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Biomedical subjects

B Boudailliez

Publications and source records attributed to B Boudailliez.

At least 73 records · Page 4Linked to original sources

Acute pancreatitis in six non-transplanted uraemic children. A co-operative study from the French Society of Paediatric Nephrology.

Ten clinical episodes of acute pancreatitis (AP) occurred in six patients (mean age 10 years, range 3-15 years) with chronic renal failure (CRF) during a 9-year period (1977-1986). The underlying cause of CRF was vesicoureteral reflux (2); urethral valves (1); ureterohydronephrosis (1); nephronopthisis (1) and a haemolytic uraemic syndrome which occurred 12 years before (1). In all patients a diagnosis of AP was established both on clinical grounds and with a serum amylase level of greater than 600 IU/l. In 3 patients laparotomy was performed because of suspected appendicitis. All patients required exclusive parenteral feeding (mean duration 25 days) and 2 patients had a partial pancreatectomy. No patient developed pancreatic pseudocysts, 2 patients experienced one relapse (3 and 21 months later) and 1 patient had two relapses and died. Mean duration of follow up was 3 years (range 1-10 years). Possible aetiological factors were: choledochal cyst (1); parotitis without a rise in mumps antibodies (1); familial dyslipidaemia but without AP in other family members (1), and aluminium intoxication with hypercalcaemia and convulsive encephalopathy treated with valproic acid in 1 patient. Severe hyperparathyroidism with radiological signs was absent in all patients. Transplantation had been performed either before AP in 2 patients (1 and 3 years before AP) or had followed AP in 1 patient (7 years after) without occurrence or relapse of AP.

Acute Disease↗

[The captopril test in nonselected hypertensive patients: absence of value for screening of renovascular patients and for unilateral small kidneys].

UNLABELLED: The decrease of blood pressure and the increase of plasma renin activity (PRA) after oral administration of captopril was evaluated in 104 consecutive hypertensive out-patients in whom the morphology of the renal arteries and parenchyma was assessed thanks to an intravenous digitalized angiography. Twenty five of these patients were excluded because of a natriuresis less than 50 mmol/24 h or non discontinuation of their treatment; 50 of these patients were classified as essential hypertension, 12 had renovascular disease (10 unilateral stenosis 2 of which significant; 2 bilateral significant stenosis); 9 had unilateral small kidneys (4 significant). The significance was ascertained on the PRA in the renal veins and/or the decrease of hypertension after surgery. The decrease of blood pressure after captopril was not different between the various groups. The increase after captopril of PRA was higher in unilateral significant lesions. However the highest post captopril PRA value was found in the essential hypertension group so that renovascular diseases could not be screened by higher post captopril PRA values. However taking into account that these latter were decreasing with age, a better discrimination of significant unilateral disease was possible in the hypertensive patients above 40 years of age. Furthermore once the diagnosis of unilateral kidney disease is established by radiological investigation, the captopril test allows to predict unilateral hypersecretion of renin with a sensitivity of 100% and a specificity of 78%. IN CONCLUSION: the captopril test does not allow to screen the non selected hypertensive patients for an efficient radiological investigation by intravenous digitalized angiography but may help to select the patients with unilateral renal disease for the renal venous PRA evaluation.

Adolescent↗

[Evaluation of maximal filtration capacity and renal functional reserve by an oral protein-loading test in adults and children].

After a protein load, the glomerular filtration increases to a maximum called maximal filtration capacity (MFC). The difference of this value with that observed in the fasting state defines the functional renal reserve (FRR). Fifty oral protein loads have been performed in 16 controls (13 adults and 3 children) and 31 patients with kidney disease or hypertension (22 adults, 9 children, 3 tests being performed twice). The load consisted in the adults of 80 g of various proteins. In the children the load consisted in the 100% of the protein intake recommended of the whole day. The urinary samples were made hourly during the 5 hours following the beginning of the meal. The MFC was not always observed during the 2 hours following the end of the meal. However the mean of the clearances measured during these 2 hours allowed an acceptable evaluation of the FRR in 42 out of 50 cases and had the advantage to reduce the errors linked to incomplete bladder voiding. In the adult controls the mean of hourly determined MFC is 156 +/- 27 ml/min, the mean of the clearances measured on the 2 hours following the meal is 136 +/- 21 ml/min and the fasting clearance 96 +/- 22 ml/min. The FRR is null in all patients with a nephropathy with a fasting clearance below 40 ml/min. It is modest or null in patients with acquired or congenital solitary kidney. Three out of five patients with proteinuria have MFC and FRR higher than the mean of the patients with comparable fasting clearances but without proteinuria suggesting that proteinuria is associated with a hyperfiltration state.

Administration, Oral↗

Comparative evaluation of bone aluminum content and bone histology in patients on chronic hemodialysis and hemofiltration.

