[Diagnostic considerations on an unusual case of thoracic ectopia of the right kidney].
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Female↗
Biomedical subjects
A Taccone
Publications and source records attributed to A Taccone.
["Porcelain gallbladder"; a pediatric case report].
A case of "porcelain gall-bladder" observed by change in a boy of 14 is reported. After discussing the anatomopathological and aetiological aspects of the syndrome, stress is laid on its exceptional nature in paediatric age and the possibility of a correlation wtih Giardiasis is examined.
Adolescent↗
Computed tomography in maple syrup urine disease.
Maple Syrup Urine Disease (MSUD) is an inherited metabolic disorder characterized by a severe, usually lethal, neonatal course in the early stages with pseudotumor cerebri and pathologically documented increased cerebral water content. CT and MRI studies in MSUD are few and the data are overlapping. This study reports CT features before and after dietary treatment in three patients; two with classical MSUD and one with an intermediate variant of MSUD. At diagnosis, CT consistently showed evidence of abnormally high lucidity involving not only white matter, but also areas of grey matter, particularly the pallidum. Furthermore, these CT changes are present both in the acute phase of classical MSUD and in an intermediate variant of the disease. The observed abnormalities evolve favorably under dietary treatment, simultaneously with clinical and neurological improvement. It is concluded that the observed CT changes indicate a diagnosis of MSUD and are relevant findings in the neuroradiologic differential diagnosis in acutely ill newborns, in which a metabolic disease may be not immediately suspected.
Humans↗
[An association between anorectal malformations and Down's syndrome].
Down's syndrome is the most frequent chromosomal anomaly in humans and sometimes is associated with anorectal anomalies. The anorectal malformations include many varieties of anatomical anomalies, which are often difficult to evaluate. The Authors believe preoperative CT or MRI of the pelvis, together with other clinical and radiological examination to be a valid mean in the preoperative prognostic evaluation. In this study they analysed the association of anorectal malformations and Down's syndrome and the absence of a genito-urinary or perineal fistula.
Anal Canal↗
[Neuroblastoma. Tumor rupture as an unfavorable prognostic factor].
Since there is little information regarding the possible prognostic significance of tumor rupture in localized neuroblastoma, we have analyzed the clinical courses of 163 children registered from 1979-1990 in 12 italian pediatric oncology Centers participating in the Neuroblastoma Cooperative Group of the A.I.E.O.P. (Italian Association for Paediatric Haematology-Oncology). Ten instances (6%) of tumor rupture were described. Ruptures occurred preoperatively in one child, during the operation in 9; among these 9, two were provoked by the surgeon to allow radical tumor excision, 7 were accidental. Of these 10 children, 7 relapsed at 3-25 months (median, 8 months) from diagnosis. Relapses were local in 5 children (2 of the 5 died), disseminated in one (who died), local + disseminated in one (presently alive with disease). Two local relapses were followed by bony or haematologic spread at 4 and 8 months, respectively. Of the 7 children who relapsed, 2 are alive in complete remission at 29, 100 months, respectively; two are alive with disease at 3 and 65 months, 3 died at 8, 15 and 24 months, respectively. We conclude that rupture of a localized neuroblastoma is a factor predisposing to relapse and may compromise the chance of cure. The surgeon should be aware of the risks connected with this complication and make any effort to avoid it.
Abdominal Neoplasms↗
Hepatic and splenic mycosis in children with acute leukemia.
Three cases of hepatic and/or splenic mycosis in children with acute leukemia are reported. Patients presented with fever not responding to broad spectrum antibiotics during or after prolonged and profound neutropenia. Noteworthy, in 1 case no abnormality in liver function tests was detectable at diagnosis. CT scan showed focal hepatic lesions in all patients. In 1 patient ultrasounds failed to detect hepatic lesions and showed only splenic lesions, while CT scan detected hepatic and not splenic lesions. Definitive diagnosis was performed by open liver biopsy and histological demonstration of fungal invasion. Cultures were negative in all cases. All patients survived with prolonged amphotericin B treatment (from 36 to 40 mg/kg), combined with flucytosine in two cases. In conclusion, this clinical entity should be taken into account by physicians taking care of neutropenic patients, even in the absence of abnormalities in liver function. CT seems to be the method of choice for diagnosis, although ultrasounds might be useful for detecting concomitant splenic lesions.
Adolescent↗
[Computerized tomography in pulmonary cystic fibrosis].
