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Biomedical subjects

G Jaeger

Publications and source records attributed to G Jaeger.

At least 19 recordsLinked to original sources

Diagnosis and clinical course of autoimmune neutropenia in infancy: analysis of 240 cases.

Primary autoimmune neutropenia (AIN) is caused by granulocyte-specific autoantibodies and occurs predominantly in infancy. Clinical presentation and diagnosis have not been well established, resulting in burdening diagnostic investigations and unnecessary treatment with granulocyte colony-stimulating factor (G-CSF). In the present study, clinical, laboratory, and immunologic data of 240 infants with primary AIN were evaluated. Suspected association with parvovirus B19 infection was investigated using serologic and DNA-based methods. Primary AIN was mainly diagnosed at the age of 5 to 15 months but was observed as early as day 33 of life. In 90% of the cases, AIN was associated with benign infections despite severe neutropenia. Spontaneous remission, shown by 95% of the patients, usually occurred within 7 to 24 months. Autoantibodies in the patient's sera were not always present, and screening had to be repeated several times until antibody detection succeeded. About 35% of the autoantibodies showed preferential binding to granulocytes from NA1 and NA2 homozygous donors. Bone marrow was typically normocellular or hypercellular, with a variably diminished number of segmented granulocytes. A significant association with parvovirus B19 infection was not found. Symptomatic treatment with antibiotics was sufficient in most patients. Eighty-nine percent of the patients received antibiotics (cotrimoxazole) for prophylaxis of infections. For severe infections or for surgical preparation, G-CSF, corticosteroids, and intravenous IgG were administered, resulting in increased neutrophil counts in 100%, 75%, and 50% of the patients treated, respectively. In combination with the detection of granulocyte-specific antibodies, the typical clinical picture allowed diagnosis of AIN without burdening investigations. Treatment with G-CSF was found to be a reliable alternative to temporarily increase the neutrophil count.

Adrenal Cortex Hormones

Invasive pulmonary aspergillosis in a critically ill neonate: case report and review of invasive aspergillosis during the first 3 months of life.

We report a fatal case of invasive pulmonary aspergillosis in a severely ill neonate and review 43 additional cases of invasive aspergillosis reported from 1955 through 1996 that occurred during the first 3 months of life. Eleven of the 44 patients had primary cutaneous aspergillosis, 10 had invasive pulmonary aspergillosis, and 14 had disseminated disease. Most infections were nosocomial in origin. Prematurity (43%); proven chronic granulomatous disease (14%); and a complex of diarrhea, dehydration, malnutrition, and invasive bacterial infections (23%) accounted for the majority of underlying conditions. At least 41% of the patients had received corticosteroid therapy before diagnosis, but only one patient had been neutropenic. Among patients who received medical and/or surgical treatment, outcome was relatively favorable, with an overall survival rate of 73%. Invasive aspergillosis may occur in neonates and young infants and warrants consideration under certain circumstances. Current therapeutic approaches consist of high-dose amphotericin B and appropriate surgical interventions.

Amphotericin B

Primary suture of anorectal abscess. A randomized study comparing treatment with clindamycin vs. clindamycin and Gentacoll.

UNLABELLED: Gentacoll (Schering-Plough A/S, DK-3520 Farum, Denmark) implant is a biologically absorbable collagen with gentamicin, which enables local use of gentamicin in an abscess cavity. PURPOSE: To increase healing rate in treatment of perianal abscess with primary suture, the effect of intraoperative parenteral clindamycin and Gentacoll was investigated against monotherapy with intraoperative clindamycin in a randomized study. METHODS: One hundred seven patients, 55 in the Gentacoll group and 52 in the control group, were enrolled in the study and followed for three months. RESULTS: Twelve patients (22 percent) in the Gentacoll group developed a new abscess and/or fistula. In the control group nine patients (17 percent) developed a recurrent abscess or a fistula. In both groups 43 patients had an uneventful course. The differences between the two groups were not significant. Of all 107 patients, 19.5 percent had recurrent disease in the follow-up period. Duration of hospitalization and reconvalescence were identical in both groups. One case of superficial thrombophlebitis was seen after clindamycin. No other adverse effects to either Gentacoll or clindamycin were seen. CONCLUSION: The study shows that Gentacoll is a safe preparation but is without value as a supplement to clindamycin in the treatment of acute perianal abscess with primary suture. The study has documented the value of this treatment under cover with a intraoperative dose of clindamycin.

Abscess

Screening of the Philadelphia variant of galactokinase in racially unmixed black Africans: first results.

In previous reports, it was emphasized that the gene GALKA of galactokinase was the predominant allele in white populations and that another allele, GALKP, which reduces red blood cell activity (RBC GALK), was common in black people. In a group of black Americans living in Philadelphia, the frequency of GALKA was found to be very close to values expected from independent estimation of white admixture. The authors have suggested that the ancestors of these blacks might have been virtually all GALKP homozygous. We have looked for carriers of GALKP genotypes among 73 black Africans; only 33 probands were shown to have a low RBC GALK. To detect white admixture, immunoglobulin allotypes Km and Gm were investigated in 50 individuals of the sample; 15 GALKP carriers with low RBC GALK and 30 of 35 individuals with normal RBC GALK shared Gm phenotypes exclusive to blacks. Our work demonstrates for the first time the polymorphism of GALK in black Africans in the absence of white admixture.

Africa

alpha-Thalassemia among sickle cell anemia patients in various African populations.

We have studied the incidence of alpha-thalassemia in normal and SS individuals from Senegal, Benin, Upper Volta, and Central Republican Africa. The alpha thal gene frequency is not significantly different in the controls from the various populations and in the SS patients from Senegal. In contrast it is compatible with increased survival of SS patients in Benin, Upper Volta. The data suggest epistatic effects of other factors in the Senegalese population.

