Abstract：
BACKGROUND:Congenital nephrotic syndrome is an uncommon disorder that may be caused by several diseases. These may be inherited, sporadic, acquired or part of a general malformation syndrome. METHODS:We reviewed the clinical characteristics, pathologic findings, and results of medical management in 30 infants who presented to Jordan University Hospital with congenital nephrotic syndrome in the years 1989 to 1999. RESULTS:Most patients (80%) had parents who were consanguineous. Most patients (80%) were born premature, with an average gestational age of 36 weeks. Most infants (77%) presented the nephrotic syndrome in the first three months of life and 26 (87%) had significant growth retardation. Twenty-five verified episodes of serious bacterial infections occurred in 18 patients. Antibiotic therapy however was successful in all these episodes. Light microscopy of the renal biopsies was consistent with the Finnish type of congenital nephrosis in most patients (83%). Chronic renal insufficiency developed in 17, and five of them needed chronic peritoneal dialysis. Most patients were given albumin transfusion and diuretic therapy especially during episodes of severe edema. Captopril alone or in combination with ibuprofen was given to eight patients, but without a response in any of them. All patients died before the age of 5 years. Most deaths occurred at an average age of 15 months (range 1-60). CONCLUSION:The Finnish type of congenital nephrosis was the most common type in our patients, most of whom died within a few months of the onset of disease. In the developing countries, the management of patients with congenital nephrosis may have to be different from that in the developed countries in view of the high cost of medical management, poor outcome, high risk of serious complications, and high mortality rate.
journal_name
J Nephroljournal_title
Journal of nephrologyauthors
Hamed RM,Shomaf Msubject
Has Abstractpub_date
2001-03-01 00:00:00pages
104-9issue
2eissn
1121-8428issn
1724-6059journal_volume
14pub_type
杂志文章abstract：:The increase in progressive kidney disease, rising numbers of patients with end-stage renal disease, organ shortages for kidney transplants and poor long-term graft survival rates underline the need for better strategies to diagnose, prevent and treat renal disease. Histological analysis, based on renal biopsies and r...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：10.5301/jn.5000205
更新日期：2012-09-01 00:00:00
abstract：:Henoch-Schonlein purpura is a clinical syndrome characterized by the association of skin, joint, and gastrointestinal symptoms. Henoch-Schonlein purpura is characterized by wide-spread vasculitis. Although the clinical symptoms of this disease are characteristic, the diagnosis is not always easy to establish because o...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2005-11-01 00:00:00
abstract：:Some patients with essential hypertension manifest increased urinary albumin excretion (UAE). Hypertensive patients with microalbuminuria manifest abnormal circadian variation of blood pressure, increased serum levels of LDL-cholesterol and lipoprotein(a), a greater rise of serum insulin in response to an oral glucose...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：1997-07-01 00:00:00
abstract：:The clinical manifestations of diabetic nephropathy are similar in type 1 and type 2 diabetes, while the renal lesions may differ. Indeed, diabetic glomerulopathy is the predominant renal lesion in type 1 diabetes, although also tubular, interstitial and arteriolar lesions are present in the advanced stages of renal d...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：10.1007/s40620-020-00797-y
更新日期：2020-10-01 00:00:00
abstract：:IgM secreting myelomas or lymphomas, including Waldenström macroglobulinemia, are associated with a varied spectrum of renal pathology, including intracapillary hyaline deposits, cryoglobulin, membranoproliferative glomerulonephritis, amyloid, monoclonal immunoglobulin deposition disease, cast nephropathy, and lymphom...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-018-0544-9
更新日期：2018-12-01 00:00:00
abstract：:Aortic intimal sarcoma is a rare tumor with poor prognosis. The most common manifestations are thromboembolic phenomena and vascular obstruction. We present a case of aortic intimal sarcoma causing bilateral renal artery stenosis which manifested as resistant hypertension and acute kidney inury. Multiple attempts to s...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.5301/jn.5000283
更新日期：2013-09-01 00:00:00
abstract：:Lupus is now regarded as a syndrome which results from several related auto-immune processes, although the exact mechanisms of how the disease arises in susceptible individuals remain obscure. When the San Carlo meetings began in 1968, much less was known about the pathogenesis of autoimmunity, but the presence of aut...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：1999-07-01 00:00:00
abstract：BACKGROUND:Cardiac surgery-associated acute kidney injury (CSA-AKI) is associated with high risk for complications and mortality. Whether renin-angiotensin system (RAS) inhibitor should be continued or withdrawn in patients with long-term use before cardiac surgery has been lack of consensus. METHODS:We performed this...
