scholarly journals The Convergence of Vasculopathy and Vasculitis: Computer Mapping Analysis of 2 Renal Biopsies in a Patient with both Systemic Sclerosis and ANCA-Related Vasculitis

2018 ◽  
Vol 8 (1) ◽  
pp. 35-44
Author(s):  
Jia Xu ◽  
Milan Rosen ◽  
Christina I. Luffman ◽  
Charles Law ◽  
Anita Laloo ◽  
...  
Keyword(s):  
Antineutrophil Cytoplasmic Antibodies ◽  
Laboratory Findings ◽  
Computer Mapping ◽  
Renal Biopsies ◽  
Mapping Analysis ◽  
Healing Phase ◽  
Initial Biopsy ◽  
Mapping Techniques ◽  
Almost All ◽  
Glomerular Tuft

Scleroderma vasculopathy and ANCA (antineutrophil cytoplasmic antibodies)-associated glomerulonephritis have rarely been reported to occur simultaneously in one patient. Herein, we report a patient who presented with a classic constellation of clinical and laboratory findings of systemic scleroderma and was subsequently found to be positive for p-ANCA. Two renal biopsies, performed 5 months apart, demonstrated typical changes of the two entities in both acute and “healed” phases, which were analyzed by computer mapping techniques. The two renal biopsies were serially sectioned and stained routinely, and with CD31 and CD34 as endothelial markers. The slides were digitized, aligned and analyzed. Each glomerular tuft was sequentially studied in terms of total area (µm2) and each biopsy was individually profiled. All arterial vessels were sequentially studied with whole vessel and luminal areas delineated and ratios calculated. The initial biopsy contained 32 glomeruli almost all with extensive fibrinoid necrosis and destruction of the capillary network. The arterial vessels (interlobular and arcuate) showed intimal edema with luminal occlusion. CD31/CD34 stains showed variable endothelial intactness but demonstrated the luminal size shifts. The second biopsy had 37 glomeruli that were either segmentally or globally sclerotic with no active changes. The vessels were now normally patent. Each glomerular tuft and arterial vessel in both biopsies was analyzed as a serial section histogram documenting these changes. These studies depict the rare occurrence of two entities together, the scleroderma kidney vasculopathy and the glomerulonephritis of ANCA-associated vasculitis syndrome both in an acute and healing phase, profiled by computer mapping techniques.

scholarly journals Lupus-like nephritis with positive anti-neutrophil cytoplasmic antibodies and negative antinuclear antibodies

2020 ◽  
Author(s):  
Joana Eugénio Santos ◽  
Rita Vicente ◽  
Beatriz Malvar ◽  
Iolanda Santos ◽  
Miguel Coimbra ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Antinuclear Antibodies ◽  
Kidney Biopsy ◽  
Kidney Injury ◽  
Steroid Treatment ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Small Vessel Vasculitis ◽  
Laboratory Findings ◽  
Clinical Criteria ◽  
Immunological Markers

Abstract Antineutrophil cytoplasmic antibodies (ANCAs) are associated with small vessel vasculitis but their prevalence is not rare in other immune diseases. In lupus nephritis (LN), their pathological role and clinical relevance have been the target of controversial views. We present a case of acute kidney injury and nephrotic syndrome in a young woman with diffuse global proliferative and membranous nephritis on her kidney biopsy, showing a full-house immunofluorescence pattern, very allusive of class IV + V LN, but lacking associated clinical criteria and laboratory findings to support the diagnosis of systemic lupus erythematosus (SLE). Furthermore, the patient presented with high titers of ANCA, steadily decreasing alongside the renal function and proteinuria improvements, with mycophenolate mofetil (MMF) and steroid treatment. The authors believe this is a case of lupus-like nephritis, in which ANCAs are immunological markers, although they are not directly involved in the pathogenesis.

scholarly journals A Case of Pyruvate Carboxylase Deficiency With Longer Survival and Normal Laboratory Findings

2021 ◽  
Author(s):  
Reza Bayat ◽  
Shahin Koohmanaee ◽  
Nejat Mahdieh ◽  
Fatemeh Kharaee ◽  
Maryam Shahrokhi ◽  
...  
Keyword(s):  
Pyruvate Carboxylase ◽  
Amino Acid Level ◽  
Tca Cycle ◽  
Type B ◽  
Laboratory Findings ◽  
Short Life Span ◽  
Pyruvate Carboxylase Deficiency ◽  
Almost All ◽  
Cycle Regulation ◽  
Recessive Defect

