PubMed Health⌕ Search

Biomedical subjects

R Haase

Publications and source records attributed to R Haase.

At least 19 recordsLinked to original sources

[Pasteurella multocida as a rare cause of neonatal meningitis].

Pasteurella multocida (P. multocida), a Gram-negative bacillus is a typical commensal in the oropharynx of animals such as dogs and cats. Human diseases caused by P. multocida are rare and include respiratory infections, sepsis and meningitis. P. multocida infections are described predominantly in patients with underlying chronic disorders, in elderly patients or in infants < or = 1 year. We describe the case of a 3-week-old boy with meningitis due to Pasteurella multocida. P. multocida was also identified in oral swab and stool cultures of the pet cat. A direct contact between baby and cat was negated by the parents. The patient recovered without any neurological sequelae.

Humans↗

[Epidural haematoma in a newborn].

Birth injuries occur occasionally but, in contrast, perinatally acquired epidural and intracerebral hematomas as well as neonatal skull fractures are extremely rare. The appropriate diagnostic and therapeutic modalities depend on the size and site of the injury as well as any accompanying diseases. We report the case of a neonatal epidural and intracerebral haematoma with skull fracture following secondary caesarean section. There was no evidence for trauma during pregnancy, birth injury or other possible causes of bleeding. The haematoma was decompressed surgically. One year after surgery the boy suffers from moderate neurological compromise and focal seizures.

Apgar Score↗

[Thrombosis of the iliac artery in a premature neonate: thrombolytic therapy using rt-PA].

Thromboembolic events in neonates are very rare. They are often associated with severe disease affecting the newborn or are secondary to central venous lines or arterial catheters. Most of the described cases of thromboses of the iliac or femoral arteries are associated with cardiac catheterisation or femoral invasive blood pressure monitoring. The relationship between single umbilical arteries and an increased incidence of structural and chromosomal anomalies is well known, but a higher rate of thromboembolic disease in infants with single umbilical arteries has not been described. Rt-PA (recombinant tissue plasminogen activator) has been successfully used in small studies and numerous case reports. To date controlled clinical trials giving guidelines for antithrombotic therapy using rt-PA are still lacking. We report the clinical course of a 700 g premature male, who was born by Caesarean section at 29 + 6 gestational weeks. On the fifth day the baby suffered from arterial thrombosis of the right pelvis axis. Antenatally a single umbilical artery was identified. Iliac arteries on the involved site appeared hypoplastic. Additionally, the prothrombin G20210A mutation was found. The patient was treated successfully using recombinant tissue plasminogen activator. In the case of a high risk of limb or organ loss due to arterial thrombosis, thrombolysis using rt-PA is justified. Appropriate rt-PA treatment has been studied for the adult but not the paediatric population. Hence, well-designed clinical trials are necessary to determine the pharmacokinetics and dynamics of thrombolytic agents in children.

Dandy-Walker Syndrome↗

High-dose therapy for patients with primary multifocal and early relapsed Ewing's tumors: results of two consecutive regimens assessing the role of total-body irradiation.

PURPOSE: Risk stratification of metastatic and relapsed Ewing's tumors (ETs) has been a matter of debate during the last decade. Patients with bone or bone marrow metastases or early or multiple relapses constitute the worst risk group in ET and have a poorer prognosis than patients with primary lung metastases or late relapses. In this article, the results of the present Meta European Intergroup Cooperative Ewing Sarcoma Study (MetaEICESS) (tandem melphalan/etoposide [TandemME]) were compared with the result of the previous study (hyper melphalan/etoposide [HyperME]), both at 5 years, in a patient population within the same high-risk stratum to determine toxicity. PATIENTS AND METHODS: Among 54 eligible patients, 26 were treated according to the HyperME protocol, and 28 were treated according to TandemME protocol. Patients received six cycles of the Cooperative Ewing Sarcoma Study treatment in HyperME and six cycles of the EICESS treatment in TandemME as induction chemotherapy. Patients also received involved-compartment irradiation for local intensification and myeloablative systemic intensification consolidation with hyperfractionated total-body irradiation (TBI) combined with melphalan/etoposide in HyperME or two times the melphalan/etoposide in TandemME followed by autologous stem-cell transplantation. RESULTS: The event-free survival (EFS) rate +/- SD in HyperME and TandemME was 22% +/- 8% and 29% +/- 9%, respectively. The dead of complication rate was 23% in HyperME and 4% in TandemME. CONCLUSION: TandemME offers a decent, albeit still not satisfactory, rate of long-term remissions in most advanced ETs (AETs), with short-term treatment and acceptable toxicity. TBI was not required to maintain EFS level in this setting but was associated with a high rate of toxic death. Future prospective studies in unselected patients are warranted to evaluate high-dose therapy in an unselected group of patients with AET.

