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Biomedical subjects

H Lackner

Publications and source records attributed to H Lackner.

At least 19 recordsLinked to original sources

The role of sonography in the evaluation of gastro-oesophageal reflux--correlation to pH-metry.

Sonography was compared to pH-metry and/or oesophagomanometry to evaluate the accuracy of sonography in the early diagnosis of gastro-oesophageal reflux. Thirty children with a mean age of 72 days (21-252 days) were studied. The results showed that specificity of sonographic diagnosis was 87.5% and sensitivity was 100% (with P less than 0.001). Sonography also proved helpful in providing both functional and morphological data in addition to pH-metric results. This study therefore suggests that sonography is useful as the first approach in the diagnosis of vomiting babies as it is non-invasive and provides sufficient diagnostic accuracy.

Female

[The effect of high-dose peroral zidovudine treatment in a 4-year-old child with HIV encephalopathy].

We report a four-year-old boy with HIV encephalopathy after vertical HIV infection. On the first admission to our hospital he showed ataxia, loose of expressive language and interstitial pneumonia. After treatment of the pneumonia the patient was started on oral zidovudine with 6 x 6 mg/kg bw/day, because of persisting neurologic symptoms and deep white matter lesions in the MR tomogram of the brain. Two months later he showed an improvement of the gait and the reappearance of the expressive language. Seven months after the start with zidovudine the MR tomogram of the brain revealed the disappearance of white matter lesions with exception of little areas of demyelinisation. No side effects of treatment were observed. The only persisting pathological clinical signs were developmental delay of about a half year and moderately hyperactive tendon reflexes of the lower extremities. Our case suggests that even oral treatment with zidovudine can have a beneficial effect on the HIV encephalopathy in infants.

AIDS Dementia Complex

[Endocrinologic function following cranial irradiation in acute lymphoblastic leukemia in childhood].

Endocrinological function was evaluated in 31 children after successful treatment of acute lymphoblastic leukaemia. All patients had received combination chemotherapy and 12-24Gy of cranial irradiation according to the German therapy protocols BFM-81, BFM-83 and BFM-86. Height, weight, bone age and pubertal development, as well as hypothalamic-pituitary function were measured. Long-term linear growth was unaffected in all patients. However, 9 patients showed subnormal serum growth hormone levels in response to pharmacological stimulation of the pituitary. All patients had normal levels of T3 and T4, but one patient showed an increased response of thyrotropin to thyrotropin releasing hormone. All prepubertal and postpubertal children demonstrated appropriate secretion of follicle-stimulation hormone (FSH) and luteinizing hormone (LH) after stimulation with LH-releasing hormone (LH-RH). 3 pubertal girls showed adequate oestradiol levels, but abnormally high levels of gonadotropins in response to LH-RH. Sexual development was normal in two of them, but the third had a late menarche and irregular menses. The significance of these findings is discussed in the context of recommendations possibly to further reduce or completely delete prophylactic cranial irradiation.

Adolescent

[Infections in the neutropenic phase following bone marrow transplantation: comparison of laminar airflow isolation with conventional isolation].

From May 1978 to September 1989 45 patients underwent 25 allogeneic and 21 autologous bone marrow transplantations (BMT) and 1 peripheral stem cell transplantation for the following indications: severe aplastic anemia (n = 4), hematological malignancies (n = 28), malignant solid tumours (n = 12) and sideroblastic anemia (n = 1). The first group of 20 patients was isolated in a conventional hospital room, while management of the aplastic phase in the second group of 25 patients was performed in a laminar air flow (LAF) unit. All patients received total decontamination. In a retrospective analysis the number of positive blood cultures during the neutropenic period was 85% in the first group, as compared with 40% in the second group, and the number of febrile episodes was 85% versus 64%, respectively. Despite the fact that the septic morbidity was lower in the LAF group, mortality during the neutropenic period (15% in group I versus 16% in group II) was unaffected and survival rate (45% in group I versus 36% in group II) did not improve. We conclude that LAF protection will only have a positive impact on survival rate if the incidence of non-infectious complications of BMT, such as organ toxicity or graft-versus-host disease, is likewise reduced.

