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Biomedical subjects

M Oddone

Publications and source records attributed to M Oddone.

At least 19 recordsLinked to original sources

Life-threatening hemorrhage from a vulvar hemangioma.

Capillary hemangioma, the most common tumor of infancy, usually does not require any treatment, but in a minority of cases it may cause morphologic, functional, or life-threatening complications. The authors report a case of a vulvar emangioma complicated by life-threatening hemorrhage not responsive to corticosteroids therapy, which showed no signs of spontaneous involution. The therapeutic approach consisted of 3 steps of selective arterial embolizations followed 5 months later by surgical excision and reconstruction of the vulva. Selective embolization rarely is used in the treatment of hemangiomas but may be required in cases of intractable bleeding, severe heart failure, large and poorly involuting hemangiomas, and as preparation for surgery. The results of the combined approach in this case are reported, and the indications discussed.

Adrenal Cortex Hormones↗

Long-term follow-up of two children with a variant of mild autosomal recessive osteopetrosis undergoing bone marrow transplantation.

Malignant autosomal recessive (AR) osteopetrosis represents an absolute indication for bone marrow transplantation (BMT). Over the last 15 years, almost 100 BMTs for osteopetrosis have been reported. The median age at transplant of most patients is 4 months. Very few cases of mild AR osteopetrosis have been described. Here, we report the good outcome of two cases of mild AR osteopetrosis with a follow-up of 5 and 6 years, respectively, after an HLA-identical sibling transplant undergone at 5 and 12 years of age, respectively. At the time of BMT, severe visual impairment was present in both children. Bone biopsy demonstrated hypermineralization with virtual obliteration of the medullary spaces, rare microfoci of hematopoiesis and marked deficiency in osteoclastic activity. Successful engraftment was complicated by hypercalcemia, controlled by a combination of bisphosphonate, phosphate infusions, vigorous hydration and calcitonin. Following BMT, radiological and histological findings showed extensive bone resorption with marked augmentation of the osteoclasts in normalized marrow. No improvement was observed in visual acuity, despite complete remodeling of skeletal abnormalities. We conclude that allogeneic BMT is the only chance of curing mild AR osteopetrosis.

Biopsy↗

Testicular microlithiasis: US findings in six pediatric cases and literature review.

The aim of this article is to report on six pediatric cases of testicular microlithiasis (TM) and to review literature reports, in order to schedule US and/or other control examinations, particularly when concomitant focal or diffuse alterations of the testicular parenchymal structure are present, considering the possible association of TM with testicular tumors. Six patients (age range 4-12 years) underwent US examination for scrotal trauma (two cases) unilateral cryptorchidism (one case) follow-up after orchidopexy for bilateral cryptorchidism (one case), and varicocele (two cases). Five examinations were performed with high-frequency probes (10/13 MHz) and seven with 5/7.5-MHz frequency transducers. Follow-up US examinations were performed at different times depending on initial clinical indications, presence of underlying disease, and initial US findings. Two of the six patients underwent three US examinations, two patients underwent two US examinations, and the remaining two patients underwent only one US examination. The patients underwent a total of 12 US examinations. Microliths were bilateral in four patients and unilateral in two patients. In these two latter cases, the contralateral testis was, in one case, cryptorchid and could not be evaluated by US; in the other case it was small and hyperechogenic with orchidopexy sequelae. In three cases microliths were distributed throughout the testis. In the remaining three cases they were present in limited areas of parenchyma. As to the importance of microliths, it was defined as mild in three cases and moderate/severe in three cases. Intratubular testicular microlithiasis is a well-proved histological finding (biopsy or autopsy). More recent is the US demonstration of TM with consequent definition of its pattern: usually bilateral hyperechogenic multiple small foci without acoustic shadows with complete or partial extension to the parenchyma. Testicular microlithiasis is a rare finding. Moreover, the pediatric cases reported in the literature are very few. However, the use of high-frequency US transducers (10-13 MHz) has recently allowed an easier demonstration of this disease also in children. Of particular interest is the study of the still-debated association of microliths with other diseases such as neoplasms. Some aspects need further investigation, namely the real incidence of microliths in the healthy population, the incidence of tumors in patients with microliths, the differences between adults and children, and the different types of follow-up at different ages. In pediatric age, if TM represents an isolated sign, patients need non-invasive US follow-up until adult age. Only if TM is in association with focal lesions of testis parenchyma is it mandatory to perform biopsy or surgical treatment.

