bluuebunny/crossref_metadata_2025 huggingface dataset:
Kaposi’s Sarcoma following Allogeneic Hematopoietic Stem Cell Transplantation for Chronic Myelogenous Leukemia
Authors: de Medeiros B.C. , Rezuke W.N. , Ricci Jr. A. , Tsongalis G. , Shen P.U.F. , Bona R.D. , Feingold J.M. , Edwards R.L. , Tutschka P.J. , Bilgrami S.
Published: April 2003

Unlike solid organ transplantation, Kaposi’s sarcoma (KS) occurs rarely following hematopoietic stem cell transplantation (HSCT). In fact, only 5 cases of KS have been reported after allogeneic or autologous HSCT. The usual treatment combines a substantial decrease in, or elimination of, immunosuppressive therapy along with local measures such as surgical excision, cryotherapy or radiation therapy. A 46-year-old woman with chronic myelogenous leukemia who had received an allogeneic HSCT previously from an HLA-identical sibling, presented on day +814 with human herpes virus-8-associated KS involving her left lower extremity. She had been on continuous immunosuppressive therapy since her transplant because of chronic graft-versus-host disease. The intensity of immunosuppressive therapy was decreased once a diagnosis of KS had been established. However, the nodular lesions continued to progress in size and number. Therefore, a course of irradiation was administered to sites of bulk disease on her legs. Furthermore, thalidomide was initiated along with a topical retinoid, alitretinoin 0.1% gel applied twice daily to the nonirradiated lesions. This approach yielded a partial response in both irradiated and nonirradiated lesions over the course of the following 7 months. Both thalidomide and alitretinoin 0.1% gel appear to be beneficial in HSCT-associated KS and exhibit tolerable side effects.
Thromboembolic Events in Beta Thalassemia Major: An Italian Multicenter Study
Authors: Borgna Pignatti C. , Carnelli V. , Caruso V. , Dore F. , De Mattia D. , Di Palma A. , Di Gregorio F. , Romeo M.A. , Longhi R. , Mangiagli A. , Melevendi C. , Pizzarelli G. , Musumeci S.
Published: April 2003

Thromboembolic (TE) events have been frequently reported in β-thalassemic patients in association with known risk factors such as diabetes, complex cardiopulmonary abnormalities, hypothyroidism, liver function anomalies, and postsplenectomy thrombocytosis. In a recent survey involving 9 Italian thalassemic centers, we identified 32 patients with TE episodes in a total of 735 subjects, of whom 683 had thalassemia major and 52 thalassemia intermedia, corresponding to 3.95 and 9.61%, respectively. There was a great variation in localization: the main one (16/32) was CNS, with a clinical picture of headache, seizures and hemiparesis. Other localizations were the pulmonary (3 patients), mesenteric (1 patient) and portal (2 patients) sites. There were 6 cases of deep venous thrombosis (2 in the upper limbs, 4 in the lower ones). Intracardiac thrombosis was found in 2 subjects and clinical and laboratory signs of DIC were observed in 2 others during pregnancy. Since our patients with TE events present a statistically significantly higher incidence of associated dysfunction (cardiomyopathy, diabetes, liver function anomalies, hypothyroidism) than those without TE events (50 vs. 13.8%), we suggest close monitoring of those patients who are at higher risk of developing TE events because of the presence of one or more of these predisposing factors.
Autoimmune Thrombocytopenia Associated with Hepatitis C Virus Infection
Authors: Hernández Fernando , Blanquer Amando , Linares Mariano , López Alfonso , Tarín Fabián , Cerveró Antonio
Published: April 2003

We have retrospectively analyzed a series of 19 patients with hepatitis C virus (HCV) infection and chronic thrombocytopenia not attributable to hypersplenism or to other causes. Antiplatelet antibodies were present in 81% of cases. Response to prednisone was observed in 6 of 7 patients and 1 of 3 patients responded to intravenous immunoglobulins. No case of reactivation of liver disease was observed during or after therapy. We consider that the possibility of an underlying mechanism should be evaluated in thrombocytopenic patients with HCV infection who do not present hypersplenism. These patients could benefit from steroid treatment.
Expression of Spectrin αI/50 Hereditary Elliptocytosis and Its Association with the αLELY Allele
Authors: Bassères Daniela S. , Pranke Patricia H.L. , Vicentim Dilmara , Costa Fernando F. , Saad Sara T.O.
Published: April 2003

Hereditary elliptocytosis (HE) is a group of hemolytic anemias characterized by the presence of elliptical erythrocytes. The underlying alterations lie in the proteins of the membrane skeleton. Defects of the αI domain of spectrin have been defined based on a decrease in the normal 80-kD αI domain and a concomitant increase in one or more lower molecular weight peptides. We have studied three Brazilian kindreds with black ancestry, who presented mild common spαI/50 HE. Our aim was to determine the molecular alteration responsible for the spαI/50 HE observed in these three kindreds and to evaluate the presence and influence of allele αLELY in the expression of this type of HE. In order to establish the molecular defect, exons 5, 6 and 11 were amplified and submitted to a nonradioactive single strand conformation polymorphism protocol. An identical band shift in exon 6 was observed in all 3 patients and their affected relatives. Direct sequencing of the amplification products of exon 6 showed the same molecular defect in all patients: a T→C substitution, responsible for the L260P mutation. Allele αLELY, detected by PCR and restriction enzyme digestion, was present in the heterozygous form in the three propositi and was associated in trans with the elliptocytogenic mutation. Blood smears of the patients with HE and αLELY in trans showed pronounced elliptocytosis, poikilocytosis and a few small red cell fragments, whereas the blood smears of their relatives, who had HE without allele αLELY, showed mild common HE with a predominance of ovalocytes and the absence of poikilocytes. We conclude that allele αLELY does not lead to the worsening of clinical conditions when associated in trans with mild HE, but can be easily distinguished by a blood smear analysis. The predominance of the L260P mutation in the kindreds studied could be related to the colonization of Brazil during the slave trade by Africans from the Benin-Togo area, where this mutation is particularly common.
Fluorescence in situ Hybridization and Surface Markers of Fine Needle Aspiration Specimen Confirm Extramedullary Myelobastoma in a Patient with Chronic Myeloid Leukemia
Authors: Karthaus M. , Meran J.G. , Wilkens L. , Soudah B. , Diedrich H. , Krauter J. , Ganser A. , Heil G.
Published: April 2003

