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Biomedical subjects

R B Galifer

Publications and source records attributed to R B Galifer.

At least 19 recordsLinked to original sources

Quantitation of androgen receptor messenger RNA from genital skin fibroblasts by reverse transcription--competitive polymerase chain reaction.

To gain further information concerning the regulation by androgen of AR mRNA expression in cultured genital skin fibroblasts (GSF), we first developed a quantitative reverse transcription-competitive polymerase chain reaction (RT-PCR). This method used an ethidium bromide stain analysis of the PCR products for the accurate quantitation of low levels of human androgen receptor (hAR) mRNA in GSF. To control for variations due to sample preparation, and to minimize the disparity of the reverse transcriptase efficiency between samples after the RT procedure, we produced an initial PCR for the glyceraldehyde-3-phosphate dehydrogenase (GAPDH) gene, and then adjusted the amount of cDNA to that of this housekeeping gene. Competitive PCR for hAR was then immediately performed on normalized cDNA with a competitor DNA that exhibited a 13 bp deletion as compared to the 163 bp for the target fragment, and the PCR products were easily separated by 3.5% agarose gel electrophoresis. This quantitation procedure involved no additional steps, such as enzymatic cleavage of the PCR products, nor the use of radioactivity. In GSF from individuals, we found that the normal amount of AR mRNA was 5.6 attomoles/microg RNA, (+/-1.0, s.e.m.) with an intra- and an inter-assay of 8.4 and 14.7%, respectively. We observed a biphasic pattern of AR mRNA expression in normal human GSF in the presence of physiological concentration of androgen. Quantitative RT-PCR of AR mRNA may be useful for studying AR mRNA expression in experimental or clinical conditions.

Dihydrotestosterone

Prognostic factors of the postoperative vomiting in case of hypertrophic pyloric stenosis.

From January 1986 to February 1994, 198 children were operated on for hypertrophic pyloric stenosis (HPS). Postoperative follow-up have been carried out in 194 cases. The children were divided into two groups: group A (n = 134; 69.1%): without any postoperative diet troubles (n = 52) or simple regurgitations (n = 82), and group B (n = 60; 30.9%) presenting more significant vomiting requiring medical treatment (n = 52) or a prolongation of parental nutrition (n = 8). A retrospective study of the different factors which can possibly explain this postoperative vomiting was carried out. The criteria having an influence are: the age (44.5 days in group A; 35.7 days in group B; (p < 0.001) the weight at the time of the operation (3921) g in group A; 3647 in group B; p = 0.01) the thickness of the pylorus at the pre-operative ultrasound scan (5.2 mm in group A; 47 in group B; p < 0.015). The other studied criteria (prematurity, birth weight, delay in diagnosis, weight loss, hydroelectrolytic abnormalities, surgical approach way-subcostal or umbilical-, surgical difficulties and operation duration) are not statistically significant. The young age (and therefore the low weight) at the time of the pyloromyotomy can easily explain the post-operative vomiting through the physiological immaturity of the lower sphincter of the esophagus. It is more paradoxical to note that these difficulties are all the more frequent because the pyloric tumor is less thick at the ultrasound scan. But this criterion is also directly related to the child's age (average thickness of 4.5 mm before the age of one month and 5.8 mm after the age of two months; p < 0.0001). These data suggest the importance of systematic medical treatment to prevent postoperative vomiting in high-risk children, in order to decrease hospital stay (4.14 days in group A; 5.20 days in group B; p < 0.0001).

Female

PCR analysis and sequencing of the SRY sex determining gene in four patients with bilateral congenital anorchia.

OBJECTIVE: We explored the possibility of a genetic anomaly in the sex determining region of the Y chromosome, SRY gene, known to be equated to the testis determining region. PATIENTS: Four patients with bilateral congenital anorchia, absence of testicular tissue, elevated FSH and a lack of testosterone response to human chorionic gonadotrophin stimulation tests were studied. MEASUREMENTS: Amplification by polymerase chain reaction of the SRY gene and direct double stranded DNA sequencing were performed with the same primers. RESULTS: The expected 648 basepairs band of SRY was detected in the four DNA samples from patients with bilateral congenital anorchia. Direct sequencing did not show any difference with the previous published sequence. CONCLUSIONS: These data suggest that, in the four patients, bilateral congenital anorchia is not related to an anomaly of the opening reading frame sequence of the SRY gene.

Child, Preschool

[Antibiotic therapy of perforated appendicitis in children: comparison between the amoxicillin-clavulanic acid and the benzylpenicillin-netilmicin-metronidazole combinations].