Adolescent↗

Unusual presentation of central nervous system relapse with oculomotor nerve palsy in a case of CD56-positive acute myeloid leukemia following allogeneic stem cell transplantation.

Allogeneic stem cell transplantation (allo-SCT) plays an important role in the treatment of infants and children with acute myelogenous leukemia (AML). Leukemic relapse after allo-SCT is responsible for a high rate of treatment failure. Extra-medullary relapse (EMR), without involvement of bone marrow, is rare compared to medullary relapse. CD56, the neural cell adhesion molecule, may contribute to the higher frequency of CNS relapse in CD56-positive AML. We observed an isolated EMR on the oculomotor nerve of a 17-month-old girl 12 weeks after cord blood transplantation (CBT), who was transplanted because of CD56-positive AML. Diagnosis of relapse was suspected clinically and confirmed by magnetic resonance imaging (MRI), and fluorescence-activated cell sorter (FACS) and chimerism analysis of cerebrospinal fluid (CSF). Therapy consisted of intra-thecal chemotherapy, CNS irradiation, and systemic immunomodulation by cyclosporin A (CsA) and basiliximab withdrawal. Twenty-one months after relapse, the patient shows full remission of symptoms and previously described oculomotor nerve infiltration.

CD56 Antigen↗

Treatment with cyclosporin A in a patient with Omenn's syndrome.

Unless treated with haematopoetic stem cell transplantation, Omenn's syndrome, a rare variant of severe combined immunodeficiency, is associated with a fatal outcome. We describe a male infant showing all the typical features of Omenn's syndrome, who was successfully treated with cyclosporin A to improve clinical condition prior to haematopoetic stem cell transplantation.

Cyclosporine↗

Arterial complications after liver transplantation.

From September 1988 through April 1998, 1,000 liver transplantations were performed on 911 patients. During the postoperative control examinations of 837 patients, we found 23 (2.74 %) with hepatic artery thromboses, 27 stenoses of the hepatic artery (3.22 %), and 6 aneurysms of the graft artery. Seventeen patients underwent retransplantation because of arterial complications. Depending on the clinical symptoms, we treated both the local situation as well as the resulting complications of inadequate arterial graft flow. The aneurysms were primarily treated surgically. The first choice of treatment of stenoses was balloon angioplasty. Early postoperative artery thromboses were also treated surgically by thrombectomy in selected cases. For the resulting biliary and local septic complications we preferred endoscopic and drainage procedures. Our clinical experiences have led us to find pretransplantation angiography recommendable, especially in the case of splanchnic artery stenoses, for bypassing from the aorta for arterial perfusion of the graft.

Adult↗

Allogeneic and autologous stem-cell transplantation in advanced Ewing tumors. An update after long-term follow-up from two centers of the European Intergroup study EICESS. Stem-Cell Transplant Programs at Düsseldorf University Medical Center, Germany and St. Anna Kinderspital, Vienna, Austria.