We report on a patient with chronic myeloid leukemia (CML) with a rapidly growing left cervical tumor 5 months after the initial diagnosis of CML. This tumor was diagnosed as a very early manifestation of extramedullary myeloblastoma by a minimally invasive method. A fine needle aspirate (26-gauge needle) was obtained from the tumor. Morphological and cytochemical analysis of the aspirate revealed 19% undifferentiated blasts. The immunophenotype was suggestive of a myeloid differentiation of the blasts (CD33+). The CML origin of the blasts was confirmed by the detection of the bcr-abl gene rearrangement in the blasts by two-color fluorescence in situ hybridization (FISH). We conclude that fine needle aspiration in combination with immunophenotyping and FISH analysis of the aspirate is a minimally invasive and rapid diagnostic tool to confirm extramedullary manifestation in CML. To our knowledge, this is the first case of extramedullary myeloblastoma confirmed by this combined technique.
Changes in Serum Thrombopoietin Levels after Splenectomy
Authors: Ichikawa Naoaki , Kitano Kiyoshi , Shimodaira Shigetaka , Ishida Fumihiro , Ito Toshiro , Kajikawa Shoji , Tahara Tomoyuki , Kato Takashi , Kiyosawa Kendo
Published: April 2003

To clarify the role of thrombopoietin (c-Mpl ligand, TPO) in ‘hypersplenic’ thrombocytopenia, we used an enzyme-linked immunosorbent assay to examine changes in serum TPO levels accompanied with splenectomy in 6 patients with liver cirrhosis, 4 patients with gastric cancer, and 2 patients with lymphoid malignancies. We also measured serum levels of other thrombopoietic cytokines such as interleukin-6 (IL-6) and erythropoietin. Platelet counts reached a maximum at day 14 after splenectomy in all subjects. In patients with liver cirrhosis, a lower elevation of platelet counts was observed compared with that in patients with gastric cancer. Serum TPO levels gradually elevated after splenectomy and reached a maximum 3.5 days after splenectomy in noncirrhotic patients, whereas peak serum TPO levels were delayed until day 7 in the cirrhosis group. IL-6 and erythropoietin showed similar kinetics between cirrhotic and noncirrhotic patients. These findings suggest that transient thrombocytosis after splenectomy may be associated with an alteration in the site of TPO catabolism by platelets from spleen to the blood and that deterioration of TPO production may play a role in thrombocytopenia in liver cirrhosis.
Pseudoreticulocytosis in a Case of Myelodysplastic Syndrome
Authors: Carulli Giovanni , Marini Alessandra , Azzarà Antonio , Lucchetti Annalia , Petrini Mario
Published: April 2003

A patient with normocytic anemia and marked and persistent reticulocytosis is presented. Causes responsible for blood loss and hemolytic diseases were excluded and, after bone marrow examination, myelodysplastic syndrome (refractory anemia) was diagnosed. In vitro reticulocyte survival studies suggested that reticulocytosis was a consequence of delayed maturation of the reticulocytes. Pseudoreticulocytosis may be an unusual presentation of myelodysplastic syndromes, because only 4 patients with such a finding have previously been reported.
Rheumatic Manifestations Preceding Adult Acute Leukemia: Characteristics and Implication in Course and Prognosis
Authors: Gur Hanan , Koren Vladimir , Ehrenfeld Michael , Ben-Bassat Isaac , Sidi Yechezkel
Published: April 2003

The manifestations and outcome of adult patients with acute leukemia (AL) were examined to study the characteristics of the rheumatological prodrome of AL and to find whether it is a marker of a distinct clinical and laboratory course and whether it has any prognostic implication. During a 10-year period, 8/139 (5.8%) of AL patients presented with rheumatic manifestations. The average duration of the arthritis syndrome preceding the diagnosis of AL was 3.25 months. The most common pattern of presentation was a reactive arthritis-like syndrome involving the large joints asymmetrically and associated with low back pain. Distinctive features suggesting a paraneoplastic arthritis were severe pain disproportionate to physical findings, a poor response to conventional antirheumatic treatment, and early significant osteopenia or lytic bone lesions. The epidemiological, clinical and laboratory characteristics of patients with or without rheumatic manifestations were comparable, except for fever on presentation, the presence of transient metabolic derangement following chemotherapy, and the initial average hemoglobin, hematocrit and serum uric acid values. Moreover, the initial outcome of the two groups was similar, as the early mortality rates were comparable (42.8% vs. 45% for patients with or without arthritis, respectively). In conclusion, rheumatic syndrome presentation of adult AL is uncommon, and apparently has no deleterious effects on initial prognosis. A timely diagnosis requires an increased awareness to distinctive features.
Ultrastructural Changes of Erythrocyte Membrane Skeletons in Chorea-Acanthocytosis and McLeod Syndrome Revealed by the Quick-Freezing and Deep-Etching Method
Authors: Terada Nobuo , Fujii Yasuhisa , Ueda Hideho , Kato Yasuko , Baba Takeshi , Hayashi Ryoichi , Ohno Shinichi
Published: April 2003

