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Biomedical subjects

J Landman

Publications and source records attributed to J Landman.

At least 55 records · Page 3Linked to original sources
PubMed1993

Combined continuous infusion etoposide with high-dose cyclophosphamide for refractory neuroblastoma: a phase II study from the Société Française d'Oncologie Pédiatrique.

PURPOSE: Patients older than 1 year with stage IV neuroblastoma who fail to achieve complete remission (CRem) have a particularly poor long-term prognosis. In an attempt to improve the outcome of these refractory patients, we tested a new drug combination. PATIENTS AND METHODS: Twenty-nine children with advanced neuroblastoma (27 stage IV and two stage III) were entered onto this phase II study. All were refractory to conventional chemotherapy and had measurable disease at the time of the trial. The regimen was a combination of high-dose cyclophosphamide (2 g/m2/d) on days 2, 3, and 4, and etoposide (VP16; 50 mg/m2/d) by continuous intravenous (IV) infusion on days 1 to 5. A pharmacokinetic study of VP16 was conducted in eight patients to determine whether the goal of persistent plasma levels between 1 and 5 micrograms/mL was achieved. RESULTS: Patients received a median of two courses, for a total of 58 courses. The median interval between each course was 32 days. In the 28 assessable patients, the overall response rate was 43%, with one CRem and 11 partial remissions (PRems). No life-threatening complication was observed in these heavily pretreated patients. The median duration of neutropenia (< 5 x 10(9)/L) was 14 days, and that of thrombocytopenia (< 50 x 10(9)/L) was 11 days. The overall incidence of sepsis was 27%. Gastrointestinal toxicity was frequent, but mild. Electrolyte disturbance with antidiuretic hormone (ADH)-like syndrome occurred in eight courses, but resolved rapidly. Grade > or = 2 hemorrhagic cystitis was observed in three courses. No cardiac toxicity was observed. There were no treatment-related deaths. Pharmacokinetic analysis showed that mean steady-state plasma levels (Css) of VP16 were greater than 1 microgram/mL during all the courses. CONCLUSION: This new drug combination appears to be effective in advanced neuroblastoma. Its toxicity remains manageable, with no life-threatening complications. Further evaluation in patients with less-advanced disease is warranted.

Adolescent↗
PubMed1992

Comparison of enteral and intramuscular vitamin A supplementation in preterm infants.

Vitamin A deficiency associated with preterm delivery is not readily reversible using the recommended supplement of 1500 IU per day. It has been reported that 2000 IU of intramuscular vitamin A administered on alternate days for 28 days will correct the deficiency. The objective of this study was to compare this regime with the practice in our nursery of giving 5000 IU of vitamin A per day with the early introduction of feeds. The vitamin A status of ten preterm infants (mean gestation 30.5 weeks) who received intramuscular vitamin supplementation was compared with that of nine infants (mean gestation 30.7 weeks) given enteral vitamin A. Vitamin A status was evaluated on the 32nd day of life using plasma retinol and retinol-binding protein (RBP) concentrations and a modified relative dose response (RDR) test. Plasma retinol and RBP concentrations were similar in the two groups shortly after birth revealing vitamin A deficiency. By the 32nd day of life, plasma retinol and RBP concentrations had risen significantly in both groups and in 70% the modified RDR was normal. Differences between the groups were not observed irrespective of the method of vitamin A administration. None of the infants developed clinical or biochemical vitamin A toxicity. In most preterm infants who tolerate feeds, vitamin A deficiency can be corrected safely by supplementing the feeds with 5000 IU of vitamin A per day.

Enteral Nutrition↗
PubMed1992

Short-term study of chimaerism after bone marrow transplantation for severe aplastic anaemia.

Chimaerism was studied early (2 weeks to 3 months) during haematopoietic reconstitution after bone marrow transplantation in 18 severe aplastic anaemia patients (acquired SAA: 14 patients; Fanconi anaemia: four patients). Fourteen patients received marrow from an identical sibling donor, one from the phenoidentical father and three from a matched unrelated donor. Peripheral blood cell DNA was first analysed by Southern blotting with a multilocus minisatellite probe (33.6.3) or a Y chromosome specific probe (pHY2.1). For all 14 patients grafted with a genotypically identical sibling donor, the post-graft DNA profile strictly matched the respective donor profile (minisatellite probe) or disclosed the Y chromosome specific band in the case of female patients grafted with a male donor. In contrast, for the one patient grafted in a mismatched situation and for two out of three patients grafted with a matched unrelated donor, the results indicated autologous bone marrow recovery. This difference between patients grafted with an identical sibling donor and those grafted in other situations is statistically significant (P less than 0.01). The 15 patients with circulating cells of donor origin were then studied by polymerase chain reaction amplification of the DNA samples. The three male patients with a female donor were studied by amplification of a Y chromosome specific sequence (DYZ1), allowing the detection of one male cell in 10(4) female cells. In all three cases, residual male nucleated cells were detected. The analysis was performed by amplification of the 33.6.3 minisatellite sequence for the 12 remaining patients. No residual recipient cells were detected within the sensitivity limit of the method which is 1% in that case. This suggests that detection of residual host cells depends on the sensitivity of the technique used.

