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Biomedical subjects

D Harms

Publications and source records attributed to D Harms.

At least 145 records · Page 8Linked to original sources

[Malignant peripheral neuroectodermal tumors. Histological and immunohistological conditions in 41 cases].

In view of the personal observation that malignant peripheral neuroectodermal tumours (MPNT) can present different histological growth patterns, 41 cases of MPNT were histologically and immunohistochemically studied. The median age of the 41 patients was 15 years (range: 9 months - 23 years). There were 27 males and 14 females. Most tumours (23/41) were located in the thoracopulmonary region. In 31/41 cases there was bone as well as soft tissue involvement. The following histopathological patterns were found: Ewing's sarcoma-like (n = 7), atypical Ewing's sarcoma-like (n = 4), neuroblastoma-like (n = 8), rhabdomyosarcoma-like (n = 8), and hemangiopericytoma-like (n = 1). In 2 cases combined patterns were noted, one tumour being characterized by neuroblastoma-like and Burkitt's lymphoma-like features. Most cases of MPNT differed from the cytological features of typical Ewing's sarcoma in that they contained hyperchromatic nuclei with distinct nucleoli. Some reticulin fibrils were found in between the cells of some cases. Immunohistochemically, 19/23 cases reacted positively to vimentin, 29/32 to neuron specific enolase (NSE), 16/28 to protein S-100, and 1/9 to glial fibrillary acidic protein. 12/24 cases reacted positively to NSE and protein S-100. Neurofilaments and desmin were not found in the formalin fixed material of the present study. The results show that most cases of MPNT can be distinguished from typical Ewing's sarcoma by cytological and histological findings. Differential diagnosis from atypical Ewing's sarcoma, neuroblastoma, and rhabdomyosarcoma is possible by immunohistochemistry.

Adolescent↗

[Early forms of compassion].

The present study deals with the problem of whether early forms of compassion are already evident in the first year of life. For this purpose, tape recordings of other children crying were played as acoustic stimuli to 210 newborns and infants. Vocal and motor reactions were observed, and numerous measures were evaluated. It was found that a) children cry with another child from birth on; b) this responsive crying becomes less frequent as the child grows older; c) the motor reactions become increasingly differentiated; d) the responsive crying does not disappear entirely despite increasing cognitive abilities, although it is partly transformed into a more restrained reaction with a sad expression; e) hungry children react more strongly than satiated children, children with siblings more often than only children, and girls more often than boys; f) responsive crying to an indifferent acoustic stimulus was observed very rarely. This leads to the conclusion that early forms of compassion already exist in the first year of life. Two such forms are described. It is assumed that compassion is already present in a primitive form at birth, and is thus inborn.

Altruism↗

[Sonographic diagnosis of alobar holoprosencephaly].

The alobar form of holoprosencephaly is characterised by a huge spherical ventricle localized in the midline. The monoventricle communicates with a large occipital cyst, the "dorsal sac". Both thalami and plexus chorioidei are fused in the midline. The interhemisperic fissure and the falx cerebri are absent as well as the corpus callosum and the septum pellucidum.

Abnormalities, Multiple↗

The association of hepatocellular carcinoma in childhood with hepatitis B virus infection.

Eleven cases of hepatocellular carcinoma (HCC) in childhood were investigated by immunohistochemistry for association with hepatitis B virus (HBV) infection. Seven of 11 cases (64%) demonstrated positivity for hepatitis B surface antigen (HBsAG), whereas all 11 were negative for hepatitis B core antigen (HBcAG). Cirrhosis was absent in all cases, and other causes for HCC in childhood were not found. All children with HBV-associated HCC died within 6 months of diagnosis. The median survival time of these children was 2 months. Only one child with HCC of trabecular subtype without HBV association is still living after 18 months. However, this child has metastases and a local recurrence. Three other children with HCC of fibrolamellar subtype are free of disease after 2, 5, and 6 years, respectively. The high number of cases of HBV-associated HCC shows the important role of HBV infection as an etiologic factor for the development of childhood HCC in middle Europe.

