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The authors report a survey of the French Group of Visceral Pediatric Surgery, concerning 985 congenital anomalies of the pyeloureteric junction in 883 children. 67.3% of them are boys. This article stresses the recent improvements in prenatal ultrasonography and diagnosis which is now able to individualize a homogeneous group of asymptomatic children ready to be repaired in the early post-natal period. Early reconstruction is associated with the greatest degree of recovery of renal function. In children, the average age is 4 years 9 months at diagnosis. In 43.6% clinical symptoms suggest an infectious problem. Pain is noted in 35.5% and an abdominal mass is discovered in 13.3%. Excretory urography (IVP), ultrasonography and cystography are still the main investigations to be done in all cases. But it is now necessary to use modern isotopic technics such as DMSA and diuretic DTPA to quantify obstruction with accuracy and follow long-term evolution after treatment. Treatment is surgical and conservative in 81.4%. Almost all of the uretero-pyeloplasties are performed according to the dismembered technic usually with temporary nephrostomy drainage. Renal reduction is reserved only for the redundant and decompensated renal pelvis. Morbidity is low (2.5%) and good results can be expected in about 95% of cases.
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PMID:[Congenital anomalies of the pyeloureteral junction in children. Multicenter study of 985 anomalies in 883 children]. 331 79

Ultrasound-guided renal cyst puncture was performed on 22 cysts which were then 95% ethanol instilled to prevent recurrence of cystic fluid. Cystic lesions disappeared on the ultrasonogram in the follow-up period of 3 to 28 months. On CT, cystic lesions became smaller size but did not disappear. Average CT numbers of the cyst were 8.75 +/- 3.83 before and 12.96 +/- 3.27 after ethanol instillation. The cystic wall became thicker. Caliceal distortion and/or pelvis compression by cystic lesions improved on IVP 2 to 3 days after ethanol instillation. The renal image on Tc-99m-DMSA scintigram showed morphological improvement and DMSA renal uptake rate increased slightly but significantly 2 to 4 weeks after ethanol instillation. There were no major complications with this procedure except for one case in which the tip of the catheter became stuck in the cyst and broke off when the catheter was removed. A slight local irritable pain was noticed in all cases. Half of the patients had hot flushes and/or somewhat drunken sense but these symptoms were only temporary. Antabuse phenomenon appeared in one case with concomitant use of a cephem antibiotics after ethanol instillation. This method of therapy is a safe non-surgical approach to treat renal cysts. 95% ethanol instillation in the cyst seems to prevent recurrence of cystic fluid.
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PMID:[Ultrasound-guided renal cyst puncture and 95% ethanol instillation. Part 2: Morphological and functional alterations]. 647 82

The authors review 178 cases of anomalies of the pyelo-ureteric junction, with a total of 189 anomalies. Almost one half of the cases were less than 5 years old (47.2%) and about 1/4 were less than 1 year old (24.7%). In girls, the incident which reveals the diagnosis is most often an infection and in boys, it is pain or haematuria. 26% of the cases had an associated urological lesion (23 cases of vesico-ureteric reflux, 8 cases of renal stones, 5 cases of mega-ureter and 4 cases of horseshoe kidney). 133 children were operated, essentially by resection of the pyelo-ureteric junction with an upstream by-pass. From the 126 cases with sufficient follow-up, 48 (38.1%) obtained great improvement, 63 (50%) a moderate improvement and 15 (11.9%), a stabilisation of the dilatation. In all cases except 3, the pyelo-ureteric junction was perfectly catheterizable. The histological lesions of the junction were variable and difficult to systematise, but usually extended beyond the junction to involve a large part of the pelvis. The variability of the caliceal dilatation makes any attempt at classification difficult. The authors recommend cystography in every case, as associated reflux was discovered in 16% of cases. Retrograde uretero-pyelography is practically no longer indicated. The isotope kidney scan with DTPA technetium, with a hyper-diuresis test, is often used, as it gives an idea about the clearance of filtration and about the degree of obstruction. Quantitative DMSA kidney scan can reveal the effects on the renal parenchyma. Conservation of the isthmus of a horseshoe kidney never interferes with the uretero-pyelic re-implantation. After correction of the anomaly of the uretero-pyelic junction, 5 cases of unilateral reflux out of 8 and 3 cases of bilateral reflux out of 10 resolved spontaneously.
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PMID:[Pyeloureteral junction syndrome in children. Apropos of 178 cases]. 687 97

