Phenotypic and pathologic evaluation of the myd mouse. A candidate model for facioscapulohumeral dystrophy.

Facioscapulohumeral dystrophy (FSHD) is an autosomal dominant disease of unknown pathogenesis which is characterized by weakness of the face and shoulder girdle. It is associated with a sensorineural hearing loss which may be subclinical. FSHD has been mapped to the distal most portion of 4q35, although the gene has not yet been identified. Distal 4q has homology with a region of mouse chromosome 8 to which a mouse mutant, myodystrophy (myd), has been mapped. Muscle from homozygotes for the myd mutation appears dystrophic, showing degenerating and regenerating fibers, inflammatory infiltrates, central nuclei, and variation in fiber size. Brainstem auditory evoked potentials reveal a sensorineural hearing loss in myd homozygotes. Based on the homologous genetic map locations, and the phenotypic syndrome of dystrophic muscle with sensorineural hearing loss, we suggest that myd represents an animal model for the human disease FSHD.

Laparoscopic resection of an abdominal wall desmoid using a modified suture traction technique: the "marionette trick".

Desmoids are rare mesenchimal tumors that may originate also inside the abdomen or in the abdominal wall. These tumors are biologically characterized by a tendency to local growth, and only rarely are they able to develop distant metastases. Surgical excision usually is the best treatment with a chance of a cure. In the few reports on intraabdominal or abdominal wall desmoids, open surgery always was performed. The first case of successful laparoscopic resection of a symptomatic anterior wall desmoid tumor with intraabdominal growth is reported. During the procedure, it was difficult to mobilize and grasp the mass using the common laparoscopic instruments, but with the help of the "marionette trick," modified suture traction technique, the tumor could be removed easily using only three trocars. With four traction sutures minimizing the wall trauma, the trick made it possible to mobilize the mass in at least, seven directions, according to the principles of physical forces and vectors. This simple trick can be helpful for other common laparoscopic procedures, avoiding the insertion of sometimes ineffective instruments through more traumatic trocars.

[Significance of prognostic parameters in acute pancreatitis]. / Significato degli indici prognostici nella pancreatite acuta.

The diagnostic and therapeutic approach to Acute Pancreatitis (A.P.) is directly related to the clinical presentation. The Authors reviewed the data of 66 patients, hospitalized between October 1989 and December 1991, to verify the effectiveness of the prognostic criteria suggested by Ranson (1974), Mercadier (1977) and Imrie (1978). A.P. was of biliary origin in the majority of the patients (63.5%); five patients (7.5%) had an acute alcoholic pancreatitis, while the aetiology was traumatic or unknown in the remaining cases. A complicated clinical course was defined by the development of pseudocyst, pancreatic abscess, digestive haemorrhage, death or prolonged hospitalization (more than 20 days). The 28.8% of the patients developed complications during hospitalization. There were seven pancreatic pseudocysts, six pulmonary complications, three renal insufficiencies, two vascular complications, two sepsies and a gastrointestinal haemorrhage. The mean hospitalization period was 15.1 days (range 1-112). The Authors conclude that the three different prognostic criteria are equally useful to test the severity of A.P. attacks allowing to identify patients with the higher risk to develop complications during hospitalization.