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Query: UMLS:C0029713 (immaturity)
 4,335 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Diffuse persistent glomerular immaturity and focal proximal tubular ectasia were seen in bilateral open renal biopsy specimens for an infant with fluid and salt depletion and slowly progressive renal failure. Subsequently, diffuse tubulopapillary renal adenoma subtotally replaced each kidney, thereby, necessitating renal transplantation. Origin of diffuse metanephric adenoma from persistent primitive epithelium of the proximal nephron is postulated and partly substantiated. We propose that this case of persistent proximal nephronic epithelial immaturity and diffuse metanephric adenoma is a variant of nephroblastomatosis and that in this case, a first trimester suicide attempt with aspirin may have initiated the maturation defect that preceded neoplastic transformation.
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PMID:Diffuse metanephric adenoma after in utero aspirin intoxication. A unique case of progressive renal failure. 21 20

Trends are changing in the management of infants and children with indirect inguinal hernias. Advances in neonatal intensive care have resulted in the survival of many small premature infants who have a high incidence of inguinal hernia. The rate of incarceration, strangulation, and gonadal infarction in these babies is twice that of the general pediatric age group. Respiratory immaturity, apnea, bradycardia, and associated neonatal conditions require special management at the time of hernia repair, usually performed just before discharge from the neonatal intensive care unit. New information concerning volume loss and depletion of germ cells beginning at 6 months of age in boys with undescended testes has stimulated the performance of orchiopexy when the patient is 1 year of age. More than 90% of boys with cryptorchid testes at the age of 1 year have an associated hernia that requires concomitant repair at the time of orchiopexy. The use of the peritoneal cavity for fluid absorptive purposes in hydrocephalus treated by venticuloperitoneal shunts or of peritoneal dialysis for renal failure and metabolic diseases such as hyperammonemia and lactic acidosis causes increased intraabdominal pressure and results in the appearance of a previously unrecognized hernia. Recognition of these and other conditions associated with a high incidence of hernial occurrence should allow early diagnosis and treatment before the development of complications. Most elective repairs of hernias are safely performed in the outpatient setting; however, some infants and children with concurrent illnesses are best managed in a "morning admissions" program, in which hospital admission occurs postoperatively.
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PMID:Current concepts in inguinal hernia in infants and children. 257

Neonatal renal failure and glomerular immaturity have been described in 1983. The present paper describes 3 cases with the same histological features. However, follow-up of the patients during 14, 18 and 4 years, respectively, showed the following: The histologic immaturity may be followed by maturation. Thus, late maturation may be a term more suitable to designate this constellation. Renal failure may not necessarily ensue, at least not during early infancy. In the 3 cases presented in this report, the histological finding is associated with renal tubular acidosis, renal diabetes insipidus and nephrocalcinosis. The association of transient glomerular immaturity (or late glomerular maturation) and renal tubular acidosis may enhance the development of nephrocalcinosis.
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PMID:Extensive glomerular immaturity associated with renal tubular acidosis, nephrogenic diabetes insipidus and nephrocalcinosis. 360 98

A case of acute renal failure in a neonate was observed in which there were no obvious predisposing factors. Renal biopsy showed marked glomerular immaturity with otherwise normal renal architecture. Light and ultramicroscopic abnormalities noted suggest that the glomerular immaturity caused an abnormally low glomerular ultrafiltration coefficient (Kf). An inadequate rate of glomerular filtration secondary to the low Kf could have precipitated the acute renal failure. The finding of isolated glomerular maturational arrest is a previously undescribed cause of neonatal renal failure.
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PMID:Neonatal renal failure and glomerular immaturity. 683 66

Superficially, the causes of voiding difficulties in children may seem quite simple, but a thorough understanding of the anatomy physiology and neurology of normal and abnormal lower urinary tract function is necessary in uncovering the etiology of often seemingly siple complaints. An ectopic ureter, neurogenic bladder, urinary tract obstruction, or simple urinary tract immaturity, for example, can all cause a child to present with the complaint of "wetting." A familiarity with all the possible causes of voiding dysfunction is required to distinguish significant from insignificant symptoms. While a "wait and see" attitude often results in the spontaneous resolution of a problem such as simple nocturnal enuresis, the misdiagnosis of a severe disorder, such as urinary outflow obstruction, may permit the insidious development of irreversible renal failure. Our purpose in writing this monograph was not to provide a comprehensive guide to the diagnosis of voiding dysfunction in children, but rather to enhance the clinician's appreciation of the complexity of these problems. Our hope is that the perspective that we have provided will obviate the diagnostic and therapeutic exaggeration of insignificant voiding complaints, while assuring that the presence of significant lesions is not overlooked.
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PMID:Voiding dysfunction in children. 741 33