Carcinoma, Hepatocellular↗

Delayed seroconversion to HIV in a hemophiliac.

In a 14-year-old boy with severe hemophilia B, HIV seroconversion was observed about 2 years after substitution therapy had been changed to heat-treated PPSB concentrates. This shows that HIV-antibodies may appear in hemophiliacs after time periods longer than generally assumed.

Acquired Immunodeficiency Syndrome↗

Malignant peripheral neuroectodermal tumors. A retrospective analysis of 42 patients.

The clinical presentation of the disease and the results of treatment in 42 patients with malignant peripheral neuroectodermal tumors (MPNT) entered into the Cooperative Ewing's, soft tissue, and neuroblastoma trials of the German Society of Pediatric Oncology were retrospectively analyzed. Within the Ewing's sarcoma trial, patients with chest wall lesions were particularly analysed for MPNT features. The period of observation ranged from 15 to 86 months; the median relapse-free time was 24 months. There were 28 male and 14 female patients, the median age of patients was 15 years (range, 9 months-23 years). Thirty-two patients had localized disease (M0), and ten patients presented with primary metastases (M1). The predominant location of the tumors was the thoracopulmonary region, followed by the extremities, the abdominal/pelvic, and head and neck region. Thirty-one of 42 tumors involved the adjacent bone. The disease-free survival according to Kaplan-Meier life-table analysis was 56% +/- 11% for Stage M0 patients at 3 years. Nine of ten patients with M1 disease showed progression of their disease. Most patients had combined modality treatment with surgery, chemotherapy and radiation therapy. Best results were obtained with extensive surgery. Radiation doses ranged from 20 to 60 Gy and could not be correlated with the outcome of the disease. Most recurrences occurred at the site of the primary tumor. In patients with primary chemotherapy after biopsy-proven diagnosis, the responsiveness of this disease to chemotherapy could be demonstrated. Combination chemotherapy containing anthracyclines and high doses of alkylating agents appeared superior.

Actuarial Analysis↗

Multidisciplinary treatment of primary Ewing's sarcoma of bone. A 6-year experience of a European Cooperative Trial.

The German Society of Pediatric Oncology in 1981 initiated the Cooperative Ewing's Sarcoma Study (CESS 81) using a four-drug combination of chemotherapy prior to definitive local control with surgery and/or radiation. From January 1, 1981 until February 28, 1985, 93 patients were registered at the trial office from 54 participating institutions in West Germany, Austria, Switzerland, and the Netherlands. On February 1, 1987, 54 of 93 patients were disease-free. Using the Kaplan-Meier life table analysis, the estimated disease-free survival (DFS) rate was 60% at 36 months and 55% at 69 months. The median period of observation was 29 months, ranging from 22 months to 69 months. Twenty-one of 93 patients (23%) had local failure, 18 of 93 patients (19%) developed systemic metastases. The local failure rate was particularly high in patients treated with radiation and was reduced when radiation planning was centralized within the study based upon the extent of disease at diagnosis. Cox regression analysis of prognostic factors showed that tumor volume was a significant factor influencing prognosis. The estimated 3-year DFS rate was 80% for patients with small tumors (volume less than 100 ml) compared to 31% for patients with large tumors (volume greater than or equal to 100 ml). In patients who had surgery for local control, the histologic response to chemotherapy was analyzed on the surgical specimen and had a strong influence on survival: 79% DFS at 3 years for patients with less than 10% viable tumor (good responders) compared to 31% DFS for patients with more than 10% viable tumor (poor responders). Tumor load and responsiveness to chemotherapy are the two major factors influencing prognosis in patients with primary Ewing's sarcoma of bone.

Adolescent↗

Clastogen-induced fragility may differentiate pancytopenia of congenital dyskeratosis from Fanconi anaemia.

A male infant is reported with congenital dyskeratosis and pancytopenia Zinsser-Engman-Cole. The bone marrow pathology showed similarities to Fanconi anaemia. Ophthalmological complications were vitreous haemorrhage, haemorrhagic cataracta complicata and glaucoma. Spontaneous and diepoxybutane-induced chromosomal fragility was within the range of normal cells but was elevated through induction with 4-nitroquinoline-oxide. These findings are contrasted with those of Fanconi anaemia.

