[Pneumothorax]. / Pneumothorax.

The presence of air between the visceral pleura and the parietal pleura with consecutive retraction of the lung from the chest wall is called pneumothorax. Regarding the genesis of the pneumothorax, a distinction is drawn between spontaneous and traumatic pneumothorax. The spontaneous pneumothorax is, depending on whether a congenital or an acquired pulmonary disease can be found, grouped into a primary spontaneous pneumothorax (PSP) without underlying lung disease and a secondary spontaneous pneumothorax (SSP) with the presence of a known lung disease. The traumatic pneumothorax is classified, depending on the cause, into penetrating and non-penetrating (blunt) traumatic events. A special form of the traumatic pneumothorax is the iatrogenic pneumothorax occurring as a result of diagnostic and/or therapeutic interventions. Clinically, a pneumothorax can range from an asymptomatic to an acute life-threatening situation. The required initial measures depend primarily on the patient's clinical condition. They vary from immediate insertion of a chest tube to wait and see with monitoring. The insertion of a chest tube is still the accepted therapeutic standard, but other procedures like aspiration of air through a needle or small catheter, particularly for small spontaneous pneumothoraces, represent alternative therapy options as well. The short-term goal is to treat possibly existing dyspnea and pain; in the long run a recurrence of the pneumothorax should be prevented. Until now, no uniform treatment algorithms or standardised therapy principles exist to achieve the therapeutic intentions of lung expansion and freedom from pain and late relapse.

Hepatitis C virus transmission in a pediatric oncology ward: analysis of an outbreak and review of the literature.

Hospital-related hepatitis C virus (HCV) infections continue to occur even after the introduction of blood donor screening. We report an outbreak of HCV in nine patients of a pediatric oncology ward in 1996/1997. Sequencing of the hypervariable genomic region 1 (HVR1) of the E2/NS1 region showed near identity between HCV isolates from these patients as evidence for infection with the same virus. Despite a detailed and careful investigation, the source of infection and the mode of virus transmission could not be established. Based on a review of the current literature about nosocomial HCV infection and HCV infection in children, hypotheses for possible means of transmission in this outbreak are discussed.

[Tufted angioma]. / Büschelartiges Angiom ("Tufted angioma").

We present a 2-year-old boy with a red, cutaneous-subcutaneous, nodule on the right elbow and a 2.5 year-old girl with an red-brown, indurated plaque on the left knee. Colour-coded doppler sonography of the boy's lesion showed vascular structures. A biopsy established the diagnosis of tufted angioma in both patients. Tufted angioma is clinically characterized by slowly spreading erythematous macules and plaques preferentially located on the upper trunk and neck in children. It is a benign tumor, malignant transformation has not been reported. The case history, clinical and histological findings contribute to the diagnosis. Tufted angioma has to be distinguished from Kaposi's sarcoma, angiosarcoma, hemangioma of infancy, sometimes bacillary angiomatosis and other cutaneous capillary malformations. Treatment of tufted angioma is difficult, various modalities like glucocorticosteroids, Interferon-alpha, flashlamp-pumped pulsed dye laser, excision and spontaneous regression have been described with varying results.

[Accomplishments of tumor surgery in pediatric nephroblastoma]. / Leistungen der Tumorchirurgie beim kindlichen Nephroblastom.

The cure rate of Wilm's tumor in childhood could be increased to more than 80% by systemic adjuvant chemotherapy in combination with operation and radiation. The importance of histological grading is discussed. Especially preoperative chemotherapy has made progress possible in reducing risks of operative techniques and has improved the prognosis for survival.

Acquired immune haemolysis by anti A 1 antibody following bone marrow transplantation.

In ABO mismatched organ or bone marrow transplants recently some cases of acquired immune hemolysis have been reported. It was felt that these life threatening complications were due to immunosuppressive treatment with cyclosporin-A. A case of severe hemolysis following mismatched BMT is reported. Here no cyclosporin-A treatment was given since the bone marrow was T-cell deprived by an E-rosetting technique. Apparently T-cell purging can under these conditions become dangerous.

