RESUMO
The question of whether distribution of chromatids to daughter cells in mitosis is a random or nonrandom process was investigated by study of the distribution of labeled chromatin in anaphase pairs at M(1) and M(2) after a pulse of tritiated thymidine. Diploid and tetraploid rat and diploid human fibroblast-like cells in serial monlayer culture were synchronized by two different methods to "purify" M(1) and M(2) anaphases: metaphase shake, and FUdR block to DNA synthesis followed by exogenous thymidine. Exposed grains of NTB-2 emulsion were counted over M(1) and M(2) anaphase pairs. An analysis (by pair) of diploid M(2) anaphase grain counts showed two discrete populations of daughters with less and with more radioactivity. A similar analysis of diploid M(1) and tetrapolid M(2) anaphases showed a single grain-count distribution. These findings may support a nonrandom model of chromatid segregation for diploid mammalian cells but do not rule out random segregation until sound mathematical models are formulated for expected random grain distributions in M(2) anaphases of cells with differing numbers of chromosomes.
Assuntos
Cromossomos/análise , Técnicas de Cultura , Mitose , Ácidos Nucleicos/análise , Análise de Variância , Animais , Autorradiografia , Contagem de Células , Linhagem Celular , Cromátides/metabolismo , Cromossomos/metabolismo , Meios de Cultura , DNA/antagonistas & inibidores , Replicação do DNA , Diploide , Fibroblastos , Histocitoquímica , Humanos , Métodos , Modelos Biológicos , Ratos , Timidina/metabolismo , Trítio , Uridina/metabolismoRESUMO
The clinical value of the routine autopsy was studied in 172 sequential cases of perinatal deaths. Of these cases 139 (81%) were autopsied including 52 fetal deaths and 87 neonatal deaths. The clinically assigned cause of death was compared with the cause of death as identified by the autopsy. In 26% (nine fetal and 27 neonatal deaths) the autopsy was the sole means of establishing the cause of death. A need for specific genetic counseling or evaluation was identified in 62 of the 139 perinatal deaths. In 30 (48%) cases the autopsy was the sole source of information highlighting this need. The autopsy is an important clinical tool providing useful information to the perinatal clinician. Besides providing specific causes of death, it can correct clinical impressions and identify genetic factors that require further assessment and counseling.
Assuntos
Autopsia , Morte Fetal/patologia , Recém-Nascido , Anormalidades Congênitas/genética , Anormalidades Congênitas/patologia , Diagnóstico Diferencial , Feminino , Morte Fetal/diagnóstico , Morte Fetal/genética , Aconselhamento Genético , Humanos , Doenças do Recém-Nascido/diagnóstico , Doenças do Recém-Nascido/genética , Doenças do Recém-Nascido/patologia , GravidezRESUMO
Segmental lung resection was performed in 5 infants with congenital lung disease. The lesions were cystic adenomatoid malformation in 2, congenital cystic disease in 2, and congenital lobar emphysema in 1. A higher than expected incidence of major segmental bronchovascular anomalies was encountered (3 of 5 patients). Other than prolonged air leak in 1 patient, complications were negligible. All patients were well from one to six and one-half years after operation. Segmentectomy is a sensible operation for congenital pulmonary disease and ultimately may have as its greatest virtue the preservation of normal lung tissue.
