Presentation
We describe a 14-year-old boy who presents with a history of dysuria and some blood in his pants. Urinalysis showed no hematuria but persistent heavy proteinuria. [sjkdt.org]
Written by leading pathologists, this second edition presents an up-to-date, concise presentation of major pathological, clinical, pathophysiological, and genetic information for over 240 diagnoses. [books.google.com]
Entire Body System
- Asymptomatic
[…] diarrhoea – Hypertension +/- – Gross hematuria +/- 14 CLINICAL FEATURESMinimal Change Nephrotic Syndrome Focal Segmental Glomerulosclerosis Membranous Nephropathy Age ( yr ) 2 - 62 - 1040 - 50 Sex ( M : F ) 2 : 11.3 : 12 : 1 Nephrotic Syndrome100 %90 %80 % Asymptomatic [slideplayer.com]
In some cases, asymptomatic proteinuria demands closer examination, including kidney biopsy, leading to the diagnosis of oligomeganephronia by school age (late-onset type). 2 This disorder is very rare in the pediatric population and even rarer in adult [scielo.br]
In some cases, asymptomatic proteinuria prompts closer examination, including kidney biopsy, and leads to the diagnosis of oligomeganephronia by school age (late-onset type). [docksci.com]
Usually the organism is destroyed more or less time, and infection is resolved or asymptomatic, but in cases of deficient cellular immunity, are released from the phagolysosome passing the cytoplasm, where they multiply freely, and the infection will [colombiamedica.univalle.edu.co]
- Anemia
Patients may present at birth with polyuria/polydipsia or signs of renal dysfunction such as anemia During the first year of life, the usual symptom is persistent anorexia with vomiting, fever, and height-weight growth delay. [dovemed.com]
Patients may present at birth with polyuria/polydipsia or signs of renal dysfunction such as anemia. During the first year of life, the usual symptom is persistent anorexia with vomiting, fever, and height-weight growth delay. [rarediseases.info.nih.gov]
The patient not only had symptoms resembling familial juvenile nephronophthisis such as polyuria and polydipsia since early childhood, growth retardation, choreoretinal degeneration, anemia and azotemia with few abnormalities of urinalysis, but also showed [jstage.jst.go.jp]
As with all such disorders, complications such as growth failure, metabolic bone disease, anemia, metabolic acidosis, and other disorders of fluid and electrolyte metabolism may occur. (Lane PH. Oligomeganephronia. [kidneypathology.com]
- Inflammation
Comprehensive in scope, this title begins with a look at normal anatomy and histology for each organ system...followed by discussions of the pathology of congenital anomalies, inflammations, non-neoplastic diseases and neoplasia. [books.google.com]
Later in immunocompetent individuals, occurs an inflammation at the site of infection, resulting in formation of a or not caseous. [colombiamedica.univalle.edu.co]
Moreover, angiotensin antagonism suppresses local growth factor, cytokine and chemokine release, with subsequent reduction of glomerular hypertrophy and sclerosis as well as tubulointerstitial inflammation and fibrosis(5). [indianpediatrics.net]
- Weight Loss
The medical condition of laryngeal histoplasmosis may present with dysphonia odynophagia, dysphagia, dyspnea, fatigue, weight loss and generalized unrest. [colombiamedica.univalle.edu.co]
Cardiovascular
- Hypertension
However, Case 1 was unusual because hypertension led to further scrutiny. [academic.oup.com]
Hypertension occurs in almost 80 - 90% of children, 30% of whom will present with complications of hypertension, including seizures or visual disturbances. Hypertension is caused by salt and water retention. [cmej.org.za]
Eyes
- Prolapse
It may account to intracranial hemorrhages. 7. mitral valve prolapse. PowerPoint Presentation: GROSSLY: Bilaterally enlarged, weighing up to 4 kgs. External surface consists solely of mass of cysts, with no intervening parenchyma. [authorstream.com]
Urogenital
- Cryptorchidism
Hypospadias, and cryptorchidism can be observed in 50% of males.We describe a case of a child affected by WHS with oligonephroma and a review of the literature of oligonephroma and olimeganephronia (OMN) with associated anomalies.CLINICAL REPORTA 9‐year‐old [deepdyve.com]
- Uremia
[…] incontinence腹圧性尿失禁 syphilis梅毒 t testicular cancer精巣癌 testicular torsion精巣(索)捻転 trabeculation肉柱形成、梁状突起、小柱形成 transitional cell carcinoma移行上皮癌 transurethral resection of the prostate (TUR-P)経尿道的前立腺切除術 tubular尿細管の tubulointerstitial尿細管間質の u urea nitrogen尿素窒素 uremia [tokyo-med.ac.jp]
Workup
X-Ray
- Small Kidney
[…] oligomeganephronia Aka: oligonephronic hypoplasia, form of renal hypoplasia, very small kidneys, reduced number of renal segments (less than7), most severe form unipapillary kidney, hypertrophied nephrons, male:female 3:1 (Pollack), progressive renal [encyclo.co.uk]
CHORUS Collaborative Hypertext of Radiology Kidney About CHORUS Disclaimer Copyright aka: oligonephronic hypoplasia form of renal hypoplasia very small kidneys reduced number of renal segments ( most severe form unipapillary kidney hypertrophied nephrons [gamuts.net]
We believe that multiple anomalies might be suggestive findings of OMN in patients, such as renal insufficiency, persistent proteinuria, and bilateral small kidneys. [link.springer.com]