Child↗

Diagnosis of neonatal hemochromatosis with MR imaging and duplex Doppler sonography.

Neonatal hemochromatosis is a rare congenital disorder which affects both fetuses and newborns. It is characterized by hepatocellular failure, often appearing on the first day of life in the form of coagulopathy, hypoalbuminemia, hypoglycemia, and jaundice. Most of the affected infants die early in life, and definitive diagnosis has often been made only by post-mortem evaluation. With the help of MRI, plus increasing awareness of the disorder, diagnosis is now often made early, even in utero. Duplex Doppler sonography does not provide information on siderosis but shows abnormalities in the liver or blood-flow patterns associated with liver disease.

Biopsy↗

Severe endobronchial obstruction in a girl with relapsing polychondritis: treatment with Nd YAG laser and endobronchial silicon stent.

Relapsing polychondritis (RP) is an uncommon disorder of unknown aetiology characterized by inflammation and destruction of the cartilaginous structures of many organs, including the tracheobronchial tree. When untreated, there is a high mortality rate, usually from respiratory obstructive complications. An 8 year old white girl, with a previous diagnosis of RP, was referred to our department for evaluation of worsening dyspnoea. Bronchoscopy showed localized inflammatory and fibrotic alterations of the mucosa, leading to severe obstruction of the left mainstem bronchus at its origin. The condition was successfully treated by endoscopic neodymium yttrium aluminium garnet (Nd YAG) laser. Re-evaluation of the patient, 7 months later, demonstrated bronchial stenosis and malacia requiring mechanical dilatation and positioning of an endobronchial silicon stent, which was well-tolerated by the patient.

Airway Obstruction↗

MRI "road-map" of normal age-related bone marrow. I. Cranial bone and spine.

We retrospectively reviewed 733 cranial and 250 spinal T1-weighted MR images of patients younger than 24 years to evaluate the bone marrow changes. The signal intensity of the bone marrow on short-TR/TE images was compared with that of fat and normal muscles in the contiguous region and graded. The signal intensity of all anatomic segments was as low as that of muscle, or inferior, in all patients younger than 3 months because of hematopoietic tissue and probably greater amounts of trabecular bone. The first anatomic segments of cranial bone to become hyperintense were the zygomatic bone and mandibular symphysis, followed by the presphenoid bone, basisphenoid, basiocciput, calvaria, and the petrous apex. After 3 years of age, most patients demonstrated pneumatization of the sphenoid sinus. We describe the most interesting changes in the developing spine, which occur in the first 2 years of life. The morphology of the vertebral bodies was evaluated. The variability of the signal and the morphology of the disks were also evaluated. Regional patterns of bone marrow signal intensity and age-related differences should not be misinterpreted as a pathologic condition.

Adolescent↗

MRI "road-map" of normal age-related bone marrow. II. Thorax, pelvis and extremities.

We retrospectively reviewed T1-weighted MR images of 381 patients aged from 7 days to 24 years to evaluate the bone marrow change in thoracic wall and shoulder, pelvis and proximal femur and upper and lower extremities. The patients included in the study were without history of bone marrow disease. A grade of from 1 to 4 was assigned to the marrow signal intensity of the examined anatomic segments. The signal intensity of all anatomic segments was as low as or lower than that of muscle in all patients younger than 2 months, reflecting underlying hematopoietic marrow. The first segments to become hyperintense were the epiphyseal/round bone ossification centers, followed by the phalanges, diaphysis, flat bones and metaphysis. Marrow signal intensity increased in all regions with age. While in the epiphysis, round bones and diaphysis bone marrow shows a diffuse and homogeneous increased signal intensity with age, in the sternum, ribs, scapulae, posterior ilium and metaphysis varying percentages of intermediate signal intensity are maintained. An orderly progression of red to yellow marrow was established.

Adolescent↗

Wilms' tumor arising in a multicystic kidney.

We describe a child with Wilms' tumor (WT) which occurred in an unequivocal multicystic dysplastic kidney (MDK). We indicate a current nonsurgical approach to MDK, but stress the probable malignant degeneration of blastemal cells, the need to search carefully for the WT in the MDK, and the necessity on 3-monthly follow-up US studies.

