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Biomedical subjects

G Porta

Publications and source records attributed to G Porta.

50 records · Page 3Linked to original sources
PubMed1987

Unexpected low prevalence of delta antibodies in the east Amazon region and São Paulo: evidence for regional differences in the epidemiology of delta hepatitis virus within Brazil.

Antibodies (anti-HD) to hepatitis delta virus (HDV) were tested by radioimmunoassay in 207 human serum samples from the eastern Amazon (states of Pará and Amapá) and São Paulo, Brazil. 42 Amazon HBsAg asymptomatic carriers were negative for anti-HD. 84 São Paulo HBsAg asymptomatic carriers were also negative. Among the 81 HBsAg patients from São Paulo with different liver diseases, only one had anti-HD. Liver biopsy of this chronic active hepatitis case was positive for HBsAg, HBcAg and HDAg in liver, by an immunoperoxidase technique. The low prevalence of HDV infections in São Paulo and eastern Amazon was unexpected and contrasts with the recent reports of high prevalence in the western Amazon region. Such regional differences emphasize the need for extensive and precise worldwide epidemiological studies of HDV.

Adult↗
PubMedDate not supplied

A new candidate region for the positional cloning of the XLP gene.

X-linked lymphoproliferative disease (XLP) is an inherited immunodeficiency characterised by selective susceptibility to Epstein-Barr virus and frequent association with malignant lymphomas chiefly located in the ileocecal region, liver, kidney and CNS. Taking advantage of a large bacterial clone contig, we obtained a genomic sequence of 197620 bp encompassing a deletion (XLP-D) of 116 kb in an XLP family, whose breakpoints were identified. The study of potential exons from this region in 40 unrelated XLP patients did not reveal any mutation. To define the critical region for XLP and investigate the role of the XLP-D deletion, detailed haplotypes in a region of approximately 20 cM were reconstructed in a total of 87 individuals from 7 families with recurrence of XLP. Two recombination events in a North American family and a new microdeletion (XLP-G) in an Italian family indicate that the XLP gene maps in the interval between DXS1001 and DXS8057, approximately 800 kb centromeric to the previously reported familial microdeletion XLP-D.

Base Sequence↗
PubMedDate not supplied

[Liver transplantation from living related donors]

OBJECTIVES: To present the experience with the first 12 living related liver transplants performed at Hospital Sírio-Libanês in São Paulo. METHODS: The donors were the fathers (6) and the mothers (6) with age ranging from 30 to 48 years. All candidates for donation were submitted to a full informed consent form, clinical and radiological evaluation and had blood withdrawn for autotransfusion. Recipient age ranged from 7 months to 10 years whereas recipient weight varied from 6.3 to 34 kg. Six patients were considered as high risk due to complications of advanced liver disease and were submitted to urgent transplantation. RESULTS: Mean donor hospital stay was 10 days with no mortality. Technical complications were observed in 4 recipients. Seven patients presented at least one episode of bacterial, viral or fungal infection. One or more biopsy proven rejection episodes were disclosed in 7 patients. Overall recipient survival was 67%, being 83% for elective cases and 50% for urgent cases. Long term follow up ranged from 8 to 25 months. Seven out of 8 survivors present excellent quality of life and normal liver function. The other patient is currently under reduced immunosuppression due to Epstein-Barr virus infection.CONCLUSIONS: These results demonstrate the safety and viability of living related liver transplantation which, in face of the current donor scarcity, should be considered as a valid option for the treatment of children with end stage liver disease.

Journal Article↗
PubMedDate not supplied

[Nutritional evaluation of children with cholestatic diseases].

OBJECTIVE: To characterize the type of malnutrition and to determine the usefulness of anthropometric indices in children with chronic liver disease (CLD). METHODS: 11 children (aged 5 - 105 mo) with CLD underwent anthropometric evaluation when they were clinically stable. The nutritional evaluation was made by the determination of Weight/Age (W/A), Height/Age (H/A) and Weight/Height (W/E) Z scores. The nutritional evaluation by Waterlow's method was also made. The fat and protein body deposits were estimated by triceps skinfold and midarm muscular circumference measurements. The analysis of the 24-hour recall was used to evaluate the quality and pattern of the feeding. RESULTS: The mean weight / age (W/A = -1.18) and height / age (H/A = - 1.26) Z scores were depressed under 1 SD, whereas mean weight / height (W/H) Z score was normal. The interpretation of the nutritional evaluation by Waterlow's method shows normal mean of the weight and almost normal mean of the height. Only three patients had normal triceps skinfold thickness (TSF) Z score, and the same occurred with five of them with the midarm muscular circumference. The quality and pattern of the feeding was adequate in only 4 patients. CONCLUSIONS: We conclude that chronic malnutrition is common in childhood CLD and that weight/height values underestimate the degree of acute malnutrition compared with TSF thickness, most likely because of the inflated patient weight caused by organomegaly. The reduction of the triceps skinfold thickness best reflected the nutritional impairment of the patients. The quality of feeding of the patients was mostly inadequate.

English Abstract↗
PubMedDate not supplied

Laparoscopic splenectomy for hematological disorders. Our experience in adult and pediatric patients.

