Multicystic dysplastic kidney detected by fetal sonography: conservative management and follow-up.

The most common cystic lesion recognized antenatally is multicystic dysplastic kidney (MCDK). Recently, conservative management without nephrectomy has been advocated. The purpose of this study was to report our experience in the conservative management of unilateral MCDK. Between 1989 and 1997, 20 children with MCDK detected by prenatal ultrasonography (US) were prospectively followed. At birth, US confirmed the prenatal findings in all cases. All patients were submitted to radioisotope scans and a micturating cystogram. Follow-up US examinations were performed annually. Mean age at diagnosis during the prenatal period was 31 weeks of gestation (range 24-38). Median follow-up time was 33 months (range 7-91). Follow-up US was performed in 19 children; 13 (68%) showed partial involution, 4 (21%) complete involution, and 2 (11%) an increase in unit size. The mean age at complete or partial involution of the lesion was 18 months. No children developed hypertension or tumors, and all maintained normal growth. In conclusion, the natural history of MCDK is usually benign, and serial US examinations show that affected kidneys frequently show involution with time.

Outcome of fetal urinary tract anomalies associated with multiple malformations and chromosomal abnormalities.

The purpose of this study was to identify prognostic factors and describe the outcome of prenatally detected renal anomalies associated with multiple malformations and chromosomal defects. Forty-one fetuses were included in the analysis. Prenatal ultrasound reports, neonatal records and autopsy information were retrospectively reviewed. Prognostic factors associated with fetal echography and clinical and laboratory findings on admission were studied. Data were analyzed by univariate analysis in which variables associated with adverse outcome were identified by the Chi-square test or Fisher exact test. The abnormalities associated with renal anomalies were divided into three groups: chromosomal defects (21%), previously described syndromes and conditions (24%), and new sporadic conditions (55%). Of 41 children admitted, 30 (76%) died during the perinatal period. The presence of oligohydramnios was significantly associated with an adverse outcome (OR=11, p=0.05). Male gender was a protective factor against death during the perinatal period (OR=0.11, p=0.01). In conclusion, prenatally detected renal anomalies associated with multiple malformations and chromosomal defects had a poor prognosis. The presence of oligohydramnios increased the risk of death, and male gender had a protective role against poor outcome.

[Idiopathic hypercalciuria: presentation of 471 cases] / Hipercalciúria idiopática: apresentação de 471 casos.

OBJECTIVE: To analyze the clinical history and evolution of children and adolescents with IH, emphasizing some of their peculiar features. METHODS: We followed 471 patients with IH at an outpatient clinic. Patients were submitted to the following protocol: abdominal X-ray, kidney and urinary tract ultrasonography; urinary ionogram, blood gas and biochemical analyses; 24-hour urine for measurement of calcium and other electrolytes and creatinine; urinalysis, urine culture and phase-contrast microscopy; second morning urine collected after fasting for measurement of calcium and creatinine. RESULTS: At the time of diagnosis, 6% of the patients were infants, 15% pre-school children, 55% school children, and 24% adolescents; 56% of them were boys. Clinical and laboratory findings were: 47% had hematuria and abdominal pain, 31% had isolated hematuria, 14% isolated abdominal pain, and 8% had urinary tract infection, nocturnal enuresis, suprapubic pain or urethralgia, or the frequency/urgency syndrome with urinary incontinence. Hypercalciuria was associated with urolithiasis in 56% of patients. There was association with hyperuricosuria in 18.5% of the cases, and hypocitraturia in 8.5% of the cases. Evolution was poor for 33% of the patients, with recurrence of nephrolithiasis, persistence of hematuria, and abdominal pain. CONCLUSIONS: IH must be diagnosed and treated with criteria in order to reduce consequences such as hematuria, abdominal pain, urinary stone formation and possible bone involvement. Signs and symptoms such as urgency and urinary incontinence, suprapubic pain and nocturnal enuresis may result from renal hyperexcretion of calcium.

Primary megaureter detected by prenatal ultrasonography: conservative management and prolonged follow-up.