Prolonged methotrexate infusions in children with acute leukemia in relapse and in remission and with medulloblastoma. Pharmacokinetics, toxicity and clinical results.

In 86 children with acute lymphocytic leukemia (ALL) and in 6 children with medulloblastoma 253 24-hour methotrexate (MTX) infusions with 150, 500, and 700 mg/m2 were performed. MTX concentrations in plasma and cerebrospinal fluid (CSF) were measured with a specific radioimmunoassay. In 131 infusions with 500 mg/m2 given to patients with ALL in remission, the MTX plasma concentration 24 h after the end of infusion did not exceed 7 X 10(-7) mol/1. Mild hematologic toxicity occurred in 22% of the treatment cycles. In contrast 8/45 infusions given to patients with ALL in relapse were associated with delayed MTX elimination followed by severe toxicity. The CSF: plasma ratio of MTX measured during 58 infusions did not exceed 11% in patients with ALL in remission, but was above this value in 13/34 infusions in patients with leukemia of the central nervous system (CNS). 24-hour MTX infusions with 500 mg/m2 were as hepatotoxic as 4- to 6-hour infusions with 3-8.5 g/m2. With MTX as single agent no remissions were achieved in 8 patients with ALL in relapse. The addition of asparaginase in 10 patients resulted in 3 complete and 2 partial remissions. In patients with ALL in first remission clinical results confirmed the value of intensive MTX therapy for disease-free survival.

[Improved prognosis in Wilms' tumor due to adjuvant combination drug therapy]. / Verbesserte Prognose beim Wilms-Tumor durch adjuvante Kombinationschemotherapie.

78 children with Wilms' tumor stage I--IV diagnosed since 1960 are presented. There were two groups of patients: one group consisting of 35 patients which received adjuvant chemotherapy for 1 year, the other group consisting of 43 patients which received no chemotherapy. Surgical excision and irradiation was identical in both groups. The prognosis was greatly improved by adjuvant chemotherapy: 4-year survival rates increased from 25 to 69%. Late side effects from radiation and chemotherapy were noted: 15/78 patients suffered from scoliosis and all patients treated by chemotherapy had a decreased lymphotoxin activity over years. The following factors appear to be related to prognosis: the extent of disease in patients determined by better techniques, histopathology of the tumor, and the nature of treatment.

[So-called histiocytosis X and malignant histiocytosis]. / Sogenannte Histiozytose-X und maligne Histiozytose.

A retrospective study of 12 cases of so called histiocytosis-X and 3 cases of malignant histiocytosis was done. It was possible to establish the differences in clinical, morphologic and cytochemical findings of both diseases. The diagnosis of histiocytosis-X can be confirmed by multinucleated histiocytes interrupted by eosinophils and plasmacells. The histopathology of malignant histiocytosis is different and is characterized by atypical histiocytes. Erythrophagocytosis throughout the tissues is seen. Typical histochemistry (acid phosphatase and naphtol-AS-acetat-esterase) findings are also helpful for diagnosis. The treatment of both diseases should be continued at least six months after disappearance of clinical apparent lesions. Combination chemotherapy with vinblastine and prednisone is suggested. In cases of histiocytosis-X in isolated lesions curettage or irradiation may be adequate. Long term remissions and presumed cures of histiocytosis-X are possible in over 70% of the cases. A strict correlation between the prognosis and the degree of involvement is confirmed. Even in cases of malignant histiocytosis, previously reported as rapidly fatal disease, combination chemotherapy may produce complete long term remissions.

Intravenous and subcutaneous desferrioxamine therapy in children with severe iron overload.