Anemia, Aplastic↗

[Pulsed Doppler sonographic determination of absolute flow velocities in the anterior cerebral artery in infants with hydrocephalus in comparison with a healthy patient sample].

In 52 infants (weight: 3174 +/- 1165 g; gestational age: 41.3 +/- 6.5 weeks) with hydrocephalus pulsed doppler recordings were obtained in the anterior cerebral arteries. For comparison 52 healthy infants (weight: 3148 +/- 1118 g; gestational age: 40.6 +/- 5.7 weeks) were investigated. In all children the maximal systolic velocity, the end-systolic velocity, the end-diastolic velocity and the pulsatility-index were measured. In the healthy control group the maximal systolic velocity was 43 +/- 14 cm x s-1, the end-systolic velocity 20 +/- 8 cm x s-1, the end-diastolic velocity 11 +/- 5 cm x s-1 and the pulsatility index was 0.75 +/- 0.10. All 9 children with minimal ventricular dilation without progression showed normal flow profiles with normal flow velocities and pulsatility-index in the anterior cerebral arteries. 17 infants with moderate, slowly progressive ventricular enlargement showed significant increase of the maximal systolic velocity (60 +/- 27 cm x s-1) and the pulsatility-index PI (0.82 +/- 0.14). There was no difference in the end-systolic and end-diastolic velocities to the healthy control group. 26 children with marked and rapid progressive hydrocephalus showed significant decrease of the end-systolic and end-diastolic velocities and an increase in the pulsatility-index. The end-systolic velocity was 15 +/- 7 cm x s-1, the end-diastolic velocity was 4 +/- 7 cm x s-1 and the pulsatility-index measured 0.91 +/- 0.18. There was no difference in the maximal systolic velocity which measured 41 +/- 17 cm x s-1. All children with increased intracranial pressure showed a pathological flow profile with a decrease of diastolic forward flow. Absent or retrograde diastolic flow in rapid progressive hydrocephalus may lead to a decrease of brain perfusion resulting in hypoxemic ischemic brain lesions. After implantation of a ventriculo-atrial shunt an increase in the end-systolic and end-diastolic velocities and a decrease of the pulsatility-index could be shown. Shunt insufficiency can be shown early by a decrease in diastolic forward flow.

Blood Flow Velocity↗

[Congenital toxoplasmosis: value of modern IgM serodiagnosis is from the example of problem cases].

Congenital toxoplasmosis may be symptomatic or asymptomatic at the time of birth. In the latter case late manifestations may occur which can be avoided by early diagnosis and treatment. In comparison with the IgM-IFT we demonstrate the specificity and high sensitivity of a recently available immunosorbent agglutination assay (ISAGA) for the detection of IgM against toxoplasma. We also present evidence for the high diagnostic value of the ISAGA in cases where congenital toxoplasmosis is suspected. Based on the presented data we propose a pattern of diagnostic methods for the diagnosis or exclusion of congenital toxoplasmosis in newborns.

Antibodies, Protozoan↗

[Congenital toxoplasmosis with delayed immune response in children. Diagnostic problems].

A newborn, delivered at term, developed a rapidly increasing hydrocephalus with intracranial calcifications and seizures during the first week of life. Clinical suspicion of congenital toxoplasmosis was at first not confirmed serologically (serum titer in the immunofluorescence test [IFT] of 1:1024 and complement-fixation reaction [CFR] of 1:40 equalling those of the mother; IgM-IFT being negative and the IFT and CFR titers significantly falling within two weeks). But in the further course of the disease the diagnosis of congenital toxoplasmosis was confirmed: 1. Microscopic tachyzoits in CSF when aged six weeks; 2. positive IgM-IFT in serum and CSF from the seventh weeks onwards; 3. steep IFT titer rise in serum and CSF from the 16th week onwards. This case demonstrates that with delayed immune response in the infected child only serial serological tests will exclude or confirm the diagnosis of congenital toxoplasmosis.

