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Biomedical subjects

H Wehinger

Publications and source records attributed to H Wehinger.

At least 19 recordsLinked to original sources

[Early abdominal manifestation of neurofibromatosis type ! in a 12-year-old boy]].

Neurofibromatosis type 1 (NF 1) is a neuroectodermal tumor in which abdominal manifestations occur in about 25% of cases in addition to the typical cutaneous lesions. A child with a severe form of an abdominal manifestation of NF 1 was treated in the Hanover Medical School by tumorresection. Pre-operative diagnosis using CT, angiography and ultrasound showed an enormous abdominal tumor constricting the mesenteric vessels. The tumor and the affected portion of the small intestine (150 cms) were removed surgically. No tumor recurrence was observed nine months after operation. Surgical removal is the only currently available therapy for treating tumors of this size.

Child↗

Cancer morbidity rates of children from the vicinity of the nuclear power plant of Würgassen (FRG).

The morbidity rates from neoplastic disease have been studied in children below the age of 15 years, residing at the time of their diagnosis within a diameter of 25 km of the nuclear power plant of Würgassen (FRG). A total of 42 patients were registered between the years of 1980 and 1988: 15 patients with neoplasms of the hematolymphopoietic system, 14 neoplasms of the central nervous system (CNS), and 13 with solid tumors outside of the CNS. The data show that children living in the vicinity of this nuclear power plant do not have an increased risk of developing neoplastic disease, as compared to the entire pediatric population of the FRG. However, a tendency for an elevated risk, not reaching the level of statistical significance, cannot be fully excluded.

Adolescent↗

[5th day convulsions of the newborn infant in rotavirus infections].

AIM OF THE STUDY: The pathogenesis of fifth day fits in newborns has not yet been explained. From 1989-1991 we observed an epidemic occurrence of 21 cases in 1917 hospitalized newborn children. After finding rotavirus in the faeces of several cases we suspected a coincidence. METHODS: We tested the faeces of 19 fifth day fitters as well as 30 healthy newborns (4.-6. day of life) for rotavirus infection. The results of all clinically indicated rotavirus examinations in neonates of 4 to 6 days of age from 1989-1991 were analysed. In 6 rotavirus positive fifth day fitters we performed an immunologic and electron microscopic examination of cerebrospinal fluid. RESULTS: 18 of 19 examined children with fifth day fits (95%) had rotavirus in the faeces. Within the healthy control group we found 12 of 30 (40%, Chi-square 12.46, p < 0.001) and in the group of sick neonates without convulsions 98 of 202 (48.5%, Chi-square 12.44, p < 0.001). This shows a statistically significant coincidence of rotavirus positive faeces with fifth day fits. Rotavirus was not present in cerebrospinal fluid. CONCLUSIONS: We suggest a causal relation between fifth day fits and rotavirus infections. Pathogenetic details remain unclear.

Cerebrospinal Fluid↗

Metaiodobenzylguanidine (mIBG) in treatment of 47 patients with neuroblastoma: results of the German Neuroblastoma Trial.

From 1984 to 1989, 47 children with relapsed, refractory, and/or metastasized neuroblastoma were treated with 131I-metaiodobenzylguanidine (mIBG) in several different treatment combinations. At initial diagnosis, 36 children had Evans stage IV and 11 stage III disease. In 16 of the 47 children, tumor recurred after complete remission prior to mIBG treatment, 26 of 47 progressed from residual or nonresponding tumor, and in 5 of 47 tumor progression during chemotherapy was observed. Altogether the children were treated with a total of 112 courses (range 1-6) with a mean dosage of 8.9 +/- 6.7 mCi/kg body weight/treatment course. Total dose was 283.2 +/- 203.7 mCi for stage III and 388.9 +/- 218.6 mCi for stage IV. Nine of 47 children reached a complete or a very good partial remission (CR and VGPR) from mIBG treatment alone, 13 of 47 achieved partial remission (PR). In an early analysis, 10 patients treated with mIBG in the neuroblastoma trial NB 85 of the German Society of Pediatric Oncology showed no significant difference in survival time compared with 30 conventionally treated children. However, the recent therapy series has been done with higher doses of mIBG, and during improved therapeutic scanning many more bone lesions could be detected than during earlier diagnostic scanning. We conclude that mIBG treatment has not yet fulfilled the expectations for it but still seems for certain indications to be a promising tool to treat neuroblastoma in the future. Moreover, the frontier of neuroblastoma detection is still advancing.

3-Iodobenzylguanidine↗

[Rational diagnosis of anemias].

An algorithmic approach to the diagnosis of anemias is presented. It starts with history, physical examination, and a basic laboratory program including erythrocyte morphology. On the basis of the reticulocyte count and MCH-value anemias are divided into hypo- and hyperregenerative as well as micro-, normo-, or macrocytic forms. Within these groups it is easy to diagnose common anemias by only a few laboratory tests. Rare forms of anemia will be recognized as such and diagnosed by special investigations.

Algorithms↗

Treatment of neuroblastoma with 131I-metaiodobenzylguanidine: experience of the Münster/Kassel Group.

I-131-metaiodobenzylguanidine was used for treatment of neuroblastoma stage IV in three children after surgery and or chemotherapy had failed to be effective. In two of the children with multilocular lesions, after an impressive improvement of clinical symptoms tumor progression was observed. Because in about 25% of children with relapsing neuroblastoma complete remission may be achieved by combining surgery, chemotherapy, and I-131-MIBG treatment, this therapeutic modality should be included in the therapeutic strategy of stage III and IV neuroblastoma.

