Renal tuberculosis: a case report.

Tuberculosis or TB (tubercle bacillus) remains a major public health problem in developing countries. Over the last decades extrapulmonary locations of the disease have become more frequent due to the increased prevalence of acquired immune deficiency syndrome and the increase number of organ transplants. The urogenital localization represents about 27% of all extra-pulmonary localizations of TB and may be due either to a disseminated infection or to a primitive genitourinary localization. The majority of patients, has pyuria, sometimes with hematuria. The diagnosis of urinary tuberculosis is based on the finding of pyuria in the absence of infection by common bacteria. The initial medical treatment includes isoniazide, rifampicin, pyrazinamide, ethambutol and streptomycin. This disease should be suspected in patients with unexplained urinary tract infections, especially if immunocompromised and/or coming from endemic areas.

Systemic lupus erythematosus patients with and without neuropsychiatric manifestations: a neck and transcranial duplex sonography study.

Neuropsychiatric manifestations are not rarely associated with systemic lupus erythematosus (SLE). Magnetic resonance angiography and positron emission tomography can provide excellent images of cerebral perfusion and metabolism whereas information is still lacking on a possible diagnostic role of ultrasound. In this study we aim to assess whether duplex sonography of neck and intracranial vessels may be useful in distinguishing patients with and without neuropsychiatric SLE (NPSLE). Neck and transcranial duplex sonography was performed by a single operator on 33 women affected by SLE (mean age +/- SD: 47.69+/-8.17 years) and on 15 healthy control subjects. Nineteen patients presented NPSLE. Pulsatility and resistivity indices (PI and RI) were automatically calculated by the ultrasound instrument in internal carotid (ICA) and middle cerebral artery (MCA), on both sides, according to standard methods. No significant haemodynamic differences were found in mean and median PI and RI values of ICA and MCA comparing SLE with NPSLE patients and with healthy control subjects. No correlation was found between MCA and ICA parameters in the same group of patients. Duplex sonography of cerebral vessels is unable to distinguish SLE and NPSLE patients. Heterogeneity of causes in the pathogenesis of NPSLE and the different vascular adaptation of cerebral macrocirculation as opposed to cerebral microcirculation may represent possible reasons that explain the inability of ultrasound to differentiate SLE patients from NPSLE patients.

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.

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.

Localized but unresectable neuroblastoma: treatment and outcome of 145 cases. Italian Cooperative Group for Neuroblastoma.

PURPOSE: To define factors that influence outcome in children with localized but unresectable neuroblastoma by retrospective investigation of response to therapy and outcome in 21 Italian institutions. PATIENTS AND METHODS: Of 145 assessable children diagnosed between 1979 and 1990, 77 were treated between 1979 and 1984 with three consecutive standard-dose (SD) protocols, and 68 between 1985 and 1990 with a high-dose (HD) protocol. All protocols included chemotherapy, followed by resection of primary tumor if feasible. If at least partial resection was achieved, consolidation therapy followed, except that from 1985 onward, patients considered disease-free following surgery received no further treatment. RESULTS: Ninety-four of 145 patients (65%) achieved a complete response (CR) or partial response (PR) with chemotherapy and 75 (52%) subsequently underwent complete resection of the primary tumor. Eighty-one patients are alive (73 without disease, eight with disease), 63 have died, and one is lost to follow-up. The 5-year overall survival (OS) rate is 55% and progression-free survival (PFS) rate 50%. Both OS and PFS correlated with response to chemotherapy, removal of primary tumor, HD therapy, and serum lactate dehydrogenase (LDH) levels. Infants (< 1 year), independent of primary tumor site, and children aged 1 to 15 years with a nonabdominal primary tumor, did better compared with children aged 1 to 15 years with an abdominal primary tumor (PFS, 72% and 64% v 30%; P < .001 and < .01, respectively). Outcome of this last group improved with the HD protocol (PFS, 40% v 23%; P = .01). CONCLUSION: In children with unresectable neuroblastoma, risk categories can be defined by age and primary tumor site. HD chemotherapy should be investigated for the poor-risk category age 1 to 15 years with an abdominal primary tumor.

Role of haematological, pulmonary and renal complications in the long-term prognosis of patients with lysinuric protein intolerance.

Three patients with lysinuric protein intolerance are reported. The first patient displayed severe haemolytic anaemia, bone marrow erythroblastophagocytosis, renal tubular disease and interstitial lung disease. Despite treatment with citrulline and low-protein diet, this child died at the age of 18 months. The second patient is now 24 years old and has chronic interstitial lung disease and focal renal glomerulosclerosis. The third patient, now 5 years old, has severe chronic interstitial lung disease. A 6-month treatment with prednisone was ineffective in the second and third patients.

[Diagnostic imaging of acute leukemia in childhood. Description of 5 cases with unusual onset]. / Diagnostica per immagini nella leucemia acuta dell'infanzia. Descrizione di 5 casi con esordio inusuale.

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.

CT of invasive pulmonary aspergillosis in children with cancer.

In treating cases of malignancy, the use of chemotherapy carries a high risk of lower respiratory tract infections, especially fungal pneumonopathy. This complication is a major cause of mortality and is often difficult to diagnose because of non-specific clinical or radiological changes, but the early recognition of invasive fungal disease is imperative. CT is an important non-invasive method for the detection and evaluation of opportunistic fungal infections. In these patients an improved survival rate can be achieved when early detection by CT leads to the prompt institution of high-dose antifungal therapy. We illustrate the spectrum of CT findings of invasive pulmonary aspergillosis encountered in children with cancer. These patients had previously been treated with high-dose chemotherapy with or without bone marrow rescue, and underwent radiological examinations because of clinical evidence of pneumonopathy. Representative cases demonstrate the clinical applications of CT in the evaluation and management of invasive fungal disease.