BACKGROUND: An update of results from the High Risk Protocol of the Meta-EICESS Study, conducted at the Pediatric Stem-Cell Transplant Centers of Düsseldorf and Vienna. In order to evaluate a possible therapeutic benefit after allogeneic SCT in patients with advanced Ewing tumors (AET), we compared outcome after autologous and allogeneic stem-cell transplantation (SCT). PATIENTS AND METHODS: We analyzed 36 patients treated with the myeloablative Hyper-ME protocol (hyperfractionated total body irradiation, melphalan, etoposide +/- carboplatin) between November 1986 and December 1994. Minimal follow-up for all patients was five years. All patients underwent remission induction chemotherapy and local treatment before myeloablative therapy. Seventeen of thirty-six patients had multifocal primary Ewing's tumor, eighteen of thirty-six had early, multiple or multifocal relapse, one of thirty-six patients had unifocal late relapse. Twenty-six of thirty-six were treated with autologous and ten of thirty-six with allogeneic hematopoietic stem cells. We analyzed the following risk factors, that could possibly influence the event-free survival (EFS): number of involved bones, degree of remission at time of SCT, type of graft, indication for SCT, bone marrow infiltration, bone with concomitant lung disease, age at time of diagnosis, pelvic involvement, involved compartment radiation, histopathological diagnosis. RESULTS: EFS for the 36 patients was 0.24 (0.21) +/- 0.07. Eighteen of thirty-six patients suffered relapse or died of disease, nine of thirty-six died of treatment related toxicity (DOC). Nine of thirty-six patients are alive in CR. Age > or = 17 years at initial diagnosis (P < 0.005) significantly deteriorated outcome. According to the type of graft, EFS was 0.25 +/- 0.08 after autologous and 0.20 +/- 0.13 after allogeneic SCT. Incidence of DOC was more than twice as high after allogeneic (40%) compared to autologous (19%) SCT, even though the difference did not reach significance (P = 0.08, Fisher's exact test). CONCLUSIONS: Because of the rather short observation period. secondary malignant neoplasms (SMN) may complicate the future clinical course of some of our patients who are currently viewed as event-free survivors. EFS in AET is not improved by allogeneic SCT due to a higher complication rate. The patient group was to small to analyze for a possible graft-versus-tumor effect.

Adolescent↗

Venous complications after orthotopic liver transplantation.

Complications involving the portal vein or the vena cava, are rare after orthotopic liver transplantation. We report on the incidence and treatment of venous complications following 1000 orthotopic liver transplantations in 911 patients. Twenty-six of the adult patients (2.7%) suffered from portal complications after transplantation, whereas complications of the vena cava were observed in only 17 patients (1.8%). Technical problems or recurrence of the underlying disease (e.g. Budd-Chiari syndrome) accounted for the majority of complications of the vena cava, whereas alteration of the vessel wall or splenectomy during transplantation could be identified as important risk factors for portal vein complications. In patients undergoing modification of the standard end-to-end veno-venous anastomosis of the portal vein due to pathological changes of the vessel wall, complications occurred in 8.3%, whereas only 2.4% of patients who received a standard anastomosis of the portal vein experienced complications of the portal vein. Furthermore, splenectomy during transplantation was also associated with an increased incidence of portal vein complications (10.5 vs. 2.2% in patients without splenectomy). Treatment was dependent on the signs and symptoms of the patients, and varied considerably between patients with portal vein complications and patients suffering from complications of the vena cava. Complications of the vena cava led to retransplantation in about one-third of the patients, whereas in patients with occlusion of the portal vein, retransplantation was necessary in only 15%, and more than half of the patients suffering from portal vein complications did not require any treatment at all. Usually, treatment of patients with portal vein complications only became necessary when additional complications such as arterial occlusion or bile duct injuries occurred.

Adult↗

Increasing prevalence of overweight among Wisconsin adults, 1989-1998.

