Neurogenic muscle hypertrophy in radiculopathy

The course of radiculopathy is sometimes associated with weakness and wasting of muscles. Very rarely in such cases, however, is hypertrophy of muscle fibres observed. Three cases are presented of sciatica with enlarged calves caused by hypertrophy of type 1 or types 1 and 2 muscle fibres. In light of the literature and the results obtained, an attempt is made to explain the cause of rare clinical symptoms and draw attention to diagnostic and therapeutic difficulties.     

Exudative discoid and lichenoid chronic dermatosis (Sulzberger-Garbe): presentation with a puzzling penile plaque*

Objective Report of two cases of exudative discoid and lichenoid dermatosis (Sulzberger-Garbe), with typical penile lesions. Differential diagnosis of persistent penile plaques. Background Following remission of the generalized eruption of exudative discoid and lichenoid dermatosis (Sulzberger-Garbe), it is not unusual to see a single remaining lesion localized on the penis. Since Sulzberger et al. (Sulzberger MB, Witten VH, Hunt JA. Puzzling persistent penile plaques. Arch Dermatol 1956:73:101-109) reviewed dermatoses presenting with puzzling persistent penile plaques in 1955, further dermatoses have evolved as important differential diagnoses. Much controversy has arisen as to the justification of exudative discoid and lichenoid dermatosis (Sulzberger-Garbe) as a disease entity. Conclusions Diagnosis of most cases of persistent plaques of dermatitis on the penis is possible with a careful examination of the entire skin, and a thorough history to discover past lesions. The possibility of malignancy makes a biopsy mandatory. Exudative discoid and lichenoid chronic dermatosis (Sulzberger-Garbe) typically responds well to systemic steroids. Intrale-sional triamcinolone acetonide has been demonstrated to be effective in the treatment of a persistent penile plaque in exudative discoid and lichenoid dermatosis (Sulzberger-Garbe), and may be helpful in discontinuing or reducing systemic therapy to a level compatible with long-term treatment.     

Nerve biopsy findings in Niemann-Pick type II (NPC)

The severe infantile form of Niemann-Pick disease type II was diagnosed in a 4-year-old girl and confirmed by demonstrating in cultured skin fibroblasts a deficiency of low-density lipoprotein-stimulated cholesterol ester synthesis of < 5% of normal. Electrodiagnostic studies revealed changes of a predominantly demyelinating motor and sensory polyneuropathy. Light microscope and ultrastructural examination of a peroneal nerve biopsy showed unique changes. Compacted myelin sheaths were disproportionately thin with marked globular irregularities in single teased nerve fibres and evidence of chronic demyelination. The majority of axons were preserved but axonal spheroids and cytoskeletal abnormalities akin to neuroaxonal dystrophy were noted. Membrane-bound multilobulated lysosomal inclusions of floccular and electron-dense material were present in Schwann cells (SC), endoneurial fibroblasts, macrophages, pericytes and endothelial cells. SC of myelinated fibres were stuffed with whorls of concentric osmiophilic membranous profiles and electron-lucent material. The findings are diagnostic and differ from those of classical Niemann-Pick disease.     

Should punch biopsies be used when high-grade disease is suspected at initial colposcopic assessment? A prospective study

The reliability and applicability of colposcopically directed cervical punch biopsy was assessed in a sample of 170 paired punch and large loop excision of cervical transformation zone (LLETZ) specimens obtained from previously untreated women who had been selected for treatment on the basis of cytology and/or colposcopic findings and in whom the entire cervical transformation zone was visible. A single punch biopsy was taken immediately before the LLETZ, and all the specimens were reviewed by a single pathologist. Nine (5.3%) punch biopsies were inadequate. In terms of whether or not there was cervical intraepithelial neoplasia (CIN), the chance-corrected kappa analysis rated overall agreement as poor (kappa = 0.21, 95% confidence limits 0.02-0.39), whereas in terms of histologic grade, it was fair to moderate (kappa = 0.32, 95% confidence limits 0.23-0.42). Punch biopsy tended to underestimate the disease. The sensitivity and specificity of colposcopically directed punch biopsy for the detection of high-grade CIN was 74% and 91%, respectively, with positive- and negative predictive values of 97% and 48%, respectively. Two microinvasive and two intraepithelial glandular lesions were missed on punch biopsy. Punch biopsy should be avoided when high-grade disease is suspected.     

Carcinoma of the papilla of vater: Are endoscopic appearance and endoscopic biopsy discordant?