Adolescent↗
PubMed1991

Treatment of hemophagocytic lymphohistiocytosis with chemotherapy and bone marrow transplantation: a single-center study of 22 cases.

Twenty-two children with hemophagocytic lymphohistiocytosis were treated with a chemotherapy regimen consisting of VP16-213, corticosteroids, and intrathecal methotrexate. A sustained clinical and biologic complete remission was obtained in 15 children and a partial remission in one child; six children died early of opportunistic infection (n = 4) or of disease progression (n = 2). Of the 16 children who were placed in first remission, 10 received maintenance chemotherapy alone, while six underwent bone marrow transplantation (HLA matched in five, HLA mismatched in one). Of the children who received chemotherapy alone, only two are in long-term remission after cessation of treatment. The remaining eight patients relapsed after a mean period of 5.4 months (range 2 to 8 months). Further treatment using the same regimen induced second remissions of short duration; death occurred after a median period of 2.3 months (range 0.5 to 6 months). A total of nine patients received allogeneic bone marrow transplantation (BMT). Among the six children transplanted in remission, four are in long-term unmaintained remission, 1 to 6 years after HLA-matched BMT. However, the relapse that occurred in one patient 1 year post BMT is difficult to interpret because the donor, the patient's 5-year-old sister, also developed the disease 1 year later. An HLA-nonidentical BMT resulted in unmaintained remission for 1 year, with autologous hematologic reconstitution followed by disease relapse. HLA-nonidentical BMT failed in three other patients with active disease at time of transplant. The poor long-term results of chemotherapy alone justify the use of related HLA-matched BMT in complete remission.

Adolescent↗
PubMed1990

Calcium regulation of antigen expression on normal and malignant human squamous cells in vitro.

In vitro, normal keratinocytes exhibit undifferentiated morphologic features and proliferate for multiple passages in low-calcium medium (less than or equal to 0.3 mmol/L) whereas, in high-calcium medium (greater than or equal to 1.0 mmol/L), these cells assume differentiation characteristics, begin to stratify, and eventually cease proliferating. In contrast, malignant keratinocytes grow well in high-calcium medium. Expression of pemphigus vulgaris antigen, a squamous cell marker, is altered on cultured normal keratinocytes by calcium. In this study we compared the effects of calcium levels on expression of cell surface antigens by UM-SCC-38, a human squamous carcinoma cell line, and normal keratinocytes cultured from newborn foreskin. Pemphigus, pemphigoid, beta 2-microglobulin antigens, as well as the epidermal growth factor receptor and the A9 germinal epithelial cell basement membrane squamous carcinoma antigen were examined. Pemphigus antigen was strongly expressed on normal and malignant cells in high-calcium but not low-calcium medium. Calcium concentration did not affect the expression of any of the other antigens tested. Thus, although calcium induces differentiation and eventual loss of proliferative capacity in normal but not malignant keratinocytes in vitro, we were unable to demonstrate differences in pemphigus vulgaris antigen expression that might be linked to the growth inhibitory effects induced by high calcium levels in nontransformed epithelial cells in culture.

Antigens, Surface↗
PubMed1990

Poor prognosis infantile fibrosarcoma with pathologic features of malignant fibrous histiocytoma after local recurrence.

In a retrospective study of infants under 1 year of age treated at our institution over a 30-year period for soft tissue tumors, eight fibrosarcomas (FS) were seen, six of which were congenital. Therapy consisted of local excision (n = 3), radiotherapy (n = 1), surgery + radiotherapy (n = 1), surgery + chemotherapy (n = 1), and surgery + chemotherapy + radiotherapy (n = 2). Among these eight patients, four are alive in first complete remission (CR) with 13, 17, 23, and 27 years of follow-up. Of the remaining four patients, two had local recurrences and are still alive in CR after re-excision of the tumor, while the other two had both local and distant relapses and died. Interestingly, in the two patients who developed distant metastases, the pathologic pattern was that of malignant fibrous histiocytoma (MFH) at the time of local recurrence. To our knowledge, no similar cases of transitions between infantile FS known for its favorable outcome and MFH have been reported in this age group. The relevance of such transitions is difficult to assess. However, given the known metastatic potential of MFH, we believe that chemotherapy regimens currently used in the management of childhood soft tissue sarcomas should be used in similar cases.

