Presentation
A 44 year old male presented with pigmentary changes at the macula noted by his optician. Best corrected visual acuity at presentation was good in both eyes. [ncbi.nlm.nih.gov]
Entire Body System
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Fatigue
After chemotherapy, she felt fatigued and noticed decreased urine output. Her serum creatinine level had risen from 0.8 to 10.1 mg/dL (71 to 893 micromol/L). [ncbi.nlm.nih.gov]
As kidney disease progresses, symptoms become more noticeable: Weight gain from the kidneys' inability to eliminate excess fluid Edema or swelling around the eyes, and in the ankles and feet Confusion and/or fatigue Loss of appetite How DDD is diagnosed [davita.com]
[…] respiratory infections Azotemia : Patients may develop acute kidney injury with the acute nephritic syndrome, which usually correlates with crescentic transformation on histology; other patients may present with advanced chronic renal insufficiency Fatigue [emedicine.medscape.com]
[…] haemolysis, anaphylaxis, decreased appetite, dizziness, dysgeusia (taste distortion), hypotension, dyspnoea and other respiratory tract symptoms, gastrointestinal upset, rash and pruritus, alopecia, muscle and joint pain, oedema, pyrexia, chills and fatigue [nice.org.uk]
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Streptococcal Pharyngitis
At 12 years of age, he was found to have macrohematuria and overt proteinuria with hypocomplementemia 2 months after streptococcal pharyngitis. [ncbi.nlm.nih.gov]
Musculoskeletal
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Restless Legs Syndrome
Complications include heart disease, bone disease, gastro-intestinal disturbances, nervous system problems such as peripheral neuropathy and restless leg syndrome (RLS), dry skin and itching, anemia, high blood pressure and a decreased ability to fight [davita.com]
Eyes
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Colorblindness
Drusen may cause colorblindness, night vision impairment and loss of vision. Treatment for DDD Like all autoimmune diseases, DDD is not curable. [davita.com]
Workup
Patients who fail to respond to treatment are referred for early kidney transplant workup and the philosophy of this group is to avoid dialysis if possible. [mayo.edu]
A low-grade plasma cell clone is less frequent. 5, 92 Accordingly, the initial workup should include a careful search for a clonal B-cell population among peripheral blood and bone marrow lymphocytes, including phenotypic and immunoglobulin gene rearrangement [bloodjournal.org]
Urine
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Pyuria
Patients with crescents on their initial renal biopsy or with large numbers of polymorphs in glomerular capillaries corresponding with sterile pyuria were more likely to have deterioration of renal function. [ncbi.nlm.nih.gov]
However, it can have a wide range of other clinical presentations, including sterile pyuria, isolated macroscopic hematuria, microscopic hematuria with subnephrotic range proteinuria, and isolated proteinuria. [renalfellow.blogspot.com]
Clinical findings such as heavy proteinuria, macroscopic hematuria, and sterile pyuria portend a worse outcome for patients. [path.upmc.edu]
In contrast, nephritic syndrome is characterized by: hematuria, pyuria, hypertension, oliguria, and azotemia. [pathwaymedicine.org]
Microbiology
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Complement Fixing Antibody
A 20-year-old man developed pneumonia and glomerulonephritis concomitantly with significantly rising Mycoplasma pneumoniae complement-fixing antibody titres. Renal biopsy showed mesangiocapillary glomerulonephritis type II (dense deposit disease). [ncbi.nlm.nih.gov]
Treatment
Median age at treatment start was 8.4 (range, 5.9-13) years. For three treatment episodes, eculizumab was the sole immunosuppressive treatment. [ncbi.nlm.nih.gov]
Prognosis
The disease occurs predominantly in children and young adults and the prognosis is variable. DDD varies considerably in incidence among different populations and has not been reported in Chinese. [ncbi.nlm.nih.gov]
The patients in whom the C3 deposits were confined to the mesangium appeared to have a better prognosis. CONCLUSIONS: The histological recurrence rate of DDD is high. [academic.oup.com]
The long-term prognosis to retain native kidney function is usually poor. [link.springer.com]
The differential diagnosis between DDD and C3GN is essential both for the treatment and for prognosis. DDD is a very rare glomerulopathy with an incidence of 2–3:1 million people. [sjkdt.org]
Etiology
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]
Over the last 30 years, marked differences in etiology and pathogenesis between type I membranoproliferative glomerulonephritis and dense deposit disease have become apparent. [ncbi.nlm.nih.gov]
Although the etiology and pathogenesis of MPGN II are unknown, it frequently progresses to end-stage renal failure. Typically in MPGN II, hypocomplementemia due to activation of the alternative complement pathway is present. [chikd.org]
MPGN may be idiopathic or secondary in etiology; most of the idiopathic and secondary MPGN exhibit similar pathological appearances [ 2 ]. [omicsonline.org]
“Membranoproliferative Glomerulonephritis” Image created by Lecturio Etiology of Membranoproliferative Glomerulonephritis If the etiology of membranoproliferative glomerulonephritis is unknown, then it is called primary or idiopathic MPGN. [lecturio.com]
Epidemiology
New chapters on glomerulonephritis associated with complement disorders, interventional treatments for hypertension, renal disease and cancer, and epidemiology and prognostic impact of acute kidney injury. [books.google.de]
We actively track the epidemiologic patterns in glomerular disease through the Mayo Clinic renal biopsy database. [mayo.edu]
Epidemiology – Indian scenario 7. [de.slideshare.net]
& alternative complement pathways Chronic serum sickness caused by repeated injection of antigen resembles MPGN Secondary Causes Many different etiologies can lead to 2 MPGN-type pattern Therefore, 1 MPGN is a diagnosis of exclusion CLINICAL ISSUES Epidemiology [basicmedicalkey.com]
Pathophysiology
We discuss the pathophysiology of C3 glomerulopathy, with evidence for alternative pathway dysregulation obtained from affected individuals and complement factor H (Cfh)-deficient animal models. [ncbi.nlm.nih.gov]
Prevention
For the latter patients, plasma replacement therapy prevents renal failure, but for the majority of patients, there is no proven effective treatment. [ncbi.nlm.nih.gov]