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Biomedical subjects

F Gauthier

Publications and source records attributed to F Gauthier.

At least 181 records · Page 10Linked to original sources
PubMed1985

[Urinary lithiasis in children. Apropos of 92 cases].

The charts of 92 children with urinary calculi were reviewed. There were 69 boys (75%) and 23 girls (25%), 20 (22%) were infants. Calculi were in kidney (74 times but were often multifocal. Fourteen children (15%) presented with a lithogenic metabolic disorder, 28 (30%) had a malformation of the urinary tract, in 4 (4.3%) a permanent urinary derivation had been performed for the treatment of a malformation or a neurologic bladder. The other children presented with apparently idiopathic lithiasis. However, urinary tract infection was found in 70%. Predisposing factors, whether stasis, urinary tract infection or some urinary malformations, were often intricate. The spontaneous elimination of calculi was rare. Even when difficult, the extraction of calculi had generally moderate functional consequences. There was one secondary renal atrophy. Relapse occurred in 11 patients. Etiologic inquiry, bacteriologic monitoring of urines and ultrasonography supervision over years are the essential means to a better evaluation of the risk of relapse.

Child↗
PubMed1985

[Surgical pulmonary biopsy in children].

During the last 2 years an open lung biopsy was performed in 16 children aged from 2 to 14 years. Fourteen of these had a chemotherapeutic induced immunodeficiency and a radiological picture of diffuse pulmonary infiltrate. A definite diagnosis was established in 11 cases: 5 pneumocystis carinii pneumonitis, 2 CMV infections, 2 pneumocalcinosis, 1 neoplastic pulmonary lymphangitis and 1 staphylococcal infection. Three had a non-specific interstitial pneumonitis. In this series there was no post-operative death and the single complication was a wound infection. The tracheal tube could be removed within the hours following the intervention in 14 of the 16 children. The review of the recent literature suggests that the open lung biopsy is a safe and accurate way for the diagnosis of pulmonary infiltrates in pediatric immunocompromised patients. It is however a very invasive procedure and it is expectable that in the next year the bronchoalveolar lavage with a fiberoptic flexible bronchoscope will be systematically attempted prior to the open biopsy.

Adolescent↗
PubMed1984

An abnormal plasma antithrombin with no apparent affinity for heparin.

Congenital antithrombin abnormality was found in several members of a French family. No history of thrombotic episodes was associated with this abnormality. Plasma antithrombin concentration as well as the rate of thrombin inactivation by defibrinated plasma in the absence of heparin were normal. However, the heparin cofactor activity was decreased by about 50% in plasma of affected patients. Accordingly, about half the amount of plasma antithrombin did not bind to gel bound heparin. Moreover the crossed immunoelectrophoretic pattern in the presence of heparin demonstrated two peaks of antithrombin, the slower one migrating as normal antithrombin when heparin was omitted from the first agarose gel. It was concluded that molecular alteration of the antithrombin molecule seemed to affect only the heparin binding site thus preventing from any rate enhancement of thrombin inactivation.

Antithrombin III Deficiency↗
PubMed1984

Inhibition of human liver cathepsin L by alpha 2 cysteine-proteinase inhibitor and the low-Mr cysteine proteinase inhibitor from human serum.

The inhibition of human liver cathepsin L by two specific proteinase inhibitors present in human serum, namely alpha 2 cysteine-proteinase inhibitor and the low-Mr cysteine-proteinase inhibitor, was studied. Kinetic parameters, including inhibition constants (Ki) and rate constants for association and dissociation (k+1 and K-1), were determined. The values found are consistent with a possible physiological function of these inhibitors to control cathepsin L activity. Furthermore, a transfer of active proteinase from the complex with either cysteine-proteinase inhibitor species to alpha 2-macroglobulin was demonstrated in vitro. Given the rate of dissociation of both cathepsin-L-cysteine-proteinase inhibitor complexes, a function of transitory inhibitor can therefore be hypothesized for these proteins and might then provide an explanation of the clearance of lysosomal proteinases.

Binding Sites↗
PubMed1984

[Fetus in fetu: report of 2 cases and analysis of the literature].

Foetus in foetu is a very unusual cause of abdominal mass in the infancy. Twenty cases only have been quoted in the literature. Two others cases are added, concerning two girls: a three months infant, second born from a genuine twin pregnancy, and a four weeks newborn whose the mass was discovered before the birth, on routine ultrasonography. In together, the diagnosis was made in operating room, then confirmed by pathologic studies. Literature data are recorded, and the difference between teratomas and foetus in foetu is point. The pathogeny remains obscure. It could result from the inclusion of a parasite twin in his bearer, become during embryologic stage of the delimitation.

