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Biomedical subjects

G Barneon

Publications and source records attributed to G Barneon.

At least 55 records · Page 3Linked to original sources

[Total colonic form of Hirschsprung disease. Apropos of 6 cases].

6 patients with total colonic aganglionosis without small bowel involvement (HCT) have been observed during a 13-years period. From this material and a review of literature, the authors stress the diagnostic and therapeutic particularities of HCT, which justify a separate analysis and a comparative study with the more typical forms of Hirschsprung's diseases. The percent of HCT out of all types of Hirschsprung's disease is 7.9%. Male to female ratio is 2:1 and familial occurrence is much higher (15 to 20%) than that seen in the classical form of Hirschsprung's diseases. A diagnosis of HCT is more difficult because of lack of specificity in clinical picture, radiologic findings and manometric evaluation. However, age at time of revelation is the neonatal period in 83% and enterocolitis is present in 25% with a higher incidence than in shorter types of Hirschsprung's disease. Therefore in HCT, laparotomy with appendicectomy and open frozen biopsies is always required for correct diagnosis. Ileostomy is mandatory. It must be done early and adequate in situation and technic. This kind of diversion has its own morbidity and mortality with a higher rate than that observed after colostomy. Definitive operation must be ideally performed at age 2. The principal of preserving and utilizing a segment of colon in a side-to-side anastomosis to the ganglionated small-bowel, followed by a pull-through ileoproctostomy has been established as a reasonable treatment for HCT since Martin's first description. 5 cases underwent a Martin repair and 1 a total colectomy.(ABSTRACT TRUNCATED AT 250 WORDS)

Cecum↗

[Juvenile fibromatosis with isolated intra-abdominal form. Apropos of 2 cases].

Among infantile fibromatosis, isolated intra-abdominal forms are the most unusual. The authors report two cases, a hepatic fibromatosis in an eleven year old girl, and a desmoïd tumor located in the jejunal mesentery in a ten month old infant. Pre-operative diagnosis is very difficult and histologic findings show a benign fibroblastic proliferation that tends to infiltrate surrounding tissues and often recur after surgical excision.

Child↗

[Pancreatic pseudo-tumor caused by bilio-pancreatic tuberculous lymphadenitis. 2 cases].

Peripancreatic tuberculous lymphadenitis is rare and of difficult diagnosis. The two cases described illustrate two clinical aspects according to the proximity of the biliary tract: either anterior lymph nodes responsible for obstructive jaundice, or posterior lymph nodes responsible for chronic epigastric pain. Endoscopic retrograde cholangiopancreatography is essential to exclude a pancreatic lesion and facilitate the interpretation of the CT images. Tuberculosis must then be suspected. In the absence of another tuberculous localisation, laparotomy is necessary to assert the diagnosis. Antituberculous chemotherapy alone, administered for 9 to 12 months, cures this form of tuberculous lymphadenitis.

Cholangiopancreatography, Endoscopic Retrograde↗

Testosterone-producing hepatoblastoma in a 3-year-old boy with precocious puberty.

The syndrome of isosexual precocious puberty (PP) associated with a primary malignant hepatic tumor is rare and previously reported in only 17 cases with poor prognosis. Twelve cases are well-documented gonadotropin-producing tumors. We here describe a new case of virilizing hepatoblastoma in a 2-year-10-month-old boy with evidence of testosterone (T) production by the tumor itself, and survival with a 3 1/2-year follow-up after an extended right hepatic lobectomy. Preoperative laboratory findings showed high levels of serum alpha-fetoprotein (AFP) and T:350,000 ng/mL and 4.92 ng/mL, respectively, which normalized after surgery. There was no circulating gonadotropin nor stimulation of the hypothalamic-pituitary axis. Testicular biopsy showed neither interstitial-cell maturation nor Leydig-cell hyperplasia. Moreover, demonstration of T secretion by tumor cells and T synthesis in presence of C14 progesterone was performed in an in vitro culture system. These data seem to provide supportive evidence of a T-producing hepatoblastoma.

Carcinoma, Hepatocellular↗

[Indefinite survival of pancreatic allografts under cyclosporin A treatment in rats].

Reports in the published literature suggest that cyclosporine A is less effective in the prevention of pancreatic graft rejection. Prolonged survival (97.9 days) of pancreatic allografts was obtained by the use of cyclosporine A in the rat, and indefinite survival was reported in 2 animals. Toxic accidents related to the compound were not observed. A complete histologic and metabolic study of pancreatic grafts showed that two types of response were possible according to whether the graft was rejected or tolerated. Cyclosporine A should be considered as a basic immunosuppressive agent for all types of allograft, including the pancreatic type.

Animals↗

[Ganglioneuroblastoma of the head of the pancreas. Report of a case in a 2-year-old boy].