With the widespread use of obstetric echography the incidence of fetal hydronephrosis has been reported more frequently. Consequently, many uropathies have been detected in asymptomatic neonates. The authors report their experience with prenatally detected primary non-refluxing megaureter. Newborns with fetal hydronephrosis were investigated by ultrasonography and micturating cystourethrogram after the beginning of chemoprophylaxis. If primary megaureter was identified, after 1 month the children underwent 99tm-DMSA, diuretic 99tm-DTPA, and intravenous urography. Eight infants with primary megaureter (bilateral in 3 cases) were identified, for a total of 11 renal units for study. All children were submitted to non-operative management. We performed ultrasonography and diuretic 99tm-DTPA during follow-up, which lasted on average 75 months. The mean cross-sectional diameter of the dilated ureter was 13.6 mm during neonatal period, and reached 8.4 mm at the end of follow-up. The renal function and the diuretic renogram remained stable throughout follow-up. Two neonates presented transitory hypertension. Our results support the notion that conservative management is safe for primary megaureter detected in asymptomatic neonates, with most cases showing spontaneous regression during a prolonged follow-up.

Predictive factors of fetal urethral obstruction: a multivariate analysis.

OBJECTIVE: The purpose of this study was to identify predictive factors of fetal urethral obstruction. METHODS: One hundred and forty-eight children with fetal hydronephrosis were admitted, submitted to a systematic protocol and prospectively followed. Possible predictive factors of urethral obstruction associated with fetal echography and clinical findings on admission were studied. The analysis was conducted in two steps. In a univariate analysis, variables associated with urethral obstruction were identified by the chi(2) test or by Fisher's exact test. Then, the variables that were significantly associated with urethral obstruction were included in a multiple logistic regression analysis. RESULTS: After final adjustment by multiple logistic regression analysis, only two variables were identified as independent predictors of fetal urethral obstruction: oligohydramnios (odds ratio, OR = 5, 95% confidence interval, CI, = 1.3-15, p = 0.01) and megacystis (OR = 9, 95% CI = 2.0-40, p = 0.004). The sensitivity and specificity of the combination of both variables were 60 and 98.5%, respectively. CONCLUSIONS: The presence of oligohydramnios and megacystis on prenatal ultrasound is highly predictive of fetal urethral obstruction.

[Diabetes insipidus as an early clinical manifestation of pineal tumor] / Diabetes insipidus como uma manifestação clínica inicial de tumor da região pineal.

OBJECTIVE: To present a pineal tumor diagnosed after long clinical course of diabetes insipidus.CASE REPORT: A ten years old male patient, with symptoms of polyuria, polydipsia and nocturia for 18 months was admitted at the Nephrology Unit with the diagnosis of nephrogenic diabetes insipidus. Six months prior to his admission, he had been submitted to a computed tomography scan, which was considered inconclusive. The diagnosis of central diabetes insipidus was confirmed by a water deprivation test. A head magnetic resonance imaging demonstrated two masses suggesting germinoma.CONCLUSIONS: Our observations show that patients with central diabetes insipidus need close clinical, laboratory and neuroradiological follow-up in order to detect the intracranially tumors in an early stage.

Prognostic factors in fetal hydronephrosis: a multivariate analysis.

With the increasing use of obstetric echography fetal hydronephrosis has been reported more frequently. The purpose of this study was to identify prognostic factors associated with adverse outcome, such as renal failure and death, in fetal hydronephrosis. One hundred and forty-eight children with fetal hydronephrosis were admitted, submitted to a systematic protocol, and prospectively followed. Prognostic factors associated with fetal echography and clinical and laboratory findings on admission were studied. The median follow-up was 39 months. The analysis was conducted in two steps. In a univariate analysis, variables associated with adverse outcome were identified by the Kaplan-Meier method. The variables that were significantly associated with adverse outcome were then included in a multivariate analysis. This analysis, using the multivariate Cox's model, was performed to identify variables that were independently associated with a worse prognosis. Only variables that remained independently associated with adverse outcome were included in the final model. After final adjustment by Cox's multivariate model, three variables were identified as independent predictors of adverse outcome: oligohydramnios, prematurity, and glomerular filtration rate lower than 20 ml/min. Thus, in the presence of oligohydramnios, prematurity, and abnormal renal function, the medical team must plan appropriate follow-up for infants at health centers prepared to investigate and treat uropathies in newborns.

