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Biomedical subjects

Z Misíková

Publications and source records attributed to Z Misíková.

At least 19 recordsLinked to original sources

[Growth and puberty in children after treatment of acute lymphoblastic leukemia].

BACKGROUND: Growth retardation and other endocrine abnormalities were recognized as sequelae of therapy of acute lymphoblastic leukemia (ALL), especially when chemotherapy was combined with cranial irradiation. The aim of our study was to establish growth and pubertal development in the group of children who had been previously successfully treated for ALL. METHODS AND RESULTS: 58 children (30 F, 28 M, age 8-18 y.) in complete initial remission lasting 4-15 y. (m 8 y.) after standard antileukemic therapy (including cranial irradiation) completed 2-9 y. ago (m 3 including y.) were studied. Standard deviation score (SDS) of standing height (SH) to chronological age (CA) and to genetic target height (GTH), index of body proportionality and timing of puberty were followed up. The final height attained 21 girls and 3 boys. The height of the boys differed neither from the average for our men, nor from their GTH. Girls: SH 148 cm-169 cm, SH of 7 girls > or = -1.5 SDS, 8 girls > or = -1.5 SDS to their GTH. The final height didn't reach yet 23 boys and 9 girls. Their SH to CA as well as SH to GTH didn't substantially differ from average. Index of body proportionality > or = 1.5 SDS in 12/30 girls and 6/28 boys. Menarche was already reached in 25 girls in age ranging from 10-15 y. (mean 11.3 y.), what was less than average for our population (13.3 y.). CONCLUSION: Even the height of prepubertal children under study didn't differ from average, the final height of girls (boys could not be evaluated because of small numbers) was significantly lower as could be expected. We suppose the early ending of puberty as a contributing factor of short stature in girls. Clear tendency to obesity especially among older girls was observed. Children who underwent antileukemic therapy deserve careful endocrinological follow-up.

Adolescent↗

[Surgical correction of genital virilization in girls with congenital adrenal hyperplasia].

Based on an analysis of 10 patients after a feminizing operation of a virilized genital in female pseudohermaphroditism caused by congenital adrenal hyperplasia on account of 21-hydroxylase deficiency the authors emphasize in conjunction with multidisciplinary collaboration the necessity of early modification and surgical repair with adjustment of the external genital before the age of two years. An excellent anatomical and cosmetic result of reconstruction with clitoroplasty emphasizes the importance of preserving the clitoris and erectile tissue to ensure adequate social, psychic and sexual development of the patient.

Adrenal Hyperplasia, Congenital↗

[Growth hormone therapy in prepubertal children with chronic renal insufficiency].

BACKGROUND: Growth retardation is a serious problem in children with chronic renal failure (CRF) despite normal endogenous growth hormone (GH) secretion. Intensive medical care and dialysis do not improve height velocity. The aim of the study was to evaluate the efficacy of GH therapy in children with growth retardation secondary to CRF. METHODS AND RESULTS: Biosynthetic growth hormone was given to 7 prepubertal children (five boys and 2 girls, age with a range 3.5-14.5 years) with severe growth retardation and CRF during 1 year. The dosage of GH was 1 IU/kg/per week. GH was given daily, sc. The patients had a full examination every 3 months. Six children completed the study. Height velocity improved with GH therapy in 5 children. Renal function deterioration was accelerated in 2 children. The serum alkaline phosphatase concentration increased and the serum calcium concentration decreased during GH treatment in all children. Glucose, thyroid gland hormone concentration and lipid concentrations remained constant. CONCLUSIONS: The study showed clearly that treatment with biosynthetic GH gave an impressive improvement of height velocity in growth retarded prepubertal children with CRF.

Adolescent↗

Early diagnosis of medullary thyroid carcinoma in a 13 years old girl.

Early stage of medullary thyroid carcinoma was diagnosed in a 13 years old girl from the family with incidence of MEN IIa. High level of calcitonin after pentagastrin stimulation was crucial for the diagnosis. Pentagastrin test as a regular screening for medullary thyroid carcinoma for all children over 3 years of families with MEN IIa is recommended.

Adolescent↗

[Treatment of hypogonadism in childhood].

Specificity of therapeutic approaches to hypogonadism in childhood and the need of high standard hormonal preparations are stressed. Male hypogonadism can in rare instances be treated even during infancy. Therapy of hypogonadism during puberty is, however, more common. The general rule for therapy of pubertal hypogonadism is to mimic physiological puberty, i.e. to begin with small doses and slowly increase them gradually to reach the level of adult dosage within 4-5 years.

Adolescent↗

Prognostic significance of testicular relapse in boys with acute lymphoblastic leukemia.

Among 107 boys with acute lymphoblastic leukemia who achieved initial complete remission after treatment according to two therapeutic protocols for standard and high risk leukemias, respectively, in 14 (13%) testicular leukemia was observed. Isolated testicular involvement was noted in 4, and combined with medullary relapse in 10 patients. The prognosis was worse in children with high risk leukemias (mainly in those with more than 20 x 10(9)/l white blood cells and/or with less than 50 x 10(9)/l platelets). Prognosis depended also on the type of relapse. It was better in isolated as well as in late testicular relapse. It was worse in combined relapses as well as in early testicular involvement. Bilateral wedge biopsy of the testes was performed in 16 boys at the end of chemotherapy and leukemic infiltration was shown in none of them. Two to 13 months thereafter, in four boys testicular relapse developed, and in 2 boys bone marrow relapse followed 9 and 13 months later. Wedge biopsy of the testes in boys with leukemia does not seem to be of importance neither for diagnosis of testicular relapse nor for improving prognosis.