In a multicentre trial we compared the clinical efficacy of amoxicillin/clavulanate used as a single-agent therapy with that of the three-agent combination usually prescribed in the post-operative period for appendicular peritonitis in children. Only bacteriologically documented peritoneal infections were included. Sixty-four patients were randomly distributed between two groups: Group A (29 cases) treated with amoxicillin/clavulanate, first administered iv (100 mg/kg/d), followed by conversion to the oral route (50 mg/kg/d) once the patient had been afebrile for 48 hours; Group B (35 cases) first treated by the iv route with benzylpenicillin (100,000 IU/kg/d) plus netilmicin (5 mg/kg/d) plus metronidazole (30 mg/kg/d), followed by conversion to the oral route for metronidazole (30 mg/kg/d). In both groups, the total duration of parenteral and oral treatment was not less than 5 days. One hundred and seventy nine bacterial strains were recovered from peritoneal fluid samples obtained during surgery; 86% of these were sensitive to amoxicillin/clavulanate. Clinical efficacy, assessed on the basis of time until return to normal temperature and gut transit and duration of hospitalization, was identical in both groups, with follow-up monitoring on day 30 showing recovery in all cases. Cure was obtained without any problems of infection in 25/29 patients in group A and in 34/35 patients in group B (non significant difference). Tolerance was excellent and identical in the two groups with the exception of three cases of thrombophlebitis which occurred in group B. The results of this study suggest that amoxicillin/clavulanate may be useful as single-agent therapy as a first-line curative treatment for appendicular peritonitis in children.

Adolescent

Evaluation of abdominal pain in Henoch-Schönlein syndrome by high frequency ultrasound.

The authors report the use of high frequency ultrasound (7.5-10 MHz probes) for the detection of intestinal complications in 14 children with Henoch-Schönlein purpura. Ultrasound gave information of 3 levels: (1) It explained the acute abdominal pain by showing in all cases the oedematous haemorrhagic infiltration of the intestinal wall, which appeared thickened (3-11 mm): lesions were diffuse (6 cases) or focal (duodenal in 5 cases, jejunal in 2 cases and ileal in one). (2) It followed the evolution of the disease: extension of lesions (5 cases) or resolution (progressive decrease of parietal thickening, reexpansion of small bowel lumen, reappearance of peristalsis). (3) It always detected surgical complication: ileo-ileal intussusception (3 cases), perforation (1 case).

Abdominal Pain

[Utero-vaginal malformations].

This review deals with the various anatomo-clinical features and the embryogenesis of utero-vaginal malformations. Three anatomo-clinical groups are distinguished from the vaginal malformations: vaginal aplasia, vaginal septa and hydrometrocolpos. Similarly, there are 3 types of uterine malformations, each corresponding to a chronologically different embryopathological anomaly: mullerian aplasia, most often responsible for utero-vaginal aplasia, mullerian fusion defects leading to bicornuate uterus, and resorption defects of the mullerian septum resulting in uterine septum. Only the utero-vaginal malformations with a vaginal component, mainly neonatal hydrometrocolpos, can really be detected during infancy and childhood, provided that the examination of the external genitalia is systematically and carefully performed. Due to their usual clinical latency during childhood, most malformations are only discovered by the end of puberty or after menarche, the main clinical signs being primary amenorrhea, cryptomenorrhea, unilateral dysmenorrhea or isolated dyspareunia. After the clinical gynecological examination, ultrasound diagnosis is very useful to explore the internal genitalia and to search for a frequently associated anomaly of the urinary tract.

Classification

[Abdominal contusions in children. Diagnosis and treatment of renal lesions. Apropos of 26 cases].

During the past ten years, we have treated 26 children with closed renal injuries. Hematuria appeared in 19 cases, but was not detected in 7 other cases, 6 of which corresponded to severe, especially pedicular, lesions. The diagnostic means for the lesion included IVP in 24 cases, ultrasonography in 23, angiography in 8 and computed tomography in 7. The treatment was medical for 18 children and surgical for 8. Observation revealed 4 cases of renal atrophy, including 1 of general atrophy without residual arterial hypertension. On the basis of this series and of a review of the literature, we study the evolution of the diagnosis and treatment of this condition with the development of new medical imaging techniques, especially of CT.

Adolescent

[Neuroblastoma, factor of early decompensation of tetralogy of Fallot].