Features of primary vesicoureteric reflux detected by investigation of foetal hydronephrosis.

Primary vesicoureteric reflux (VUR) diagnosed on investigation of foetal hydronephrosis accounts for many antenatally detected uropathies. In order to study foetal VUR and its consequences, newborns with foetal hydronephrosis were investigated by ultrasound, micturating cystourethrogram and 99mTechnetium-dimercapto-succinic acid (DMSA), after beginning of chemoprophylaxis. Twenty-eight infants with VUR (bilateral in 15 cases) were identified giving a total 43 renal units for study. There was a predominance of males (86%), moderate/severe reflux (84%) and renal damage (51%). Presence of renal damage was correlated with the severity of reflux. VUR should be investigated in cases of foetal hydronephrosis and our results support that renal damage is frequently congenital and not secondary to urinary tract infection.

[Fetal hydronephrosis: postnatal management and follow up]. / Hidronefrose fetal: abordagem pós-natal e seguimento.

OBJECTIVE: Fetal hydronephrosis is being detected with increasing frequency and probably this observation will increase as the quality of ultrasound equipment improves. This study evaluated etiology and postnatal clinical outcomes of prenatally detected hydronephrosis. METHODS: In a period of 13 years 148 infants were referred with fetal hydronephrosis. The initial evaluation, after prophylaxis, included ultrasound and voiding cystography. Sequential evaluation was determined by initial findings. RESULTS: Postnatal predominant diagnosis were pelviureteric junction obstruction (24%) and hydronephrosis without obstruction (18%). Follow up median was 38.7 months. Renal function deteriorated in 13 (8.8%) children and 11 (7.4%) died during follow up. Bad prognosis was associated with urethral obstruction. CONCLUSIONS: Fetal hydronephrosis is a clue of urinary tract anomalies. Urinary tract infections and delay in postnatal diagnosis must be prevented in children with this uropathy.

[Hypertension: retrospective study in children from a pediatric nephrology unit]. / Hipertensão arterial: estudo retrospectivo em crianças referendadas a uma unidade de nefrologia pediátrica.

300 hypertensive children, from newborn to 18 years old,were studied, retrospectively, from 1975 to 1992, 105 from those lost for follow-up. In 244 the diagnosis of the primary disease, predominantly glomerulopathy, was established. 122 had chronic renal failure (CRF). From them, 74 arrived at end stage renal disease (ESRD) and 13 have been transplanted. From 12 submitted to surgical treatment, hypertension has been controlled in 8. 54 died, 48 with CRF and 38 with ESRD. Difficulties in controlling those patients are discussed, mainly therapy diet, the use of hypotensive drugs on dialysis treatment. Our conclusion is for the necessity of a precocious diagnosis for hypertension and primary diseases, of a trained interprofessional team as well as availability of adequate equipment to children, mainly for dialytic therapy. For many of these children, renal transplantation is the only definite therapy.

Aspects of Brazilian paediatric nephrology.

Brazil is a large country with different population densities in its five geographical regions, each of which has severe but unevenly distributed socioeconomic problems which affect the health care system. This makes the accurate assessment of renal disease in the large paediatric population which comprises 40% of the total population especially difficult. This paper analyses the experience of one paediatric nephrology unit. Urinary tract infection affecting 44% of the patients was the most common disease. There was a high incidence of vesicoureteric reflux and renal scarring. The proportion of poststreptococcal glomerulonephritis did not exceed 11%, whilst nephrotic syndrome in its various forms represented 20% of the patients. End-stage renal failure was common and difficult to manage. Renal transplantation could not meet the demand for a variety of reasons, thus there has been an alarming annual increase in the number of patients on dialysis. Other diseases encountered in smaller numbers included acute renal failure, other glomerulopathies (mainly IgA), tubulopathies and renal lithiasis.