Laparoscopic splenectomy has gained increasing acceptance in the surgical management of a variety of splenic disorders, in particular hematological diseases. In this series, we report our experience with 20 patients (male:female ratio of 4:16 with median age of 16 years, range 5-49 years) who underwent this procedure because of ITP in 9 cases, spherocytosis in 7 and Cooley disease, sickle cell anemia, dyserythropoietic and hemolytic anemia in one case each. The patient was placed in a supine position using a fourtrocars technique. We did not perform pre-operative splenic artery embolization in any case. Spleen lower pole and its posterolateral attachments were dissected first, using electrocautery and endoclips. Vascular hilar isolation was achieved with an EndoGIA stapler and the spleen was removed by morcelation within a retrieval bag (16 cases) or via a 4-5 cm left subcostal incision (4 cases). One patient required conversion to open technique (conversion rate 5 %), because of uncontrolled bleeding from splenic hilum. Mean operative time was 165 min (range 100-240 min), mean splenic size was 13.5 cm (range 11-20 cm), with weight ranging between 140 and 1060 g and estimated blood loss was 151 ml (75-280 ml). No patient required a blood transfusion. Median postoperative hospital stay was 4 days (range 3-8 days). Postoperative complications occurred in 2 patients (10%), with no mortality rate in this series. Regarding the low complication rate and the advantages of a small abdominal trauma in the postoperative period, such as less postoperative pain, faster hospital discharge and better cosmetic results, the laparoscopic approach for elective splenectomy in hematological disorders has a substantial benefit for the patient.

Adolescent↗
PubMedDate not supplied

[Liver transplantation in children: initial experience of the Instituto da Criança of the Hospital das Clínicas of Universidade de São Paulo].

The initial experience at the Instituto da Criança do Hospital das Clínicas--School of Medicine of São Paulo University with liver transplantation in children is presented. A staff experienced in the management of children, including pediatric surgeons, hepatologists, critical care specialists, anesthesiologists, and other has been joined to draw therapeutic protocols. Afterward, more than 100 experimental liver transplant were performed in animals of medium size (dogs and pigs). From September, 1989 to July 1991, 12 liver transplants were performed on 9 children (3 retransplants) ranging in age from 2.5 to 17 years, being 5 boys and 4 girls. The donors had been selected according to the ABO blood group and body weight. Just once a blood A+ recipient received a liver from a blood O+ donor. The regular postoperative immunosuppression consisted of triple therapy with cyclosporin, prednisone and azathioprine. The postoperative stay in the Intensive Care Unit ranged from 3 to 24 days, according to the necessity of ventilatory support. These was no intraoperative mortality, arterial or venous thromboses, or early biliary complications. The overall survival is 78% (7/9). Primary non-function of the graft was the cause of death in two of our children. Although the number of cases is still small our results are comparable to those of the best liver transplant centers in the world.

Adolescent↗
PubMedDate not supplied

[Echographic diagnosis of hip dysplasia in neonates. Reflections on two years' work at our ultrasonography center].

The paper evaluates the role of ultrasound examinations of the hip joint in neonates by comparing the results of a one-year screening study (non-selected group) with the findings in a selected group of neonates with a high risk of hip dysplasia. The introduction to the paper contains a general discussion of the clinical aspects of hip dysplasia and describes the main anatomical findings using echography.

Hip Dislocation, Congenital↗
PubMedDate not supplied

Chronic active hepatitis associated with anti-liver-kidney microsome antibody type 1.

A 3 years old girl presented with clinical feature of an acute hepatitis-like illness, with jaundice, hepatosplenomegaly, high alanine aminotransferase activity (ALT) and high gamma-globulin values. We were able to demonstrate high titre of anti-liver-kidney microsome antibodies type 1 (LKMA1) in the serum of this patient using immunofluorescence, ELISA (Enzyme-linked immunoabsorbent assay) and Western blot (WB) analysis. This observation together with the liver morphology and after excluding other possible causes of hepatitis established the diagnosis of chronic active hepatitis associated with anti-LKM1 antibody. Immunosuppressive therapy was therefore started immediately.

Autoantibodies↗
PubMedDate not supplied

[Computed tomography in the follow-up of patients nephrectomized for adenocarcinoma].

A retrospective study was carried out of 159 CT examinations acquired in 64 patients who underwent unilateral nephrectomy for renal-cell carcinoma (34 in the left-hand side and 30 in the right-hand side). Thirty-nine patients were examined twice in the three years following nephrectomy; 25 were examined three or more times over a 5-year period after nephrectomy. Acute or chronic inflammatory disease was demonstrated in 6 patients (4 abscesses were detected by means of clinico-radiologic signs and 2 by means of cytology after CT-guided needle biopsy). Twenty patients had local recurrences 2-7 cm phi: in 17 of them the diagnosis was confirmed by CT-guided percutaneous FNAB; 15 patients had liver metastases, in 3 of them associated with local recurrences; 26 patients were free of both local recurrences and distant metastases. The authors analyze the value of CT in the definition of post-nephrectomy anatomic alterations, in the early detection and staging of local recurrences, and in the differentiation between postoperative fibrosis and complications. Moreover, the value is stressed of CT-guided FNAB of suspicious lesions. Our study suggests the value of a methodical CT follow-up of asymptomatic post-nephrectomy patients. CT immediately after surgery is also recommended to serve as a baseline reference for subsequent examinations. In our series, CT was accurate in the early detection of both local recurrences and distant solitary metastases.

Adult↗