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.

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.

Listeria monocytogenes brain abscesses in a girl with acute lymphoblastic leukaemia after late central nervous system relapse.

A case of Listeria monocytogenes bacteraemia and meningitis with intracerebral abscesses in a girl with acute lymphoblastic leukaemia in relapse is reported. The clinical features included subacute onset with fever and marked irritability followed by seizures, meningism and confusion. The pathogen was isolated from blood and cerebrospinal fluid. Computerised tomography of the brain showed two intracerebral parenchymal localisations, in the left frontal lobe and in the right occipital lobe, respectively. The patient survived this severe infection without neurological sequelae. 2 months later she underwent allogeneic bone marrow transplantation without major complications. This case report should alert pediatric oncologists about the possible occurrence of severe intracerebral listerial infections in the immunocompromised child and suggests that this infection can be treated successfully and should not necessarily preclude continuation of antineoplastic treatments.

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.

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.

["Porcelain gallbladder"; a pediatric case report]. / La "colecisti a porcellana"; contributo di un caso in età pediatrica.

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.

[Retroperitoneal teratoma. Description of a case]. / Teratoma retroperitoneale. Descrizione di un caso.

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.

[Axial computed tomography in anorectal malformations: a pre- and postoperative indication?]. / Tomografia axial computerizada en las malformaciones anorectales: una indicacion pre y postoperatoria?

Ten patients with anorectal malformations were studied by preoperative CT: well developed sphincteric muscular structures (puborectal sling of levator ani, muscular striated complex and external sphincter) were present in six patients. In three patients muscular structures were poorly developed (case 2a with rectovaginal fistula, case 5a with rectocloacal fistula and case 7a with prostatic fistula). In one case with bladder fistula, the sphincteric musculature was not identified at all. Eleven patients were postoperative studied by pelvic CT: Two of 11 cases had a good sphincteric control. The other nine patients were incontinent. In three of the nine incontinent patients, the colon was appropriately place, but puborectal muscle and sphincteric complex were hypoplastic. In the other six cases CT showed a pulled through colon out of the sphincteric structures. Four of them presented major bony sacral abnormalities and/or lesion of the sphincteric muscle structures. The remaining two patients with an evident pulled-through colon out of a normally developed sphincteric region and without sacral anomalies, were selected for a secondary PSARP procedure. In one of these two patients the operation brought about a complete resolution of the fecal incontinence. The pelvic CT is a useful examination in the anorectal malformation for the preoperative prognostic evaluation of continence, while postoperative CT is essential in selecting those incontinent patients who need a secondary PSARP for the treatment of fecal incontinence.

[An association between anorectal malformations and Down's syndrome]. / Associazione tra malformazioni anorettali e sindrome di Down.

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.

[2 cases of osteomyelitis in acute leukemia in the induction phase of treatment]. / Due casi di osteomielite in fase di induzione di leucemia acuta.

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.

[Ewing's sarcoma in preschool children. Description of 6 cases]. / Sarcoma di Ewing in età prescolare. Descrizione di 6 casi.

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.

[Advantages of a permanent venous access in children treated for cancer. Preliminary results]. / Vantaggi di un accesso venoso permanente in bambini trattati per malattie oncologiche. Risultati preliminari.

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)

[Neuroblastoma. Tumor rupture as an unfavorable prognostic factor]. / Neuroblastoma. Rottura tumorale come fattore prognosticamente sfavorevole.

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.

[Unusual pelvic pathology treated with posterior sagittal anorectoplasty]. / Patologia pelvica rara trattata secondo i principi della PSARP.

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.

[Renal changes in acute leukemia in children at onset. Incidence and prognostic value]. / Alterazioni renali nella leucemia acuta del bambino all'esordio. Incidenza e valore prognostico.

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.

[Borderline between the normal and the pathological in the radiological diagnosis of the posterior male urethra and female urethra in childhood]. / Limiti tra il normale ed il patologico nella diagnostica radiologica dell'uretra posteriore maschile e dell'uretra femminile in età pediatrica.

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.

MRI of hepatic focal nodular hyperplasia: a report of two new cases in the pediatric age group.

At present, magnetic resonance is a useful modality for the diagnostic assessment of focal nodular hyperplasia of the liver. In the pediatric age group, diagnosis for this kind of pathology is more effective and conclusive when performed by MR, because of the limited variety and the restricted expression of primary hepatic tumors. MR diagnosis is based on the presence of a central connective tissue that is scar hyperintense on T2-weighted sequences, which can be regarded as a typical finding, though not specific. The other criteria used to differentiate it from primary malignant tumors on MRI are the homogeneity of the tissue surrounding the scar and its signal intensity, which is similar to that of adjacent normal hepatic parenchyma.

[The radiologic picture of total colonic aganglionosis]. / Quadro radiologico dell'aganglia totale del colon.

The radiological findings pathognomonic of Hirschsprung's disease have been known for quite a long time. However, in addition to those diseases characterized by rectum or rectum-sigmoid aganglionosis (as in the classic form of Hirschsprung's disease), in pediatric pathology of surgical interest severe affections exist characterized by aganglionosis extending from the rectum to the entire colon, and exceptionally as far as the small bowel (ultra-long Hirschsprung's disease). The authors report on the results they obtained in radiological studies of 6 cases of total colonic aganglionosis by means of the current radiological procedures, i.e. plain abdominal films and barium enema. As for total colonic aganglionosis, no diagnostic radiological parameters have been assessed yet. The authors compare their results with those taken from international literature: microcolon with reflux into mega-ileum (2 out of 6 cases) appears to be the most suggestive X-ray finding for total colonic aganglionosis. Finally, the role is discussed of radiological studies in the diagnostic approach to total colonic aganglionosis. The authors conclude that, even though radiological investigations are indispensable, they are often not conclusive. Therefore, an accurate evaluation of the proximal extension of aganglionosis can be made only at surgery.