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P Gugliantini

Publications and source records attributed to P Gugliantini.

At least 19 recordsLinked to original sources
PubMed1999

Constitutional osteochondrodysplasias identifiable at birth. A short review on the state of the art in radiodiagnosis in the late 20th century.

INTRODUCTION: The value of a systematic radiologic analysis in constitutional osteochondrodysplasias remains underestimated by both neonatologists and radiologists. We report the clinical experience of the Department of Neonatology and Neonatal Intensive Care Unit of St. John Hospital in Rome with constitutional osteochondrodysplasias identifiable at birth. MATERIAL AND METHODS: We reviewed 2120 cases of newborns hospitalized in our unit from January 1996 to August 1998 and here submitted to at least one direct radiograph of chest and abdomen (the so-called "babygram"). All the newborns were clinically assisted by the same three neonatologists and radiologically followed by the only pediatric radiologist, the external consultant for diagnostic imaging of the Intensive Care Unit of the Neonatology Department. RESULTS, DISCUSSION AND CONCLUSIONS: We diagnosed 14 cases of constitutional osteochondrodysplasias (.66%): 4 of them (28.27%) belong to the group considered by the European Society of Pediatric Radiology (ESPR) as lethal before or immediately after birth, while the other 10 (71.43%) belong to different groups of the ESPR classification. Thus, we arbitrarily grouped them into a single pathologic condition, based on their two main features: being generally not lethal and always or very often identifiable at birth. These 10 cases were: 1 campomelic dysplasia, 3 achondroplasias, 2 asphyxiant thoracic dysplasias type Jeune, 1 cherubinic dysplasia, 2 osteogenesis imperfectae, 1 osteopetrosis. We justify the relatively high incidence of constitutional osteochondrodysplasias in our study (.66% versus an average incidence of .076% reported in the world population) on the basis of: a) an increasing number of high-risk newborns in our intensive care unit; b) an improvement in our clinical and radiologic diagnostic skills. We conclude that the state of the art of the diagnosis of constitutional osteochondrodysplasias is still based on the first plain X-ray examination performed at birth because of cardiorespiratory and/or abdominal diseases in the newborn.

Humans↗
PubMed1989

Mucolipidosis II: correlation between radiological features and histopathology of the bones.

Twelve cases of Mucolipidosis II (I-cell disease) with a wide range of severity of skeletal involvement were studied. Pathological findings in two cases provided helpful information in understanding the radiographic features of dysostosis multiplex. Inhibition of the growth plate cartilage calcification and rickets-like lesions were observed in the metaphyses. Enhanced subperiosteal remodelling and paratrabecular fibrosis were also evident in the diaphyses. High levels of parathormone were found in one case. This finding supports the hypothesis that bone lesions may be secondary, at least in part, to damage in such viscera as the kidney and/or the liver and that they are mediated by vitamin D and parathormone.

Bone and Bones↗
PubMed1989

[High-resolution computed tomography in the diagnosis of branchial otodysplasias. A report on 10 cases (15 ears)].

Computed Tomography (CT) is not yet widely employed in the evaluation of temporal bone diseases in pediatric patients. Our experience is reported in the study of branchial otodysplasias (10 cases, 15 ears) by means of a high-resolution program. Indications, advantages, limitations and risks of this technique are discussed. Some technical details are reported, such as number and type of scans, length of data acquisition, thickness and feed of slices, electronic image reconstruction, and radiation doses given to the lens. The method employed to immobilize younger patients is also described. CT findings in the single cases are summarized in the tables. The most significant images of 5 cases are also reproduced. A careful analysis of the cases in our series highlights the advantages provided by the use of high-resolution CT in the evaluation of the temporal bone in pediatric patients, especially as far as dysplasias are concerned.

Adolescent↗
PubMed1986

[Rib notchings in Cooley's anemia].

In 3 cases of Cooley's anemia rib notchings were found. In the first case the clinical pattern did not allow to exclude postductal type of aortic coarctation and thus was performed a venous digital aortography (SVC) showing the integrity of the aortic arch and the absence of intercostal arterial collateral circulation. Only two previous descriptions of rib notchings in thalassemia were found in literature, and the authors shortly describe the 3 observed cases and suggest some possible pathogenetic interpretations either of rib notchings in thalassemia or of the relatively late appearance (adolescence) of rib notchings.

Adolescent↗