Epidemiology of hepatitis C and G in sporadic and familial porphyria cutanea tarda

From 1995 to 1997, we prospectively evaluated the prevalence of hepatitis C virus (HCV) RNA in 124 patients with porphyria cutanea tarda (PCT) from Northern France (83 sporadic and 41 familial PCT). Serum samples were analyzed for ferritin, transaminases, HCV antibodies, and HCV RNA. In addition, genotyping of HCV and searches for HCV infection risk factors (blood transfusion, iv drug abuse, and surgical intervention) were performed. Twenty-six of 124 patients (21%; 95% CI: 13.9-28) were positive for serum HCV antibodies. All of them were also positive for HCV RNA. The prevalence of HCV infection was higher in the sporadic PCT group (26.5%, 22 out of 83) than in the familial PCT group (9.7%, 4 out of 41). Risk factors for hepatitis C infection were found to be significantly increased in the HCV-positive group when compared with the HCV-negative PCT group. In all HCV-positive patients with a risk factor, the suspected date of exposure to the virus always preceded the clinical onset of PCT. The HCV genotype pattern in PCT patients was similar to that observed in nonporphyric HCV patients in western European countries. Serum ferritin level was increased in both HCV-positive and HCV-negative porphyric patients. Transaminase levels were significantly higher in HCV-infected PCT patients. Sixty-seven out of 124 patients were retrospectively studied for hepatitis G virus (HGV) infection. Six of these 67 patients (8.9%; 95% CI: 2.1-15.8) were positive for HGV RNA. None of the six HGV-infected patients were positive for HCV RNA. The HGV-infected patients did not differ statistically from those without HGV infection with regard to age, ferritin, transaminase levels, and PCT treatment. These results support the view that sporadic cases of HGV infection may occur frequently. This study of a large cohort of HCV and PCT patients further documents an increasing gradient in HCV prevalence from northern to southern Europe, and shows that HCV infection acts as a triggering factor of PCT. Finally, the HGV prevalence found in the PCT patients was comparable with that found in French blood donors, suggesting that HGV is not a PCT triggering factor.     

Cutaneous pigmentation, only manifestation of porphyria cutanea tarda in a HIV-1 positive patient

We investigated dopamine D1 receptors in the putamen and caudate nucleus with positron emission tomography in six patients with narcolepsy and five healthy controls using [11C]NNC 756 as ligand. The caudate-to-cerebellum and putamen-to-cerebellum ratios of [11C]NNC 756 were within normal limits in patients with narcolepsy. No evidence of increased D1 receptor binding in narcolepsy was found.     

Cytochrome P450 induction, uroporphyrinogen decarboxylase depression, porphyrin accumulation and excretion, and gender influence in a 3-week rat model of porphyria cutanea tarda

An experimental model of porphyria cutanea tarda, consisting of depressed hepatic uroporphyrinogen decarboxylase (URO-D) activity and accumulation of highly carboxylated porphyrins in the liver, was produced in 3 weeks in Fischer 344 rats. A single administration of a polychlorinated biphenyl mixture (Aroclor 1254) to iron-loaded female rats maintained continuously on delta-aminolevulinic acid supplemented drinking water produced the porphyric state. Without iron loading, URO-D activity appeared slightly less inhibited (33% of normal vs 23% of normal) but porphyrin accumulation was dramatically less (70 vs 605 micrograms porphyrin/g liver). Similar treatment in male rats produced URO-D activities of 54 and 70% of normal with and without iron loading, respectively, and porphyrin concentrations of 76 and 17 micrograms/g. When hexachlorobenzene was substituted for Aroclor 1254 treatment in female rats, URO-D activity was 61 and 69% of normal (with and without iron loading, respectively) and liver porphyrin concentrations were 96 and 25 micrograms/g, respectively. Hexachlorobenzene did not produce significant porphyric effects in male rats. Aroclor 1254 induced CYP1A to a greater extent in females than in males and to a greater extent than hexachlorobenzene, which showed a greater propensity to induce CYP2B. Overall correlation between URO-D activity depression and porphyrin accumulation was highest when fitted to an exponential curve, indicating the importance of the extreme of the depression URO-D activity in evoking experimental porphyria cutanea tarda.     

