+ Resolve Article
+ Follow Us
Follow on FacebookFollow on Facebook
Follow on TwitterFollow on Twitter

+ Translate
+ Subscribe to Site Feed
GeoScience Most Shared ContentMost Shared Content

Nephrotic syndrome in the 1st year of life

, : Nephrotic syndrome in the 1st year of life. Australian & New Zealand Journal of Medicine 6(1): 84

Among the various primary conditions which may be associated with a nephrotic syndrome at birth or within the 1st year of life, the best known is the congenital nephrotic syndrome of finnish type (CNF) characterized by irregular pseudocystic dilatation of proximal tubules. This disease, very frequent in Finaland, is often familial with an autosomal recessive mode of inheritance. Patients are steroid resistant, but the cause of death is usually not uraemia but infection or severe diarrhoea with electrolyte imbalance. The second condition is idiopathic nephrosis including minimal change disease, diffuse mesangial proliferation and focal segmental glomerular sclerosis. As opposed to CNF, infants with early onset nephrosis may respond to steroid therapy as older children do and may even recover. However, there are no histopathological criteria which allow the certain differentiation of idiopathic nephrosis from CNF. The third condition is diffuse mesangial sclerosis (DMS), a clinicopathological entity which can occur as an isolated finding or be associated with male pseudohermaphroditism and/or Wilms' tumour (Drash syndrome). From a morphological point of view, DMS is easy to differentiate from CNF because of the characteristic pattern of involvement of the glomeruli. From a clinical point of view, the nephropathy, almost always characterized by a nephrotic syndrome, has two distinct features: it is most often diagnosed in the first 2 years of life and it progresses rapidly to end-stage renal failure, which usually occurs before the age of 3 years. The clinical findings in 36 patients with DMS are presented. The nephropathy was isolated in 22 infants and associated with male pseudohermaphroditism and/or Wilms' tumour in 14. The early onset of the nephropathy, its familial incidence and its association with Drash syndrome suggests an antenatal dysgenetic process. Recent studies indicate a direct role of WT1 (a Wilms' tumour gene) in the aetiology of Drash syndrome, and mutations of this gene have been found in 3 patients herein reported. It remains to be demonstrated whether or not the WT1 gene is also altered in patients with isolated DMS.


DOI: 10.1007/bf00857534

Other references

Simons, F.Estelle.R., 2004: Epinephrine (adrenaline) in the first-aid, out-of-hospital treatment of anaphylaxis. Epinephrine (adrenaline), the initial treatment of choice for systemic anaphylaxis, is an alpha- and beta-adrenergic agonist with bidirectional, cyclic adenosine monophosphate-mediated pharmacological effects on target organs, and a narrow therape...

Dantzer R., 1973: Methodology of comparative trials in veterinary therapeutics part 1 general principles. Annales de Recherches Veterinaires 4(2): 305-313

Gryzhankova L.N.; Saenko G.N.; Karyakin A.V.; Laktionova N.V., 1973: Concentration of some metals in algae from the sea of japan. Okeanologiya 13(2): 259-263

Jackson, B.T.; Kinmonth, J.B., 1974: The normal lymphographic appearances of the lumbar lymphatics. Clinical Radiology 25(2): 175-186

Blunt M.H., 1972: Hemo globin function in the developing goat and sheep. Animal Blood Groups & Biochemical Geneticsp 1: 53-54

Meixner, J., 1943: Uber die Umbildung einer Turbellarienart nach Einwanderung aus dem Meere ins Susswasser. Internationale Revue der Hydrobiologie Leipzig, 434-6: 458-468

Carballas, M.C.rballas, T.C.baneiro, A.V.llar, M.; Leiros, M.; Guitian-Ojea, F., 1984: AC soils on granite, in Galicia (northwestern Spain) with special reference to Atlantic rankers. III. Organic fraction. Anales de edafologia y agrobiologia (pub 1984) 42(11-12): 1781-1814

Leca, A.P., 1969: Osteo-articular manifestations of congenital hemolytic anemia. Les Cahiers du College de Medecine des Hopitaux de Paris 10(16): 1275-1287

Aviv, G.; Elpers, L.; Mikhlin, S.; Cohen, H.; Vitman Zilber, S.; Grassl, G.A.; Rahav, G.; Hensel, M.; Gal-Mor, O., 2017: The plasmid-encoded Ipf and Klf fimbriae display different expression and varying roles in the virulence of Salmonella enterica serovar Infantis in mouse vs. avian hosts. Salmonella enterica serovar Infantis is one of the prevalent Salmonella serovars worldwide. Different emergent clones of S. Infantis were shown to acquire the pESI virulence-resistance megaplasmid affecting its ecology and pathogenicity. Here, we...

Van Bemmel, A.C.V.; Zwart, P.; Peters, J.C., 1962: Report on births and deaths occurring in the Gardens of the Royal Rotterdam Zoo during the years 1959 and 1960. Causes of death in 163 mammals and 677 birds are summarized in two tables. Pasteurella pseudotuberculosis was isolated from four monkeys, a Gazella arabica, a Damaliscus philippse and a Cygnus melancoryphus.