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Preliminary results of this retrospective-prospective analysis of renal hypertension in 110 children indicate that hypertension may be secondary to a wide variety of acute progresive, and chronic renal diseases which may be either congenital or acquired. Affected children may be detected at any time from infancy through adolescence. Symptoms usually associated with acute glomerulonephritis (i.e., headache, swelling, nausea, vomiting, anorexia, fatigue, dizziness, and fever) occur in both acute and chronic renal diseases associated with hypertension. Headache and swelling are the most common symptoms in this series. Peripheral edema, rales, and increased heart size were found in between 10 and 25% of these children. Differential diagnosis may be approached by a consideration of causes of acute and chronic hypertension. The child with chronic renal disease usually presents with a long history of fatigability, poor growth, and pallor, and laboratory tests reveal elevation of the creatinine and BUN along with anemia, hypocalcemia, and hyperphosphatemia. In contrast, the child with acute renal disease and hypertension presents with a history of prior good health followed by the abrupt onset of signs and symptoms of renal disease; laboratory tests usually reveal modest elevations of creatinine and BUN, anemia is unusual, an abnormal urinalysis is common, and serum calcium and phosphorous levels are usually normal. Renovascular and asymmetric renal parenchymal disease represent uncommon but important conditions because surgery may be curative. Treatment may be surgical, medical, or combined. Surgical conditions include renal trauma, hydronephrosis, asymmetric renal disease, and renal arterial disease. Adequate blood pressure control without medication can be expected following surgery in instances of unilateral involvement with a normal contralateral kidney. Meticulous assessment of the contralateral kidney is needed to determine that it is normal. If surgery is unsuccessful or is not indicated, pharmacologic therapy is initiated with a stepwise regimen starting with the mildest agent (e.g., thiazides) and then adding additional antihypertensive drugs when adequate blood pressure control has not yet been achieved. The goal of therapy is the lowest, safest, tolerated blood pressure levels. Long-term, carefully designed studies of antihypertensive agents for children with renal hypertension are not available. The need for collection and critical analysis of data concerning the clinical course of children with renal hypertension is evident from a review of the literature and from the preliminary data presented in this series. The presentation of such information and a critique of outcome variables will provide a basis for program planning for affected children and improvement in patient care where indicated.
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PMID:Renal hypertension in children. 99 44

Reported in this paper is the case of a female newborn with mesenchymal hamartoma of the liver. High-accuracy ultrasonography diagnostics were applied to the mother admitted for examination for suspicion of fetal hydronephrosis in the 31st week of pregnancy. A 7 x 4 cm compartmental process was delineable in the abdominal region of the foetus and was variably viewed in ultrasonography in cystic or solid form. A girl, 2,650 g in body weight and 47 cm in body length, was delivered by caesarean section in the 35th week of pregnancy. Postnatal nutritional disorders were accompanied with recurrent vomiting. Sonography, computed tomography and scintigraphy were performed, and the prenatally diagnosed process was identified as a cystic growth in the right liver lobe with enclosure of the V. cava inferior. A typical hepatolobectomy was performed on the newborn on her 15th day of age, with the V. cava enclosing part being left unextirpated. Mesenchymal hamartoma of the liver was the histological diagnosis. Only one prenatally diagnosed case of this kind had been known from literature before.
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PMID:[Prenatally diagnosed mesenchymal hamartoma of the liver]. 161 Jul 66

Salt losing nephropathy, occurring predominantly in male infants, has been reported in association with a spectrum of urologic diseases including obstructive uropathy and massive, infected vesicoureteral reflux (VUR). This has been called pseudo-hypoaldosteronism (PHA) or alternatively, pseudo salt-losing congenital adrenal hyperplasia (CAH), and is thought to reflect a tubular unresponsiveness to aldosterone. We report our experience with six cases, discuss one case in detail and review the 39 cases previously reported. A one month old male infant presented with a left upper quadrant mass. Signs and symptoms included vomiting, dehydration, hyponatremia and hyperkalemia. This suggested the diagnosis of CAH for which therapy was instituted. Ultrasonographic examination subsequently revealed the mass to be a urinoma in an infant with posterior urethral valve (PUV) and obstructive hydronephrosis.
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PMID:Salt losing nephropathy simulating congenital adrenal hyperplasia in infants with obstructive uropathy and/or vesicoureteral reflux--value of ultrasonography in diagnosis. 174 73

We report a case of metastatic ureteral tumor resulting from gastric cancer in a 56-year-old female. She had undergone distal gastrectomy for gastric cancer in our hospital 3 years earlier, on the histological diagnosis of poorly differentiated adenocarcinoma with absolute curative resection. In March, 1987, she visited our hospital complaining of microscopic hematuria and lumbago. Intravenous pyelography and left retrograde pyelography revealed the stenotic change of the left ureter and hydronephrosis. Endoscopic ureteral biopsy was performed, and the histological diagnosis was an inflammatory change of the ureter. But the hydronephrosis increased, so partial ureterectomy was performed. The histological examination confirmed adenocarcinoma in the left ureter resulting from gastric cancer. From the 340th postoperative day, she complained of general fatigue and vomiting, and gastroscopy revealed recurrent gastric cancer.
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PMID:[A case of metastatic ureteral tumor]. 219 72