Assuntos
Pulmão/anormalidades , Pneumonectomia/métodos , Brônquios/anormalidades , Feminino , Humanos , Lactente , Recém-Nascido , Pulmão/patologia , Masculino , Enfisema Pulmonar/congênito , Veias Pulmonares/anormalidades , Síndrome do Desconforto Respiratório do Recém-Nascido/cirurgiaRESUMO
Three basic types of microscopic biliary structures at the portahepatis were distinguishable in infants with biliary atresia: bile ducts, collecting ductules of biliary glands, and biliary glands. Correlation between the type of biliary structure and the quantity and quality of post-operative bile flow was possible in 23 instances. At 2 weeks after operation, the 11 patients in whom a bile duct was identified had a daily bile flow of 68.0 +/- 11.5 mL. Bilirubin concentration in the bile was 13.6 +/- 3.3 mg/dL and total daily bilirubin excretion was 8.77 +/- 2.74 mg. In contrast, bile flow in 12 patients having only collecting ductules and/or biliary glands in the porta hepatis was 19.1 +/- 3.9 mL and bilirubin concentration in bile was 1.7 +/- 0.3 mg/dL. Thus, total daily bilirubin excretion was 0.34 +/- 0.08 mg (P less than 0.001). Postoperative cholangitis occurred only in patients with ducts. It is concluded that only bile ducts communicate with the intrahepatic biliary system and drain bile after hepatic portoenterostomy.
Assuntos
Ductos Biliares/anormalidades , Bile/metabolismo , Fígado/patologia , Ductos Biliares/patologia , Colangite/etiologia , Duodeno/cirurgia , Humanos , Lactente , Jejuno/cirurgia , Fígado/cirurgia , Complicações Pós-OperatóriasRESUMO
Survival after Kasai's operation for "noncorrectable" biliary atresia is influenced by (1) age, (2) large bile ducts, and (3) concentration of the bile bilirubin. Critical values are: age less than 10.5 wk, any evidence of a large bile duct, and a bile bilirubin concentration greater than 8.8 mg/100 ml. Using these 3 factors, a predictive model is able to identify a favorable group with an 89% expected 4 yr survival.
Assuntos
Ductos Biliares/anormalidades , Análise Atuarial , Ductos Biliares/patologia , Ductos Biliares/cirurgia , Colorado , Feminino , Humanos , Lactente , Intestinos/cirurgia , Laparotomia , Fígado/patologia , Fígado/cirurgia , Masculino , Métodos , Modelos Teóricos , MortalidadeRESUMO
Seven of 114 (6%) patients treated in Denver for biliary atresia had a variant of the disease in which only microscopic bile ducts or ductules were identified at the porta hepatis, hepatic lesions were present, even in the neonatal example, and intrahepatic biliary hypoplasia was uniformly present. The excised bile duct cysts consisted of fibrosis or scar tissue and with little or no epithelial lining. Six patients had corrective surgery. The first patient was treated by choledochoenterostomy and became totally obstructed. Definitive operation in this patient and all subsequent patients consisted of excision of all extrahepatic duct structures and Roux-en-Y portoenterostomy. Two patients died, the one who did not have surgical correction and a second who had correction at age 35 weeks. The other five patients have been followed for 18 to 158 months and are anicteric but have some degree of residual liver damage. We conclude that so-called correctable biliary atresia occurs in less than 10% of cases, that because of coincident biliary hypoplasia, "cure" is not a possible outcome, and that the treatment of choice is complete surgical excision of the extrahepatic bile ducts and Roux-en-Y portoenterostomy.
