Compensatory renal growth in children with unilateral renal tumor treated by nephron-sparing surgery or nephrectomy.

INTRODUCTION: In children with an unilateral renal tumor, nephron-sparing surgery (NSS) results in a more adequate renal function adaptation compared to nephrectomy. In the present study, we investigated whether nephron-sparing surgery is followed by a different renal structure adaptation compared to nephrectomy. METHODS: Sixteen patients with unilateral renal tumor treated by nephrectomy (Group 1) and 10 treated by nephron-sparing surgery (Group 2) were enrolled in the study. Kidney volume was estimated by ultrasonography, using the formula for a prolate ellipsoid. Kidney volume was adjusted to the patient's weight and kidney laterality and expressed as a percentage of the expected volume of two kidneys in a healthy child. Total kidney volume (TKV) corresponded to the volume of the contralateral kidney in Group 1 patients, and to the volume of contralateral kidney + kidney remnant in Group 2 patients. Renal function was evaluated by serum creatinine values adjusted for sex and age and expressed as standard deviation scores (SDS). RESULTS: Group 2 patients presented with a greater indexed TKV compared to Group 1 patients, (97.4 +/- 18.8 % vs. 77.0 +/- 17.7 %; p = 0.005). Indexed TKV below the reference range for healthy controls with two kidneys was found in 4 of 10 Group 2 patients vs. 14 of 16 Group 1 patients (p = 0.017). In both patient groups, correlation analysis of indexed TKV and creatinine SDS showed a negative correlation (r = - 0.47; p = 0.01). CONCLUSION: In children with unilateral renal tumor, NSS is followed by a more adequate compensatory restoration of TKV compared with nephrectomy.

Gastric emptying time in cyclic vomiting syndrome in children.

Pathogenesis of cyclic vomiting syndrome (CVS) is still uncertain. Aim of our study has been to verify the role of gastric emptying time in children affected by CVS. We studied 9 children with CVS who underwent measurement of gastric emptying time by real-time ultrasonography after administration of a mixed, solid-liquid meal. In all the children gastric emptying time resulted in normal range. Our data suggest that mobility abnormalities of the stomach don't play a key role in the pathogenesis of CVS.

Role of radiography and ultrasonography in the diagnosis of the pediatric gastro-esophageal reflux disease.

Twenty-four hour esophageal pH-monitoring is gold standard for evaluate pathological GERD. Role of radiography and ultrasonography in the diagnosis of gastro-esophageal reflux disease (GERD) has been studied. Our results have been shown that radiography and ultrasonography have a limited role in the diagnosis of pathological GERD. However, such investigations an useful the follow-up of patients affected by pathological GERD.

The role of MRI in the intestinal complications in cystic fibrosis.

Fibrosing colonopathy is a complication recently detected in children with cystic fibrosis (CF), and is thought to be associated with the use of high-strength pancreatic enzymes. The goal of this study was to evaluate the effectiveness of magnetic resonance (MR) in detecting possible pathologic gut findings in patients with CF under pancreatic enzyme treatment. Twenty-five patients with CF and pancreatic insufficiency, all under treatment with high-dose pancreatic enzymes, were studied by MR. MR was performed on a 1.5-T magnet by T1-weighted, 2D-FLASH fat-suppression, breath-hold sequences before and after intravenous administration of gadolinium, and by T2-weighted Turbo Spin-Echo (TSE) and Half-Fourier Acquisition Single-Shot Turbo Spin Echo (HASTE) fat-suppression, breath-hold sequences. A superparamagnetic negative oral contrast agent was given 1.5 h before the examination. MR showed a wall thickening of the terminal ileum and the ascending colon (>4-12 mm) in 22 patients; nine of them (wall thickness >4-6 mm) showed both a moderate hyperintensity of the bowel wall on T2-weighted sequences and an enhancement after intravenous gadolinium on T1-weighted sequences; 13 patients (wall thickness >6-12 mm) showed both a great wall enhancement after intravenous gadolinium and an increased signal intensity of the bowel wall on T2-weighted sequences. Fecal impaction without bowel wall involvement was detected in three patients. MR proved to be a useful, noninvasive, diagnostic tool for the evaluation of patients with CF and fibrosing colonopathy. The signal hyperintensity on T2-weighted sequences and the great wall enhancement after intravenous gadolinium administration indicating an acute edematous condition, provide the clinicians useful information for the therapeutic adjustment.

