Frequency of the severe combined immunodeficiency disease gene among horses in Morocco.

Severe combined immunodeficiency disease (SCID) of horses is an autosomal, recessive hereditary disease occurring among Arabian or crossbred Arabian horses. The genetic defect responsible was previously identified as a 5-base pair deletion in the gene encoding the catalytic subunit of the DNA dependant protein kinase (DNA-PKcs). This study was carried out to determine the frequency of SCID and identify horses carrying the gene for SCID among Arabian and Arabian crossbred stallions and mares in Morocco using a DNA-based test. Twenty-one horses were SCID carriers: 14 (7%) Arabians, 6 (4%) Arab-Barbs and one (33%) Anglo-Arab. After analysing their genealogy, 3 imported stallions were identified that disseminated the mutant gene of DNA-PKcs in Morocco.

[Stomach duplications apropos of a case revealed by digestive hemorrhage. Value of ultrasonic endoscopy. Review of the literature]. / Les duplications gastriques à propos d'un cas révélé par une hémorragie digestive. Intérêt de l'écho-endoscopie. Revue de la littérature.

Double stomach is a relatively rare inborn malformation. Manifestations are usually observed during the neonatal period or in early childhood. In adults, complications usually lead to diagnosis. Echographically and endoscopically, the malformation takes on the aspect of a tumour of the gastric mesenchyme. Echo-endoscopy is required for diagnosis. Surgery is the only treatment with or without exeresis of the gastric collar surrounding peripheral often pediculated lesions. Laparoscopic control may be helpful. Other procedures may be adapted on a case by case basis. Prognosis depends on associated malformations.

[Perforation of duodenopyloric ulcers. Prognostic factors and therapeutic choices. Retrospective study of 140 patients]. / Perforations des ulcères duodéno-pyloriques. Facteurs pronostiques et choix thérapeutiques. Etude rétrospective de 140 malades.

The treatment of perforation of duodenopyloric ulcers varies according to the risks of immediate mortality and long-term recurrence. The authors carried out a retrospective study on 140 duodenopyloric ulcers which had been treated over a 13 year period. The study high identified 3 factors of immediate mortality. Age over 70 years, admission delayed by more than 24 h and preoperative hemodynamic shock. Global mortality in this series is 8.6% and varied between 2.3% and 66.7% according to the absence or presence of these 3 risk factors, respectively. The authors recommend a simple operative protocol guided exclusively by the risk factors for mortality. The presence of a single factor requires simple suture of the perforation. Suture of the perforation and vagotomy should be performed in the absence of risk factors.

[Technique of isoperistaltic esophageal gastroplasty. Comparative study of in vitro arterial vascularization]. / Technique de gastroplastie oesophagienne isopéristaltique. Etude comparative de la vascularisation artérielle in vitro.

Operative survival is impaired by anastomotic fistula after esophageal resection and reconstruction. Hypovascularisation of esophageal substitute is essential. Stomach is a useful esophageal substitute however proximal resection of lesser curvature compromises vascularisation of the highest point of fundus which is usually sutured to esophagus. To improve this a new S shape esophagoplasty with isoperistaltique stomach was achieved. Fifteen cadaveric stomachs were assigned to three equal groups prepared as follow: narrow J shape stomachs, large J shape stomachs, large S shape stomachs. The arterial tree was studied following injection of contrast media barium from the right gastroepiploic artery. Proximal arteries of the S shape stomachs were well filled comparing to the others. This was allowed by keeping intact the tronc of the left gastric artery branches, severing the left gastroepiploic artery close to the spleen, and dividing the proximal fundal region as a curve.

Lupus psychosis: differentiation from the steroid-induced state.

Steroid induced psychosis in SLE is rare but clinically important, and often difficult to distinguish from lupus cerebritis. We report a patient with SLE who became depressed following an increase in her steroid dosage. Based on her clinical presentation and high levels of antibodies to P ribosomal proteins (both in CSF and serum) a diagnosis of lupus cerebritis was made. Steroid dosage, time intervals, and the duration of mental changes may help in differentiating steroid psychosis from lupus cerebritis. No single laboratory test sufficient to establish a definitive diagnosis of lupus cerebritis is available at the present time. However, elevated levels of antibodies to P ribosomal proteins may assist in confirming the diagnosis of this condition.

