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Biomedical subjects

Y Revillon

Publications and source records attributed to Y Revillon.

At least 19 recordsLinked to original sources

[Cystic adenomatoid malformation of the lung revealed in a newborn infant by an image of a lung abscess].

BACKGROUND: Cystic adenomatoid malformation, a rare pulmonary malformation, usually appears as a cystic mass, radiologically. It may be infected and confusion has also arisen in distinguishing it from pneumonia with pneumatoceles. CASE REPORTS: A full-term boy suffered from severe neonatal respiratory distress. Pregnancy had been uneventful despite the fact that his mother had insulin-dependent diabetes. Prenatal ultrasonographies did not reveal any abnormality. On day 2, X-rays showed a right pulmonary mass that appeared solid. The patient was treated for E Coli sepsis. Subsequently, the pulmonary mass became lacent, cystic, fluid-filled, resembling an abscess; the CT scan confirmed these features. As the lesion increased in volume, a limited resection was performed. Histologic examination showed adenomatoid proliferation of bronchiolar elements with formation of cysts and necrosis. CONCLUSION: Infection of cystic adenomatoid malformation may supervene the first days of life resulting in a lung abscess appearance.

Cystic Adenomatoid Malformation of Lung, Congenita

Management of oxalosis.

Oxalosis is the final stage of primary hyperoxaluria type I (PHI) when reduction of GFR produces oxalate accumulation. It involves bones, arteries, eyes, heart, nerves, etc. The management of oxalosis starts with prevention of nephrocalcinosis and renal failure by diluting urine and by inhibiting oxalate crystal formation either by increasing the urinary citrate or the urinary pyrophosphate. At endstage renal disease (ESRD) there is no dialysis modality for avoiding the progression of oxalosis. Combined liver/kidney transplantation (LKT) represents the most effective approach. The European PHI transplant registry recently reported 64 LKT with a five-year patient survival of 80% and progressive healing of oxalosis. Four children who received LKT in our unit are reported, all of whom are alive with a follow-up of three months to five years. Bone disease completely healed in one case after three years, but retinal deposits persisted despite improvement of visual acuity. A special perioperative protocol must be applied for protecting the graft from oxalate released from the stores. Laboratory follow-up including oxalemia and urinary crystal volume helps to adjust the individual prescriptions. This intensive management must be continued as long as oxalate stores persist.

Combined Modality Therapy

Liver transplantation in two cases of propionic acidaemia.

Orthotopic liver transplantation (OLT) was performed in two patients with propionic acidaemia, a 7-year-old boy and a 9-year-old girl, diagnosed with a severe neonatal form with high risk of metabolic decompensation. In both cases the metabolic liver functions recovered within the 12 postoperative hours; no clinical symptoms of propionic acid toxicity, metabolic acidosis, severe hyperammonaemia, hyperglycinaemia or haematological abnormalities were observed. In both cases insulin-dependent diabetes mellitus occurred early after OLT (persisting in the boy's case). Severe post-transplantation complications were observed (acute rejection and CMV infection in both patients) which did not trigger metabolic decompensation. The boy developed chronic rejection and vanishing bile duct syndrome due to incomplete hepatic arterial thrombosis. He required permanent in-patient care with chronic hyperammonaemia and neurological sequelae involving the basal ganglia and died 15 months after OLT. The girl left hospital after 2 months and is presently leading a normal life with almost no dietary protein restriction (40 g protein per day). Urinary urea excretion and daily protein intake increased after liver transplantation. Propionyl- and tiglylglycine disappeared immediately after OLT. Urinary methylcitrate and 3-hydroxypropionate remained at concentrations corresponding to those before OLT. However, the total of all characteristic metabolites of organic acid analysis was reduced to 50-60% of the values before OLT in both patients. Propionylcarnitine was still detected at significant concentrations. Plasma odd-chain fatty acid concentrations decreased continuously after OLT only in the girl's case. Tissue of both transplanted livers showed increased odd-chain fatty acid concentrations 9 and 15 months after OLT, respectively, in both patients. We consider that at present OLT should only be performed in severe forms of propionic acidaemia.

Acidosis

[Chronic septic granulomatosis revealed by neonatal pulmonary aspergillosis].

