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Biomedical subjects

François Cachat

Publications and source records attributed to François Cachat.

12 recordsLinked to original sources

Arterial hypertension after surgical closure of omphalocele and gastroschisis.

Arterial hypertension has been reported as a complication of surgical closure of an abdominal wall defect. No report studying the incidence, the characteristics and the clinical significance of hypertension after surgical correction of an omphalocele or gastroschisis has been published so far. The medical records of all newborns with surgically corrected gastroschisis or omphalocele identified in two centers were retrospectively evaluated. Arterial hypertension was defined as a mean daily systolic and/or diastolic blood pressure value higher than the 95 percentile for age and/or weight, according to literature data. The timing of surgery, weight gain, plasma creatinine and the use of diuretics or vasoactive drugs were compared between the groups with and without hypertension. Seventy-two patients were identified and included in the study, 29 with omphalocele and 43 with gastroschisis. Those with omphalocele were born at a mean age of 37.3+/-2.6 weeks with a mean birth weight of 2,971+/-715 g, and those with gastroschisis were born at 36.1+/-2.0 weeks with a mean birth weight of 2,527+/-498 g. Blood pressure values of 66 patients were available for analysis. Of the omphalocele patients, 46.2% (12/26) developed systolic hypertension, compared to 17.5% (7/40) of the patients with gastroschisis (P =0.024). Hypertension was always transient, lasting an average of 4 and 1 day in the omphalocele and gastroschisis groups, respectively. Two patients with omphalocele were given anti-hypertensive therapy. There was no difference between patients with or without hypertension regarding weight gain, use of vasoactive drugs or diuretics, mean weekly creatinine values or the timing of surgery. Newborns with an abdominal wall defect frequently present with transient arterial hypertension. Hypertension occurs significantly more often, is more severe and lasts longer in patients with omphalocele than in patients with gastroschisis. In both groups, hypertension is transient and rarely requires therapy. The cause of hypertension remains unclear.

Female↗

Severe hypertension and massive proteinuria in a newborn with renal artery stenosis.

Renal vein thrombosis and the congenital nephrotic syndrome have been associated with nephrotic-range proteinuria/nephrotic syndrome and hypertension in the newborn period. We describe a newborn with severe hypertension and proteinuria secondary to unilateral renal artery stenosis. Proteinuria completely disappeared with blood pressure control (with sodium nitroprusside and an angiotensin-converting enzyme inhibitor). Although renin was not measured, we speculate that proteinuria might have been induced by a high renin state, and was controlled by the angiotensin-converting enzyme inhibitor.

Angiotensin-Converting Enzyme Inhibitors↗

[Urinary calculi epidemiology in children].

We identified 40 pediatric patients with urolithiasis. There were 27 boys and 13 girls. Initial symptoms were abdominal pain, with or without microscopic hematuria in 40% of the cases, and urinary tract infection/pyelonephritis in 25% of the cases. Stones were made of struvite (35% of the cases), calcium-phosphate (25%) or calcium-oxalate (20%). The high prevalence of struvite stones reflects the importance of urinary tract infection a major cause of urolithiasis in that specific age group. Hypercalciuria was the most common urinary biochemical abnormality, found in more than 50% of the children. In the absence of a spontaneous passage of the stone, extra-corporeal shock wave lithotripsy represents an excellent therapeutic option. This article emphasizes the importance of stone analysis and extensive biochemical investigations in children with urolithiasis, in order to avoid recurrence and potential progression towards chronic renal failure.

Child↗

[Medical treatment of urinary lithiasis].