Influence of clinical and hemodynamic characteristics on signal-averaged electrocardiogram in postoperative tetralogy of Fallot.

This study examines the relation between signal-averaged electrocardiographic measurements and the occurrence of spontaneous ventricular arrhythmias in 86 patients with a postoperative right bundle branch block after repair of tetralogy of Fallot; special attention was given to the influence of age, body surface area and right ventricular systolic pressure on signal-averaged electrocardiograms. Twenty-eight of the 86 patients had significant ventricular arrhythmias on 24-hour ambulatory monitoring. A positive linear correlation was found between filtered QRS duration and age at evaluation or body surface area (r = 0.45, p = 0.00001; r = 0.54, p < 0.00001, respectively) and between amplitude of the last 40 ms of the filtered QRS and right ventricular systolic pressure (r = 0.48, p < 0.001). A negative linear correlation was found between amplitude of the last 40 ms of the filtered QRS and age at evaluation or body surface area (r = -0.27, p = 0.01; r = -0.34, p = 0.002, respectively). When the age of the patients or the body surface area was considered with an analysis of covariance, the presence of ventricular arrhythmias was associated with a higher amplitude of the last 40 ms of the filtered QRS. In addition, an amplitude of the last 40 ms of the filtered QRS > 170 microV had an excellent sensitivity (100%) and a good specificity (88%) for identifying patients with both right ventricular systolic hypertension and spontaneous ventricular arrhythmia. Thus, adjustment of signal-averaged parameters for age at evaluation and body size is mandatory when studying postoperative tetralogy of Fallot.

[Management of patients over 75 years of age with digestive or abdominal disease. A series of 660 cases]. / Prise en charge des patients âgés de plus de 75 ans présentant une pathologie digestive ou abdominale. A propos de 660 observations.

Over a period of 3 years, the authors cared for 660 patients over 75 years of age with diseases of the digestive tract. In 180 cases, the initial hospitalization had been previously planned and in 480 (72.7%) emergency admission was necessary. The diagnosis had not been confirmed in 90% of the emergency admissions. Half of the patients suffered from major illness (ASA III, IV). Planned hospitalization in a surgical unit involved cancer in 60% of the cases among which about one-half were colorectal cancers. In this latter group, mean hospitalization was 12 days and hospital mortality was 9.7%. For the patients with emergency admissions, diagnosis was only established after exploratory examinations (echography 296, endoscopy 205, opacification of the digestive tract 157...). No diagnosis could be established in 1.6% of the patients. Four types of emergency patients could be identified: occlusions 39.6%, painful abdomen 37%, haemorrhage 13.9% and jaundice 7.7%. In these groups, surgery was required in 64.7%, 69.1%, 72% and 40.5%, and mortality was 21.9%, 20.3%, 21.6% and 8.1% respectively. Duration of hospitalization was however similar (12 days). The overall mortality in this series was 14% (5.7% in non-operated patients and 16.7% in operated patients with a distinction between planned operations, 9.7%, and emergency operations, 20.3%). The two main risk factors were underlying disease and the need for emergency surgery. Age over 90 was also aggravated prognosis.

[Should routine exploratory laparotomy be performed in the presence of an abdominal wound? Discussion apropos of 176 cases]. / Le dogme de la laparotomie exploratrice doit-il être maintenu en présence d'une plaie de l'abdomen? Discussion à propos de 176 observations.

