[Hyperthyroidism in a premature infant due to transplacental passage of maternal thyrotropin receptor antibodies]. / Frühgeborenenhyperthyreose durch diaplazentar übertragene TSH-Rezeptor-Antikörper der Mutter.

The duration of the stimulating effect of transplacental transferred thyrotropin-receptor-antibodies (TRAb) is discussed by the example of a 23 years old woman suffering from Graves' disease with a severe hyperthyroidism. She became pregnant six weeks after the diagnosis was obtained and then discontinued her antithyroid medication on her own responsibility. On a check-up in the 20th week of pregnancy, a hyperthyroidism was once more found, leading to a therapy with propylthiouracil, which however, was again interrupted by the patient a few weeks later. In the 32nd week, she gave birth to a male child that already presented with distinct signs of thyrotoxicosis and developed a continuous deterioration of the condition, including a tachycardia with up to 190 beats per minute, fever, tremor and a respiratory disorder. Assay of the newborn serum revealed a severe hyperthyroidism. The TRAb level was 180 U/l (normal range < 15). A therapy with propranolol and prednisolone was initiated, leading to a significant improvement of the general condition. Nevertheless, after 12 days, there was still no notable decrease of the hormone levels. Therefore an antithyroid medication was started, which caused normal thyroid hormone levels within 9 days. However, after the therapy was stopped, a hyperthyroidism was again observed within one week, requiring another, low-dose antithyroid medication, which was administered for 26 days. After this period, the TRAb level was down to 25 U/l and no more hyperthyroidism was found. The biological half-life of the TRAb was 20 days in our case.

[Inflammatory fibroid polyp of the stomach as an unusual differential diagnosis of epigastric tumor]. / Entzündlich fibroider Polyp des Magens als ungewöhnliche Differentialdiagnose eines Oberbauchtumors.

We describe the case of a 5 months old infant with an inflammatory fibroid polyp of the stomach. The girl presented with inflammatory symptoms and a tumor in the upper abdomen which was confirmed by sonography and computer-tomography. This very rare benign tumor has not yet been described in an infant.

Neuroblastoma screening: arguments from retrospective analysis of three German neuroblastoma trials.

The justification for a neuroblastoma screening program has been discussed controversially. The analysis of 701 patients of the German neuroblastoma trials NB 79, 82, and 85 provides additional information on this subject. The basis of our investigation was the good prognosis of stage I and II patients (92% survival 5-10 years after diagnosis) compared with 66% in stage III and 11% in metastatic disease. The correlation of age and stage (p less than 0.0001), a median progression time of 14.6 months (range 3.4-33.5 mo) from localized to metastatic disease as observed in 18 patients, the high incidence of asymptomatic diseases in stages I (49%) and II (30%) patients and the cost-benefit estimation arguments in favor of a screening program. The key problem for the lab part is the lower incidence of abnormal catecholamine metabolite excretion in stage I and II patients. The origin of 89% of metastatic disease from intraabdominal sites suggests that ultrasonography may be of additional value.

Low grade malignant non-Hodgkin's lymphomas and peripheral pleomorphic T-cell lymphomas in childhood--a BFM study group report.

Non-Hodgkin's lymphomas (NHL) are classified as low or high grade malignant lymphomas of either B or T cell origin. Cells of low grade malignant NHL (LG-NHL) of B cell type represent either follicular or postfollicular, cells of LG-NHL of T cell type postthymic maturation stages in the lymphoid differentiation pathway. LG-NHL often fail to undergo long-term complete remission or cure in adults, no matter what type of conventional therapy is applied. In three pediatric therapy studies frequency and probability of continuous complete remission (pCCR) were studied for LG-NHL and peripheral pleomorphic T-cell lymphomas (PTCL). Of 432 evaluated patients only six children (1.4%) were qualified as LG-NHL. LG-NHL of T cell type were diagnosed in three children (one T-zone lymphoma, two PTCL of small cell type), LG-NHL of B cell type in another three cases. Two children presented with nodular type of centroblastic/centrocytic lymphoma (CB/CC), one with lymphoplasmocytoid type of immunocytoma (IC). In addition to the two patients with low grade malignant PTCL, there have been also four children with high grade malignant PTCL. The analysis showed that there was not significant difference for event free survival of children with LG-NHL and HG-NHL, respectively (pCCR: 0.63 and 0.82, p = 0.29). In contrast, when comparing high and low grade PTCL versus all other types of childhood NHL (non-PTCL), a significant difference seems to exist (pCCR for PTCL: 0.44, for non-PTCL: 0.83; p = 0.02). However, for further concisive conclusions more patients are needed for evaluation.