The shapes of acanthocytic erythrocytes have been thought to be related to changes of the organization of the membrane components. In this study, acanthocytes from a patient with chorea-acanthocytosis and a patient with McLeod syndrome were examined. These acanthocytes had a greater tendency to form spikes than normal erythrocytes in an incubation culture system, indicating their weakness against membrane tension. The membrane skeletons of erythrocytes from both patients were examined by our erythrocyte-splitting method followed by the quick-freezing and deep-etching method. The ultrastructural organization of the membrane skeletons was heterogeneous even in individual erythrocytes, indicating regional changes in the compactness of their meshworks. In the process of erythrocyte splitting, some erythrocyte membranes became reversed and protruded into the cytoplasmic side. In these regions, there were fewer filamentous structures. The focal membrane skeletal changes, which are probably one of the factors that induce the phenotype of an acanthocytic erythrocyte, were demonstrated for the first time by this method.
Effect of Granulocyte/Colony-Stimulating Factor on the Onset of the Adult Respiratory Distress Syndrome
Authors: Takahashi Yufuko , Kobayashi Yutaka , Chikayama Satoshi , Ikeda Motomi , Kondo Motoharu
Published: April 2003

To evaluate the effect of granulocyte/colony-stimulating factor (G-CSF) on the onset of the adult respiratory distress syndrome (ARDS), we investigated whether the incidence of ARDS due to pulmonary infection differed between the G-CSF group which received chemotherapy with G-CSF and historical controls without G-CSF. We evaluated 132 patients with hematological malignancy in complete remission without any main organ dysfunction who had been treated between April 1983 and December 1997. We compared the incidence of ARDS due to pulmonary infection between those who received G-CSF and those who did not. There was no remarkable difference in the number of patients, gender, age, or distribution of primary diseases between the two groups. The intensity of chemotherapy was not considered to significantly differ between the two groups, though the chemotherapy regimens administered differed slightly. In the G-CSF group, the duration of neutropenia was significantly shorter and the frequency of documented infection was significantly decreased. We could not find any relationship between ARDS due to pulmonary infection and any anticancer agent or antibiotics. There was no relationship between the kind of G-CSF and the incidence of ARDS due to pulmonary infection (per chemotherapy session; p > 0.10, per case; p > 0.30, χ2 test). The incidence of ARDS due to pulmonary infection per chemotherapy session was 4.21%, and showed a higher tendency in the G-CSF group (p < 0.100, χ2 test). The incidence of ARDS due to pulmonary infection per case was 25.4% and was significantly higher in the G-CSF group (p < 0.025, χ2 test). The incidence of ARDS due to pulmonary infection was higher in the G-CSF group than in the controls, suggesting that G-CSF promotes the development of ARDS due to pulmonary infection.
Effect of Desferrioxamine in Acute Haemolytic Anaemia of Glucose-6-Phosphate Dehydrogenase Deficiency
Authors: Al-Rimawi H.S. , Al-Sheyyab M. , Batieha A. , El-Shanti H. , Abuekteish F.
Published: April 2003

The effectiveness of desferrioxamine (DFO) in ameliorating the severity of the acute haemolysis of glucose-6-phosphate dehydrogenase (G6PD) deficiency was studied in 167 children with G6PD deficiency during an acute haemolytic crisis. All patients received packed cell transfusion on admission if their Hb levels were <8 g/dl, which was repeated as needed. Eighty patients also received a single dose of DFO 30–40 mg/kg by slow intravenous infusion (DFO group). The remaining 87 children did not receive DFO (control group). The need for more than one transfusion was less frequent in the DFO group as compared to the control group (p = 0.01). The need for late transfusion (transfusion after 36 h of admission) was also less in the DFO group (7%) compared to 21% in the control group (p = 0.02). On average, children in the DFO group needed less packed red blood cells (16.5 ml/kg body weight) than the control group (22.8 ml/kg body weight) and the difference was highly significant (p = 0.0001). We conclude from this study that DFO in a small dose is effective in the treatment of acute haemolytic crises of G6PD deficiency. It shortens the duration of the crisis and decreases the amount of blood transfusion needed.
Portal Vein Thrombosis in Paroxysmal Nocturnal Haemoglobinuria
Authors: Tomizuka Hiroshi , Hatake Kiyohiko , Kitagawa Seiichi , Yamashita Keisuke , Arai Hiroyoshi , Miura Yasusada
Published: April 2003

A 28-year-old man was hospitalized with nausea, vomiting, abdominal pain and low-grade fever. He had a 6-month history of paroxysmal nocturnal haemoglobinuria (PNH), and laboratory data showed anaemia and liver dysfunction. An abdominal ultrasonography showed ascites and portal vein thrombosis. After receiving antithrombotic treatment, the portal vein thrombosis did not extend. Portal vein thrombosis is very rare but should be considered when we encounter liver dysfunction associated with PNH as well as hepatic vein thrombosis. Ultrasonography is very useful in detecting portal vein thrombosis and facilitating early diagnosis. Warfarin is very effective in preventing exacerbation of portal vein thrombosis in PNH.
Bacteremia by Gram-Negative Bacilli in Patients with Hematologic Malignancies
Authors: Martino Rodrigo , Santamaría Amparo , Muñoz Luz , Pericas Roser , Altés Albert , Prats Guillem , Sierra Jordi
Published: April 2003