Management of abdominal wounds is presently the subject of discussion between the partisans of routine laparotomy and those preferring "armed" surveillance. Results of study of a series of 176 abdominal wounds subjected to surgical dogma showed: that the diagnosis of non penetrating wounds (17.6%) was not always evident, due either to their anatomical localization (frontier region wounds) or to insufficient local exploration in urgent cases (6.6% of false-negatives), that the existence of serious clinical signs (50 cases) was always associated with one or more visceral lesions, requiring urgent laparotomy with a morbidity of 20% and a mortality of 8% (4 cases), that in the case of asymptomatic penetrating wounds (96 cases), routine laparotomy did nevertheless allow the diagnosis of visceral lesions in 50 cases (including 23 major lesions) but was of no utility in 46 cases (31.5% of blind laparotomies for the total series). The elevated proportion of useless laparotomies (30% in the literature), the result of a dogmatic attitude, or the risk of a delayed intervention (5 to 8%) in the series practising the selective method, led to a modification in the authors' attitude. The existence of serious signs should obviously result in a laparotomy. In their absence, and when confronted with a penetrating or doubtful (frontier region) wound, an exploratory celioscopy is proposed to ensure complete abdominal exploration, to confirm the presence or absence of penetration, to treat minimal lesions and to perform a classical laparotomy in case of evident necessity.

[Influence of age and body surface on the signal averaged high amplification ECG after complete repair of tetralogy of Fallot]. / Influence de l'âge et de la surface corporelle sur le signal moyenné de l'ECG haute amplification après réparation complète des tétralogies de Fallot.

The aim of this study was to assess the influence of age and body surface area on the signal averaged ECG after complete repair of Tetralogy of Fallot. Fifty eight patients operated for Tetralogy of Fallot in whom the postoperative ECG showed right bundle branch block without any significant arrhythmia on Holter monitoring or exercise stress testing underwent signal averaged electrocardiography. A very significant relationship was observed between age and body surface area and the duration of the filtered QRS (r = 0.45, p = 0.0004 and r = 0.49, p = 0.00009 respectively) or the average voltage of the last 40 milliseconds (r = -0.49, p less than 0.03 and r = -0.31, p less than 0.02 respectively). Therefore, signal averaged ECG in patients operated for Tetralogy of Fallot varies with age and morphology. An adjustment of the parameters of the signal averaged ECG with respect to clinical characteristics is necessary before assessing the value of this technique for identifying patients at risk of developing ventricular arrhythmias.

[Gallstone obstruction of the common bile duct, a severe form of biliary lithiasis. Choice of treatment]. / L'empierrement du cholédoque, une forme grave de lithiase biliaire. Quel traitement choisir?

Collection of 10 or more stones in the extrahepatic common bile duct causes lithiasic obstruction of the CBD, a fairly rare entity observed in less than 10% of cases of bile duct lithiasis. This study is based on 35 cases recorded over 10 years, including 60% in patients aged more than 75 years. Endoscopic treatment was attempted in 28% of patients and was totally successful in one case our of four only because of insufficient removal of obstruction after sphincterotomy. Surgery, either necessary (8 cases) or systematic (25 cases) combines cholecystectomy, choledocotomy and biliary fiberendoscopy for a complete treatment minimizing the risks of residual lithiasis. While biliodigestive anastomoses prevailed (58%) during the first years of this study, external biliary drainage was most often chosen during the last 5 years. This surgery in aged patients still entails high morbidity (25%) and considerable mortality (9%). Better efficacy may be provided in the future by the combination of fiberendoscopic means and of lithotrity for aged subjects at high surgical risks.

[Holter monitoring, exercise test and atropine test in isolated congenital atrioventricular block in children]. / Evaluation par enregistrement Holter, épreuve d'effort et test à l'atropine des blocs auriculo-ventriculaires congénitaux isolés de l'enfant.

Twenty-four patients with isolated congenital heart block were investigated by 24-hour Holter monitoring at an average age of 9.3 +/- 5.5 years. Six patients were symptomatic and 18 were asymptomatic. Eight asymptomatic patients underwent exercise stress tests and an atropine test was performed in 10 asymptomatic patients to evaluate the capacity to accelerate the heart rate. The symptomatic patients were older than the asymptomatic patients. None of the parameters which analyse ventricular rate were significantly different in the two groups of patients. Significant ventricular arrhythmias (Lown Grade 2 or over) were recorded in 1 symptomatic and 3 asymptomatic patients. The incidence of these ventricular arrhythmias increased with age and degree of bradycardia. The percentage increase in ventricular rate after atropine correlated with what was observed on effort (r = 0.95, p = 0.01) but there was no relationship between the ventricular rates during these two tests and those recorded on Holter monitoring. The results of this series of children with isolated congenital heart block show the Holter parameters cannot distinguish symptomatic from asymptomatic patients. The exercise stress and atropine tests gave very similar results but their prognostic value has not yet been established.

