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Biomedical subjects

R Drut

Publications and source records attributed to R Drut.

At least 19 recordsLinked to original sources
PubMed1998

Rhabdomyosarcoma of extrahepatic biliary tree: initial treatment with chemotherapy and conservative surgery.

BACKGROUND: The very low frequency of rhabdomyosarcoma (RMS) of the extrahepatic biliary tree has impeded the development of a standardized form of treatment. PROCEDURE: Based on the good response of embryonal RMS to chemotherapy, we used a multi-drug protocol as the initial treatment of a 3-year-old girl after obtaining adequate transparietohepatic biliary drainage. The treatment achieved complete remission. Later conservative laparoscopic surgery revealed only residual scar tissue. The patient is alive and well 1 year after remission. DISCUSSION AND CONCLUSIONS: Management of this difficult tumor using modern less invasive techniques for diagnosis and treatment, with the help of preoperative chemotherapy, prevented extensive damage to the biliary tree and allowed complete recovery.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1998

[Human papillomavirus, neonatal giant cell hepatitis and biliary duct atresia].

We previously recognized the presence of HPV-DNA in cases of idiopathic neonatal giant cell hepatitis (INGCH) and extrahepatic biliary duct atresia (EBDA) in archivated tissue using the PCR technique. In order to investigate a possible vertical transmission we looked for the presence of HPV-DNA in cervical swabs in the mothers along with formalin-fixed paraffin-embedded hepatic tissue from 3 infants with INGCH and 4 patients with EBDA by nested-PCR. Cervical smears showed koilocytosis consistent with HPV infection in 2 cases. Delivery was vaginal except for one that was by cesarean section. All infants were males. Amplification of HPV-DNA was demonstrated in all cases, the types being concordant in infants and mothers. Although this is a small group, the findings appear in line with previous data. The presence of the same type of HPV-DNA in the infants' livers and their mothers' cervical swabs is another argument supporting the possibility of vertical transmission of the virus.

Biliary Atresia↗
PubMed1997

Cytologic characteristics of peripheral neuroectodermal tumors in fine-needle aspiration smears: a retrospective study of three pediatric cases.

Cytologic diagnosis of peripheral neuroectodermal tumors (PNT) on fine-needle aspiration (FNA) smears represents a challenge to the cytopathologist. Usually ancillary studies are used to achieve definitive diagnosis. We retrospectively examined FNA material from three cases of PNT with the aim of identifying their features. Positive and negative cytologic findings were recognized. Positive features for PNT included the presence of: rather uniform appearance of the cells, which display scant but almost always-present perinuclear clear cytoplasm (suggesting a bland epithelial tumor); nuclei with distinctively smooth nuclear membrane contour, finely granular chromatin, and one or two small nucleoli (suggesting neuroendocrine anlage); and organization of the cells singly or in cohesive clusters. Negative findings included the absence of: frequent mitotic figures, large nucleoli, nuclear pleomorphism, cellular debris, histiocytes, and polymorphonuclear leucocytes. The smears appeared clean, with small, uniform cells having features suggesting a neuroendocrine epithelial tumor. These findings may prove useful for accurate cytologic diagnosis and differentiation of PNT from other small blue round cell tumours (SBRCT) of soft tissues without the use of ancillary studies since, when properly evaluated, cytomorphology of the latter group of tumors is more heterogeneous than generally believed.

Abdominal Muscles↗
PubMed1997

Aneuploid nucleomegaly of bronchial cells in ataxia-telangiectasia: cytologic recognition in bronchial brushings.

Ataxia-telangiectasia (AT) is an autosomal recessive disorder of childhood onset characterized by cerebellar ataxia and cutaneous and conjunctival telangiectasias, which affects many systems and organs. One histologic feature of AT is the presence of enlarged dystrophic nuclei, predominantly in satellite cells of sympathetic ganglia and dorsal roots. This paper describes the recognition of nucleomegaly of respiratory cells in bronchial brushings of a 9-year-old patient with AT. The enlarged nuclei displayed smooth nuclear contour, coarse and clumped chromatin granules, and one or two conspicuous nucleoli. The average size was 0.1015 mm in the AT case and 0.0573 mm in control cells. Ploidy analysis demonstrated an aneuploid population of cells with a DNA index of 1.31 and a S-G2M fase of 4.48% in the AT, while the control nuclei showed normal diploid values. To our knowledge, this is the first report of a description of aneuploid nucleomegaly of bronchial cells detected in bronchial smears from a patient with AT. Given that malignant transformations are usually preceded by ploidy alterations, it seems likely that the presence of an aneuploid cell population probably correlates with the increased cancer risk observed in AT patients. Cytopathologists must bear in mind these morphologic features of aneuploid nucleomegaly exhibited by certain cell populations when examining a smear from AT patients. Moreover, this finding may even represent a clue for diagnosis of AT in cases in which the disease has gone unrecognized.

Aneuploidy↗
PubMed1997

Malignancy-associated membranoproliferative glomerulonephritis.