Urinary stone disease is frequent, and characterized by a high recurrence rate. Prevention of recurrent urolithiasis is possible using an appropriate diet with or without medications. Patients should be encouraged to have a high fluid intake. For an adult, urine volume should exceed 2000 ml/day. Diet modification should be done according to the various metabolic factors contributing to the formation of the stone (ie, hypercalciuria, hyperoxaluria, hypocitraturia, hyperuricuria, and so forth). Calcium intake should be around 1000 mg/day, protein intake limited to 1.2 g/kg/day, and salt intake kept to less than 100-150 mEq/jour. For uric acid urolithiasis, patient should limit uric acid intake to less than 500 mg/day. If these dietary manoeuvers fail, one can use thiazide diuretics to treat hypercalciuria, potassium citrate to correct hypocitraturia or sodium bicarbonate to alkalanize urine and prevent uric acid stone formation.

Decision Trees↗

[Infective lithiasis].

We report the case of a 69-year-old woman, with a BMI of 42.9, suffering from bilateral struvite calculi and who raised end stage renal failure. Urease-synthesizing bacteria, leading to the hydrolysis of urea into ammonium and to an alkaline urine (pH > 7.2), are required for struvite stone formation in humans. Struvite component constitutes the majority of staghom calculi. Patients with struvite stones could lose renal function because of obstructive or pyelonephritic episodes and surgical interventions on the kidney. Therapeutic success needs a follow up by a specialized uro-nephrologist team as soon as possible.

Aged↗

[Cysteine lithiasis].

Cystinuria is a common inherited amino-aciduria resulting in abnormal urinary excretion of cystine and the dibasic aminoacids, lysine, arginine and ornithine. Formation of cystine kidney stones, recurrent infections and subsequent renal failure are the main complications of the disease. Recently, the gene SLC3A1 and SLC7A9, encoding the two subunits rBAT et b0,+AT of the proximal renal transporter complex, have been identified. In this article, we report the medical history of a 30-year-old patient and discuss the recent molecular progress, the clinical evolution, and the medical treatment of the cystinuria.

Adult↗

[Massive hyperoxaluria].

Primary hyperoxaluria type I is a rare inborn error of metabolism caused by a deficiency of a liver-specific peroxisomal enzyme. It manifests by increased oxalate production that ultimately results in kidney failure, due to urolithiasis and nephrocalcinosis, and finally induces systemic oxalosis and risk of premature death. Primary hyperoxaluria type 2 is mainly responsible of urolithiasis. Enteric hyperoxaluria is a commonly seen adverse event of Crohn disease or after extensive intestinal resection. These affections represent the main etiologies of massive hyperoxaluria. If not recognized very soon and adequately treated, these conditions can progress rapidly to end stage renal failure.

Child↗

[Radiologic and biochemical studies of urinary lithiasis].

Clinical investigations of a patient with urolithiasis include a careful history and radiological and biochemical evaluation. Stone analysis by infrared spectrophotometry remains the most important step. These investigations are essential in order to understand why a patient developed urolithiasis and, most importantly, how to avoid its recurrence in the future. Simple exams are often enough in a patient with a single urolithiasis episode. But biochemical evaluation should be extensive in a child, or an adult with several urolithiasis episodes or with renal insufficiency.

Decision Trees↗

Lymphomatoid granulomatosis in a renal transplant patient.

Lymphomatoid granulomatosis is a rare angiocentric and angiodestructive pulmonary angiitis considered as a variant of the lymphoproliferative disorder group. Patients with organ transplantation are at an increased risk for post-transplant lymphoproliferative disorders secondary to their immunosuppression. However, lymphomatoid granulomatosis has rarely been described in patients with renal transplantation. It often presents with severe pulmonary signs. We describe a case whose initial presentation was an isolated VIth nerve palsy. We review the radiological and pathological findings and discuss the etiopathogenesis and therapeutic options of this particular lymphoproliferative disorder. With careful and stepwise reduction in her immunosuppression, our patient showed a complete disappearance of her lymphomatoid granulomatosis, and she is clinically well more than 3 years after the diagnosis, with good kidney function.

Adolescent↗

Ureteral obstruction in neonatal mice elicits segment-specific tubular cell responses leading to nephron loss.