To compare the characteristics of bacteremic infections by different aerobic gram-negative bacilli (GNB) in patients with hematologic malignancies, we studied 54 consecutive monomicrobial bacteremias by Enterobacteriaceae (EB), 15 by Pseudomonas aeruginosa, 43 by other non-glucose-fermenting GNB (NGFGNB) and 11 by other GNB. Patients with EB and P. aeruginosa bacteremia usually developed the infection after intensive chemotherapy for leukemia or during a hematopoietic stem cell transplantation, while most infections in outpatients off therapy were due to NGFGNB. A significant proportion of bacteremias by EB (37%) and P. aeruginosa (40%) were accompanied by severe morbidity (septic shock, pneumonia or deep-seated organ infections) vs. only 7% of other NGFGNB (p < 0.01). Most infections by these latter bacteria were catheter-related bacteremias (80 vs. 2% of EB, p < 0.005), while most EB infections (61%) were uncomplicated bacteremias of unknown source (vs. 14% of other NGFGNB, p < 0.005). Appropriate antibiotics alone cured 98% of EB and 73% of P. aeruginosa bacteremias but only 26% of other NGFGNB (p < 0.005 for both differences), which were cured by catheter removal in 70%, usually after failure of antibiotic treatment. In conclusion, our results suggest that there are significant differences in the patient populations and clinical characteristics of bacteremic infections by the classic GNB (EB and P. aeruginosa) and the new NGFGNB in adults with hematologic malignancies.
The Polysaccharide, PGG-Glucan, Enhances Human Myelopoiesis by Direct Action Independent of and Additive to Early-Acting Cytokines
Authors: Turnbull J.L. , Patchen M.L. , Scadden D.T.
Published: April 2003

β-Glucans stimulate leukocyte anti-infective activity, enhance murine hematopoietic recovery following bone marrow injury and mobilize murine progenitor cells from bone marrow. This study evaluated the in vitro hematopoietic potential of the β-glucan, PGG-glucan, on human bone marrow mononuclear cells (BMMC) and CD34+ BMMC compared with protein cytokines. In the presence of submaximal concentrations of recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF; 0.5 ng/ml), PGG-glucan significantly increased BMMC myeloid colony formation comparable to the increase observed with either interleukin-3 (rhIL-3) or stem cell factor (rhSCF). Moreover, the addition of PGG-glucan to cultures containing GM-CSF + IL-3 or GM-CSF + SCF significantly augmented granulocyte-macrophage colony production above baseline, demonstrating that PGG-glucan acts independently of those early-acting cytokines and can enhance their activity in an additive manner. Anti-PGG-glucan monoclonal antibody specifically abrogated the growth-enhancing effect of added PGG-glucan in a saturable manner and other control carbohydrate polymers failed to affect colony formation. Further, PGG-glucan was not associated with induction of IL-6, GM-CSF production and removal of accessory cells by CD34+ cell isolation did not alter the PGG-glucan effect. These data demonstrate that PGG-glucan acts on committed myeloid progenitors to enhance human hematopoietic activity by a mechanism of direct action independent of IL-3 or SCF and independent of secondary cytokine stimulation.
Evidence of Lymphocyte Alveolitis by Bronchoalveolar Lavage in Thalassemic Patients with Pulmonary Dysfunction
Authors: Filosa A. , Esposito V. , Meoli I. , Baron I. , Romano L.
Published: April 2003

Pulmonary dysfunction represents one of the least studied complications in thalassemic patients. Probably, it is due to the absence of pulmonary symptoms. There are few works in the literature, and contradictory results have been published. The aim of this study was to define the spirometric pattern and the possible causes of lung impairment by testing bronchoalveolar lavage (BAL) with pathological pulmonary function tests (PFTs). Furthermore, diffusion capacity tests for carbon monoxide corrected for hemoglobin value (Dco*) were performed. We studied 48 thalassemic patients (27 F and 21 M), with an age range from 8 to 23 years, divided into two groups on the basis of PFTs results. Thus, group A was formed by 16 patients with restrictive spirometric patterns of whom 14 had also reduced Dco* values and group B consisted of 32 patients with normal PFTs and Dco* values. Patients of group A underwent chest high-resolution computing tomography (CHRCT) and BAL whose fluid was analyzed by microbiologic and cytological assays. A pathological CHRCT picture was present in 8 patients. Nine out of 16 patients who accepted to undergo BAL had a chronological age greater than 17 years with a mean bone age of 13.9 years. BAL results showed lymphocyte alveolitis in 6 patients and a normal cytogram in 3, while alveolar iron-laden macrophages were present in 4 out of 6 patients with alveolitis and 2 out of 3 patients with normal cytogram. Moreover, all examined BAL fluids showed a normal CD4/CD8 ratio, while only 2 patients showed an altered serum CD4/CD8 ratio. We demonstrated the presence of (1) lung-restrictive syndrome in 16 of the oldest thalassemic patients; (2) lymphocyte alveolitis in 6 patients, and (3) a picture of interstitial fibrosis by CHRCT in 8 of them. All these data are suggestive of a diagnosis of interstitial lung disease secondary to thalassemia. BAL helped to identify the presence of alveolar iron-laden macrophages that represented a local defense mechanism against free iron. This latter finding therefore might be the primary cause of the lung impairment promoting an oxidative damage. Further studies are needed to investigate this hypothesis and therapeutical potentials.
Estimation of Stem Cell Fractions in Peripheral Blood Stem Cell Harvest by Using an SE-9000 Hematology Analyzer
Authors: Saigo Katsuyasu , Sugimoto Takeshi , Takeuchi Satoshi , Kosaka Yoshiyuki , Ryo Ryukichi , Kumagai Shunichi
Published: April 2003

We inquired whether stem cell fractions in peripheral blood stem cell harvest could be precisely detected by using a hematopoietic progenitor cell (HPC) counting system applied to an automated hematology analyzer, SE-9000. Although there was an apparent increase in the HPCs 20 h after storage in nondiluted conditions, samples diluted with RPMI-1640 containing 1.0 mg/ml EDTA-2K showed a relatively stable number of HPCs. There was a significant relationship between HPCs and CD34 cells (n = 75, r = 0.769). This method may represent the least expensive and most time-effective way for stem cell estimation in harvest products.
Sensitivity to Interferon-Alpha and Interferon-Stimulated Gene Factor 3 Binding Activity in Human Chronic Myelogenous Leukemia Cell Line KT-1
Authors: Yamauchi Hayato , Sakai Ikuya , Narumi Hiroshi , Soga Shinji , Fujita Shigeru
Published: April 2003