Adolescent

Imipenem-antibiotic monotherapy in juvenile cancer patients with neutropenia.

From 1984 to 1987 two consecutive groups of juvenile cancer patients (n = 45) with fever and neutropenia corresponding in all criteria were examined. In half of the total of 90 febrile episodes and septicemias, a conventional antibiotic combination therapy (Pseudomonas-active penicillin/cephalosporin of the third generation/aminoglycoside) was instituted. In the remaining half imipenem was used as an antibiotic monoagent. In 66% and 60% of the febrile episodes treated with antibiotic combination therapy and with imipenem, respectively, septicemia was confirmed by positive blood cultures. Nineteen febrile episodes occurred in the myeloaplastic phase after bone marrow transplantation. In a comparative study of imipenem as monotherapy versus an antibiotic combination therapy the results obtained with imipenem were superior in many regards. No resistance developed necessitating change of antibiotic therapy. Coagulase-negative Staphylococci, primarily responsible for catheter-associated septicemia, were susceptible. Duration of fever and thus duration of treatment were shorter. The incidence of side effects and costs were lower. Therefore, imipenem as an antibiotic monotherapy in febrile cancer patients with neutropenia appears to be more efficacious than the conventional combination therapy, even during myeloaplasia following bone marrow transplantation. The results and rationale of this retrospective analysis are discussed.

Adolescent

[Primary and secondary inoperable teratoid hepatoblastoma--multidisciplinary management].

Hepatoblastomas are the most frequent malignant liver tumors of childhood. Prognosis mostly depends on their resectability. Unresectable tumors are almost invariably fatal, unless their size can be decreased by chemotherapy and/or irradiation to permit surgical removal. In the present case report we discuss the clinical course of a 15 months old boy, who was admitted with primary inoperable teratoid hepatoblastoma. After alternating intravenous and intraarterial chemotherapy as well as radiation therapy, the tumor could be successfully resected. Specific problems of histological diagnosis and of the therapeutic management are discussed, and an overview of the recent literature in behalf of the histological basis, therapeutic possibilities and prognostic factors of hepatoblastomas is given.

Antineoplastic Combined Chemotherapy Protocols

Busulfan/cyclophosphamide plus bone marrow transplantation is not sufficient to eradicate the malignant clone in juvenile chronic myelogenous leukemia.

Bone marrow transplantation plays an essential role in the successful treatment of both juvenile and adult chronic myelogenous leukemia. Recently, it has been reported that conditioning with high doses of busulfan can successfully replace total body irradiation (TBI), in patients with acute myelogenous leukemia as well as adult chronic myelogenous leukemia. We report here the case of a 29-month-old boy with juvenile chronic myelogenous leukemia (JCML) transplanted with HLA-identical bone marrow after conditioning with busulfan, etoposide and cyclophosphamide. Successful engraftment was followed by early relapse on day 67. A second HLA-identical transplant was performed following myeloablative treatment with TBI. Engraftment was once again successful and the patient remains free of disease more than 24 months after transplantation. We conclude that busulfan is insufficient in eradicating JCML and that TBI is required prior to transplantation.

Antineoplastic Combined Chemotherapy Protocols

[Teratoma of the stomach in a 4-day-old newborn infant].

Teratoma of the stomach is a very rare tumor. Nearly all previously reported patients were males. We report a 4 day-old male infant, who presented with a palpable abdominal mass. A preliminary diagnosis of teratoma was made by abdominal x-ray, sonography and MR imaging. Surgery was performed and diagnosis of teratoma of the stomach was confirmed by histological examination of the tumor. Because teratoma of the stomach is always a benign neoplasm surgery is curative and no further therapy is needed.

Diagnosis, Differential

[Ingestion of caustic substances in childhood].

