[Rare case of mucopolysaccharidosis 1. Clinical observations].
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Biomedical subjects
Publications and source records attributed to A Morabito.
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The importance of perinatal infection in the mother-foetus transmission of HbsAg positive hepatitis is stressed. The role of type A hepatitis has no influence and that of the NANB is little known. The Authors maintain that in no case is the hepatitis virus a valid motive for the interruption of pregnancy, even when it occurs in the first months of gestation.
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The Authors report their experience with 738 children observed and operated for minor diseases in the period January 1989-June 1993. The children were hospitalized for day surgery or one day surgery. After some surgical, anaesthesiological, and postoperative pathophysiological considerations, and on the basis of their results (reduced local and general postoperative complications), the Authors confirm the benefit of this type of organization in terms of reduced nosocomial infections, reduced psychological traumas and increased socio-economic advantages.
A case of retroperitoneal Hemangiopericytoma in a 9 year old boy is presented. Hemangiopericytoma is a rare soft tissue tumor with unpredictable biological behaviour and a high local recurrency rate. Its ubiquity, the different grades of malignancy, the lack of correlation between clinical and histological feature are discussed. It is emphasized the hard preoperative diagnosis of the painless "mass", that can be reached only by histology. Increased cellularity, prominent mitotic activity, and foci of necrosis or hemorrhage suggest a malignant form rather than a benign one. Classifying Hemangiopericytoma among mesenchymal tumors, radical surgery is the treatment of choice whenever possible, associated with pre or post operative chemotherapy according to National Protocol RMS 88.
The Authors after a review of the embriology, physiology and pathophysiology of diaphragm, report three cases of congenital or acquired diaphragmatical hernias. They discuss advantages and disadvantages of different surgical techniques on the basis of their technical experience as well as results obtained.
The Authors reviewed their series of 28 consecutive elderly patients operated on for gastric cancer in the Division of General and Oncological Surgery--Department of Surgery and Surgical Emergencies of the University of Perugia from January 1993 to December 1994. Thirteen subtotal gastrectomies associated with resection of the nearby organs in 5 cases, six total gastrectomies, two gastrojejunal bypass, and seven atypical resections of the middle third of the stomach were performed. The Authors focus their attention on this atypical technique pointing out the advantages compared to other procedures as well as criteria for patients selection. Gastric resection of the middle third seems a worthwhile procedure for well selected elderly patients, resulting in a good quality of life with low morbidity and mortality.
The operative treatment of thyroid pathology has to be distinguished for benign and malignant pathology. The major complications associated with thyroid surgery are injury to the recurrent laryngeal nerve and hypoparathyroidism. Postoperative hypoparathyroidism is rarely the result of inadvertent removal of all parathyroid glands but, non commonly, is due to disruption of their blood supply. Postoperative hypoparathyroidism results in patients with symptomatic hypocalcemia, transitory and permanent hypoparathyroidism. Subtotal thyroidectomy is used for the treatment of benign thyroid pathology while tumors are treated by total thyroidectomy with neck dissection.
The operative management of patients with hyperparathyroidism is controversial. High rates of persistent hypercalcemia and postoperative hypoparathyroidism are seen in multiple hyperplasia and bilateral neck exploration. Patients undergoing unilateral neck exploration with removal of a single parathyroid adenoma have a rapid clearance of PTH which declines within hours after surgery. There is a sensitive immunometric assay (IRMA) for the intact molecule which demonstrates a decline in 15 minutes during surgery. Intraoperative measurement of PTH may be complementary to surgical skill and histopathologic information and may modulate extension of neck exploration.