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PubMedDate not supplied

Rewriting the histological classification of lupus nephritis.

The World Health Organization (WHO) classification of lupus (SLE) nephritis was published almost 20 years ago, and there is world-wide recognition of its utility in the diagnosis and treatment of SLE glomerulonephritis (GN). However, a number of problems have been recognized: The classification of severe segmental (WHO class III > or = 50%) and membranous glomerulonephritis (MGN) complicated by the lesions of severe segmental or diffuse GN (WHO classes Vc > or = 50% or Vd) is ambiguous; The implications of non-immune complex pathogenic mechanisms are not acknowledged and SLE interstitial nephritis and vasculitis are not included in the current classification. We propose a revision that retains the classes of the current WHO classification. Informed by investigations utilizing the WHO classification, it places severe segmental GN in class III (segmental GN) and mixed MGN and segmental and diffuse GN in class V (MGN). It optimizes the use of electron and fluorescence microscopy in defining controversial and ambiguous lesions. It develops subclasses based upon pathogenic insights gained since the advent of the WHO classification. Finally, it recognizes the need to include the disease specific lesions of SLE tubulointerstial nephritis and vasculitis.

Humans↗

PubMed1988

Amebic liver abscess in a European patient: zymodeme classification of Entamoeba histolytica.

This is a case report of a 36-year-old patient who developed an amebic liver abscess after a stay in the Sudan. He was first misdiagnosed as having pneumonia of the right lower lobe. Following establishment of the correct diagnosis, the patient recovered fully after metronidazole treatment. The fecal culture in Robinson's medium yielded extensive growth of Entamoeba histolytica. Electrophoretic characterization proved it to be a zymodeme XIX, which is one of the zymodemes associated with pathogenicity in the host. This first report of a zymodeme classification of E. histolytica in Germany should initiate further epidemiological studies.

Adult↗

PubMedDate not supplied

[Concept of freedom of infection of animal flocks].

Totally negative results of epidemiological investigation of random samples do not prove the absence of the infection as the pathogen may be restricted to only a few animals in the herd for a long time. The statement "absence of infection" is critical for such situations. The question is raised, whether the statement "absence of infection" should be generally avoided. Classification of herds and flocks according to the prevalence of the pathogen would be more valid for the implementation of control measures.

Animals↗

PubMed2003

Classification and rescue of ROMK mutations underlying hyperprostaglandin E syndrome/antenatal Bartter syndrome.

BACKGROUND: Mutations in the renal K+ channel ROMK (Kir 1.1) cause hyperprostaglandin E syndrome/antenatal Bartter syndrome (HPS/aBS), a severe tubular disorder leading to renal salt and water wasting. Several studies confirmed the predominance of alterations of current properties in ROMK mutants. However, in most of these studies, analysis was restricted to nonmammalian cells and electrophysiologic methods. Therefore, for the majority of ROMK mutations, disturbances in protein trafficking remained unclear. The aim of the present study was the evaluation of different pathogenic mechanisms of 20 naturally occurring ROMK mutations with consecutive classification into mutational classes and identification of distinct rescue mechanisms according to the underlying defect. METHODS: Mutated ROMK potassium channels were expressed in Xenopus oocytes and a human kidney cell line and analyzed by two electrode voltage clamp analysis, immunofluorescence, and Western blot analysis. RESULTS: We identified 14 out of 20 ROMK mutations that did not reach the cell surface, indicating defective membrane trafficking. High expression levels rescued six out of 14 ROMK mutants, leading to significant K+ currents. In addition, two early inframe stop mutations could be rescued by aminoglycosides, resulting in full-length ROMK and correct trafficking to the plasma membrane in a subset of transfected cells. CONCLUSION: In contrast to previous reports, most of the investigated ROMK mutations displayed a trafficking defect that might be rescued by pharmacologic agents acting as molecular chaperones. The evaluation of different disease-causing mechanisms will be essential for establishing new and more specific therapeutic strategies for HPS/aBS patients.

Animals↗

PubMed2001

Cerebellar/spinocerebellar syndromes.

Spinocerebellar syndromes are a heterogeneous group of neurological disorders clinically characterized by dysequilibrium, progressive incoordination of gait and limbs, and speech and eye movement disturbances. Clinical classification and differential diagnosis are intricate due to the great variability of the phenotypic, pathogenic, neuropathological and genetic aspects of these diseases. Spinocerebellar syndromes may present as sporadic, nongenetic, disorders or as familial forms. Clinical and genetic classifications of autosomal dominant and recessive spinocerebellar ataxias are briefly reviewed. Distinguishing clinical features, diagnostic procedures, and frequency of specific genotypes in Italian patients are presented.

