 children (32 females, 20 males; mean age 9y 8mo, SD 5y 5mo, range 5mo-17y) presenting with their first episode of myelitis caused by EV71-TM (n=11), MOG-TM (n=10), AQP4-TM (n=9), MS-TM (n=13), and UNC-TM (n=9). In the EV71-TM group, lesions were distributed throughout the cord and enhancement of nerve roots (ventral and dorsal) was common. The MOG-TM group had lesions distributed throughout the cord and most commonly longitudinally extensive transverse myelitis and lesions involving the grey matter alone on axial scans. The AQP4-TM group had lesions distributed in the cervicothoracic spine, cavitation, and contrast enhancing lesions. All patients with AQP4-TM had an abnormal brain MRI scan. The MS-TM group characteristically had multiple short segment lesions of the cord involving the cervicothoracic spine. The UNC-TM group did not have distinctive spinal MRI findings but had a relative paucity of lesions on their brain MRI scans. There are neuroimaging findings that are helpful in differentiating between myelitis associated with EV71, MOG, AQP4, and multiple sclerosis in children. These features may be useful early in the presentation of transverse myelitis while awaiting infectious/immunological testing, and/or further demyelinating events. Magnetic resonance imaging can help identify aetiologies for children presenting with a first episode of myelitis. Entervirus-71-associated myelitis lesions are distributed throughout the cord and enhancement of nerve roots is common. Lesions distributed throughout the cord are commonly seen in myelin oligodendrocyte-associated myelitis. Aquaporin-4-associated myelitis lesions are distributed in the cervicothoracic spine, cavitation and contrast enhancing lesions are common. Short segment lesions in the cervicothoracic spine are commonly seen in multiple sclerosis-associated myelitis.

Surgical approach and management outcomes for junction tuberculous spondylitis: a retrospective study of 77 patients

BackgroundJunction tuberculous spondylitis involves the stress transition zone of the spine and has a high risk of progression to kyphosis or paraplegia. Problems still exist with treatment for spinal junction tuberculosis. This study investigated the surgical approach and clinical outcomes of junction spinal tuberculosis.MethodsFrom June 1998 to July 2014, 77 patients with tuberculous spondylitis were enrolled. All patients received 2–3 weeks of anti-tuberculous treatment preoperatively; treatment was prolonged for 2–3 months when active pulmonary tuberculosis was present. The patients underwent anterior debridement and were followed up for an average of 29.4 months clinically and radiologically.ResultsThe cervicothoracic junction spine (C7-T3) was involved in 15 patients. The thoracolumbar junction spine (T11-L2) was involved in 39 patients. The lumbosacral junction spine (L4-S1) was involved in 23 patients. Two patients with recurrence underwent reoperation; the drugs were adjusted, and all patients achieved bone fusion. The preoperative cervicothoracic and thoracolumbar kyphosis angle and lumbosacral angle were 31.4 ± 10.9°, 32.9 ± 9.2°, and 19.3 ± 3.7°, respectively, and the corresponding postoperative angles were ameliorated significantly to 9.1 ± 3.2°, 8.5 ± 2.9°, and 30.3 ± 2.8°. The preoperative ESR and C-reactive protein level of all patients were 48.1 ± 11.3 mm/h and 65.5 ± 16.2 mg/L which decreased to 12.3 ± 4.3 mm/h and 8.6 ± 3.7 mg/L at the final follow-up, respectively. All patients that had neurological symptoms achieved function status improvement at different degrees.ConclusionFor spinal tuberculosis of spinal junctions, anterior debridement, internal fixation, and fusion can be preferred and achieved. If multiple segment lesions are too long or difficult for operation of anterior internal fixation, combining posterior pedicle screw fixation is appropriate.