Biopsy↗

[Problems in children with growth hormone deficiency in Slovakia].

The authors give an account of 71 patients treated on account of complete growth hormone deficiency in Slovakia. Before the age of five the diagnosis was established in 24 children, after the age of 10 in 17 patients. Nine patients suffer from panhypopituitarism, while isolated deficiency of growth hormone was recorded in 34 patients. Perinatal pathology plays an important role in the aetiology. A tumour caused the disorder in five children. Treatment of the majority of patients was not systematic and was inadequate because of shortage of growth hormone. When regular substitution treatment was started before the age of 8 years, the therapeutic results were very satisfactory. The authors draw attention to the danger of further deterioration of care of children with growth hormone deficiency for economic reasons.

Child↗

[Somatic development after successful therapy of acute lymphoblastic leukemia].

The authors evaluated 55 subjects after successful treatment of acute lymphoblastic leukaemia in childhood as regards height, body weight and sexual maturation. In 23 the final height could be evaluated, which was somewhat lower than the mean height of the population, lower than could be expected according to the genetic potential and height before the diseases with a more evident deficit in women. There was an obvious tendency to overweight. Early onset of menarche was recorded.

Adolescent↗

Non-Hodgkin's lymphoma in children. Results of therapy.

Twenty-one children with non-Hodgkin's lymphoma (NHL) entered intensive chemotherapy study according to the protocol 6481. Sixteen patients with extraabdominal involvement achieved long-lasting remission, 15 of them have been disease-free for 17 till 77 months. Five children suffered from intraabdominal NHL, 4 of them expired because of progression of the disease within 4 months after diagnosis, 1 patient has been disease-free for 43 months. Actuarial disease-free survival is 0.76 for the whole group, 0.8 for those with extraabdominal involvement and 0.20 for children with intraabdominal forms of non-Hodgkin's lymphoma. Protocol 6481 is highly effective for extraabdominal forms of childhood non-Hodgkin's lymphomas but insufficient for abdominal forms.

Abdominal Neoplasms↗

Consequences of intensive therapy of acute lymphoblastic leukemia in children in initial complete remission lasting more than five years.

Complex clinical, neurological and psychological examination was carried out in 23 children with acute lymphoblastic leukemia (ALL), being in complete initial remission from 5 to 12 years and without medication following the successful intensive therapy according to the protocol 0171 from 2 1/2 to 9 years. Somatic deficiency was observed in 2 children. Slightly hypoplastic bone marrow haemopoesis was found in 2 children. All children had normal haemogram. In 7 children lowered values of E-rosettes and in 3 children lowered levels of serum IgA were detected. Two children were HBsAg positive, in one of them with mild fibrosis was found at biopsy. Pathological neurological findings occurred in 7 children; pathological electromyogram (EMG) and electroencephalogram (EEG) in 8 and 5 children, respectively. Deviations of intellectual development were observed in 13 children. Deviations found can be connected in particular with long-term antileukemic therapy, especially with neurotoxic effects of some components of therapeutic regimen used (methotrexate, brain irradiation).

Acute Disease↗

Our experience with childhood histiocytosis X.

A retrospective analysis of the course of histiocytosis X in 16 children treated at our hospital over the past 15 years is presented. Nearly all of the patients were at generalized stages of the disease. In this study the staging according to Greenberg was used. The disease had a favorable course at Stages I and II, 1 patient at Stage III and 2 at Stage IV died. All the children were treated by chemotherapy, in some of them combined with radiotherapy. Individual patients differed from one another with respect to chemotherapeutic regimens. The actuarial survival rate was 78% for the whole group. Better results were obtained after a less intense but long-term treatment than after an intense but interrupted chemotherapy.

Child↗

Treatment of acute lymphoblastic leukemia in children. Long-term results of two trials.

Eighty-eight children younger than 15 years with acute lymphoblastic leukemia were treated with two similar protocols. Both regimens consist of multidrug combinations, with CNS prophylaxis. Sixty-four patients were enrolled on first protocol 0171 with vincristine and prednisone induction therapy. After remission induction patients were randomized on two arms: regimen A which consists of intensive courses of methotrexate with vincristine and prednisone reinductions and administration of cyclophosphamide. Regimen B is similar to regimen A, only courses of methotrexate are alternated with courses of 6-mercaptopurine during consolidation and intensification therapy. In maintenance phase both regimens have daily administration of 6-mercaptopurine and twice weekly administration of methotrexate, with vincristine and prednisone reinductions. The second protocol 0276/A is similar to regimen B of protocol 0171, but L-asparaginase is administered in the end of induction phase and total duration of therapy since induction of complete remission is prolonged from 2.5 to 4 years. Complete remission rate ranged from 92 to 96% in the patients achieving complete remission. In protocol 0171 actuarial proportion of children alive in complete remission for more than 10 years is 60% at present. The actual median survival of all children treated with protocol 0171 is 46+ months and actual median duration of complete remission is 43+ months. There was no significant difference between patients treated with methotrexate alone and those treated with combination of methotrexate and 6-mercaptopurine during consolidation and intensification phase. In protocol 0171 the relapse rate was 28% and the death rate in complete remission was 15%. More than 70% of complete responders in protocol 0276 is in continuous complete remission from 2+ to 57+ months, relapse rate is only 4% at present and the death rate in complete remission is 9% in this protocol.

Adolescent↗