BACKGROUND: To elucidate the sequence of renal responses leading to nephron loss in obstructive nephropathy, we examined the evolution of segmental nephron cellular changes consequent to chronic unilateral ureteral obstruction (UUO) in the neonatal mouse. METHODS: Neonatal mice were subjected to UUO or sham-operation, and kidneys were harvested 5, 12 or 19 days after surgery. Proximal tubules (PT), distal tubules (DT) and collecting ducts (CD) were identified with lectins. Histomorphometric quantitation was made for cellular necrosis, apoptosis, proliferation, tubular dilatation, tubular basement membrane (TBM) thickening, interstitial collagen, and glomerular maturation. The distribution of hypoxic tissue was determined using pimonidazole as a marker. Additional studies were performed by mechanically stretching monolayer cultures of mouse proximal tubular and collecting duct cells, and measuring apoptosis. RESULTS: Neonatal UUO induced an arrest of glomerular maturation throughout the period of study. Chronic UUO induced hypoxia, tubular necrosis, proliferation, and TBM thickening in the PT, but stimulated apoptosis in the DT and CD. Tubular dilation in the obstructed kidney was most severe in CD and least severe in PT. Tubular cell apoptosis closely paralleled tubular dilation (P < 0.05), and fibrosis surrounding individual tubules also correlated with tubular dilation (P < 0.001). Mechanical stretching of cultured mouse tubular cells induced apoptosis directly proportional to the magnitude of axial strain: apoptosis was consistently greater in CD than in PT cells (P < 0.05). CONCLUSIONS: Following UUO, the co-localization of hypoxia with cellular proliferation, necrosis, and TBM thickening of the PT is consistent with ischemic injury resulting from vasoconstriction. In contrast, a selective dilation of the distal portion of the nephron (DT and CD), which results from the greater tubular compliance there, leads to stretch-induced epithelial cell apoptosis, along with a progressive peritubular fibrosis. Nephron loss in the obstructed developing kidney likely results from complex, segment-specific cellular responses.

Animals↗

Recovery from release of ureteral obstruction in the rat: relationship to nephrogenesis.

BACKGROUND: Obstructive nephropathy is a major cause of renal insufficiency in infants and children. Despite release of unilateral ureteral obstruction (UUO) in the first five days of life in the rat, renal growth is impaired, while glomerular filtration rate (GFR) is preserved at one month, but decreases markedly by one year. To test the hypothesis that renal recovery from UUO depends on the stage of nephrogenesis at the time of relief of obstruction, renal recovery from relief of five days UUO following completion of nephrogenesis (days 14 to 19) was compared with UUO during nephrogenesis (days 1 to 5). METHODS: Rats underwent UUO or sham operation at one day of age, with relief five days later. In additional groups of neonatal rats, the operation was at 14 days, with relief at 19 days. Three months later, blood pressure, GFR, urine flow, sodium and potassium excretion, and kidney weight were measured. In addition, the number of glomeruli, glomerular maturation, glomerular diameter, tubular atrophy, and interstitial fibrosis were determined in each kidney. The effects of five-day UUO on number of glomeruli was determined also in adult rats one month following relief of obstruction. RESULTS: Three months following relief of UUO during days 14 to 19, renal growth was decreased by 50%, compared to a 30% reduction following relief of UUO during days 1 to 5 (P < 0.05). The number of glomeruli was reduced by approximately 50% regardless of the timing of UUO, but glomerular size was reduced only in rats with UUO from days 14 to 19. Blood pressure and tubular atrophy were increased, and GFR, urine flow, sodium and potassium excretion were decreased in the postobstructed kidney of both neonatal groups. In the adult rat, the five-day UUO did not result in a decrease in the number of glomeruli. CONCLUSIONS: In the period immediately following nephrogenesis, the kidney is particularly susceptible to long-term injury from temporary UUO. This suggests that a delay in relief of significant ureteral obstruction should be avoided if diagnosed in the perinatal or neonatal period.

Aging↗