Choledochal cyst: a 10-year experience

BACKGROUND: Choledochal cyst is a rare abnormality usually found in infancy and childhood and mainly in females. Outcome depends on early diagnosis, complete resection of the cyst and proper hepaticoenterostomy.OBJECTIVE: The aim of this paper is to evaluate the outcome of 18 patients operated in ten years. METHODS: Retrospective chart review. RESULTS AND DISCUSSION: Age ranged between 20 days and 13 years (mean 3 years). Fifteen (83%) were female and 03 (17%) male. Signs presented included 15 (83%) with jaundice, 11 (61%) with dark urine, 10(55%) with pale colored stools, 09 (50%) with abdominal pain and palpable mass in only 02(11%). Ultrasonography identified the cyst in 14 (77%) patients. Endoscopic retrograde cholangio-pancreatography demonstrated a long common duct in three of four cases. Anatomical types were: 09 type I, 01 type II, 07 type IV and 01 type V. Except for one patient who had a type V cyst, all patients underwent cyst resection plus Roux-en-Y hepaticojejunostomy. There were two (11%) early postoperative deaths related to deterioration of liver function. Thirteen (86%) of the 15 patients available to follow-up were symptom-free without late complications at a mean period of three years, and two (14%) patients developed chronic pancreatitis.     

Myasthenia gravis with the electrographic response of a myasthenic syndrome. Report of a case

It is reported a case of a 61 years-old man with progressive asthenia, disfagia, disphonia and diplopia, of variable intensity during the day, who had a very good response to anticolinesterasic drugs and corticosteroids. The repetitive stimulation tests at low frequency, resulted in large increment (maximum 275%) of the basal voltage after exercise. At high frequency he also had a large increment. Radiological and laboratory investigation three times in a seven-year period was normal, without evidence of any neoplasia. Muscle biopsy showed a type II muscle fiber atrophy. The repetitive stimulation tests repeated three times, was typical of myasthenia gravis in one occasion and in the other two, typical of myasthenic syndrome (increment of 418%). A discussion about other cases with similar findings is made after a review of the literature.     

Wilms' tumor of the uterus: the first report of the literature

The first case of primary Wilms' tumor of the uterus is described. It occurred in a 14-year-old girl. Microscopically, embryonal tubules, glomeruli, and blastema were identified within a myxomatous stroma. No teratomatous elements were found. The tumor arose in the uterus, permeated the endocervical canal, filled the vagina, and infiltrated the Douglas cul-de-sac and the left broad ligament through the posterior vaginal fornix. Comments are made on the histogenesis and histologic pattern of the tumor.     

Teneurin proteins possess a carboxy terminal sequence with neuromodulatory activity

We have previously shown that a bioactive neuropeptide-like sequence is present at the carboxy-terminus of the teneurin transmembrane proteins. We have subsequently called this peptide 'teneurin C-terminal associated peptide' (TCAP). The sequence encodes a peptide 40 or 41 amino acids long flanked by a cleavage motif on the amino terminus and an amidation motif on the carboxy terminus, characteristic of bioactive peptides. This sequence is highly conserved in all vertebrates. A TCAP-like sequence is encoded by each of the four teneurin genes. We have therefore examined the neurological role TCAP-1 may play in mice and rats. In situ hybridization studies showed that the teneurin-1 mRNA containing the TCAP-1 sequence is expressed in regions of the forebrain and limbic system regulating stress and anxiety. A synthetic version of amidated mouse/rat TCAP-1 was prepared by solid-phase synthesis and used to investigate the in vitro and in vivo activity. TCAP-1 induces a dose-dependent change in cAMP accumulation and MTT activity in immortalized mouse neurons. Administration of synthetic TCAP-1 into the basolateral amygdala significantly increases the acoustic startle response in low-anxiety rats and decreases the response in high-anxiety animals in a dose-dependent manner. When 30 pmol TCAP-1 is administered into the lateral ventricles each day for 5 days, the sensitization of the rats to the acoustic startle response is abolished. These data indicate that TCAP may possess functions that are independent of the teneurin proprotein and together, the teneurins and TCAP, may represent a novel system to regulate neuronal function and emotionality.     

Validation of a health survey questionnaire focusing on risk factors for coronary artery disease in a group of public employees in Juiz de Fora, Minas Gerais State, Brazil

The validity of a health survey questionnaire used in a family health promotion program to identify individuals at risk of developing coronary artery disease (CAD) was studied in a random sample of 297 State public employees in Juiz de Fora, Minas Gerais State, Brazil. The sample was stratified by sex, and subjects were 40 years of age or older. Results obtained in interviews were compared to laboratory and clinical data. We assessed the questionnaire's sensitivity, specificity, and positive and negative predictive values in relation to questions about current or previous history of diabetes, hypertension, smoking, and high cholesterol. Among individuals with at least one altered clinical or laboratory test, 81% (total sensitivity) were identified by the referred risk inquiry when reporting at least one risk factor. Sheffield and New Zealand tables were used to determine the individuals with increased risk of developing CAD and to assess if they would also be identified by the health survey questionnaire. The sensitivity for identifying this subgroup was 100%.     

Globin haplotypes of human T-cell lymphotropic virus type I-infected individuals in Salvador,Bahia, Brazil,suggest a post-Columbian African origin of this virus

The city of Salvador, Bahia, Brazil, has sociodemographic characteristics similar to some African cities. Up to now, it has had the highest prevalence of human T-cell lymphotropic virus type I (HTLV-I) infection (1.74%) in the country. To investigate which strains of HTLV-I are circulating in Salvador, we studied isolates from 82 patients infected with HTLV-I: 19 from the general population, 21 from pregnant women, 16 from intravenous drug users, and 26 from patients and their family attending a neurologic clinic. Phylogenetic analysis from part of the LTR fragments showed that most of these isolates belonged to the Transcontinental subgroup of the Cosmopolitan subtype (HTLV-Ia). Only one sample from a pregnant woman was closely related to the Japanese subgroup, suggesting recent introduction of a Japanese HTLV-I lineage into Salvador. betaA-Globin haplotypes were examined in 34 infected individuals and found to be atypical, confirming the racial heterogeneity of this population. A total of 20 chromosomes were characterized as Central African Republic (CAR) haplotype (29.4%), 31 (45.6%) were characterized as Benin (BEN) haplotype, and 17 (25%) were characterized as Senegal (SEN) haplotype. Five patients' genotypes (14.7%) were CAR/CAR; 10 (29,4%), BEN/BEN; 9 (26.5%), CAR/BEN; 2 (5.9%), BEN/SEN; and 7 (20.6%), SEN/SEN. One patient's genotype (2.9%) was CAR/SEN. The betaA-globin haplotype distribution in Salvador is unusual compared with other Brazilian states. Our data support the hypothesis of multiple post-Columbian introductions of African HTLV-Ia strains in Salvador, Bahia, Brazil.