Presentation
He also presented an abnormal ultrasonography, which revealed asymmetric kidneys. Through renal biopsy, the diagnosis of oligomeganephronia (OMN) was confirmed. OMN is a very rare form of renal hypoplasia, and late-onset in adulthood is even rarer. [ncbi.nlm.nih.gov]
On Admission POA Help "Present On Admission" is defined as present at the time the order for inpatient admission occurs — conditions that develop during an outpatient encounter, including emergency department, observation, or outpatient surgery, are [icd10data.com]
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]
Entire Body System
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Enuresis
One patient presented in infancy (10 days of age), the others between 12.8 and 14.5 years (mean 13.7 years), with long-standing polydipsia and polyuria, enuresis, and growth retardation. [read.qxmd.com]
Respiratoric
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Tachypnea
He was admitted immediately after birth because of tachypnea and multiple anomalies, i. e., cleft lip, cleft palate, lowset and malformed ear, and retentio testis. [ci.nii.ac.jp]
Eyes
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Antimongoloid Slant
In addition, the two patients had similar multiple anomalies; microcephaly, prominent glabella, hypertelorism, antimongoloid slant, epicanthal folds, broad nose, cleft lip and palate, down-turned mouth, short philtrum, micrognathia, low set ears, hypospadias [ncbi.nlm.nih.gov]
In addition, the two patients had similar multiple anomalies; microcephaly, prominent glabella, hypertelorism, antimongoloid slant, epicanthal folds, broad nose, cleft lip and palate, downturned mouth, short philtrum, micrognathia, low set ears, hypospadias [onlinelibrary.wiley.com]
Workup
X-Ray
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Multiple Renal Cysts
renal cysts Q61.1 Polycystic kidney, infantile type Q61.11 Cystic dilatation of collecting ducts Q61.19 Other polycystic kidney, infantile type Q61.2 Polycystic kidney, adult type Reimbursement claims with a date of service on or after October 1, 2015 [icd10data.com]
Serum
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Creatinine Increased
Regarding our patient, proteinuria was the first clinical manifestation, and two years later serum creatinine increased. [zdoc.site]
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]
The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment. [orpha.net]
An ideal learning tool for beginners and a valuable reference for experienced healthcare professionals, its interactive, case-based approach allows you see to diagnosis and treatment in a clinical context and test your knowledge with review questions [books.google.com]
Prognosis
(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]
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]
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). [orpha.net]
Glomerular density in renal biopsy specimens predicts the long-term prognosis of IgA nephropathy. Clin J Am Soc Nephrol. 2010;5(1):39–44. PubMed CrossRef Google Scholar 6. [link.springer.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]
Etiology
Oligomeganephronia, characterized clinically be early renal failure and histologically by large, sparse glomeruli, is of undetermined etiology. [ncbi.nlm.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]
Associations distal limb deficiencies with micrognathia (MIM.246560) Etiology PAX2 germline mutations ( 11168927 ) See also paired box transcription factors (PAXs) PAX2 renal development renal hypoplasia retinal defects renal-coloboma syndrome References [humpath.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]
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]
[…] disease. 3 This is the so-called hyperfiltration theory of renal damage, which is based on the notion that the few remaining hard-working nephrons will eventually fail, resulting in progressive glomerulosclerosis. 1 In order to understand better the pathophysiology [scielo.br]
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]
Prevention
Prevention - Oligomeganephrony Not supplied. [checkorphan.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]
Currently, there is no effective treatment and preventive measures for OMN because of unclear mechanism. Regular follow-up is needed. Patients with OMN are often born with a lower birth weight than a normal newborn. [zdoc.site]
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]