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Biomedical subjects

Y Aigrain

Publications and source records attributed to Y Aigrain.

At least 91 records · Page 5Linked to original sources

Development of human renal function: reference intervals for 10 biochemical markers in fetal urine.

Evaluation of fetal renal function by analysis of fetal urine sampled in utero may improve perinatal care after a prenatal diagnosis of bilateral obstructive uropathy. We provide reference intervals for 10 fetal urinary compounds and examine their variation with gestational age. Forty-one fetuses with bilateral obstructive uropathy (urine sampled between 20 and 38 weeks of gestational age) had normal, healthy values for serum creatinine (< or = 50 mumol/L) at ages 1-2 years. These cases were thus assumed to represent a reasonable approximation to healthy values. Sodium and beta 2-microglobulin concentrations significantly decreased with gestational age; calcium, ammonia, and creatinine significantly increased; glucose, phosphorus, chloride, urea, and total protein concentrations did not vary. Our results provide reference values for prenatal evaluation of fetal renal function and suggest that glomerular filtration of macromolecules and tubular reabsorption of glucose and phosphorus are mature by 20 weeks of gestation, whereas tubular reabsorption of sodium and beta 2-microglobulin increases progressively during the second half of gestation.

Biomarkers↗

Pancreaticoduodenectomy in childhood for rare pancreatic ductal anomalies--case reports.

In the past 9 years, 3 children suffering rare pancreatic disorders have undergone cephalic pancreaticoduodenectomy (Whipple procedure) required after the failure of initial conservative treatments. Ductal anomalies such as pancreas divisum or predominant Santorini duct can be managed conservatively (especially by papillotomy of the minor papilla), unless the extent of the fibrotic lesions of the pancreas jeopardizes the final evolution of the pancreatic disease. Intrapancreatic gastric duplications remain extremely rare. Even to-day, diagnosis was made only on pathologic specimens after radical surgery. Better knowledge of this rare pathology, with emphasis on its radiological features, may help better assessment. Hemoductal pancreatitis is evocative of the coexistence of gastric duplication and pancreatic aberrant duct. However, when fibrotic and necrotic changes in the duodenopancreatic region are considerable, assessment of this anomaly becomes difficult. Radical though it is, pancreaticoduodenectomy may be necessary to treat cephalic pancreatic lesions in childhood after the failure of reasonable attempts of conservative treatment.

Child, Preschool↗

Interleukin-6 and interleukin-1 beta production in a pediatric plasma cell granuloma of the lung.

Plasma cell granuloma (PCG) is a pseudotumor of unknown origin. It is frequently accompanied by acute-phase clinical and biological signs that resume after complete surgical removal, suggesting production of soluble mediators. We therefore investigated the role of cytokines in a previously healthy 10-year-old boy with a PCG of the lung and systemic symptoms. In this case, very high serum levels of interleukin-1 beta (IL-1 beta) and interleukin-6 (IL-6) were found before tumor excision, associated with inflammatory signs including major hyper-gamma-globulinemia. Pathologic analysis of the tumor showed an accumulation of fibroblasts and plasma cells producing immunoglobulins. Local production of IL-1 beta and IL-6 could be demonstrated at the messenger RNA (mRNA) level by the reverse transcriptase polymerase chain reaction and could be attributed to inflammatory cells by in situ hybridization and immunohistochemistry, whereas plasma cells exhibited membrane expression of the IL-6 receptor. Postsurgery follow-up showed rapid normalization of serum IL-1 beta and IL-6, whereas inflammatory protein levels decreased. This confirms the local production of cytokine within the PCG and raises the question of whether a dysregulation of cytokine production initiates the disease.

Base Sequence↗

Duodenal and esophageal manometry in total colonic aganglionosis.

Eleven children with total colonic aganglionosis were studied by esophageal and duodenal manometry. They were separated into two groups: group 1, with ileal involvement, and group 2, without ileal involvement. The results of the digestive motility studies were compared with the extent of ileal involvement of the aganglionosis and with the amount of artificial nutritional support required. All children showed abnormalities on both esophageal and duodenal manometry, suggesting complete digestive tract motility involvement. Recent advances in understanding Hirschsprung's disease and manometrial abnormalities suggest a primary motility disorder in children with total colonic aganglionosis.

Child, Preschool↗

Duodenal manometry in postobstructive enteropathy in infants with a transient enterostomy.