This study was aimed at evaluating CT sensitivity in identifying the signs of pulmonary cystic fibrosis (CF). The chests of 39 patients (16 males and 23 females, mean age 19.1 years) were examined by CT: all patients had been given a clinical score according to Schwachman and Kulckzycki criteria. Thickened bronchial walls were observed in all cases, which are typical of peribronchitis. Bronchiectases were present in 87% of cases; their extent, pattern and localization were exactly shown on CT scans. Bronchoceles were seen on CT scans in 64% of patients; less frequent was the finding of atelectases and subpleural bullous emphysema. In a great number of patients (64% and 82%, respectively) pleural thickening and hilar adenopathy were demonstrated on CT scans. In conclusion, our results confirm CT as a more sensitive method than conventional radiography to identify and locate the signs of pulmonary CF. The early identification of the lesions is of high prognostic value, since the early detection and treatment of bronchoceles may prevent permanent bronchiectasis.
Adolescent↗
[Unusual pelvic pathology treated with posterior sagittal anorectoplasty].
Posterior sagittal approach is very useful for the correction of anorectal malformations, but it can be used also for the treatment of pelvic or perineal masses. The authors present 3 patients affected one by a perineal hamartoma, one by a rectal duplication and one by a retrorectal cystic teratoma. All the patients were treated following the PSARP technique, using the Penã's bipolar electro-stimulator just in order to respect all the muscles of the anorectal sphincters. Using this procedure the authors were able to preserve the full anorectal continence in all the 3 patients.
Anus Neoplasms↗
[Current role of CT in pediatric ophthalmology].
In spite of the recent and substantial improvements in MR technique, some problems still exist relative to its applications in routine clinical exams in pediatric ophthalmology. The main problems are: inadequate MR equipment, long examination time, and MR inability to demonstrate intraocular calcifications. Ocular and orbital ultrasound (US) studies are highly operator-dependent, and US utility has been especially described in evaluating ocular, but not orbital, lesions. In order to verify the actual role of CT in pediatric ophthalmology, the CT scans of 58 children with ophthalmologic pathologies, performed over a 2-year period, were reviewed and compared with definitive diagnoses. Seven separate CT findings for each pathologic condition were independently analyzed and correlated with histology. In agreement with other CT series, optic nerve gliomas were invariably intraconal, whereas histiocytosis-X, Ewing's sarcoma, olfactory neuroblastoma (esthesioneuroblastoma), metastatic neuroblastoma and nephroblastoma were extra-conal. Rhabdomyosarcoma, principally extraconal, frequently involved the intraconal and preseptal spaces, with permeative destruction of the osseous orbit and frequent intra/extracranial spread. Orbital spread was mainly observed in vascular tumors. CT showed great accuracy in evaluating punctuate calcifications in retinoblastomas and in metastatic neuroblastomas, and bone fragments within a zone of destruction in histiocytosis-X. Various characteristics of CT attenuation values were observed in pathologic tissues, and high attenuation and marked contrast enhancement were particularly observed in metastatic neuroblastomas and rhabdomyosarcomas. In congenital orbital abnormalities and inflammatory diseases, CT readily detected ocular malformations (microphthalmos and colobomata).(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
Leigh disease: value of CT in presymptomatic patients and variability of the lesions with time.
Three cases of Leigh disease (subacute necrotizing encephalomyelopathy) have been investigated recently in our institute by CT. Bilateral, low attenuation areas were observed in the basal ganglia in all cases. These areas corresponded to the typical necrosis areas seen pathologically in this disease. In our study there were further interesting CT appearances: the peripheral enhancement of low-density zones and the discovery of the lesions before the clear onset of clinical symptoms.
Brain Diseases, Metabolic↗
[The hemophilic child. Post-traumatic cerebrospinal complications. Diagnostic protocol].
The authors report their experience on 30 hemophilic children controlled at the Pediatric Institute G. Gaslini, Genoa, Italy; among the 30 patients, there were 4 cases of hemophilia A, and 1 of Von Willebrand disease. Various degrees of trauma in different sites had caused central neurological complications. In all cases CT, both in the immediate post-traumatic phase and later on, allowed both an early diagnosis and the follow-up of hemorrhagic lesions and late complications, even in absence of significant neurologic symptomatology; medical replacement treatment and neurosurgery, when needed, allowed a positive resolution of all cases. The authors believe this early-phase and follow-up diagnostic protocol, together with strict clinical and laboratory controls, to allow a prompt interdisciplinary therapeutic approach, which has preventive aims as well.
Cerebral Hemorrhage↗
[Retroperitoneal teratoma. Description of a case].
The Authors describe a case of asymptomatic retroperitoneal teratoma discovered on a routine examination in a six year-old male. Diagnosis was made pre-operatively on the base of CT scan imaging. Surgery allowed radical excision of the teratoma, which turned to be a totally benign entity.
Child↗
[Ewing's sarcoma in preschool children. Description of 6 cases].
Ewing's sarcoma (ES) is rarely diagnosed in the pre-school age; these few cases however present a number of difficult diagnostic and therapeutic problems. The Authors describe a series of six such cases aged less than 4 years diagnosed in the period 1974-1987. Standard treatment was modified with the purpose to reduce acute toxicity and late sequelae. These modifications are described in details. Four/6 patients are presently alive disease-free from 29 to 87 months from diagnosis (median, 34 months). Two patients died from acute toxicity. The Authors suggest that young children with ES may be treated successfully with proper adjustments of current protocols. Increased aggressiveness of chemotherapy regimens may compensate for reduced radiotherapy; however, the high susceptibility to infection of youngsters has to be taken into account and makes mandatory a careful monitoring during phases of profound myelodepression.