1. We report two cases of acute mercury vapour intoxication in humans. The mercury vapour was released from smelting alloys (gold-mercury amalgam). The alloy was apparently contaminated with an unknown amount of mercury. 2. Within half an hour of the incident, the victims began having moderate headache, nausea, lumbar pain and shortness of breath at rest. The patients were treated with BAL (2,3 dimercaptopropanol), followed by DMSA (2,3 dimercaptosuccinic acid). 3. Serial measurements of mercury metal in plasma and in urine were made for ten days. 4. The results suggest that in spite of the treatment, relatively high concentrations of mercury remain in the plasma for a very long time, and this could be explained by the progressive release of mercury from red blood cells and tissues after oxidation. However, BAL and DMSA did not seem to be the most efficient antidotes. They reduce the plasma inorganic mercury uptake at concentrations of < 50 micrograms I-1.
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PMID:Elemental mercury vapour toxicity: treatment and levels in plasma and urine. 771 4

Over the past 4 years 18 children were observed at the La Timone-Enfants Hospital in Marseille with renal lesions consequent to nonpenetrating abdominal traumas. Hematuria was reported in 14 cases (7 macroscopic and 7 microscopic) and all cases suffered from abdominal and/or lumbar pain. In relation to the diagnostic iter used: 18 echographies, 15 urographies, 13 TAC, 2 ascendant pyelographies, 2 scintigraphies with DMSA, 1 echo-Doppler and one angiography were performed. Renal lesions were classified as: 8 type 1, 5 type 2, 4 type 3, 1 type 4. Four cases were operated and one case underwent percutaneous drainage of an urohematoma. Following an analysis of these case and a revision of the literature, the authors emphasise that the evolution and progress in the diagnosis and treatment of this important aspect of traumatic pathology in children enables an almost complete recovery of kidney function in almost all cases.
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PMID:[Kidney injuries in children]. 780 Dec 10

Over a 40-month period, 24 consecutive children (1-15 years) underwent laparoscopic nephrectomy. The indication for surgery was a poorly functioning kidney (<6% on DMSA isotope scanning) secondary to a variety of causes, with or without pain or infection. Four cannulas were used in each patient. The kidney was approached through a small incision in the upper paracolic gutter without mobilization of the colon. The procedure was successful in all but one child, who had conversion to open technique because of poor laparoscopic viewing. In 12 children who required nephroureterectomy, the distal ureter was approached by an open technique through either a small extension of the iliac fossa cannula site or a Pfannenstiel incision for combined bladder surgery. The average operating time for laparoscopy was 85 (range 40-160) minutes. The children undergoing nephrectomy or nephroureterectomy alone had an average hospital stay of 2 (range 1-4) days. There were no laparoscopic or surgical complications. Laparoscopy provides a safe and successful approach to pediatric nephrectomy. The technique combines well with an open approach to the distal ureter when nephroureterectomy is indicated.
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PMID:Transperitoneal laparoscopic nephrectomy in children. 984 66

Cushing's disease (CD), the chronic endogenous hypercortisolism derived from an ACTH-secreting pituitary adenoma, and multiple osteochondromatosis (MO), a congenital mesoderm dyschondroplasia, represent two distinct rare neoplastic diseases. Clinical appearance of MO usually occurs during the first-second decade of life. In fact, the growth of osteochondromas parallels the patient's growth, then becoming quiescent after the closure of the epiphyses and the achievement of final stature. Here we describe an uncommon case of a patient with a long-term history of childhood-onset CD, who surprisingly developed MO during the third decade of life, after the remission of CD. Indeed, a female patient had been followed for CD from the age of 12 to the age of 24 years, when CD definitively remitted. At the age of 26 the patient complained progressively worsening backache and pain at level of hips and feet. Standard radiography of skeleton showed multiple bone dysmorphisms at level of the four limbs, spine and pelvis consistent with multiple osteochondromas and exostoses. A diagnosis of MO was performed. Total body bone scintigraphy with 99mTc-MDP revealed an increased uptake of the radioligand, suggesting an increased metabolic turnover in correspondence of the majority of the osteochondromas. However, the negativity of the majority of the lesions at 99mTc-DMSA scintigraphy and the histological diagnosis of benign osteochondroma of the only positive lesion at 99mTc-DMSA evidenced that the high metabolic activity of the osteochondromas was not due to malignant transformation. However, the activity of the lesions was highly surprising considering that they usually become quiescent after the achievement of the final stature. In last analysis, the uncommon characteristics of MO and, particularly, its occurrence after stable remission of hypercortisolism, suggests a possible role of glucocorticoids in influencing the clinical course of the skeletal disease. The inhibitory effect of hypercortisolism on bone growth and maturation could explain the block in the proliferation of skeletal lesions during the developmental age, where CD was in the active phase, and the opposite effect of stimulation of the ostochondromas growth during stable normalization of cortisol secretion, after CD remission.
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PMID:Uncommon clinical course of multiple osteochondromatosis in a patient with a long-term history of Cushing's disease. 1213 92