Diagnosis, Differential↗
PubMed1984

[Urethrorrhagia in boys].

Fourteen boys, aged 2 to 13, have been examined in the last six years for blood spotting from urethra between voidings, rarely associated with some degree of dysuria. No anomaly was seen on IVP, except in two cases which showed mild stenosis of the posterior urethra. Endoscopic evaluation was completely negative in 5 cases; moderate inflammation or hypervascularisation, some what a subjective aspect, was noticed in 4 cases; a definite "posterior urethritis" already described by others in the literature was demonstrated in 5 cases. The etiology of such a lesion remains unknown; no story of external or internal trauma was reported. Although these urethrorrhagia are of benign nature, we do not feel like others (1) that endoscopy should be considered as unnecessary, but on the contrary that it should help for correct evaluation and follow up of these patients, rather prone to be lost especially when no treatment has been prescribed.

Adolescent↗
PubMed1984

[Choleperitonitis after hepatoportocholecystostomy for atresia of the biliary tract].

The gallbladder, the cystic and the choledocal ducts are patent in one fifth of biliary atresia cases, and can be used for the corrective operation. The main interest of this hepatoportocholecystostomy (HPC) is to prevent the cholangitis episodes which are the most severe complication of successful hepatoportoenterostomies (HPE). However a singular complication of HPC has been noticed by previous authors: the choleperitonitis. We report a recent case in which this complication was diagnosed by a routine ultrasonography at the 3rd week of the uneventful postoperative course of a HPC performed in an 1 1/2 month old infant. An early reoperation (HPE) had a good result with restoration of a satisfactory bile flow. The child is anicteric with a follow-up of one year. In our experience with 208 corrective operations between 1969 and 1981, HPC was used in 38 cases, with restoration of bile flow in 17 cases. A choleperitonitis was noticed in 4 cases in this series, and in a 5th additional recent case. Three children were not suitable for reoperation because of very poor condition and died within 3 to 17 months after HPC. Two children were reoperated upon early with a good result. We advocate the HPC procedure despite the risk of choleperitonitis and emphasize the interest of early postoperative routine ultrasonography, especially when stools remain acholic at the 4th postoperative week.

Bile↗
PubMed1983

Rat alpha 1-cysteine proteinase inhibitor. An acute phase reactant identical with alpha 1 acute phase globulin.

alpha 1-Cysteine proteinase inhibitor was isolated from normal and acute phase rat serum. The procedure, which includes successive fractionations on AcA 34, Cibacron blue Sepharose, DEAE-Sephacel, and hydroxyapatite, but avoids the use of an affinity chromatography step on a cysteine proteinase gel, led to the preparation of two electrophoretically distinct components. These resolved to a single band of apparent Mr = 68,000 when analyzed by sodium dodecyl sulfate-polyacrylamide gel electrophoresis. A slightly higher Mr was estimated from gel filtration studies, possibly related to the rather high carbohydrate content (15.25% of the dry weight). The purified protein exhibited strong inhibiting capacity towards papain with a Ki of 5 X 10(-11) M. As shown by immunochemical quantitation as well as functional activity, a 6-10-fold increase in the alpha 1-cysteine cysteine proteinase inhibitor content was recorded in inflammatory serum, thus demonstrating the protein to be a typical acute phase reactant. Its partial physicochemical characterization (Mr, isoelectric point, extinction coefficient, amino acid and carbohydrate compositions) shows large similarities with alpha 1 acute phase globulin whose biological function remains unknown, and which is present at the same concentration (about 0.5 g/liter) in normal serum. Identity between the two molecules has been further demonstrated by double immunodiffusion analysis since a continuous precipitating line was observed when purified alpha 1-cysteine proteinase inhibitor was reacted against anti-alpha 1 acute phase globulin and anti-alpha 1-cysteine cysteine proteinase inhibitor antibodies.

Amino Acids↗
PubMed1983

Comparison of immunochemical and biological properties of human anti thrombin during blood clotting and upon interaction with exogenous thrombin.

Modification of immunological and biological properties of human antithrombin were studied in plasma-serum pairs and in defibrinated plasma supplemented with human thrombin. Modified antithrombin obtained through whole-blood clotting or upon addition of exogenous thrombin appeared the same with regards to its electrophoretic or biological properties. However, amounts of thrombin higher than that physiologically available, had to be used to obtain a "serum-like" antithrombin in thrombin supplemented plasma suggesting different pathways for this transformation. This was in agreement with the observation in plasma of a modification of antithrombin antigenic properties upon thrombin addition whereas no difference was demonstrated when comparing serum to normal plasma. It may be concluded that the inactivation of antithrombin and the appearance of electrophoretically modified forms in normal serum is not mainly due to the formation of enzyme-inhibitor complexes and therefore that proteolytically modified, enzyme-free forms of antithrombin demonstrated in purified systems (Fish et al. 1979) could be of physiological relevance.

