Interruption of aortic arch and hypoplastic left heart syndrome.

Interruption of the aortic arch and hypoplastic left heart syndrome in the same patient is exceptional. In the combined collections of the Registry of Cardiovascular Disease (St. Paul, Minnesota, U.S.A.) and the Registry of Congenital Heart Defects of the Rijksuniversiteit Gent (Gent, Belgium) three specimens were found with this unusual combination. These cases are herein described and compared with four similar cases previously reported in the literature.

Effects of shipping, handling, adrenocorticotropic hormone, and epinephrine on alpha-tocopherol content of bovine blood.

In 2 studies, plasma, erythrocyte, and neutrophil alpha-tocopherol concentrations were monitored in beef cattle after shipping, handling, and sample collection. On the basis of alpha-tocopherol results, an additional 2 studies were designed to measure the effects of administration of adrenocorticotropic hormone (ACTH) and epinephrine on the alpha-tocopherol concentration in the aforementioned blood constituents and on creatine kinase (CK) activity in Holstein calves. In the first of these studies, 15 beef cattle that had recently arrived at the feedlot consumed feed supplemented daily with 1,000 IU of dl-alpha-tocopheryl acetate. Values for initial blood samples indicated that CK activity was high. Although plasma alpha-tocopherol concentration indicated that vitamin supplementation was adequate, RBC and neutrophil alpha-tocopherol values were generally nondetectable. After 4 weeks of supplementation, plasma alpha-tocopherol concentration increased (P < 0.05), and neutrophil and RBC alpha-tocopherol values became measurable in most of the cattle. In the second study, 6 beef heifers had decreased (P < 0.05) plasma, RBC, and neutrophil alpha-tocopherol values after multiple periods of handling and blood sample collection. In the third and fourth studies, 10 tamed Holstein heifer calves, 5 of which were administered ACTH and epinephrine to simulate stress effects on blood alpha-tocopherol concentrations and CK activity. In study 3, the vitamin E-adequate heifers had increased blood CK (P < 0.001) activity and cortisol (P < 0.01) concentration, and decreased (P < 0.05) neutrophil alpha-tocopherol concentration after hormone injections.(ABSTRACT TRUNCATED AT 250 WORDS)

Neutrophil function in normal and Chediak-Higashi syndrome cats following administration of recombinant canine granulocyte colony-stimulating factor.

The effects of recombinant canine granulocyte colony-stimulating factor (rcG-CSF) on leukocyte counts and neutrophil function in clinically normal cats and in cats heterozygotic and homozygotic for Chediak-Higashi syndrome (CHS) were examined. CHS is a genetic disease characterized by neutropenic episodes and defects in a variety of phagocyte functions. Short-term administration of rcG-CSF at 10 micrograms/kg body weight resulted in a five- to tenfold increase in circulating granulocytes by day 10 of administration and normalizes CHS neutrophil counts by day 3. The drug was specific for neutrophils as determined by differential cell counts. Neutrophil chemotaxis under agarose and phagocytosis of Escherichia coli were characterized following administration of rcG-CSF in vivo. Granulocytes elicited by rcG-CSF show enhanced chemotactic abilities toward activated serum, increased spontaneous migration, and an enhanced ability to ingest opsonized E. coli. At a concentration of 1 nM rcG-CSF in vitro, chemotaxis and spontaneous migration were increased, with no effect on phagocytosis. CHS neutrophil function was improved by administration of rcG-CSF in all parameters studied, although the defect in chemotaxis was present throughout the treatment period. We conclude from this study that neutrophils elicited by rcG-CSF are functionally enhanced and that rcG-CSF may be a viable therapy for CHS and other related disorders.

Evaluation of clinical signs of disease, bronchoalveolar and tracheal wash analysis, and arterial blood gas tensions in 13 horses with chronic obstructive pulmonary disease treated with prednisone, methyl sulfonmethane, and clenbuterol hydrochloride.

We evaluated the efficacy of 3 treatments for chronic obstructive pulmonary disease in horses: prednisone (400 mg/horse, PO, daily; n = 7), methyl sulfonmethane (10 g/horse, PO, q 12 h; n = 6), and clenbuterol hydrochloride (0.4 mg/horse, PO, q 12 h; n = 7). A fourth group acted as controls (n = 6) and was not treated. The treatment period lasted 10 days. Each horse was a member of 2 different groups for 10 days, separated by an 18-day interval of no treatment. All horses were housed together in an outdoor pen without bedding. Horses were fed alfalfa/grass hay mix ad libitum from a large feeder. The same batch of hay was fed throughout the study. Multiple physical and laboratory variables were monitored prior to, during, and at the end of each 10-day trial period. Changes in lung sounds, respiratory effort, degree of anal movement, nasal discharge, temperature, respiratory rate, or heart rate were not significant. Changes in arterial blood gas tensions, tracheal wash or bronchoalveolar lavage cytologic findings, or phagocyte function were not significant. All horses were tachypneic and most were tachycardic. The median value for PaO2 was below normal for all horses. All tracheal wash and most bronchoalveolar lavage cytologic findings represented a suppurative response. Negative linear correlation was observed between PaO2 and degree of respiratory effort in these horses (eg, as PaO2 decreased, the degree of respiratory effort increased).