Adolescent

[When should cholecystectomy in acute cholecystitis be planned?].

Acute cholecystitis may be treated either by removal of the inflamed gallbladder during the acute stage of the disease or by conservative measures followed later by cholecystectomy. Many authors recommend delayed operation in view of the possibly higher postoperative mortality with early operation. 394 early cholecystectomies for acute cholecystitis have been performed between 1970 and 1979 at the University Hospital of Basle. 14 patients died postoperatively, representing a mortality rate of 3.5%. Large series in the literature show similar mortality of 3.2-4.5%. In four prospective randomized studies no significant difference of the mortality rate has been demonstrated. One retrospective study of the two methods showed a reduction in mortality rate from 7.4% for late operation to 2.7% for early cholecystectomy. Based on our own studies and on the literature, we have come to the conclusion that early cholecystectomy must be recommended for acute cholecystitis. Its advantages are shorter hospital stay, less patient discomfort since there is only one hospitalization, and reduction of costs. These advantages are also coupled with a similar or even lower mortality rate.

Acute Disease

HLA-A and B antigens in AKA pygmies.

HLA-A and B antigens were studied in 543 AKA pygmies. The present analysis showed two characteristics of this pygmoid group: the absence of HLA-A1, A11, B8 and Bw38 and the high frequency of Aw30, B17, B27, B37, B40 and Bw39. The strongest gametic associations were found with the haplotypes Aw30, B37 and A3, B5.

Black People

Gc, Tf, Hp subtype and alpha 1-antitrypsin polymorphisms in a Pygmy Bi-Aka sample.

Protein polymorphism is studied in more than 900 serum samples during different investigations conducted in a Bi Aka Pygmy group. The Gc, Tf and alpha 1-antitrypsin subtype polymorphisms were determined after isoelectric focusing while the haptoglobins alpha and alpha 2-peptides were studied on PAGE. A high frequency of the Hp2 gene is noted while Hp1F and Hp1S gene frequencies are similar. According to the Gc1S and Gc2 gene frequencies this group falls within the cluster of the melanoderm populations such as the Sara, Bantu and Peulhs. The two subtypes of TfC1 and TfC2 are present in this sample. TfC3 is absent. The TfD1 variant frequency is one of the highest observed in African groups. The alpha 1-antitrypsin polymorphism corresponds to the presence of the three PiM subtypes. No other variants are observed, neither PiS nor PiZ. For the first time a highly significant association is described between the TfD1 and Gc1A1 (GcAb) genes. Family pedigrees do not permit the ascertainment of the linkage between the two loci.

Black People

Gc (vitamin D binding protein) subtype polymorphism and variants distribution among Saharan, Middle East, and African populations.

This article presents the results obtained by electrophoretic analysis of the group specific component polymorphism in more than 1,250 serum samples from populations living in the Sahara, the Middle East, and equatorial Africa. In addition to the alleles Gc1F and Gc1s, five variants, including one previously unknown, were found. The distribution of the alleles herein described permits speculation on exchanges and relations among the groups considered. The lowest frequencies of the gene Gc2 correspond to regions where sunlight is stronger. There is also a north-south gradient in the Gc1F gene frequency. This seems to parallel the gradient seen in skin pigmentation.

Africa, Northern

Population genetic studies of the Aka pygmies (Central Africa): a survey of red cell and serum enzymes.

Blood samples collected in a single Pygmy tribe, the Aka, living in Bokoka district (Central African Empire) were investigated with respect to the phenotype and gene frequencies of the following 12 enzyme systems: acid phosphatase, adenosine deaminase, adenylate kinase, carbonic anhydrase, esterase D, glucose-6-phosphate dehydrogenase, malate dehydrogenase, phosphoglucomutase 1, phosphoglucomutase 2, phosphogluconate dehydrogenase, superoxide dismutase and serum cholinesterase variants (locus E1 and E2). The data obtained in the study of genetic polymorphisms of this isolated and inbred population show a specific pattern with the following characteristics: the very low frequency of PGDB and pa alleles; the existence of two rare PGM variants at the PGM2 locus, typical PGM26Pyg (4.2%) and PGM29 (0.2%); the high frequency of the pr allele (10.8%) and CAII2 (8.22%) and ESD2 genes (18.4%). Furthermore, at the G6PD locus four distinct alleles have been found: the negroid GdA- (4%) and GdA+ (16%), the common GdB+ (79.2%)--, and the rare Gd+Ibadan Austin (0.7%). Cholinesterase typings disclosed the presence of the uncommon E1f and E1s genes distributed within a single breeding unit. The results are compared with other data previously reported on South African Khoisan and some Negroid populations; the particular genetic background of Pygmies is discussed.

Black People

Genetic variants of human glucose-6-phosphate dehydrogenase in a Saharian and Pygmy family.

In two African communities, inhabitants of a Western Sahara oasis and Bi-Aka Pygmies (Central Africa), a genetic study of the distribution of G6PD phenotypes has been undertaken. Obtained data show the existence in both groups of slow electrophoretic variants with no enzyme deficiency or moderately reduced activity. Biochemical characterization of G6PD types was performed. In the Saharian family in which inheritance pattern of mutant G6PD was investigated, two alleles were found, the Negroid marker GdA- and Gd+Madrona, segregating among the different members. In the Pygmy family the Gd+Ibadan-Austin gene was detected. The incidence of these mutations in the groups studied, a comparison with similar G6PD variants observed in other African populations and the geographic distribution of these slow molecules are discussed in this paper.

Africa, Central