[Acute myelogenous leukemia in children under 2 years of age: studies and treatment results in 23 children in the AML therapy study BFM-78]. / Akute myeloische Leukämie bei Kindern unter 2 Jahren: Untersuchungen und Behandlungsergebnisse bei 23 Kindern der AML-Therapiestudie BFM-78.

23 out of 151 patients of the childhood AML study BFM-78 were less than two years of age at the time of diagnosis, 10 of them being less than one year old. The incidence of M5-subtype was high in infants with 12/23 (52%) compared with 20/128 (16%) in those 2 to 17 years of age. The percentage of boys was 44% in the young children compared with 56% in the older ones. Initial skin infiltrations were seen in five infants with monoblastic subtypes (M4, M5) and in only three patients more than two years of age. The incidence of liver and spleen enlargement greater than or equal to 5 cm below the costal margin was significantly higher in young children. Due to infectious complications frequent therapy-free intervals and/or reduced drug dosages were necessary in the 8-week induction treatment regimen. The prophylactic cranial irradiation with 12 Gy in the first year of life and 15 Gy in the second year has so far not caused any long-term sequelae. The results were similar to those in older children: 18/23 (78%) of the infants achieved complete remission compared with 101/128 (79%) of those 2 to 17 years old. With a follow-up period of 16 to 49 months the probability of continuous complete remission (disease-free interval) was 52% in children under the age of 2 and 54% in those more than 2 years of age. We conclude that with the improved prognosis an intensive chemotherapy is justified in infants with acute myelogenous leukemia.

[Gonadal dysgenesis--possible variation of clinical, hormonal, cytogenetic and histologic findings]. / Gonadendysgenesie--Variationsbreite klinischer, hormoneller, zytogenetischer und histologischer Befunde.

Errors of gonadal differentiation are influencing the sex differentiation on the whole in man. Since the methods in clinical cytogenetics improved, an exact chromosome investigation became possible, which enables the necessary therapy, the appropriate medical and psychological care, and the genetic counselling. Regarding our own observations, general surveys on the frequencies of the different syndromes and on the occurrence of the single clinical symptoms are given.

Invasive dysgerminoma in a girl with 45,X/46,X; mar mosaicism.

We report a 16-year-old girl with features of Turner's syndrome from whom an invasive dysgerminoma was removed. Cytotoxic drugs were given for the next 12 months. Mosaicism of two karyotypes (45,X/46,X; mar) was found in various tissues. The literature is reviewed with special regard to cytogenetic findings and prognosis of malignant growth and differentiation of dysgenetic gonads.

"ADAM complex" (amniotic deformity, adhesions, mutilations)--a pattern of craniofacial and limb defects.

We report eight patients with the craniofacial defects and limb anomalies of the (amniotic deformity, adhesions, mutilations) ADAM complex. Facial abnormalities comprise clefts and distortion and dislocation of craniofacial structures; limbs show various combinations of amputation, secondary syndactyly, and constriction. From previous reports and our cases it is obvious that the clinical picture of the ADAM complex varies enormously; a less severe type combines cleft lip and palate with amputations or amniotic bands. Clinical and experimental data suggest that these malformations are of symptomatic (exogenous) origin. Nosologic differentiation from other conditions phenotypically similar but of genetic etiology is important for genetic counseling. Observation of the ADAM complex in two members of a family suggests that genetic factors might operate in some cases.