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Biomedical subjects

R B Galifer

Publications and source records attributed to R B Galifer.

At least 37 records · Page 2Linked to original sources

[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↗

[Congenital abnormalities of the peritoneo-vaginal process].

The different clinical manifestations resulting from abnormalities of the development of the peritoneovaginal process are reviewed: inguinal hernias, abnormal testicular descent, herniated ovary, hydrocele of the tunica vaginalis, hydrocele of the spermatic cord. The pathogenesis is studied on the basis of the embryology of the vaginal processus. The surgical aspects are discussed.

Cryptorchidism↗

[Surgical problems posed by abnormalities of testicular migration].

Based on their experience and on a review of the literature, the authors study the surgical aspects of the undescended testis. A precise definition excluding the retractile testis is essential. Although the understanding of the pathogenesis remained incomplete, the present data give support to an early orchidopexy performed by a specialized surgeon following the failure of a human chorionic gonadotropin test.

Cryptorchidism↗

[Intestinal malformations and congenital heart diseases].

A series of 21 patients with both congenital heart disease and intestinal malformation seen over a 12-year period is reported. The intestinal malformations were: anorectal malformations (11 cases), duodenal atresia (5), omphalocele (4) and common mesentery (1). Congenital heart diseases consisted of: atrial septal defect (ASD) (10 cases), ventricular septal defect (VSD) (2), tetralogy of Fallot (2) and miscellaneous cardiopathies. In patients with anorectal malformations ASD and VSD predominated (6/11 cases) and multiple malformations syndromes were present in 8 cases, including trisomy 13, Vater syndrome, skeletal (4), neurological (3) and renal abnormalities (3); 3 children died. Duodenal atresia was always associated with left-to-right shunt: VSD (3), ductus arteriosus (2), complete atrioventricular canal (1) and trisomy 21 (2); one child died. Omphalocele coexisted with VSD (2), tetralogy of Fallot (1), dual outlet right ventricle (1), trisomy 21 (1) and multiple malformations syndromes (3); 2 children died. The patient with common mesentery had left-to-right shunt. Comparison of this series with data from the literature showed that children with congenital heart disease have a much higher incidence of intestinal malformations than those with normal heart and that they frequently present with multiple malformations (chromosome aberrations or multiple organ lesions). This multiple malformation complex is particularly common in anorectal malformations where the incidence of congenital heart diseases is 9 to 14%, with predominance of VSD and tetralogy of Fallot. In patients with duodenal atresia trisomy 21 is extremely frequent, and the incidence of cardiopathies is 18%; in the absence of trisomy 21 the cardiopathy is complex.(ABSTRACT TRUNCATED AT 250 WORDS)

Abnormalities, Multiple↗

[Congenital anomalies of the pyeloureteral junction in children. Multicenter study of 985 anomalies in 883 children].

The authors report a survey of the French Group of Visceral Pediatric Surgery, concerning 985 congenital anomalies of the pyeloureteric junction in 883 children. 67.3% of them are boys. This article stresses the recent improvements in prenatal ultrasonography and diagnosis which is now able to individualize a homogeneous group of asymptomatic children ready to be repaired in the early post-natal period. Early reconstruction is associated with the greatest degree of recovery of renal function. In children, the average age is 4 years 9 months at diagnosis. In 43.6% clinical symptoms suggest an infectious problem. Pain is noted in 35.5% and an abdominal mass is discovered in 13.3%. Excretory urography (IVP), ultrasonography and cystography are still the main investigations to be done in all cases. But it is now necessary to use modern isotopic technics such as DMSA and diuretic DTPA to quantify obstruction with accuracy and follow long-term evolution after treatment. Treatment is surgical and conservative in 81.4%. Almost all of the uretero-pyeloplasties are performed according to the dismembered technic usually with temporary nephrostomy drainage. Renal reduction is reserved only for the redundant and decompensated renal pelvis. Morbidity is low (2.5%) and good results can be expected in about 95% of cases.

Adolescent↗

[Acute intestinal invagination in infants and children, critical evaluation of the diagnostic and therapeutic strategy. Apropos of a series of 163 cases].

This study concerns a homogenous series of 163 cases of intussusceptions in children, seen in the Visceral Pediatric Surgical Unit of Montpellier from 1974 to 1985. The authors differentiate and compare two periods with regard to the year 1980: the first one (P1 = 64 cases) during which, surgery was always the rule;the current one (P2 = 99 cases) where non operative treatment is the modality of choice under precise conditions. Synthetic analysis and comparison of the different therapeutic groups which have been distinguished in every period lead to the following data. 14% of intussusceptions are directly operated whatever the period or the modality of management probably because this group represents the absolute contra-indications of a barium enema--It concerns children with a long duration of the disease, obstructive or peritonitis signs and late diagnosis--Laparotomy is mandatory and finds a high percentage of leading points and ileo-ileal forms. Intestinal resection was necessary in 59.5% of cases. Because of this and a poor general condition, morbidity was high and Hospital stay was long. 56.4% of intussusceptions are operated after failure of barium enema reduction. This hydrostatic irreducibility is probably explained by the prevalence of ileo-colic forms (53.3%) in this group. A shorter duration of the disease (less than 2 days in 43.1%) and a strong majority of idiopathic intussusception (90.5%) are surely responsible of a high percentage (80%) of successful manual reduction without the need of a resection.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute Disease↗