The authors report the case of a tetralogy of Fallot associated with a neuroblastoma secreting large quantities of noradrenaline. Anoxic decompensation of the tetralogy of Fallot occurred early at the age of 4 months with cyanotic crises associated with an impression of abdominal pain. A systemic-pulmonary anastomosis was performed and the cyanosis regressed but the crises persisted and investigations showed the presence of a neuroblastoma. The tumour was treated by surgery and chemotherapy and has not recurred after 2 years' follow-up. Complete repair of the tetralogy of Fallot was performed secondarily at the age of 2. In this case, the high concentration of circulating catecholamines was probably a factor of early decompensation of the tetralogy of Fallot by infundibular spasm. A review of the literature revealed 26 cases of cardiac disease with a peripheral neurogenic tumour. Two pathogenic mechanisms are suggested: an embryological abnormality of cardiac cell migration from the neural crest and chronic stimulation of neuroblastic cells by chronic hypoxia.

Humans

[Abdominal injuries in children. Diagnosis and treatment of renal lesions. Apropos of 26 cases].

During the past ten years, we have treated 26 children with closed renal injuries. Hematuria appeared in 19 cases, but was not detected in 7 other cases, 6 of which corresponded to severe, especially pedicular, lesions. The diagnostic means for the lesion included IVP in 24 cases, ultrasonography in 23, angiography in 8 and computed tomography in 7. The treatment was medical for 18 children and surgical for 8. Observation revealed 4 cases of renal atrophy, including 1 of general atrophy without residual arterial hypertension. On the basis of this series and of a review of the literature, we study the evolution of the diagnosis and treatment of this condition with the development of new medical imaging techniques, especially of CT.

Abdominal Injuries

Single-stage correction of posterior hypospadias (178 cases). Comparison of three techniques: free skin graft, free bladder mucosal graft, transverse pedicle preputial graft.

Three techniques have been developed for single-stage repair of posterior hypospadias: free skin grafts, free bladder mucosal grafts, and pedicle preputial grafts. This multicenter retrospective study of 178 children who underwent surgery for posterior hypospadias was designed to compare the results achieved with these procedures. Free skin grafts (15 cases) resulted in the most frequent complications, and in particular the most severe strictures; in our opinion this technique should be abandoned. Pedicle preputial grafts (133 cases) gave the greatest number of successes from the outset, and should be preferred whenever the dimensions of the prepuce are sufficient for urethroplasty. Results with bladder mucosal grafts (30 cases) were not as good as with preputial grafts, but this technique remains the only solution when the meatus is in a very posterior position, and for children who have already undergone multiple operations.

Adolescent

[Neonatal obstruction].

Neonatal obstruction is a radio-clinical syndrome, common to a wide range of malformative anomalies which alter the normal anatomy or function of the digestive tract at any level. The main etiologies are reviewed with emphasis on specificity due to embryology, topography and clinical presentation. It is relatively easy to diagnose neonatal obstruction and relate it to a definite cause. The successful treatment of such obstruction requires a pluridisciplinary collaboration of specialized teams.

Constriction, Pathologic

Antibiotic therapy of perforated appendicitis in children: a comparison of amoxycillin/clavulanate with a combination of benzylpenicillin, netilmicin and metronidazole.

This multicentre trial compared the clinical efficacy of amoxycillin/clavulanate used as a single-agent therapy with that of the three-agent combination usually prescribed in the post-operative period for appendicular peritonitis in children. Only bacteriologically documented peritoneal infections were included. Sixty-four patients were randomly distributed between two groups: Group A (29 cases) treated with amoxycillin/clavulanate, first administered iv (100 mg/kg/day) followed by conversion to the oral route (50 mg/kg/day) once the patient had been afebrile for 48 h; Group B (35 cases) first treated by the iv route with benzylpenicillin (100,000 IU/kg/day) plus netilmicin (5 mg/kg/day) plus metronidazole (30 mg/kg/day) followed by conversion to the oral route for metronidazole (30 mg/kg/day). In both groups, the total duration of parenteral and oral treatment was not less than five days. A total of 180 bacterial strains were recovered from peritoneal fluid samples obtained during surgery; 86% of these were sensitive to amoxycillin/clavulanate. Clinical efficacy, assessed on the basis of time until return to normal temperature and gut transit and duration of hospitalization, was identical in both groups, with follow-up monitoring on day 30 showing recovery in all cases. Cure was obtained without any problems of infection in 25/29 patients in group A and in 34/35 patients in group B (non-significant difference). Tolerance was excellent and identical in the two groups with the exception of three cases of thrombophlebitis which occurred in group B. The results of this study suggest that amoxycillin/clavulanate may be useful as single-agent therapy as a first-line curative treatment for appendicular peritonitis in children.

Amoxicillin

[Solid tumors of the adrenal gland in children (excluding neuroblastomas). A study of a series of 18 cases].