Follow-Up Studies↗

[Diagnostic imaging of acute leukemia in childhood. Description of 5 cases with unusual onset].

The authors, after reviewing the main features of the so called "leukemic osteopathy", remark how the natural history of leukoses has changed thanks to progress in both diagnosis and treatment. Indeed, the condition is now diagnosed early and its remission is quick. Thus, the cases with X-ray-evident bone involvement are quite uncommon: hence the need to use, at AL onset, imaging techniques which are more sensitive to bone marrow changes. To this purpose, MR imaging has proved a valuable technique which can demonstrate even the early stages of pathologic conditions affecting bone marrow.

Acute Disease↗

Relapsing polychondritis in childhood: a rare observation studied by CT and MRI.

Relapsing polychondritis is very rare in children. The diagnosis must be based on a combination of clinical and pathologic features. CT is very useful for an accurate and rapid assessment of laryngo-tracheo-bronchial involvement and the typical finding is lumen narrowing by wall thickening and collapse of the supporting cartilaginous structures. The role of MR imaging should be complementary to CT.

Child↗

Distribution of radionuclides in the environment in northern Italy after the Chernobyl accident.

Soon after the Chernobyl nuclear accident, the air-pumping stations in Pavia (northern Italy) were alerted. In a few days, a rapid increase in radionuclide concentration in air particulates was observed. Consequently, an environmental radioactivity monitoring programme was started in which several matrices such as soil, grass, vegetables and cows' milk were subjected to direct gamma-ray spectrometry. The radioactivity distribution and its variation with time is presented, discussed and compared with other available data. Detection limits, precision and accuracy are also reported, and depth profiles in soils for 137Cs are presented and correlated with soil quality parameters. A survey of environmental radioactivity in soil, in a search for residual Chernobyl fallout, was carried out and a map of the 137Cs distribution over a large area in northern Italy is presented and discussed.

Accidents↗

[Blocked transcolostomy colonography in the identification of anorectal malformations].

The study of anorectal malformations has considerably improved over the last years also thanks to the use of such diagnostic techniques as CT and MR imaging, which allow a better knowledge of perineal region anatomy and the demonstration of the development of sphincteric structures. The most important diagnostic phase for the identification of anorectal malformations consists in the detection of rectal fistulas. The authors report on their experience with barred water-soluble contrast enema in the study of anorectal anomalies. Over 2 years, 23 patients with anorectal malformations (10 males and 13 females) previously submitted to colostomy, were studied with this method. Fistulas were detected in 21 cases; in the extant 2 patients (both females affected with Down syndrome), no fistulas were detected, not even at surgery, which was posterior sagittal anorectoplasty according to Peña-De Vries. In our experience, the most frequent anomalies were recto-bulbo-urethral fistula in males and low recto-vaginal fistula in females. The authors suggest the use of this method as a routine diagnostic examination, because of its proven reliability.

Anal Canal↗

[Epilepsy with laughing seizures, hypothalamic hamartoma and precocious puberty. Contributions of MRI, computed EEG topography (CET) and ambulatory EEG (A-EEG)].

The concurrence of gelastic (laughing) seizures, hypothalamic hamartoma and precocious puberty constitutes a well defined epileptic syndrome in children; moreover mental retardation, neuropsychological deterioration and behavioral disorders have been often observed in these patients. In two cases we studied by means of MRI the appearance and the site of the hamartoma (in the posterior part of the hypothalamus with extension toward the third ventricle). The EEG study was performed by means of repeated recordings, of Computed EEG Topography (CET) and of Ambulatory EEG (A-EEG): in both patients during interictal periods paroxysmal EEG discharges prevailing in temporal or fronto-temporal regions and slight abnormalities of the background activity in the same areas were detected. Laughing seizures were recorded in each patient particularly by means of A-EEG: in case 1 bursts of high-voltage activity ("theta" waves) followed by depression of the background rhythm and by irregular spike discharges located in left temporal region were observed; in patient 2 irregular generalised spike discharges followed by slow waves or by depression of the background activity were seen. The presence of local abnormalities in both patients can support the hypothesis that the cortex, especially of the temporal anterior lobe, is involved in the origin of the laughing seizures. The significance of the mechanisms of secondary generalization as regards the seriousness of the epilepsy and of the mental impairment in these patients is also suggested.

Brain Mapping↗