Assuntos
Atresia Biliar/cirurgia , Ductos Biliares/cirurgia , Atresia Biliar/patologia , Ducto Colédoco/cirurgia , Duodeno/cirurgia , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Jejuno/cirurgia , Fígado/cirurgia , Masculino , Complicações Pós-OperatóriasRESUMO
Analyses of bile bilirubin during the first month after Kasai operations, and of the liver biopsies obtained at the time of initial surgery, were done in 67 patients with biliary atresia. Bilirubin excretion (milligrams per day) was determined as the product of the bile volume and its concentration. Operative liver biopsies were evaluated for fibrosis, bile duct proliferation, bile stasis, giant cell transformation, and parenchymal degeneration; the severity of each abnormality was graded on a scale of 0 to 4. A forward stepwise regression procedure using the Cox proportional hazards model identified the relationship between survival and covariants. Thirty-nine of 67 patients died. Of these, 38 excreted less than 6 mg of bilirubin per day during the first postoperative month. The other patient died of a coexisting anomaly. Nine other patients who excreted less than 6 mg of bilirubin per day are alive but are either jaundiced or awaiting transplantation. Nineteen patients who excreted greater than or equal to 6 mg of bilirubin per day are alive (mean follow-up, 61 months) with normal or near-normal liver function. The severity of liver fibrosis, bile duct proliferation, and bile stasis did not correlate with survival, whereas giant cell transformation and parenchymal degeneration were highly significant (P less than or equal to .000 and P less than or equal to .0003, respectively). Twenty-six infants with grade 1-4 giant cell transformation or grade 2-4 parenchymal degeneration had a mean survival of 11 months.(ABSTRACT TRUNCATED AT 250 WORDS)
Assuntos
Atresia Biliar/cirurgia , Bile/análise , Atresia Biliar/metabolismo , Atresia Biliar/mortalidade , Bilirrubina/análise , Biópsia , Humanos , Lactente , Fígado/patologia , Portoenterostomia Hepática , PrognósticoRESUMO
Twenty of 30 patients with bronchopulmonary dysplasia (BPD) had major tracheobronchial abnormalities, which in 18 could be incriminated as contributing to their symptoms. There were 15 examples of tracheobronchial stenosis, 13 of tracheomalacia, nine of bronchomalacia, and one of tracheal web. Sixteen patients had operations. Tracheostomy was successful as a temporizing measure in ten patients although there was significant morbidity. Balloon dilatation of tracheobronchial stenosis produced temporary (2) or long-term (2) improvement in four patients. Electroresection of tracheobronchial stenosis was successful in the three instances it was employed. Lobectomy for lobar emphysema was curative in both patients. Aortopexy for tracheomalacia improved all three patients. We conclude that (1) symptomatic major airway lesions are not uncommon manifestations of BPD, (2) many of the lesions are amenable to surgical therapy, and (3) bronchoscopic evaluation should be considered early in the course of infants with BPD.
Assuntos
Displasia Broncopulmonar/cirurgia , Displasia Broncopulmonar/complicações , Displasia Broncopulmonar/patologia , Broncoscopia , Feminino , Humanos , Lactente , Recém-Nascido , MasculinoRESUMO
A prenatal sonographic diagnosis of extrahepatic biliary atresia was made and, 76 hours after birth, operatively confirmed. A standard Kasai operation was performed, with the exception of the use of an ancillary appendiceal conduit to provide biliary drainage of an independent bile duct draining the right anterior hepatic segment. Hepatic and ductal histology were identical to those usually found in biliary atresia in a 6- to 8-week-old infant. The child is well at 16 months.
Assuntos
Atresia Biliar/cirurgia , Atresia Biliar/diagnóstico , Atresia Biliar/patologia , Feminino , Humanos , Recém-Nascido , Masculino , Gravidez , Diagnóstico Pré-Natal , UltrassonografiaRESUMO
Among 280 infants under 1 year of age with congenital heart disease autopsied at the University of Colorado Health Sciences Center between 1959 and 1978, there were six instances of grade IV1 pulmonary artery hypertension. Five were patients with ventricular septal defect (four associated with other cardiovascular malformations). The sixth was a patient with atrioventricular canal. The youngest was 2 1/2 months of age. Advanced degrees of pulmonary hypertensive arteriopathy (grade IV or more) have been said to be rare in infants, especially under the age of 1 year. The fact that all of these cases occurred within the last few years of the study suggests the possibility of improved supportive care leading to the prolonged survival of infants who might otherwise have died prior to developing severe disease. In addition, the role of altitude in accelerating the arteriopathy must be considered in the present series. In any case, this unexpected increase in the frequency of severe pulmonary hypertensive arteriopathy should stimulate consideration of early surgical correction of the underlying cardiovascular malformation, especially in areas of relatively high altitude.