Cerebellar Ataxia↗

PubMed2000

Primary vasculitides and vasculitis confined to skin: clinical features and new pathogenic aspects.

Cutaneous vasculitis is a heterogeneous group of disorders, and may occur with virtually all syndromes of vasculitis. It can occur as an isolated dermatologic disorder or as a manifestation of a potentially life-threatening systemic vasculitis. Cutaneous manifestations vary depending on the underlying cause, the size of the vessel involved and the severity and type of inflammation. In this short review, the classification, the characteristic skin manifestations of the primary vasculitides and new pathogenic aspects are discussed.

Animals↗

PubMed1999

Genetic analysis of turbot pathogenic Streptococcus parauberis strains by ribotyping and random amplified polymorphic DNA.

Ribotyping and RAPD profiling of a collection of 18 Streptococcus parauberis strains isolated from diseased turbot in Galicia (NW Spain) was performed in order to analyze the possible genetic variability within this bacterial fish pathogen. In addition, the value of this technique for intraspecific classification and epidemiological studies was evaluated. Ribopatterns of DNA digested with three endonucleases and hybridized with a cDNA probe complementary to highly conserved sequences in the 16S and 23S rRNA genes showed a great homogeneity among the turbot isolates. Compared with ribotyping, RAPD appeared to be a reliable and fast technique for discriminating between isolates of S. parauberis on the basis of their farm of isolation and, therefore, represents a powerful tool for epidemiological studies of this fish pathogen.

Animals↗

PubMed1975

[Epidemiology, terminology and statistical classification of intestinal infections].

On the basis of critical survey of literature data and analysis of morbidity of intestinal infections in 5 towns of Donbass for 20 years and also of studying 5148 case histories an attempt is made of rational explantation of the causes of growth during the last years of the incidence of Sonne dysentery salmonelloses, escherichioses and other diseases caused by conditionally-pathogenic microorganisms. The importance of improvement of nomenclature and of statistical classification of intestinal infections was demonstrated.

Bacterial Infections↗

PubMed1993

Prevalence of contagious pathogens of bovine mastitis and use of mastitis control practices.

A cross-sectional study of 1,032 dairy herds in Ohio was conducted to determine the prevalence of the major contagious pathogens of mastitis (Streptococcus agalactiae and Staphylococcus aureus) and the use of common mastitis control measures. Herd owners were surveyed by mail concerning their use of mastitis control measures. The survey focused on treatment of nonlactating cows, postmilking teat dipping, culling practices, milking machine maintenance, treatment for clinical mastitis, and premilking hygiene practices. Nearly 90% of questionnaires were returned. The prevalence of Streptococcus agalactiae and Staphylococcus aureus was determined by use of bulk-tank milk samples. Most herds (n = 802) met the criteria for classification into 1 of 4 groups: (1) Free of contagious pathogens, as determined by inability to isolate coagulase positive staphylococci (CPS) and esculin-negative CAMP positive streptococci (ENCPS) from 3 bulk-tank milk samples, (2) CPS, but not ENCPS, isolated from at least 1 sample, (3) ENCPS, but not CPS, isolated from at least 1 sample, (4) both ENCPS and CPS isolated from at least 1 sample. The number of herds in which both ENCPS and CPS were isolated was low; therefore, these herds were grouped with herds in which ENCPS alone was isolated for the evaluation of mastitis control practices related to herd pathogen status. Herd somatic cell count (SCC) was determined using Dairy Herd Improvement Association data by calculating the geometric mean SCC from individual cow test day SCC. Twelve months of SCC data from 741 herds were included in this study.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

PubMed1997

[Atherosclerosis. Formal pathogenesis, classification and functional significance].

Atherosclerosis and its complications determine the majority of deaths in the western world, followed by malignant tumors. The present work introduces a classification of stages of atherosclerotic disease based on relevant pathogenic and therapeutic concepts, elaborated by H. Stary. At the present, we are able to relate different lesion types to a time course and partly to interferences between participating cell populations as well as to special pathogenic stimuli. From the therapeutic view, this knowledge is fundamental for preventive as well as interventional strategies like gene therapy. Distinct atherosclerotic plaques reveal a different composition and architecture, which may account for the variable risk for further complications of lesions showing the same size and degree of stenosis. In combination with an advanced clinical and diagnostic characterization of atherosclerotic lesions, the present concept might contribute to a better and differential therapy of atherosclerosis.

Arterial Occlusive Diseases↗

PubMedDate not supplied

Metastatic bacterial endophthalmitis: a contemporary reappraisal.