This is a case report of a two years old young boy whose abdominal tumor was discovered by a routine examination. The mass was a huge, solid and painless one, located anteriorly in the right upper quadrant of the abdomen. Abdominal X-ray, IVP and ultrasonography showed a calcified mass with a normal right kidney. Urinary catecholamine excretion was significantly increased and the diagnosis of extrasurrenal prerenal sympathoblastoma was preoperatively suspected. Transverse laparotomy founded an encapsulated solid tumor, widening the duodenal loop and developing in the head of the pancreas. A cephalic duodenopancreatectomy was performed with an un eventful post-operative course. Histopathological examination confirmed it was a ganglioneuroblastoma of the head of the pancreas with lymphatic metastasis and local invasion of the neighbouring pancreas with immature tissues. The child was then treated with radiotherapy (35 Grays) and chemotherapy (Oncovin-Endoxan alternated with Oncovin-Adriamycine). The boy is now in good health with a two years follow up. Malignant pancreatic tumors are exceedingly rare in the pediatric age group (about 40 cases in the literature). Most of them are carcinoma and sympathoblastoma is quite exceptional (Research has yielded only one case : Bienaymé, 1976). The presence of a nervous tumor in such an unusual position explains the lack of precision in the preoperative location and brings up the difficult question of prognosis. It leads to pathogenic hypothesis.

Child, Preschool↗

Somatostatin treatment of psoriasis.

Somatostatin treatment was administered to 20 psoriatic patients according to the following protocol: Continuous infusion (250 micrograms/h) for at least 2 days followed either by short infusions (1 h) at 8 A.M. and 8 P.M. (12 cases) or by repeating the initial 2-day infusion (eight patients). Before treatment (day 0) and on day 6, biopsy specimens were taken for routine examination (12 patients) and for ultrastructure (seven patients). In vitro immunological studies were carried out on peripheral blood lymphocytes (six patients) on day 0 and day 8. In two patients, somatostatin was stopped because of serious side effects. Thus, clinical results were evaluated in 18 patients, on day 30. In ten of them no improvement whatsoever occurred, two had a partial clearing and an almost complete remission was achieved in six others. Ultrastructural studies showed, on day 6, enlargement of the intercellular spaces with deposits of granular material of glucidic composition, associated with features of cellular damage. Percentages of T and B cells were unmodified but a significant depression of mitogenic stimulation by PHA and ConA was clearly observed on day 8. Even if somatostatin treatment may have a beneficial effect in some patients it seems much less valid than other well-known therapies for psoriasis.

Adult↗

[Ultrasonography before surgical operation of retroperitoneal malignant tumors in childhood (author's transl)].

Abdominal tumors in childhood are diagnosed almost always by palpation only, the retroperitoneal localization of these are confirmed by I.V.P. The new gray scale ultrasound machine seem to be very useful in the staging of the masses before the surgical operation. For the correct appreciation of the sensibility of gray-scale ultrasound, 6 Wilm's tumors, 6 neuroblastoma and 1 malignant retroperitoneal fibroma were reviewed. These children were echographied just before the surgical procedure and the surgeon's reports were compared with ultrasonics findings. If the sensibility is good to give measurement of the masse and to establish the situation of the tumor toward the midline of the abdominal cavity, the visualisation of the great vessels was not so obvious. However these hepatic structures were well established in all cases. The renal vein was never seen. At the end of this report it's possible to describe differential echographic findings between Wilm's tumor and neuroblastoma. --A Wilm's tumor is a mass well limited in periphery, confused with the kidney and with a frequent sonolucent rim sign. --A Neuroblastoma is a very irregular tumor well separated of the kidney without sonolucent area in its limit. U.S. made after an I.V.P. is in all cases very useful to surgeons. The two methods with their additional findings make almost always arteriography without interest.

Adolescent↗

[Homocystinuria. Histological and ultrastructural study. Report of a case (author's transl)].

The histological and ultrastructural appearance of the skin is reported in a case of homocystinuria. By optical microscopy, the collagen fibers appear granulose and non fascicular in the superficial dermis; in the deeper layers they are rare; the elastic network of the medium and lower dermis is thick and disrupted. Electron microscopically, some fibroblasts exhibit large autophagic vacuoles; the collagen fibers are normal while the elastic fibers are broken and dense; the dermal capillaries are normal. Vitamin B6 has shown its action by recoloring the patient's hair.

Child↗

[Acroangiodermatitis developing on paralytic limbs (author's transl)].

4 cases of acroangiodermatitis developing on paralytic feet are reported. Acroangiodermatitis might be an entity different from Mali's syndrome and Stewart-Bluefarb's syndrome. Paralysis could generate the dermatologic lesions by increasing venous stasis and enhancing arteriovenous channels.

Acrodermatitis↗

A medulloblastoma in a baboon (Papio papio).

A transitional medulloblastoma was found in the cerebellum of a young female baboon. The primary lesion that permitted its discovery was papillary and retinal edema in the peripapillary zone.

Animals↗

[Xanthogranuloma in adults. Clinico-pathological study of a case].

The authors report a case of naevoxanthoendothelioma in a 58-year-old woman. 24 cases of naevoxanthoendothelioma in adults have already been reported in the literature. If the age of the patient is considered, nosologic problems may arise, especially with the strictly cutaneous reticulohistiocytosis of Senear and Caro. Biological and ultrastructural features allow the authors to develop some hypotheses about pathogenesis of the disease.

Diagnosis, Differential↗