During the past three and half years (Jan, 1984-Jun. 1987), 14 cases of ureteropelvic junction obstruction presented with abdominal pain were encountered in the Department of Pediatrics, Mackay Memorial Hospital. Eight cases were male and six female. Their ages ranged from 4 years old to 12 years old. The duration of abdominal pain lasted for several days in 5 cases, several months in 4, and several years in another 5 cases. The abdominal pain was usually recurrent. It occurred once every 2 to 6 months. The location of pain was predominantly in the left abdomen and the periumbilical area. Cramping was the most characteristic symptom. Besides abdominal pain, several cases also had vomiting or abdominal mass. Laboratory examination showed some cases to have microscopic hematuria and others pyuria. All were proved by renal ultrasonography to have varying degrees of hydronephrosis. Intravenous pyelography or retrograde pyelography also confirmed the diagnosis of ureteropelvic junction obstruction. In 9 of the 14 cases, surgical intervention was performed to correct abnormalities. Six cases were followed up regularly; all showed improvement, however, one developed ureteropelvic junction obstruction on the contralateral kidney one year later. The remaining five cases who did not receive surgical treatment continued to have recurrent abdominal pain.
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PMID:[Ureteropelvic junction obstruction presenting with abdominal pain: a analysis of fourteen cases]. 227 77

The determination of acetylcholinesterase (AChE) has been shown to be as specific as alphafetoprotein (AFP) for the prenatal detection of open neural tube defects although AFP remains the method of choice. This paper describes a semi-automated technique for the analysis of acetylcholinesterase in amniotic fluid that: A) reduces the cost of the procedure; B) allows for a larger number of samples to be run at a time; and C) provides for more accurate and reproducible procedures and results. Six fetuses with neural tube defects (2 with gastroschisis and 3 where one twin was dead) were detected and found to have elevated AChE, TChE and 2 bands by electrophoresis. Quality control procedures using both pure enzyme and amniotic fluid with low and high levels of the enzyme are described. The analysis of 340 amniotic fluids of normal pregnancies indicates that the normal value for AChE is 5.17 +/- 2.63 mU/ml (97% confidence interval for the mean 4.84-5.49 mU/ml. A group of 27 abnormal pregnancies provides evidence that fetal vomiting and regurgitation, fetal demise, multiple cysts syndrome, idiopathic IUGR, arthrogryposis multiplex, hydrocephaly (stenosis of aqueductus), trisomy 21, trisomy 18, hydronephrosis, pyloric stenosis, heart malformation, ectopia cordis and multiple gestation produce elevated levels of pseudocholinesterase (PChE) in amniotic fluid. The use of pseudocholinesterase levels in amniotic fluid for prenatal diagnosis is proposed and discussed in view of its elevated levels in abnormal pregnancies where AChE is normal. The normal values for PChE are 23.86 mU/ml (mean) and 5.83 for standard deviation. Electrophoretic analysis was performed on all samples with values higher than one standard deviation above the mean.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Determination of cholinesterase and acetylcholinesterase in amniotic fluid. Uses in prenatal diagnosis and quality control. 242 50

A case of lupus cystitis in a 23-year-old male is reported. The patient began to complain of diarrhea and vomiting in October, 1985. When the diagnosis of systemic lupus erythematosus (SLE) was established at the Department of Internal Medicine in our hospital, he was referred to our clinic for examination of pollakisuria on November 22. DIP revealed a loss of bladder distensibility, and bilateral hydronephrosis and hydroureter. Transurethral cold cup biopsies revealed subcutaneous edema. A diagnosis of lupus cystitis was made and he was treated with steroids, which resulted in symptomatic and radiographic improvement.
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PMID:[A case of lupus cystitis]. 273 71

We report a case of a 37 year old man who presented with vomiting and colicky abdominal pain and who was found to have microscopic haematuria. Radiological-examination showed a right hydronephrosis apparently caused by a paracaecal tumour extending to involve the right ureter. This was resected and proved histologically to be a mesenteric fibromatosis. The patient has none of the factors predisposing to the development of this lesion, in particular Gardner's syndrome. Eight months following surgery he appears to have made a full recovery. Previous publications on this rare intra-abdominal neoplasm are reviewed.
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PMID:Mesenteric fibromatosis with ureteric stenosis. 342 65

Nephrogenic diabetes insipidus usually presents with polyuria, polydipsia, fever, vomiting, dehydration and failure to thrive. However, in infancy polyuria may be absent because of dehydration and reduced glomerular filtration rate. In 2 cases the main presenting feature was hypotonia, with marked head lag. Family studies confirmed the X-linked mode of inheritance of the disease; in case 1 the disease appeared to have arisen as a new mutation in the mother, and in case 2 the carrier status was traced back to the great-grandmother. Pitfalls in the diagnosis and detection of the carriers are discussed. Treatment with thiazide diuretics and prostaglandin synthesis inhibitors is effective in reducing urine volumes and polydipsia. The early detection of the disease and adequate management may prevent such complications as megacystis, mega-ureter and hydronephrosis, with resulting renal failure. Mental and physical retardation may also be avoided.
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PMID:Nephrogenic diabetes insipidus presenting with infantile hypotonia. A report of 2 cases. 373 62

The recent published literature dealing with urinary tract calculi during pregnancy has been summarized. Our own experience with 17 patients, (0.08% of the deliveries) in a recent 12-year interval has been described. Emphasis must be placed on the safety and limitations of renal ultrasonography. Excretory urography should be performed in patients with urinary infection not responding after 48 hours of antibiotic therapy, with declining renal function, with massive hydronephrosis on renal echography, or with pain and dehydration from vomiting. The timing of postinjection films is critical; a 3-hour film and, if needed, a 6-hour film are recommended. Criteria for intervention (nonoperative or operative) include calculous pyelonephritis, persistent massive hydronephrosis with impairment of renal function, and protracted pain or sepsis.
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PMID:Renal calculi in pregnancy. 389 20


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