journal_title：Journal of nephrology
pub_type： 杂志文章,多中心研究
doi：10.1007/s40620-019-00657-4
更新日期：2019-12-01 00:00:00
abstract：BACKGROUND:Previous studies have shown that bleeding times have positive predictive values of only 5% for perioperative bleeding in unselected populations. Nevertheless, performing bleeding times prior to all renal biopsies is common in nephrology practice. METHODS:We report complications of 112 renal biopsies done at...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2001-07-01 00:00:00
abstract：BACKGROUND:This study is part of a 3-country study testing whether normal levels of hemoglobin (Hgb) delay the progression of left ventricular (LV) growth in chronic kidney disease (CKD) patients not on dialysis. METHODS:This was an open-label, randomized, multicenter, controlled trial conducted in 27 tertiary-care ho...
journal_title：Journal of nephrology
pub_type： 杂志文章,多中心研究,随机对照试验
doi：
更新日期：2008-11-01 00:00:00
abstract：BACKGROUND:Dialysate calcium (Ca) concentration should be viewed as part of the integrated therapeutic regimen to control renal osteodystrophy and maintain normal mineral metabolism. Thus, a correct ionized calcium mass balance (Ca++MB) during hemodialysis (HD) is crucial in the treatment of renal osteodystrophy. The G...
journal_title：Journal of nephrology
pub_type： 杂志文章,随机对照试验
doi：10.5301/JN.2011.6385
更新日期：2011-11-01 00:00:00
abstract：BACKGROUND:In biopsy-proven idiopathic nephrotic syndrome (INS), immunoglobulin M (IgM) and C1q are occasionally deposited in the mesangium. In pediatric nephrology, the significance of mesangial IgM or C1q deposits is controversial, based on previous reports. The aim of this study was to explore the clinical significa...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.5301/jn.5000133
更新日期：2013-03-01 00:00:00
abstract：:Autosomal dominant polycystic kidney disease (ADPKD) may present with a variety of extrarenal manifestations. Only 1 case of spontaneous coronary artery dissection has been reported so far in ADPKD. Here we report a case of coronary artery dissection occurring in a 38-year-old woman with ADPKD and an unremarkable medi...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2009-05-01 00:00:00
abstract：:Given the alarming predictions of the potentially devastating future epidemic of end-stage renal disease (ESRD), further knowledge is still urgently needed on this topic. The real dimensions of the problem are still unclear. It is difficult to compare Europe and the USA because different criteria are used in data coll...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2005-03-01 00:00:00
abstract：:Calciphylaxis, or calcific uremic arteriolopathy, is a vascular ossification-calcification disease involving cutaneous or visceral arterioles, with ischemic damage of the surrounding tissues, usually in the setting of chronic kidney disease. Pathogenesis is still unclear and probably comprises the participation of vas...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：10.1007/s40620-015-0192-2
更新日期：2015-10-01 00:00:00
abstract：:Gonadal dysfunction is a frequent finding in men with chronic kidney disease and with end-stage renal disease. Testosterone deficiency, usually accompanied by elevation of serum gonadotropin concentrations, is present in 26-66% of men with different degrees of renal failure. Uremia-associated hypogonadism is multifact...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：10.5301/JN.2011.8481
更新日期：2012-01-01 00:00:00
abstract：:Primary renal non-Hodgkin's lymphoma is very rare in childhood. A six-year-old boy presented with bilateral non-obstructive multinodular nephromegaly and renal failure. Percutaneous needle biopsy showed large-cell lymphoma. The patient was started on chemotherapy. A right nephrectomy was done when systemic hypertensio...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：2002-09-01 00:00:00
abstract：:Hepatitis C virus (HCV) infection is now considered a systemic disease due to the occurrence of extra-hepatic manifestations. Among these, the renal involvement is frequent. HCV infection, in fact, is strongly associated with proteinuria and chronic kidney disease (CKD) and negatively affects the prognosis of renal pa...