BACKGROUND: Pulmonary aspergillosis is now the main cause of death in chronic granulomatous disease (CGD); it may occur before the age of one year and then often reveals CGD. CASE REPORT: A male newborn was referred to hospital at 27 days of age for fever (39 degrees C), hemodynamic failure and biological inflammation syndrome caused by pulmonary infection. Chest CT scan revealed multiple and bilateral intraparenchymatous nodules. An open lung biopsy showed histiocystic granuloma with multinucleated giant cells. Culture of tracheal, bronchoalveolar lavage samples and lung biopsy grew positive for Aspergillus fumigatus. Impaired chemiluminescence production by neutrophils was detected, enabling the diagnosis of CGD. It was later confirmed by the study of neutrophils functions. The child recovered after 12 months of parenteral amphotericin B therapy. CONCLUSION: A febrile multifocal pneumopathy occurring in infancy should lead to consider the possibility of CGD which may be confirmed by the chemiluminescence test.

Aspergillosis

[Effects of intraperitoneal insufflation on hematogenous seeding of abdominal infections. Preliminary results of an experimental study in rats].

Most laparoscopic procedures require the creation of a pneumoperitoneum. In order to evaluate the potential hazards of bacteriemia related to insufflation, we conducted a study in the rat. Two groups of 20 Wistar rats were used for this study. Peritonitis was induced by opening the terminal ileum. Twenty-four hours later, 20 rats were insufflated at a mean pressure of 6 mm Hg (Group I). After one hour of insufflation, an hemoculture was performed via direct intracardiac puncture and in the other group of 20 non-insufflated rats (Group NI). Five of the 18 hemoculture were positive in the Gr. I (27.7%) and 6 out of 20 in the Gr. NI (30%) (chi 2 = 0.238 p = 0.62 non significant difference). These results suggest that insufflation does not facilitate hematogenous dissemination of bacteria from intraperitoneal sepsis in this animal model.

Animals

Motility of the transplanted small bowel: a manometric study in the piglet.

Complete transection of sympathic and parasympathic nerves is an inevitable consequence of intestinal transplantation. The aim of this experiment was to study if extrinsic denervation alters the motor function of the graft. Ten large-white piglets were used. In Group 1 control (5 animals), a segment of distal ileum was isolated on its intact neurovascular pedicle as a Thirty-Vella loop. In Group 2 study (5 animals), a Thiry-Vella loop of distal ileum was transplanted as a free autograft. The motility of intestinal loops was studied by intraluminal pressure recording at the 8th and 15th postoperative days, on conscious animals fasted for 24 hours. The measured patterns were period (P), duration (D), maximal amplitude (A) and speed of migration (S) of phases III of migratory myoelectric complexes (MMCs). One hundred and seven MMCs were recorded and compared between Group 1 and Group 2. The results were as follows: mean (+/- sem) of Group 1 vs mean (+/- sem) of Group 2. P (min) = 68.5 (+/- 8.1) vs 54.2 (+/- 3.2)(NS); D (min) = 5.9 (+/- 0.2) vs 5.9 (+/- 0.1) (NS); A (cmH2O) = 73.9 (+/- 4.1) vs 83.4 (+/- 3.2) (NS); S (cm/min) = 5 (+/- 1.1) vs 6.2 (+/- 1) (NS). The motor function of the loops was studied following intraluminal perfusion of a cholinergic compound (carbachol) at doses of 1, 2, 3, 4 and 5 mg. The mean and the maximal pressures were recorded during 3 minutes from the 2nd minute following injection of carbachol.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals

[Video-assisted pneumonectomies: lessons learned from an experimental study on an animal model].

In order to test the feasibility of video-assisted pneumonectomy, we conducted an experimental study in an animal model. We performed 12 attempts of video-assisted pneumonectomy (7 on the right side and 5 on the left side) in pigs with an average weight of 43.5 kg. We used a combined technique of conventional and endoscopic dissection. A complication-free pneumonectomy was possible in 7 animals. Among the remaining 5 animals, 6 vascular injuries occurred, one of which was lethal. Two other animals died during the procedure for unknown reasons. We conclude that: 1) the vascular risk of video-assisted pneumonectomy appears to be prohibitive; 2) the value of animal models for vats feasibility studies is questionable because of the major differences in anatomical conditions.

Animals

[Mid-term outcome of 2 cases with maple syrup urine disease: role of liver transplantation in the treatment].

