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The cochlear nuclei--pathology.

This article is a sequel to one dealing with the anatomy of the cochlear nuclei. It employs the combined peripheral/central approach in reviewing some basic principles of the pathology of central deafness, involving the cochlear nuclei. The combined approach shows the relative status of the cochlear elements and of the superior ventral cochlear nucleus in various conditions. Anoxic encephalopathy, including that of erythroblastosis, involves the stated nucleus but spares the peripheral hearing organ. This, as well as the findings in other conditions, reflects on the employment of amplification or the implantation of electrodes. The spheroid cells, the nerve cells of concern of the superior ventral cochlear nucleus (SVCN), show an anatomic frequency gradient, low ventral to high dorsal. In any case of deafness expected to involve the SVCN and showing an audiogram frequency pattern, the frequency gradient must be included in the pathologic investigation. When the organ of Corti is completely destroyed, the spheroid cells may show transsynaptic atrophy.

Aged↗

Micro-angiopathic haemolytic anaemia and an unusual lactate dehydrogenase iso-enzyme pattern in metastatic adenocarcinoma. A case report.

Micro-angiopathic haemolytic anaemia is an unusual but well-recognized complication of metastatic adenocarcinoma. A young black man presented with red cell fragmentation, compensated disseminated intravascular coagulation and leuco-erythroblastosis. Metastatic adenocarcinoma of the stomach was suspected but only demonstrated at autopsy. An unusual finding was the elevation of the slow-moving lactate dehydrogenase iso-enzyme (LDH5) level in the plasma which was shown to be produced by metastatic tumour cells recovered from the bone marrow.

Adenocarcinoma↗

[Subacute idiopathic autoimmunehemolytic anemia with prolonged aplastic phase and erythremic reaction (author's transl)].

A case of idiopathic autoimmune hemolytic anemia with subacute onset and peculiar course is reported. In the beginning of the disease there was a long lasting severe aplastic phase, during which numerous blood transfusions were required. The low antiglobuline test titre compared with the severe hemolytic activity and the ineffective erythropoesis suggested first of all the presence of pure red cell anemia. Later on blood regeneration with erythroblastosis started and resembled the picture of erythremic myelosis. The transient course of the disease with full normalisation of all blood values and continuous remission lasting now for more than one year is a peculiar variant of idiopathic autoimmune hemolytic anemia.

Aged↗

[Immune response of (C57BL/6xA/Sn)F1 mice in mycoplasm-virus infection].

Mixed Rauscher leukemia virus (RLV) and M. arthritidis infection of (C57BL/6xA/Sn)f1 mice-hybrids, highly resistant to RLV, was accompanied by a progressive inhibition of rosette-forming cells (RFC) and plaque-forming cells (PFC), resulting in the induction of malignant erythroblastosis identical by cytology to Rauscher leukemia. The mice-hybrids infected with A. laidlawii and RLV developed significant splenomegaly on the 21st day of the infection, and their immune response was almost entirely suppressed, but both RFC and PFC populations as well as the spleen weight returned to the initial level by the 62d day the infection. A possible role of mycoplasm in the induction and development of Rauscher leukemia is discussed.

Animals↗

5-Azacytidine increases fetal hemoglobin production in a patient with sickle cell disease.

5-Azacytidine, given to one sickle cell patient as 9 doses of 30 mg/m2 each at intervals of 8 hr, rapidly increased the number of red cells containing HbF (F cells), decreased the amount of HbS in F cells, and decreased the MCHC of F cells. Although a significant rise in the peripheral blood hemoglobin from 8 gm/dl to 10-12 gm/dl was obtained, painful vaso-occlusive crises continued to occur. Therapy was associated with no marrow toxicity, although erythroblastosis appeared after each course of therapy. Increased HbF production was associated with demethylation of two restriction enzyme sites 5' to the two gamma-globin genes. Five other restriction sites around the gamma-globin genes were unchanged. 5-Azacytidine is considered potentially mutagenic and carcinogenic in man and, therefore, further experimental treatment should be limited only to severely affected sickle cell patients.

Adult↗

Diagnosis and management of bilateral theca lutein cysts in a normal term pregnancy.