Ten children with transfusion dependent anemias (thalassemia, sideroblastic anemia, congenital pure red cell aplasia) received either intravenous desferrioxamine (DF) in increasing doses up to 450 mg/kg at the time of transfusion or daily subcutaneous DF up to 110 mg/kg on an outpatient basis. No patient on intravenous DF reached a negative iron balance. All children with a subcutaneous DF dose of more than 60 mg/kg obtained a negative iron balance with a net iron excretion (transfusion iron already substracted) between 206 to 810 mg (mean 496 mg) monthly. The effectiveness of regular subcutaneous DF on liver storage iron could be confirmed in 4 patients by liver biopsy, showing a decrease between 40-60% iron after 12-14 months of chelation therapy. So far the daily iron excretion has remained constant with a given dose of DF over a period up to 15 months. Even if poor compliance in some patients is taken into account, it is possible with this method of treatment to prevent further accumulation of iron in chronically transfused children.

[Bone marrow transplantation for aplastic anaemia (author's transl)]. / Knochenmarktransplantation bei aplastischer Anämie.

From March 1975 until May 1980 twelve patients with severe aplastic anemia were grafted with bone marrow from HLA-identical siblings by the Munich Cooperative Group for Bone Marrow Transplantation. Six patients are alive between 10 months and more than 5 years after grafting with normal blood values and marrow. One patient is treated as an out patient for chronic localized graft-versus-host disease (GvHD), five patients are well and without treatment. Six patients have died, one patient with a cerebral hemorrhage the day before transplantation, three patients following rejection of grafts 32, 40 and 55 days after grafting, one patient with severe GvHD 85 days after grafting and one patient, probably with interstitial pneumonia, following cerebral hemorrhage. Three of 6 patients who were conditioned with Cyclophosphamide (CY) only died following rejection of the graft. Two adults who were conditioned with CY and "total lymphoid irradiation" and three children, who wer given unirradiated leukocyte concentrates from the marrow donor after grafting, did not reject their grafts. The results of the Munich-Cooperative Group for Bone Marrow Transplantation are comparable to those of large, specialized centers for bone marrow transplantation, they indicate possibilities of cure of severe aplastic anemia by marrow grafts from HLA-identical siblings. They confirm that better results are obtained with earlier transplantation in the course of the disease.

Sinus histiocytosis with massive lymphadenopathy and epidural involvement.

Sinus histiocytosis with massive lymphadenopathy (SHML) was recognized as a new clinical-pathological entity in 1969. Up to the present 134 cases have been described. The disease is characterized by prominent cervical lymph node enlargment. Microscopic features include marked dilatation of sinuses with intrasinusal histiocytes and lymphophagocytosis. About 70% of the patients reported were affected during the first 2 decades of life. The disease is held to be benign on account of spontaneous resolution in some patients. A follow-up survey of 72 patients showed disappearance of the symptoms 10 years after the original diagnosis in 24 patients. In 42 patients the disease still persisted 6 months to 21 years later. Six patients died, but only one of them as a result of the disease. Extranodal involvement was seen in the orbit, eyelid, respiratory tract, skin, bone, salivary glands, and testis. In two cases, one of which will be reported here, paraparesis resulted from infiltration of the epidural space. Treatment with prednisolone was tried in some cases with excellent results. In our case treatment with prednisolone and vinblastine resulted in the disappearance of the neurological symptoms.

Antibody incubation of human marrow graft for prevention graft versus host disease.

An in vitro incubation of incompatible donor bone marrow by xenogenic anti-T-cell globulin (ATG) suppressed an otherwise lethal GvH reaction in animal models. An application of this principle to clinical bone marrow transplantation was successfully tried in three patients with acute lymphoblastic leukemia. Preparation of the specific anti-human T-cell globulin (ATCG-H) was carried out by absorption of anti-human thymocyte globulin with liver-kidney homogenate, chronic lymphocytic leukemia cells of B-cell type, and erythrocytes. Subsequent testing revealed that the serum still reacted with human T-cells but no longer reduced the number of colony-forming units in culture (CFU-C). All three bone marrow recipients were treated by chemotherapeutic conditioning and total body irradiation followed by grafting of in vitro treated bone marrow from HLA-identical siblings. The transplantation of the bone marrow was well tolerated and no major side effects were encountered. No patient so far (24, 7, 6 months) has shown any signs of GvHD. The in vitro pretransplantation treatment of bone marrow with anti T-globulin may be a new approach to the prevention for GvHD in man.