An 11-year-old girl with an abdominal desmoplastic round cell tumor, treated with chemotherapy, presented with gross hematuria and proteinuria. Renal biopsy revealed type I membranoproliferative glomerulonephritis (MPGN). The association of a malignant tumor and MPGN is extremely unusual in children, and the pathogenesis of the renal lesion under these circumstances is unknown.

Abdominal Neoplasms↗
PubMed1997

[Clinico-endoscopic spectrum of gastritis associated with Helicobacter pylori in pediatrics].

In order to gain further knowledge about the clinical and endoscopic features of chronic gastritis (CG) associated with Helicobacter pylori (H py) we retrospectively analyzed 81 pediatric cases. All were biopsy-proven. The cases were divided in two groups: Group (1988-1992) included 21 cases. These represented the early stage of clinical recognition of the disease. Group 2(1993-1995) comprised 60 cases and represents the stage in which the disease was mandatory. Mean number of cases/year was 4.2 and 20 for group 1 and 2, respectively. Recurrent abdominal pain (RAP) was the most frequent clinical symptom (74/81; 91%), followed by upper digestive tract hemorrhage (UDTG) (34/81; 41.9%). The combination gastroesophageal reflux (GER) and esophagitis (E) was found in 52/81 (64.2%) of the children. Endoscopically, granularity of the mucosa was more frequently found in cases with RAP (47/74), GER (28/36) and E, while a smooth mucosa predominated in patients with UDTH (23/34). Our findings strongly suggest that symptomatic CG with H py in children expresses peculiar clinical and endoscopic features. Since RAP was present in 91% of the cases it appears adequate to include this disease in the differential diagnosis of it. These clinical manifestations have not been previously linked to CG with H py. Better understanding of the clinical and endoscopic spectrum of CG with H py results in adequate treatments and possibly prevention of gastric (and esophageal) diseases found in adults.

Abdominal Pain↗
PubMed1997

[Gastrointestinal autonomic tumor associated with von Recklinghausen's disease].

Gastrointestinal autonomic nerve (GAN) tumor is a rare type of gastrointestinal stromal tumor that is presumed to arise from the enteric autonomic plexus. Occasionally it develops associated with von Recklinghausen's disease (VRD). A 63-year-old-woman with VRD and two GANs in the ileum is reported, and the literature of this combined findings reviewed.

Autonomic Pathways↗
PubMed1996

Nonimmune fetal hydrops and placentomegaly: diagnosis of familial Wiedemann-Beckwith syndrome with trisomy 11p15 using FISH.

We have studied a family in which four members of the same generation were affected with Wiedemann-Beckwith syndrome (WBS). Trisomy 11p15 was demonstrated using molecular probes in interphase nuclei of formalin-fixed paraffin-embedded placenta from a stillborn fetus and in peripheral blood lymphocytes from two liveborn female relatives. Clinical examination showed nonimmune hydrops and placentomegaly in two siblings and multiple phenotypic abnormalities consistent with WBS in the two other relatives. Paternal karyotype of the stillborn infants demonstrated a reciprocal translocation (46,XY,t(10;11) (q26;p15)) explaining the origin of the extra 11p15 material. This study illustrates the advantages of FISH for interphase analysis of chromosome aberrations otherwise not detected even by conventional cytogenetic analysis and documents that nonimmune hydrops associated with placentomegaly may be the presenting features in familial WBS.

Adult↗
PubMed1996

[Undifferentiated gastric carcinoma of lymphoepithelioma type with Epstein-Barr virus].

We are presenting the case of a 62-year-old man with a gastric tumor which turned out to be an undifferentiated carcinoma lymphoepithelioma type. The epithelial nature of the cells was confirmed by immunohistochemistry. The presence of Epstein-Barr virus, limited to the epithelial cells, was demonstrated by in situ hybridization. These findings confirm data from the literature and support the possible etiopathogenic relationship between Epstein-Barr virus and undifferentiated carcinoma lymphoepithelioma type of the stomach. We are unaware of any other report of this type of neoplasm in our country.

Carcinoma, Squamous Cell↗
PubMed1995

Paracoccidioidomycosis: diagnosis by fine-needle aspiration cytology.

A 4-yr-old girl presented with constitutional symptoms, abdominal swelling, ascites, and cervical lymphadenopathy. Fine-needle aspirate smears of a cervical lymph node revealed numerous round yeasts, many of them with several peripheral buds fitting the pattern of Paracoccidiodes brasilensis. This appears to be the first case in which the diagnosis of this deep mycosis was achieved by FNAC. The procedure may prove useful for rapid diagnosis in cases like the present one which represents the so-called acute disseminated form which affects mainly children and immunosuppressed patients and can be rapidly fatal.

Biopsy, Needle↗
PubMed1995

Biphasic intraabdominal desmoplastic small round cell tumor: fine-needle aspiration cytology findings.