Theca lutein cysts are benign neoplasms known to be associated with twins, molar pregnancy and erythroblastosis fetalis, but they are only rarely associated with a normal singleton pregnancy. Their natural course is postpartum spontaneous regression. Documented cases were noted because of abdominal pain or dystocia. This paper reports theca lutein cysts which were asymptomatic and were first noted ultrasonographically in a singleton gestation at term. In a few published cases and in a larger number of unpublished cases, apparent confusion as to the benign nature of these cysts has led to unwarranted bilateral oophorectomy. Asymptomatic theca lutein cysts undoubtably occur more commonly than they are reported. With the increasing use of ultrasonography and cesarean section, this condition probably will be observed more frequently in the future. At the time of laparotomy in late pregnancy with ovaries which appear pathological, tissue biopsy and evaluation is of critical importance in order to avoid unnecessary castration in this group of young women.

Adolescent↗

Viral-associated alterations in hematopoiesis in the mouse.

A variant of Rauscher leukemia virus, designated RLV-A, induces a protracted hematopoietic dysplasia characterized by hepatosplenomegaly with erythroblastosis, severe terminal anemia, thrombocytopenia, and erythroblastemia. Erythrocyte and platelet survival is reduced and the ferrokinetic data suggest that iron utilization is faulty. Stem cells (CFU-S, CFU-C, CFU-MK, CFU-E, BFU-E) are reduced in the bone marrow but increased in terminal spleens. The cause(s) of these viral-associated alterations in stem cell numbers is not known.

Animals↗

Differentiation properties of avian tumor cell lines: analysis using chicken fetal antigen and other markers.

Three avian lymphoblastoid tumor cell lines were compared for the expression of the serologically complex cell surface antigen, chicken fetal antigen (CFA), and other properties associated with differentiation. Two Marek's disease cell lines (CU-12 and CU-36) and a cell line associated with lymphoid leukosis (CU-10) were all found to differ qualitatively in the expression of CFA. While both Marek's disease cell lines exhibited overall phenotypes similar to those of T-lymphocytes, the lymphoid leukosis cell line possessed characteristics in common with either immature lymphocytes or cells of the reticuloendothelial system. The specific patterns of CFA expression on these lymphoid tumor cells may be related to the particular level of differentiation blockage during tumorigenesis as has been proposed for erythroblastosis. The lymphoid leukosis cell line exhibited a pattern of CFA expression that would not distinguish between the possibility of differentiation blockage versus retrogenic expression. Since CFA is known to be associated with a series of cell surface glycoproteins, the exact relationship between individual molecules, the level of lymphoid differentiation, and changes associated with tumorigenesis may now be examined.

Animals↗

[Haemoglobinosis C/beta-thalassemia double heterozygosity in an Algerian patient with total suppression of haemoglobin A synthesis (author's transl)].

The authors describe the case of a young Algerian, aged 32, suffering from mild icterus, accompanied by a marked splenomegaly. The blood count revealed a moderate degree of anaemia with reticulocytosis, pronounced anisocytosis, micro-spherocytes, bulls eye cells, folded cells, hypochrome cells, a marked polychromasia and a mild erythroblastosis. Present also were hyperbilirubinaemia, raised plasma haemoglobin, zero haptoglobin, a reduced osmotic fragility and half-life of erythrocytes. Haemoglobin electrophoresis showed 17.25% haemoglobin F, 62.8% haemoglobin C+A2 and no haemoglobin A. The genetic study indicated that the patient was a double heterozygote C/beta thalassaemia, his mother and his son both suffering from this disease. This thalassemic gene of type beta (0) totally inhibited the synthesis of haemoglobin A, the defect found in our patient.

Adult↗

Aetiology of developmental enamel defects not related to fluorosis.

