[Extrafascial excision of the rectum or total meso-rectal excision?]. / Résection extrafasciale du rectum ou excision mésorectale totale? Etude anatomochirurgicale.

Total mesorectal excision is a valuable technique in preventing local recurrences cancer. However the use of the word "mesorectum" is inaccurate anatomically, and the implication that total excision of all the perirectal fat contained within the perirectal fascia in all patients with rectal cancer will minimize local recurrences remains contentious. The term extrafascial excision of the rectum is more accurate. He may contribute as well to a better understanding of the surgical technique allowing all surgeons to improve their own results.

Intrascrotal hernia of the ureter and fatty hernia.

Intrascrotal hernia of the ureter is a rare event. We describe here one such case. There are two anatomic types of such ureteral hernias. The paraperitoneal type has a peritoneal indirect sac, which pulls the ureter with it. The extraperitoneal ureteral hernia is without a peritoneal sac. In such cases, which are almost always indirect hernias, there is usually a large amount of fat. It is, in fact, retroperitoneal fat, which slides, and pulls the ureter with it by gravity. Such a case is a genuine prolapse of the retroperitoneal structures. This anomaly, which has been rarely studied, is worth knowing about, because the ureter may be damaged during hernia dissection. The surgeon should be cautious when discovering huge fatty hernias, and should avoid the excision of fat and simply return the fatty mass to its normal place after its separation from the cord.

[Recklinghausen's disease and gastrointestinal stromal tumors]. / Maladie de Recklinghausen et tumeurs stromales digestives.

Gastro-intestinal stromal tumors associated with Recklinghausen's disease should be considered in the current concept of the stromal tumors with reference to recent advances in immuno-chemistry. In this setting, there is an high potential of maluignancy. For the treatment of these lesions, surgery is the main tool. Frequency of malignant digestive diseases associated with Recklinghausen disease should be kept in mind.

[Intrascrotal herniation of the ureter]. / Hernie urétérale intrascrotale.

We describe one case of intra-scrotal hernia of the right ureter. The anomaly was recognized on a pre-operatory urogram. It was an extra-peritoneal ureteral hernia. There are two types of ureteral hernia: the para-peritoneal hernia with a peritoneal sac; the extra-peritoneal type with only a fatty hernia. The mecanism of the latter illustrated by our case is a prolapse of the retro-peritoneal fat. It is not possible to practice an urogram before the cure of every inguinal hernia, so the prevention in this rare situation of the damage of ureter is to be cautious in the resection of huge lipomas and sliding fat when operating on inguinal hernias.

[Intravenous immunoglobulin in children with Guillain-Barré syndrome]. / Imunoglobulina endovenosa em crianças com síndrome de Guillain-Barré.

We report our experience with intravenous immunoglobulin (IVIG), plasmapheresis and supportive care in 13 patients with the Guillain-Barré syndrome. Seven of 13 patients received IVIG, 2 plasmapheresis and 4 supportive care. At 15th day after IVIG administration, all patients in this group had improved at least one disability grade. In the plasmapheresis group, 1 improved at 5th day after the procedure. Two of the 4 patients that received supportive care improved at 20th day of evaluation. In the IVIG group, the final scores were lower and had no relapses. These results suggest faster clinical improvement with IVIG when compared with supportive measures.

[The use of clinical score to estimate the severity of an acute attack of asthma] / Uso de escore clínico para a avaliação da gravidade da crise de asma.

OBJECTIVE: To identify patients with acute asthma who require intensive care, using a clinical score.METHODS: Retrospective analysis of 299 patients who were admitted to the hospital for acute asthma, between 1993 and 1996. Two groups were selected: group I (GI) with 26 patients admitted to the Intensive Care Unit; group II (GII) with 52 patients admitted to the Emergency Room, matched by age and sex with those of GI. A clinical score to assess the groups had the following items: heart rate, respiratory rate, retractions, cyanosis, alertness level, previous treatment, previous hospitalization, and duration of attack. The score ranged from 0 to 2 points for each of those, summing up 11 points.RESULTS: The clinical parameters that discriminated well the 2 groups were: retractions (p<0.001), cyanosis (p<0.01) and alertness level (p<0.005). Through a discriminate analysis of the clinical score components, 22/26 patients were recognized as GI and 40/52 patients were recognized as GII, showing an accuracy of 85% and 77% respectively for GI and GII.CONCLUSION: The clinical score was useful to discriminate patients with severe attack of asthma who might require intensive care, and should be employed at hospital admission.

[Evaluation of the complement system and immunoglobulin G levels in cord blood. Relationship with gestational age and intrauterine growth]. / Avaliação do sistema complemento e da imunoglobulina G no sangue do cordão umbilical de recém-nascidos. Relação com a idade gestacional e adequação do crescimento intra-uterino.

