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Biomedical subjects

D L Gibbs

Publications and source records attributed to D L Gibbs.

At least 19 recordsLinked to original sources

Congenital diaphragmatic defects and associated syndromes, malformations, and chromosome anomalies: a retrospective study of 60 patients and literature review.

Congenital diaphragmatic defects (CDDs) may occur in malformation syndromes of varied causes. Syndromic cases of CDDs due to chromosomal defects, autosomal recessive, autosomal dominant, or X-linked inheritance have been described. In order to determine the frequency and nature of syndromes, malformations, and chromosome abnormalities associated with CDDs, we reviewed the records of all patients with CDDs evaluated over a 4-year period. During the 4-year interval, a total of 60 patients was evaluated. Of these, 29 had a therapeutic or spontaneous abortion, and 31 received postnatal care. On prenatal ultrasonography, 20 of 60 (33%) of patients with CDDs had additional anomalies. Additional anomalies, besides CDDs, were present in 15 of 31 (48%) of liveborn patients on newborn evaluation. In total, 16 patients with multiple anomalies were evaluated. Of these, 12 of 16 (75%) had additional abnormalities detected by prenatal ultrasonography. The 4 of 16 (25%) without additional anomalies on prenatal sonography had multiple anomalies found neonatally, lethal multiple pterygium syndrome being diagnosed in one case. Prenatal chromosome analysis was performed in 7 of 16 patients, and all had postnatal karyotypes. All initial karyotypes were normal. Tetrasomy 12p was documented on postnatal fibroblast analysis in one case who had percutaneous umbilical blood sampling (PUBS). Syndromes diagnosed postnatally in 7 of 16 patients (44%) include: Fryns syndrome (2), Simpson-Golabi-Behmel syndrome (2), tetrasomy 12p (1), Brachmann-de Lange syndrome (1), and lethal multiple pterygium syndrome (1). We were unable to make a specific diagnosis in 9 of 16 patients (56%) with multiple malformations. In patients with CDDs, a normal prenatal karyotype, especially if obtained by PUBS, and absence of other detected abnormalities by fetal ultrasonography, do not exclude the presence of other major anomalies, including chromosome abnormalities and severe multiple malformation syndromes.

Chromosome Aberrations

Neurodevelopmental outcome after open fetal surgery.

PURPOSE: This study examined the neurological and age-appropriate developmental outcomes of 11 children who underwent open fetal surgery. RESULTS: Nine children have normal neurological outcomes, and nine have normal development. CONCLUSION: Intensive postnatal care, intracranial hemorrhage, and requirement for prolonged respiratory support were associated with a worse neurological and developmental prognosis.

Central Nervous System Diseases

Fetal hydrops after in utero tracheal occlusion.

In utero tracheal occlusion accelerates fetal lung growth in experimental animals. Following tracheal occlusion, animals with an intact diaphragm can develop hydrops; presumably the enlarged lungs increase intrathoracic pressure, compress the fetal heart, and occlude venous return. In contrast, in animals with a diaphragmatic hernia, even excessive lung expansion has not led to hydrops because the lung can expand through the diaphragmatic defect into the abdomen. The authors had assumed that the diaphragmatic defect in human fetuses with congenital diaphragmatic hernia would provide a similar "release valve" if excessive lung growth occurred. A recent case proved this assumption wrong. At 26 weeks' gestation, an in utero fetal tracheal occlusion was performed on a human fetus with a diaphragmatic hernia. Over the next 9 days there was rapid lung expansion and overdistention, compression of the fetal heart, and hydrops. These findings mimic those seen in fetuses with congenital high airway obstruction syndrome and has implications for in utero treatment of congenital diaphragmatic hernia.

Adult

Familial diaphragmatic agenesis: an autosomal-recessive syndrome with a poor prognosis.

Diaphragmatic agenesis is a severe form of congenital diaphragmatic hernia for which an autosomal recessive form of inheritance has been proposed. The authors report six families with 13 pregnancies with diaphragmatic agenesis in which inheritance followed an autosomal recessive pattern, including the first reported case of bilateral diaphragmatic agenesis in twins. None of the thirteen affected fetuses survived. Familial diaphragmatic agenesis appears to be a distinct clinical entity with a worse prognosis than posterolateral diaphragmatic hernia.

Adult

Worldwide study of cefoperazone susceptibility.

In vitro susceptibility to cefoperazone of more than one million clinical aerobic bacterial isolates was evaluated in 369 hospitals in Japan, the United States, Canada, France, Austria, and Hungary. Standard versions of the disk diffusion method were used according to interpretive criteria approved in the respective countries, and comparisons were made among the countries and among various body sites. Susceptibility of organisms varied little within most species among the countries. Minor differences in methodology and in interpretive criteria may explain some of the variations observed among countries and hospitals. The susceptibility of key pathogens to cefoperazone among countries ranged from 91% to 98% for Escherichia coli, 73% to 91% for Pseudomonas aeruginosa, 90% to 95% for Klebsiella pneumoniae, 73% to 92% for Enterobacter cloacae, 74% to 92% for Staphylococcus aureus, and 91% to 97% for Proteus mirabilis. Susceptibility of isolates from the urinary tract did not vary markedly from that in other body sites.