Patients who ingest caustic substances continue to pose difficult problems in diagnosis and management. Flexible fiberoptic endoscopy and radiological studies have helped to assess the extent of damage caused by corrosives. A number of non surgical techniques are available for therapy, mainly corticosteroids and antibiotics. In a retrospective study from January 1976 till October 1988 we analysed 86 children aged from 1 month to 11 years. The purpose of our analysis was to check the outcome in patients with corrosive burning treated by standardized therapeutic management. Alkaline household substances caused the injury in 75% of our patients. No lesions were found in 19 patients. There were 21 patients with first degree, 28 with second degree ans 18 with third degree lesions. The diagnosis was confirmed by endoscopy within the first 48 hours. Therapeutic management in 2nd and 3rd degree lesions was based on corticosteroids, antibiotics and early bougienage of the oesophagus over an endless thread. Three patients with 2nd degree and all 18 patients with 3rd degree lesions underwent bougienage. Corticosteroids were given for two to four weeks, depending on the severity of the lesion. Our results demonstrate that early diagnosis and therapy can help to reduce stenosis and prolonged illness of these patients. In addition, public health efforts should be made to educate people about the dangers of caustic substances.

Burns, Chemical

Successful treatment of CMV retinitis with ganciclovir after allogeneic marrow transplantation.

Cytomegalovirus (CMV) infection of the retina is a well recognized complication in patients with the acquired immune deficiency syndrome but is rarely seen after bone marrow transplantation (BMT). Among a variety of drugs ganciclovir so far appears to be the most effective therapy for CMV retinitis, but in previous studies relapses occurred in all patients in whom ganciclovir was interrupted. We report the clinical findings in a 22-year-old BMT recipient who developed bilateral exudative CMV retinitis 64 days after BMT despite prophylactic treatment with high-titer CMV-immunoglobulins and transfusions of CMV-negative blood products and donor bone marrow. During a 12 day course of treatment with 7.5 mg/kg/day of ganciclovir the CMV retinitis improved and viruria ceased on day 4 of therapy. In contrast to the previous reports, CMV retinitis in this patient continued to improve even after ganciclovir was stopped and eventually complete healing of all intraretinal lesions as well as total reconstitution of the visual acuity was achieved. He is now free of disease and without relapse of CMV retinitis more than 1 year after transplantation.

Adult

[Cyclosporin A in severe aplastic anemia in children].

Severe aplastic anemia should be treated with bone marrow transplantation if possible. Various clinical and experimental data support the view that the major pathogenetic defect in SAA is a dysregulated cellular immune response which in turn has a negative effect upon hematopoiesis. Therefore a large percentage of patients react favorably to treatment with immunosuppressive agents as antithymocyte globulin and high-dose methylprednisolone. Data concerning the efficiency of Cyclosporine A treatment are limited until now. We present in this report our own experience with Cyclosporine A treatment in three children with severe aplastic anemia who lack a bone marrow donor. Included is also a review of the present therapeutic possibilities.

Adolescent

[Malignant bone marrow infiltration in nuclear magnetic resonance tomography].

Magnetic resonance imaging (MRI) is currently the only non-invasive method detecting changes of bone marrow. While yellow bone marrow produces a high signal intensity, which is similar to subcutaneous fat, any other cellular infiltration of the bone marrow causes a decrease of signal intensity because of replacement of marrow fat cells. In this report we describe two patients, who underwent MRI because of clinical symptoms of coxitis, in order to exclude Perthes disease. Both cases showed decreased signal intensity of the bone marrow particularly of the proximal femura, highly indicative of cellular bone marrow infiltration. Bone marrow aspiration in these patients showed acute lymphocytic leukemia in one case and marrow infiltration by neuroblastoma cells respectively in the other case. Because of its high sensitivity, MRI is also suitable in detecting focal bone marrow disease. For these cases a biopsy of these focal bone marrow lesions can be performed in order to stage the disease properly as in the presence of neuroblastoma or malignant lymphoma presenting with localized disease, however, with focal bone marrow metastases on MRI. In addition MRI can also be used to follow up the disease as well as therapy by showing the regression of these bone marrow lesions.

Adrenal Gland Neoplasms