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Biomedical subjects

G Zacchello

Publications and source records attributed to G Zacchello.

89 records · Page 5Linked to original sources
PubMed1980

Primary intestinal lymphangiectasia. Comparison between endoscopic and radiological findings.

Two children with primary intestinal lymphangiectasia (PIL) are presented, both of whom had been prescribed an MCT supplemented diet low in long chain fatty acids. The hypoproteinemic edema improved in one patient, but persisted in the other. Following several years of dietary therapy, both subjects were re-evaluated, and a duodenoscopy and radiography of the digestive tract were performed. The duodenoscopic procedure evidenced two types of lymphangiectatic plaques on the surface of the duodenal mucosa: one form had a diameter of less than 1 mm, while the other exceeded 3 mm. The smaller lesions were seen in the child with the more favorable clinical course, whereas both types were observed in the other patient. Radiological examination disclosed the typical anomalies of intestinal lymphangiectasia only in the subject who had not responded to the dietary regimen. These results suggest that endoscopy affords a more precise assessment of the anatomic injury and could play an important role in formulating an accurate clinical diagnosis.

Child↗
PubMed1979

Nephrotic syndrome during treatment with alpha-mercaptopropionylglycine.

We report 2 cases of cystinuria in which a nephrotic syndrome developed during treatment with alpha-mercaptopropionylglycine. This syndrome resolved after withdrawal of the drug and it did not recur when the alpha-mercaptopropionylglycine was given again in lower doses. The hypothesis is made that the nephrotic syndrome was dose-related. The alpha-mercaptopropionylglycine must be used in doses less than 50 mg./kg per day with regular monitoring of 24-hour urinary protein in cystinuric children.

Amino Acids, Sulfur↗
PubMedDate not supplied

[Levels of myoglobin in umbilical cord blood of infants born by spontaneous labor and cesarean section].

The levels of myoglobin in the umbilical cord blood of 20 full-term infants were studied in relation to spontaneous vaginal delivery and elective cesarean section, using the Rapitex Myoglobin (Behring Institute, Marburg, FRG). The content of myoglobin in umbilical cord blood was similar and within normal limits (less than 50 micrograms/l). We conclude that semiquantitative methods are not useful at birth to detect rhabdomyolysis and myoglobinuria secondary to the stress of vaginal delivery.

Adult↗
PubMedDate not supplied

[Membranous nephropathy associated with chronic hepatitis caused by B virus].

We report 6 cases with membranous glomerulonephritis associated with chronic hepatitis B virus infection, who came under our observation for microscopic haematuria in 2 cases, haematuria and proteinuria in 1 case, and nephrotic syndrome in the others. At the examination all 6 children were found to have hepatomegaly with raised serum transaminase activity. All the patients were positive in the serum for HBsAg and anti-HBc, 3 were HBeAg and anti-HBe negative. Liver biopsy showed features of chronic hepatitis with moderate signs of activity. Renal biopsy was consistent with membranous glomerulonephritis in all patients. With a fluorescent antibody technic HBeAg was found to be deposited in diffuse granular fashion, along glomerular capillary walls, together with IgG, in 2 out of 3 cases stained, but no deposition of HBsAg was detected in all the patients. Steroid therapy was started from 18 to 33 months. Urine analysis became negative in 4 cases and persisted normal in the follow-up. One patient with haematuria developed nephrotic syndrome and one died from miliary tuberculosis. Our findings suggest that the clinical outcome is favourable in children with membranous glomerulonephritis and chronic active hepatitis and that the pathogenesis probably is not unique.

Child↗
PubMedDate not supplied

[Medullary sponge kidney. Description of a pediatric case].

The case of a 11 year old boy with medullary sponge Kidney and nephrolithiasis discovered because of abdominal pain is described. Functional tubular impairment (concentrating defect, distal tubular acidosis) was present. No hypercalciuria nor hyperparathyroidism was detected. The diagnosis of medullary sponge Kidney was confirmed histopathologically. The pediatric cases described in the literature are characterized by a higher incidence of concentrating defect and acidosis, while the adults subjects often show hypercalciuria and or hyperparathyroidism. The careful study of tubular functions in the pediatric cases appears to be very useful to understand which are primary tubular disturbances and which are only secondary.

Abdomen, Acute↗
PubMedDate not supplied

[Diagnostic and therapeutic protocol in malformative uropathies prenatally diagnosed].

The increased frequency of prenatal detection of urinary tract abnormalities has brought to the attention of the pediatrician a high number of neonates and infants with asymptomatic hydronephrosis, which represents the most common form of uropathy. As it becomes clear that hydronephrosis does not necessarily mean obstruction, the purpose of the evaluation is to identify those infants in whom hydronephrosis will cause progressive renal deterioration, because of an ongoing restriction to urinary outflow. In the present paper, the discussion of the post-natal diagnosis and management focuses on the embryologic background, the pathophysiology of the obstruction and the rationale to utilise the different diagnostic techniques (ultrasonography, associated with the use of the Doppler; intravenous urography; avoiding cystourethrography; renal scintigraphy).