Pyruvate carboxylase deficiency (PCD) is a rare autosomal recessive defect in a biotin-containing enzyme, Pyruvate carboxylase, which is considered as an enzyme of TCA-cycle regulation, gluconeogenesis, lipogenesis, and biosynthesis of neurotransmitters. Increased lactate to pyruvate ratio and decreased three hydroxybutyrates to acetoacetate are the main biochemical features of PCD. The elevated level of Citrulline, Proline, and Lysine with a short life span has been reported previously. Patients’ survival in almost all cases is below three months. Here, the authors aimed to report a girl with manifestations of Type B of PCD and longer survival (two-year and four-month-old). This patient did not have any changes in amino acid level, which was a unique case of Type B of PCD.

scholarly journals Laboratory Profiles of Pleural effusion Collected from Children: Experience at a Tertiary Care Hospital in Bangladesh

2013 ◽  
Vol 5 (1) ◽  
pp. 26-27
Author(s):  
Mahmudul Hasan ◽  
Md Rafiqul Islam ◽  
Abdul Matin ◽  
Ranjit Ranjan Roy ◽  
Md Abdullah Yusuf ◽  
...  
Keyword(s):  
Pleural Effusion ◽  
Pleural Fluid ◽  
Tertiary Care ◽  
Cross Sectional Study ◽  
Care Hospital ◽  
College Hospital ◽  
Laboratory Findings ◽  
Cross Sectional ◽  
Medical College ◽  
Almost All

Background: Pleural effusion occurs in many reasons. Laboratory tests are necessary to find out the causes. Objective: This study was an attempt to know the laboratory findings of pleural effusion. Methodology: This cross-sectional study of thirty (30) admitted cases with pleural effusion confirmed by chest radiography and aspiration of pleural fluid from one (1) year to twelve (12) years age of either sex were collected purposively. This study was carried out from July 2009 to February 2010 in the Department of Pediatrics at Rajshahi Medical College Hospital. All information were recorded in pre tested semi structured questionnaire. Results: Color of pleural fluid was straw in 56.7%, clear in 30.0%, blood stained in 13.3%. In this study, lymphocyte predominance among 56.7% cases, acid fast bacilli in 3.3% cases and raised protein of more than 3gm/dl and sugar less then 60mg/dl in 93.3% cases in pleural fluid. Conclusion: In almost all cases protein is raised and sugar is less in pleural fluid. DOI: http://dx.doi.org/10.3329/jssmc.v5i1.16201 J Shaheed Suhrawardy Med Coll, 2013;5(1):26-27

Brucellosis: A Retrospective Evaluation

2003 ◽  
Vol 33 (3) ◽  
pp. 151-153 ◽  
Author(s):  
Meltem Işikgöz Taşbakan ◽  
Tansu Yamazhan ◽  
Deniz Gökengin ◽  
Bilgin Arda ◽  
Mahmut Sertpolat ◽  
...  
Keyword(s):  
Diagnostic Techniques ◽  
Test Results ◽  
Duration Of Treatment ◽  
Laboratory Findings ◽  
Treatment Regimens ◽  
Unpasteurized Milk ◽  
Clinical Presentations ◽  
History Of ◽  
Almost All ◽  
Antimicrobial Regimen

One hundred and sixty-six presumed brucellosis patients were included in the study. These patients were classified as primary (91), relapse (18) and suspected (57) cases according to their clinical presentations, and serologic and microbiologic test results. Primary and relapse cases were evaluated retrospectively according to age, sex, residence, routes of transmission, clinical and laboratory findings, treatment regimens, duration of treatment, and relapse rates. Of the 109 primary and relapse patients, 57 were male and 52 female. The ages of the patients ranged between 16–75 (mean age 40.2). The percentages of the urban and rural residence of the patients were 41.3% and 58.7%, respectively. The most common mode of transmission was consumption of unpasteurized milk and milk products (67.9%). Malaise, fever and sweating were the most frequently observed symptoms (96.3%, 95.4%, 91.7%, respectively). The most common signs were fever (97.2%), splenomegaly (59.6%), and hepatomegaly (37.6%). The liver was the most frequently involved organ (21.1%). Almost all (99.1%) patients were serologically positive. However, the positivity rate of culture was low (15.6%). The most frequently preferred antimicrobial regimen was rifampin and doxycycline combination. The relapse rate was 8.3%. Brucellosis is still prevalent in Turkey as in many other countries in the Mediterranean basin. The clinical presentation of the disease may show regional variations. Patients with a history of occupational or nutritional contact with the bacterium and with a compatible clinical picture should be examined using appropriate diagnostic techniques before any attempt to prescribe an antimicrobial.