Long-term outcomes of varicella zoster virus infection-related myelitis in 10 immunocompetent patients

ObjectiveTo describe the clinical presentation and long-term disease outcomes of varicella zoster virus (VZV) infection–related myelitis (VZVM) in immunocompetent patients. MethodA series of 10 immunocompetent patients with VZVM were retrospectively observed and followed (3–96 months). ResultsThe onset of myelitis was timed in relation to the appearance of VZV-associated rash (−3 to 50 days). Rash locations included the cervical (5), thoracic (2), and lumbar (3) dermatomes, whereas myelitis localized to the cervical (6) and thoracic (9) spinal cord and the medulla (1). Spinal MRI revealed extensive longitudinal transverse myelitis in nine patients, with multiple segmental lesions (≥2 segments) evident in five patients. Aquaporin-4, myelin oligodendrocyte glycoprotein, ganglioside Q1b, and ganglioside T1b antibodies were detected in some patients. Three patients fulfilled the 2015 diagnostic criteria for neuromyelitis optica spectrum disease, of whom two relapsed. Seven patients were treated with intravenous antivirals and methylprednisolone, with the remaining three patients receiving methylprednisolone only. Ongoing immunosuppressive therapy was provided for two patients who experienced relapses. To date, no patients have reported VZV reactivation. Over the course of follow-up, the Expanded Disability Status Scale (EDSS) score deceased from 4.9 to 2.6 on average. ConclusionsVZVM runs a relatively benign course in immunocompetent patients, although relapses can occur depending on patient immune status. A comprehensive evaluation of patient's autoimmune condition is recommended.

TCTAP C-186 One-stage Endovascular Treatment for Complicated Peripheral Arterial Occlusive Disease with Multiple Segmental Lesions

TCTAP C-186 One-stage Endovascular Treatment for Complicated Peripheral Arterial Occlusive Disease with Multiple Segmental Lesions

Multilineage somatic activating mutations in HRAS and NRAS cause mosaic cutaneous and skeletal lesions, elevated FGF23 and hypophosphatemia

Pathologically elevated serum levels of fibroblast growth factor-23 (FGF23), a bone-derived hormone that regulates phosphorus homeostasis, result in renal phosphate wasting and lead to rickets or osteomalacia. Rarely, elevated serum FGF23 levels are found in association with mosaic cutaneous disorders that affect large proportions of the skin and appear in patterns corresponding to the migration of ectodermal progenitors. The cause and source of elevated serum FGF23 is unknown. In those conditions, such as epidermal and large congenital melanocytic nevi, skin lesions are variably associated with other abnormalities in the eye, brain and vasculature. The wide distribution of involved tissues and the appearance of multiple segmental skin and bone lesions suggest that these conditions result from early embryonic somatic mutations. We report five such cases with elevated serum FGF23 and bone lesions, four with large epidermal nevi and one with a giant congenital melanocytic nevus. Exome sequencing of blood and affected skin tissue identified somatic activating mutations of HRAS or NRAS in each case without recurrent secondary mutation, and we further found that the same mutation is present in dysplastic bone. Our finding of somatic activating RAS mutation in bone, the endogenous source of FGF23, provides the first evidence that elevated serum FGF23 levels, hypophosphatemia and osteomalacia are associated with pathologic Ras activation and may provide insight in the heretofore limited understanding of the regulation of FGF23.

Assessment of the diagnostic value of CT and X-ray enterography for small intestinal Crohn disease

To investigate the value of CT and X-ray enterography in the diagnosis of small intestinal Crohn disease(CD). Data of 39 CD cases confirmed by surgery and pathology who underwent CT and X-ray enterography were analyzed retrospectively. All the patients had complete CT data, 28 cases had X-ray intestinal barium meal data, and 18 had sinus tract enterography. CT enterography showed mural thickening(>4 mm) in 34(87.2%) patients, mural gas in 7(17.9%), mural edema in 7(17.9%), mural fat in 4(10.3%), increased enhancement of bowel wall(>10 HU) in 37(94.9%), multiple segmental lesions in 33(84.6%), single segmental lesions in 6(15.4%), mesenteric lymphadenopathy(>5 mm) in 13(33.3%), vascular bundle thickening in 9(23.1%), cellulitis in 12(30.8%), peritoneal abscess in 10(25.6%), phlegmon in 8(20.5%), incomplete intestinal obstruction in 14(35.9%), seroperitoneum in 22(56.4%), and fistulization in 4(10.3%). CT enterography did not demonstrate the change of mucosa such as strip ulcer or cobblestone. Among the 28 cases of small bowel X-ray enterography, 23 cases(82.1%) presented with multiple segmental lesions, 5(17.9%) with single segmental lesions, 18(64.3%) with strip ulcer, 16(57.1%) with cobblestones, 4(14.3%) with intestinal fistula, while no bowel wall and extraintestinal complication of CD disease was observed. Among the 18 cases of sinus tract enterography, 13 cases (72.2%) presented with intestinal fistula, 12(66.7%) with peritoneal abscess, 8(44.4%) with sinus tract. CT enterography can demonstrate exactly the diseased bowel wall and extraintestinal complication of CD disease, which is important to evaluate the extent of CD and guide the treatment, however strip ulcer and cobblestone sign cannot be demonstrated. The X-ray enterography is available to demonstrate the characteristic changes of CD such as trip ulcers and cobblestones, but is difficult to show the bowel wall and extraintestinal inflammatory mass and abscesses. The sinus tract enterography is easy to demonstrate the intestinal fistula and intra-abdominal abscess. Combination of these methods is more beneficial to guild the diagnosis and treatment.