Chronic granulomatous disease with gastric antral narrowing: a study and follow-up by MRI.

We report the case of a boy with chronic septic granulomatosis (CSG) who presented with a marked gastric antrum narrowing which was evaluated by MRI after oral intake of magnetic contrast particles, and after gadolinium i.v. administration. In particular, a mammillated aspect of the gastric wall in the antral region was seen. Follow-up by MRI clearly showed the gradual resolution of hyperemic wall thickness, after medical management. The antral stenosis resolved after 3 months. Magnetic resonance imaging provides detailed evaluation of gastric wall inflammation in course of CSG.

Respiratory status of infants with esophageal atresia.

In infants with esophageal atresia (EA), lung opacities on a chest radiograph (CXR) are usually considered the cause of respiratory distress (RD). However, in some instances signs of RD and CXR changes show no correlation. The aim of this study was to investigate the pathogenesis of RD in EA patients with a normal CXR. In 41 infants with EA, CXR findings were correlated with clinical manifestations and blood-gas analysis data. The degree of abnormal gas exchange was quantitated by the arterial/alveolar oxygen tension ratio (a-ARO2). Of the 41 infants, 39(95%) presented with RD. No lung opacities were found in 130 of 294 CXRs examined (44%). An a-ARO2 below 0.75 (lower limit of normal) was calculated in 215 of 247 arterial blood samples analyzed (87%). When a temporal correlation was established, RD with a clear CXR was characterized by signs of extra- and intrathoracic airway obstruction, often associated with an a-ARO2 below 0.75. The degree of hypoxemia was greater than the degree of hypercapnia. We conclude that in infants with EA, RD with a clear CXR is related to both tracheomalacia and upper-airway obstruction that may cause miliary atelectasis not detected by conventional CXR with intrapulmonary shunting and hypoxemia.

Integrated diagnostic imaging of primary thoracic rhabdomyosarcoma.

We report a rare case of primary thoracic rhabdomyosarcoma in a girl who was referred with acute chest pain, hacking cough, and wheezing. A chest X-ray revealed a complete opacity of the right hemithorax. Ultrasound revealed a right-sided pleural effusion and a solid mass above the liver dome, suggesting a neoplastic disease, which quickly led to further specific examination. Use of CT and MRI together with bone scintigraphy completed the investigation. The biopsy specimen showed a pattern of alveolar rhabdomyosarcoma. This case was reported to emphasize the role of US in the evaluation of a child with hemithorax opacity.

[Bilateral intravesical ureteroceles associated with hydroureteronephrosis and hyperechogenic spots in kidneys. Report of a neonatal case]. / Ureterocele intravescicale bilaterale associato ad idroureteronefrosi e spots iperecogeni renali. Descrizione di un caso ad esordio neonatale.

Ureterocele is a cystic dilatation of the terminal ureter. In children it is most commonly ectopic and associated with the duplex collecting system, less frequently it is intravesical and associated with the single collecting system. We present the radiological and ultrasound findings in a male newborn with bilateral intravesical ureterocele associated with hydroureteronephrosis and hyperechogenic spots in both kidneys. In the literature this association has never been reported in neonatal age.

Procalcitonin as a marker of nosocomial infections in the neonatal intensive care unit.

OBJECTIVE: To determine accuracy of procalcitonin concentrations for diagnosing nosocomial infections in critically ill neonates. DESIGN: Case-control study. SETTING: Neonatal intensive care unit of a teaching hospital. PATIENTS: Twenty-three neonates with nosocomial infection. Four controls matched for duration of hospital stay and birth date were chosen for each case patient. MEASUREMENTS AND RESULTS: PCT concentrations were measured by the LUMItest procalcitonin kit at onset of signs of infection and after recovery. Range of PCT concentrations (ng/ml) was 2.0 to 249.1 in case patients and 0.08 to 1.0 in controls (sensitivity and specificity, 100%). PCT values returned to normal (<1.0 ng/ml) by day 3 to 7 of appropriate antibiotic therapy. CONCLUSIONS: Measurement of PCT concentrations may be useful for early diagnosis and monitoring of infectious complications in neonates during their stay in the neonatal intensive care unit.

Adult height in girls with central precocious puberty treated with gonadotropin-releasing hormone analogues and growth hormone.