Assuntos
Cardiopatias Congênitas/complicações , Hipertensão Pulmonar/etiologia , Artéria Pulmonar/patologia , Altitude , Feminino , Cardiopatias Congênitas/patologia , Comunicação Interventricular/complicações , Comunicação Interventricular/patologia , Humanos , Hipertensão Pulmonar/patologia , Lactente , MasculinoRESUMO
Seven years' experience (1965-1972) with neonatal pulmonary hemorrhage at the University of Colorado was reviewed. Pulmonary hemorrhage still occurs frequently as a preterminal event in hyaline membrane disease. As an isolated "pure" entity, however, pulmonary hemorrhage has shown a marked decline. This phenomenon is probably related to advances in neonatal intensive care. The radiographic pattern of "pure" pulmonary hemorrhage in the newborn is most commonly a diffuse alveolar infiltrate. Since pure hemorrhage is now an uncommon entity, the radiologist can reasonably suggest this diagnosis on rare occasions only, in the clinical setting of profound hypoxia.
Assuntos
Hemorragia/diagnóstico por imagem , Doenças do Recém-Nascido/diagnóstico por imagem , Pneumopatias/diagnóstico por imagem , Humanos , Doença da Membrana Hialina/complicações , Recém-Nascido , Masculino , RadiografiaRESUMO
Histopathological features of nonsuppurative destructive cholangitis have been described in primary biliary cirrhosis, chronic graft-vs-host disease, and chronic rejection of human liver allografts. To determine whether or not susceptibility to injury of interlobular bile ducts was related to the original hepatobiliary disease requiring transplantation, we compared the histopathology of allografts transplanted into two groups of patients. The first group consisted of patients whose original hepatobiliary diseases primarily affect bile ducts: primary biliary cirrhosis, sclerosing cholangitis, and biliary atresia. The second group consisted of patients whose original hepatobiliary diseases do not result in injury to interlobular bile ducts. In 32 liver allografts studied histopathologically, the original disease did not recur. Interlobular bile duct damage occurred, however, in 17 of the 32 and resembled that seen in primary biliary cirrhosis. The spectrum of bile duct injury included mononuclear inflammation of the bile duct epithelium, destruction of the bile duct epithelium, and dissolution of the bile duct. There was no relationship between the frequency or severity of bile duct damage and original hepatobiliary disease, age, sex, duration of allograft survival, or cause of death. We conclude that nonsuppurative destructive cholangitis occurs frequently in liver allografts and represents one component of allograft rejection. This lesion resembles, in many respects, that seen in primary biliary cirrhosis so that histopathological differentiation may be difficult.
Assuntos
Cirrose Hepática Biliar/etiologia , Transplante de Fígado , Adolescente , Adulto , Ductos Biliares/anormalidades , Carcinoma Hepatocelular/patologia , Carcinoma Hepatocelular/cirurgia , Pré-Escolar , Colangite/patologia , Colangite/cirurgia , Feminino , Rejeição de Enxerto , Humanos , Lactente , Cirrose Hepática Biliar/patologia , Cirrose Hepática Biliar/cirurgia , Neoplasias Hepáticas/patologia , Neoplasias Hepáticas/cirurgia , Masculino , Pessoa de Meia-IdadeRESUMO
We investigated the role of lipid peroxidation as the mechanism mediating copper toxicity in isolated rat hepatocytes and the modulating effect of vitamin E. Hepatocytes, isolated from rats fed diets containing deficient (E-), sufficient (E+), and excess (E++) amounts of vitamin E, were incubated with CuCl2 (0-2400 microM) for 150 min. Dose and time-dependent decreases in hepatocyte viability (determined by trypan blue exclusion and lactate dehydrogenase release) due to copper toxicity correlated with production of malonyldialdehyde in E- and E+ hepatocytes. However, malonyldialdehyde generation did not accompany copper toxicity in E++ cells. Copper toxicity was enhanced in E- compared to E+ and E++ hepatocytes as assessed by cell viability studies and ultrastructural plasma membrane bleb formation. In vitro vitamin E repletion of E- hepatocytes restored resistance to copper and decreased malonyldialdehyde production proportionately. Thus vitamin E deficiency appeared to increase the susceptibility of hepatocytes to copper toxicity. We conclude that lipid peroxidation may not be the mechanism by which copper is toxic to isolated hepatocytes but that the site of injury may be thiol-rich cellular proteins.