Defective in vitro motility of polymorphonuclear leukocytes of homozygote and heterozygote Chediak-Higashi cats.

The in vitro migratory responses of neutrophils of homozygote and heterozygote Chediak-Higashi cats were defective in an under-agarose assay when compared to the behavior of phagocytes of control cats. The linear distances traversed by the leading front of migrating Chediak-Higashi neutrophils toward streptococcal culture supernatant, zymosan-activated serum or buffer were reduced and smaller numbers of Chediak-Higashi phagocytes populated the resulting migration areas than did cells of control animals. The relative migration parameters of the Chediak-Higashi phagocytes, however, did not differ from the corresponding parameters of control neutrophils in the presence of streptococcal culture supernatant. Therefore, phagocytes of homozygote and heterozygote Chediak-Higashi cats recognized and responded equally well to the bacterial stimuli as did cells of control animals but traveled shorter distances primarily because of a reduced inherent motility. Similar results were also obtained when the feline phagocytes were attracted by zymosan-activated serum. In addition the relative migration parameters of the neutrophils of homozygote Chediak-Higashi cats were reduced and the normalized spatial distributions of their migrating cells were significantly different in the presence of 100% and 20% zymosan-activated serum when compared to the corresponding migration parameters of carrier and control animals. Defective recognition or responses to the higher concentrations of these host-derived attractants complicated, therefore, the already reduced inherent motility of the phagocytes of homozygote Chediak-Higashi cats.

Influence of technical parameters on the in vitro motility of equine neutrophils in the presence of streptococcal culture supernatant.

To identify the influence of technical factors on the in vitro motility of equine neutrophils towards streptococcus culture supernatant in an under-agarose assay, we studied the changes in eight cell migration parameters. The distances the phagocytes travelled by directed, random and spontaneous migration increased with incubation time, cell concentration and the gelatin and serum contents of the migration plates. The contribution of chemotaxis to the phagocyte migrations, however, decreased simultaneously. The directed and random, though not the spontaneous, migrations of the phagocytes increased also when the chemoattractant wells were placed closer to the cell wells but so did the influence of the chemokinetic activity of the bacterial culture supernatant on phagocyte motility. In contrast, preincubation of migration plates with the chemoattractant, the agarose content of the migration plates and contamination of the granulocytes with non-migrating, mononuclear cells did not substantially affect the in vitro migrations of the neutrophils. The changes in the in vitro motility of the equine neutrophils by these technical factors were, in general, comparable to those reported for human cells attracted by a variety of host-and bacteria-derived chemoattractants.

Chemotactic and chemokinetic activity of Streptococcus faecalis culture supernatant for equine neutrophils.

Although equine neutrophils did not respond towards formylated methionyl peptides, Streptococcus faecalis culture supernatant caused an in vitro stimulation of equine neutrophil motility when measured by an under-agarose assay. The migration of neutrophils towards the culture supernatant increased sigmoidally with the logarithmic concentration of the culture supernatant in the chemoattractant wells. The streptococcal culture supernatant was chemokinetic because it stimulated the random motility of the phagocytes. Because granulocytes migrated further towards the supernatant than could be explained by the chemokinetic activity of the bacterial products, the streptococcal culture fluid also exerted a chemotactic effect on the leukocytes. The chemotactic activity of the supernatant was further confirmed by the changes in the orientation of the migrating cells during incubation. These results indicate that bacteria produce cytotaxins other than formylmethionyl peptides which are recognized by equine neutrophils.

Effect of transportation stress on bronchoalveolar lavage fluid analysis in female horses.

Four bronchoalveolar lavages were performed sequentially on 9 control and 8 transport-stressed female horses. Alterations in results of fluid cytologic analyses, microbial content, and phagocyte function of recovered pulmonary macrophages in all horses were determined. Seemingly, absolute and relative increase in the number of inflammatory cells detected in the second bronchoalveolar lavage fluid of control horses was the result of irritation of the first lavage. This increased response was not observed in transport-stressed horses until 5 days after transport (third lavage; 10 days after initial lavage). Seemingly, delayed inflammatory response was the result of the transport stress. Microbial content and macrophage function were not significantly different between the 2 groups (P greater than 0.05).

Hypoplastic left heart syndrome and 45X karyotype.

A review of 63 patients with 45X karyotype (Turner's syndrome) admitted to a hospital from 1972 to 1985 showed that 20 (32%) had one or more major cardiac malformations (mostly coarctation and aortic stenosis). Four (20%) died in the neonatal. One infant had mitral stenosis and severe aortic stenosis and died at the age of 35 days. The three (15%) other patients who died had a typical hypoplastic left heart syndrome, with an atretic aortic valve in two and pinpoint aortic valve in one. Turner's syndrome was clinically suspected in three of the cases. One of these had mosaicism (46XX,45X) the others had a 45X pattern. During the same period (1972-85) 39 patients (14 girls and 25 boys) were admitted with diagnosis of hypoplastic left heart syndrome. Three (21.4%) girls had a 45X karyotype. The high incidence of hypoplastic left heart syndrome in Turner's syndrome and of Turner's syndrome in girls with hypoplastic left heart syndrome suggests that hypoplastic left heart syndrome can be another expression of the 45X karyotype.

