An Asian case of fibroblastic rheumatism: clinical, radiological, and histological features

We report a case of fibroblastic rheumatism (FR) in a 61-year-old woman. The patient showed sclerodactyly and polyarthritis that involved both her hands and feet joints. Levels of C-reactive protein and matrix metallopeptidase-3 were within normal range. We diagnosed her condition as FR according to both the clinical features characterized with the destructive change of multiple joints and the histological sample. This is the first FR published case of FR in an Asian individual, and 23 published cases were reviewed.     

Gorham-Stout disease: radiological,histological, and clinical features of 12 cases and review of literature

Clinical Rheumatology - Gorham-Stout disease (GSD) is an exceedingly rare disease characterized by progressive osteolysis and angiomatosis. We investigate the features of this disease and evaluate...     

Antiphospholipid syndrome and palindromic rheumatism: a new possible association

Objective The aim of this study was to report six patients with palindromic rheumatism (PR) in whom signs, symptoms, and/or serologic evidence of antiphospholipid syndrome (APS) developed.Methods The medical histories of the patients were reviewed with special emphasis on age, gender, duration of PR, and lapse of time until antiphospholipid antibodies were detected or APS was diagnosed. Three representative cases are described.Results Two patients were women and four were men. Their mean age was 49.3 years (range 36–80), and the mean duration of PR was 5.5 years (range 3–8). In all patients, raised titers of antiphospholipid antibodies were found on two or more occasions. Two patients developed clinical pictures compatible with APS, two showed symptoms which may be attributable for APS, and raised titers of antiphospholipid antibodies were found in only two.Conclusion It seems that the appearance of these two uncommon conditions together is more than coincidental and may point to a previously unreported clinical association.     

肺泡癌50例临床资料及31例高分辨率CT分析

目的分析肺泡癌的临床诊断及胸部 CT 的影像学特征,提高肺泡癌的诊断水平。方法对1993至2003年间我院收治的1050例新发肺癌病例中经病理证实的50例肺泡癌的临床资料及1998至2005年中31例患者的高分辨率 CT 影像进行分析。结果 50例肺泡癌患者中,女30例,男20例,平均年龄为(58.4±10.7)岁。最常见的首发症状为咳嗽(20/50)。24例因肺部阴影而就诊的患者中20例无任何临床症状,多为早期结节型,病程最长4年。31例高分辨率 CT 检查的患者中8例为粟粒型,11例表现为结节型的患者中小泡征7例,钙化征7例;12例肺炎型患者中9例次出现低CT 值的实变影、假空洞征、网状影、磨玻璃影,2例出现碎石路征和 CT 血管造影征(增强扫描造影剂高峰时低密度的实变中血管影清晰可见)。结论肺泡癌仅占肺癌的4.76%(50/1 050)。肺泡癌尤其是结节型患者无临床症状者比例较高,且病程较长;定期行胸部 X 线检查很重要;胸部 CT 的特征性表现有助于诊断和鉴别诊断。     

Pituitary xanthogranulomas: clinical features,radiological appearances and post-operative outcomes

Background

Xanthogranulomas are inflammatory masses most commonly found at peripheral sites such as the skin. Sellar and parasellar xanthogranulomas are rare and present a diagnostic challenge as they are difficult to differentiate from other sellar lesions such as craniopharyngiomas and Rathke’s cleft cysts pre-operatively. Their radiological imaging features are yet to be clearly defined, and clinical outcomes after surgery are also uncertain. This study reviews clinical presentation, radiological appearances, and clinical outcomes in a cohort of patients with pituitary xanthogranulomas.

Methods

A prospectively maintained pituitary surgery database was screened for histologically confirmed pituitary xanthogranulomas between May 2011–December 2016. Retrospective case note assessments were then performed by three independent reviewers. Patient demographics, clinical presentations, imaging, and clinical outcomes were analysed.

Results

During the study period 295 endoscopic endonasal pituitary surgeries were performed. Six patients had confirmed pituitary xanthogranulomas (2%). Patients most commonly presented with visual field deficits and/or endocrine dysfunction. Common imaging features included: a cystic consistency, hyperintensity on T1-weighted MR images, and contrast enhancement either peripherally (n?=?3) or homogenously (n?=?3). The most common pre-operative endocrine deficits were hyperprolactinaemia and hypoadrenalism (at least one of which was identified in 4/6 patients; 66%). Thirty-three percent (2/6) of patients presented with diabetes insipidus. The most common post-operative endocrinological deficits were adrenocortical dysfunction (66%) and gonadotropin deficiency (66%). Visual assessments normalised in all six patients post-operatively. Gross total resection was achieved in all patients, and at median follow up of 33.5 months there were no cases of tumour recurrence.