Assuntos
Cobre/toxicidade , Peroxidação de Lipídeos , Fígado/efeitos dos fármacos , Deficiência de Vitamina E/metabolismo , Vitamina E/farmacologia , Animais , Membrana Celular/efeitos dos fármacos , Membrana Celular/ultraestrutura , Sobrevivência Celular/efeitos dos fármacos , Células Cultivadas , Relação Dose-Resposta a Droga , Fígado/citologia , Fígado/metabolismo , Fígado/ultraestrutura , Masculino , Malondialdeído/metabolismo , Microscopia Eletrônica , Distribuição Aleatória , Ratos , Ratos Endogâmicos , Vitamina E/administração & dosagemRESUMO
We report a child with wheezing and respiratory distress of 53 months duration which was undiagnosed and unresponsive to treatment. Necropsy examination of trachea and bronchi showed narrowing by chronic inflammation and fibrosis of mucosa and submucosa. Despite intensive investigations, the aetiology and pathogenesis remained undetermined.
Assuntos
Bronquite/patologia , Traqueíte/patologia , Brônquios/patologia , Bronquite/etiologia , Doença Crônica , Feminino , Humanos , Lactente , Esclerose , Traqueia/patologia , Traqueíte/etiologiaRESUMO
To examine the role of oxidant damage to subcellular membranes in the pathogenesis of copper hepatotoxicity, the effects of dietary copper overload and varying states of vitamin E on biochemical, histological, and ultrastructural features of rat liver were investigated. Weanling male rats were pair-fed for 8 weeks on diets containing normal or high levels of copper in combination with either deficient, sufficient, or excessive vitamin E. Hepatic microsomes and mitochondria, isolated by differential centrifugation, showed similar enrichment and recovery among all experimental groups. Evidence of in vivo peroxidation of membrane lipids (generation of conjugated dienes and thiobarbituric acid reacting substances) was present in mitochondrial but not microsomal preparations from copper-overloaded rats. Serum aspartate aminotransferase, alanine aminotransferase, and cholylglycine (which were increased in all copper-overloaded rats), as well as mitochondrial thiobarbituric acid-reacting substances, were more elevated in vitamin E-deficient rats. In copper-overloaded rats, liver histology showed changes of acute and chronic hepatocyte injury with mild periportal fibrosis; electron microscopy showed abundant copper-containing lysosomes and dilated cristae of hepatocyte mitochondria, findings similar to those in the liver of humans with copper-overload disorders. These findings suggest that an oxidant injury to hepatocyte mitochondria may be one of the initiating factors in hepatocellular damage that leads to hepatic lesions in copper-overload states in humans.
Assuntos
Cobre/toxicidade , Peroxidação de Lipídeos , Lipídeos de Membrana/metabolismo , Microssomos Hepáticos/ultraestrutura , Mitocôndrias Hepáticas/ultraestrutura , Deficiência de Vitamina E/metabolismo , Animais , Cobre/administração & dosagem , Masculino , Microscopia Eletrônica , Microssomos Hepáticos/metabolismo , Mitocôndrias Hepáticas/metabolismo , Ratos , Ratos Endogâmicos , Vitamina E/fisiologiaRESUMO
We conducted experiments with a previously described rabbit model of Escherichia coli-induced preterm pregnancy loss. Does at 70% gestation were inoculated hysteroscopically with 0.2 ml of Escherichia coli (10(5) colony-forming units per milliliter) or saline solution. Animals were randomly assigned to either receive treatment with ampicillin-sulbactam (begun 1 to 2 hours before inoculation and continued for up to 7 days) or to receive no therapy. Animals were killed after delivery or after 7 days. Saline solution-inoculated animals had no pregnancy loss. Of the Escherichia coli-inoculated animals, those treated with ampicillin-sulbactam had significantly fewer deliveries, fewer positive cultures, and more live fetuses than the untreated animals (p less than or equal to 0.001). Cultures from multiple sites, amniotic fluid prostaglandin levels, and maternal progesterone levels were obtained, and the placenta, uterus, and fetal lung were histologically evaluated. In the second phase of the study, the Escherichia coli-inoculated animals were treated with ampicillin-sulbactam at one of three times: at inoculation or 2 or 4 hours after inoculation. The Escherichia coli-inoculated does treated with ampicillin-sulbactam at or before inoculation had significantly fewer deliveries, fewer positive cultures, and more live fetuses than the Escherichia coli-inoculated does in which treatment was delayed 4 hours (p less than or equal to 0.01).
