Assuntos
Pielonefrite/diagnóstico por imagem , Infecções Urinárias/diagnóstico por imagem , Criança , Humanos , Córtex Renal/diagnóstico por imagem , Pielonefrite/diagnóstico , Pielonefrite/etiologia , Radiografia , Cintilografia , Sensibilidade e Especificidade , Bexiga Urinária/diagnóstico por imagem , Refluxo Vesicoureteral/complicaçõesRESUMO
Fifteen children with renovascular hypertension as a result of aortic thrombosis were followed for a mean of 26 months (range 5 to 58 months) to determine outcome. As neonates, all patients had hypertension and elevated plasma renin activity. Of 11 patients studied with radionuclide renography and scintigraphy, 10 had abnormal renal blood flow; three had complete absence of unilateral perfusion. On follow-up examination all children were normotensive; five children ages 5 to 24 months required antihypertensive medication. Of 15 children, 14 had normal statural growth; all had normal serum creatinine, plasma renin activity, and calculated glomerular filtration rate values. Patients with complete absence of renal perfusion unilaterally remained functionally anephric; children with less severe perfusion deficits had improved perfusion as shown by radionuclide renography and scintigraphy. We believe that many patients with aortic thrombosis and renovascular hypertension who have had aggressive antihypertensive therapy in the neonatal period will have good renal function and increased perfusion to the affected kidney 2 years later.
Assuntos
Doenças da Aorta/fisiopatologia , Hipertensão Renovascular/fisiopatologia , Trombose/fisiopatologia , Doenças da Aorta/diagnóstico por imagem , Doenças da Aorta/etiologia , Cateterismo Periférico/efeitos adversos , Creatinina/sangue , Seguimentos , Taxa de Filtração Glomerular , Humanos , Hipertensão Renovascular/diagnóstico , Hipertensão Renovascular/etiologia , Recém-Nascido , Prognóstico , Estudos Prospectivos , Cintilografia , Renina/sangue , Trombose/diagnóstico por imagem , Trombose/etiologia , Ultrassonografia , Artérias UmbilicaisRESUMO
Renal function was studied in 74 subjects who, between 1966 and 1972 (ages 1 to 6 years) had had blood lead levels (PbB) greater than or equal to 100 micrograms/dl (range 100 to 471 micrograms/dl, median 142 micrograms/dl) and in 21 sibling controls. PbB measured in 1983 in study subjects remained significantly higher than in sibling controls (mean +/- 1 SD 14.5 +/- 4.5 vs 11.6 +/- 2.6 micrograms/dl, P less than 0.01). The two groups did not differ in development of hematuria or leukocyturia. The frequency of elevated serum creatinine concentration, depressed creatinine clearance, elevated protein excretion, low urinary osmolality, elevated serum beta 2-microglobulin (beta 2-M), and elevated fractional excretion beta 2-M % X 100 was similar in the two groups. Mean values for these tests were similar in study subjects compared with sibling controls. Mean systolic blood pressure was significantly higher in study subjects compared with that in sibling controls (117 +/- 12 vs 109 +/- 10 mm Hg), but the control group contained a preponderance of females and the study group had more overweight females; mean diastolic blood pressure was similar in the two groups. We conclude that in our adolescent subjects who had had lead poisoning 9 to 17 years earlier, there is little if any evidence of chronic nephropathy.
Assuntos
Nefropatias/induzido quimicamente , Intoxicação por Chumbo/complicações , Pressão Sanguínea , Peso Corporal , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Rim/fisiopatologia , Nefropatias/fisiopatologia , Testes de Função Renal , Chumbo/sangue , Estudos Longitudinais , Masculino , Urina/análiseRESUMO
The therapeutic efficacy of oral glycine was tested in a 3-year-old girl with isovaleric acidemia. An oral leucine load (25 mg/kg) caused a rise of the blood levels of isovaleric, lactic, and pyruvic acids as well as an increase of urinary excretion of the ketone bodies. These changes did not occur when oral glycine (250 mg/kg) was given with the leucine. Glycine supplementation favored the formation of isovalerylglycine, a nontoxic conjugate of isovaleric acid which is excreted rapidly. Excretion of isovalerylglycine rose threefold when leucine and glycine were administered simultaneously. Chronic glycine therapy was tolerated well and may have prevented one episode of ketoacidosis.
Assuntos
Erros Inatos do Metabolismo dos Aminoácidos/terapia , Glicina/uso terapêutico , Valeratos/sangue , Acidose/terapia , Administração Oral , Pré-Escolar , Esquema de Medicação , Feminino , Glicina/administração & dosagem , Glicina/análogos & derivados , Glicina/urina , Humanos , Cetoácidos , Corpos Cetônicos/urina , Lactatos/sangue , Leucina/metabolismo , Piruvatos/sangueRESUMO
Since hypocitraturia in distal renal tubular acidosis, we screened the asymptomatic children in three families with familial dRTA, by comparing their 24-hour urine citrate excretion to values obtained in 45 normal children. Subsequent acid loading uncovered four new cases of dRTA suspected because of the finding of hypocitraturia. Because hypocitraturia probably contributes to nephrolithiasis/nephrocalcinosis and subsequent renal damage in dRTA, affected family members were treated with alkali (4 mEq/kg/day), which normalized urine citrate in three children; in a fourth child citrate excretion rose but was not normal. Measurement of urine citrate excretion was superior to other currently proposed screening tests for dRTA (first morning urine pH and sediment, urine concentration).
Assuntos
Acidose Tubular Renal/diagnóstico , Citratos/urina , Acidose Tubular Renal/genética , Acidose Tubular Renal/urina , Adulto , Criança , Feminino , Humanos , Túbulos Renais Distais , MasculinoRESUMO
In the study of children with language problems, particularly in reading and writing, it has been observed that some have an outstanding ability to produce artistic pictures and objects. These productions are perceptive, well organized and generally contain much action. Despite their pictorial skill these patients may have only a rudimentary use of coded symbolic graphic forms. Others display moderate ability in reading and writing. These patients frequently have the disorganized overacctive behavior and the motor clumsiness that is so common in the dyslectic child; some, however, are biologically effective. From this material we entertain the hypothesis that picture (artistic) productions are generated by the sub-dominant cerebral hemisphere, and that this function is quite distinct from the coded graphic operations resident in the dominant hemisphere. If this hypothesis is correct, it would seem socially benefical to allow these patients to develop their unique artistic ability to its full capacity, and not to overemphasize the correction of the disturbed coded symbol operations in remedial training.
Assuntos
Arte , Criatividade , Dislexia/psicologia , Adolescente , Criança , Dominância Cerebral/fisiologia , Dislexia/fisiopatologia , Humanos , Idioma , MasculinoRESUMO
A patient is described in whom lactic acidosis of very severe degree was found to coincide with the presence of beta-methylcrotonic acid and rho-hydroxyphenyllactic acid in urine in large amounts, while beta-hydroxyisovaleric acid was found to be a relatively minor excretion product. Beta-methylcrotonic acid is demonstrated, for the first time, to be present in blood and CSF. These findings are discussed in relation to the patients previously reported to have beta-methylcrotonylglycinuria and raise the possibility that our patient's organic aciduria may be secondary to acquired disease rather than to an inborn error of metabolism.