Cardiac transplantation in a neonate with endocardial fibroelastosis.

An investigational orthotopic cardiac transplantation was performed to manage subendocardial fibroelastosis in a neonate. No unmanageable technical complications arose from the transplantation. Postoperative observation showed the infant developed normally except for moderate cerebral palsy.

Malignant rhabdoid tumour of the kidney expressing neurofilament proteins. Immunohistochemical findings and histogenetic aspects.

A malignant rhabdoid tumour (MRT) of the kidney was studied immunohistochemically using a large panel of monoclonal antibodies. All tumour cells were marked by a vimentin antibody. Variable amounts of the tumour cells were stained by antibodies specific for neurofilament proteins. Keratin, desmin and GFA, however, were not expressed. Antibodies reacting with lymphohaematopoietic antigens as well as with normal renal tissue and renal neoplasms, especially Wilms' tumours, (BA-1, BA-2, BA-3) and an antibody directed against Tamm-Horsfall protein failed to stain the tumour. These results, especially the expression of neurofilaments demonstrated for the first time in a MRT, point to a derivation of the demonstrated tumour from the neural crest. Analysis of the heterogeneous immunohistological marker expression and of the different primary sites of MRTs suggest that these highly malignant neoplasms are a histogenetically heterogeneous group, irrespective of their characteristic light microscopic appearance.

[Soft tissue tumors in childhood]. / Weichteiltumoren im Kindesalter.

Soft-tissue tumours and soft-tissue sarcomas are different in children and in adults, and therapy varies significantly according to histology, age and stage of disease. The most common soft-tissue sarcoma in children, rhabdomyosarcoma, can be cured in about 60% of cases by a combined therapy involving surgery, chemotherapy and radiotherapy. All children with soft-tissue sarcomas should be treated according to multi-center treatment protocols and should be referred to specialized oncology units. Modern diagnostic techniques and new treatment modalities will increase our knowledge and further raise the cure rate.

No adverse prognostic influence of hepatitis B virus infection in acute childhood lymphoblastic leukemia.

In the years 1980-1985 72 children with acute lymphoblastic leukemia were diagnosed and treated by intensive combination chemotherapy (BFM protocols 79, 81, 83). Of these children 33 acquired a Hepatitis B-virus-carrier state with 1983 as the peak year of incidence. Both groups of patients, the infected and the uninfected ones, were comparable as to prognostic factors. All except 8 patients are off chemotherapy after a total duration of treatment of 1 1/2 or 2 years. Probability for event-free survival (life table analysis, maximum observation time 82 months, minimum 12 months) is equal (0.77 vs. 0.75) in both groups. With 3 exceptions, all HBV-infected patients still carry the HBs-antigen in the serum; 22 of the 30 living patients in the infected group developed anti-HBc.

[Cytomegaly infection in infancy in a family with demonstrated HIV antibodies]. / Frühkindliche Cytomegalie-Infektionen in einer Familie mit HIV-Antikörpernachweis.

Cytomegalovirus (CMV) was isolated from the urine of babies from two consecutive pregnancies of a mother. The first baby had died of pneumonia at the age of six months, two years before the second confinement (twins of different sexes). The male twin was examined at the age of eight months on account of generalized lymphomas, pulmonary infiltrates and thrombopenia. He excreted CMV in the urine, and HIV antibodies were found in the serum. All members of the family (also the baby who died in 1983, from whom serum samples had been stored at -20 degrees C) finally proved to be carriers of antibodies against HIV. The parents and the female twin did not show any clinical symptoms indicating a HIV infection; however, CMV was also isolated from a urine sample from the mother.

[Intestinal cryptosporidiosis in suspected acquired immune deficiency]. / Intestinale Kryptosporidiose bei Verdacht auf erworbene Immunschwäche.

In a 13-year-old boy--with haemophilia A, positive tests for HTLV-III antibodies, and lymphocytopenia--there occurred watery voluminous stools which persisted over many months. Cryptosporidia were demonstrated microscopically in the stool. Cryptosporidiosis should be considered in patients with acquired immune deficiency who have diarrhoea.

Fatal outcome of acute thrombocytopenic purpura associated with a viral infection in an 11-year-old child.

A case of fatal intracranial hemorrhage is reported in an eleven year old girl with acute idiopathic thrombocytopenic purpura following a viral infection. The patient was randomized to the IgG-arm of the ITP therapy study. Immunoglobulin administration was not followed by a raise of the thrombocyte count. Neither the IgG therapy nor intensive therapeutic measurements were able to prevent the fatal course of cerebral hemorrhage in this case. Pathological and immunological findings indicate that our patient suffered from a fulminant ITP which must be considered as a part of a still active viral disease.