Conclusions

The prevalence of pituitary xanthogranulomas in our series is higher than that suggested in the literature. Surgery restored normal vision to all cases, however four patients (67%) required long-term hormonal replacement post-operatively. Imaging features such peripheral rim enhancement, a suprasellar tumour epicentre, and the absence of both calcification or cavernous sinus invasion were identified as potential indicators that together should alert clinicians to the possibility of pituitary xanthogranuloma when assessing patients with cystic sellar and parasellar tumours.

     

Late onset hepatic failure: clinical, serological and histological features

The clinical, laboratory and histological features of 47 patients with what is defined as late onset hepatic failure are reviewed. Twenty-five of the patients were female and 22 male with a median age of 45 years. Hepatic dysfunction was severe as evidenced by the prolongation of prothrombin time (median = 32 sec, range = 17 to 120 sec). In only four cases was a viral etiology proven (2 hepatitis B, 2 hepatitis A) although the similarity of the clinical features to patients with fulminant viral hepatitis--apart from the longer period of illness prior to the onset of encephalopathy (median = 9 weeks, range = 8 to 24 weeks)--made non-A, non-B infection a possibility in the remainder. There were also similarities to chronic active hepatitis with low titer antibodies to smooth muscle or antinuclear factor in 17% and elevation of the serum IgG in 49%. Liver biopsy in 5 of 8 survivors more than 1 year after initial presentation showed chronic active hepatitis in three. Lobular inflammatory infiltrate, bridging necrosis and multilobular collapse were the features of the acute stage of illness in both the survivors and fatal cases. The patients given corticosteroids did not have a statistically significant improvement in survival, and overall mortality for the series was 81%. Hepatic transplantation, successfully performed in one patient, would appear to offer the best chance of survival for the majority of these patients.     

Bacteraemic melioidosis pneumonia: impact on outcome, clinical and radiological features

OBJECTIVES: Melioidosis is an endemic disease in South-east (SE) Asia and bacteraemia in melioidosis is associated with high mortality. We describe some clinical and radiological features of bacteraemic pneumonia due to Burkholderia pseudomallei as well as a comparison with bacteraemic patients without pneumonia. METHODS: Patients with positive blood cultures for B. pseudomallei from October 1997 to November 2001 were included. Patients were grouped as 'Pneumonia' and 'Non-pneumonia' according to clinical and radiological features. RESULTS: Eighteen (60%) out of total 30 patients were in the pneumonia group. There was no significant difference in age, WBC count, platelet counts and bilirubin levels between the groups. However the 'Pneumonia' group had higher incidences of hyponatraemia, acidosis, diabetes with poor control, renal impairment and shorter length of stay. Twelve (66%) of 18 patients in the pneumonia group required ICU admission compared to none in the non-pneumonia group; all required mechanical ventilation. Only 13/30 (43%) patients had initial empiric antibiotic therapy that is appropriate for melioidosis. The pneumonia group also had significantly higher mortality (13/18, 72%) rate than the non-pneumonia group (3/12, 25%, P=0.03). Chest radiographs were non-specific. 7/18 (38%) had unilobar involvement of the lung, mostly left sided; the rest had multilobar or bilateral involvement. Six (33%) had pleural effusion. No patient had cavitary lung disease. Visceral abscesses (spleen, liver and prostate) were also common in ultrasound and CT scans in both groups. CONCLUSION: (1) Bacteraemic melioidosis with pneumonia carries high mortality with most patients dying early. (2) Radiological features of melioidosis pneumonia are non-specific. (3) Clinicians who treat patients from SE Asia need to be aware of this condition to institute early and appropriate antibiotic therapy.     

Giant sigmoid diverticulum: clinical and radiological features.

Two case reports of giant sigmoid diverticulum associated with diverticular disease of the sigmoid colon are presented. The clinical and radiological features of 30 similar cases found in the literature are reviewed. Our two cases represent the largest recorded diverticulum and the oldest recorded patient with this condition.     