[Effect on peripheral blood cells in children with acute leukemia during remission by intramuscular injected of arabinosyl-cytosine (author's transl)]. / Effektivität intramuskulär verabreichten Cytosin-Arabinosids bei der ambulanten Behandlung von Kindern mit akuter Leukämie in Remission.

Investigation was done in children with acute leukemia in complete hematological remission. It was tested whether out-patient treatment by intramuscular administration of arabinosylcytosine (Ara-C) may obviate the continuous intravenous Ara-C regimen. Every therapy cycle lasted 5 days. Changes in peripheral blood cell counts after 5 days of continuous intravenous. Ara-C infusion against 5 days of intramuscular Ara-C application given every 12 h were tested. 129 cycles of intramuscular application and 11 cycles of intravenous application were evaluated. Evaluation was done by the Friedmanntest. There was a significant decrease in blood cell counts after intramuscular Ara-C treatment. When administered intramuscularly during the first cycle Ara-C was effective for at least 3 weeks, whereas after repeated cycles the decline of blood cells was only demonstrable for 14 days. A comparison of the effect between intravenous and intramuscular routes revealed similar results. No local side effects were noted when Ara-C was given intramuscularly.

[Neuroblastoma in children. Clinical staging and management (author's transl)]. / Neuroblastom im Kindesalter. Klinik, Diagnostik und Therapiemöglichkeiten.

Sixty-four children with neuroblastoma stage I to III c are presented. The coordinated management utilizing surgical excision, irradiation (2--5.000 rad) in stage II and III and multiagent chemotherapy is described. Favorable sites were abdomen (30) and thorax (22). In ten cases the primary site was unknown. The prognosis is influenced by several factors: Patients under 1 year of age without evidence of bone or bone marrow metastases have a favorable outcome (13/15). Nearly all patients with lesions of bone or generalized tumor in bone and/or bone marrow (stage III b and III c) failed to attain long term disease free survival despite combination chemotherapy and the use of radiation therapy (33/34). No relation between histological or biochemical characteristics and prognosis could be found. Biochemical determinations however are useful as an index of response to treatment. Prognosis was independent from sex. A review of the literature and on attempt to improve the therapeutic efficancy in stage III are reported.

[Juvenile rhabdomyosarcoma. Diagnosis and new therapeutic possibilities]. / Rhabdomyosarkom im Kindesalter Diagnostik und neue Therapiemöglichkeiten.

Seventeen children with rhabdomyosarcoma stage I to III diagnosed since 1973 are presented. The coordinated management utilizing surgical excision, irradiation (5000 rad) and systemic adjuvant multiagent chemotherapy is described. The favorable sites were head and neck sites and genitourinary region. The mean survival is at present 19 months. 14 children are alive and well. 13 children had no tumor recidivation, 1 child had 8 months after beginning of the therapy lung metastases which after irradiation with 2000 rad disappeared. 3 children (stage III) died 9, 14 and 20 months after diagnosis during therapy by metastases. Acute and late effects on normal tissues from radiation and chemotherapy were noted in 12 cases. A review of the literature and therapeutic alternatives in the future are indicated.

Sinus histiocytosis with massive lymphadenopathy and paraparesis: remission with chemotherapy. A case report.

A case of sinus histiocytosis with massive lymphadenopathy (SHML) in which cranial nerve function was impaired is described. Severe paraparesis due to an epidural block at the C2 and C5-T2 levels was also present. Treatment with cytotoxic agents resulted in a dramatic decrease in the size of the involved lymph nodes and disappearance of neurological symptomatology. A review of the literature revealed that, although the disease is benign in its clinical course, the process is not restricted to the lymph nodes. The case reported here is the second case in which the epidural space was affected.