GnRH analogues (GnRHa) represent the treatment of choice in central precocious puberty (CPP), because arresting pubertal development and reducing either growth velocity (GV) or bone maturation (BA) should improve adult height. However, in some patients, GV decrease is so remarkable that it impairs predicted adult height (PAH); and therefore, the addition of GH is suggested. Out of twenty subjects with idiopathic CPP (treated with GnRHa depot-triptorelin, at a dose of 100 microg/kg im every 21 days, for at least 2-3 yr), whose GV fall below the 25th percentile for chronological age, 10 received, in addition to GnRHa, GH at a dose of 0.3 mg/kg x week s.c., 6 days weekly, for 2-4 yr; and 10 matched for BA, chronological age, and duration of GnRHa treatment, who showed the same growth pattern but refused GH treatment, served to evaluate the efficacy of GH addition. No patient showed classical GH deficiency. Both groups discontinued treatment at a comparable BA (mean +/- SEM): 13.2 +/- 0.2 in GnRHa plus GH vs. 13.0 +/- 0.1 yr in the control group. At the conclusion of the study, all the patients had achieved adult height. Adult height was considered to be attained when the growth during the preceding year was less than 1 cm, with a BA of over 15 yr. Patients of the group treated with GH plus GnRHa showed an adult height significantly higher (P < 0.001) than pretreatment PAH (160.6 +/- 1.3 vs. 152.7 +/- 1.7 cm). Target height (TH) was significantly exceeded. The group treated with GnRH alone reached an adult height not significantly higher than pretreatment PAH (157.1 +/- 2.5 vs. 155.5 +/- 1.9 cm). TH was just reached but not significantly exceeded. The gain in centimeters obtained, calculated between pretreatment PAH and final height, was 7.9 +/- 1.1 cm in patients treated with GH combined with GnRHa; whereas in patients treated with GnRHa alone, the gain was just 1.6 +/- 1.2 cm (P = 0.001). Furthermore, no side effects have been observed either on bone age progression or ovarian cyst appearance and the gynecological follow-up in the GH-treated patients (in comparison with those treated with GnRHa alone). In conclusion, a gain of 7.9 cm in adult height represents a significant improvement, which justifies the addition of GH for 2-3 yr during the conventional treatment with GnRHa, especially in patients with CPP, and a decrease in GV so marked as to impair PAH, not allowing it to reach even the third centile.

Crossed renal ectopia with fusion.

The case of three-year old girl with right to left crossed renal ectopia with fusion is described. This malformation presented only microhematuria and urinary tract infections. The diagnosis was established by means of biochemical and radiographic investigations.

Delayed gastric emptying in an infant with Sandifer syndrome.

The case of an infant with Sandifer syndrome is reported. Real-time ultrasonography showed delayed gastric emptying time, which returned to normal when the patient was asymptomatic. The importance of gastric motility investigations in Sandifer syndrome is stressed since delayed gastric emptying could play a role in the pathogenesis of this disease.

Ultrasound in the management of hepatic veno-occlusive disease in three children treated with dactinomycin and vincristine.

Three children, treated with dactinomycin and vincristine without radiotherapy showed hepatic toxicity consistent with diagnostic criteria for hepatic veno-occlusive disease (VOD). Two patients were affected with Wilms' tumor and the third with malignant fibromatosis. The clinical manifestations of VOD were mild and regressed in all patients after supportive therapy. Serial ultrasonography (US) was performed in all cases and was useful in confirming the diagnosis and in evaluating the severity of the disease. US features of VOD were hepatomegaly, gallbladder wall thickening, ill-defined borders of the hepatic vessels, and ascites. Parenchymal heterogeneity was still present after several months as a result of the hepatic injury. The hazy appearance of the portal vessels and the gallbladder wall thickening seemed directly correlated with the degree of hepatic involvement in the early phase of VOD.

Enucleative surgery for stage I nephroblastoma with a normal contralateral kidney.