[Incidentally discovered adrenal tumors. Apropos of 20 cases]. / Les incidentalomes surrénaliens. A propos de 20 cas.

According to Glazer, the term of incidentaloma describes an adrenal tumor discovered incidentally during acomputed tomographyscan and by extension to all masses discovered whatever the imaging process. Twenty patients have been operated during the last 10 years. The discovering circumstances are not specific, except the ecography and CT Scan. Complementary examinations include biological tests aiming at showing a possible hormonal secretion and more specific examinations MIBG scintygraphy and magnetic resonance imaging. The tumors often large (80% more than 5 cm long) and solid (75%) have all been removed by transperitoneal approach (13) or lombotomy. Post operative course was approach unevertful. The histological study showed 6 metastasis, 5 benign kystic lesions, 3 hematomas, 3 adenomas, 2 adrenalcortical carcinoma and 1 ganglioneuroma. A review of the literature series leads us to evaluate the frequency and risk of incidentalomas and to consider the necessary examination and the strategy to be adopted in front of such lesions. The evaluation of hormone status, the recourse to specific imaging seems necessary whereas the guided needle biopsy does not present any risk and should be reserved for very special cases. A decisional scheme is proposed, which ains at removing all voluminous and/or secreting tumors. Small lesions behind 35 mm will have to be examined closely and will be removed if they grow larger or persist.

[Traumatic hemoperitoneum of splenopancreatic origin. Apropos of 155 cases. Can a non-surgical treatment be proposed?]. / Les hémopéritoines traumatiques d'origine spléno-hépatique. A propos de 155 cas. Peut-on proposer un traitement non opératoire?

From 1985 to 1990, we treated 155 patients presenting with a hemoperitoneum secondary to a splenic or hepatic injury (diagnosis established by sonography, puncture and washout and/or laparotomy). These were 39 children and 116 young adults (average age 33 years). Fifty-eight of them (37%) suffered from multiple injuries (11 children, 47 adults). Splenic lesions were observed in 110 cases and hepatic lesions in 45. Ninety-one patients were operated, 55 in emergency and 36 later, while a watch-and-wait policy was applied to 64 (42%), with repeated clinical and complementary (ultrasound and/or CT) examinations. The indication for surgery was based on the clinical findings and the necessity of blood transfusion (more than 40 ml/kg/24 h in children, more than 2 to 5 U/24 h in adults, according to the context). This attitude allowed us to avoid operating two-thirds of the children and one third of the adults. We assess the limitations of this method.

[Infectious endocarditis of the tricuspid valve in patients with endocardial pacemakers]. / Endocardites infectieuses tricuspidiennes sur sonde de stimulation permanente endocavitaire.

Right heart endocarditis in patients with permanent cardiac pacing catheters is uncommon but not an exceptionally rare complication. Two dimensional echocardiography provides diagnostic and prognostic information. The authors report two cases which illustrate the fact that many workers recommend explanation of the pacing catheter and that tricuspidectomy with or without valve replacement may sometimes be necessary.

[Incidence and factors favouring ventricular arrhythmia after surgical repair of tetralogy of Fallot]. / Incidence et facteurs favorisant les arythmies ventriculaires après réparation de tétralogie de Fallot.