Child, Preschool↗
PubMed1990

Successful pregnancies in women on regular hemodialysis treatment.

Successful pregnancies in women on regular hemodialysis treatment are infrequent but increasing. We present two such cases; both pregnancies were diagnosed early, and hemodialysis was intensified, leading to significant reductions in predialysis serum urea levels (70-100 mg/dl). One case was particularly unusual in that systemic lupus erythematosus was the underlying disease, and the patient had no residual renal function at the time of conception. Both patients delivered by cesarean section at 32 and 35 weeks, and their infants are well at ages 2 years and 18 months, respectively. The management and the complications associated with such pregnancies are discussed.

Adult↗
PubMed1989

Radiological colpocephaly: a congenital malformation or the result of intrauterine and perinatal brain damage.

The term colpocephaly, meaning disproportional enlargement of the occipital horns of the lateral ventricles, was considered in the past to be a distinct congenital malformation acquired in early intrauterine life. During the last few years several cases were reported in whom a variety of intrauterine and perinatal causes could be associated with this radiological picture. We report on 9 children with radiological colpocephaly in whom intrauterine and/or perinatal injury to the developing brain seemed to be the cause of colpocephaly. It is evident from our observations that "radiological colpocephaly" is a non-specific finding caused frequently by CNS damage acquired during intrauterine and perinatal life.

Brain Diseases↗
PubMed1988

Breast milk jaundice in preterm infants.

A retrospective study was performed comparing bilirubin levels in 40 preterm newborns with uncomplicated courses fed a combination of premature breast milk and formula to those of 60 comparable preterm newborns fed formula only. A significantly higher bilirubin level was noted in the group fed the combined diet on the 6th day of life and on the day of discharge. Seventy-six and seven tenths percent of the preterm infants fed breast milk and formula met the criteria for phototherapy, whereas only 45 percent were treated in the group fed formula alone. Our findings indicate that premature breast milk might cause early and late increase in bilirubin levels in healthy preterm newborns.

Bilirubin↗
PubMed1988

Fracture of the clavicle in the newborn. An ultrasound diagnosis.

Forty-one cases of clavicle fracture in newborn babies were examined by both radiographic and ultrasonic methods. No substantial difference has been found between these two modalities. It is suggested that ultrasound should be the procedure of choice in the diagnosis of clavicle fracture. This is especially true in those cases where impaired movement of the arm is the only clinical sign.

Birth Injuries↗
PubMed1988

Myoglobinuric renal failure in a newborn after traumatic delivery.

Fetal and neonatal asphyxia is the main cause of transient or acute renal failure (ARF) in neonates. Rhabdomyolysis and subsequent myoglobinuria have been rarely reported in neonates. We describe a case of ARF in a newborn infant in whom asphyxia, birth trauma and hypovolemic shock precipitated rhabdomyolysis which contributed to ARF.

Acute Kidney Injury↗
PubMed1987

An infant with multiple deformations born to a myasthenic mother.

An infant with multiple deformations born to a mother with untreated myasthenia gravis presented with arthrogryposis multiplex, craniofacial dysmorphism, kyphoscoliosis of the thoraco-lumbar spine, severe hypotonia, absence of the sucking reflex, and other neurological deficits. The neurological state of the infant supported the diagnosis of congenital myasthenia gravis, but the negative Tensilon test and the lack of clinical improvement after prolonged Mestinon treatment ruled out this diagnosis. We believe that the multiple deformations and reduced fetal movements are related to the maternal myasthenic environment associated with mild polyhydramnion.

Abnormalities, Multiple↗
PubMed1986

Early diagnosis and intrauterine therapy of meconium plug syndrome in the fetus: risks and benefits.

Two cases are reported of patients in the third trimester of pregnancy in whom routine ultrasound examination revealed progressive dilatation of the fetal intestines. Intestinal obstruction was suspected and amniocentesis as well as amniography with Urografin were done. In both cases a regression in dilatation of bowel was noted on ultrasonography. The Urografin swallowed by the fetus was deemed to have relieved the obstruction of the fetal intestine. The babies were delivered in good condition and passed large amounts of watery meconium per rectum. In spite of the risks inherent in the use of Urografin amniography, as well as of amniocentesis, there appear to be a great number of advantages to the treatment of meconium obstruction of the fetus in utero.

Adult↗
PubMed1985

Neonatal factor XIII deficiency.

We describe a patient diagnosed in the neonatal period as having factor XIII deficiency who presented with persistent umbilical bleeding. Factor XIII deficiency is the only coagulation factor deficiency that cannot be detected by classical hemostatic tests, and a rapid diagnosis is vital during the first decade of life. A newborn presenting with persistent umbilical stump bleeding should be screened for factor XIII deficiency when routine coagulation tests prove normal.

Blood Coagulation Tests↗