Biomedical subjects
Publications and source records attributed to J Janda.
Authors describe a case of a very severe poisoning, the prothrombin-time was less than 10% of the normal value and the child developed hepatic coma. We suppose, the favourable outcome may have been influenced by the treatment with Silymarin in combination with high doses of G-Penicillin.
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We describe a case of a 9-year-old boy with the severe acute liver injury caused by idiosyncrasy after the administration of valproate. The liver biopsy performed during the first days after the onset of the disease revealed the necrosis of more than 50% of hepatocytes but the injury was almost fully reversible which was well demonstrated by the second biopsy five months later. After five years an evidence for the immunologic idiosyncrasy caused by valproate was still present, which was demonstrated in vitro by blastic transformation of lymphocytes from the patient. The boy is quite healthy and epilepsy is under control of suximide. The family was informed that the boy could be treated with valproate never more.
Dialysis and transplantation of the kidney have become in Czechoslovakia part of routine therapy of chronic renal failure (ChRF) in children and adolescents. The authors examined 16 children aged 4-17 years (x = 10.5) treated in a chronic dialyzation programme (ChDP) by haemodyalisis and 20 children after transplantation of a cadaverous kidney, age 4.5-16.5 years (x = 11.5), incl. 14 patients with a stable function of the graft and six patients during the period of acute rejection of the graft. The authors tested the basic parameters of humoral and cellular immunity in all three groups of children. In the group of the CRI a significant drop of the level was found only in serum IgA, the other parameters, incl. examination of sub-populations of lymphocytes CD3+, CD4+ and CD8+, did not differ significantly from normal values. In patients with a stabilized function of the graft treated by Cyclosporin A, prednisone, and in some cases also with azothioprim, the authors found a significantly lower absolute number of lymphocytes of the mentioned sub-populations as compared with controls and patients included in a ChDP. During the period of acute rejection the absolute number of CD3+, CD4+, CD8+ increased significantly, as compared with the period of stabilized function of the graft. Thus patients in CRI do not manifest despite the serious affection of the organism serious changes of immune reactivity within the scope of examinations made by the authors. Monitoring of immunity parameters in patients with transplants can contribute to evaluation of the effect of immunosuppression (it can draw attention to low dosage of drugs) and in the context with other clinical and laboratory examinations it can contribute to the diagnosis of acute rejection.
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The authors examined a total of 107 healthy subjects--neonates (n = 13), infants (n = 23), toddlers (n = 16), 3-6-year-old children (n = 15) and school children (n = 40). They correlated the urinary Ca excretion mmol/kg/24 h) with the Ca/cr index which was obtained by examination of postprandial urine sample. They revealed a significant correlation of the excretion and index in infants p less than 0.001, in school children p less than 0.01 with a regression coefficient of 0.9 and 0.7 resp. The index was significantly age dependent, the value of the 95th percentile in neonates was 1.38, in infants 1.025, in toddlers 1.26, in three-year-old children 0.835 and in school children 0.56. The urinary Ca/cr values must be thus interpreted in relation to the child's age; the index declines with age. The reason for high indices in young children is the low creatinine concentration in the excreted urine which rises significantly with age. Examination of the Ca/cr index should be part of routine screening in all cases with suspected nephropathy. The accepted upper range of the index, 0.6 (mmol/mmol), applies only to school children, but not to younger children.
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In 1984-1989 at the First Paediatric Clinic of the Motol Faculty Hospital six patients with the diagnosis of infantile cortical hyperostosis (Caffey-Silvermann syndrome) were hospitalized. Non-familial concurrence of the disease was involved. All children developed the disease before the age of 4.5 months, four before the age of three months. Among the six patients was only one girl. As to pathological findings there was a markedly elevated FW in 5/6, leucocytosis in 5/6, thrombocytosis in 3/4, eosinophilia in 3/6, elevated alkaline phosphatases in 2/6. The affection was four times on the mandible, once in the area of the hip joints and in one instance it was multifocal. The authors present one detailed case-history with extreme affection of the clavicle which was originally taken for a bone tumour.
Reduction of the renal parenchyma in experimental animals can cause glomerulosclerosis in the single kidney and lead sometimes to functional failure. Data on the influence of nephrectomy or agenesis on the later fate of the patient in human medicine are controversial. The authors examined a total of 40 patients with single kidneys. In 23 agenesis was involved (15 boys, 8 girls) aged 6-22 years (mean = 11.9, s = 4.3). In 17 nephrectomy was performed (9 boys, 8 girls); time interval after nephrectomy 1-26 years (mean = 7.8, s = 3.9). Three quarters of all patients excreted increased amounts of albumin (detected as microalbuminuria by immunoturbidometry). The glomerular filtration rate (GFR) was slightly reduced in about one third of the patients but none suffered from renal insufficiency. In one half of the group the authors detected a slightly impaired concentrating capacity with an increased sodium fraction. In one third marginal diastolic hypertension was recorded. In half the group the single kidney was hypertrophic. Regression analysis did not reveal deterioration of the GFR in relation to age but revealed increasing renal hypertrophy in relation to height with accelerated hypertrophy during puberty. There was also a significant drop of the systolic blood pressure in relation to age in patients with agenesis. In none of the other parameters differences were found between the group with agenesis and nephrectomy. Patients with a single kidney deserve prolonged ambulatory follow up. In the investigated group the single kidney influenced in a substantial way also the selection of occupation.