The present report describes the case of a 9-yr-old boy with an abdominal desmoplastic small round cell tumor (DSRCT) which on fine-needle aspiration cytology and histology revealed a biphasic pattern, making initial diagnosis difficult. Epithelial-like clusters of cells and loosely-arranged poorly-differentiated cells with scant cytoplasm associated with cells having a larger nucleus and multinucleated larger cells represented the smears' counter-part of epithelial clusters and lobules and sarcomatous-like tissue recognized in the histologic sections. Multinucleated cells were common in the sarcomatous-like areas of the tumor. The biphasic pattern was highlighted by immunohistochemistry. Keratin and epithelial membrane antigen stained predominantly or only the epithelial component, while desmin diffusely decorated the sarcomatous areas and the epithelial cells as a paranuclear cytoplasmic dot. Immunosera 013 mainly stained the sarcomatous component.

Abdominal Neoplasms↗
PubMed1995

Detection of human papillomavirus in juvenile laryngeal papillomatosis using polymerase chain reaction.

We examined the presence and subtypes of human papillomavirus (HPV) in 20 paraffin-embedded samples (from 12 patients) of juvenile laryngeal papillomatosis using the polymerase chain reaction (PCR). The biopsies had been stored for months to 12 years. Due to the great genetic variability of HPV, we selected a conservative sequence of the viral genome (L1 region) to identify the vast majority of the subtypes. Positive results were obtained by one-step PCR amplification with the MY09-11 consensus primers (L1 region) in only 10 of the cases. After a two-step amplification (nested-PCR) with GP5-6 primers the 20 samples proved to be positive demonstrating the higher sensitivity of this method. In order to amplify a highly variable region of the genome (E6), specific primers for HPV types 6 and 11 (H6/11 L1-R2) were used. 7/12 patients were positive for this subtype. Since more that one subtype has been reported in the same sample, the presence of HPV 6-11 sequences does not exclude that other subtypes might be involved. The results of this study show that: 1) HPV is present in JLP. 2) The most frequent HPV subtype involved was from the 6-11 group. 3) PCR can be successfully used in archived tissue routinely processed in a laboratory of pathology.

Base Sequence↗
PubMed1995

Cytomegalovirus-associated Ménétrier's disease in adults. Demonstration by polymerase chain reaction (PCR).

Ménétrier's disease (MD) is a rare form of hypertrophic or hyperplastic gastropathy characterized by conspicuous thickening of the gastric mucosal folds and foveolar (crypt) hyperplasia. We examined the presence of cytomegalovirus (CMV) in 2 cases of MD in adults, one associated with gastric carcinoma, using the polymerase chain reaction (PCR). None of the cases showed intranuclear inclusions consistent with CMV infection. Both revealed, besides the features of MD, a peculiar pattern of "chronic active plasmacellular gastritis". Although the samples had been stored in formalin for more than 10 years CMV-DNA was recovered with good yield from both samples. The demonstration of CMV in MD in adults may helps to explain present knowledge of the complex relationships between this virus and gastric mucosa, and strongly suggests a pathogenetic role of the virus with variable phenotypic expression along the years.

Cytomegalovirus Infections↗
PubMed1994

Fetal renal maldevelopment with oligohydramnios following maternal use of piroxicam.

A female neonate, born by cesarean section at 37 weeks of gestation, presented with respiratory distress syndrome, right pneumothorax and anuria. A sonogram showed increased echogenicity, with neither hydronephrosis nor macroscopic cysts. Peritoneal dialysis was started on the 14th day because of renal insufficiency, but the newborn died on the 33rd day. Family history was unremarkable, except that the mother received piroxicam at about the 26th week of gestation. A sonogram at the 28th week showed oligohydramnios. Histopathological study of the kidneys revealed crowded glomeruli and only few differentiated proximal convoluted tubules in the inner cortex, abnormally differentiated microcystic tubules and microcystic glomeruli in the outer cortex. Periodic acid-Schiff staining showed only traces of brush border in the dilated tubules of the outer cortex. Immunoperoxidase staining for epithelial membrane antigen was positive in the luminal border of all tubules. Electron microscopy confirmed the presence of brush border remnants and other proximal tubular characteristics in some segments. The renal abnormality bears some similarities to that found in familiar renal tubular dysgenesis, but it fits better with those described after maternal use of angiotensin converting enzyme inhibitors and nonsteroidal anti-inflammatory drugs. The lesion in this case appears to have resulted from fetal exposure to piroxicam. Recently, a second pregnancy ended in a completely normal female newborn.

Abnormalities, Drug-Induced↗
PubMed1994

[Pathologic findings in diethylene glycol poisoning].

We are reporting the necropsy findings of 7 patients poisoned with diethylene glycol-contaminated propolis (a rubbery substance produced by bees from vegetal resins). Besides the well-known features of hydropic necrosis of centrolobular areas in the liver and renal tubules we found acute pancreatitis with diffuse enzymatic fat necrosis which in two of the cases was considered the secondary cause of death, and acute demyelinating lesions in the central and peripheral nervous system. Six out of the 7 cases showed glomerular PAS-positive arteriolar hyalinosis at the vascular pole, in two of them widely disseminated. Differing from the findings reported in ethylene glycol poisoning we could not find calcium oxalate crystals in any of the cases. The pancreatic, central and peripheral nervous system lesions as well as the glomerular arteriolar hyalinosis have not been previously described in the literature in relation with diethylene glycol poisoning.

Aged↗