Metastatic bacterial endophthalmitis remains a challenge to the clinician despite the success of antibiotics in reducing its frequency and severity. Controversy currently surrounds the management of this condition because of uncertainty about the value of and indications for vitreous surgery. We review 72 cases of metastatic endophthalmitis from the past decade, including five not previously published. The spectrum of causative bacteria changed significantly during this period, with displacement of meningococcus by Bacillus cereus as the most frequently reported agent and an increasing incidence of infection by organisms of low pathogenicity in immunologically compromised hosts. We propose a new classification scheme for metastatic endophthalmitis based on the location (anterior or posterior segment) and extent (focal or diffuse) of the primary intraocular infection. Focal and anterior cases appear to have a good prognosis, while posterior diffuse disease nearly always leads to blindness. Our analysis of outcomes suggests that systemic antibiotics are more valuable in metastatic than in postoperative or traumatic endophthalmitis and that intraocular antibiotic injection and vitrectomy make only a limited contribution to successful treatment in metastatic infection. We recommend a clinical approach to metastatic endophthalmitis that minimizes exposure of patients to the risks of invasive procedures.

Adolescent↗

PubMed1984

Primary inoculation complex of skin by Mycobacterium chelonei.

We report a patient with a primary inoculation complex of the skin caused by Mycobacterium chelonei, a facultative pathogen that belongs to group IV of Runyon's classification of atypical mycobacteria. This organism is seldom responsible for disease in humans, although cutaneous, pulmonary, heart, bone, and disseminated infections have been reported. An unusual cutaneous manifestation of this organism is presented with a review of the literature.

Child↗

PubMed1990

Acute chagasic cardiopathy produced by a strain of Trypanosoma cruzi (type I) in an experimental model.

We have carried out a study of the tissular tropism of the strain Y of T. cruzi considering that the different strains of T. cruzi show a great instability in their pathogenic properties with the aim of proving that the classifications of his parasite based in its tissular tropism are not reliable given that these characteristics are subject to change as can be seen by comparing our results with those of other authors. In our study, the Y. strain of T. cruzi shows a strong pancytotropic action, specially with marked cardiotropic aspects. The lack of affectation in the lymphatic ganglion and the small proportion of spleen lesions (8%) as well as the absence of pseudocysts at this level is surprising in a strain which was described as eminently reticulotropic. Our data show that this strain produces cardiac pseudocysts without lesions of the parasitized muscle fiber. The above mentioned data evidence that the biological behavior of a strain and specially its tissular tropism are susceptible to present variations with time.

Acute Disease↗

PubMed2026

Mast cell leukemia with complex genomic alterations in an elderly patient with prior hematologic and solid malignancies: a case report.

INTRODUCTION: Mast cell leukemia (MCL) is the rarest and most aggressive variant of systemic mastocytosis (approximately 1% of cases), with a median survival of under 2 years. Diagnosis requires ≥20% atypical mast cells in the marrow aspirate, and the disease frequently overlaps with myeloid neoplasms. CASE PRESENTATION: An 86-year-old man with paranasal sinus diffuse large B-cell lymphoma in remission since 2017 after R-CHOP and methotrexate, and prostate adenocarcinoma treated in 2019, presented with acute pancytopenia, presumed to represent lymphoma relapse. Serum tryptase exceeded 11 999 ng/mL; the aspirate showed 20% pleomorphic mast cells (CD117+, weak CD25, CD2-), confirming aleukemic MCL. Formal CMML criteria could not be confirmed due to the unavailability of monocyte differential data; however, the findings raised suspicion for an associated myeloid neoplasm, with SM with an associated hematological neoplasm remaining an alternative classification. Karyotyping was normal; next-generation sequencing revealed pathogenic variants in TP53, RB1, DAXX, ASXL1, TET2, and SRSF2, and a rare extracellular-domain KIT p.D419del. He declined inpatient midostaurin, deteriorated rapidly, and died 2 weeks later. CLINICAL DISCUSSION: This case illustrates a therapy-related MCL (plausible but unconfirmed given the non-leukemogenic profile of methotrexate and the focal nature of prostate stereotactic body radiation therapy) with a suspected associated myeloid neoplasm and complex pathogenic mutations. The KIT p.D419del extracellular domain variant is a rare non-D816V mutation; the canonical D816V was not detected on NGS, though the presence of a low-variant allele fraction D816V cannot be fully excluded due to assay sensitivity. Despite midostaurin, the disease remained aggressive. CONCLUSION: Persistent unexplained cytopenias warrant heightened suspicion of MCL, and comprehensive genomic profiling clarifies diagnosis, distinguishes overlapping myeloid disease, and informs prognosis in this aggressive, refractory neoplasm.

case report↗

PubMed1987

[Significance of Yersinia enterocolitica and thermophilic Campylobacter for water hygiene].

