Cases reported "Nephrotic Syndrome"

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1/8. Heavy chain deposition disease: the disease spectrum.

    A 45-year-old white woman was found to have microscopic hematuria during her annual physical examination. After a negative urologic workup, she returned 5 months later with nephrotic syndrome, renal insufficiency, and hypocomplementemia. Renal biopsy showed a nodular sclerosing glomerulopathy that could not be further characterized because of inadequate tissue for immunofluorescence. The patient returned 8 months later with chronic renal failure. A repeat renal biopsy showed deposits composed of immunoglobulin g (IgG) heavy chain and complement components C3 and C1 along glomerular, tubular, and vascular basement membranes, with negativity for kappa and lambda light chains, findings consistent with heavy chain deposition disease (HCDD). The heavy chain subclass was exclusively IgG3. Staining with monoclonal antibodies to epitopes of the constant domains of IgG heavy chain showed a CH1 deletion, indicating a truncated heavy chain. On review of the previously reported cases of HCDD, common clinical presentations include nephrotic syndrome, renal insufficiency, hematuria, and, in some cases, hypocomplementemia. In most patients, the hematologic disorder is mild, without overt myeloma. light microscopy shows a nodular sclerosing glomerulopathy, and heavy chain deposits are detectable within basement membranes throughout the kidney by immunofluorescence and electron microscopy. There is no effective treatment for this condition, and virtually all patients progress to chronic renal failure.
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2/8. Congenital nephrotic syndrome responsive to captopril and indometacin.

    Two children with congenital nephrotic syndrome are described (one with Finnish-type nephrosis, the other with diffuse mesangial sclerosis). Both children have had a prolonged and sustained clinical response with good physical health and normal growth patterns using captopril and indometacin as their sole treatment. No adverse effects have been noted. We recommend a trial of indometacin and captopril treatment in cases of congenital nephrotic syndrome.
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3/8. Congenital nephrotic syndrome with clinical hypothyroidism.

    A 15 month old boy with typical features of congenital nephrotic syndrome (CNS) is reported, who in addition to the renal pathology had an associated clinical hypothyroidism with low levels of total and free thyroxine and triiodothyronine and an elevated serum TSH. Improvement in the physical parameters and mental status from thyroid hormone replacement therapy is documented.
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4/8. Two paraneoplastic syndromes in a patient with ovarian cancer: nephrotic syndrome and paraneoplastic cerebellar degeneration.

    BACKGROUND: A paraneoplastic syndrome-a rare, indirect system disturbance located distant from a neoplasm-has been reported in association with ovarian cancer. CASE: Two paraneoplastic syndromes-nephrotic syndrome and paraneoplastic cerebellar degeneration (PCD)-developed in a patient who had no symptoms from ovarian cancer. She presented with the nephrotic syndrome. Evaluation for metastasis, including computed tomography (CT) scan and ultrasonography, showed a slightly enlarged right ovary and a 2-cm lower paraaortic lymph node on the right side. A 5-cm ovarian mass and multiple paraaortic lymph nodes were excised, and six cycles of carboplatin and paclitaxel were given. nephrotic syndrome symptoms had greatly subsided at 6 weeks after surgery. At 7 months after surgery, symptoms of PCD developed yet CT, CA-125 levels, and physical examination showed no evidence of ovarian cancer. CONCLUSION: To our knowledge, development of two paraneoplastic syndromes in one patient with ovarian cancer has not been reported. nephrotic syndrome resolved with treatment of the neoplasm whereas PCD did not.
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5/8. Latent cerebral hypoperfusion in a boy with persistent nephrotic syndrome.

    Pediatricians are often faced with both bad temper and decreased daily activity in children with persistent nephrotic syndrome. These problems, which might affect both mental and physical development, have been explained as a consequence of general fatigue due to systemic edema and/or long-term hospitalization. However, other factors, such as cerebral hypoperfusion, may be involved. We experienced a case of a boy with steroid-resistant nephrotic syndrome who showed diffuse cerebral hypoperfusion on single photon emission computed tomography. Diffuse cerebral hypoperfusion dramatically resolved as the nephrotic syndrome remitted. His bad temper, decreased daily activity, and delay of speech and motor development also improved. In our patient, cerebral hypoperfusion might have been associated with his mental problems, physical problems, and delayed development of speech.
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6/8. Arterial thrombosis due to minimal change glomerulopathy secondary to nonsteroidal anti-inflammatory drugs.

    The authors report a 49-year-old woman who had been treated with diclofenac for her back pain. nephrotic syndrome followed by occlusion of the right profound femoral and popliteal arteries occurred. Successful thrombectomy was performed. Another nonsteroidal anti-inflammatory drug (NSAID) was administered by injection during hospitalization. Renal biopsy showed minimal change glomerulopathy (MCG). Remission was delayed despite large-dose steroid administration. The delayed remission in this patient may be caused by impaired renal function and the possible effect of a second period of NSAID administration. The authors present the first case of femoral arterial thrombosis associated with nephrotic syndrome secondary to NSAID-associated MCG. Careful history-taking and physical examination may detect early causes and complications of nephrotic syndrome. Any rechallenge of NSAID should be avoided.
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7/8. Cerebral venous thrombosis in adult nephrotic syndrome due to systemic amyloidosis.

    Although venous thrombosis is one of the common complications in nephrotic patients, cerebral venous thrombosis (CVT) is rarely reported. CVT is so difficult to be detected by conventional diagnostic methods that it is sometimes overlooked despite its potential severity. We report a 79-year-old female with nephrotic syndrome due to systemic amyloidosis who suddenly altered mental status during her hospitalization. The underlying etiology had been not identified by physical examinations, various laboratory data, and repeated computed tomography, and finally she died. The post-mortem examination showed a massive thrombus impacted in intracranial left-sided transverse and sigmoid sinus. This case suggests that CVT can occur in a nephrotic patient who presents unexplained neurological signs and symptoms, which might not be detected only through conventional diagnostic tests.
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8/8. Concurrent FSGS and Hodgkin's lymphoma: case report and literature review on the link between nephrotic glomerulopathies and hematological malignancies.

    BACKGROUND: The link between the nephrotic syndrome (NS) and malignancy was first described in 1922. In solid tumors, the NS is most often due to membranous glomerulonephropathy, whereas in common hematological malignancies, minimal-change disease predominates. Focal segmental glomerulosclerosis (FSGS) is among the least frequently reported renal lesion associated with malignancy. methods: We report a case of the simultaneous diagnoses of FSGS and Hodgkin's lymphoma, and review the literature on various nephrotic glomerulonephropathies associated with common leukemia and lymphoma. RESULTS: Although nephrotic glomerulonephropathies rarely occur in association with acute leukemia, they have often been described in chronic lymphocytic leukemia (CLL). Membranoproliferative glomerulonephropathy and membranous glomerulonephropathy are the most common lesions observed in CLL. Nephrotic glomerulonephropathies have also been well documented among patients with lymphomas, in particular, Hodgkin's lymphoma. While minimal-change disease is most commonly found in association with Hodgkin's lymphoma, more diverse and complex renal lesions are associated with non-Hodgkin's lymphoma. FSGS remains a rare association with hematological malignancies. CONCLUSIONS: Nephrotic glomerulonephropathies are not only linked to solid-organ tumors, but also to hematological malignancies. A thorough evaluation, including a physical examination for lymphadenopathy and organomegaly, as well as a hematological evaluation, must be performed in all patients presenting with nephrotic glomerulonephropathies.
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