OBJECTIVES: Overweight is an important condition that increases the risk of chronic diseases such as diabetes, heart disease, and cancer. We examined trends in overweight prevalence among Wisconsin adults from 1989 to 1998 and assessed progress in reaching the overweight 2000 goal for Wisconsin. METHODS: We used data on the self-reported height, weight, and calculated body mass index from the Wisconsin Behavioral Risk Factor Survey to examine overweight prevalence trends among Wisconsin adults. RESULTS: The prevalence of overweight has increased over 40% during the past decade from 23% of adults in 1989, to 34% of adults in 1998. The increase occurred in men and women, blacks and whites, and all age groups. In 1998, 330,000 more adults were overweight in Wisconsin, compared to 1989. CONCLUSION: As rates of overweight continue to increase, the incidence and prevalence of many chronic diseases will also increase. Effective population-based efforts are required to reduce caloric intake and increase physical activity among Wisconsin adults.

Adult↗

Variations of surgical reconstruction in liver transplantation depending on vasculature.

BACKGROUND: From September 1988 through April 1998, 1000 liver transplantations were performed on 911 patients. The standard technique for liver re-vascularization to guarantee an optimal blood inflow during transplantation was modified in 19% of the cases on the arterial side and in 5.6% of the cases on the portal side as a result of unusual anatomical features and pathological changes in the vasculature of the organ recipient. In 113 transplantations, successful reconstruction of accessory vessels of the graft (12 left and 101 right hepatic arteries) was performed without complications. It is our opinion that preoperative diagnosis of the vasculature (stenoses of the celiac trunk etc.) of the organ recipient by duplexsonography and angiography is necessary. Even with the help of these tests, it is extremely difficult to diagnose a "steal" syndrome in the splenic artery: for example, 31 of 40 patients with poor liver function received postoperative therapy for newly diagnosed "steals". RESULTS AND CONCLUSIONS: There is no increase in complications (stenosis and thrombosis) with modifications of arterial reconstruction (4.9 vs 6.3%); however, with modification of portal reconstruction the increase is from 2.4% to 8.3%.

Adult↗

Oral combination chemotherapy in the management of AIDS-related lymphoproliferative malignancies.

An oral combination chemotherapy regimen initially developed for AIDS-related non-Hodgkin's lymphoma includes lomustine (CCNU), etoposide, cyclophosphamide, and procarbazine. This regimen takes advantage of oral administration, the in vitro synergy of these drugs and their first-line efficacy in lymphoma, and the ability of lomustine and procarbazine to cross the blood-brain barrier. This regimen was used to treat 38 patients with AIDS-related non-Hodgkin's lymphoma. The overall objective response rate was 66% (34% complete response rate) with a 5% CNS relapse rate, and a median survival duration of 7.0 months. One-third of the patients survived for 1 year, 11% for 2 years, and half of the patients survived free from progression of their lymphoma. On the basis of these results, this oral regimen was modified and administered to 5 patients with AIDS-related primary CNS lymphoma as part of a sequential combined-modality chemotherapy and radiation regimen. Rapid progression of CNS disease was observed in this group of patients, with a median survival duration of 1.0 month. The identical regimen was administered to 7 patients with AIDS-related Hodgkin's disease: we observed a 71% partial remission rate and a median survival duration of 7.0 months. Myelosuppression remains the most significant clinical toxicity. Our results with this oral regimen appear comparable to those of standard intravenous combination chemotherapy regimens in patients with AIDS-related non-Hodgkin's lymphoma.

Administration, Oral↗

Effect of small doses of iodine on thyroid function in patients with Hashimoto's thyroiditis residing in an area of mild iodine deficiency.