Assuntos
Ampicilina/uso terapêutico , Infecções por Escherichia coli/prevenção & controle , Morte Fetal/prevenção & controle , Complicações Infecciosas na Gravidez/prevenção & controle , Sulbactam/uso terapêutico , Animais , Modelos Animais de Doenças , Quimioterapia Combinada/uso terapêutico , Feminino , Injeções Intramusculares , Gravidez/sangue , Progesterona/sangue , Coelhos , Fatores de TempoRESUMO
BACKGROUND: There has been controversy about whether early reperfusion of myocardial infarcts causes further necrosis mediated by reactive oxygen species or other mechanisms. Unequivocal evidence that therapeutic agents given only during reperfusion can prevent, rather than delay or modify, injury has been sparse. Failure to account for variables, such as collateral blood flow, that influence infarct size independently and attempts to measure infarct size too early in reperfusion may have limited the sensitivity and specificity of some previous studies. METHODS AND RESULTS: After 90 minutes of coronary occlusion and 48 hours of reperfusion in a canine model, we examined the effect on infarct size of intravenous infusion of N-(2-mercaptopropionyl)-glycine (MPG), a diffusible antioxidant. Infarct size and region at risk were measured by post-mortem dual perfusion with triphenyl tetrazolium chloride and Evans blue dyes, and regional myocardial blood flow was measured with radioactive microspheres. Infusion of MPG 100 mg.kg-1.h-1, beginning either 15 minutes before the onset of reperfusion or 30 minutes after the onset of reperfusion and continued until 4 hours of reperfusion and followed by an intramuscular dose, reduced infarct size, normalized for both region at risk and the level of collateral blood flow, by 60% and 45%, respectively. When infusion of MPG was limited to the last 15 minutes of ischemia and the first hour of reperfusion only, the normalized infarct size was reduced by 26%. Heart rate, blood pressure, and their product did not differ among the four groups studied. The plasma half-time of MPG was < 10 minutes. In in vitro experiments MPG was a scavenger of hydrogen peroxide but not of superoxide radical. CONCLUSIONS: After 90 minutes of coronary ligation, infusion of the diffusible hydrogen peroxide scavenger, MPG, for several hours, beginning as late as 30 minutes after the onset of reperfusion, substantially reduced infarct size measured 48 hours later. In this model, necrosis caused by processes during reperfusion may be more extensive than necrosis caused by ischemia alone. Since infusion of this agent for only the first hour of reperfusion was considerably less effective, it appears that most of the oxidant injury leading to necrosis occurred after the first 60 minutes but within the first 4 hours of reperfusion.
Assuntos
Sequestradores de Radicais Livres , Infarto do Miocárdio/tratamento farmacológico , Traumatismo por Reperfusão Miocárdica/prevenção & controle , Reperfusão Miocárdica , Tiopronina/uso terapêutico , Animais , Circulação Coronária/efeitos dos fármacos , Cães , Peróxido de Hidrogênio , Infusões Intravenosas , Masculino , Infarto do Miocárdio/patologia , Miocárdio/patologia , Fatores de Tempo , Tiopronina/administração & dosagemRESUMO
Four cases of congenital cystic adenomatoid malformation of the lung are presented with ultrastructural analysis. In some of the cases the ultrastructure is similar to that described in normal fetal airways during the early embryonic phase of lung development. Varying degrees of differentiation are noted, confirming what has previously been observed ultrastructurally in this lesion. One new finding is the presence of tonofilaments in one of our cases, which may represent a metaplastic change. Although the exact nature of the insult is unknown, there may be a developmental defect affecting epithelial differentiation as well as the interaction between the developing pulmonary mesenchyme and distal respiratory units.