This is a survey of the French South-East Group of Research in Pediatric Surgery (GRECPSE) concerning 18 Solid Adrenal Tumors, observed during a 20-years period (1969-1989) in 11 boys and 8 girls. 13 are tumors of the adrenal cortex (9 adenomas and 4 carcinomas). Clinical virilization is found in 7, cushing's syndrome in 3; while feminizing tumor, Conn's syndrome and non hormone-secreting form are quite exceptional (1 case each, respectively). 5 are adrenal pheochromocytomas (4 benign tumors and 1 malignant with local invasion). Ultrasonography, CT Scan and MIBG Scintigraphy are modern procedures which permit localization of the small and non palpable tumors with high accuracy. They make easier surgical management. Differentiation between benign and malignant tumors of the adrenal glands still remains the main problem. The course of the disease is surely the only distinguishing parameter. However weight tumor and selective histopathological characteristics are useful in predicting the prognosis and behavior of such tumors = Mitotic activity for adrenocortical tumors, extensive areas of necrosis and small cells for pheochromocytomas have the highest discriminating value.

Adolescent

[Endoscopic treatment of vesico-ureteral reflux using a sub-mucous injection of Teflon paste in children. Apropos of 337 patients (491 ureters)].

We reviewed our experience with endoscopic sub-ureteral injection of polytetrafluoroethylene (PTFE) in 337 children (84% females, 16% males). Four hundred and ninety one refluxing ureters were injected (93% primary reflux, 7% secondary reflux after reimplantation, correction of exstrophy of the bladder or neurogenic bladder). Reflux was: - stage I: 7.8% - stage II: 32% - stage III: 38% - stage IV: 20% - stage V: 2.2% Follow-up cystograms were performed one 428 ureters, 6 to 30 months after injection. 80% of children are cured after one injection while 4% required two injections in order to attain the same result. Improvement in the grade of reflux (minimal residual disease) was noted in 10% of the patients and in 6% the procedure failed. In the latter group, 16 children were operated on without difficulty; lymphatic migration of PTFE was noted in two cases. In the 33 patients with secondary reflux, the endoscopic treatment was associated with an 80% cure rate, in the 26 patients presenting a reflux on duplex systems, cure rate was 65%. This is a simple procedure performed on an outpatient basis; in our experience with 491 injections there were no complications at the time of injection nor there any postoperative ureteric obstruction. Endoscopic PTFE injections are less efficient than surgical reimplantation in the treatment of vesico-ureteral reflux. Teflon paste may not be the ideal material, other substances are presently being investigated. This technic is limited by the caliber of the urethra in young boys (Charrière 14).

Adolescent

[Renal tumors in children, excluding Wilms' tumor. Apropos of 29 cases].

The authors present a multisource study of 29 kidneys tumors (Wilms excepted) in children. The most common types (18 cases) are tumors from metanephrogen blastema (congenital mesoblastic nephroma, nephroblastomatosis and nodular renal blastema, multilocular cystic nephromas. Mature kidney tumors are much more rare in childhood (8 cases): they are epithelial tumors (adenoma or adenocarcinoma) or mesenchymal neoplasms. In one case, histological study don't permit to list a malignant tumor to one of previous categories. Finely, among secondary tumors, two localisations of hemopathy are reported. After a limited exposure of cases and review of the literatures the authors emphasize the anatomo-clinical aspects, the value of paraclinical exams and the treatment of each type of tumor.

Female

[Fibrous epithelial polyps of the pyelo-ureteral junction in children. Presentation of 3 cases and review of the literature (33 cases)].

Primary ureteral tumors are rare and benign ones constitute only a small portion. Of the benign neoplasms, fibro epithelial polyps are probably the most common and could be considered to be an exceedingly rare cause of Hydronephrosis in children. The exact etiology of benign ureteral polyps is not certain but it is likely that they have a congenital one. They are mesodermal in origin and consist of a thick fibrous stalk covered by a layer of normal transitional epithelium. They are more commonly found in boys (80.6%), who present usually with abdominal pains or renal colics. Fibro-epithelial polyps often rise in the proximal ureter and renal pelvis, especially at the left side (69.4%). Radiographic signs are those of a non typical uretero-pelvic Junction obstruction. IVP and retrograde ureterogram usually demonstrate a long, thin, filiform, lucent filling defect within the ureteral lumen with moderate hydronephrosis. Appropriate treatment of these benign tumors is local excision with or without segmental resection. Prognosis should be considered as excellent without a report of recurrence. A review of the literature revealed 33 cases of ureteral polyps in children. We report here 3 additional cases of obstructing fibro epithelial polyps, observed in boys aged respectively 4, 8 and 9 years.

Child