Clinical Protocols↗
PubMedDate not supplied

[Chronic renal failure secondary to vesicoureteral reflux: is the prevention possible?].

Vesicoureteral reflux (VUR) is the commonest cause of end-stage renal failure reported by the ITALKID (Italian Register of Chronic Renal Failure) (CRF) in children (0-15 years). Herein we report 131 children (53 M and 78 F) with a diagnosis of VUR made in our Centre between 1987 and 1994. Diagnosis was made in 69% of cases (85/131) during the 1st year of life and in 31% of cases (47/131) afterwards. VUR was demonstrated following a prenatal ultrasonography suspicion in 24/131 and after symptoms such as urinary tract infection (94/131) and poor growth (6/131). 75/131 of patients were treated conservatively (continuous chemoprophylaxis) while 56/131 underwent an antireflux surgery. CRF was found in 14% (19/131) of cases even though the diagnosis of VUR was performed in most of the cases (79%) during the 1st year of life. In conclusion prenatal ultrasonography has a low sensibility in the diagnosis of VUR. In addition, an early diagnosis made during the 1st year of life do not prevent CRF. The study was carried out in a Centre where patients with urological problems are referred from a large part of Northern East of Italy and this may explain the high prevalence of CRF and of children receiving a surgical treatment.

Adolescent↗
PubMedDate not supplied

Renal immunohistochemical distribution, plasma levels and urinary excretion of endothelin after kidney transplantation.

We investigated the immunohistochemical distribution of endothelin (ET) in 22 graft biopsies from kidney-transplanted patients. Like normal kidney tissue, 6/22 biopsies showed either no or only very weak ET positivity in the vascular endothelium. In the other 16 cases ET staining was marked on the vascular endothelium and wall (9/16), inflammatory infiltrates (11/16), glomeruli (7/16) and tubules (5/16). ET positivity in glomeruli and inflammatory infiltrates correlated with the degree of glomerular damage and interstitial inflammation but no correlation was found between the immunohistochemical results and the clinical variables considered. ET plasma levels (ET-PL) in patients (5.13 + 1.77 pmol/L) did not differ significantly from age-matched healthy controls (3.76 + 0.93 pmol/L), nor did ET urinary excretion (ET-U/CR-U) (33.94 + 21.89 and 24.94 + 8.5 pmol/mmol/L respectively). Neither ET-PL nor ET-U/CR-U was correlated with histological and immunohistochemical data or with the clinical variables. Our study suggests a potential role of ET as a local pro-inflammatory and growth factor in renal allografts and confirms its importance in the sequence of events involved in the progression of kidney damage.

Adolescent↗
PubMedDate not supplied

The Italian Registry of Pediatric Chronic Peritoneal Dialysis: a ten-year experience with chronic peritoneal dialysis catheters.

OBJECTIVE: To analyze the data from 347 peritoneal catheters implanted in 249 pediatric patients aged < or = 15 years at start of chronic peritoneal dialysis (CPD). DESIGN: Restrospective study of the data collected between 1986 and 1995, in 20 dialysis centers, from the Italian Registry of Pediatric Chronic Peritoneal Dialysis. Data collection for each pediatric catheter included: catheter type, site and technique of insertion, complications, duration, and reason for removal or replacement. RESULTS: Fifty catheters were inserted in patients under 2 years of age, 50 in patients aged 2 - 5 years and 247 in patients over 5 years of age. Catheter types included 307 (88.5%) Tenckhoff (286 double cuff, 21 single cuff) and 40 (11.5%), double-cuff, Valli-type catheters. All catheters were surgically implanted and omentectomy was performed in 83.5% of cases; the entry-site was in the midline in 136 cases (39.2%) and paramedian in 211 (60.8%). During 6076 CPD months we observed 274 catheter-related complications: 182 catheter infections (exit-site and/or tunnel infection), 23 leakages, 19 obstructions, 19 cuff-extrusions, 14 dislocations, 6 hemoperitoneum, 10 other (incidence of one complication every 21.8 dialysis-months). A significant reduction of catheter-related complications occurred in the last five years, compared with the first 5 years. One hundred and six catheters were removed due to catheter-related causes: infection (83 cases), obstruction (11), dislocation (4), outer-cuff extrusion (3), leakage (2), bowel incarceration (2), and bowel infarction (1). Catheter survival was 72.2% at 12 months, 52.3% at 24 months, 32.8% at 36 months, and 25.7% at 48 months. Significantly lower catheter survival was found in younger children (0 - 2 years) compared with two other age groups (2 - 5 years, and > 5 years). No significant correlation was found between catheter survival and catheter entry-site (midline vs paramedian). CONCLUSIONS: Catheter-related infections were confirmed to be the most common complication and most frequent cause of peritoneal catheter removal. In addition, catheter survival rate was worse in younger children, indicating that more effort should be made to improve peritoneal catheter survival particularly in this age group.

Adolescent↗