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Biomedical subjects

F Janssen

Publications and source records attributed to F Janssen.

At least 55 records · Page 3Linked to original sources
PubMed1989

Pyoderma gangrenosum with pulmonary involvement.

A 60-year-old woman had a typical pyoderma gangrenosum with monoclonal IgA gammopathy and atrophic gastritis. Two years after the onset of her skin disease, she had evidence of pulmonary abscesslike involvement. Corticosteroid therapy led to healing of skin and lung diseases. This case stresses the multisystemic manifestations of neutrophilic dermatoses with special attention to pulmonary involvement.

Drug Administration Schedule↗
PubMed1988

Long-term follow up of a case of severe congenital chloride diarrhoea.

A patient with congenital chloride diarrhoea (CCD) is reported. The diagnosis on the 5th day of life was based on the watery stools with characteristic ionic composition. The neonate presented with abdominal distension necessitating laparotomy on day 1, followed by transverse colostomy on day 3, owing to persistent abdominal swelling. Total parenteral nutrition (from 1 to 3 months) followed by an enteral semi-elemental diet (from 3 to 5 months) was required to reduce the quantity of stools and to obtain a satisfactory nutritional state. The colostomy was closed at 16 months. Growth, psychomotor development and renal function are normal at 6 years. Criteria for therapeutic evaluation are discussed.

Blood Chemical Analysis↗
PubMed1987

Atypical neutrophilic dermatosis with subcorneal IgA deposits.

A 26-year-old woman had a chronic vesiculopustular and ulcerating skin disease associated with fever and arthritis. Cutaneous biopsy specimens showed an extensive infiltration of the dermis and epidermis by neutrophils. Direct immunofluorescence (IF) revealed linear subcorneal IgA deposits. Indirect IF showed IgA antibodies reactive with the subcorneal zone of normal epidermis. The disease responded to dapsone therapy. The association between neutrophilic dermatoses, including pyoderma gangrenosum, subcorneal pustular dermatosis, and related entities, and IgA involvement, either IgA gammopathies and/or intraepidermal IgA deposits, is emphasized. Intraepidermal IgA deposits are possibly involved in the pathogenesis of our patient's condition and of other cases of unusual neutrophilic dermatoses.

Adult↗
PubMed1985

Ureteral stenosis after kidney transplantation: true incidence and long-term followup after surgical correction.

Between March 1965 and December 31, 1982, 421 kidney transplantations were performed in our department. The over-all incidence of ureteral stenosis was 5.5 per cent. However, when the number of patients at risk at various times after transplantation was considered the probability for ureteral stenosis to develop was 4.6 per cent at 1 year, 7.7 per cent at 2 years and 9.7 per cent at 5 years. Preoperative and postoperative complications were frequent. The wound infection rate was 21.9 per cent and 2 of the 24 patients died of septic shock. Graft survival rate after definitive surgical correction of ureteral stenosis was 71 per cent at 1 and 2 years, and 65 per cent at 3 years. Late results justify the efforts to re-establish correct urinary drainage of the graft.

Follow-Up Studies↗
PubMed1985

Acute valproate intoxication with fatal outcome in an infant.

A healthy twenty-month-old boy ingested a maximal dose of valproate from which about 750 mg/kg were absorbed. Cerebral coma, which lasted for twenty hrs, was followed by an undisturbed period of approximately sixteen hrs. Death from cardiorespiratory failure due to severe bronchopneumonia occurred 46.5 hrs after the ingestion of the drug. The serum valproic acid concentration reached a peak of 1061 micrograms/ml within three hours, and fifteen minutes before death it had fallen to 187 micrograms/ml. The half-life of 16.6 hrs was within the range usually found. Metabolic acidosis, hypernatraemia and hyperosmolarity could be corrected, unlike the hypocalcaemia, which developed later. Bilirubin, GOT, GPT, gamma-GT, alkaline phosphatase, blood glucose, diastase, urea, creatinine, haemoglobin as well as PT and PTT and the platelet count were all normal. Leucopenia with 1,600 per microliter developed only during the bronchopneumonial stage. The histo-pathological findings were acute hypoxic damage of the myocardium, kidneys and certain neurones of vulnerable areas of the brain (neuronal microvesiculation and tigrolysis) in addition to a severe cerebral oedema in the final stage. A morphological substrate of an acute valproate encephalopathy was not demonstrable. The liver showed no necrosis or cholostasis. The vertebral marrow was inconspicuous. All the results indicate that liver function was not impaired in spite of the initial maximal concentration of valproic acid. In all probability the patient might have survived the acute valproate intoxication had it not been for the bronchopneumonia.