journal_title：Journal of nephrology
pub_type： 杂志文章,实务指引
doi：10.1007/s40620-018-0523-1
更新日期：2018-10-01 00:00:00
abstract：BACKGROUND:In the general population and in hemodialysis patients epicardial adipose tissue (EAT) has been associated with increased mortality and cardiovascular events. Weight loss and lipid lowering therapies reduced EAT in the general population. It is unknown whether sevelamer, a phosphate (Pi) binder that lowers c...
journal_title：Journal of nephrology
pub_type： 杂志文章,随机对照试验
doi：10.1007/s40620-016-0310-9
更新日期：2016-10-01 00:00:00
abstract：BACKGROUND:Proteinuria results from increased glomerular permeability and is associated with retraction and effacement of the highly specialized interdigitating podocyte foot processes. In podocytes (glomerular epithelial cells [GECs]), p130Cas localizes diffusely to the cytoplasm and connects focal adhesion proteins a...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.5301/jn.5000261
更新日期：2013-09-01 00:00:00
abstract：:Epicardial adipose tissue (EAT) is localized between the myocardial surface and visceral layer of the pericardium. It is a metabolically active organ that secretes several cytokines which modulate cardiovascular morphology and function. EAT may interact locally with coronary arteries through paracrine secretion mechan...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：10.1007/s40620-018-0491-5
更新日期：2018-12-01 00:00:00
abstract：:The expansion of genomic medicine is furthering our understanding of many human diseases. This is well illustrated in the field of nephrology, through the characterization, discovery, and growing insight into various renal diseases through use of Next Generation Sequencing (NGS) technologies. This review will provide ...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：10.1007/s40620-017-0448-0
更新日期：2018-02-01 00:00:00
abstract：INTRODUCTION:Lack of adherence may influence the efficiency of phosphate binders. Our clinical hypothesis was that if lanthanum carbonate (LC) might offer some advantage over sevelamer (S), that that would be in those patients in whom serum phosphate (P) levels were not controlled by S or in those requiring high doses ...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2010-11-01 00:00:00
abstract：OBJECTIVES:HERMEX is a population-based study which tries to evaluate the relative weight of cardiovascular risk factors in inhabitants of Extremadura, Spain. This report presents the data about chronic kidney disease (CKD) in a Spanish population sample. METHODS:For an observational cross-sectional population-based s...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.5301/jn.5000221
更新日期：2013-07-01 00:00:00
abstract：:Structural and functional alterations affecting the aging kidney predispose to an increased risk of acute renal failure (ARF) in the elderly. This is a common problem becoming more relevant because of an increase in life expectancy. The epidemiology of ARF in the elderly is far from being well assessed, because of the...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：10.5301/jn.5000141
更新日期：2012-01-01 00:00:00
abstract：BACKGROUND:Non-heart-beating donors suffer a period of warm ischaemia (WI) injury, resulting in higher rates of delayed graft function compared with heart-beating donors. Endothelin is believed to play a pathophysiological role in the intense vasospasm associated with WI. This study aimed to investigate renal function ...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2009-01-01 00:00:00
abstract：:Minoxidil is frequently used in patients with advanced renal disease who have been unresponsive to other antihypertensive agents. We describe a case of a 50-year-old man with chronic renal failure who was hospitalized complaining of a 2-week history of sore throat that had progressed to severe oral lesions and multipl...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2007-01-01 00:00:00
abstract：:Cutaneous periarteritis nodosa (PAN) is a clinical feature characterized by chronic, benign course; its pathogenesis is unknown. In patients submitted to renal transplantation cutaneous PAN is a rare complication. We report a case of cutaneous PAN associated with the reappearance of hepatitis B antigen 16 years after ...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2001-09-01 00:00:00
abstract：BACKGROUND:This study aimed to evaluate the health-related quality of life (HRQOL) and burden on family caregivers of chronic dialysis patients and to analyze which factors were associated with it. METHODS:A cross-sectional multicentric study was carried out with 221 patient/caregiver pairs. General population Short F...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2004-11-01 00:00:00
abstract：:Gitelman syndrome (GS), an inherited disorder due to loss of function in mutations of the gene encoding the distal convoluted tubule Na-Cl cotransporter (NCCT), is characterized by hypokalemia metabolic alkalosis, hypomagnesemia and hypocalciuria. A 18-year-old girl was admitted to our hospital with a history of muscl...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2010-09-01 00:00:00