Cefoperazone

Relationship between circulating interferon and anti-interferon antibodies and impaired natural killer cell activity in systemic lupus erythematosus.

Interferon (IFN) production and response are impaired in a high percentage of systemic lupus erythematosus (SLE) patients. In addition, elevated serum levels of alpha-IFN or anti-alpha-IFN antibodies are present in some SLE patients. This study examined the relationship of circulating IFN and anti-IFN antibodies to the impairment of natural killer (NK) cell function in SLE. All 15 SLE patients studied had measurable circulating alpha-IFN, while the normal controls had minimal serum IFN. Neither patient nor control sera contained any detectable anti-alpha-IFN activity. However, most of the SLE patients demonstrated defects in NK cell function. Because these defects in NK cell function appeared to be associated with circulating IFN, but not anti-IFN, antibodies, the effect of prolonged in vitro IFN exposure on NK cell function of peripheral blood mononuclear cells was determined. It was found that prolonged exposure to IFN induced both an apparent defect in IFN response and a definite impairment of baseline NK cell function. These results suggest that prolonged elevation of circulating alpha-IFN levels could be responsible, in part, for the defects in natural cytotoxicity present in SLE.

Autoantibodies

Generation of natural killer cells and lymphokine-activated killer cells in human AB serum or fetal bovine serum.

Interleukin 2 (IL-2) can induce or enhance the cytotoxic activity of natural killer (NK) cells and lymphokine-activated killer (LAK) cells. The effects of fetal bovine serum (FBS) and human AB serum (HABS) on the IL-2-induced NK cell and LAK cell activities of large granular lymphocytes (LGL) were measured, respectively, against the NK-sensitive cell line K562 and the LAK-sensitive cell line Daudi. FBS and HABS were essentially equivalent in their effects on IL-2-dependent NK activity with prolonged culture. However, with prolonged incubation from 1 to 5 days of PBMC in the presence of IL-2, there was considerably less generation of LAK cell activity in FBS (Day 3: 5.3 +/- 3.4 LU/10(7) cells) compared to HABS (Day 3: 44.6 +/- 4.2 LU/10(7) cells) (P less than 0.05). These differences in IL-2-dependent LAK cell generation did not appear to be due to the lot of the FBS or to activating factors present in individual samples of HABS. Similarly, the suppressive effects of FBS could not be reversed with increasing concentrations of IL-2 ranging from 10 to 100 U/ml. Importantly, the presence of FBS in the cultures resulted in more cell death (15.9 +/- 5.6%) at 4 days of culture compared to HABS (1.8 +/- 1.0%) (P less than 0.05). These results suggest that FBS may inhibit generation of LAK effector cells, but not NK cells in cultures containing IL-2 and that the use of HABS as a culture supplement is preferable to FBS in studies of human LAK cell function.

Animals

A comparative study of once versus twice daily treatment of superficial dermatophyte and yeast infections with tioconazole (1%) cream.

Two open, multicentre studies were performed to evaluate the efficacy of 1% tioconazole cream (Trosyd, Pfizer) as a once daily treatment for patients with common superficial dermatophyte and yeast infections (t. pedis, t. versicolor, t. corporis, t. cruris, candidiasis). In Study I, forty-eight patients were treated once daily and forty-nine were treated twice daily. The clinical response rate at the last treatment visit was 96% and 98%, respectively, in the two treatment groups. In Study II, all 107 patients were treated once daily, and the clinical response rate was 95% at the last treatment visit. The mycological cure rates were 85% and 91% in the Study I groups, and 94% in Study II. At the long-term follow-up evaluation (approximately 4 weeks after treatment) favourable clinical responses were seen in 88% of patients in both the Study I groups, and in 94% of patients in Study II. Long-term mycological cures were seen in 80% of patients in both groups of Study I, and in 94% of patients in Study II. Side-effects of treatment were generally transient, mild and coincident with disease symptoms, and occurred in six patients (6%) in Study I and in four patients (4%) in Study II. The results of both studies have shown no clinically or statistically significant differences in toleration or efficacy between once daily and twice daily treatment regimens with 1% tioconazole cream for the treatment of superficial dermatophyte infections.

Adolescent

Cefoperazone disk diffusion susceptibility test: confirmation of the tentative interpretive criteria, Pseudomonas aeruginosa cross-resistance, and determination of quality control performance limits.