Assuntos
Pulmão/anormalidades , Membrana Celular/ultraestrutura , Núcleo Celular/ultraestrutura , Citoesqueleto/ultraestrutura , Retículo Endoplasmático/ultraestrutura , Epitélio/ultraestrutura , Feminino , Humanos , Corpos de Inclusão/ultraestrutura , Recém-Nascido , Pulmão/ultraestrutura , Masculino , Microscopia Eletrônica , Mitocôndrias/ultraestrutura , Organoides/ultraestrutura , Ribossomos/ultraestruturaRESUMO
The clinical and pathologic features of 23 cases of hepatocellular carcinoma occurring in patients younger than age 35 years (mean age, 17.4 years) were analyzed. Ten of these (43%) were the fibrolamellar oncocytic variant (FLO), characterized by large polygonal neoplastic hepatocytes and lamellar bundles of collagen. The remainder (non-FLO) showed the usual wide range of gross and histologic patterns typical of hepatocellular carcinoma in older age groups. Overall, hepatocellular carcinoma was more common in females than in males. The FLO variant was characterized by a longer duration of symptoms prior to diagnosis, increased frequency of resectability of the tumor, and infrequency of mitoses. Of particular importance is the fact that 5 of 10 patients with the FLO variant are alive and clinically free of disease 1 1/2 to 8 years postoperatively, while none of the 13 patients with non-FLO hepatocellular carcinoma is alive and free of disease. There was no significant difference between the two groups in mean age at diagnosis, presence of single versus multiple hepatic tumors, vascular invasion, or tumor necrosis. Although cirrhosis was present in three non-FLO patients and none of the FLO patients, the difference was not statistically significant. The prognosis of hepatocellular carcinoma in young patients does not appear to differ from that in older patients, with the exception of the fibrolamellar oncocytic variant, a variant which is common in younger patients.
Assuntos
Carcinoma Hepatocelular/patologia , Neoplasias Hepáticas/patologia , Adolescente , Adulto , Carcinoma Hepatocelular/cirurgia , Criança , Pré-Escolar , Terapia Combinada , Feminino , Humanos , Fígado/patologia , Neoplasias Hepáticas/cirurgia , Masculino , Microscopia Eletrônica , Necrose , Invasividade Neoplásica , Estudos RetrospectivosRESUMO
Nine families have been reported in which male newborns presented with X-linked myotubular (centronuclear) myopathy. Little is known about the biochemical basis of this disorder or about its natural history in utero. We report a family in which an infant with myotubular myopathy presented in utero with polyhydramnios, poor fetal movement, and fetal cardiac arrhythmias. Shortly after birth the infant died from severe respiratory insufficiency. Gas chromatography-mass spectrophotometry for serum organic acids showed a large octanoic acid peak, but total acyl-CoA dehydrogenase activities in liver were normal. The maternal family history was significant for two perinatal male deaths. Postmortem examination revealed generalized muscle wasting, cardiac enlargement, cryptorchidism, and flexion contractures. Examination of muscle showed numerous fibers that had enlarged, centrally located nuclei and perinuclear clear zones. The muscle fibers were hypotrophic and predominantly of type I. Biopsy specimens of the muscles of the mother and maternal aunt had increased numbers of centrally located nuclei. Neurologic examination was normal. The case demonstrates the typical clinical course, pathology, and family history of severe X-linked myotubular myopathy. In addition, it confirms the reported detection of fetal cardiac arrhythmias and documents what may be an abnormality in fatty acid oxidation.