OBJECTIVE: Several studies have suggested that iodine may influence thyroid hormone status, and perhaps antibody production, in patients with autoimmune thyroid disease. To date, studies have been carried out using large amounts of iodine. Therefore, we evaluated the effect of small doses of iodine on thyroid function and thyroid antibody levels in euthyroid patients with Hashimoto's thyroiditis who were living in an area of mild dietary iodine deficiency. METHODS: Forty patients who tested positive for anti-thyroid (TPO) antibodies or with a moderate to severe hypoechogenic pattern on ultrasound received 250 microg potassium iodide daily for 4 months (range 2-13 months). An additional 43 patients positive for TPO antibodies or with hypoechogenicity on ultrasound served as a control group. All patients were TBII negative. RESULTS: Seven patients in the iodine-treated group developed subclinical hypothyroidism and one patient became hypothyroid. Three of the seven who were subclinically hypothyroid became euthyroid again when iodine treatment was stopped. One patient developed hyperthyroidism with a concomitant increase in TBII titre to 17 U/l, but after iodine withdrawal this patient became euthyroid again. Only one patient in the control group developed subclinical hypothyroidism during the same time period. All nine patients who developed thyroid dysfunction had reduced echogenicity on ultrasound. Four of the eight patients who developed subclinical hypothyroidism had TSH concentrations greater than 3 mU/l. In 32 patients in the iodine-treated group and 42 in the control group, no significant changes in thyroid function, antibody titres or thyroid volume were observed. CONCLUSIONS: Small amounts of supplementary iodine (250 microg) cause slight but significant changes in thyroid hormone function in predisposed individuals.

Adult↗

Mutations of the ret protooncogene in German multiple endocrine neoplasia families: relation between genotype and phenotype. German Medullary Thyroid Carcinoma Study Group.

It has been suggested that not only the position but also the nature of the mutations of the ret protooncogene strongly correlate with the clinical manifestation of the multiple endocrine neoplasm type 2 (MEN 2) syndrome. In particular, individuals with a Cys634-Arg substitution should have a greater risk of developing parathyroid disease. We, therefore, analyzed 94 unrelated families from Germany with inherited medullary thyroid carcinoma (MTC) for mutation of the ret protooncogene. In all but 1 of 59 families with MEN 2A, germline mutations in the extracellular domain of the ret protein were found. Some 81% of the MEN 2A mutations affected codon 634. Phenotype-genotype correlations suggested that the prevalence of pheochromocytoma and hyperparathyroidism is significantly higher in families with codon 634 mutations, but there was no correlation with the nature of the mutation. In all but 1 of 27 familial MTC (FMTC) families, mutations were detected in 1 of 4 cysteines in the extracellular domain of the ret protooncogene. Half of the FMTC mutations affected codon 634. Mutations outside of codon 634 occurred more often in FMTC families than in MEN 2A families. In all but 1 of 8 MEN 2B patients, de novo mutations in codon 918 were found. These data confirm the preferential localization of MEN 2-associated mutations and the correlation between disease phenotype and the position of the ret mutation, but there was no correlation between the occurrence of hyperparathyroidism or pheochromocytoma and the nature of the mutation.

Amino Acid Sequence↗

Long-term treatment of acromegalic patients with repeatable parenteral depot-bromocriptine.

We studied the efficacy and tolerability of a repeatable long-acting parenteral depot-bromocriptine preparation (Parlodel LAR) in 14 acromegalic patients, 10 of whom had received oral bromocriptine therapy previously, 2 of them showing intolerance to oral bromocriptine. Patients received i.m. injections of 50-100 mg depot-bromocriptine at 4-week intervals for 3-24 months (median 6). Growth hormone profiles were assessed by four daily samples at 4-week intervals. Main daily growth hormone levels decreased from 52.1 +/- 12.3 micrograms/l (mean +/- SEM) to 19.4 +/- 4.7 micrograms/l on the day of injection. In 6 patients, growth hormone values were lowered by more than 50%, whereas IGF-I levels decreased only slightly and growth hormone values during the oral glucose tolerance test remained non-suppressible. Tumour sizes were not affected. Two women became pregnant and were delivered of healthy babies. Side-effects typical of bromocriptine occurred frequently on the days of injection and diminished in most patients after 2 months of therapy despite increasing dosage. Compared with previous oral bromocriptine therapy, 9 of 10 patients preferred the depot preparation, whereas the reduction of growth hormone levels was similar during both treatments. In conclusion, depot-bromocriptine should be considered for acromegalic patients intolerant to oral bromocriptine.

Acromegaly↗