The prevalence and factors favorising postoperative ventricular arrhythmias in 62 patients undergoing surgical repair of tetralogy of Fallot between 1971 and 1982 were analysed. Nineteen patients (31 %) had significant ventricular arrhythmias (Lown greater than or equal to 2) on Holter monitoring or exercise stress testing. The arrhythmia could only be recorded after stress testing in 5 patients. The patient's age at operation, the duration of follow-up and age at evaluation were significantly greater in the group with ventricular arrhythmias. However, age at operation was closely related to the other two parameters and a discriminating analysis showed that the age at operation was not a favorising factor for the occurrence of ventricular arrhythmias (p = 0.23), in contrast to the duration of follow-up (p = 0.0015) and age at evaluation (p = 0.0007). No relationship was found between ventricular arrhythmias and the following factors: previous anastomosis, outflow patch, necessity of a ventriculotomy or reoperation, presence of residual ventricular septal defect, postoperative systolic right ventricular pressure, severity of intraventricular conduction defects, and effort tolerance. These results suggest that after surgical repair of tetralogy of Fallot, the incidence of ventricular arrhythmias increases with time. The main problem is to identify patients with a high risk of sudden death, i.e. those with high degree ventricular arrhythmias and poor haemodynamic results of repair.

[Traumatic rupture of the bladder. Apropos of 11 cases]. / Les ruptures traumatiques de la vessie. A propos de onze cas.

Eleven cases of rupture of bladder were observed within 7 years. Four of them were due to a direct trauma on a full bladder (group I), the other seven occurred during a complex trauma, constantly with a fracture of the pelvis (group II). In group I, the rupture was situated on the apex of the bladder. The symptoms were a suprapubic pain in four patients, a hematuria in two, and an anuria in one. In the four patients, intraperitoneal fluid was clinically evident, and confirmed ultrasonographically. An urography was performed in two patients and showed an intraperitoneal leak in our patient, and intravesical clots in the other one. The four patients were operated on within 48 hours, and were successfully treated by suture and vesical catheter. In group II, the vesical injury was revealed by hematuria (2 patients) hemorragic shock (2 patients) or both (2 patients). In the last patient, an abdominal pain after 10 days was the only symptom. Clinical signs of hemoperitoneum were present in only four patients and were confirmed ultrasonographically in two out of five patients. The urogram was normal in two of four patients. In one patient it showed a small bladder, and in another one a vesical leak. Five patients were operated on among where three in emergency and two after 9 and 10 days. Four patients with intraperitoneal rupture were successfully tread by suture of the bladder. Among three patients with extraperitoneal rupture, one had an additional tear of the mesentery and died from intravascular coagulation, the second was successfully treated by a suture, and the last one by a vesical catheter only.(ABSTRACT TRUNCATED AT 250 WORDS)

[Muco-epidermoid cancer of the esophagus. Review of the literature. Report of a case with 9 years' follow-up]. / Les cancers muco-épidermoïdes de l'oesophage. Revue de la littérature. Rapport d'un cas avec recul de neuf ans.

Mucoepidermoid carcinoma of esophagus is a distinctly uncommon neoplasm. Diagnosis depends on precise microscopic criteria. Microscopic features should show distinct epidermoid features with evidence of intracellular mucus secretion. Their origin is generally believed to be esophageal submucosal glands or their ducts. In a survey of the literature, only 15 cases were found to have been reported. A case of a 43 year old man with a 9 year follow-up is presented. Prognosis of mucoepidermoid carcinoma is the same as ordinary squamous cell carcinoma of the esophagus.

[Volvulus of the sigmoid colon]. / Volvulus du côlon sigmoïde.

The authors report of 34 cases of sigmoid colon volvulus occurring in young subjects (15%) and in elderly subjects (60% over the age of 75 years). The diagnosis is frequently suggested by the clinical features and the history (30% of patients have a history of a previous identical episode) and is confirmed by a plain abdominal x-ray and/or an opaque enema. Endoscopy is performed in every case, except in the presence of peritoneal signs, in an attempt to perform detorsion and colonic intubation under direct vision in order to avoid emergency surgery; this procedure is effective in 87% of cases. Emergency surgery has a high mortality rate (43.5%). Elective or deferred emergency surgery after preparation is much safer (6.6% mortality). In patients with multiple diseases, non-surgical conservative management after detorsion too frequently results in repeated complications, leading to decompensation of the concurrent illnesses and a mortality rate of 34%. Endoscopic detorsion followed by surgical resection after a short 3 to 4 day preparation seems to be the best guarantee of therapeutic success.