Hepatic mass in Budd-Chiari syndrome: CT and MRI findings

The North Karelia Project, a community-based demonstration project for prevention of cardiovascular diseases since 1972 in Finland, was successful in reducing the population levels of the major cardiovascular risk factors. A net decline in risk factors and coronary heart disease mortality was observed in North Karelia in the 1970s. Thereafter, the mortality from coronary heart disease has declined markedly in all of Finland. The aim of the study was to find out how the cancer mortality has changed in North Karelia during this longer follow-up period. Age-adjusted mortality trends were calculated for the male population aged between 35 and 64 years in the province of North Karelia, and in all of Finland for the period 1969-91, using the official mortality data. The trends and the changes were calculated using general linear model procedures. During the 20-year period, cancer mortality declined in North Karelia by 45.4% and in all of Finland by 32.7% (P = 0.006 for difference). The greater decline in North Karelia occurred particularly in the second decade of the follow up, and lung cancer. The results support the hypothesis that reduction in the population levels of the cardiovascular risk factors lead to beneficial changes in cancer mortality rates, but such changes take longer time to manifest than for coronary heart disease.     

The C282Y mutation in the haemochromatosis gene (HFE) and hepatitis C virus infection are independent cofactors for porphyria cutanea tarda in Australian patients

The interaction of free allergen with two (or more) IgE molecules bound to the high-affinity receptor for IgE (FcepsilonRI) on mast cells and basophilic granulocytes results in the release of inflammatory mediators. The role of allergen-specific IgG antibodies in the allergic reaction in human beings is less clear. We produced two chimeric IgE antibodies, hIgE-Dp2A and hIgE-Dp2B, directed to two nonoverlapping epitopes (A and B) of the house dust mite allergen Der p 2. Chimeric IgG1 and IgG4 variants of these antibodies were produced also. Basophil activation by the house dust mite allergen Der p 2 was induced after sensitization of basophils with a mixture of chimeric hIgE-Dp2A and hIgE-Dp2B antibodies but not after sensitization by the individual IgE antibodies alone. Basophil activation was also shown after sensitization with hIgE-Dp2A and stimulation with Der p 2 incubated with hIgG1-Dp2B or hIgG4-Dp2B antibodies. Both IgE and IgG antibodies directed to the other nonoverlapping epitope complemented the sensitization by the hIgE-Dp2A antibody. Nonsensitized basophils were not activated by the Der p 2/hIgG-Dp2 mixtures. These results indicate that allergen-specific IgG can complement an IgE-dependent reaction and therefore under certain conditions can act as an anaphylactic antibody.     

CT and MRI features of cavernous haemangioma of internal auditory canal

A left internal auditory canal (IAC) cavernous haemangioma is reported in a 45-year-old Saudi male. The lesion was associated with rapidly deteriorating hearing loss and facial nerve dysfunction. CT showed a calcified enhanced IAC lesion while T1 weighted MRI showed an isointense contrast enhancing lesion bulging into the porus acousticus. The imaging features of the three usual IAC lesions--meningioma, acoustic neuroma and cavernous haemangioma--were compared. Calcification/ossification appear more commonly in cavernous haemangioma than in the other two lesions while facial nerve dysfunction is a clinical hallmark of IAC cavernous haemangioma.     

Clinical features of relative focal hyperfusion in patients with intracerebral hemorrhage detected by contrast-enhanced xenon CT

Optimization of the skeletal muscle contraction during cardiac assist is important to achieve maximal cardiac assist and yet avoid overstimulation that may injure skeletal muscle. Dynamic cardiomyoplasty suffers from lack of an objective, reproducible, and accurate technique to measure skeletal muscle shortening fraction after wrap and training of the muscle. A recruitment curve is considered the best way to select the proper stimulation level to achieve supramaximal contraction without overstimulating the muscle. A fluoroscopic technique of determining latissimus dorsi recruitment curve was evaluated in five goats undergoing dynamic cardiomyoplasty with an anterior cardio-subcutaneous wrap. Two pairs of stainless steel targets (0.5 and 1 cm of diameter) were implanted on each side of the muscle wrap. One pair of sonomicrometer crystals was also implanted. Displacement of the targets was measured under fluoroscopy at five different stimulation levels. Correlation coefficients between targets on the inside surface of the wrap and the sonomicrometer crystals, and targets on the outside surface of the wrap and the sonomicrometer crystals were 0.71 (P < 0.05) and 0.60 (P < 0.05), respectively. Targets on the inside surface of the wrap were more accurate than targets on the outside surface of the wrap for measurement of skeletal muscle shortening fraction and establishment of a recruitment curve. Adverse effects from the targets were not observed.     