Features Analysis of Lower Extremity Arterial Lesions in 162 Diabetes Patients

Objective. This study aimed to investigate the angiographic manifestations of lower extremity atherosclerotic steno-occlusive disease in patients with diabetes. Materials and Methods. A total of 162 patients with diabetes were enrolled in this study. The angiographic findings of lower extremity arterial lesions were evaluated according to location (iliac, femoral, popliteal, and crural artery), type (stenosis or occlusion), and length (<5 cm, 5–10 cm, and >5 cm). Results. A total of 131 of 162 (80.9%) diabetics showed multiple segmental lesions, and 19.1% (31/162) presented single segmental lesions in the lower extremity artery. Crural artery was the mainly involved location (39/162, 85.8%). Among the recorded 660 lesions of 162 cases, 437 (66.2%) were occlusion lesions, while 223 (33.8%) were stenosis lesions. Of 437 occlusion lesions, 308 lesions (70.5%) were in crural artery. More than 10 cm occlusion lesion (242/392, 61.7%) was the main manifestation in crural artery, especially in anterior (92/127, 67.2%) and posterior tibial arteries (91/124, 73.4%), which was higher than that in iliac artery (8/33, 24.2%), popliteal artery (53/157, 33.8%), and femoral artery (11/78, 14.1%). Conclusion. In diabetic subjects with lower limb artery ischemia, the vascular involvement is extremely diffuse and particularly severe in crural arteries, with high prevalence of more than 10 cm occlusion lesions.

Using Intermittent Pneumatic Compression Therapy to Improve Quality of Life for Symptomatic Patients With Infrapopliteal Diffuse Peripheral Obstructive Disease

Although the acute effect of intermittent pneumatic compression (IPC) therapy had been documented for patients with symptomatic peripheral arterial obstructive disease (PAOD), its efficacy in improving quality of life (QOL), especially for those with infrapopliteal diffuse lesions, remains unclear. Thirty-one patients with infrapopliteal diffuse or multiple segmental lesions were enrolled in the study. Based on receipt of IPC therapy (3 h daily for 3 months), patients were allocated to a study (n=23) or control (n=8) group. The 6-min walking test, transcutaneous oxygen tension (TcPO₂), and QOL evaluated with the Short-Form 36 questionnaire were measured at the beginning and end of the study. In the QOL analysis, scores for physical functioning, physical and emotional role functioning, bodily pain, and general and mental health showed significant changes after IPC therapy. In the 6-min walking test, duration, and the initial and absolute claudication distances were significantly increased in the study group. The TcPO₂ also significantly increased in the distal end of the target limb after IPC therapy. Patients at high risk for amputation with infrapopliteal diffuse or multiple segmental lesions can improve their walking ability, TcPO₂ of the target limb and QOL after IPC therapy.

Endovascular revascularization for lower extremity atherosclerosis obliterans in elderly patients:a report of 86 cases