In order to compare hemofiltration (HF) and hemodialysis (HD) in connection with the risk of aluminum overload and renal osteodystrophy, double bone biopsies after double tetracycline labeling and a desferrioxamine test were performed in 12 patients on HF and 15 patients on HD. The aluminum concentration was low (less than 0.6 mumol/l) both in the dialysate and the substitution fluid. The duration of treatment (about 2 years) and the cumulative doses of Al(OH)3 and CaCO3 were comparable in the two groups. None of the patients was taking 1 alpha-OH-D. The aluminum balance during an HF run ranged from -22 to +1.8 mumol/l, the balance being positive only when the plasma aluminum was less than 0.5 mumol/l. Basal plasma aluminum and its increase induced by desferrioxamine were comparable in the two groups. Bone aluminum content was also comparable, but was about 10 times higher than in 7 nonuremic controls. Bone aluminum content and plasma aluminum increase after desferrioxamine were correlated to the Al(OH)3 cumulative dose. None of the patients had florid osteomalacia with increased osteoid thickness, and only 1 in each group had traces of stainable aluminum. The mineralization front was decreased in 8 of 12 HF and in 9 of 14 HD patients, so that no difference was observed between the means of the two groups. The predominant histological bone picture of the patients was osteitis fibrosa which was present in 10 of 12 HF and in 13 of 15 HD patients. Mean osteoclast count and active resorption surface were comparable in the two groups, but was increased (5-10 times the mean of the controls).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[Treatment of hydronephrosis in newborn infants. Apropos of 18 cases diagnosed by antenatal ultrasonography].

Results are reported of therapy of 18 neonates in whom obstetrical ultrasound imaging had demonstrated uni- or bilateral pyelo-caliceal dilatation due to stenosis of pyelo-ureteral junction (18 children--2 hydronephroses). Neonatal biologic, ultrasound and urographic examinations confirmed presence and assessed importance of the hydronephrosis. As a function of findings therapy consisted of immediate operation (10 neonates), delayed surgery (5 cases) or surveillance (3 cases). Severe uni- or bilateral forms, classified stage III or IV were operated upon immediately, sometimes as emergencies. Less severe cases (stage III) were operated upon after 1 to 3 months, and the minor forms (stage II) were the object of simple surveillance. The operative technique used for treatment of the hydronephros was the Andersson-Hynes operation, anastomosis in 9 cases involving microsurgical procedures. Emphasis is placed on the serious nature of these neonatal forms, often bilateral or associated with contralateral uropathy. In utero shunts during the antenatal period are excluded, therapy being decided after birth. Based on their experience, the authors underline the reliability of early microsurgical pyeloplasty which appears to be the operation of choice in neonates at the present time.

Female↗

[Cavernous hemangioma of the bladder in children. Apropos of a case].

Benign tumours of the urinary bladder are very rare in children. A new case of cavernous haemangioma of the bladder is reported in a five year old boy presenting with macroscopic haematuria. This tumour is a hamartoma which usually presents with painless haematuria. In about 30% of cases, the bladder tumour is associated with angiomatous lesions in other parts of the body. The diagnosis may be suspected on intravenous urography or ultrasound examination, but it is generally made at cystoscopy. Biopsy and transurethral resection must be avoided because of the risks of haemorrhage. Partial cystectomy appears to be the most effective method of treatment.

Child↗

[Long-term follow-up of abused children (apropos of 65 cases)].

The authors work in an area located north of Paris, which contains roughly a population of .75 million, of which 82,000 are children less than 3 years of age. The center and main city of the area is the town of Amiens. The recruitment of their cases under study was essentially hospital based, which explains the relative severity of the trauma observed. The children were followed by various observation systems. Seventy-four children, of which more than two-thirds were less than 3 years of age were followed for a period varying from two to ten years. Forty-three percent of these children have been maintained in their biological families; the others have been placed. With this system only nine recurrences of abuse have been observed (12%), and only two instances of death have been observed. Other studies have demonstrated higher proportions. However, in two-thirds of the children observed, there were minor or major psychological or even psychiatric sequelae. Protection services have tried to stay away from institutional placement as much as possible; when necessary, the foster home system or adoption has been used. Striking is the lack of means available to help abusing families. Efforts should be made to assist the abusing families stay together.

Battered Child Syndrome↗

Varicella vaccine in children with chronic renal insufficiency.

This study reports the antibody response and clinical follow-up of uraemic children awaiting kidney transplantation after administration of the Oka-strain varicella vaccine (Varilirix). Seroconversion was observed in 20 out of 23 patients found to be seronegative when tested by the fluorescent antibody to membrane antigen technique, and an antibody booster response was observed in 41 out of 47 seropositive patients. Mild clinical varicella occurred in 5 vaccinated patients and herpes zoster in 3 initially seropositive ones. Nevertheless, a dramatic decrease in the incidence of both varicella and herpes zoster was observed in a series of 330 consecutive transplantations after the introduction of the varicella vaccine.

Adolescent↗

[Cerebral and ocular abnormalities with anterior pituitary insufficiency of familial nature].