Sonography, CT, CT sialography, MRI and MRI sialography in investigation of the facial nerve and the differentiation between deep and superficial parotid lesions

Three types of veneer restorations (VRs) were evaluated for recognition by two groups of observers to study the aesthetic result. The different types of VRs were: porcelain, direct resin composite and indirect resin composite. One month after insertion of the VRs, colour transparencies were made of smiling patients randomly selected from a group of 112 patients participating in a clinical trial. The slides were evaluated by five dentists who were not familiar with the patients and by 25 beautician students (BS). Dentists were asked to locate the VRs which were present in the patients and to specify the type of VR. BS were only asked to locate the VRs. To trace a possible relationship between the aesthetic result of the treatment and a number of variables, ANOVA was applied to evaluate the variables: 'discolouration of the teeth' before treatment, 'type of VR' and 'number of VRs'. Agreements in judgement were expressed in Cohen-Kappa coefficients. The results showed that the dentists could locate the VRs quite well (Kappa coefficient 0.64 +/- 0.28) but for BS this was lower (Kappa coefficient 0.43 +/- 0.27). The more VRs were made in one patient, the more difficult it was to locate them correctly. The other variables had no significant effect on the recognition of the VRs. It was not possible for the dentist observers to differentiate between the types of VRs.     

Hepatic hamartoma in adults. Apropos of a case documented by MRI

A mixed hamartoma of the liver in a 39 year old man is reported. Abdominal ultrasound revealed a 4 cm inhomogeneous echogenic mass with acoustic shadowing. MRI T1 weighted images showed a inhomogeneous low intensity mass with lower gadolinium enhancement than normal liver, a moderate low signal intensity on proton density weighted images, and heterogeneous isosignal intensity on T2 weighted images. Peripheric calcifications were found on pathologic examination.     

Value of MRI, CT and findings in staging of gynecologic malignancies

PURPOSE: The present study was designed to compare the value of MRI, CT and clinical examination in local tumor staging of gynaecologic malignancies. PATIENTS AND METHODS: 99 patients with clinically suspected carcinoma of the cervix uteri, ovarian carcinoma or tumor recurrence after gynaecologic cancer underwent all three staging modalities. Furthermore CT and MRI were compared in detecting lymph node metastasis and peritoneal implants. RESULTS: MRI was superior to CT and clinical staging in local tumor staging with an accuracy of 77% for cervical carcinoma and of 88% for recurrent tumors, whereas CT achieved an accuracy of 65% and 55% and clinical staging 60% and 63% accuracy for carcinoma of the cervix and recurrent cancer. Especially for local staging of these two tumor entities MRI is very useful. MRI and CT reached comparable results in the detection of ovarian tumors with an accuracy of 73% for MRI and 69% for CI. Both imaging modalities also showed equal results in the detection of lymph node metastasis, so that primary the cost saving use of CT tumor staging for ovarian lesions and lymph node metastasis should be favoured.     

MRI and clinical features in amyotrophic lateral sclerosis

MRI of the brain and spinal cord was performed in 21 patients with amyotrophic lateral sclerosis (ALS), 8 normal volunteers and 16 neurological disease controls. High signal was seen in the intracranial corticospinal tract in 16 of the 21 patients on T2-weighted and in 10 on proton density (PD)-weighted images. In one patient, the high signal on T2-weighted images became less marked with progression of the disease. Low signal intensity was seen in the motor cortex in 12 of the 21 patients. High signal in the anterolateral column of the spinal cord on T1 weighted images was seen in 14, and high signal in the lateral corticospinal tract on T2 weighted images was seen in 7 of the 21 patients. The relationship between the abnormal images and upper motor neurone signs remained unclear. High signal intensity was seen in the corticospinal tract in the brain on T2-weighted images in two normal volunteers and four disease controls, and on PD weighted images in three disease controls. Low signal intensity in the motor cortex on T2 weighted images was seen in three normal volunteers and four disease controls. However, high signal intensity was seen in the intracranial corticospinal tract on T1 weighted images in five patients with ALS who showed pronounced upper motor neurone signs including spastic paraparesis, but not in controls. Thus, abnormalities on MRI in the brain and spinal cord should be considered in the diagnosis of ALS, and high signal intensity of the intracranial corticospinal tract on T1-weighted images may reflect the severe pathological changes of the upper motor neurones in ALS.     