OBJECTIVE: To evaluate the complement system in cord blood and its relationship with the degree of maturation and intrauterine growth. METHODS: Serum levels of C(3), C(4) and CH(50) were measured in premature, small for gestational age and appropriate for gestational age newborns. The activation of complement system was searched by clivage product determination C(3d) and C(4d). Serum IgG levels were also determined in all children. RESULTS: The levels of C(3), C(4), CH(50) and IgG were significantly lower in preterm (p < 0,001); C(4) and IgG values were also significantly lower in small for date than those in normal newborn. C(3d) and C(4d) were not detected, indicating that the complement system had not been activated. CONCLUSIONS: The lower complement component levels and IgG in newborn are related to gestational age as well as to intrauterine growth.

[Acute hepatic failure associated with valproic acid in children. Report of 3 cases]. / Insuficiência aguda hepática associada ao ácido valpróico na infância. Relato de três casos.

We report the cases of three epileptic children who developed hepatotoxicity induced by valproic acid. Two patients had developmental delay. Including the one who died, all patients were receiving polytherapy (carbamazepine in two and phenobarbital in one). The patients age ranged from 2 years and 8 months to 5 years and 1 month. The onset of hepatic complications occurred within 6 months of valproate therapy in two patients and 12 months in one. All patients developed the classical clinical signs of hepatotoxicity. Vomiting, edema and jaundice were the initial symptoms. Fever occurred in two patients. The serum levels of glutamic oxaloacetic transaminase were mildly elevated with a maximum of 194 IU. The bilirubin levels ranged from 5.5 to 19.8 mg%. Two patients recovered clinically and showed normalization of the laboratory abnormalities and one had fatal course. The hepatotoxicity must be considered as a side effect of valproic acid mainly in children under two years age, with polytherapy regimen and neurologic damage. The hepatic insufficiency can be reversible.

Extrapyramidal disorder secondary to cytomegalovirus infection and toxoplasmosis after liver transplantation.

A boy underwent liver transplantation for postnecrotic cirrhosis secondary to Wilson's disease. The patient had no neurological clinical manifestations prior to the transplantation. The patient developed dysarthria, dysphagia, spasticity, rigidity, and intention and resting tremor of all extremities. Cranial computerized tomography revealed hypodensity of the thalamus, basal ganglia and external capsule. Anti-cytomegalovirus IgM became positive. At autopsy, there were severe pathological changes at the thalamus and basal ganglia.

Evaluation of complement activation in premature newborn infants with hyaline membrane disease.

Fifteen premature newborns with hyaline membrane disease causing acute respiratory distress were evaluated for complement activation. A high intrapulmonary right-to-left shunt and marked arterial-alveolar oxygen difference indicated the severity of the respiratory failure. Twenty preterm healthy infants served as controls. Total haemolytic activity, plasma concentrations of complement components and regulatory proteins (C3, C4, C1-inhibitor, factors H and I) as well as activation products (C3a, C3dg, C1rsC1-inhibitor, C3b(Bb)P) gave no evidence of significant complement activation. Functional activity of the ubiquitous regulatory protein C1-inhibitor was significantly reduced without impact on classical pathway activation. These data suggest that, in contrast to the adult form of respiratory distress syndrome, the low-pressure pulmonary oedema characterising hyaline membrane disease is not mediated by activation of the complement system.

[Spontaneous rupture of the spleen during chronic pancreatitis. A report on two new cases (author's transl)]. / Rupture spontanée de la rate au cours des pancréatites chroniques. A propos de deux nouveaux cas.

Spontaneous spleen rupture during chronic pancreatitis is scarcely seen. About 2 personal cases, the authors study 15 published cases. The spleen break can occur in patients with a known chronic pancreatitis, or with pancreatic signs, or without any symptomatology. Diagnosis is hard: spontaneous spleen break often looks like an acute pancreatitis. The high frequency of associated left pleural effusion is significant. Arteriography is helpful when it is done. Splenectomy, often too late, is the right treatment.

[Late ileal metastases from a skin melanoma with mainly hemorrhagic symptoms. Report of one case (author's transl)]. / Métastases iléales tardives d'un mélanome cutané périphérique. Manifestations hémorragiques prédominantes. A propos d'un cas.

Intestinal metastase from a malignant melanoma are rare and raise difficult diagnostic problems especially when far from the initial tumour. Although acute intussusception is the usual clinical presentation, one should recognise the possibility of atypical symptoms: e.g. repeated digestive hemorrhage or resistant anemia. A sutdy of past history may lead to the discovery of a malanoma. Treatment is unfortunately surgical and palliative to releave the complication.