PubMed Health⌕ Search

Biomedical subjects

D Di Bella

Publications and source records attributed to D Di Bella.

45 records · Page 3Linked to original sources

[Biliary lithiasis in thalassemia major].

The presence of cholelithiasis was diagnosed by ultrasonography in 10 patients with thalassemia major aged 16 to 33 years. Other 10 patients aged 7 to 19 years showed acalculous cholecystopathy. Serum liver enzymes and ferritin levels, as well as splenectomy do not influence significantly the production of gallstones. Significant differences were observed in the age of patients with gallstones when compared to subjects without gallstones or with acalculous cholecystopathy. Although, in the last years, the high transfusional regimen has decreased the incidence of cholelithiasis, the frequent liver disease could be a cause of acalculous cholecystopathy in younger thalassemic patients.

Adolescent↗

[Influence of transfusion regime on precocious fusion of the proximal humeral epiphysis in thalassemia major].

The precocious fusion of the proximal humeral epiphysis has been reported in adolescents with thalassemia major. We evaluated the incidence of this skeletal abnormality in two groups of patients characterized by a different timing of the hypertransfusional regimen (i.e., before or after 10 years of age). Precocious epiphyseal fusion was significantly more common among patients who started hypertransfusional regimen after 10 years of age. Starting hypertransfusional regimen since early life may prevent epiphyseal fusion as a result of a permanent block of marrow proliferation.

Adolescent↗

[A case of Hallermann-Streiff syndrome with rapidly fatal course].

The Hallermann-Streiff syndrome is a rare affection characterized by beaked nose, dyscephaly, hypotrichosis, cataracts, micrognathia and proportionate short stature. The most severe complication in the syndrome is respiratory embarrassment. Narrow air passage with abnormal glottic closure, prenatal growth deficiency, immunodeficiency and sometimes associated cardiovascular anomalies, can predispose these patients to pulmonary infections. The Authors describe a new-born with Hallermann-Streiff syndrome, who, after a brief period of obstructive apnea successfully treated with CPAP, developed a severe pulmonary infection that caused his death at the age of 61 days.

Age Factors↗

[Congenital pulmonary lymphangiectasia with chylothorax. Different evolution of 2 cases with severe neonatal respiratory distress].

The Authors report on 2 cases of congenital pulmonary lymphangiectasia with chylothorax and severe respiratory distress which were characterized by a different clinic course. One of the newborns, in fact, died after a few days of life, while the other has survived with the complete regression of chylothorax. The Authors also add some physiopathologic remarks about pulmonary lymphatic circulation, in order to focus the different evolution of the 2 cases and the pulmonary lymphangiectasia treatment.

Chylothorax↗

[Congenital hip dysplasia in Sicily].

The Authors have analyzed the results of the ultrasound exams of the hips made in the Department of Pediatric Radiology, of the Catania University between 1993-1996 on 2000 little patients aged from 0 to 7 months. The aim of the examination was to define the frequency of the congenital dysplasia of the hip among the population of South-East Sicily because in literature there are no sure data. After a keen analysis of these data one can affirm that the incidence of the congenital dysplasia of the hip among the population of South-East Sicily is of 2.55% with the overwhelming prevalence in the females (M/F = 1/5). This percentage is thus comparable to the national one, and this allow us to confirm the great importance of the mass screening to prevent the congenital dysplasia of the hip even in Sicily.

Female↗

[The VACTERL association: a report of a clinical case with hepatic cystic lymphangiectasis].

VACTERL association includes three or more of the following six anomalies: V (vertebral anomalies), A (anal atresia), C (cardiac abnormalities), TE (tracheo-esophageal fistula and/or esophageal atresia), R (reno-urinary anomalies) and L (limb defects). VACTERL cases are classified as "associated" when other than the typical six defects are present in the same infant, or "isolated" when they are not. We report a case of VACTERL association "associated" that presents an hepatic cystic lymphangiectasia that was never described before in literature. We also consider the most important factors involved in the aetiology of the typical anomalies.

Abnormalities, Multiple↗