To analyze the outcome and risk factors of endovascular revascularization of lower limb artery for elder patients. From January 2006 to November 2008, 86 elder patients (98 ischemia limbs) underwent endovascular revascularization due to lower limb ischemia. Age of this group were 60 to 82 years old with a mean of (70 +/- 6) years old. Fifty-four limbs (55.0%) had severe intermittent claudication, 28 limbs (28.6%) had rest pain, 11 limbs (11.2%) had ulcer, and 5 limbs (5.1%) had gangrene. Sixty-six limbs were mono segment disease, including 25 aorta-iliac lesions, 33 femoral-popliteal lesions and 8 infra-popliteal lesions. Thirty-two limbs were multiple segment disease involving 2 or 3 segment lesions. Mortality, morbidity, primary patency, secondary patency and limb salvage were retrospectively analyzed. Risk factors on outcome were also evaluated. Ten limbs underwent angioplasty, while the rest 88 limbs underwent angioplasty plus primary stent implantation. The total operation success rate was 95.9%. Perioperative mortality within the first 30 d was 0. Perioperative morbidity within the first 30 d was 5 cases (5.1%), including 2 myocardial infarction, 2 major amputations and 1 irreversible contrast-induced nephropathy. Follow-up duration were 1 to 35 months with a mean of (18 +/- 10) months. Eighty-three (96.5%) patients had effectively follow-up. Mortality was 2.3% (2 cases died due to myocardial infarction). Primary patency rate was 83.7%, secondary patency rate was 94.9% and limb salvage rate was 95.9%. Risk factor analysis showed that diabetes mellitus, critical ischemia and multiple segment lesions were associated with worse patency. Endovascular treatment is effective, safe and repeatable revascularization for elder patients of Critical lower limb ischemia. Patients with diabetes mellitus, critical ischemia and multiple segment lesions should be paid more attention because their rather worse outcome.

Infrainguinal cutting balloon angioplasty in de novo arterial lesions

This prospective, non-randomized study evaluated the short- and mid-term feasibility, safety, primary patency, and limb salvage of cutting balloon percutaneous transluminal angioplasty (CB-PTA) for the treatment of peripheral arterial occlusive disease (PAOD). All data were collected for 128 consecutive patients who underwent CB-PTA to improve infrainguinal arterial circulation between January 2003 and July 2007. One-hundred thirty-five limbs with PAOD (claudication, n = 19; critical limb ischemia [CLI], n = 116) were treated. Patency was evaluated by clinical examination and duplex ultrasonography. A total of 203 lesions (183 stenoses, 20 occlusions) were treated in 66 femoropopliteal and 69 infrapopliteal arterial segments. The TransAtlantic Inter-Societal Consensus (TASC) classification of the primary lesions was A in 41.5%, B in 45.2%, C in 8.2%, and D 5.1%. Mean follow-up was 16.1 +/- 9.7 months. The overall technical success rate was 96.3% and the complication rate was 8.9%. There were two (1.5%) perioperative deaths. The primary patency rate was 82.1% at 12- and 24-months in patients with claudication (femoropopliteal lesions). The 1- and 2-year results for femoropopliteal and infrapopliteal lesions in patients with CLI were: primary patency 64.4% and 51.9 %, respectively; limb salvage 84.2% and 76.9%; survival 92.6% and 88.5%. More distal lesions and TASC classification were significant independent risk factors for outcome (P < .05). Treatment of multiple segment lesions was an independent predictor of a favorable outcome (P = .04). CB-PTA is safe and feasible for the treatment of infrainguinal arterial occlusive disease, with relatively low mid-term restenosis rates compared to other endovascular treatments. However, these data cannot be extrapolated to potential outcomes for lesions >10 cm in length. Further follow-up will be necessary to evaluate the long-term results of CB-PTA.

Glomerular tip changes in childhood minimal change nephropathy

Segmental glomerular lesions at the tubular opening, or tip changes, are found in the renal biopsies of adults in many disorders, including some initially considered to show minimal change nephropathy. The hypothesis was that similar tip changes occurred in children. We reviewed a consecutive series of 50 biopsies, diagnosed as minimal change nephropathy, from 49 children. Segmental lesions were found in five biopsies. One biopsy showed lesions at the glomerular hilum. The patient was in remission at follow-up. Four biopsies showed only tip changes. Three patients were in remission, two on no treatment at follow-up, and one on ciclosporin. The other had chronic hepatitis B infection, with persistent proteinuria and segmental lesions at different sites in glomeruli. The other 44 children were nearly all in remission, 18 without treatment at follow-up, and the rest on various immunosuppressants, but one had persistent proteinuria and multiple segmental lesions. Series of childhood minimal change nephropathy, similar to this one, are likely to include cases of the glomerular tip lesion, under the original definition of minimal change nephropathy plus tip changes. This should make little difference in clinical practice, because the clinical course should resemble that of minimal change nephropathy.