The aetiological factors in enamel defects of a non-fluoride nature can be divided into systemic and local. The systemic factors comprise a variety of conditions: genetically determined, chromosomal anomalies, congenital defects, inborn errors of metabolism, neonatal disturbances, infectious diseases, neurological disturbances, endocrinopathies, nutritional deficiencies, nephropathies, enteropathies, liver diseases and intoxications. The genetically determined enamel defects include amelogenesis imperfecta, which may occur as an isolated phenomenon or as part of other disorders such as epidermolysis bullosa, pseudohypoparathyroidism and taurodontism. The congenital defects include heart disorders and unilateral facial hypoplasia and hypertrophy. Among the inborn errors of metabolism are: galactosaemia, phenylketonuria, alkaptonuria, erythropoietic porphyria and primary hyperoxaluria. Neonatal disturbances are important in the development of enamel hypoplasia, foremost among these are premature birth and hypocalcaemia. The latter causes postnatal hypoplasias, which, however, are never seen in breast-fed children. Haemolytic anaemia, mostly in conjunction with erythroblastosis foetalis, may cause enamel hypoplasia. In children with neurological disturbances a rather large number have enamel hypoplasias, and these changes may be a significant aid in neurological diagnosis. When the tetracyclines were introduced, many children had these drugs prescribed in the period when the teeth were undergoing mineralization. The result was a yellow-brown stain of the affected teeth. In recent years, however, there appears to have been a reduction in the incidence of tetracycline staining. As for local causes the most important are traumatic injuries and periapical osteitis of primary teeth.

Amelogenesis Imperfecta↗

Monoclonal antibody to chicken fetal antigens on normal erythroid cells and hematopoietic-lymphoid tumor cell lines.

Hybridoma cell lines secreting antibodies to chicken fetal antigens (CFAs) were generated by the fusion of mouse P3X63Ag8 myeloma cells with spleen cells from a mouse immunized with intact SC chicken strain one-day-hatched red blood cells. Immunodepletion studies show monoclonal anti-CFA to be detecting a subset of the Mr 50,000 CFA molecules recognized by polyclonal anti-CFA. Monoclonal anti-CFA is erythroid specific against in vivo-derived hematopoietic-lymphoid cells. Exceptions to the erythroid specificity of monoclonal anti-CFA include failure to react with avian erythroblastosis virus-transformed erythroid cells both before and after butyric acid-induced differentiation and reactions with reticuloendotheliosis virus-transformed immature lymphoid cells and chicken embryo cells. Immunofluorescence and 125I binding analyses utilizing monoclonal anti-CFA show reticuloendotheliosis virus cells to possess high levels of CFA even though the CFA determinant does not appear to be a 125I-labeled immunoprecipitable Mr 50,000 molecule. The unique property of monoclonal anti-CFA that permits it to distinguish among surface membrane antigens of normal and neoplastic cells of the same lineage makes it an important tool for future investigations of normal and abnormal cell differentiation.

Animals↗

Radiology of non-immune hydrops fetalis.

A woman presented with polyhydramnios when 33 weeks pregnant, and three weeks later delivered an infant with idiopathic non-immune hydrops fetalis. Ultrasonic scanning before birth showed a severely edematous infant. An abdominal radiograph revealed the classical "Buddha" position of fetal hydrops. The cause of the infant's hypoalbuminemia could not be determined, but the infant responded quickly to intravenous albumin and to thoracentesis for the large pleural effusions. As non-immune hydrops becomes proportionately more common, radiologists should be aware that the diagnosis of "hydrops fetalis" is not synonymous with "erythroblastosis."

Adult↗

Chicken fetal and adult antigen expression on erythroleukemia cells before and after induced differentiation.

Avian erythroblastosis virus strain R (AEV)-transformed, cloned erythroleukemia cells from three different ages of SC strain chickens were analyzed before and after differentiation induced by 1.0 mM butyric acid for expression of chicken fetal antigens (CFAs) and chicken adult antigens (CAAs) and for hemoglobin expression. Immunofluorescent analyses show the loss of individual CFA determinants from erythroleukemia cells with induced differentiation, although there appeared to be no correlation between CFA loss and onset of hemoglobin production. Erythroleukemia cells were examined by cell surface labeling followed by immunoprecipitation with antisera specific to CFAs and CAAs. Erythroleukemia cells expressed CFAs and CAAs on their membranes that are not reported to be expressed by the target cell of AEV. The expression of CAAs and the enhanced expression of CFAs by erythroleukemia cells may be due to limited cellular differentiation, alterations in regulatory controls of genes coding for CFAs and CAAs, or increased levels of production of previously undetected CFAs and CAAs following AEV transformation. Control and induced erythroleukemia cells expressed CFAs and CAAs that differed both quantitatively and qualitatively from normal erythroid cells. Molecular weight variations of CFAs and CAAs observed in the erythroleukemia cells may represent glycolyzation differences between AEV-transformed cells and normal erythroid cells.