The mechanism of responsiveness of chronic myelogenous leukemia (CML) cells to interferon (IFN)-α was examined by using two subclones of CML cell line KT-1 which exhibited significantly different sensitivities to the antiproliferative and apoptosis-inducing effects of IFN-α. IFN-stimulated gene factor 3 (ISGF3) formation by IFN-α was reduced in the IFN-α-resistant subclone compared to the IFN-α-sensitive subclone. We conclude that the level of ISGF3 formation is responsible for the difference in IFN-α responses between these subclones.
The Fitness-for-Work Evaluation of a Young Patient with Essential Thrombocythemia
Authors: Merkel Drorit , Moshe Shlomo , Tal Orna , Eldad Arieh
Published: April 2003

We describe a case of a military cadet, a candidate for submarine service, who was diagnosed with essential thrombocythemia (ET). The estimated risk in the literature for major clinical complications in ET patients is about 5–20%. Our report concerns a young asymptomatic patient where the estimated risk is probably much lower. We think that the activity in the marine corps, which at times is involved with some degree of dehydration, might increase the risk for thromboembolic events. The possibility of a major thromboembolic complication occurring at sea could complicate and endanger the patient’s life, especially when an evacuation to a hospital is required. A submarine officer has a responsibility to other people’s life who depend on his skills and ability. In case of a major complication which can cause sudden incapacitation, the consequences can be catastrophic to many. Opposed to the considerations to disqualify the young cadet stands the fact that his training was expensive, and that it would be unfortunate to disqualify a very talented young person. We thus recommended to clear the cadet as a marine officer in teaching jobs, in sites were medical assistance is available in a short period of time. In our opinion he should be disqualified from serving in combat jobs where there is a greater risk for him and for the men under his command.
Circadian Rhythm of Arginine Vasopressin in Hepatorenal Syndrome
Authors: Pasqualetti Paolo , Festuccia Vittorio , Collacciani Antonio , Acitelli Pierluigi , Casale Raffaele
Published: April 2003

The etiology of hepatorenal syndrome is still incompletely understood, but the nonosmotic release of arginine vasopressin (AVP) seems to have an important role. Since AVP presents a well-defined daily periodicity in its production and secretion, the aim of the study was to investigate the circadian rhythm in its circulating plasma concentrations in patients with hepatorenal syndrome, compared with healthy controls. Venous blood samples were drawn during a 24 hour period at 2 hourly intervals from a peripheral vein in 10 healthy subjects and in 10 patients with hepatorenal syndrome for the determination of the circulating plasma levels of AVP by radioimmunoassay. Statistical analysis was carried out by the ‘cosinor’ method. The controls presented a significant (p < 0.002) circadian rhythm in the AVP circulating levels, whereas no rhythm (p > 0.05) was found in patients. The circadian rhythms were significantly (p < 0.005) different between the two groups, patients having higher mean daily concentration and lower circadian variation of AVP. These results confirm the existence of a well-defined circadian rhythm of AVP in healthy subjects, whereas the hepatorenal syndrome patients present a complete loss of the circadian rhythm. It could be hypothesized that primitive damage in circadian time-keeping regulates the circadian rhythm of vasopressinergic neurons, or has a secondary effect on the peripheral vasodilatation in the course of advanced liver disease. This great upset in the temporal and functional organization, together with that of the renin-angiotensin-aldosterone system, could play an important role in promoting and/or in the maintenance of the hydroelectrolyte imbalance that characterizes the hepatorenal syndrome.
Multiple African Honeybee Stings and Acute Renal Failure
Authors: Hommel Didier , Bollandard Frank , Hulin Alain
Published: April 2003

In 1956, the Brazilian government brought honeybees (Apis mellifera andansonii) from South Africa. A group of these bees crossed the Amazon and reached Venezuela where they have killed between 30 and 50 people in the last 4 years [1]. In 1993, we observed the first case of rhabdomyolysis, hepatic dysfunction and acute renal failure (ARF) following multiple stings by African bees (A. mellifera andansonii) in French Guiana.
A Simple Histological Method Differentiating AL-Type from AA-Type Amyloidosis
Authors: Horita Shigeru , Nitta Kosaku , Honda Kazuho , Yumura Wako , Nihei Hiroshi
Published: April 2003

Amyloidosis is a disease caused by deposition of amyloid protein in various tissues and organs, and is also an important cause of chronic renal failure, usually occurring within 3 years of the development of nephrotic syndrome. To date, 13 types of amyloid proteins have been identified in humans, regardless of systemic or localized amyloidosis. Amyloid proteins derived from serum amyloid A (AA), immunoglobulin light chains (AL), transthyretin, and β2-microglobulin are constituents in systemic amyloidosis. Despite the close histological resemblance of each amyloid deposit, the pathogenesis appears to differ according to individual amyloidosis.
Urinary Sodium, Potassium, Chloride, Calcium, Magnesium, Phosphate, Urate and Urea in Healthy Subjects Aged 5–18 Years
Authors: Donati-Genet P. , Donati R. , Colombo J.P. , Lüthy C.M. , Bianchetti M.G.
Published: April 2003

The determination of urinary electrolytes is helpful in the diagnosis of children with dyselectrolytemia or metabolic acid-base imbalance of unknown origin [1–3]. The urinary excretion of electrolytes has mostly been evaluated by investigating 24-hour urine specimens [4]. The collection of such urine samples is laborious in children without bladder control, in those unable to comply while playing or at school, or in those with acute electrolyte abnormalities. The usefulness of measuring urinary solutes in relation to creatinine in urine has been suggested [5].
Pregnancy in Women on Chronic Dialysis
Authors: Romão Jr. J.E. , Luders C. , Kahhale S. , Pascoal I.J.F. , Abensur H. , Sabbaga E. , Zugaib M. , Marcondes M.
Published: April 2003

