Spontaneous Regression of Budd-Chiari Syndrome (Hepatic Venous Occlusion) in a Young Female

ABSTRACT. Hultcrantz R, Angelin B, Einarsson K, Friman L (Departments of Internal Medicine and Roentgenology, Serafimer Hospital, and Department of Internal Medicine, Huddinge University Hospital, Karolinska Institute, Stockholm, Sweden). Spontaneous regression of Budd-Chiari syndrome (hepatic venous occlusion) in a young female. Acta Med Scand 1987; 221:503–7. A case of occlusion of the hepatic veins in an 18-year-old girl is presented. The onset was sudden with massive ascites and markedly impaired general condition. The diagnosis was based on liver biopsy and angiograms of the caval and hepatic veins as well as of the celiac artery. No predisposing factors could be found. The patient was treated conservatively with laparo-centesis and diuretics. Clear improvement was seen after two weeks, and after four weeks she had no ascites and could be discharged. All liver function tests were then normalized. After three months, all diuretics could be withdrawn, and in the following 11 years she has remained completely recovered. The case illustrates that also widespread thrombi of the hepatic veins may sometimes rapidly dissolve spontaneously, with apparent total reconstitution of hepatic function. This case is unusual since previously reported cases have had high mortality rates and, in surviving cases, operative procedures or large doses of diuretics have been required to control the ascites.     

Is chronic inflammatory change in the prostate the major cause of rising serum prostate‐specific antigen in patients with clinical suspicion of prostate cancer?

AIM: To evaluate the cause of elevated prostate-specific antigen (PSA) in patients with transrectal needle biopsy negative for prostate cancer. METHODS: Serum PSA concentration, prostate volume, and pathologic findings were examined in 223 patients with negative biopsy for prostate cancer. The degree of prostate inflammation was determined by the extent and degree of inflammation shown by biopsy specimens and is expressed as an inflammation score (range: 0-36). RESULTS: A significant correlation was found between PSA concentration and prostate total volume (P=0.0001). Prostate chronic inflammation showed no correlation with PSA concentration (P=0.485, F=0.488). After allocating patients to normal PSA (4 ng/mL) groups, we found that serum PSA concentrations in both groups were predominantly affected by prostate total volume. CONCLUSIONS: An increase in prostate volume appears to be the major contributor to a high serum PSA concentration in patients with negative biopsy for prostate cancer. However, in contrast to previous reports, there was no correlation between the degree of prostate chronic inflammation and serum PSA concentrations.     

NATURE AND HEALING OF TIBIAL SHAFT FRACTURES IN ALCOHOL ABUSERS

Alcohol abuse is associated with an increased risk of osteopeniaand fractures. Previous histomorphometric studies on iliac crestbone have found decreased bone formation and increased boneresorption in alcohol abusers but it has not been establishedwhether alcohol abuse has any effect on the anatomical locationor the healing time of tibial shaft fractures. We studied, retrospectively,199 adult male patients hospitalized for isolated tibial shaftfracture in the city of Malmö, Sweden, between 1980 and1990. Forty-nine of the patients had earlier been registeredat the Department of Alcohol Diseases and were judged to beproblem drinkers. Abusers sustained their tibial shaft fracturesmore often by falling at ground level (P<0.0001) or froma higher level (P=0.009) and the fractures were more often obliquethan transverse (P=0.002) as compared with non-abusers. Healingtime was impaired in abusers who had sustained a transversefracture (P=0.035), but no difference was observed in healingtime in those with an oblique fracture. We found no differencebetween the abusers and the non-abusers regarding duration ofhospital stay, fracture location, amount of displacement, occurrenceof open fractures or the rate of complications.     

Laboratory screening method for selection of healthy volunteers

The aim of laboratory screening in Phase I is to exclude subjects with subclinical illness, who might be at increased risk in the study, and who might also adversely influence interpretation of the results. A new method for laboratory screening, based on Bayesian probability theory, is proposed, which consists of: 1. Drawing up a list of diseases to be excluded. 2. Defining for each disease, the maximum acceptable risk that an included subject could be affected by it. 3. Identifying one test for each disease. 4. Using a contingency table to calculate the specificity of the test and integrating the estimated prevalence of the disease from epidemiological data. 5. Applying the percentage obtained by the calculation of specificity to the previously determined distribution of values in the volunteer population to identify the threshold value for inclusion. Use of this deductive method in screening volunteers for Phase I trials affords increased security of selection, while reducing the number of non-pertinent exclusions because of laboratory findings.     

Dural sinus thrombosis: study using intermediate field strength MR imaging

The magnetic resonance (MR) images of six patients with thrombosis of a dural sinus were reviewed. The diagnosis had been verified by computed tomographic scans in three patients and arteriograms in two; in the sixth patient, only MR imaging was used to confirm the clinical syndrome. In all patients, high-intensity signal was seen from the thrombus within the affected dural sinus on all echoes. This persistent signal intensity allowed intravascular clot to be distinguished from normal causes of increased signal such as flow-related enhancement (entry phenomenon) and even-echo rephasing. MR imaging demonstrated the cause of the thrombosis in three patients: two were secondary to adjacent tumors, and one was secondary to unsuspected mastoiditis. Complications such as infarction were also demonstrated. Using MR imaging, one can easily and safely diagnose thrombosis of a dural sinus. MR should be the imaging method of choice in patients suspected of having thrombosis of a dural sinus.     