Antineoplastic Combined Chemotherapy Protocols↗
[Advantages of a permanent venous access in children treated for cancer. Preliminary results].
Current therapy for children with cancer includes a variety of invasive procedures many of which require repeated venous access over a considerable period of time. Such procedures are poorly tolerated by children and by their veins. Recently it has become possible to undertake the majority of such procedures by means of permanent indwelling silastic catheters improving the quality of life of the children and their parents and increasing the scope of therapeutic intervention. In the period July '83 - August '84 we have used 46 of these catheters in 45 children with malignant disease, 12 with acute myeloid leukaemia, 12 with neuroblastoma, 7 with B cell leukaemia-lymphoma, 6 with rhabdomyosarcomas, 2 with Ewing's Sarcoma, 2 with Wilms' tumor and 1 case each of Hodgkin's disease, teratocarcinoma, osteosarcoma and juvenile chronic myeloid leukaemia. The children's ages ranged from 2 months to 14 years; 22 were male and 23 female. The catheters were inserted under general anaesthesia (duration 20-40 minutes) usually without difficulty, except for a single patient in whom no suitable vein could be found. No complications connected with the placement of the catheter were observed. Subsequent management of the catheter was initially complicated and time-consuming, but was subsequently simplified so that acceptance by parents, children and nursing staff was eventually excellent. The duration of use of 46 catheters ranges from 7 to 350+ days; 24 catheters are presently in use at 30-350+ days from insertion. Eight children died as a result of disease progression and two of sepsis with the catheter in place.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
[2 cases of osteomyelitis in acute leukemia in the induction phase of treatment].
Whereas children with Acute Leukemia are highly susceptible to infectious complications, the occurrence of acute osteomyelitis is extremely rare in these patients. The authors describe two such cases in children at onset of an acute lymphoblastic and of a myelomonocytic leukemia, respectively. In the former case, the clinical course has been characterized by the progressive involvement of several joints and bones. A citrobacter Freundii was isolated in the synovial fluid of an involved knee. This complication was successfully treated with proper antimicrobic agents and surgical toilet, while the patient was vigorously treated for her leukemia, achieving a complete remission. The latter case developed a right humerus osteomyelitis from an Enterobacter. The patient failed to respond to antibiotics, and his leukemia also turned refractory to antiblastic therapy. The difficulty in the differential diagnosis among the X-graphic aspects of leukemic, inflammatory and degenerative disease of bones are discussed by the authors. Some pathogenetic hypothesis of leukemic osteomyelitis are also presented.
Acute Disease↗
[Borderline between the normal and the pathological in the radiological diagnosis of the posterior male urethra and female urethra in childhood].
The authors, with the support of a large case report, point out several varieties of the standard urethrography of a normal urethra and the correlations between macroscopic anatomy, radiologic anatomy and physiology. They stress the importance of a good knowledge of the urethral anatomy and of the urethrographic physiology in order to avoid considering as pathologic a really normal radiologic finding.
Age Factors↗
[Renal changes in acute leukemia in children at onset. Incidence and prognostic value].
Microscopic leukemia infiltration of kidneys is a common autoptic finding in children for ALL before starting specific treatment. However, a palpable renal enlargement is uncommon. The authors have performed an intravenous pyelogram (IVP) in 139 pediatric cases of acute leukemia, 117 of whom ALL, the remaining 22 ANLL. ALL patients were divided in 3 groups; Group 1 was made of 18 children treated with IGG-74 protocol, independently by any prognostic factor; Group 2 included 46 patients presenting one or more negative factors; Group 3 was of 53 cases with no unfavourable factor. Abnormal IVP was found in 4/18 (22,2%) Group 1, 9/46 (19,5%) Group 2, 2/53 (3,8%) Group 3 ALL patients. The most common anomaly was a bilateral renal enlargement with normal or slightly compromised renal function. Only 3 out of 117 ALL children had palpable renal masses. All 15 children acquired CR within one month from starting therapy together with normalization of IVP. Six out of 15 of these cases died, one is alive with disease, 8 are alive in CR. Except for two cases, all presented other negative prognostic factor associated with abnormal IVP. One of 22 ANLL children had an abnormal IVP: only one kidney was involved with the unique mechanism of intraparenchimal hemorrhage and hydronephrosis due to the filling of renal pelvis and ureter by multiple coarse cloths. In conclusions, major renal alterations are present in about 10% of ALL children, rarely in ANLL. This finding is commonly associated with unfavorable prognostic factors.
Acute Disease↗