Successful pregnancy outcome is an uncommon occurrence in women requiring chronic dialytic treatment, and the most adequate dialysis therapy in the management of these pregnant patients has not been established. During the period 1988–1995, we estudied the outcome of 17 pregnancies in dialyzed females, with an average age of 28.2±5.9 years (range: 18–38 years). Seven women had adequate urine volume (>800 ml/24 h). Five patients started dialysis after conception and the remaining 12 pregnancies were diagnosed after 6–72 months on dialysis. Fourteen women were maintained on hemodialysis (HD) and 3 on continuous ambulatory peritoneal dialysis (CAPD). The HD schedule was increased to 3 h 5–6 times weekly, and CAPD was increased to six 2-liter exchanges/day. Mean serum urea was 78.6 ± 27.4 mg/dl (range 45–110); serum creatinine was 6.5 ± 3.7 mg/dl (3.3–9.8 mg/dl); and hematocrit was 28.9 ± 3.3 vol% (22–35 vol%). Anemia was partially controlled with rHuEpo in 8 patients. Significant problems were polyhydramnios in 7 cases (5 HD/2 CAPD), oligohydramnios in 1 (HD), gestational diabetes in 2 (CAPD), premature labor with spontaneous abortion at the 19th, 22nd and 28th weeks of gestation (2 HD/1 CAPD), hypertension in 8 (7 HD/1 CAPD), and sterile eosinophilic peritonitis in 1 case (CAPD). Mean gestational age at delivery in 14 successful pregnancies (12 HD/2 CAPD) was 32.3 ± 2.6 weeks (27–36 weeks) and mean baby weight was 1,400.7 ± 579.1 g (range 720– 2,650 g). No congenital fetal abnormality was observed. Respiratory distress was observed in 6 infants, with 2 deaths (1 HD/1 CAPD) in the first week after delivery. In this study, successful pregnancies were reported in 70.6% of dialyzed women with uremia, with hemodialysis having a rate of fetal survival of 78.6% and CAPD with 33.3%.
Variable Presentation of Primary Hyperoxaluria Type 1 in 2 Patients Homozygous for a Novel Combined Deletion and Insertion Mutation inExon 8 of the AGXT Gene
Authors: von Schnakenburg Christian , Hulton Sally A. , Milford David V. , Roper Helen P. , Rumsby Gill
Published: April 2003

Two unrelated patients of Pakistani origin presented with primary hyperoxaluria type 1 (PH1) at 4 months and 3 years of age, respectively. While the younger patient failed to thrive and suffered from early renal failure, the older one showed a relatively benign history with urolithiasis as the main feature of the disease. In both patients the diagnosis was confirmed by assessment of alanine:glyoxylate aminotransferase catalytic and immunoreactivity in liver biopsy specimens. The underlying genetic defect was found to be a combined deletion and insertion in exon 8 which alters the reading frame of the protein. The nucleotide change introduces a Stu1 restriction site which facilitated typing of additional family members. Both patients and a further affected brother were homozygous for this mutation, while their parents were heterozygous for it. This mutation is the first deletion/insertion identified in PH1. Although rare in our PH1 patient cohort (2.5% of alleles), the finding of 2 homozygous apparently unrelated individuals of the same ethnic origin suggests that it may prove worthwhile to screen other Asian patients for this mutation. These PH1 cases present further evidence that factors other than genotype contribute significantly to the clinical presentation and severity of PH1.
A Rat Model of Chemical-Induced Polycystic Kidney Disease with Multistage Tumors
Authors: Ito Fumio , Toma Hiroshi , Yamaguchi Yutaka , Nakazawa Hayakazu , Onitsuka Shiro , Hashimoto Yasunobu
Published: April 2003

Diphenylthiazole (DPT) induces polycystic kidney disease in the rat which serves as a model of human acquired cystic disease of the kidney. However, DPT administration alone does not produce neoplastic changes in renal cysts. We examined the effect of N-nitrosomorpholine (NNM), a carcinogen, in rats bearing DPT-induced renal cysts. Forty Sprague-Dawley rats were divided into four groups: DPT/NNM, DPT, NNM, and nontreated groups. DPT was administered throughout the experimental period, and NNM was given from weeks 4 to 7 after the start of the experiment. The rats were sampled from weeks 39 to 48, and histopathological examinations of the excised kidneys were performed. Multiple cystic changes were observed in all the DPT-treated rats in both DPT and DPT/NNM groups which were absent in almost all other rats. Solid adenomatous lesions were observed in the NNM-treated rats: in 7 of 9 and in 3 of 10 rats in the DPT/NNM and NNM groups, respectively. Cystic adenomatous lesions were found in 4 of 9 rats in the DPT/NNM group exclusively and not in the other groups. Combined DPT and NNM administration to rats produced an animal model showing neoplastic changes in renal cysts resembling microscopically renal cancer lesions in human acquired cystic disease of the kidney (on hematoxylin and eosin staining).
Nephrotic Syndrome in Strongyloidiasis: Remission after Eradication with Anthelmintic Agents
Authors: Wong Teresa Y.H. , Szeto C.C. , Lai Fernand F.M. , Mak C.K. , Li Philip K.T.
Published: April 2003

