Biomedical subjects

H L Müller

Publications and source records attributed to H L Müller.

93 records · Page 6Linked to original sources

PubMedDate not supplied

[Endocrinologic after-care of brain tumor patients: initial retrospective analysis of HIT-study documented auxologic and endocrine parameters].

BACKGROUND: In 1987, a pilot trial (HIT 88/89) was initiated by GPOH to investigate the effectivity and toxicity of intense preradiation chemotherapy. The aim of this study was to improve the treatment of children with malignant brain tumors, but provided no standardized recording of auxological and/or endocrinological parameters. In 1993 a sub-project, "Endocrinological late effects", was started during the ongoing study to examine the late effects of treatment, and a retrospective evaluation was performed in order to standardize parameters. PATIENTS: We considered auxological and hormonal data from those patients (n = 133; 78 m, 55 f) who were alive at the time of the survey, and had completed tumor therapy. RESULTS: Analysis of the growth pattern of the children after the end of therapy indicate impaired growth. Group 1 (n = 77, CA < 10 yrs): Mean height SDS at tumor diagnosis was +0.32 (n = 77), and fell constantly after completion of therapy to -0.75 (n = 16) after 3-4.5 yrs. Group 2 (n = 56, CA > 10 yr): Height SDS-CA fell from +0.24 (n = 45) at the time of diagnosis to -0.62 (n = 14) after 3-4.5 yrs. Unfortunately, only body height and weight were recorded regularly, data on sitting height, pubertal stage and bone age were incomplete. Endocrine evaluation after end of therapy was insufficient. CONCLUSIONS: A standardization of endocrinological follow up is necessary as soon as possible, for an adequate description of the endocrinological late effects.

Adolescent↗

PubMedDate not supplied

Disseminated malignant ectomesenchymoma (MEM): case report and review of the literature.

Malignant ectomesenchymoma (MEM) is a rare soft tissue tumor believed to arise from a pluripotent migratory neural crest cell and composed fo both a mesenchymal element and a neuroectodermal element. The authors report the case of an 11-month-old male who presented with a local abdominal MEM and systemic metastases into lungs, liver, bones, and bone marrow. This is the first reported case of an MEM with initial bone marrow dissemination. The tumor consisted of a neuroblastoma component and a mesenchymal component with sarcomatous features. Diagnosis and therapy were complicated by the histological heterogeneity of the tumor, which also influenced the clinical appearance and course in this case. A literature search revealed 15 other evaluated cases that arose in soft tissue and had adequate clinicopathologic data. Complete surgical resection was the mainstay of treatment, and chemotherapy also appeared to be important. In all reported patients (n = 3) with initial metastases or bone marrow dissemination, as in this case, no cure could be achieved. In patients with disseminated MEM, new therapeutic approaches such as high-dose chemotherapy followed by stem cell rescue should be considered, similar to the current strategy in patients with stage VI neuroblastoma or soft tissue sarcoma.

3-Iodobenzylguanidine↗

PubMedDate not supplied

Acute lymphoblastic leukemia with severe skeletal involvement: a subset of childhood leukemia with a good prognosis.

Skeletal radiographic abnormalities are common in children with acute lymphoblastic leukemia (ALL). The impact of severe skeletal involvement (SI) on survival and the correlation between SI and biological markers were analyzed. Therefore, radiographs and medical charts of 106 ALL patients who received a skeletal survey at the time of diagnosis and were treated at the University Children's Hospital Würzburg between 1974 and 1995 were reviewed. On the basis of the skeletal survey, SI was quantified using a score. Fifty-nine patients (55%) showed radiographic abnormalities defined as metaphyseal banding (48%), periosteal reactions (11%), osteolysis (33%), osteosclerosis (31%), or osteopenia (22%). Children with severe SI (n = 32) presented with a higher rate of severe radiographic abnormalities such as geographic destructive osteolysis (37%; P < .001) and periosteal reactions (28%; P < .05) compared with children with moderate SI (0% and 4%, respectively). Patients with severe SI showed a lower peripheral blast count (P < .05) at diagnosis, a more frequent "prednisone good response" (P < .05), and a higher survival rate (83 +/- 7%; P < .05) than patients without SI (54 +/- 9%). Patients with moderate SI (n = 27) showed a higher hemoglobin concentration (P < .05), an enlargement of liver (P < .05) and spleen (P < .01), a higher BFM risk factor (P < .01), but still a higher survival rate (73 +/- 11%) than patients without SI (NS). Patients with severe SI had a higher (P < .001) DNA content of leukemic cells as measured by DNA index (DI) than patients without SI. Thirty-one percent of patients with severe SI, 22% of patients with moderate SI, and no patient without SI had a DI > 1.16. No patient with a DI < 1.0 presented with severe SI. The number of radiographic abnormalities in patients with SI correlated with the DI (rho: 0.46; P < .001). However, patients with euploidy (DI = I) and severe SI also had a higher (P = .05) survival rate (70 +/- 18%; n = 15) than euploid patients without SI (49 +/- 11%; n = 24). Of the patients with severe SI, 78% had common ALL and 22% had an ALL type other than common ALL (P < .05). In patients with ALL types different from common ALL, severe SI was also associated with higher survival rates. We conclude that on the basis of clinical features, two distinct subgroups could be identified in terms of SI. Patients with clinically relevant severe SI had a better prognosis, a higher DI, and more frequently a common ALL than patients without SI. However, the impact of severe SI on prognosis was independent of DI and type of leukemia.

Adolescent↗