Antibodies, Bacterial↗

Epithelioid sarcoma in children and adolescents. An immunohistochemical study.

Six cases of epithelioid sarcoma were studied by conventional light microscopy and immunohistochemistry. The six cases account for 1.4% of the 417 cases of soft tissue sarcoma collected at the Paediatric Tumor Registry, Kiel. The average age of the five male and one female patient was 10.8 years (median: 13 years). Particular clinical findings included the location of the tumours; three were found in the pelvis, two in the head and neck, and one in the hand. Four patients are living without disease, and one patient died of disease three years after diagnosis. Histologically, four of the six tumours revealed multinucleated giant cells. Immunohistochemically using a panel of mono- and polyclonal antibodies all cases stained positively for vimentin, cytokeratin, epithelial membrane antigen (EMA), and human milk fat globulin (HMFG-2). Five cases were positive for neuron specific enolase (NSE), and three stained positively for protein S-100. A positive reaction for alpha-1-antichymotrypsin was noted in two cases. These immunohistochemical findings attest to the multidirectional differentiating capabilities of epithelioid sarcoma and support the concept of derivation from a multipotent mesenchymal stem cell.

Adolescent↗

Diagnostic pitfalls in solid childhood tumors.

Differential diagnosis of round and spindle cell type tumors in childhood is difficult. Diagnosis can be facilitated by additional immunohistochemical analysis (case 1) or detection of characteristic histological patterns (case 2). Rapid growth is not an exclusive feature of malignant tumors, but can also be observed in benign tumors and tumor-like conditions of young patients (case 3).

Adolescent↗

Sarcomatous Wilms' tumour with clear cells and hyalinization. A study of 38 tumours in children from the SIOP nephroblastoma file.

The International Society of Paediatric Oncology (SIOP) nephroblastoma trial and studies 1, 2 and 5 include 33 sarcomatous Wilms' tumours with a clear cell pattern and five with a hyalinizing pattern among 909 renal tumours. Bone metastases developed in 22 (67%) and one patient (20%) respectively compared with 14 patients (1.6%) in the rest of the series. Both subgroups had clear cells and hyalinization. A constant and characteristic feature of the clear cell tumours was an alveolar vascular pattern which aids in the recognition of this tumour even in fibrous and sclerotic forms. Sarcomatous Wilms' tumour with a hyalinizing pattern had homogeneous areas of sclerosis with clustering of tumour cells but lacked the vascular pattern. Two tumours showed atypia similar to that in malignant rhabdoid tumour of the kidney but had no cytoplasmic inclusions. Five year recurrence-free survival for the entire series was 68%, for sarcomatous clear cell tumours, 42% and for sarcomatous tumours with hyalinizing pattern, 20%.

Adolescent↗

Small-cell pediatric tumors: histology, immunohistochemistry, and electron microscopy.

Histology, immunohistochemistry, and electron microscopy give an account on what is known of the correlation between histologic appearance and prognosis in this important group of tumors. Ultrastructural and immunohistochemical findings are presented that frequently are useful in establishing a diagnosis, but also serve to elucidate the histogenesis of some tumors, especially those that lack characteristic features.

Child↗

[Malignant peripheral neuroepithelial tumors in childhood].

The different therapy modalities and course of disease of 42 patients with a malignant peripheral neuroepithelial tumor are retrospectively analyzed. Therapy was completed in 31 children, 25 of whom had a primary localized tumor and 6 a disseminated neuroepithelioma. 17 of the children with a localized illness survive disease free in contrast to no survivor in the group with a disseminated tumor. The effective chemotherapy combining vincristine, adriamycin, ifosfamide and actinomycin D must be complemented by an efficacious local control because malignant peripheral neuroepithelioma tend to recur locally. The prospective analysis of newly diagnosed patients and a standardized therapy regimen will show if malignant peripheral neuroepithelioma represents a distinct tumor entity, different from a Ewings-Sarcoma.

Adolescent↗