Intestinal motility was studied in 11 children with a transient enterostomy secondary to a neonatal organic small intestine obstruction (5 total colon Hirschsprung's disease, 2 necrotizing enterocolitis, 1 intussusception, 3 ileal atresia). Eight children presented with a postobstructive enteropathy (severe grade I [5], moderate grade II [3]) and three were considered as controls (grade III). They were assigned to one of the three groups on the basis of the duration of parenteral nutrition and constant rate enteral nutrition needed and the oral feeding tolerance. Barium small intestine transit showed no persistent partial obstruction or peritoneal adhesions. The abnormal inert marker transit times were statistically correlated with the clinical groups as well as duodenal manometric abnormalities. Manometric recordings were characterised by the absence (grade I) or abnormal phase III (grade II) of the migrating motor complex and decreased motility index (grades I and II). This study confirms that this enteropathy is due to a chronic alteration in motility induced by prenatal or postnatal obstructions.

Barium Sulfate↗

Postobstructive enteropathy in infants with transient enterostomy: its consequences on the upper small intestinal functions.

Repeated or prolonged organic obstruction of the small intestine in the neonatal period can lead to severe refeeding problems, despite a transient ostomy. These problems are thought to result from a postobstructive enteropathy (POE) of the apparently normal small intestine segment above the obstruction. Ten infants with a POE, characterized by limited oral caloric and carbohydrate intakes and increased ostomy effluent, were compared with 8 controls with an enterostomy and a normal postoperative refeeding pattern. There was no statistical difference in the histomorphometric appearance of the mucosa or its digestive or absorptive capacity (brush-border hydrolases, glucose transport) between the two groups. The effluent and duodenal floras of the two groups were similar. However, all POE patients showed significant abnormal peristalsis characterized by barium and carmin transit times. This suggests that repeated or prolonged obstruction in the neonatal period could lead to a POE, caused by chronic motricity abnormalities of the small intestine above the obstruction. Although this POE is more frequent after small bowel atresia, it may also occur with other conditions causing prenatal and postnatal intestinal obstruction.

Enterostomy↗

Tracheal rupture in a newborn during a complicated delivery. Diagnosis and surgical repair.

Rupture of the trachea is an exceptional obstetrical lesion. The infant reported in this paper, at 1 hour of age, developed respiratory distress with pneumomediastinum, bilateral pneumothorax and subcutaneous emphysema. This resulted from the fact that the trachea had ruptured, within 1 cm of the carina, during the difficult delivery. When the child was 23 days old, operation proved necessary because extubation was not feasible. The stenotic portion of the trachea was resected and continuity restored by end-to-end anastomosis. The tracheal lumen at the site of the anastomosis proved normal by bronchoscopic examination 4 months after the operation. There is only one similar case in the literature. The etiology of this rupture is discussed.

Birth Injuries↗

[Congenital diaphragmatic hernia. Value of preoperative stabilization].

Seventy-two patients with congenital diaphragmatic hernia (CDH) diagnosed in the first 12 hours of live have been reviewed retrospectively. Forty-eight patients born before 1985 (group I) were compared to 24 patients born between 1985 and 1989 (group II). The management was different for the 2 groups. Group I was operated on immediately. For group II, delayed surgery after stabilization was preferred. For this group, stability was sought before, during and after surgery. Therapy was first aimed at optimizing ventilation and oxygenation by way mechanical ventilation. Pharmacologic agents were used in an attempt to decrease pulmonary vascular resistance and improve cardiac output. The survival rate was 37.5% before 1985, 62.5% after 1985 (P less than 0.01). Main prognostic factors were Apgar score, paCO2, pH, ventilation index, alveolar-arterial difference in oxygen and oxygenation index. The stabilization before surgery improved the survival rate.

Carbon Dioxide↗

[Prognosis of prune belly syndrome].

The Prune Belly syndrome (PBS) is unfrequent. Fourteen cases have been followed in our unit during the last 20 years. Four infants (29%) died during the first months of life, because of neonatal sepsis (2 cases) or end-stage renal failure (2 cases). Among the other 10 cases, 6 (43%) had normal glomerular filtration rate at a mean age of 10 years 6 months (6 months to 15 years), 4 had chronic renal failure, including 3 cases who developed end-stage renal failure at 8, 8 years 8 months and 17 years respectively. Resection of an urethral obstruction was performed in 2 cases. This surgical indication remains widely accepted, while the current tendency is to limit ureteral surgery in PBS. Orchidopexy was performed in 4 children, 3 of them less than 6 years 6 months of age. Fertility of these early operated children remains to be established, as all adults reported in the literature remain sterile when orchidopexy was not performed or was performed after age 6.