The taxonomy of Yersinia enterocolitica as well as their classification methods are still unreliable with respect to their pathogenicity. Therefore, a basis of evaluation was established by using extended biochemical tests (API-20-E, API-50-CH) and electrophoretic methods for identifying strains from human, food and water sources. All strains show great inhomogenicity, which was higher for non-human strains than for those isolated from clinical specimens. Thermophilic Campylobacters are especially prevalent in wild living fowl thus being a potential risk for surface water contamination. The results of the study show that high colony counts were found only during winter, whereas in summer the concentration lay below the infective dose. Furthermore, thermophilic Campylobacters were isolated only from samples that contained a higher number of fecal indicator organisms.

Bacteriological Techniques↗

PubMed1993

Epidermolysis bullosa acquisita and bullous systemic lupus erythematosus. Diseases of autoimmunity to type VII collagen.

Autoimmunity to C7 is a genetically predisposed condition that results in the production of predominantly IgG class basement membrane autoantibodies that may cause basement membrane damage and subepidermal blisters by at least two pathogenic mechanisms. Autoimmunity to C7 cuts across traditional disease classifications (EBA versus bullous SLE), presents with heterogeneous clinical and pathologic features, mimics other diseases, and may be difficult to diagnose and treat. Autoimmunity to C7 is associated with susceptibility to SLE and perhaps inflammatory bowel disease.

Autoantibodies↗

PubMed1997

Classification of Vibrio vulnificus strains by the carbohydrate composition of their capsular polysaccharides.

Pathogenic bacteria are often classified on the basis of the complex polysaccharides found on the surface, usually capsular polysaccharides or lipopolysaccharides. It is common in clinical practice to use reactivity with antisera specific to the various cell surface carbohydrates for this purpose. In this work, we describe a chemotyping method for bacterial capsular polysaccharides which is based on a carbohydrate analysis of an acid hydrolysate of the capsule. High-performance anion-exchange chromatography at high pH (HPAE) with electrochemical detection, which is used for analysis of the hydrolysate, shows preferential sensitivity for sugars. A single acid hydrolysis condition is chosen for screening a large collection of bacterial isolates and a computerized autosampler is used to make possible a large number of rapid analyses. This procedure does not yield a quantitative carbohydrate analysis for the sample but produces a fingerprint which can be used to discriminate among isolates which have different capsular polysaccharide structures. The procedure has been applied to a collection of 120 isolates of Vibrio vulnificus, a water-born species common in shellfish which causes septicemia in immunocompromised individuals, most often from eating of raw oysters. The collection of bacterial isolates includes strains from both clinical cases of septicemia and from such environmental sources such as sea water, sediments, and shellfish. Our results show that a number of unusual sugars including many amino sugars are found in these polysaccharides and that a wide variety of capsular carbotypes in V. vulnificus may be readily distinguished by the HPAE fingerprint.

Anion Exchange Resins↗

PubMed2002

Schizophrenia and bipolar disorder: similarities in pathogenic mechanisms but differences in neurodevelopment.

Over the past 100 years, the Kraepelinian classification of psychoses has dominated our approach to schizophrenia and bipolar disorder. However, controversy as to the nature of the illnesses--whether they can be viewed as completely distinct, essentially the same, or occupying different points along a psychosis spectrum--has intensified in recent years. This paper reviews the evidence for these differing opinions, examining both the commonalities between the two diseases and the distinctions. A genetic propensity towards psychotic disorders is widely acknowledged; more recent studies suggest a considerable overlap in genetic susceptibility to schizophrenia and bipolar disorder. The influence of early environmental effects, such as obstetric complications, on schizophrenia is also established but little such evidence exists for bipolar disorder. Structural abnormalities of the brain of developmental origin as well as neuropsychological deficits have been clearly identified in schizophrenia but less evidence has been found in bipolar disorder. The most plausible explanation is that one or more susceptible genes are shared between schizophrenia and bipolar illness, and can be thought of as predisposing individuals to psychosis, perhaps by producing a dysregulation of the dopaminergic response to stress. Other genes and environmental factors are likely to have more specific effects and contribute to producing the patterns that psychiatrists recognize as 'classical' schizophrenia and mania. In particular, genes involved in early cortical development and early neurodevelopmental insults causing developmental impairment may put individuals on a trajectory towards schizophrenia rather than bipolar illness.

Adult↗