Q-fever presenting as an autoimmune disease: case report and review

Open Medicine ◽  
2011 ◽  
Vol 6 (6) ◽  
pp. 727-731 ◽  
Author(s):  
Maria Gamaletsou ◽  
Achilleas Gikas ◽  
Nikolaos Sipsas
Keyword(s):  
Autoimmune Disease ◽  
Monoclonal Gammopathy ◽  
Erythema Nodosum ◽  
Q Fever ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Laboratory Findings ◽  
Chronic Q Fever ◽  
Disease Case ◽  
Antibodies Monoclonal ◽  
Autoimmune Phenomena

AbstractQ fever is a worldwide zoonosis caused by the intracellular bacterium Coxiella burnetti. Autoimmune phenomena associated with the disease may obscure the clinical picture, and in many reports mislead physicians to an initial diagnosis of an autoimmune disease. We present a case of chronic Q-fever, complicated by myocarditis/pericarditis, where patient’s initial signs, symptoms and laboratory findings (i.e., protracted fever, oligoarthritis, erythema nodosum, positive antineutrophil cytoplasmic antibodies, monoclonal gammopathy) seemed to suggest an autoimmune disease. We also review the literature for autoimmune phenomena associated with Q-fever.

scholarly journals Cocaine-Induced Midline Destructive Lesions (CIMDL): A Real Challenge in Diagnosis

2021 ◽  
Vol 18 (15) ◽  
pp. 7831
Author(s):  
Michele Di Cosola ◽  
Mariateresa Ambrosino ◽  
Luisa Limongelli ◽  
Gianfranco Favia ◽  
Andrea Santarelli ◽  
...  
Keyword(s):  
Complete Blood Count ◽  
Correct Diagnosis ◽  
Venereal Disease ◽  
Antibody Test ◽  
Diagnostic Procedures ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Laboratory Findings ◽  
Detailed Medical History ◽  
Recurrent Sinus ◽  
Conclusive Diagnosis

The prolonged use of intranasal cocaine can destroy the nasal architecture with the erosion of the palate, turbinates, and ethmoid sinuses causing cocaine-induced midline lesions (CIMDL). The CIMDL display a clinical pattern mimicking variable diseases. The aim of this study was to highlight the difficulties in reaching a correct diagnosis through the evaluation of eight new cases. The diagnostic procedures followed in these patients included: detailed medical history, clinical and histological examination, computed tomography and magnetic resonance imaging, laboratory findings (complete blood count, sedimentation rate, antinuclear antibody test, rheumatoid factor, venereal disease research laboratory test, leishmaniasis and fungal serology, antineutrophil cytoplasmic antibodies ANCA test), and chest X-ray. All patients complained of epistaxis, halitosis, nasal scabs and obstruction, decreased sense of smell and/or taste, oro-nasal regurgitation of solids and liquids with recurrent sinus infections, and chronic facial pain. On clinical examination, all patients showed palate perforation with variable nasal structure involvement and presented a strong positivity for ANCA tests with a p-ANCA pattern. The followed protocol for the CIMDL diagnosis allowed for a relatively quick and conclusive diagnosis in all patients. A multidisciplinary approach is mandatory in the management of CIMDL, involving dental professionals, maxillofacial surgeons, and psychologists.

scholarly journals MON-693 Strongyloides Stercoralis Hyper Infestation Syndrome in Type 2 Diabetes: An ANCA Positive Case Report from India

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Kalpana Dash ◽  
Surekha Tippisetty ◽  
Vamsi Krishna Kolukula
Keyword(s):  
Molecular Mimicry ◽  
Past History ◽  
Parasite Load ◽  
Strongyloides Stercoralis ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Total Leukocyte Count ◽  
Stool Examination ◽  
Laboratory Findings ◽  
Precise Diagnosis ◽  
History Of

Abstract A 53-year old male patient with T2D and hypertension for 13 years, presented to Apollo Sugar Clinic, Raipur with recurrent abdomen pain, but no vomiting, constipation, diarrhea or fever. The patient has a past history of asthmatic bronchitis, recurrent eosinophilia, and one month prior to hospitalization, recovered from erythematous maculopapular serpentine rash measuring 6.0 x 1.5 cm over the abdomen post anti-allergic treatment. The patient was neither suffering from immunosuppressive condition nor was on immunosuppressant therapy, had normal vitals but the laboratory findings revealed high total leukocyte count and raised absolute eosinophil count (52%). The provisional diagnosis was made as hypereosinophilic enteritis in a long-standing T2D and hypertension. Immunological tests antinuclear antibody (ANA) and antineutrophil cytoplasmic antibodies (ANCA) resulted in positive ruling out vasculitis. Stool examination detected rhabditiform larvae of S Stercoralis and diagnosed as Strongyloides hyper infestation syndrome. The patient was managed with IV antibiotics, IV fluids, IV insulin and when abdomen pain reduced started with the oral diet. To remove larvae load, unlike routine treatment, the patient was put on albendazole and ivermectin for three consecutive days. After 15 days follow-up patient was completely asymptomatic; at 6 weeks TLC, stool test, ANCA and ANA titer were negative indicating no parasite load. These antibodies detected could be due to molecular mimicry triggered by parasite antigens which may help in diagnosing and monitoring the disease course. ANA and ANCA positive results have been rarely reported in the past for S stercoralis. This unique case of S stercoralis infestation in T2D may enlighten the health care physicians to investigate for this infestation in immunocompromised T2D patients with pain abdomen. Precise diagnosis with timely management can prevent steroid therapy due to eosinophilic enteritis which can be harmful to the patients.