Abnormalities, Multiple↗

[Currarino syndrome: an association not to be overlooked].

The Currarino triad consists of an anorectal malformation, a presacral tumor and a sacrum defect. A new case is reported in a female neonate. The diagnosis was suspected because of delayed emission of meconium associated with an occlusion syndrome. It was confirmed by bone imaging, ultrasonography and magnetic resonance imaging. A colostomy was performed on day 7 then closed on day 43 after several rectal dilation were carried out. A presacral lipoma was operated on at 10 months. The 56 cases reported in the literature are reviewed.

Abnormalities, Multiple↗

[Neonatal proteus mirabilis adrenal gland abscess].

The authors describe a case of unilateral adrenal abscess in a neonate, which was diagnosed at 13 days of age by abdominal ultrasonography. The newborn presented with persistent high white blood cells after Proteus mirabilis omphalitis. Magnetic resonance imaging and needle aspiration confirmed the diagnosis. This is the second case of Proteus mirabilis neonatal adrenal abscess reported in the literature. Percutaneous drainage allowed complete recovery.

Abscess↗

Urinary bladder diverticula and the Ehlers-Danlos syndrome in children.

Two large bladder diverticula that induced severe micturition disturbances were surgically removed in a 5-year-old boy with Ehlers-Danlos syndrome (EDS). One year after surgery, a new, asymptomatic diverticulum developed. This case is discussed in the light of nine similar cases that have already been published. It is concluded that EDS diverticula form a distinct group characterized by postoperative recurrence. They are not due to vesicourethral obstruction but probably result from an anomaly of the vesical wall. They are responsible for micturition difficulties and urinary tract infections. Their unavoidable recurrence means that surgery should be restricted only to symptomatic patients.

Child↗

[Upper jejunal atresia: treatment using jejunal resection, duodenal modeling and duodeno-jejunal or ileal anastomosis. Results in 13 newborn infants].

13 high jejunal atresias, including 6 type IIIb apple peel small bowel deformities, have been treated by a one stage surgical procedure, including proximal jejunal resection, tapering duodenoplasty and end-to-end duodeno-jejunal or duodeno-ileal anastomosis. All 13 infants survived. There were no anastomotic leak or malfunction. 7 jejunal atresias with normal distal small bowel were fed a mean of 9 days post-operatively (range 4 to 12 days). Intra-venous fluids were discontinued at a mean of 18 days post-operatively (range 13 to 24 days). Patients, all premature babies, were discharged on the mean 30th post-operative day (range 24 to 40). 5 jejunal atresias with apple peel deformity were fed with an average post-operative delay of 21 days (range 12 to 24). All intra-venous fluids were discontinued at a mean of 48 days post-operatively (range 35 to 90). Average hospital stay was 78 days (45 to 120). In one case, an ileal diaphragm, unnoticed during the first operation was subsequently resected at age 45 days and an iterative tapering duodenoplasty was performed at the age of 8 months. Recovery was then uncomplicated and the child was discharged on a normal oral feeding at the age of 10 months. Functional obstruction of the intestine at the site of anastomosis often occurs in high jejunal atresias. 70% of mortality resulted from operative complications in atresias with apple peel deformity. Intestinal distension is the cause of anastomotic malfunction. After jejunal resection, the duodenum remains dilated and anastomosis cannot function properly.(ABSTRACT TRUNCATED AT 250 WORDS)

Anastomosis, Surgical↗

[Neurocristopathy. The association of Hirschsprung's disease-ganglioneuroma with autonomic nervous system dysfunction in 2 children].

Two children with the coexistence of long segment Hirschsprung's disease, ganglioneuroblastoma with Ondine's curse in one case and autonomic nervous system impairment in the other are presented. All lesions may be related to aberrations of neural crest cell growth, their migration, or differentiation. A genetic etiology, and the link between Hirschsprung's disease and multiple endocrine adenomatosis is discussed. The combination of Hirschsprung's disease and a neurocristopathy strongly suggests that exploration of the autonomic nervous system and APUD system be carried out.

Autonomic Nervous System Diseases↗