Vein of Galen aneurysm: MRI with a fast gradient refocusing pulse sequence.

A case of vein of Galen aneurysm, studied with a fast gradient refocusing pulse MRI sequence, is presented. Fast MRI sequences allowed the acquisition of additional hemodynamic information that was not available with either routine MRI or angiography.

Mucopolysaccharidosis: thickening of dura mater at the craniocervical junction and other CT/MRI findings.

Cranial CT and/or MRI imaging of 8 patients with mucopolysaccharidosis (MPS) was retrospectively evaluated. Two patients had MPS IH, 1 had MPS IS, 1 had MPS IVA and 4 had MPS IV. CT and MRI showed thickening of dura mater at the cranio-cervical junction, causing narrowing of the subarachnoid space, in all the patients examined. Spinal cord compression was detected in 4 patients. Other findings were: white matter alterations, mild to severe hydrocephalus, skull dysplasia and odontoid dysplasia. White matter alterations were evident as large areas and as multiple dispersed spots of prolonged T1 and T2 value. Reduced gray/white matter contrast was demonstrated on T2-weighted MRI images. It is important to examine the cranio-cervical junction carefully for thickening of dura mater in all patients with mucopolysaccharidosis examined by CT or MRI, because of the generally progressive clinical course of MPS. In patients with symptomatic cord compression, surgical intervention should be considered.

[High-resolution computerized tomography. Staging of lobular lesions in cystic fibrosis]. / Tomografia computerizzata ad alta risoluzione. Stadiazione delle lesioni lobulari nella fibrosi cistica.

Forty-four patients affected with cystic fibrosis (CF) were examined by means of high-resolution Computed Tomography (HRCT) of the chest; the sensitivity of this technique was evaluated in identifying small pulmonary structure alterations, and its role outlined. In all cases, HRCT scans allowed early lobular shape anomalies to be detected and secondary pulmonary lobular lesions to be correlated with disease evolution. A classification for lobular lesions was proposed: Stage I - Thickening of the bronchovascular bundle and/or intralobular septa and/or middle interstitial compartment. Unessential changes in lobular ventilation. Preservation of lobular shape. Stage II - Intralobular emphysema. Acinar filling with normal ventilation (A) or diffuse hypoventilation of the lobule (B). Preservation of lobular shape. Stage III - Lobular consolidations. Preservation of lobular shape. Stage IV - Patch distribution of lesions characterized by derangement of lobular structures. Changes in normal lobular shape. The identification of these lesions confirms HRCT as the most sensitive technique for the early visualization and localization of signs of CF bronchopneumonopathy. A broader experience coming from the systematic use of HRCT in chronic inflammatory lung diseases would increase our knowledge of pathogenetic processes and allow therapeutic perspectives to be improved.

High-resolution computed tomography in cystic fibrosis.

The sensitivity of high-resolution computed tomography (HRCT) in identifying the pulmonary lesions of cystic fibrosis (CF) was evaluated. Thirty-nine patients (16 males, 23 females; mean age 19.1 years) were examined by chest HRCT. According to Shwachman and Kulczycki criteria, the clinical score of the patients ranged from 40 to 95, thus covering most possible variations of lung disease severity. All the patients presented diffuse thickening of bronchial walls, expression of the characteristic CF bronchial inflammation. Bronchiectases were the second most common lung lesions: discrete dilatation of bronchi was observed in 87% of cases; the localization, pattern and extent of bronchiectasis were accurately detected by HRCT. Pleural thickening and hilar adenopathy were frequently identified (in 64% and 82% of the patients, respectively). Bronchoceles were seen in 64% of the patients; atelectasis (33%) and subpleural bullous dystrophic emphysema (28%) were observed less frequently. On HRCT, the localization of the disease processes within the secondary pulmonary lobule was possible in all patients. In agreement with international literature, the identification of these lesions confirms HRCT as the more sensitive technique for early visualization and location of the manifestations of CF bronchopathy. A larger range of experience coming from a systematic use of HRCT in chronic inflammatory lung diseases would increase our knowledge of pathogenetic processes and allow improvement of therapeutic perspectives.

[Mucopolysaccharidoses: evaluation of the cranium by computed tomography and magnetic resonance]. / Mucopolisaccaridosi: valutazione del cranio con tomografia computerizzata e risonanza magnetica.

Cranial CT and/or MRI examinations were performed in 8 patients affected with mucopolysaccharidosis. Two patients were affected with mucopolysaccharidosis IH,1 had mucopolysaccharidosis IS, 1 had mucopolysaccharidosis IV A and 4 presented mucopolysaccharidosis VI. CT and MRI showed white matter changes consisting of symmetric and diffuse hypodense areas on CT, large areas and multiple spots of prolonged T1 and T2 value on MRI. Reduced gray/white matter contrast was demonstrated on T2-weighted sequences. Furthermore, CT and MRI showed thickening of the dura mater at the cranio-cervical junction, which caused subarachnoid space narrowing in all patients. Spinal cord compression was detected in 4 patients. Additional findings were mild to severe hydrocephalus, skull dysplasia and odontoid dysplasia. Owing to the progressive clinical course of all mucopolysaccharidosis, the authors stress the importance of a careful study of the cranio-cervical junction in all the patients with mucopolysaccharidosis examined by CT or MRI in order to demonstrate essential thickening of the dura mater. In the patients with symptomatic cord compression, surgery should be considered.

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.