Antithrombins↗
PubMed1983

Protease inhibitor localization in control and streptozotocin-diabetic skeletal muscles.

alpha 1-Antitrypsin and alpha 1-inhibitor-3 were localized for the first time inside skeletal muscle cells. Their content, especially that of alpha 1-inhibitor-3, was greatly reduced following streptozotocin-induced diabetes. alpha 1-Antitrypsin and alpha 1-inhibitor-3 were also observed in the vascular components and interstitial space surrounding both control and diabetic soleus muscles as revealed by immunofluorescence. In diabetic muscles, the non-myofibre locale of alpha 1-inhibitor-3 was reduced, and to a lesser extent, alpha 1-antitrypsin. Both myofibre and extracellular patterns were reversed to control levels by insulin replacement.

Acute-Phase Proteins↗
PubMed1983

Immunofluorescent localization of four serum proteinase inhibitors in normal rat liver.

Four serum proteinase inhibitors, alpha 1-macroglobulin, alpha 1-proteinase inhibitor, alpha 1-inhibitor3 and alpha 2-acute phase macroglobulin, were localized in rat liver by immunofluorescent techniques. alpha 1-Macroglobulin was observed predominantly in the sinusoids and alpha 1-inhibitor3 in hepatocytes. In contrast, alpha 1-proteinase inhibitor was localized in two sites, sinusoids and parenchymal cells. The fourth inhibitor tested, alpha 2-acute phase macroglobulin, was barely detectable in normal liver. However, some appeared to be present in the extrahepatocyte space.

Acute-Phase Proteins↗
PubMed1983

[7 cases of calyceal diverticula in children].

Seven cases of calyceal diverticula were seen from 1967 to 1981 in children aged 2 to 16 years at the moment of diagnosis. Symptoms were: gross hematuria (2 cases), urinary infection (2 cases), recurrent abdominal pain (2 cases) or enuresia. All children had a single diverticulum. Five of the 7 diverticula were located at upper renal pole. One of the children presenting with hematuria and renal colic had an oxalic calculous within diverticulum. Three small diverticula did not require treatment and remained uncomplicated with a follow-up of 14,18 and 60 months. Four complicated diverticula (1 from oxalolithiasis, 1 from hematuria and 2 from urinary infection) required surgical removal, by partial nephrectomy (1 case) or deroofing operation with intradiverticular ligation of the communication channel (3 cases). Results were good in 3 children. Removal of adjacent parenchyma with a residual cavity was necessary 5 years later in the fourth child. The majority of children calyceal diverticula seem to be from congenital origin, but some authors suggest that the y could result from vesico-tubular reflux. The possibility of late complications from small and asymptomatic diverticula has been emphasized by many authors. The deroofing operation is certainly the elective procedure when a large or complicated diverticulum requires surgical treatment.

Adolescent↗
PubMed1982

Biosynthesis and secretion of alpha 1-antitrypsin in primary cultures of rat hepatocytes. Characterization of differently glycosylated intracellular and extracellular forms.

The biosynthesis and secretion of alpha 1-antitrypsin was studied in rat hepatocyte primary cultures. After labeling with [35S]methionine an alpha 1-antitrypsin with an apparent molecular weight of 49000 estimated by sodium dodecyl sulfate/polyacrylamide slab gel electrophoresis was immunoprecipitated from the cell homogenate. This intracellular form of alpha 1-antitrypsin could be deglycosylated by endoglycosidase H treatment indicating that its oligosaccharide chains were of the high-mannose type. Pulse-chase experiments showed that about 30 min after its synthesis the transformation of the 49000-Mr alpha 1-antitrypsin to a protein with an apparent molecular weight of 54000 began. Only this 54000-Mr protein was secreted by the hepatocytes. The 54000-Mr alpha 1-antitrypsin was not sensitive to endoglycosidase H, but sensitive to neuraminidase, and it incorporated [3H]galactose and [3H]fucose indicating that its oligosaccharide chains were of the complex type. In the presence of tunicamycin, which blocks the formation of N-asparagine-linked oligosaccharide chains, an unglycosylated alpha 1-antitrypsin with an apparent molecular weight of 41000 was found in the cells as well as in the medium. However, tunicamycin decreased the secretion of alpha 1-antitrypsin by 60-70%, whereas the secretion of albumin remained unaffected. In the presence of colchicine the secretion of both alpha 1-antitrypsin and albumin was impaired. The results demonstrate the importance of glycosylation for the secretion of alpha 1-antitrypsin.

Animals↗