Alpharetrovirus↗

Placentomegaly due to fetal congestive failure in a pregnancy with a sacrococcygeal teratoma.

Conditions associated with placentomegaly in which the disproportionate placental enlargement is understood include erythroblastosis fetalis, intrauterine infections and chorioangioma. This report suggests that in a pregnancy complicated by a large sacrococcygeal teratoma, placentomegaly results from high-output fetal cardiac failure secondary to the mass acting as an arteriovenous fistula.

Adult↗

Biology of Saryupari Brahmins of Chhattisgarh, central India.

In July-November, 1986, Saryupari Brahmins of Chhattisgarh (Central India) have been investigated for the distribution of ABO blood groups, Rh factor, ABH secretion, PTC taste sensitivity and seven morphological traits. The important findings in brief are as follows: 1. The frequency of O blood group is slightly dominant (38.91%) and gene frequency exhibit r > q > p pattern. Statistical analysis (D/sigma = 0.5447) show that the population is in genetic equilibrium. 2. Estimation of the expected rate of erythroblastosis foetalis occurred in this population is calculated to be 8-16 cases in every 10,000 pregnancies. 3. High incidence (53.82%) of non-secretors of ABH substances in saliva suggest that this group may belong to Caucasoid stock. 4. The ability to taste the chemical PTC has a high frequency (61.81%) in this group. 5. R/L type of handclasping (58.15%), leg folding (65.38%) and L/R type of arm folding (58.93%) is dominant. L-type of handedness (5.48%) is moderately high. 6. Tongue rollers (93.28%) and folders (53.77%) are more among them. 7. Free type of ear lobe (68.68%) is the common feature in this population. 8. The above observations were compared in details with other Brahmin populations reported from India with special reference to the descendant groups residing in Madhya Pradesh (Central India). Furthermore, the results obtained on growth studies, and dermatoglyphic studies (palm and sole) from our published literature were also included so as to have some idea on the genetical, behavioural, dermatoglyphic and growth norms of this group.

ABO Blood-Group System↗

Influence of expression and cis-acting sequences from avian leukosis viruses (ALVs) on stability of (ALV)-based retrovirus vectors.

Defective avian leukosis virus (ALV)-based vectors expressing the neo and LacZ genes were constructed under the control of cis-acting elements originated from 4 avian retroviruses: avian erythroblastosis virus (AEV), Rous associated viruses 1 (RAV-1) and 2 (RAV-2), and the Schmidt Ruppin strain of Rous sarcoma virus subgroup D (SR-RSV-D). We used these vectors to study the long-term stability of beta-galactosidase expression (encoded by the LacZ gene) in a permanent cell line from quail fibroblasts (QT6). Infection of the immortalized QT6 cell line with these vectors resulted in unstable beta-galactosidase expression. We determined whether this instability of provirus expression was correlated with: (1) presence of G418 selection; (2) deletion in the proviral genome; (3) hypermethylation of the proviral genome; (4) position of the neo and LacZ genes in the proviral genome; and (5) the transcriptional activity of the long terminal repeat (LTR) elements of proviral vectors. We observed that G418 selection pressure applied to infected QT6 cells lead to a more stable LacZ gene expression. Moreover, our results suggest a correlation between the stability of proviral gene expression and the level of gene expression driven by the LTR elements and depending on the strain origin of these.

Alpharetrovirus↗

Expression of embryonic zeta-globin and epsilon-globin chains in a 10-year-old girl with congenital anemia.

A 10-year-old Danish girl with congenital anemia is described. At birth, she had severe anemia and erythroblastosis and was transfused a number of times during the first year. The need for transfusions has since declined steadily. Her reticulocyte counts varied between 2% and 15%, and her bone marrow aspirate showed some dyserythropoietic features. Her hemoglobin F level was consistently elevated, up to as much as 41%. Her erythrocytes had a normal level of I antigen but an undetectable level of i antigen. Moreover, embryonic zeta-globin and epsilon-globin chains were present in some of her circulating erythrocytes. These findings may represent the manifestations of a new variant of congenital anemia.

Adolescent↗