Brain↗
PubMed1984

Islet-cell antibodies (ICA-CFICA) and HLA genotypes in 107 IDD patients and their first-degree relatives.

The prevalence of ICA and CFICA in relation to HLA-DR genotypes was analyzed in 107 insulin-dependent diabetic (IDD) patients with a duration of IDD ranging from onset to 30 years, and 247 nondiabetic first-degree relatives. In 46 patients tested at onset of IDD, the prevalence of ICA and of CFICA was 61 and 50%, respectively; with the longer duration of IDD, the prevalence of CFICA decreased more rapidly than that of ICA. No significant association was observed between ICA and any HLA allele in patients tested from onset till 2 years after onset. However, a higher prevalence of ICA was found in DR3 positive patients with a duration of IDD of more than 2 years (p less than 0.02). Among the healthy relatives, the prevalence of ICA was 7% in parents and 13% in siblings; one out of 101 siblings had CFICA. No association was found between ICA and any HLA marker. The presence of ICA in sibs was independent of the number of haplotypes they shared with the IDD proband: among the 13 ICA-positive healthy sibs, one shared 2 haplotypes, nine shared 1 haplotype and three shared no haplotype with the proband, which is not different from the random distribution.

Adolescent↗
PubMed1983

Elbow arteriovenous fistula (EAVF) for chronic hemodialysis in small children.

Elbow arteriovenous fistulae (EAVF) were constructed in 10 children aged 27 mo to 10 yr. There were no operative failures. Follow-up ranged from 5 to 22 mo. Three episodes of thrombosis occurred in a single child. The fistula was recanalized each time. The creation of an EAVF is a simple surgical procedure. Children weighing as little as 10 kg were efficiently dialysed for prolonged periods of time with this vascular access device.

Arteriovenous Shunt, Surgical↗
PubMed1982

Mobility of endogenous ecotropic murine leukemia viral genomes within mouse chromosomal DNA and integration of a mink cell focus-forming virus-type recombinant provirus in the germ line.

Characterization of endogenous ecotropic Akv proviruses in DNA of low and high leukemic mouse strains revealed the presence of one to six copies of the Akv genome per haploid genome equivalent integrated in the germ line. Low leukemic strains analyzed so far contained only one complete copy of the Akv proviral DNA. The site of integration varied among strains, although genetically related strains often carried the Akv proviral gene in the same chromosomal site. The different substrains of the AKR mouse displayed the presence of variable numbers (two to six) of Akv genomes. In all substrains one Akv genome was present in an identical chromosomal site; this locus probably comprised the progenitor genome. Closely related substrains had several Akv proviral DNAs integrated in common sites. The accumulation of Akv genomes in the germ line of the AKR/FuRdA strain is likely the result of independent integration events, since backcross studies with the Akv-negative 129 strain showed random segregation of all six proviral loci. The AKR/Cnb strain carried a recombinant provirus in the germ line. This provirus resembled in structure the AKR mink cell focus-forming viruses, which are generated by somatic recombination during leukemogenesis. Therefore, the germ-line amplification of Akv proviral DNAs occurs most likely through infection of embryonic cells by circulating virus.

AKR murine leukemia virus↗