Early and advanced stages in palindromic rheumatism patients: Test characteristics of three classification criteria and discrimination potential

Aim of the workIt is to identify the classification characteristics of diagnostic criteria in the early (≤3 year) and advanced stage (>3 year) of palindromic rheumatism (PR).Patients and methods269 patients of PR were selected out of those with recurrent intermittent arthritis and were divided into two groups with ≤3 year (n = 99) and >3 year (n = 170) disease duration. The clinical characteristics and the positive incidence according to 3 classification criteria: Pasero and Barbieri (1986); Hannonen et al. (1987) and Guerne and Weisman (1992) were compared. Furthermore, the positive incidences of each criterion were calculated.ResultsThe mean age of the patients was 39.2 ± 10.8 years and M:F 1.2:1. The agreement of the Hannonen et al. (1987) and Guerne and Weisman (1992) criteria was the highest with 0.63 of κ value. When the positive incidences by each criteria were compared between groups of the ≤3 year and >3 year, both Hannonen et al criteria (76.8% and 91.2%) and Guerne and Weisman (62.6% and 85.8%) were significantly different (p = 0.001). The criterion that showed the obvious difference between early and advanced stages were as follow: (Pasero and Barbieri) >5 attacks in the last 2 years and ≥3 joints involved in different attacks; (Hannonen et al.) subsequent attacks in at least three different joints and for (Guerne and Weisman) six-month history of brief-sudden-onset and recurrent episodes of monoarthritis and ≥3 joints involved in different attacks.ConclusionsThe individual PR criteria showed different classification characteristics in ≤3 year and >3 year.     

复发性风湿症63例临床分析及1.5～3年随访结果

目的 提高对复发性风湿症临床特点、治疗方法和预后的认识。方法 分析63例复发性风湿症患者的临床资料．观察他们对治疗的反应并对其进行1．5-3年的随访。结果 本组患者平均发病年龄（40±11）岁，平均病程（8±7）年。主要表现为复发性急性关节炎和（或）关节周围软组织炎症，全身大多数关节可累及．46％有发作诱因．67％发作频率逐年增加。非甾体类抗炎药和改变病情的抗风湿药对本症有不同程度的疗效。5％患者的症状完全消失，90％长期反复发作，还有5％进展为RA。结论 复发性风湿症是一种主要表现为复发性急性关节炎和（或）关节周围炎的临床综合征，青霉胺和磷酸氯喹对本症有效，其预后存在多样性。     

Breast cancer in Greenland-selected epidemiological,clinical, and histological features

Summary Fifty-seven breast cancers were diagnosed among indigenous Greenlandic women from 1950 to 1974. An additional 22 cases registered between 1975 and 1979 represent a minimum number and were only used as basis for minimum incidence rates. Changes in age-adjusted rate, age-specific incidence pattern, and relative risk were consistent with an upward shift from a population of low risk between 1950 and 1969 to one of intermediate risk from 1970 onward, a finding that relates well to increased urbanization and westernization. The risk of breast cancer in Greenland may be associated with consumption of saturated fats but is seemingly not correlated to total fat intake which has always been on a par with high-risk Danish levels. An association with diet may in reality have been stronger than suggested but weakened by a counterbalancing effect of high fertility, especially in the youngest age groups. Evaluation of histological features and survival did not suggest differences which could favorably compare with findings in white population groups contrary to reported results from the population of Japan, also one of low risk and of mongoloid origin. Further studies should consider dietary intakes, endocrine variations, and breast fluid secretion with special attention to girls at the age of menarche.This work has been supported by Sygekassernes Helsefond (Grant No. H 11/51-76)     

Autoimmune hepatitis. Physiopathologic, clinical, histological, and therapeutic features

Autoimmune hepatitis is characterized by an inflammation of the portal tract with lymphocytes and plasma cells, an hypergammaglobulinemia and a variety of circulating autoantibodies. The presence of smooth muscle antibodies and/or antinuclear antibodies define type 1. Type 2 is characterized by the presence of liver-kidney--microsomal antibodies. Environmental, genetic and infectious factors may explain the autoreactivity of T cells. Different non specific clinical features may be present. Sometimes the presentation may be an acute hepatitis; in the remainder, the disease may not be recognized until liver damage is advanced. Hypergammaglobulinemia and presence of circulating autoantibodies are the key for diagnosis. The association of prednisolone in combination with azathioprine remains the established treatment. If relapse or non response occur, other immunosuppressive therapy such as cyclosporin may be useful. Liver transplantation is reserved for (sub)fulminant forms and end stage liver disease.     