Carcinoma of the papilla of Vater is classified as periampullary cancer representing 5% of all gastrointestinal tract malignancies. Early and accurate diagnosis is important for those patients with a tumor of the papilla, as the prognosis is more favorable than in other periampullary neoplasms. Endoscopically obtained biopsies from suspicious papillae can detect an early tumor, although even for skilled pathologists it is often difficult to differentiate carcinomas from noninvasive lesions on the basis of forceps biopsies. The purpose of this study was to assess the preoperative diagnostic accuracy of duodenoscopy appearance and biopsy in all cases with suspicion of tumor. Thirty patients with suspicion of carcinoma of the papilla of Vater and with final diagnosis established by pancreatoduodenectomy were included in this retrospective study. In each case, a comparison was made between endoscopic biopsy and duodenoscopic appearance. Duodenoscopic appearance sensitivity and accuracy for malignancy were 86% and 83%, respectively, whereas endoscopic biopsy sensitivity and accuracy were 65% and 67%, respectively. Although preoperative diagnosis of carcinoma of the papilla of Vater is useful for making therapeutic decisions, the diagnostic value of the endoscopic appearance was superior to endoscopic biopsy in this series. Presented at the 2003 American Hepato-Pancreato-Biliary Association Congress, Miami, Florida, February 27-March 3, 2003. Supported by FADA-CAPES/PROP 200J (M.L.D.).     

Human immunodeficiency virus (HIV)-related chronic relapsing inflammatory demyelinating polyneuropathy with multifocal unusual onion bulbs in sural nerve biopsy

Summary This is obviously the first report on a case with a spontaneous sensu strictu relapsing variant of human immunodeficiency virus (HIV)-related polyneuropathy. Its manifestation preceded LAS. Intrathecal HIV-antibodies developed between the most severe third and fourth episode. Analysis of sural nerve biopsy was consistent with a multifocally accentuated chronic inflammatory demyelination, characterized by unusual onion bulb-like Schwann cell formations with irregular voluminous layers, electron density, aggregation of filaments, multiple indented nuclei, and numerous enclosed collagen pockets. A direct or immune-mediated indirect specific influence on Schwann cell morphology by HIV might be discussed. Virus-like particles and ultrastructural markers of HIV were not detectable.     

超声引导徒手穿刺活检在乳腺癌诊断中的应用

目的探讨高频超声引导徒手穿刺活检在乳腺癌诊断中的应用价值。方法回顾性分析36例乳腺癌经高频超声引导自动活检枪徒手穿刺活检情况。结果36例可疑乳腺癌经超声引导徒手穿刺活检均取得满意的病理材料,病理诊断为乳腺癌,与术后病理结果一致,诊断率100%,其中1例未明确病理组织学分型,对组织学分型诊断率为97.2%。所有穿刺活检病例均未出现严重并发症。结论乳腺癌应用高频超声定位徒手穿刺活检可以获得明确的病理诊断,操作简单方便、安全可靠。     

The Evolution of Nonimmune Histological Injury and Its Clinical Relevance in Adult-Sized Kidney Grafts in Pediatric Recipients

To describe the evolution, risk factors and impact of nonimmune histological injury after pediatric kidney transplantation, we analyzed 245 renal allograft protocol biopsies taken regularly from the time of transplantation to 2 years thereafter in 81 consecutive rejection-free pediatric recipients of an adult-sized kidney. Isometric tubular vacuolization was present early after transplantation was not progressive, and was associated with higher tacrolimus pre-dose trough levels. Chronic tubulo-interstitial damage and tubular microcalcifications were already noted at 3 months, were progressive and had a greater association with small recipient size, male donor gender, higher donor age and female recipient gender, but not with tacrolimus exposure. Renal function assessment showed that older recipients had a significant increase in absolute glomerular filtration rate with time after transplantation, which differed from small recipients who showed no increase. It is concluded that progressive, functionally relevant, nonimmune injury is detected early after adult-sized kidney transplantation in pediatric recipients. Renal graft ischemia associated with the donor-recipient size discrepancy appears to be a greater risk factor for this chronic histological injury, suggesting that the exploration of additional therapeutic approaches to increase allograft perfusion could further extend the graft survival benefit of adult-sized kidneys transplanted into small children.     

Clinical Significance of an Early Protocol Biopsy in Living-Donor Renal Transplantation: Ten-Year Experience at a Single Center

We report here our 10-year experience of a biopsy performed at day 14 after transplantation in 304 patients with stable graft function. The factors that may have influenced subclinical rejection were analyzed according to histology. The incidence of subclinical rejection was 13.2%. Addition of mycophenolate mofetile (MMF) as a primary immunosuppressant significantly decreased the incidence of subclinical rejection compared with patients without such treatment (odds ratio, 0.23; p < 0.05). On the other hand, HLA-DR antigen mismatch (odds ratio, 2.39) and unrelated donor (odds ratio, 2.10) were also significantly associated with decreased subclinical rejection (p < 0.05). The incidence of acute rejection in patients with normal findings was lower than in those with borderline changes or subclinical rejection (0.23 +/- 0.05 vs. 0.48 +/- 0.07 and 0.60 +/- 0.11, respectively; p < 0.05). The graft survival rates in patients with subclinical rejection were lower than in patients with normal or borderline changes at 1 (88.4% vs. 97.9% and 99.1%; p < 0.05), 5 (77.8% vs. 96.2% and 95.9%; p < 0.05) and 10 (62.3% vs. 96.2% and 93.7%; p < 0.05) years. Thus, a protocol biopsy performed on day 14 after transplantation is useful for predicting graft survival. Triple therapy including MMF, related donor and HLA-DR antigen match are important factors for reducing subclinical rejection in living-donor renal transplantation.