Three families presenting one or several cases of brain or ophthalmic abnormalities and an hypopituitarism at least by one of the members have been observed. In the first family, the mother and one of her sons present bilateral choroidoretineal coloboma with amblyopia; one of these two suffers as well from panhypopituitarism. In the second family two premature twins, a brother and his sister, present a syndrome with hypophyseal dwarfism and ophthalmic abnormalities, consisting in the boy's case in an peripapillary depigmentation with no visible sight trouble whereas girl's is showing an extreme microphthalmia with major mental retardation. In the third family two 2nd degree cousins present a panhypopituitarism but only one of the two reveals through neuroradiological investigations corpus callosum and septum lucidum agenesia. The karyotype is normal in all the cases. An hereditary mechanism appears clearly in the first family. It is possible in the second, probable in the third one.

Abnormalities, Multiple↗

[Reactive arthritis in children].

Reactive arthritis is arthritis in which, although the nature of the responsible infection is known or suspected upon serological grounds, attempts at recovering the pathogen from the synovial fluid have failed. One of the main pathogenetic problems is the multiplicity of etiologic agents. Some are exogenous and may be related to the articular tropism of certain microorganisms, to immunologic depression due to an antecedent or coincident infection, and to successive reinfections by the same pathogen or by others which may promote an exacerbation of the disease. Others are endogenous and attention should be given to the local or systemic presence of an antigen as well as, in some instances, to the persistence of residual forms of infecting agents, which are more readily demonstrated with current bacteriological and serological methods. Although reactive arthritis is to be distinguished from septic arthritis, it can no longer be clearly differentiated from the classical post-infectious rheumatism. Once it has been produced, the antigenic stimulation is responsible for an immunologic response which tends to check systemic extension but may also produce tissue damage in the host. Some patients have circulating immune complexes which may bind to the joint, thereby damaging it. In other patients, particularly those who are HLA B27 positive, host-pathogen cross-reactions are demonstrated. Actually, the most frequent pathogenetic sequence seems to be a combination of two or more of these mechanisms, as there are reasons to believe that presence of the pathogen in situ is not required for the persistence of the inflammatory process. Reactive arthritis was first reported in adults following either sexually transmitted urethritis due to chlamydiae, mycoplasma or gonococci, or hepatitis B or an intestinal infection due to Yersinia, Campylobacter, Shigella, Klebsiella or Salmonella. Later, it was described in pediatric patients, particularly in Scandinavia where, for genetic reasons, the HLA B27 group is prevailing. Reactive arthritis seems less frequent in caucasian ethnic groups and above all in Latin Americans among whom HLA B27 carriers are more uncommon; however, it must be pointed out that they have not been as extensively studied and that other etiologic factors may still remain to be discovered. The course and etiology of the different forms of arthritis share certain characteristics which have been determined through a better knowledge of these conditions: onset occurs one or several weeks after a respiratory, urinary or, most often in children, digestive infection. This episode is unremarkable or latent and often overlooked.(ABSTRACT TRUNCATED AT 400 WORDS)

Antigen-Antibody Complex↗

[Prenatal ultrasonic diagnosis of malformation uropathies: 13 cases].

The systematic prenatal diagnosis of 5 cases of urinary tract malformations out of 2 500 pregnancies in 1982 in a special care maternity, or 2 per 1 000 is compared with the discovery of 10 cases in 28 months in 17 960 foetuses, or 0.55 per 1 000 whereas the overall frequency varies from 0.3 to 5.25 per 1 000 in infants. We studied 13 children whose prenatal abnormalities were 2 unilateral ureterohydronephrosis and 7 bilateral dilatations associated to 2 ureteroceles, 1 multicystic dysplasia and 1 ureteral duplicity. All of these except two were confirmed at birth. The exceptions consisted in one unilateral dilatation and one bilateral ureterohydronephrosis which turned out to be dysplastic kidneys unknown during pregnancy. Three ureteral duplicities were also ignored. The results are the following: Three cases were severe: one therapeutic abortion after 27 weeks, one intra-uterine decompression followed after few days by a preterm delivery and neonatal death and one provocated preterm delivery (36 weeks). All the others underwent decompression during the first hours of their life. Five cases can be considered unsuccessful: 2 abortions, the death of a 22 days old infant with an unilateral multicystic dysplasia and 2 bilateral malformations with renal failure, one of which was associated to an unilateral dysplastic kidney. Reliability of sonography as well as its interest in prognosis and prenatal evaluation of renal function are demonstrated.

Amniotic Fluid↗

[Regular dominance of thumb ankylosis with mental retardation transmitted over 3 generations].

A young girl 12 old, sent to us for obesity, and coxa-epiphysiolysis showed signs of mental retardation and bilateral thumb ankylosis. The fact that the mother was also affected by both of these signs, led to a more detailed genetic research. The latter revealed that not only the daughter, the mother, but also their own mother and may be, the sister, the grand-mother and the great-aunt of the patient had a retardation, a slight dysmorphia, a type A brachydactylia, signs of obesity and an identical ankylosis of both thumbs. This vertical inheritance, affecting apparently females only, but not associated with a high rate of miscarriage, has, it seems, never been reported. The characteristics of this family are being considered and discussed.

Adult↗