12:1769 ) Oligomeganephronia: type of hypoplasia with small kidney but hypertrophied nephrons due to compensatory hypertrophy caused by reduced number of nephrons Diagrams / tables Images hosted on other servers: Renal hypoplasia Microscopic (histologic [pathologyoutlines.com]
Treatment
The goal of treatment for Oligomeganephronic Renal Hypoplasia is to slow the progression of disease. [dovemed.com]
Signs and Symptoms of Oligomeganephronia Low birth weight Hypoosmolar urine, maximum gravity of urine is 1.007–1.012 Thirst, vomiting and dehydration Proteinuria End-stage renal disease around the age of 10 years Treatment of Oligomeganephronia Treatment [urology-textbook.com]
[…] indication for second-line immunosuppressive treatment. [cmej.org.za]
Prognosis
Prognosis With the increasing metabolic demands on the kidney during growth, a decline in renal function is seen resulting in chronic renal failure at a mean age of 10 years (range 6 months to 20 years). Last updated: 10/9/2014 [rarediseases.info.nih.gov]
(Outcomes/Resolutions) The prognosis of Oligomeganephronic Renal Hypoplasia is dependent upon the severity of the signs and symptoms and associated complications, if any The condition is progressive. [dovemed.com]
Steroid Responsive NS : Good prognosis ( MCNS ) Steroid Resistant NS : Poor prognosis ( FSGS ) 30 Congenital Nephrotic Syndrome Presents in first 3 months of life Anasarca, hypoalbuminaemia, oliguria { Finnish’ Type } Antenatally detectable : – Raised [slideplayer.com]
Prognosis With the increasing metabolic demands on the kidney during growth, a decline in kidney function is seen resulting in chronic kidney disease at a mean age of 10 years (range 6 months to 20 years). * European Reference Network The documents contained [orpha.net]
Cystitis Tests o Home Kidney Tests o Home Urine Protein Tests (Kidney Function) o Home Prostate Cancer Tests * Kidney Health: Home Testing: o Home Microalbumin Tests (Kidney) o Home Urine Protein Tests (Kidney) o Home Urinary Tract Infection (UTI) Tests Prognosis [checkorphan.org]
Etiology
Oligomeganephronia, characterized clinically be early renal failure and histologically by large, sparse glomeruli, is of undetermined etiology. [ncbi.nlm.nih.gov]
Etiology The etiology of oligomeganephronia is not fully understood, but is hypothesized to be caused by premature termination of nephrogenesis. [rarediseases.info.nih.gov]
TERMINOLOGY Abbreviations Oligomeganephronia (OMN) Synonyms Oligomeganephronic hypoplasia Definitions Renal hypoplasia with marked compensatory nephron hypertrophy occurring both sporadically and due to specific genetic disorders ETIOLOGY/PATHOGENESIS [basicmedicalkey.com]
This is the first documented report of an intrauterine fetal demise of one twin in association with oligomeganephronia in the other, and suggests that these two conditions may be related etiologically. [karger.com]
Epidemiology
Epidemiology Prevalence is unknown. The male to female ratio is of 3:1. Clinical description Oligomeganephronia is usually detected on routine ultrasound screening with small but normal shaped kidneys. Birth weight is often below the average value. [rarediseases.info.nih.gov]
Summary Epidemiology Prevalence is unknown. The male to female ratio is of 3:1. Clinical description Oligomeganephronia is usually detected on routine ultrasound screening with small but normal shaped kidneys. [orpha.net]
Epidemiology Frequency United States Exact frequency of oligomeganephronia is difficult to determine. [emedicine.medscape.com]
Pathophysiology
Written by leading pathologists, this second edition presents an up-to-date, concise presentation of major pathological, clinical, pathophysiological, and genetic information for over 240 diagnoses. [books.google.com]
Learning objectives• Filtration barrier• Pathophysiology of FSGS• Genetics of FSGS• Management of FSGS• Recurrence of FSGS in Graft• Management of Recurrence 3. Glomerular Filtration Barrier 4. [slideshare.net]
Pathophysiology Oligomeganephronia results from arrested development of the metanephric blastema at 14-20 weeks' gestation, with subsequent hypertrophy of glomeruli and tubules in the kidney. [emedicine.medscape.com]
( Drug induced ) – Penicillamine, Gold, NSAIDS, Pamidronate, Mercury, Lithium 9 Idiopathic Minimal Change disease ( >80 % ) Mesangial proliferation Focal segmental Glomerulosclerosis Membranous Nephropathy Membranoproliferative glomerulonephritis 10 Pathophysiology [slideplayer.com]
Prevention
Prevention - Oligomeganephrony Not supplied. [checkorphan.org]
Prenatal testing is another possibility for prevention or awareness, and this can be done through molecular genetic testing or ultrasounds at later stages of pregnancy. [en.wikipedia.org]
• Galactose: Block the binding site or change the configuration of the free soluble factor, preventing it from binding to the podocytes• Podocyte stem cells therapy [slideshare.net]
Normal assent of these fused kidneys is prevented because they are caught by the root of the inferior mesenteric artery. Usually asymptomatic because its collecting system develops normally and the ureters enter the bladder. [authorstream.com]