Strongyloides stercoralis infection is known to be important because of its potential for life-threatening disseminated infection in immunosuppressed hosts. Apart from direct invasion into nearly every organ in systemic infection, evidence suggests that immunological reaction also plays a role in the pathogenesis of the disease, including both uncomplicated and disseminated infections. However, Strongyloides-related glomerulonephritis has not been well documented. We present a case of steroid- and cyclophosphamide-resistant nephrotic syndrome complicated by disseminated strongyloidiasis which responded to anthelmintic agents. The remission of nephrotic syndrome after treatment of Strongyloides infection strongly suggests the possibility of Strongyloides-associated glomerulonephritis. Nephrotic patients in endemic areas of Strongyloides infection should have the differential white cell count checked. Strongyloides infection should be ruled out in patients with eosinophilia before immunosuppressants are initiated to prevent the complication of disseminated strongyloidiasis.
Nonrelated Living-Donor Kidney Transplantation: Medical and Ethical Aspects
Authors: Colakoglu Murat , Yenicesu Müjdat , Akpolat Tekin , Vural Abdülgaffar , Utaş Cengiz , Arinsoy Turgay , Sindel Şükrü , Akoglu Emel , Yasavul Ünal , Turgan Çetin , Caglar Şali
Published: April 2003

Several patients with end-stage renal disease went to Bombay for renal transplantation from nonrelated living donors and then returned to Turkey for posttransplantation follow-up. The aims of this study are to evaluate the long-term results of renal transplantation from nonrelated living donors in Turkish patients with end-stage renal disease and to discuss the ethical and social aspects of nonrelated kidney donation. One hundred and twenty-seven patients (89 males, 38 females; mean age 38.1, range 17–63 years) were investigated retrospectively. None of the patients went to Bombay on our advice. All transplantations were performed between 1991 and 1995. The mean follow-up period after transplantation was 34.2 (range 1–68) months. Graft survival rates were 85, 83, and 57% after 3 months and 1 and 5 years, respectively. Patient survival rates were 94, 93, and 92% after 3 months and 1 and 5 years, respectively. Seven patients died within the first 3 months after the transplantation. Surgical problems, infections, acute rejection, ciclosporin nephrotoxicity, and hepatic problems were common complications. We conclude that medical and surgical complications occur frequently in paid kidney transplantation, but most of these complications can be prevented by adequate preoperative management, and precautionary measures should be taken to prevent commercialization of renal transplantation before the spread of emotionally related living kidney donation.
Accumulation of Nσ-(Carboxy-methyl)lysine and Changes inGlomerular Extracellular MatrixComponents in Otsuka Long-EvansTokushima Fatty Rat:A Model of Spontaneous NIDDM
Authors: Kushiro Masahiko , Shikata Kenichi , Sugimoto Hikaru , Ikeda Kazuyoshi , Horiuchi Seikoh , Makino Hirofumi
Published: April 2003

Increases in extracellular matrix (ECM) and changes in its components have been documented in the glomeruli of diabetic nephropathy. Advanced glycation end products formed by glycoxidation have been shown to induce the synthesis of ECM components and transforming growth factor beta (TGF-β), suggesting that advanced glycation end products may be involved in the etiology of imbalance of ECM components in diabetic glomerulosclerosis. The Otsuka Long-Evans Tokushima Fatty (OLETF) rat is an inbred strain that spontaneously develops non-insulin-dependent diabetes mellitus which progresses to diabetic glomerulosclerosis. NΕ-(carboxymethyl)lysine (CML) is known to be formed by glycoxidation. To clarify the involvement of glycoxidation in diabetic nephropathy, we examined the localization of CML, ECM components, and TGF-β1 in the glomeruli of OLETF rats. The amounts of α3(IV) collagen, type VI collagen, and fibronectin were significantly increased in the glomeruli of OLETF rats, whereas the heparan sulfate proteoglycan levels were decreased. After 6 months of age, CML levels were significantly increased in the mesangial area of the glomeruli in these animals. The overexpression of TGF-β1 preceded the increase in glomerular ECM components. The present study demonstrated that the accumulation of CML precedes the changes of glomerular ECM components in the glomeruli during the course of diabetic nephropathy, suggesting that glycoxidation may be one of the major causes of diabetic glomerulosclerosis.
Chronic Renal Failure: An Overview from a Pediatric Perspective
Authors: Saborio Pablo , Hahn Seunghoon , Hisano Satoshi , Latta Kay , Scheinman Jon I. , Chan James C.M.
Published: April 2003

We present data on the costs and impact of chronic renal failure, the primary renal diseases leading to end-stage renal disease in children, and review the adaptive responses and the pathophysiology and complications of uremia in experimental animals and in man. A treatment strategy is summarized.
Serum Ionized versus Total Magnesium in Patients with Chronic Renal Disease
Authors: Saha H. , Harmoinen A. , Nisula M. , Pasternack A.
Published: April 2003

Background: Recent technology has made it possible to assess serum ionized magnesium with user-friendly ion-selective electrodes similar to measurement of the serum calcium concentration. Methods: We measured the serum ionized (Mg2+) and total magnesium (tMg) concentration in 69 patients with chronic kidney disease (38 men, 31 women, age 22–85 years) and in 75 control subjects. Results: In control subjects the reference ranges were as follows: serum Mg2+ 0.45–0.67 mmol/l, and tMg 0.67–0.96 mmol/l. In patients with chronic renal failure serum Mg2+ was 0.57 ± 0.05 mmol/l and tMg 0.80 ± 0.11 mmol/l, and in transplant recipients receiving cyclosporine 0.53 ± 0.05 and 0.71 ± 0.10 mmol/l, respectively. The correlation coefficient between serum Mg2+ and tMg was r = 0.73 (p < 0.001) in the patient group and r = 0.75 (p<0.001) in control subjects. Serum tMg was below the reference range in 15 of 69 measurements. However, serum Mg2+ was below the reference range in only 1 of these 15 samples. Hence, 14 of 69 cases with low tMg but normal Mg2+ were false-positive with respect to hypomagnesemia. Conclusion: We conclude that tMg may overestimate the incidence of hypomagnesemia, and the measurement of Mg2+ may be of benefit when studying patients with expected hypomagnesemia.
Plasma Soluble Fas and Soluble Fas Ligand in ChronicGlomerulo nephritis
Authors: Sano Hirotake , Asano Kiyoji , Minatoguchi Shinya , Hiraoka Jitsuko , Fujisawa Koh , Nishigaki Kazuhiko , Yasuda Norio , Kumada Hirokazu , Takemura Masao , Ohashi Hiroshige , Seishima Mitsuru , Fujiwara Takako , Fujiwara Hisayoshi
Published: April 2003