Neuropathy associated with “benign” anti-myelin-associated glycoprotein IgM gammopathy: Clinical,immunological, neurophysiological pathological findings and response to treatment in 33 cases

We studied 33 patients presenting with a peripheral neuropathy associated with non-malignant anti-myelin-associated glycoprotein (MAG) IgM monoclonal gammopathy (MG) in an attempt to delineate their clinical, immunological, electrophysiological and pathological characteristics; we also reviewed our experience concerning long-term follow-up and therapy. Peripheral neuropathy associated with non-malignant anti-MAG IgM MG was observed mostly in males (sex ratio 7.2), and mean age at onset was 67 years (range 46–81). A predominantly sensory pattern was noted in more than 80% of cases, although some patients were affected by a predominantly motor peripheral neuropathy. Although disease progression was slow in most cases, 45% of patients suffered severe disability, and in 2 cases, the patient's death appeared to stem directly from the neuropathy. The electrophysiological findings were indicative of a demyelinating process in 90% of cases, and electron microscopic examination of nerve biopsy specimens demonstrated widening of the myelin lamellae in more than 95% of cases. Most of our patients showed a disappointing response to steroids and chemotherapy or plasma exchanges. Intravenous immune globulin, evaluated in 17 patients, had a transient, mostly subjective effect in 35% and led to a clear-cut improvement in 24% of cases. We did not observe any correlation between the severity of the clinical picture and the anti-sulphoglucuronyl paragloboside antibody titre; in individual cases, clinical improvement occurred without lowering of IgM levels. Although the severity and the rate of progression may greatly vary from patient to patient, the combination of clinical, electrophysiological and pathological features delineates a characteristic pattern in peripheral neuropathy associated with non-malignant anti-MAG IgM MG.     

Adverse events in phase one studies: a study in 430 healthy volunteers

Summary All the clinical, laboratory and electrocardiographic adverse events detected during 24 Phase I studies in the same unit over a 5 y period are reported here. 430 healthy male volunteers were involved, corresponding to 5488 days of follow-up.The overall incidence of adverse events was 13.5%, with a significant difference between active drug (15.3%) and placebo (7.4%) treatments. There were 69 distinct types of adverse events. Headache was the most frequent symptom (2%). There were severe adverse events in 20 cases (0.36%), with an incidence of 20/430 per subject (4.6%). There were no deaths or life-threatening events.Although the main objective of Phase I studies is to determine the maximum dose tolerated, cause-effect relationships with adverse events are hard to establish, because of the frequency of adverse events with placebo, and because of the limited number of subjects included such studies.     

Autosomal dominant centronuclear myopathy]

In a family 6 members in 3 generations were affected by centronuclear myopathy (CNM) of autosomal dominant inheritance. The apparent onset was in the early forties and the disease progressed slowly. Limb weakness was predominant. Strabismus was present in 5 cases and calves hypertrophy in 3. Serum creatinine kinase was always within the normal range. In one case myotonic bursts were found at electromyography. In 2 cases brain stem auditory evoked potential studies demonstrated abnormal prolongation of interpeak latencies I-III and favoured subclinical nervous system involvement. Muscular biopsies showed typical features of centronuclear myopathy with 50 to 80% central nuclei. In two cases immunocytochemical labelling of dystrophin showed staining in the sarcoplasm in favour of an arrest in the morphogenesis of developing myofiber. Others families with autosomal dominant CNM in the literature and also some sporadic adult cases had similar clinical features.     

糖尿病周围神经病97例临床特征与神经电生理分析

目的:探讨糖尿病周围神经病(DPN)患者的临床特征与神经电生理变化。方法:分析97例DPN患者的临床特征,比较DPN组和对照组的神经传导速度(NCV)、远端潜伏期、远端波幅3个参数。结果:①临床特征以肢体麻木(59%)最多见、其次为疼痛(42%)。②患者组NCV、远端波幅值低于对照组,远端潜伏期比对照组延长;两组3个参数比较,除腓总神经远端波幅、尺神经感觉传导速度和正中、尺、腓肠神经远端潜伏期外,差异均有统计学意义(P<0.05)。结论:①DPN患者临床特征以肢体麻木和疼痛最多见;②检测NCV、远端潜伏期、远端波幅,能较早发现临床患者。     

Surgical Treatment of an Early Epileptic Encephalopathy with Suppression-Bursts and Focal Cortical Dysplasia

Summary: A case of early epileptic encephalopathy (EIEE) with suppression-bursts or Ohtahara's syndrome, associated with focal cortical dysplasia is reported. Infantile spasms and brief tonic unilateral seizures began on the fifth day of life. Interictal EEG demonstrated an asymmetrical "suppression-burst" pattern with no wake or sleep cycling. Seizures were refractory to all antiepileptic drug (AED) and steroid therapy. Magnetic resonance imaging (MRI) showed right frontotemporal cortical thickening. After three weeks of an ineffective medical treatment a preoperative evaluation with single photon emission computed tomography (SPECT) and electrocorticography (ECoG) was performed to characterize epileptogenic focus. Surgical resection of the precentral area was performed at age 1 month. Neuropathologic examinations confirmed diagnosis of focal cortical dysplasia by identifying cytoarchitectural disarray and ectopic neurons located deep in subcortical white matter. During follow-up, 1-year postoperative the child had a single febrile seizure. Neurologic examination showed minor developmental delay and slight left-sided weak ness.