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The paper reports two cases of lipoprotein glomerulopathy (LPG) in children. The Sanger sequencing results in 2 cases indicated apolipoprotein E gene mutation[c.127 (exon3) C>T, p.R43C (p.Arg43Cys); c.494 (exon4) G>C, p.R165P (p.Arg165Pro),respectively]. Renal pathological presentation of two children showed that a large number of lipoprotein emboli were formed in the glomerular capillary loop, and the diagnosis of LPG was confirmed. The onset of LPG has no specific clinical manifestation, which is easy to be undiagnosed or misdiagnosed. Renal biopsy is a diagnostic means, glucocorticoid treatment is ineffective, and long-term lipid-lowering treatment may be required for LPG.
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Objective To discuss the clinical characteristics and prognosis of lipoprotein glomerulopathy (LPG) in chil-dren. Method Clinical data of one pediatric LPG patient were retrospectively analyzed. The clinical features and prognosis of childhood LPG were summarized based on literature review. Results A nine years old girl presented with frequent urination. The ifrst urine test revealed hematuria and proteinuria. After one week anti-infection treatment, the hematuria and proteinuria were continued. The serum albumin was slightly reduced. The hyperlipidemia and mild anemia were emerged. Kidney biopsy showed that enlarged glomeruli, with dilated capillary loops and weak eosinophilic lipoprotein thrombi in the capillary lumina under the light microscope;layered or tuftedemboluscontaining particulated lipid vacuoles under electron microscope. Gene sequencing identified APOE Tokyo (Leu141-Lys143→0). The diagnosis of LPG was confirmed. The lipid-lowering therapy was administrated and the disease was alleviated. Conclusion LPG is a rare disease in children. The level of blood lipid was signiifcantly increased, and the hormone therapy was ineffective. Kidney biopsy is the main basis for diagnosis. The genetic testing can prompt the genetic background. Lipid lowering therapy can relieve the progress of the disease.
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Objective:To provide reference for the treatment of lipoprotein glomerulopathy and to investigate the participation of clinical pharmacists in the whole treatment course. Methods: Three different treatment regimens, including immunosuppressive thera-py, dual plasma filtration therapy and lipid-lowering combined with reducing urinary protein therapy was respectively adopted for one patient with lipoprotein glomerulopathy, and the efficacy was evaluated. Clinical pharmacists assisted physicians in deciding treatment regimen, performed pharmaceutical care, adjusted medication and analyzed the prognosis of the patient during the follow-up. Results:1. Immunosuppressive therapy was ineffective for the patient;2. The dual filtration acted quickly, while the expense was high and the disease was easy to relapse;3. The third therapy was relatively safer and more economical with long-term effect. Conclusion:Combi-nation therapy of lowering lipid and reducing urinary protein is the most suitable treatment regimen for the patient with lipoprotein glo-merulopathy. Clinical pharmacists play an important role in the whole course of treatment to assist physicians in obtaining the maximum benefit of patients.
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To report a Chinese female adult suffering with lipoprotein glomerulopathy. Methods Light , electron microscopy and immunofluorescence were performed with renal tissue from biopsy. Results Mass proteinuria and nephrotic syndrome were seen as clinical features in this patient. Obvious expansion of glomerular capillary cavities, full of positive sudan 3 lipoprotein thrombi, was observed. Electron microscopy demonstrated cavities were full of various foaming lipid deposition in cluster and layer arrangement. Repeat renal biopsy after two months found expansive cavities decreased remarkably and lipoprotein thrombi were replaced by mesangial proliferation and sectional sclerosis gradually. Conclusion This case is diagnosed by pathology.
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Objective To further elucidate the clinical manifestations and pathological characteristics of lipoprotein glomerulopathy (LPG) . Methods Data of 7 LPG patients were reviewed retrospectively. Clinical manifestations were recorded on the day of renal biopsy. Biochemical profiles of lipid and lipoproteins were examined. Plasma concentration of apoE was determined with radial immunodiffusion assay. Biopsy specimens were processed for light microscopy, immunohistochemistry staining and electron microscopy. Glomerular deposition of apoA, apoB and apoE were detected using monoclonal antibodies on cryostatic sections in all patients. Results All the seven patients presented with edema, microscopic hematuria, heavy proteinuria, anemia and enlarged kidney size. Most of them, the levels of serum creatinine were normal. Biochemical profile revealed that the levels of triglyceride, apoB and apoE were elevated markedly. In all cases, increments of glomerular size and lipoprotein thrombi that occupied capillary lumica in the glomeruli were observed. Immunohistochemistry staining showed that the thrombi were strongly positive for apoA, apoB and apoE. Granules and various size of vacuoles in the thrombi were observed under electronic microscopy. Conclusion Compared with the previous reports of LPG from other countries, some unique clinical and pathological features are found in this group of Chinese LPG patients.