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Biomedical subjects

J Hoza

Publications and source records attributed to J Hoza.

At least 19 recordsLinked to original sources
PubMed1998

[Lactate acidosis in childhood].

BACKGROUND: Secondary lactate acidosis is found in children with hypoxaemia, with impaired tissue perfusion, in hepatic and renal failure or in intoxications. Primary lactate acidosis is usually caused by hereditary metabolic disorders. The objective of the trial was to analyze the causes which lead in childhood to the development of primary hyperlactacidaemia. METHODS AND RESULTS: The authors examined during 1995-1996 the lactate and pyruvate concentration in 479 children referred by paediatric and neurological departments with a suspect hereditary metabolic disturbances. A raised lactate in blood or cerebrospinal fluid > 2.3 mmol/l was found in 230 children incl. 49 where a metabolic disorder was detected. Ten children had impaired cytochrome c oxidase, two children had a combined deficience of NADH dehydrogenase and cytochrome c oxidase, three children had a deficience of the pyruvate dehydrogenase (PDH) complex, one child had a deficience of ATP synthase and seven children suffered from impaired beta-oxidation. Glycogenosis type I, III or IX was found in 13 children. In three children organic aciduria was found, two children had an impaired urea cycle and three children impaired fructose metabolism. In five children a low level of free and total carnitene was found as a result of valproate treatment. A significant increase of the lactate level by more than 1 mmol/l during an oral glucose load was found in 11 of 16 children with impairment of the respiratory chain or PDH complex. In 58 children concurrently lactate in blood and cerebrospinal fluid assessed but no correlation of lactate levels was found. CONCLUSIONS: In patients with suspect hereditary metabolic disorders examination of lactate, pyruvate and alanine levels can be considered a screening test for detection of mitochondrial disorders. It remains difficult to reveal the cause of hyperlactacidaemia in a sick child even if a wide range of laboratory methods are used which contribute to the diagnosis of hereditary metabolic disorders.

Acidosis, Lactic↗
PubMed1997

[Enzyme therapy in children with severe forms of Gaucher's disease].

The enzyme therapy with Ceredase in patients with Gaucher's disease is at present probably the most expensive treatment in the whole world. One-year treatment of an adult patient with Gaucher's disease costs more than 7 million crowns. Indications for treatment in individual patients as well as financial provisions are so far problematic in the Czech Republic. From a total of 28 patients of varying age with Gaucher's disease diagnosed by the authors Ceredase was administered to two boys with a severe course of the disease. Within one year of treatment the health status of both children improved, growth became normal, the spleen diminished in size by 20-35%, haematological manifestations of hypersplenism are receding, there was a 32-46% decline of the activity of serum chitotriosidase and biochemical parameters of the disease improved.

Child↗
PubMed1994

The effect of long-term glucocorticoid therapy on glucocorticoid receptor content and on steroid response to ACTH.

The effect of long-term glucocorticoid therapy for systemic diseases on glucocorticoid receptor (GR) content and on basal and ACTH-stimulated levels of plasma and salivary cortisol 17 alpha-hydroxy-progesterone, androstenedione, 11 beta-hydroxyandrostenedione, DHEA, its sulfate and sex hormone-binding globulin (SHBG), as well as on basal levels of aldosterone, was investigated in a group of 24 children treated with prednisone for at least 8 months. The therapy was interrupted 24 h before the ACTH test and before plasma and saliva sampling. The control group consisted of 21 healthy children of corresponding age and sex. The patients were divided into two subgroups with normal and subnormal basal cortisolemia, they also differed in their response to ACTH. The GR levels in patient groups were indistinguishable from those found in controls. No correlation was found between GR content and basal levels of the above steroids or their response to ACTH. The best markers, apart from basal cortisolemia, for evaluation of the degree of suppression of adrenal function appeared to be the response of salivary (but not of plasma) cortisol and 17 alpha-hydroxy-progesterone to ACTH. Surprisingly, significantly lower levels of SHBG levels, which rose markedly after ACTH, were found in all the patients.

Adolescent↗
PubMed1994

Juvenile chronic arthritis--bone mineral density in relation to corticosteroid therapy.

Bone demineralization often accompanies juvenile chronic arthritis (JCA). Fourteen patients with confirmed diagnosis of JCA had their bone mineral density (BMD) measured with the use of dual photon X-ray absorptiometry. The results obtained were compared to the Lunar BMD DPXA standards. Seven patients received Prednisone in doses of more than 0.16 mg/kg/day for more than 6 months and 7 patients (sex and age matched) never received any steroids at all. In the first group BMD was decreased in 7 patients (100%), in the second group BMD was mildly decreased in 2 patients (28%). Due to the difference in BMD in both groups, it is obvious that corticosteroids have substantial influence on bone demineralization in JCA.

Adolescent↗
PubMed1994

Calciuria in children with juvenile chronic arthritis.