The effectiveness of percutaneous transluminal angioplasty for the treatment of critical limb ischemia: A 10-year experience

To determine the efficacy, safety, and long-term results, including continued clinical improvement and limb salvage, of percutaneous transluminal angioplasty (PTA) in patients with critical limb ischemia (CLI). From August 1993 to March 2004, 138 limbs in 111 patients with CLI (rest pain in 62 [45%] and ulcer/gangrene in 76 [55%]) were treated by PTA. In iliac lesions, stents were placed selectively for primary PTA failure: residual stenosis (>30%) or pressure gradient (>5 mm Hg). Stent placement was limited in infrainguinal lesions. The most distal affected arteries treated with angioplasty were the iliac artery in 45 limbs (33%; iliac group), the femoropopliteal artery in 41 limbs (30%; FP group), and tibial arteries in 52 limbs (37%; BK group). All analysis was performed according to an intent-to-treat basis. Reporting standards of the Society for Vascular Surgery and the International Society for Cardiovascular Surgery were followed to evaluate initial success, and late follow-up status was evaluated with the Kaplan-Meier method. Patency was evaluated by using ultrasound scanning and ankle-brachial pressure index measurement. There was one (0.9%) perioperative death. Twenty stents were placed selectively in 14 iliac arteries. Mean follow-up was 14.7 months (range, 1-75 months). Overall, initial technical and clinical success rates were 96.4% and 92.8%, respectively. The cumulative primary, assisted primary, and secondary patency; continued clinical improvement; and limb salvage rates +/- SE at 5 years were 31.4% +/- 10.4%, 75.5% +/- 5.7%, 79.6% +/- 5.5%, 36.1% +/- 10.0%, and 89.1% +/- 4.0%, respectively. In each subgroup, the primary, assisted primary, and secondary patency; continued clinical improvement; and limb salvage rates at 3 years were 51.6%, 94.7%, 97.8%, 65.1%, and 95.0%, respectively, in the iliac group; 49.4%, 72.2%, 76.4%, 57.4%, and 92.7%, respectively, in the FP group; and 23.5%, 41.8%, 46.1%, 51.1%, and 77.3%, respectively, in the BK group. Of the 12 predictable variables, hypertension, multiple segment lesions, more distal lesions, and TransAtlantic Inter-Society Consensus classification type D were significant independent risk factors for the outcomes ( P < .05; univariate log-rank test and Cox regression multivariate analysis). PTA is a feasible, safe, and effective procedure for the treatment of CLI. The high limb salvage rate is attributed to the high assisted primary and secondary patency rates despite the low primary patency rate. Angioplasty can be the primary choice for the treatment of CLI due to iliac and infrainguinal arterial occlusive disease.

Hepatic involvement in hypereosinophilia: sonographic findings.

Hypereosinophilic syndrome may cause eosinophil-related tissue damage to various organs. The purpose of this paper is to describe sonographic findings in 13 patients with hypereosinophilia in whom the liver was involved. The diagnosis in these 13 patients was based on liver biopsy in seven patients with bone marrow biopsy in six patients. Eight patients had hypereosinophilic syndrome and five patients had clonorchiasis. All 13 patients had mild to marked hepatomegaly. Seven of 13 patients showed multiple round or oval hypoechoic (n = 6) or variably echogenic (n = 1) lesions measuring 1 to 2 cm with poorly defined margins in both lobes of the liver. Four patients had one or two hypoechoic lesions 3 to 4 cm in size, with geographic pattern and poorly defined margins. Two patients showed diffuse hepatomegaly with increased parenchymal echogenicity. The number of lesions and the extent of diffuse lesions seem to be proportional to the degree of eosinophilia. Hypereosinophilia may produce multiple small focal hepatic lesions or diffuse segmental or lobar echogenic lesions simulating primary or metastatic tumor of the liver.

Spinal intramedullary cysticercosis.