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Fc region fragments derived from the enzymatic cleavage of human IgG have been shown to induce human peripheral blood-derived B cells to differentiate into Ig secreting cells (ISC). The synthetic peptide p23, corresponding to residues 335 to 357 in the Fc region of human IgG1, represents a region of the molecule responsible for stimulation of ISC formation. Fc region-induced ISC formation requires at least two signals; one supplied by Fc region activators and one supplied by a T cell-derived factor(s). In this report we show that the coculture of human PBMC with pFc' or p23, results in the release of factor(s) that resemble IL-6 in its pattern of biologic activity. This conclusion is based on the observations that supernatants from Fc region-stimulated PBMC cultures contained increased levels of elements that scored as positive in two assays for IL-6: the B9.9 hybridoma growth and the CESS cell differentiation assays. Moreover, RNA from Fc region-stimulation PBMC contained increased levels of IL-6 cDNA-hybridizable elements. Finally, it was observed that rabbit anti-IL-6 inhibited the ability of supernatants derived from Fc region-stimulated PBMC cultures to induce B9.9 cell proliferation as well as p23-induced ISC formation in intact PBMC cultures. Fc region fragments induce both monocytes and T cells to produce IL-6. Taken together, these results indicate that IL-6 is produced in Fc region-stimulated PBMC cultures and is involved in B cell activation by these activators.
The authors explain the principle of central venous catheters (CVC) for long-term use, indicators for insertion, mode of insertion and principles of care of children with these special CVC. The authors inserted since 1982 42 CVC of the Broviac-Hickmann type in 32 children (age 22 days--15 years; body weight 2.4-17 kg). Since 1987 they inserted 16 CVC for long-term use with a subcutaneous capsule to 16 children and adolescents (age 1 years-17 years; body weight 11.5-73.5 kg). These CVC were inserted under general anaesthesia, in catheterization theatre. There were no technical complications. The authors treated and followed under standard protocol a group of 14 children with 20 CVC of the Broviac type (age at onset of treatment 22-715 days; body weight 2.4-10 kg) as well as the above described group of 16 children and adolescents with CVC for long-term use with a subcutaneous capsule. During treatment of children with Broviac catheters five technical complications were observed (mechanical damage of the CVC); in children with CVC with a subcutaneous capsule one technical complication was recorded (damage of the chamber of the Chemoport Vygon system, by incorrect puncture). The authors searched for thromboembolic and infectious complications. In four of 20 children with Broviac catheters purulent phlebitis of the cannulated veins was found (all died)--one thromboembolic complication developed on average after 234 days of use of Broviac catheters.(ABSTRACT TRUNCATED AT 250 WORDS)
We report a 34-year-old woman with a cervical chordoma consisting of an intraspinal and extraspinal portion. The extraspinal component of the tumor invaded the deeper structures of the left side of the neck, and was subjected to repeated partial removal. It was misinterpreted as pleomorphic adenoma of salivary gland origin, whereas the intraspinal portion was a neurilemmoma with unusual mucous degeneration.
Reported is a nasal oncocytoma with locally invasive properties in a 86 year old man. The tumor was histologically found to consist of cells rich in cytoplasm, with striking eosinophilia, granularity, and predominance of glandular architecture. Electron microscopy revealed typical oncocytes with abundant mitochondria in the cytoplasm, with many of them compressing the nuclei of tumor cells. The tumor reported was distinguished from unambiguously benign oncocytomas by its mitotic activity and locally invasive character. These properties should be noted in histologic diagnosis. Locally delimited but radical tumor removal is the adequate therapy.
Acute non-suppurative tubulointerstitial nephritis was recorded within a five-month interval in 1988 in one girl and two boys aged 15, 16 and 12 years. The common feature was non-selective proteinuria, slight glycosuria, anaemia, a sedimentation rate of more than 100 mm/2 hrs hyperatotaemia not calling for dialyzation treatment (268, 354 and 266 mumol/l plasma creatinine resp.), a markedly impaired concentrating capacity (540, 593 and 520 mOsm/kg urine resp.). In all patients circulating serum immunocomplexes were elevated (PEG-IKEM). One patient developed acute uveitis at the onset of the disease, the remainder after 5 and 6 months resp. and in all there was a tendency of a protracted course and relapses resp. In two patients uveitis was diagnosed by an aimed examination by means of a slit lamp at a time when there were not yet any clear signs of affection of the eyes. All patients were subjected to percutaneous renal biopsy which revealed an interstitium with uneven lymphoplasmacytic cellulization with infrequent eosinophil and neutrophil polynuclear cells. Electron microscopy revealed sections of varying size with fibrotization of the tubular basal membrane; the glomerular changes were not typical. All patients had prednisone treatment and their renal functions were gradually restored. Despite extensive serological examinations, the aetiology was not cleared, however before the onset of the disease the patients had penicillin, cotrimoxazol and erythromycin resp.
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In their review the authors discuss the history of Alport's syndrome, its nomenclature, incidence, genetics, clinical diagnosis. The authors emphasize electron microscopic findings and criteria of the diagnosis (haematuria or renal failure in the family, progressive nervous deafness, typical changes of the basal glomerular membrane (GBM) and ophtalmological findings of lenticonus or perimacular spots. Familial haematuria (FH) is according to the authors defined as haematuria in several members of the family. Based on data in the literature and the authors' experience, the authors discuss the differential diagnosis of FH where Alport's syndrome is relatively rare. A far more frequent unit is benign familial haematuria characterized morphologically as isolated thinning of the GBM. In some cases these patients are threatened by iatrogenic damage from unnecessary and invasive diagnostic method. The finding of thinned GBM and normal renal function in the parents and grandparents suggest a favourable prognosis also in child patients. Cases of familial glomerulonephritis or idiopathic syndrome with glomerulosclerosis or familial IgA nephropathies are relatively rare. Familial haematuria is are relatively rare. Familial haematuria is relatively frequent (according to the authors 20% of all obscure haematurias) and their diagnosis is based on systematic examination of the urine in other members of the patient's family who also suffer from haematuria.