Langerhans' cell histiocytosis and juvenile xanthogranuloma of the orbit. Clinicopathologic, CT, and MR imaging features

The clinical, radiologic, and histopathologic features of two main disorders of the orbit are discussed. Group I, Langerhans cell histiocytosis (histiocytosis X, Class I), is caused by proliferation of X histiocytic Langerhans' cells. Group II is juvenile xanthogranuloma, and Class II is related to the proliferation of non-X histiocytic (monocyte-macrophage) cells. The two diseases are of unknown cause and differ in their clinical, radiologic, and histopathologic features.     

Catecholamine uptake, accumulation, and release in acute porphyria

Hypertension and tachycardia are well known features of acute porphyria and have been shown to be related to increased circulating catecholamines. The mechanism by which circulating catecholamines are increased was studied using the isolated perfused rat heart and human platelets as a model of adrenergic neuronal function. It was found that neither delta-aminolevulinate (ALA) nor porphobilinogen (PBG) blocked uptake or caused release in the isolated perfused rat heart. Platelets from six patients with acute prophyria, three in remission and three latent, with matching normal controls were studied with regard to their uptake of [(3)H]norepinephrine in the presence of ALA or PBG. It was found that ALA and PBG significantly reduced uptake and accumulation of [(3)H]-norepinephrine in patients with acute porphyria; however, no similar reduction in uptake and accumulation was observed in the platelets of normal controls. Therefore, it appears that there is a latent defect in the catecholamine uptake and (or) accumulation of platelets of patients with acute prophyria which only manifests itself in the presence of ALA or PBG. If platelet uptake serves as a model of adrenergic neuron uptake, this suggests that elevated circulating catecholamine levels during acute attacks of acute porphyria are caused at least partially by blockade of re-uptake into the sympathetic neurons.     

Imaging of spinal intradural arachnoid cysts: MRI, myelography and CT

Imaging studies in eight patients with surgically-confirmed spinal arachnoid cysts were analyzed retrospectively. All patients had preoperative MRI of the spine and seven preoperative myelography with postmyelographic CT. In all cases the correct diagnosis could be made preoperatively on the basis solely of MRI. The diagnosis could also be established from myelography and postmyelographic CT in six of the seven cases. In one case myelography and CT simply demonstrated an intradural extramedullary mass.     

Cystic meningioma: neuroradiological (MRI, CT) and macroscopic intraoperative appearance. A case report

Differential diagnosis of intracranial cystic meningiomas may present difficulties in about 10-15% of cases where anatomo-pathological alterations such as intratumoral necrosis, cystic cavity, hemorrhage or lipomatous infiltration are present. These alterations are responsible for an unusual radiological appearance which may suggest a false diagnosis. We describe a case of meningioma with a cystic appearance in which MRI was more helpful than CT, because it suggested an extra-axial meningiomatous lesion and thus allowed more precise surgical planning.     

1,5 Tesla MRI in focal nodular hyperplasia. Review of the literature apropos of 7 cases

There are increasing reports of unusual clinical features and atypical courses of syphilis in patients with acquired immunodeficiency syndrome. Recently, we had the opportunity to study an HIV-positive female patient with strong manifestations of secondary syphilis. The case is discussed together with the implications of secondary syphilis in her concomitant ocular affliction. Moreover, we comment on the clinico-therapeutic controversies brought about by the association of infection with Treponema pallidum and HIV.     

SPECT, CT, and MRI in head injury: acute abnormalities followed up at six months

We report a case of cervicofacial necrotizing fasciitis that developed after blepharoplasty, an occurrence that, to our knowledge, has not previously been reported in the medical literature. A patient who presented to our institution 3 days after undergoing blepharoplasty of the upper eyelid was diagnosed as having fulminant fasciitis involving extensive areas of the face, scalp, and neck. We review the case in detail and discuss clinical and radiological clues to diagnosis, surgical and medical management, wound care, and subsequent scar contracture. This case emphasizes the need for individualized, appropriate postoperative care and for an awareness of this rare, potentially fatal complication. Early recognition and aggressive treatment of cervicofacial fasciitis can arrest its rapid progression and prevent devastating sequelae.