It has been reported that glomerular cells with apoptosis and positive Fas immunoreactivity are seen in proliferative glomerulonephritis (PGN). Fas induces apoptosis when it binds to Fas ligand (Fas-L) or soluble Fas-L (sFas-L). However, soluble Fas (sFas) blocks apoptosis by inhibiting binding between Fas and Fas-L or sFas-L. That is, Fas, Fas-L, and sFas-L are inducers of apoptosis, but sFas is an inhibitor of apoptosis. We studied the relationship between the plasma levels of sFas and sFas-L in 32 patients with various types of adult chronic glomerulonephritis. Patients with serum creatinine levels >1.5 mg/dl (132.6 µmol/l) were excluded. The plasma levels of sFas-L were within the normal limits in all patients. The plasma levels of sFas in the patients with minimal-change (n = 8) and membranous nephropathy (n = 7) were similar to the age- and sex-matched controls. However, the plasma sFas levels were significantly elevated in patients with mesangial PGN (n = 10) and membranoproliferative glomerulonephritis (n = 7)(3.4 ± 0.9 and 3.9 ± 1.5 ng/ml, respectively) as compared with the age- and sex-matched controls (controls: 2.1 ± 0.4 and 2.2 ± 0.6 ng/ml, respectively). In PGN, according to increase of histological grade and decrease of creatinine clearance, the number of TUNEL-positive cells in glomeruli is decreased in spite of an increase of the Fas positivity, and plasma sFas is increased. The degree of proliferative change is determined by the balance between proliferation and apoptosis and/or necrosis. Therefore, increased plasma sFas in PGN may inhibit apoptosis in glomeruli and may be one of the progressing factors in PGN. Thus, we conclude that an increase in plasma sFas levels is important to the protection of apoptosis in PGN.
Light Chain Deposition Disease Detected by Antisera to a Variable Region of the κ1 Light Chain Subgroup
Authors: Takeda Shin-ichi , Takazakura Eisuke , Haratake Joji , Hoshii Yoshinobu
Published: April 2003

A 59-year-old male started maintenance hemodialysis, and 80 months later died of congestive heart failure. Just before death he had no urine, and no monoclonal immunoglobulin could be demonstrated in the serum on immunoelectrophoresis. An autopsy showed deposition of periodic acid-Schiff-positive and Congo red-negative material in the interstitium and vessel walls of almost all organs and tissues. In the glomeruli there was a multifocal nodular accumulation of the material. Electron microscopy showed finely granular, not fibrillar deposits. In spite of these impressive lesions on light microscopy, the material was not stained with commercially available antisera to light chains. However, the material was positive for antisera to the variable region of the ĸ1 light chain subgroup. This is the first case with light chain deposition disease that showed positive staining only with antisera to the variable region of the light chain.
Clinical and Morphometrical Interrelationships in Patients with Overt Nephropathy Induced by Non-Insulin-Dependent Diabetes mellitus
Authors: Matsumae Tomoji , Jimi Shiro , Uesugi Noriko , Takebayashi Shigeo , Naito Setsuya
Published: April 2003

To clarify the relationships between clinical and renal structural findings in non-insulin-dependent diabetes mellitus (NIDDM), we studied 19 renal biopsy specimens from patients with overt diabetic nephropathy induced by NIDDM. By a conventional biopsy examination using light, immunofluorescent and electron microscopy, we excluded patients with any non-diabetic renal diseases which may have caused urinary abnormalities. Using standard stereological methods, the glomerular filtration surface area per nephron (S-Filt/neph), mesangial volume per glomerulus (V-Mes/glom), mesangial matrix volume per glomerulus (V-MM/glom) and the width of the glomerular basement membrane (GBM) were measured in nonoccluded glomeruli. The hyaline change in the arteriole was analyzed semiquantitatively using light microscopy as an index of arteriolar hyalinosis. Light-microscopic findings demonstrated minimal lesions in 4 cases, focal and/or segmental sclerotic lesions in 4 cases, and diffuse mesangial expansion in 11 cases (with and without nodular lesions in 9 and 2 cases, respectively). Mean glomerular volume of an open glomerulus in diabetic patients was 1.6 times the reference value obtained from nondiabetic patients with mild proteinuria and/or hematuria and normal renal function. Clinical parameters, including the duration of diabetes, urinary protein excretion and creatinine clearance were all related to S-Filt/neph, V-Mes/glom, V-MM/glom and the width of the GBM. In addition, V-MM/glom, the width of the GBM and the index of arteriolar hyalinosis were closely interrelated. Based on these findings, diabetic renal changes, including an increase in the mesangial matrix, thickening of the GBM and arteriolar hyalinosis, appeared to progress in parallel, and may reflect various clinical manifestations in patients with overt diabetic nephropathy induced by NIDDM without nondiabetic renal lesions.
Familial Lecithin:Cholesterol Acyltransferase Deficiency with Renal Failure in Two Siblings
Authors: Muthusethupathi M.A. , Padmanabhan R. , Date A. , Jayakumar M. , Rajendran Suguna , Vijayakumar R.
Published: April 2003

We encountered 2 siblings with corneal opacities, anemia, decreased concentration of plasma esterified cholesterol, chronic renal failure and markedly reduced levels of plasma lecithin:cholesterol acyltransferase. Renal biopsy in 1 patient showed clear lacunae containing characteristic dense bodies in the glomerular and tubular basement membranes and the interstitium. One of the 2 siblings received a kidney transplant and is doing well after 9 years. This is the first report of this rare metabolic disease from India.