[Polyimpaction or a 3d dentition? A report of an unusual clinical case].
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Biomedical subjects
Publications and source records attributed to R Bianco.
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Experience in health education programmes for diabetics is reported. Such education is considered a priority since it is an essential aspect of treatment. Only education can prevent acute attacks and reduce the consequences of later diabetic complications. Elsewhere education programmes have been organised by Anti-diabetic Centres or group meetings. The present health education programme however was conducted at the Day Hospital. The programme was " personalized " to allow for personal control of the disease taking place in the patients' environment and attended not only by diabetics, but also by their families, subjects at risk and non-diabetics interested in this problem.
A newborn female with a phenotype intermediate between the Neu and the COFS syndromes is described. The hypothesis of the two conditions representing different degrees of severity of the same autosomal recessive mutation in the homozygote state is discussed.
We describe a 3-month-old female with a de novo interstitial deletion of the long arm of chromosome 1 (1q23-25). Clinical features are failure to thrive, psychomotor retardation, cleft lip and palate, short metacarpals, metatarsals and fingers and a severe congenital heart disease. The four previously reported patients with the same deletion share with ours the distinctive pattern of anomalies of the face and limbs; therefore, it seems now possible to delineate a proximal 1 q deletion syndrome.
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A 3-month-old male infant with clinical and radiological features of Kniest disease is reported. Additional findings were severe Pierre Robin syndrome and external hydrocephalus. The patient was retarded in mental and motor development. He died at 4 months of age from the complications of tracheostomy. The parents were both normal clinically and radiologically, thus the disease in the child was presumably due to a new mutation. The reported familial cases of Kniest disease suggest autosomal dominant inheritance. The differential diagnosis is discussed in detail.
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An 8-year-old girl with narrow nose, small mouth, maxillary hypoplasia, cleft palate, hypodontia and hypohidrosis is described. Her scalp hair was coarse, dry and wiry. Microscopic examination showed the hair to be twisted at irregular intervals on its long axis, as seen in pili torti. Her mother has the same features; as a child, she had identical hair and is now bald. Both mother and daughter display signs and symptoms of Rapp-Hodgkin's ectodermal dysplasia. The autosomal dominant inheritance of the disease is further supported by the findings in this family.
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We have developed an in vivo model for chronic evaluation of prosthetic heart valves using juvenile domestic sheep. This report summarizes the results of a study conducted to assess a new bileaflet prosthetic valve. Nine juvenile sheep underwent mitral valve replacement using standard cardiopulmonary bypass techniques including mild hemodilution, systemic hypothermia, and cold fibrillatory arrest. The average time on cardiopulmonary bypass was 57 min. There were no surgical or anesthetic complications. Two (22%) early deaths occurred due to prosthetic annular size disproportion (1) and preexisting pneumonitis (1); postmortem examination of both implanted devices revealed normal function. The remaining seven animals (78%) remained clinically well and underwent left and right heart catheterization, angiography, and sacrifice after the 150th postoperative day. Left ventriculograms demonstrated normal valve function in all cases. The average mitral transvalvular gradient, as determined by simultaneous pulmonary capillary wedge and left ventricular and diastolic pressure, was 5.4 mm Hg. Thus we have developed a new in vivo model that provides a successful model for chronic evaluation of prosthetic valves using a sheep model. There are several features contributing to the success of our model. First, to decrease the possibility of bacteremia and seeding of the prosthesis, a single incision is used and intraoperative monitoring lines minimized. Second, we use a short cardiopulmonary bypass run (range 52-62 min), with no period of ischemia. Third, fresh sheep blood is transfused immediately following bypass to prevent anemia. Fourth, gastric decompression is used to prevent ruminal bloating, with the resulting vena caval compression that decreases blood return while on bypass.
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A multicenter clinical trial has been fulfilled by 18 Paediatric Hospital Centers in the regions of Piedmont and Aosta Valley, on 139 cases, concerning the use of a new macrolide, miocamycin, in the therapy of various infective diseases, such as upper and lower respiratory tract infections, ear and nose, throat, teeth affections, minor surgery, some contagious diseases. The results of this trial show a good effectiveness of the drug, consisting in a rapid disappearance of the clinical symptoms, a high degree of systemic and gastroenteric tolerability, and very poor side effects.