Chronic arsenic toxicity due to drinking arsenic-contaminated water has been one of the worst environmental health hazards affecting eight districts of West Bengal since the early eighties. Detailed clinical examination and investigation of 248 such patients revealed protean clinical manifestations of such toxicity. Over and above hyperpigmentation and keratosis, weakness, anaemia, burning sensation of eyes, solid swelling of legs, liver fibrosis, chronic lung disease, gangrene of toes, neuropathy, and skin cancer are some of the other manifestations. A cross-sectional survey involving 7683 participants of all ages was conducted in an arsenic-affected region between April 1995 and March 1996. Out of a population of 7683 surveyed, 3467 and 4216 people consumed water containing As below and above 0.05 mg/L, respectively. Except pain abdomen the prevalence of all other clinical manifestations tested (e.g., pigmentation, keratosis, hepatomegaly, weakness, nausea, lung disease and neuropathy) were found to be significantly higher in As exposed people (water As > 0.05 mg/L) compared to control population (water As level < 0.05 mg/L). The prevalence of pigmentation and keratosis, hepatomegaly, chronic respiratory disease and weakness rose significantly with increasing arsenic concentrations in drinking water. The respiratory effects were most pronounced in individuals with high arsenic water concentrations who also had skin lesion. Therapy with chelating agent DMSA was not found to be superior to placebo effect. However, therapy with DMPS caused significant improvement of clinical condition of chronic arsenicosis patients as evidenced by significant reduction of total clinical scores from 8.90 +/- 2.84 to 3.27 +/- 1.73; p < 0.0001. Efficacy of specific chelation therapy for patients suffering from chronic As toxicity has further need to be fully substantiated. However, supportive treatment could help in reducing many symptoms of the patients. Treatment in hospital with good nutritious diet has been found to reduce symptom score in a subset of placebo treated patients in West Bengal during the course of DMSA and DMPS trial. People should be advised to stop drinking As contaminated water or exposure to As from any other source. The various clinical manifestations should be treated symptomatically.
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PMID:Chronic arsenic toxicity: clinical features, epidemiology, and treatment: experience in West Bengal. 1263 24

We report a patient with DTPA and DMSA uptake on unsuspected bone metastases. He had severe pain due to grade 3 hydronephrosis of his left kidney. When Tc-99m DTPA and DMSA renal scanning were performed for preoperative evaluation, abnormal radiopharmaceutical uptake on the iliac area was noted. Pancreatic adenocarcinoma metastases to bone were subsequently defined. This patient is a very demonstrative case in respect of having all DTPA, DMSA and HDP uptakes in bone metastases. The type of the tumor has to be added to the list of extrarenal uptake of DTPA and DMSA as a rare cause.
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PMID:Incidental DTPA and DMSA uptake during renal scanning in unknown bone metastases. 1687 10

Urinary tract infections (UTIs) are the most common source of bacterial infections among young febrile children. Accurate diagnosis of acute pyelonephritis (APN) and vesicoureteral reflux (VUR) is important because of their association with renal scarring, leading in the cases to long-term complications. However, the gold standard examinations for both are either DMSA scan (for APN and scar) or cystography (for VUR) and present limitations (feasibility, pain, cost, etc.). Procalcitonin, a reliable marker of bacterial infections, was demonstrated to be a good predictor of both renal parenchymal involvement in the acute phase and late renal scars. Furthermore, it was also found to be associated with high-grade VUR and was the key tool of a clinical decision rule to predict high-grade VUR in children with a first UTI. Therefore, procalcitonin may certainly be found playing a role in the complex and still debated picture of which examination should be performed after UTI in children.
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PMID:Procalcitonin: a key marker in children with urinary tract infection. 2127 26


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