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 24,015 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Preeclampsia-eclampsia is still one of the leading causes of maternal and fetal morbidity and mortality. Despite active research for many years, the etiology of this disorder exclusive to human pregnancy is an enigma. Recent evidence suggests there may be several underlying causes or predispositions leading to the signs of hypertension, proteinuria, and edema, findings that allow us to make the diagnosis of the "syndrome" of preeclampsia. Despite improved prenatal care, severe preeclampsia and eclampsia still occur. Although understanding of the pathophysiology of these disorders has improved, treatment has not changed significantly in over 50 years. Although postponement of delivery in selected women with severe preeclampsia improves fetal outcome to a degree, this is not done without risk to the mother. In the United States, magnesium sulfate and hydralazine are the most commonly used medications for seizure prophylaxis and hypertension in the intrapartum period. The search for the underlying cause of this disorder and for a clinical marker to predict those women who will develop preeclampsia-eclampsia is ongoing, with its prevention the ultimate goal. This review begins with the clinical and pathophysiologic aspects of preeclampsia-eclampsia (Part 1). In Part 2, the experimental observations, the search for predictive factors, and the genetics of this disorder will be reviewed.
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PMID:Preeclampsia. Part 1: clinical and pathophysiologic considerations. 1221 68

Preeclampsia-eclampsia is still one of the leading causes of maternal and fetal morbidity and mortality. Despite active research for many years, the etiology of this disorder exclusive to human pregnancy is an enigma. Recent evidence suggests there may be several underlying causes or predispositions leading to the signs of hypertension, proteinuria, and edema, findings that allow us to make the diagnosis of the "syndrome" of preeclampsia. Despite improved prenatal care, severe preeclampsia and eclampsia still occur. Although understanding of the pathophysiology of these disorders has improved, treatment has not changed significantly in over 50 years. Although postponement of delivery in selected women with severe preeclampsia improves fetal outcome to a degree, this is not done without risk to the mother. In the United States, magnesium sulfate and hydralazine are the most commonly used medications for seizure prophylaxis and hypertension in the intrapartum period. The search for the underlying cause of this disorder and for a clinical marker to predict those women who will develop preeclampsia-eclampsia is ongoing, with its prevention the ultimate goal. This review began with the clinical and pathophysiologic aspects of preeclampsia-eclampsia (Part 1). Now, in Part 2, the experimental observations, the search for predictive factors, and the genetics of this disorder are reviewed.
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PMID:Preeclampsia. Part 2: experimental and genetic considerations. 1221 69

Pre-eclampsia is still one of the leading causes of maternal and fetal morbidity and mortality. Despite active research for many decades, the etiology of this disorder exclusive to human pregnancy is an enigma. Recent evidence suggests there may be several underlying causes or predispositions leading to endothelial dysfunction and causing the signs of hypertension, proteinuria, and edema--findings that allow us to make the diagnosis of the "syndrome" of pre-eclampsia. It is obvious that a single mechanism responsible for the syndrome pre-eclampsia does not exist. Instead, several mechanisms can act together and even multiply each other. The search for the underlying cause of this disorder and for a clinical marker to predict which women will develop pre-eclampsia is ongoing, with its prevention being the ultimate goal.
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PMID:Pre-eclampsia--still a disease of theories. 1506 37

While increased risk of cardiovascular disease (CVD) in patients with hyperlipidemia, chronic kidney disease (CKD), or end-stage renal disease (ESRD) is well documented, transient hyperlipidemia or intermittent renal disease as a consequence of relapsing nephrotic syndrome (NS) has not been studied. To investigate this enigma, 62 patients, between 25 and 53 years of age, who had steroid-responsive/dependent NS during childhood, were identified from the records of the Division of Pediatric Nephrology at Yale School of Medicine. Forty patients were located and contacted to ascertain symptoms or occurrences of CVD via a telephone interview. At the time of follow-up, 23-46 years after cessation of NS, none of these patients had ESRD or CKD. Three patients had experienced a myocardial infarction (MI): a 32-year-old male with a family history of CVD; a 41-year-old male with a history of heavy smoking, hypertension, diabetes mellitus, and elevated cholesterol; a 31-year-old male after a cocaine overdose. The occurrence of events (8%) and mortality from CVD (none) in this cohort of patients is comparable to patients of a similar age in the general population and is lower than that of patients of the same age who are on dialysis. The data suggest that relapsing NS during childhood does not place patients at increased risk for CVD mortality or morbidity compared with the general population. Consequently, it would appear that factors related to persistent proteinuria or renal insufficiency, rather than transient proteinuria and renal disease, contribute to the CVD documented in patients with CKD or ESRD.
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PMID:The risk of cardiovascular disease in adults who have had childhood nephrotic syndrome. 1508 19

Maternal hypertension and proteinuria after 20 weeks gestation defines preeclampsia. Severe preeclampsia is defined by severe hypertension or massive proteinuria, with or without symptoms or altered laboratory tests. With an incidence of 4-7%, preeclampsia remains a major cause of maternal and neonatal morbidity and mortality. Admission into a hospital is crucial to monitor both mother and fetus. The only treatment is delivery. Management of blood pressure and prevention of eclampsia with magnesium sulfate is indicated in severe preeclampsia. Despite numerous studies attempting to elucidate the exact etiopathogenesis of this complex multifactorial disease, prediction or prevention of preeclampsia is not available. Preeclampsia has been named the "disease of theories" and remains to date a challenging enigma for the scientific community.
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PMID:[Recent data on the physiopathology of preeclampsia and recommendations for treatment]. 1577 58