Cefoperazone disk diffusion test and minimum inhibitory concentration comparison studies were performed on 421 recent bacterial isolates, using 30- and 75-micrograms commercially prepared disks. Acceptable correlation coefficients (-0.82 to -0.86) and very major (false-susceptible) interpretive error rates (less than 1%) were obtained with both disk concentrations. The interpretive criteria for both disks were identical. Using the preferred 75-micrograms disk, the Thornsberry et al. criteria (J. Clin. Microbiol. 15:769-776, 1982) of greater than or equal to 18 mm = susceptible (less than or equal to 32 micrograms/ml) and less than or equal to 14 mm = resistant (greater than 64 micrograms/ml) resulted in only 5.5% of strains having indeterminate-range zone diameters; the 30-micrograms disk had 6.9% of strains with indeterminate zone diameters. The 75-micrograms disk, excluding the testing of enterococci, minimized the very major and other interpretive errors to less than 5%. Larger zone diameters will contribute few technical problems with either disk concentration. Data from 1,320 zone diameters submitted for each quality control strain indicated no significant (P greater than 0.05) difference between disks made by the three major manufacturers, and consistent results were obtained within each laboratory with numerous lots of Mueller-Hinton agar (except for one manufacturer). Individual daily test and accuracy quality control ranges were calculated from clinical investigator laboratory data at 16 hospitals based on mean zone sizes and from an additional 8 laboratories with both mean and median calculations. The quality control data were nearly identical, and ranges calculated by the two methods were very similar. Susceptibility tests of Pseudomonas aeruginosa indicate that the cefoperazone disk or minimum inhibitory concentration test would accurately predict P. aeruginosa susceptibility test results for other pseudomonas-active cephalosporins (cefsulodin and ceftazidime), thus producing no very major interpretive errors.

Cefoperazone

Vibrio parahaemolyticus in Brazilian coastal waters.

Vibrio parahaemolyticus has been increasingly recognized as an important cause of acute diarrheal illness in Asia, Europe, Africa, Australia, and North America. We investigated whether this pathogen was also present in Brazil and found that 10% of the fish samples studied were contaminated with this halophilic vibrio. Positive cultures were obtained in three of 64 fish (5%) and in seven of 36 shell fish (19%).

Animals

The interaction in vitro between human polymorphonuclear leukocytes and Neisseria gonorrhoeae cultivated in the chick embryo.

Cultivation of Neisseria gonorrhoeae in the allantoic cavity of 10-day chick embryos ensured the following necessary properties for subsequent quantitive in vitro phagocytosis studies of viable gonococci: log phase of growth, resistance to the cidal effect of fresh human serum, maintenance of colonial type, and absence of clumping. Employing a modification of the Maaloe technique, phagocytosis of log-phase type 1 and 2 gonococci by human PMN leukocytes did not occur in the presence or absence of serum. These findings indicate that log-phase type 1 and 2 gonococci possess antiphagocytic surface factors Stationary-phase organisms of the same colonial type were ingested and rapidly killed by human PMN leukocytes under similar experimental conditions, thus emphasizing the necessity to employ log-phase gonococci in the study of phagocytosis and antiphagocytic surface factors. Log-phase type 4 gonococci were ingested and rapidly killed by human PMN leukocytes in the presence of fresh human serum but not heat-inactivated serum or in the absence of serum. Morphologic studies demonstrated that log-phase viable gonococci attach to the surface membrane of human PMN leukocytes. Interiorization of avirulent but not virulent organisms was observed in the presence of fresh human serum. Gonococci-human PMN leukocyte interactions thus provide a model for the investigation of the nonimmunologic and immunologic parameters associated with the attachment and ingestion stages of phagocytosis.

Animals

Chorioamniotic membrane separation: a potentially lethal finding.

Sonographic detection of chorioamniotic membrane separation (CMS) has been considered a benign incidental finding. We now report 6 cases of CMS identified by prenatal ultrasound; 1 in an otherwise normal pregnancy and 5 following fetal surgery. Following membrane separation, amniotic bands formed and compromised the umbilical cord in 4 cases leading to 2 fetal deaths. In the first case, CMS was detected by ultrasound at 22 weeks' gestation in an otherwise uncomplicated pregnancy. Because CMS was considered benign and umbilical cord blood flow was ample, the mother was followed by intermittent sonographic examinations. Fetal demise occurred 2 weeks later, clearly due to umbilical cord strangulation by an amniotic band. Surprised by this unexpected outcome, we reviewed our experience with CMS after hysterotomy for fetal surgery. Out of more than 40 fetal surgical cases, we have 5 cases in which CMS was recognized after hysterotomy. Three of these fetuses had umbilical cord compromise by a band of amniotic membrane leading to 1 fetal death. This experience demonstrates that membrane separation may be associated with amniotic band formation which can lead to cord strangulation and fetal compromise. Following fetal surgery, serial ultrasound evaluation and close fetal monitoring are indicated. In otherwise unremarkable pregnancies, clinician awareness of the possibility of amniotic band formation following CMS should be heightened. In either situation, knowledge of this potential life-threatening complication may identify cases in which cord compromise requires emergent delivery or fetoscopic release of the strangulating amniotic band.

Adult