Demineralization of bone is a frequent finding in children with juvenile chronic arthritis (JCA). Recently there have been reports about hypercalciuria accompanying JCA. This is believed to be associated with increased bone resorption due to cytokines and immobility of the patients and steroid treatment. In 12 patients with confirmed diagnosis of JCA basic biochemical indices of bone metabolism, were performed (S-Ca, P, ALP, U-Ca/U-creatinine, U-P). Bone mineral density (BMD) was measured using DPXA method and results obtained were compared to the Lunar BMD DPXA standards. In spite of decreased BMD, no significant hypercalciuria was found and other mentioned biochemical indices of bone and mineral metabolism were normal as well.

Adolescent↗
PubMed1993

Class I associations and frequencies of class II HLA-DRB alleles by RFLP analysis in children with rheumatoid-factor-negative juvenile chronic arthritis.

A total of 94 patients with juvenile chronic arthritis (JCA) was tested for HLA class I by serology and for class II by RFLP typing. Early onset JCA (EOPA) is associated with HLA-A2, DR5 and DR8 in both males and females. The combination (joint occurrence) of these JCA associated alleles (A2, DR5, DR8) is frequently seen in patients with chronic iridocyclitis. Late onset pauciarticular disease has an increased frequency of HLA-B27, especially in males. Our data confirm that polyarticular JCA with early childhood onset (< or = 4 years) is associated with DR5 and DR8 and has a different immunogenetic background from polyarticular JCA with later childhood (> 4 years) onset (associated with DR4).

Adolescent↗
PubMed1993

The SAPHO syndrome.

We report two cases of the SAPHO syndrome (synovitis, acne conglobata, pustulosis palmoplantaris, hyperostosis and osteitis). This syndrome has been published in the pediatric and rheumatological literature in recent years.

Acne Vulgaris↗
PubMed1991

A model for the role of HLA-DQ molecules in the pathogenesis of juvenile chronic arthritis.

Restriction fragment length polymorphism (RFLP) typing of MHC-class II loci DRB, DQA1, DQB1, DQA2 and DPB1 was performed in 94 patients with seronegative juvenile chronic arthritis (JCA) and 184 random controls. Analysis of allele frequencies and MHC-class II 4-loci haplotypes indicate: (1) Susceptibility to JCA is more strongly associated with the HLA-DQ subregion than with the HLA-DR subregion, especially in early onset pauciarticular JCA (EOPA-JCA). (2) Haplotype and sequence analysis show two independent MHC-class II associations for susceptibility to EOPA-JCA, one located in DQA1, the other in DPB1. (3) Two RFLP defined patterns of the DQA1 locus, DQA1.5 (DQA1*0501) and DQA1.8 (DQA1*0401, *0601) are strongly associated with the disease. (4) Analysis of amino-acid (AA) sequences coded in exon 2 of DQA1 reveals an AA sequence of six AAs common to all three associated DQA1 alleles. This suggests a model that includes a functional role for HLA-DQ molecules in the pathogenesis of JCA.

Alleles↗
PubMed1991

Sulphasalazine and Delagil--a comparative study in patients with juvenile chronic arthritis.

Thirty nine consecutive patients with pauciarticular and polyarticular juvenile chronic arthritis were randomised to treatment with either sulphasalazine or Delagil (chlorochinum diphosphoricum) in a parallel 6 month clinical trial. We compared clinical and laboratory signs of activity before and after the treatment. Of 21 patients with Sulphasalazine 10 were improved, 7 unchanged and in 4 patients had the therapy to be withdrawn. Of 18 patients with Delagil 5 were improved, 12 without effect and withdrawal was necessary in 1.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed1989

Comparative effects of methylphenidate on ADD girls and ADD boys.

The effects of 0.3 mg/kg methylphenidate were compared for 12 ADD boys and 12 ADD girls participating in a summer treatment program for children with behavior and/or learning problems. Previous investigations have suggested that ADD girls may differ from ADD boys in some important respects. No information exists regarding whether the effects of the most common treatment for these children, methylphenidate, has comparable effects on boys and girls. The boys and girls were matched for age and IQ. The results revealed equivalent and beneficial effects of methylphenidate for both boys and girls. Methylphenidate therefore would appear to be as useful a treatment for ADD girls as for ADD boys.

Attention Deficit Disorder with Hyperactivity↗
PubMed1989

[Sulfasalazine in the treatment of chronic juvenile arthritis].

In 38 children with juvenile chronic arthritis the authors tested Sulphasalazine in an open clinical trial. The drug was administered for 26 weeks, 20-30 mg/kg/day. The action of the drug was followed up with regard to clinical (number of criteria of juvenile chronic arthritis, functional state, period of morning stiffness, number of affected joints painfulness of joints) and laboratory (red cell sedimentation rate, circulating immunocomplexes) indicators. A favourable effect (improvement of at least five of the above indicators) was recorded in 42.1% of the children. Undesirable effects were found in 11 children (30%) incl. six where they were the cause of discontinuation of treatment. All undesirable effects were reversible. The majority of them (exanthema, headache, leukopenia, microscopic haematuria) developed during the first 3-4 weeks of treatment.

Adolescent↗