Posttransplant recurrence of inherited focal segmental glomerulosclerosis (FSGS) is still an enigma owing to the evident paradox of the molecular origin of proteinuria. A young girl with FSGS for WT1 mutation (IVS9+4C>T) and Frasier syndrome received a renal transplant at the age of 11 years. After an initial good outcome with recovery of renal function, proteinuria re-appeared after 7 days and steadily increased up to a nephrotic range. Determination of plasma permeability activity showed concomitant high Palb (0.7). At this point, plasmapheresis was started and after nine cycles with 1500 mL exchange and albumin re-infusion, proteinuria decreased to normal range and is still normal after 3 years. This is the first description of posttransplant recurrence of proteinuria in Frasier syndrome that should be included in potential outcome of renal transplant in this category of patients. This observation confirms the concept that recurrence of proteinuria may occur in inherited forms of FSGS so far reported only for patients carrying NPHS2 mutations and reinforces the idea on multifactorial origin of the disease.
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PMID:Posttransplant recurrence of proteinuria in a case of focal segmental glomerulosclerosis associated with WT1 mutation. 1678 May 44

Pre-eclampsia, a syndrome of pregnant women, is one of the leading causes of maternal and fetal morbidity and mortality. Despite active research, the etiology of this disorder remains an enigma. Recent work has, however, provided promising explanations for the causation of the disorder and some of its phenotypes. Evidence indicates that the symptoms of hypertension and proteinuria, upon which the diagnosis of pre-eclampsia is based, have several underlying causes. Nevertheless, the treatment of pre-eclampsia has not changed significantly in over 50 years. This review describes the most recent insights into the pathophysiology of pre-eclampsia from both basic and clinical research, and attempts to provide a unifying hypothesis to reconcile the abnormalities at the feto-placental level and the clinical features of the maternal syndrome. The novel findings outlined in this review provide a rationale for potential future prophylactic and therapeutic interventions for pre-eclampsia.
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PMID:Mechanisms of disease: Pre-eclampsia. 1693 75

Chyluria is the passage of chyle into urine, and develops as a result of communication between the lymphatic system and the urinary system. It is an unusual manifestation of lymphatic filariasis reported mainly from South Asian countries. We report the case of a 38-year-old man from an endemic area who presented with passage of milky urine. Physical examination did not reveal any lymphadenopathy or lymph oedema. Urine tests revealed nephrotic range proteinuria. A 99m technetium sulphur colloid lymphoscintigraphy confirmed connection between lymphatic vessels and the urinary tract. Predominant chyluria with no overt lymphatic filariasis remains an enigma.
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PMID:An occult filarial infection presenting as chyluria with proteinuria: a case report and review of literature. 2266 20

An epidemic of chronic kidney disease of unknown origin has emerged in the last decade in Central America and has been named Mesoamerican nephropathy. This form of chronic kidney disease is present primarily in young male agricultural workers from communities along the Pacific coast, especially workers in the sugarcane fields. In general, these men have a history of manual labor under very hot conditions in agricultural fields. Clinically, they usually present with normal or mildly elevated systemic blood pressure, asymptomatic yet progressive reduction in estimated glomerular filtration rate, low-grade non-nephrotic proteinuria, and often hyperuricemia and or hypokalemia. Diabetes is absent in this population. Kidney biopsies that have been performed show a chronic tubulointerstitial disease with associated secondary glomerulosclerosis and some signs of glomerular ischemia. The cause of the disease is unknown; this article discusses and analyzes some of the etiologic possibilities currently under consideration. It is relevant to highlight that recurrent dehydration is suggested in multiple studies, a condition that possibly could be exacerbated in some cases by other conditions, including the use of nonsteroidal anti-inflammatory agents. At present, Mesoamerican nephropathy is a medical enigma yet to be solved.
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PMID:CKD of unknown origin in Central America: the case for a Mesoamerican nephropathy. 2515 Aug 55

Nephrotic syndrome is defined as the association of massive proteinuria, hypoalbuminaemia, edema, and hyperlipidemia. It is separated to steroid-sensitive or steroid-resistant (SRNS) forms in respect to the response to intensive steroid therapy. SRNS usually progresses to end-stage renal failure. According to the North American Pediatric Renal Trials and Collaborative Studies SRNS constitutes the second most frequent cause of ESRD in the first two decades of life. Unfortunately, there is no curative treatment for majority of patients. Majority of the SRNS patients have the histologic picture of focal segmental glomerulosclerosis. Interestingly, the risk of recurrence in the kidney graft in patients with hereditary SRNS is lower than in those who do not have genetic background. The etiology and pathogenesis of SRSN has remained enigma for decades. The discovery of 39 dominant or recessive SRNS genes enabled better understanding of the function of the glomerular podocytes and slit membrane. Hildebrandt's group has shown that 85% of the SRNS cases with onset by 3 months of age and 66% with onset by 1 year of age can be explained by recessive mutations in one of four genes only (NPHS1, NPHS2, LAMB2, or WT1). The same group used modern diagnostic techniques such as the next generation sequencing and tested a large international cohort of SRNS patients (n = 1783 families). The diagnostic panel included 21 genes with a recessive mode of inheritance and 6 genes with a dominant mode of inheritance. Single-gene cause was detected in 29.5% (526 of 1783) of the families with SRNS that manifested before 25 years of age. The identification of causative single-gene mutations may have important therapeutic consequences in some cases. This is very important for patients who carry mutations in a gene of coenzyme Q10 biosynthesis (COQ2, COQ6, ADCK4, or PDSS2). In these patients the treatment with coenzyme Q10 may be indicated. Also, patients with recessive mutations in PLCE1 may respond fully to the treatment with steroids or cyclosporine A. The patients with CUBN may benefit the treatment with vitamin B12. The detection of causative mutations may also be very important for familial genetic counseling and for prenatal diagnosis.
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PMID:Steroid Resistant Nephrotic Syndrome-Genetic Consideration. 2744 91


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