Spinal intramedullary cysticercosis is an uncommon manifestation of neurocysticercosis. We review our experience with eight cases of intramedullary cysticercosis. Eight patients who were surgically treated for spinal intramedullary cysticercosis between 1982 and 1991 were retrospectively reviewed, and the final outcomes were assessed. In six patients, the cysticercosis involved the thoracic cord, whereas in the other two, the cysticercosis was cervical in location. Only one patient had multiple soft tissue calcifications, as revealed by plain radiography. Myelography indicated an intramedullary lesion in each of seven patients; two of the seven patients had partial myelographic block, suggesting the segmental nature of the lesion. Cerebrospinal fluid studies were noncontributory. One patient had three cysts, whereas the other seven had one cyst each. Four patients had adjacent soft purulent materials, which were revealed by histopathological examination to be granulation tissue. The neurological statuses of seven patients improved after surgery. Six patients were followed up for a mean of 30.6 months (3 mo-5 yr). Three could resume their previous occupations, two others could manage their daily activities, and one required only minimal assistance for daily activities. The outcome of intramedullary cysticercosis is not as dismal as reported earlier, and patients with paraplegia also have favorable outcomes. A preoperative diagnosis of cysticercosis can be suspected in an endemic area in the presence of multiple soft tissue calcifications and segmental lesions revealed by myelography or magnetic resonance imaging studies.

Spinal Intramedullary Cysticercosis

OBJECTIVE: Spinal intramedullary cysticercosis is an uncommon manifestation of neurocysticercosis. We review our experience with eight cases of intramedullary cysticercosis. METHODS: Eight patients who were surgically treated for spinal intramedullary cysticercosis between 1982 and 1991 were retrospectively reviewed, and the final outcomes were assessed. RESULTS: In six patients, the cysticercosis involved the thoracic cord, whereas in the other two, the cysticercosis was cervical in location. Only one patient had multiple soft tissue calcifications, as revealed by plain radiography. Myelography indicated an intramedullary lesion in each of seven patients; two of the seven patients had partial myelographic block, suggesting the segmental nature of the lesion. Cerebrospinal fluid studies were noncontributory. One patient had three cysts, whereas the other seven had one cyst each. Four patients had adjacent soft purulent materials, which were revealed by histopathological examination to be granulation tissue. The neurological statuses of seven patients improved after surgery. Six patients were followed up for a mean of 30.6 months (3 mo-5 yr). Three could resume their previous occupations, two others could manage their daily activities, and one required only minimal assistance for daily activities. CONCLUSION: The outcome of intramedullary cysticercosis is not as dismal as reported earlier, and patients with paraplegia also have favorable outcomes. A preoperative diagnosis of cysticercosis can be suspected in an endemic area in the presence of multiple soft tissue calcifications and segmental lesions revealed by myelography or magnetic resonance imaging studies.

頚椎症性筋萎縮症手術例の検討

We retrospectively evaluated the surgical results of cervical spondylotic amyotrophy in 39 patients (34 men and 5 women) who had been managed between 1973 and 1995. The average age at the time of surgery was 51.2 years (range, 33 to 82 years). Subjects showed muscle atrophy at the shoulder girdle (17 patients), at the forearm and hand (12 patients), and throughout the whole upper extremity (10 patients). Activities of daily living were preoperatively impaired by the muscle weakness. Neurological findings revealed 27 myelopathies and 12 radiculopathies. Anterior interbody fusion (Robinson-Smith) was performed in 35 patients (60 intervertebral discs) and expansive laminoplasty (Hirabayashi) was performed in 4 patients.The average duration of follow-up was 64 months (range, 6 to 164 months). The activities of daily living improved in 31 patients (79%), but in eight patients (21%) they were still impaired. The eight patients preoperatively had all showed myelopathy. In cases of developmental spinal canal stenosis (under 12mm of the sagittal diameter), multiple segmental (over three intervertebral discs) lesions, and generally narrow subarachnoid space, anterior interbody fusion gave poor results. In such cases we recommend the expansive laminoplasty.

Different clinicopathological types of segmental sclerosing glomerular lesions in adults

Renal biopsies and kidneys from 111 adult patients with segmental sclerosing glomerular lesions were put into different morphological groups by pathologists unaware of clinical findings. 'Overload changes' (n = 11) were large glomeruli with hilar lesions. Ten of these patients had reduced renal mass. In the 'glomerular tip lesion' (n = 10) there were changes at the tubular origin in otherwise normal glomeruli. All patients in this group had the nephrotic syndrome, completely responsive to steroids. 'Mesangial hypercellularity with diffuse segmental changes at the glomerular tip', corresponding to 'early classical focal segmental glomerulosclerosis' (n = 18), differed from the glomerular tip lesion in that glomeruli were larger and hypercellular. Most patients with this classification had the nephrotic syndrome, several were given additional immunosuppression, and less than half had complete remission. 'Diffuse multiple segmental lesions', corresponding to 'late classical focal segmental glomerulosclerosis' (n = 16), presented usually with the nephrotic syndrome, and most of these patients had no response to immunosuppression. Unlike most other groups, in 'focal segmental sclerosing lesions' (n = 56) there were genuinely focal lesions, not in every glomerulus. Most patients had asymptomatic proteinuria. Pathological study is able to classify segmental lesions that would often be grouped as 'focal segmental glomerulosclerosis' into types with different pathogenesis, clinical findings, appropriate treatment, response to treatment, and prognosis.