scholarly journals Clinical presentations and long-term prognosis of childhood-onset polyarteritis nodosa in a single centre: a retrospective study

2020 ◽  
Author(s):  
Jeong-Seon Lee ◽  
JoongGon Kim ◽  
Soyoung Lee
Keyword(s):  
Polyarteritis Nodosa ◽  
Treatment Guidelines ◽  
Clinical Manifestations ◽  
Diagnosis And Treatment ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Childhood Onset ◽  
Single Centre ◽  
Laboratory Findings ◽  
Paediatric Patients

Abstract Background: Polyarteritis nodosa (PAN) is a systematic necrotizing vasculitis involving medium-sized arteries. Childhood-onset PAN ranges from mild to severe systematic disease causing damage and early mortality. As it is a rare disease in children, there is less study on clinical features, diagnosis and treatment than adults. And diagnosis occurs relatively late in most paediatric patients with polyarteritis nodosa. Therefore, this study aimed to investigate clinical manifestations, laboratory findings, treatment strategies, and long-term outcomes among patients with childhood-onset polyarteritis nodosa treated at a single-centre in Korea. We further aimed to evaluate the usefulness of the Birmingham Vasculitis Activity Score (BVAS) and Five-Factor Score (FFS) in paediatric patients.Methods: We retrospectively analysed data collected from patients with childhood-onset PAN treated at our institution from March 2003 to February 2020. Results: Nine patients (6 male and 3 female) were included in the study. The median ages at symptom onset and diagnosis were 7.6 (3–17.5) and 7.7 (3.5–17.6) years, respectively. The median follow-up duration was 7.0 (1.6–16.3) years. Eight patients were diagnosed with systemic PAN, while one was diagnosed with cutaneous PAN. All patients exhibited skin manifestations, while five exhibited Raynaud’s phenomenon. Organ involvement was observed in one patient. Constitutional symptoms such as fever, arthralgia, myalgia, and weight loss were also observed. Antineutrophil cytoplasmic antibodies were absent in all patients. The median BVAS at diagnosis was 8 (range: 2–29), and one patient had an FFS of 1. Prednisolone was initially used for induction in all patients, and other drugs were added in cases refractory to prednisolone. All patients survived despite some complications such as intracranial haemorrhage or digital necrosis requiring amputation.Conclusions: Early diagnosis and treatment may minimise sequelae in patients with childhood-onset PAN. Further multicentre studies are required to clarify the unique characteristics of childhood-onset PAN and establish treatment guidelines, early detection strategies, and biomarkers. Trial registration: Retrospectively registered.

scholarly journals Acute debut of neuroacanthocytosis in clinical practice

2016 ◽  
Vol 97 (6) ◽  
pp. 971-973
Author(s):  
K L Zagidullina ◽  
N A Popova
Keyword(s):  
Clinical Case ◽  
Autosomal Dominant ◽  
Correct Diagnosis ◽  
Cognitive Disorders ◽  
Blood Analysis ◽  
Whole Body ◽  
Laboratory Findings ◽  
Dominant Type ◽  
Dominant Disease ◽  
Almost All

Neuroacanthocytosis is a rare autosomal dominant disease, which in its clinical manifestation is characterized by choreiform hyperkinesis, mental and cognitive disorders, signs of polyneuropathy and cardiomyopathy, and the basis of the disease is presence of modified erythrocytes (acanthocytes) in peripheral blood. The disease is characterized by autosomal dominant type of inheritance (the gene was mapped on chromosome 9q21), sporadic cases are possible. Description of a clinical case of a 63-year old patient with neuroacanthocytosis delivered by an ambulance with a preliminary diagnosis of stroke is provided. The patient complained of severe general fatigue, whole body shivering, and involuntary compulsive uncontrollable movements in the limbs, body, and face. The patient noted changes in her voice, probably due to compulsive movements of her tongue, lightheadedness, and shaky walk. Almost all physical and laboratory findings were within normal. Taking into account acuteness of the disease, its attack at the time of hypertensive emergency and patient’s age, circular cause was suggested, computed tomography of the brain and magnetic resonance imaging were performed. The results of neuroimaging ruled out the pathology of cerebral circulation. Ultrasound of neck vessels revealed nothing abnormal. Purposeful repeated blood analysis revealed that 85% of erythrocytes were acanthocytes and after that the correct diagnosis was made. The peculiarities of this clinical case are acute manifestation of symptoms and relatively late debut of the disease.

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