Captopril in severe childhood hypertension--reversible anaemia with high dosage.

Experience with chronic inhibition of the angiotensin-converting enzyme in children is limited to cases refractory to all other forms of treatment. In reports dealing with the use of captopril (Capoten-R) in children no important side-effects are mentioned. This report describes a 7-year-old boy with severe hypertension secondary to haemolytic uraemic syndrome. Good pressure control was obtained after introduction of captopril. However, under the high initial dosage, pronounced anaemia developed within the first 3 months of treatment. The anaemia responded to dose-reduction while pressure control was maintained. Serial echocardiographic studies were performed. They illustrate the beneficial haemodynamic effects of captopril in the follow-up of children under antihypertensive treatment. Some recommendations are made on the use of captopril in children.

Isolation of horse mononuclear cells, especially of monocytes, on Isopaque-Ficoll neutral density gradient.

Horse mononuclear cells were separated from whole blood using neutral density gradient centrifugation on Isopaque-Ficoll. The resulting cell suspension was comparable in composition with similarly prepared human and bovine mononuclear cell preparations. The relative concentration of monocytes was increased by the use of a gradient with density lower than that originally proposed by Böyum (Böyum, A. 1968. Scand. J. Clin. Lab. Investig. 21 supple. 97:77-89). Contamination by neutrophils was limited either by using a gradient medium of lower density or by replacing Isopaque-Ficoll by Percoll-0.9% NaCl. Although the density of the Isopaque-Ficoll appears to be the main determinant in the isolation method of Böyum, the mechanism of separation of the cell population is complex and a substantial variability of the results can be expected.

Comparative study in angiocardiography in children, evaluating the side-effects of non-ionic and ionic contrast media.

The study concerns 127 consecutive patients, presenting congenital heart disease, with an age distribution from neonatal to 15 years (mean 5 years). With non-ionic Amipaque, 164 injections were performed, with ionic Isopaque Coronar 81. Isopaque Coronar caused in 51% of the cases a clear discomfort for the patient; with Amipaque the side-effects were reduced to 9% (p less than 0.001). The mean body temperature rise with Amipaque was only 0.09 degrees C, against 0.28 degrees C with Isopaque Coronar (p less than 0.001). The changes in the heart rate reached 8.35% with Amipaque and 15.2% with Isopaque Coronar (p less than 0.001). Amipaque caused a mean of 1.8 extrasystoles per injection; Isopaque Coronar 2.2 (p less than 0.05). The quality of the angiogram with Amipaque was quite better than with Isopaque Coronar (p less than 0.0025). The non-ionic Amipaque is a much safer and better tolerated compound that the ionic Isopaque Coronar for paediatric angiocardiography.

Surgery for arteriovenous fistulas and dilated vessels in the right lung after the Glenn procedure.

A superior vena cava-right pulmonary artery (SVC-RPA) anastomosis was constructed in a 2-year-old boy with tetralogy of Fallot. Ten years later and 5 years after "corrective" surgery without removal of the shunt, cyanosis and heart failure developed. Stereocineangiography and lung scanning revealed arteriovenous fistulas and dilated vessels in the right lung. The SVC-RPA anastomosis was taken down, the SVC being reimplanted in the right atrium and the RPA end being closed with a few stitches. Neither lobectomy nor pneumonectomy was performed. Immediately after the operation and during a follow-up period of almost 2 years, the boy has remained asymptomatic. Whenever a correction is planned in a patient with SVC-RPA anastomosis, the vessels of the right lung should be examined by scanning and angiography. If important arteriovenous fistulas do exist, the affected lung should be excluded from the pulmonary artery circulation.

A new case of the trisomy 9P syndrome. Report of a patient with unusual chromosome findings (46,XX/47,XX, + i (9p) and a peculiar congenital heart defect.

A girl is described for whom an unusual chromosome constitution was found and who had a peculiar congenital heart defect. The girl showed the main clinical features of the trisomy 9p syndrome, such as psychomotor retardation, microcephaly and brachycephaly, enophthalmos, antimongoloid eye slant, hypertelorism, abnormal ears, a globulous nose, downward slanting mouth, hypoplasia of phalanges and abnormal palmar creases. In addition, the girl had an incomplete harelip, a cleft palate and a peculiar congenital heart defect, a ventricular septal defect with pulmonary valve stenosis and a marked hypoplasia of the pulmonary trunk, including the bifurcation. Chromosome analysis revealed a mosaicism with normal and abnormal mitoses (47 chromosomes). The extra chromosome was a metacentric E16-like chromosome, which on the basis of the G, R, Q and Giemsa-11-banding could be identified as an isochromosome of the short arm of chromosome 9 (46,XX/47,XX, +i (9) (pter leads to cen leads to pter)). The patient consequently had a partial tetrasomy of the short arm of chromosome 9.