Chronic hepatitis C in elderly patients: clinical, histological and virological features

Some clinical, histological and virological features, efficacy and safety of interferon (IFN) therapy were evaluated in elderly patients with chronic hepatitis C (CHC). We enrolled 22 patients aged 65-75 (mean age: 68.3 +/- 3.17 years); 15 males and 7 females. In all cases the hepatitis C virus RNA (HCV-RNA) was determined before, during and after the therapy, and HCV sub-types were established; 15 patients underwent hepatobiopsy. At entry, the duration of disease was: 6 patients 1-3 years, 2 patients 4-10 years, 14 patients 11-30 years; alanine-aminotransferase (ALT) = (3.17 +/- 1.15) x N (N = normal value); aspartate-transaminase (AST) = 2.28 +/- 1.6 x N; gamma-glutamyl-transpeptidase (gGT) = 1.4 +/- 1.1 x N; platelets = 164,000 +/- 66,000/mm(3); histological pattern: 2 mild chronic active hepatitis (CAH), 5 CAH, 2 severe CAM, 6 CAH with liver cirrhosis (LC); histological activity index (HAI) (14 patients) = 11.14 +/- 4.5 (range 5-17); scores according to Scheuer: lobular 2.28 +/- 1.13, portal 2.71 +/- 0.99, fibrosis 2.35 +/-1.33; HCV-RNA +ve: 20 patients, HCV-RNA -ve: 2 patients; HCV-subtypes: 1b 20/20 (100%), 1b+1a 1/20 (5%), 1b+ 2a 1/20 (5%). Treatment was applied to 18 patients, for 3-12 months; 5 received alpha-IFN2a; 5 received alpha-IFN2b, 3 lymphoblastoid IFN, all at a dose of 3 mU thrice per week; 3 patients received 6 mU beta-IFN thrice per week. Therapy over 6 months was applied to 16 patients: Complete response (CR) was observed in 8 patients (50%), one of them was with long-term CR (over 12 months after therapy); 5 have had relapse and 2 patients are still under treatment. Partial response (PR) was observed in 4 patients (25%), no response (NR) in 4 patients (25%). Side effects were moderate and self-limited. Loss of HCV-RNA was shown in some patients with PR and in all patients with CR, but only temporarily.     

Distinctive clinical,psychological, and histological features of postinfective irritable bowel syndrome

OBJECTIVE: Irritable bowel syndrome after gastroenteritis is well recognized. Our aim was to determine whether postinfective IBS (PI-IBS) has histological or clinical features that are distinct from those of IBS patients with no history of preceding infection. METHODS: A total of 75 consecutive IBS outpatients and 36 healthy control subjects completed a questionnaire detailing symptoms, mode of onset, and previous psychiatric history. All underwent a full diagnostic workup including rectal biopsy, which included immunostaining and quantification for lamina propria or intraepithelial T lymphocytes, serotonin-containing enterochromaffin (EC), and mast cells. Patients were divided according to onset of symptoms into PI-IBS (n = 23) or non-PI-IBS (n = 52) patients. RESULTS: Diarrhea predominance occurred more frequently in PI-IBS (70%) than in non-PI-IBS (42%) patients (p = 0.03). A history of previous treatment for anxiety or depression was present in 26% of PI-IBS patients compared to 54% of non-PI-IBS (p = 0.02). Biopsy results for all patients were normal using conventional criteria; however, quantification revealed that PI-IBS showed increased EC cells compared to those of non-PI-IBS patients (p = 0.017) and controls (p = 0.02). Lamina propria T lymphocytes were increased in PI-IBS (p = 0.026) and non-PI-IBS (p = 0.011) patients compared to controls. Mast cells were increased in non-PI-IBS patients (p = 0.054) compared to controls. CONCLUSIONS: Individuals with PI-IBS are a clinically distinct subgroup characterized by diarrheal symptoms, less psychiatric illness, and increased serotonin-containing EC cells compared to those with non-PI-IBS.