3-Iodobenzylguanidine↗

Hereditary hypofibrinogenemia with fibrinogen storage in the liver.

A family with hereditary autosomal dominant hypofibrinogenemia is described. The outstanding feature is massive deposition of fibrinogen/fibrin within hepatocytes, faintly visible in routine microscopic sections, but clearly demonstrable by immunohistologic techniques. Circulating fibrinogen shows normal electrophoretic mobility of A alpha-, B beta-, and gamma-chains. We assume that the hereditary defect in this family interferes with fibrinogen release from hepatocytes. Clinically there are fluctuating slight elevations of serum transaminase levels. Hemostasis and wound healing are undisturbed.

Adult↗

[Influence of vitamin E on the development of bronchopulmonary dysplasia in neonates with artificial ventilation. A prospective randomized study].

In a prospectively randomized study we investigated the influence of vitamin E on the development of bronchopulmonary dysplasia (BPD) in neonates with artificial ventilation. Out of 78 neonates two randomized groups of each 39 were formed; one was treated, the other served as control group. We evaluated the cases of those children who remained on respiratory therapy for more than 3 days and who survived 14 days or longer, 19 in each group. The vitamin E treated infants received 20 mg/kg vitamin E i.m. daily during artificial ventilation, but not longer than 14 days. A total of 5 infants (13%) developed BPD, 3 in the vitamin E group, 2 in the control group. All patients with BPD died after 1/2-4 months. After 3 months 17 treated and 16 untreated infants survived. X-rays of the lungs were normal in 12 and 13 respectively. These results do not suggest a beneficial effect of vitamin E in respirator-treated newborn infants.

Bronchopulmonary Dysplasia↗

[Propionic acidemia associated with hypertrophic pyloric stenosis and bouts of severe hyperglycemia (author's transl)].

A newborn is presented with hyperexcitability, drowsiness and later-on with frequent vomiting and muscular hypotonia. Examination of the urine by gas chromatography-mass spectrometry lead to the diagnosis of propionic acidemia which was confirmed enzymatically in fibroblasts. Two unusual features were encountered in this case: There were severe bouts of hyperglycemia with blood glucose values up to 396 and 747 mg/100 ml; furthermore x-ray studies and autopsy revealed a hypertrophic pyloric stenosis.

Autopsy↗

[Haemolytic streptococci of the serological group B and pneumococci - new life-threatening bacteria in newborn wards (author's transl)].

In 1978 353 premature and mature newborns were admitted to our neonatal and intensive care unit. 34 children developed bacterial infections. B-streptococci were the offending organisms in 6 cases, pneumococci in 1 case, 5 of these 7 infections were of the early-onset type, 2 of the late-onset type. The early-onset type disease manifests itself by respiratory distress and shock. It is transmitted vertically from mother to child before or during birth. 3 of our 5 affected children died. The late-onset type disease occurs after 5-10 days, is transmitted as horizontal, mostly nosocomial infection, and presents as sepsis with meningitis or - as in one of our cases - with osteomyelitis. In addition to the 7 sick infants 8 of the 353 admitted newborns were colonized without signs of disease. Early diagnosis, therapy, and prophylaxis of these infections present still unsolved problems.

Cross Infection↗

[Connatal leukaemia and pseudoleukaemia--two cases with cytochemical findings (author's transl)].

Two newborn infants with hyperleukocytosis and blast cells in the peripheral blood are compared. Case 1 represents a connatal leukemia, which could be characterized as monocytic by the naphthol-ASesterase reaction, which was inhibited by NaF. The child died after a few hours. Case 2 must be diagnosed as "pseudoleukemia" in mongolism. The blast cells were classified as lymphoblastic on the basis of a strong granular beta-glucuronidase reaction. The patient achieved spontaneous remission.

Diagnosis, Differential↗

[Controlled desferrioxamine treatment of congenital anaemia and transfusion siderosis (author's transl)].

A 19-year-old patient with congenital Blackfan-Diamond anaemia has been maintained on a regular red cell transfusion schedule since he was two months old. From the age of four he has received desferrioxamine injections at regular intervals with different dose levels. In spite of the treatment with the chelating agent secondary siderosis developed with typical endocrinological abnormalities and cardiac arrhythmias. Increasing the dose of desferrioxamine to 16 g/24 h resulted in an iron excretion of 184 mg/24 h. Such an intensification of treatment finally produced a negative iron balance and the cardiac arrhythmias disappeared. Desferrioxamine therapy should be done under controlled circumstances only, an iron balance is mandatory, and the dose should be adjusted to the results of the balance.

Adolescent↗

[Treatment of ALL in children. Results and side effects with a modification of protocol memphis VII (author's transl)].

42 patients with ALL were treated according to the following protocol: induction with vincristine + prednisone (+/- L-asparaginase), CNS-prophylaxis with cranial irradiation (2400 rads) and intrathecal methotrexate, maintenance for 3 years with 6-MP 50 mg/m2/d p.o. + MTX 75-150 mg/m2/2 wk i.v. X 4, alternating in a cyclic fashion with 6-MP 50 mg/m2/d p.o. + cyclophophshamide 600 mg/m2/2 wk i.v. X 4. The observation time is 24-67 (median 49) months. The actuarial complete remission curve shows 40% continuous complete remissions at 36 months and 30% at 60 months.--The frequency and temporal distribution of typical infectious complications are presented. The incidence of varicella was comparable to that in a southgerman normal control group (5,7% per year). During treatment there were two zoster manifestations per one varicella case, the incidence of zoster being 1 case per 106 patient-months, viz 11,4% per year.

Adolescent↗