多発分節性病変を有する腸骨・大腿動脈領域閉塞症に対する血行再建術の評価

多発分節性病変を有する腸骨・大腿動脈領域閉塞症に対する血行再建術の評価

Critical analysis of cineangiographic criteria for diagnosis of arrhythmogenic right ventricular dysplasia

Biplane 30-degree RAO and 60-degree LAO RV selective cineangiography was performed in 21 patients with significant ventricular arrhythmias (ventricular tachycardia in 14, salvos in three, and complex PVCs in seven) and a high presumption of arrhythmogenic RV dysplasia (ARVD), and in a control group of 10 presumed normal individuals. Comparing the two series revealed the lack of specificity of some angiographic images usually reported as suggestive signs of ARVD, such as slow dye evacuation of RV during the levophase and deep fissuring in the anterior wall with a “pile of plates” image. Inversely, localized morphologic and contraction abnormalities in the RV free wall were more sensitive and specific signs for diagnosis of ARVD; these were localized akinetic or dyskinetic bulges sometimes giving a true image of aneurysm (90%), wide and deep fissuring of the apex or of the inferior wall (33%), and large areas of akinesia. By order of frequency, these abnormalities were found on the apex in 71%, on the inferior wall in 52%, on the anterior wall in 48%, in the subtricuspid area in 38%, and on the pulmonary infundibulum in 33%. These localized lesions can suffice for the diagnosis of RV dysplasia in the absence of associated pathologies, such as ischemic heart disease or congenital defects. Usually a global RV systolic dysfunction is associated in ARVD, as confirmed by greater RV volumes (134 ± 26 vs 79 ± 10 ml/m 2 for RVEDV, p < 0.001; 76 ± 34 vs 32 ± 6 ml/m 2 for RVESV, p < 0.001), and lower RV ejection fraction (58 ± 18% vs 47 ± 8%, p < 0.001) in the ARVD group compared to controls. Nevertheless, normal RV volumes and ejection fraction can be observed in some localized forms with mono- or bisegmental lesions in which RV systolic dysfuntion is absent or moderate, and extensive forms with multiple segmental lesions where RV systolic dysfunction is constant and often severe. Six out of 21 patients in the ARVD group exhibited obvious global or segmental LV dysfunction, indicating the possibility of biventricular forms, as previously reported in other publications.

Surgical Considerations in Extracranial Occlusive Lesions

The criteria for selection of a patient with symptomatic cerebral arterial insufficiency as a candidate for surgical therapy differ in various institutes. For this reason it is difficult to interpret the data on incidence provided by large statistics. The symptomatology differs according to the main type of the occlusion: The carotid type with recurrent dizziness or instability, contralateral numbness or even paralysis of one limb, transient ipsilateral blurring of vision, and deterioration of the ability to do mental work; the vertebrobasilar type with transient periods of vertigo, weakness of the limbs, visual disturbances (of both eyes), and cerebellar ataxia. In the past decade major surgical principles in reconstructive arterial surgery have been stabilized to a large extent. The most favored methods are: Endarterectomy eventually combined with a patch graft angioplasty. This procedure is specifically suitable for lesions of the carotid bifurcation and in the internal carotid and vertebral artery area. Resection of the diseased segment and insertion of a venous or heteroplastic prosthesis bypass procedures are employed in lesions of branches of the aortic arch, especially if there are multiple segmental lesions. The application of this technique depends on the extent and location of the lesion and the condition of the patient (intraversus extrathoracic approach). The best candidates for operation are patients with intermittent attacks of cerebral arterial insufficiency who are neurologically intact. Second best are the patients with progressive neurologic deficits and those with a stable neurologic deficit.