BACKGROUND: Dietary treatment of maple syrup urine disease remains difficult; chronic nutritional support in the child does not always avoid acute crises so that liver transplantation may represent an alternate choice in some cases. CASE REPORTS: Two gypsy cousins were born by an interval of 4 days; both had maple syrup urine disease and were similarly treated from the first days of life. They were given exchange transfusions followed by diet restricted in the branched chain amino acids, maintaining normal growth and plasma leucine concentrations under 7 mg/100 ml. Laura, at 10 years, was retarded at school. Compliance to school attendance was limited by her diet problems. Helen suffered at 7 yr 3 mo from liver failure due to hepatitis A virus infection which required liver transplantation. Protein intake was normal 1 week later. At 10 years, she presented with the same degree of school retardation as her cousin, and was placed in the same class. CONCLUSION: Liver transplantation may be effective for treating metabolic problems in MSVD without significative difference between outcome post classic treatment or post liver transplantation.

Child

[The role of brachytherapy in the therapeutic strategy of vesico-prostatic rhabdomyosarcoma in children].

Because of its ballistic and physical characteristics, brachytherapy (BT) represents the optimal irradiation treatment in pediatric tumors, especially in prostate or bladder rhabdomyosarcoma. In a limited series of 15 children treated with BT (including three patients treated with salvage BT), 8 out of 9 patients surviving with NED benefited from conservative treatment among 12 patients treated with BT as first-line treatment. Sequelae were minimal consisting of one grade I cystitis and one asymptomatic vesical and ureteral reflux. BT allows administration of high doses to limited volumes sparing normal tissues. BT--whenever possible--represents the treatment of choice compared with external radiotherapy. Indications of BT depend on tumor accessibility and size (usually < or = 4 cm).

Adolescent

Thoracoscopic ultrasonic localisation of lung nodules: initial results of an in-vitro and in-vivo study.

The main problem in thoracoscopic resection of lung nodules is the difficulty in localising the target. In the following we describe the use of an ultrasonographic, deflectable linear array probe of 7.5 MHz which was first tested during an in-vitro study. This study has provided useful information with respect to the US inspection of normal parenchyma and lung nodules. Initial results in clinical use in 14 patients demonstrate some limitations linked to the difficulty in manoeuvering the probe and to the remaining air in the parenchyma. However, endoscopic US is an interesting additional tool during thoracoscopic exploration of the lung.

Equipment Design

[Characteristic aspects of vascular complications in renal transplantation in children].

In a series of 859 transplantations performed in children and adolescents from January 1973 to December 1992, vascular thrombosis accounted for 18% of all graft losses (2nd cause of graft failure), and 57% of early failures (within 2 months). Renal veins and arteries were equally affected. The young age of the donors and recipients constituted the main risk factor, thrombosis accounting for 40 and 37% respectively of all graft losses in donors and recipients < 5 years old. This explained the lower survival rate of these age groups after grafting, as reported in most published pediatric series. The prophylactic use of a low molecular weight heparin in high-risk grafts may help to decrease its incidence. A renal artery stenosis, located a few cm beyond the anastomosis in 2/3 of cases, was observed in 10% of grafts. Often severe hypertension (HBP) with cerebral complications, responsible for permanent sequelae in some cases, was the prominent symptom. Anti-hypertensive drug therapy was sufficient to control HBP in 40 cases out of 72, and in 10 of these, a spontaneous regression of the stenosis was remarked within a few months after repeated angiography. Transluminal angioplasty was used on 26 stenosis and was effective in 2/3 of cases; the recurrence rate, however, was 27%.

Adolescent

Intraoperative detection of pheochromocytoma with iodine-125 labelled meta-iodobenzylguanidine: a feasibility study.

We evaluated the feasibility of intraoperative detection of pheochromocytoma sites after injection of meta-iodobenzylguanidine labelled with iodine-125. Six patients with multiple or recurrent pheochromocytoma were injected for intraoperative detection. During surgery, all count rates were recorded using a CdTe detector diode. Tumour foci were found in all cases. Tumour count rates ranged from 50 to 1000 counts per second (mean approximately 400). Blood activity, used as a reference level, ranged from 10 to 50 counts per second (mean approximately 35). In all patients, the intraoperative probe was helpful to the surgeon and facilitated the discovery of the pathological foci even when they were small (< or = 1 cm). Complete resection under probe control was correlated with postoperative normalization of urinary normetanephrine excretion. The use of a probe designed to detect low-energy gamma-ray radionuclides bound to a highly specific molecule provides an accurate detection tool which is well adapted for ectopic localizations and for small foci.

3-Iodobenzylguanidine