PURPOSE: Tumor enucleation is not recommended for children with nephroblastoma and a normal contralateral kidney. However, in adults with unilateral low stage renal cell carcinoma tumor enucleation may offer an alternative to radical nephrectomy, since functioning renal tissue is preserved without a greater risk of residual microscopic disease. Enucleative surgery may be more reasonable in children with nephroblastoma, because the risk of relapse can be reduced with chemotherapy. Therefore, we prospectively evaluated the feasibility of enucleative surgery in children with stage I unilateral nephroblastoma. MATERIALS AND METHODS: Between 1992 and 1995, 13 children with nephroblastoma and a normal contralateral kidney were consecutively admitted to our surgical unit. Possible candidates for tumor enucleation were evaluated according to certain criteria, including stage I disease at diagnosis, well-defined margins on post-contrast computerized tomography and at least 50% of the functioning kidney could be preserved. Preoperative and postoperative chemotherapy was given in all cases. RESULTS: Of 4 children with preservation of more than 50% of the functioning kidney 3 were considered eligible for enucleation. The tumors, which were confined to the mid kidney in 2 children and upper renal pole in 1, were successfully enucleated without hypothermia or vascular occlusion. All 3 children are disease-free at 49, 48 and 26 months of followup, respectively. Renal function has been almost completely restored postoperatively. CONCLUSIONS: These preliminary data suggest that enucleative surgery may be a reasonable option in select children with stage I nephroblastoma and a normal contralateral kidney.

[Spondylitis in infancy. A case report]. / Spondilodiscite in età pediatrica. Presentazione di un caso.

The authors report on a case of a spondylodiskitis in three year old child. Diskitis is an inflammatory process of the intervertebral disk space, not often described in pediatric age. The etiology is infective and generally presents with lumbar spine localization. Clinically it is characterized by lumbar pain at times radiating to a lower extremity. Symptoms are aspecific and radiological signs do not appear for several weeks after the onset of symptoms. Interest in this pathology derives from the fact that it may be more common than previously recognized. This case offered us the opportunity to review principal symptoms of diskitis and to describe the main diagnostic technics used.

Primary pulmonary rhabdomyosarcoma in childhood: clinico-biologic features in two cases with review of the literature.

The cases of two children under three years of age with primary pulmonary rhabdomyosarcoma and no associated lung malformations are reported and a review of the literature is presented. In both, complete surgical removal of the tumor was performed and histologic examination revealed embryonal subtype. Flow cytometric assessment showed a tumor-cell diploid DNA content. Postoperative radio- and chemotherapy were carried out, but in spite of treatment both girls died because of disease progression, fourteen and nine months after diagnosis. The importance of associated cystic lung malformations and DNA content in predicting clinical outcome of primary pulmonary rhabdomyosarcoma is evaluated.

[The interpretation of a negative intradermal reaction to tuberculin in a case of tuberculous lymphadenitis]. / Interpretazione di una intradermoreazione alla tubercolina negativa in un caso di linfadenite tubercolare.

This work aims at describing a particular case of tuberculous lymphadenitis with negative tuberculin skin test in a five-year child who has afterwards developed Hodgkin disease. BK presence is initially demonstrated through the examination of a first bioptic sample of a lymph-node, then confirmed by the presence in the serum of mycobacterial antigens on the peripheral lymphocytes and monocytes. Nine months later the second bioptic sample puts in evidence the presence of Reed-Sternberg cells. The follow-up of our patient took place at the Pediatric Department of the University of Rome "La Sapienza" and lasted an overall period of twenty days. The child was hospitalized twice and underwent a large number of radiological and laboratory tests. The therapy consisted only of medical specific treatment. Our study results lead us to evaluate the presence of immunological suppressive mechanisms in some particular tuberculosis cases, justifying the tuberculin skin-test negativity. When Hodgkin's disease has turned up, it has been too difficult to outline a precise temporal sequence of events, we could only advance hypothesis.

[Budd-Chiari syndrome in children: case report]. / Sindrome di Budd-Chiari in età pediatrica. Descrizione di un caso.

This work aims at describing a particular case of Budd-Chiari syndrome in a ten-year-old girl. In our patient, the subjective and objective features of this disease, which is already rare during childhood, are associated with a well-assessed polyposis in the blind intestine (intestinum caecum). In our opinion, a possible connection between these two pathological events, a connection unheard of in the literature so far, is an interesting issue to be dealt with in this case report. The follow-up of our patient took place at the Pediatric Hospital of the University of Rome "La Sapienza" and lasted an overall period of 6 months. The girl was hospitalized twice and she underwent a large number of radiological and laboratory tests. The therapy consisted only of medical treatment and support measures. Liver transplant is regarded as the only chance to obtain a full recovery, but it has not yet been performed on our patient. It is by comparing those researches carried out on similar cases with the outcome of our personal study, that we come to the conclusion that, as for our patient, the Budd-Chiari syndrome can be classified as an idiopathic syndrome. Moreover, the fact that this syndrome and the polyposis of caecum occur at the same time in a patient can probably be considered as a coincidence.