C57BL/6J mice homozygous for the cpk gene exhibit an autosomal recessive (AR) form of polycystic kidney disease (PKD), similar to human ARPKD, with massive collecting duct cysts. These cysts are lined by epithelial with an immature phenotype. Since renal expression of epidermal growth factor (EGF) is also significantly decreased in affected mice, we hypothesized that renal EGF is necessary for normal developmental maturation of the collecting duct. To determine if the lack of EGF may be a decisive factor in the initiation and/or growth of collecting duct cysts, we administered exogenous EGF (1 microgram/g body wt subcutaneously) daily for Postnatal Days 3-9 (a critical period for collecting duct maturation) to C57BL/6J-cpk mice. EGF but not sham or albumin treatment retarded the development of PKD, reduced the degree of renal failure associated with the disease, and prolonged the survival of cystic mice. Sulfated glycoprotein-2 gene expression, a marker of immaturity in collecting duct cells, was reduced in cystic kidney by EGF treatment. This finding indicates that EGF treatment was associated with an increase in the maturation of the collecting duct epithelial cells. These findings support the view that decreased EGF may play a significant role in promoting the enlargement of collecting duct cysts in a hereditary model of ARPKD and that PKD involves defective and/or arrested collecting duct cell maturation.
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PMID:Epidermal growth factor ameliorates autosomal recessive polycystic kidney disease in mice. 778 94

Kaspareit-Rittinghausen described a rodent model of inherited polycystic kidney disease (PKD), the Han:SPRD rat [1, 2], in which heterozygotes develop renal cysts and renal failure (in males) over several months, whereas homozygous animals develop rapidly progressive renal enlargement that leads to death in a few weeks. In this study, we examined selected elements of the pathogenesis of this disease in heterozygotes and homozygotes from birth to advanced disease. Heterozygous male rats developed slowly progressive renal cystic disease with interstitial fibrosis and azotemia seen by six months of age. Female heterozygotes developed slowly progressive renal cystic disease, but did not develop interstitial fibrosis or azotemia. Epithelial cells lining cyst cavities showed various degrees of morphologic immaturity. Cyst walls also developed basement membrane thickening, especially in areas of cellular immaturity, suggesting an interrelationship between this basement membrane thickening and cellular dedifferentiation. Thickened basement membranes were associated with increased immunoreactivity for type IV collagen, laminin, and fibronectin. Homozygous rats developed massive renal enlargement, marked azotemia, and died near three weeks of age. Renal c-myc proto-oncogene expression was elevated in homozygous cystic infants and in adult heterozygotes. In situ hybridization showed high levels of c-myc mRNA in cyst epithelia, suggesting abnormal regulation of cellular proliferation in the cells lining cysts, as seen in other models of PKD. The Han:SPRD rat is the only well-documented animal model of inherited PKD with an autosomal-dominant inheritance pattern and appears to have several features which resemble human ADPKD.
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PMID:Autosomal-dominant polycystic kidney disease in the rat. 845 52

Myocardial calcification has been rarely described in premature infants after myocardial infarction and myocarditis with coxsackievirus B1. In adults and older children, metastatic myocardial calcification has been reported in chronic renal failure. We report a case of myocardial calcification in a 680-gm preterm infant after a prolonged course of renal failure complicated by secondary hyperparathyroidism. Subclinical myocardial injury was evidenced by a high serum creatine phosphokinase MB band concentration, which probably provided a susceptible substrate for the deposition of calcium crystals, because the multiplication product of serum calcium and inorganic phosphorus levels transiently exceeded 75 mg x mg/100 ml, indicating serum saturation during the course of secondary hyperparathyroidism. We report this case as an unusual complication of renal immaturity in extremely low birth weight infants and an indication of a relatively intact parathyroid glandular function in them. Hypoxia, myocardial dysfunction, and renal failure are common complications in such infants, and in the presence of renal failure, the serum levels of calcium and inorganic phosphorus should be maintained below the pathologic level to avoid ectopic calcification of the tissues, including the myocardium.
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PMID:Myocardial calcification in an extremely low birth weight infant with chronic renal failure and secondary hyperparathyroidism. 851 2

Clinical and morphological analysis was made to assess 9 cases of minimal change disease (MCD) and 30 cases of mesangial glomerulonephritis (GNMES) recognized by light microscopy with unfavourable course. Case selection was based exclusively on the clinical course suggesting a possibility of early sclerosis (long-term steroid resistance, frequent recurrences, rare short-lasting remissions, hypertension, renal failure). It was found that the unfavourable clinical course was clearly more frequently associated with electron microscopic than light microscopic changes. Marked increase of the matrix was observed also in those glomeruli in which light microscopy did not reveal any changes or only the signs of immaturity. It was also noticed that in those cases in which the assessment of mesangial matrix increase (which means the onset of sclerosis) is not certain, it is useful to make a morphometric analysis of electron microscopic material.
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PMID:Clinical and morphological (including morphometric) aspects of minimal change disease and mesangial glomerulonephritis with unfavourable course in children. 1062 18

Cranioectodermal dysplasia (CED) is a rare autosomal recessive disease with characteristic craniofacial, skeletal, and ectodermal-derived tissue abnormalities. In this disease, tubulointerstitial nephropathy (TIN) has been reported as one of the life-threatening combinations. Here we report a sporadic case of CED showing signs of renal failure during the perinatal period. Renal biopsy at the age of 6 months revealed TIN consisting of marked interstitial fibrosis with inflammatory cell infiltration accompanied by scattered tubular atrophy. Glomeruli were often sclerosed and others showed prominent immaturity; the findings are supportive of progressive deterioration of renal function in this infant. This case suggests that TIN in CED can occur during the fetal period and progress rapidly, leading to end-stage renal failure in infancy.
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PMID:Renal failure due to tubulointerstitial nephropathy in an infant with cranioectodermal dysplasia. 1649 15


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