Renal dysplasia

Rieger syndrome Eye defecfs Abnormal skelefal paffeming and renal dysplasia... 

Serum potassium concentrations were determined at the end of a 2-hour infusion of amphotericin deoxycholate (1 mg/kg/day) in a 2-year-old girl with systemic candidiasis receiving long-term hemodialysis for renal dysplasia (85). The potassium concentration was 6.7 mmol/1, despite dialysis against a 1.5 mmol/1 potassium bath just before the infusion. The next dose was given during dialysis, and the serum potassium concentration was 2.6 mmol/1 after the infusion. 

A 14-month-old girl with chronic renal insufficiency due to renal dysplasia was empirically treated with ceftazidime and vancomycin for fever. Her calculated creatinine clearance was 10 ml/minute/1.73 m. She erroneously received vancomycin 1.5 g in 3 doses 6 hours apart. Her serum creatinine concentration increased and her vancomycin concentrations remained markedly high (338 mg/1 5 hours after the third dose). The half-Ufe of vancomycin was 145 hours. Hearing loss developed. Continued charcoal hemoperfusion and hemodialysis were used to treat the disorder. Thrombocytopenia was noted as significant consequence of hemoperfusion. The patient did not fully recover her previous renal function and became dialysis dependent. The audiogram normalized by 6 months. 

Curr Opin Pediatr 17 690-694 Poznanski A (1969) A simple device for administering barium to infants. Radiology 93 1106 Quan L, Smith WL (1973) The VATER association vertebral defects, anal atresia, T-E fistula with esophageal atresia, radial and renal dysplasia a spectrum of associated defects. J Pediatr 82 104-107... 

Teratogenicity A woman who took candesartan 8 mg for 2 years before and throughout her pregnancy for idiopathic hypertension delivered a boy by cesarean section at 34 weeks who subsequently died following respiratory difficulties [51 ]. The autopsy showed hypoplasia, renal dysplasia, and calvarial hypoplasia with brain malformation. The authors noted that the known risk of fetal toxicity associated with candesartan may be ignored by prescribers. 

Pregnancy Ababy boy was bom at 35 weeks gestation to a mother being treated with oxcarbazepine 1400 mg/day. The baby developed hyponatremia by day of life four, and a withdrawal syndrome by day seven. He was additionally found to have severe left hydronephrosis and bilateral hyperechogenic renal parenchyma compatible with renal dysplasia on kidney ultrasoxmd, as well as mild aortic stenosis, a bicuspid aortic valve, patent foramen ovale, and patent ductus arteriosus on echocardiogram [112 ]. 

Mackie GG, Stephens FD (1975) Duplex kidneys a correlation of renal dysplasia with position of the ureteral orifice. JUrol 114 274-280... 

Jeune syndrome (asphyxiating thoracic dystrophy) Small thorax, polydactyly, rhizomelic limb shortening, trident pelvis, biliary dysgenesis and pancreatic dysplasia Renal dysplasia, juvenile nephronopthisis, hydroureters, multicystic kidneys AR (2008500)... 

Short rib polydactyly syndromes type I (Saldino Noonan) and type II (Majewski) Short ribs and limbs, genital and visceral anomalies, biliary dysgenesis, pancreatic dysplasia Renal dysplasia, cystic kidneys AR (263530)... 

Tuberous sclerosis Hypopigmentated macules, adenoma sebaceum, retinal and brain tumors, seizures, mental retardation Renal angiomyolipomas (40-80%), renal dysplasia, cortical cysts, renal vascular anomalies, renal cell carcinomas, polycystic kidney disease AD (191100) new mutations in about 85% of cases, variable expressivity, incomplete penetrance TSCl, TSC2 and further gene loci... 

Prune belly syndrome Deficient abdominal muscles, urinary obstruction/ distension, cryptorchism, malrotation of the gut, clubfeet, limb reduction anomalies Urethral atresia, ureteral duplication, bladder distension, hydronephrosis, renal dysplasia Mostly sporadic, heterogeneous (100100)... 

Renal angiomyoliposmas (40-80%), renal dysplasia, cortical cysts, renal vascular anomalies, renal cell carcinomas, polycystic kidney disease... 

Mandell J, Peters CA, Retik AB (1990) Current concepts in the perinatal diagnosis and management of hydronephrosis. Urol Clin N Amer 17 247-262 Matsumoto JS (1986) Acquired lesions involving the ureter in childhood. Semin Roentgen 21 166-167 McGrath MA, Estroff J, Lebowitz RL (1987) The coexistence of ohstruction at the UPJ and UVJ. AJR 149 403-406 Mckie GG, Stephens FD (1975) Duplex kidneys a correlation of renal dysplasia with position of ureteral orifice. J Urol 114 274-280... 

Rosenfeld DL, Lis E (1993) Gartner s duct cyst with single vaginal ectopic ureter and associated renal dysplasia or agnesis. J Ultrasound Med 12 775-778 Shozu M, Akasofu K, Harda T, Kubota Y (1991) A new cause of female pseudohermaphrodism placental aromatase deficiency. J Clin Endocrinol Metab 72 560-566 Siegel MJ (1995) Pediatric sonography, 2nd edn. Raven Press, New York, pp 441-442... 

Very frequently more than one organ system is involved. This is also reflected by the so-called VATER or VACTERL association that combines vertebral anomalies, anorectal atresia, cardiac failure, tracheoesophageal fistula, esophageal atresia, renal dysplasia, and limb malformation. 

Table 10.2. Renal dysplasia. Various manifestations of renal dysplasia, including cystic and syndromal forms, adapted according to Bernstein (1992), Kissane (1990), Hoyer (1996) and Watkins (1997)...

Multicystic renal dysplasia Obstructive renai dysplasia ... 

Bernstein J (1971) The morphogensis of renal parenchymal maldevelopment (renal dysplasia). Pediatr Clin North Am 18 395-407... 

Huttunen NP (1976) Congenital nephrotic syndrome of Finnish type. Study of 75 cases. Arch Dis Child 51 344-348 Jeon A, Cramer BC, Walsh E et al (1999) A spectrum of segmental multicystic renal dysplasia. Pediatr Radiol 29 309-315... 

Patten RM, Mack LA, Wang KY et al (1990) The fetal genitourinary tract. Radiol Clin North Am 28 115-130 Persky L, Izant R, Bolande R (1967) Renal dysplasia. J Urol 98 431-435... 

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