Meningomyelitis due to nematode infection in four cats.

Spinal cord parasitic migrations in cats are uncommon. This report describes four cases of chronic hindlimb paraparesis in cats associated with nematode infection. Complete neurologic, hematologic, serum chemistry and radiographic examination was performed on all animals. Computed tomographic (CT)-myelographic examination at the lumbar area in one cat showed a slight swelling of the spinal cord. Necropsy examination of the spinal cord revealed generalized edema and marked submeningeal hemorrhage at the thoracic region in three cats. On histopathologic examination, numerous sections of adult nematodes and eggs were present in histological sections of the affected spinal cord segments in all cats. The morphologic features of the nematode, location and appearance of the lesions suggest that the parasite responsible for the paralysis in these cats is Gurltia paralysans.

Histología y ultraestructura de los osteodermos fósiles de glyptodon clavipes y holmesina sp. (xenarthra: cingulata) / Histology and ultrastructure of the fossil osteoderms of glyptodon clavipes and holmesina sp. (xenarthra: cingulata) / Histologia e ultraestrutura dos osteodermos fósseis de glyptodon clavipes e holmesina sp. (xenarthra: cingulata)

Este trabajo comprende un análisis estructural de los osteodermos fósiles de Glyptodon clavipes (Glyptodontidae) y Holmesina sp (Pampatheriidae) recolectados en el sitio de Muaco (Municipio Colina, Estado Falcón, Venezuela). Para el estudio microanatómico, las muestras fueron observadas mediante luz polarizada y microscopía electrónica de barrido. Los osteodermos de los escudos escapular y pélvico de Holmesina sp. son hexagonales, compuestos por una sola figura con una superficie perforada por pequeñas depresiones circulares de profundidad variable. En G. clavipes, la mayoría de los osteodermos son hexagonales y están constituidos por una figura central con una pequeña depresión y delimitada por un surco central, rodeada por seis u ocho figuras periféricas, divididas entre sí por surcos radiales. A nivel microscópico, la superficie de los osteodermos de G. clavipes está texturizada por un número mayor de porosidades que en Holmesina sp. Un corte histológico del osteodermo de Holmesina sp. muestra una capa superficial, integrada por fibras mineralizadas, y una zona más profunda de hueso compacto, perforada por canales vasculares. En contraste, las escutas de G. clavipes revelan una región trabecular más amplia, con numerosas osteonas y zonas de resorción irregulares.

This work is a structural analysis of the fossil osteoderms of Glyptodon clavipes (Glyptodontidae) and Holmesina sp. (Pampatheriidae) colected at the Muaco site (Colina Municipality, Falcon State, Venezuela). For the microanatomical study, the samples were observed under cross polarized light and scanning electronic microscopy. In Holmesina sp., the osteoderms of the anterior and posterior bucklers are hexagonal, composed of a single figure with the surface perforated by circular depressions of variable depths. In G. clavipes, most of the osteoderms are hexagonal and formed by a central figure with a small depression and defined by a central sulcus and surrounded by six to eight peripheral figures, separated from each other by radial sulci. At the microscopical level, the surface of the osteoderms of G. clavipes is texturized by a greater number of pits than in Holmesina sp. A histological section of a Holmesina osteoderm shows a superficial layer, integrated by mineralized fibers, and a deep area of compact bone perforated by vascular channels. In contrast, osteoderms of G. clavipes have a more extensive trabecular region with osteons and irregular resortion areas.

Este trabalho compreende uma análise estrutural dos osteodermos fósseis de Glyptodon clavipes (Glyptodontidae) e Holmesina sp (Pampatheriidae) recolhidos na região de Muaco (Município Colina, Estado Falcón, Venezuela). Para o estudo micro anatômico, as amostras foram observadas mediante luz polarizada e microscopia eletrônica de varredura. Os osteodermos, dos escudos escapular e pélvico de Holmesina sp., são hexagonais, compostos por uma só figura com una superfície perfurada por pequenas depressões circulares de profundidade variável. Em G. clavipes, a maioria dos osteodermos é hexagonal e está constituída por uma figura central com uma pequena depressão e delimitada por um sulco central, rodeada por seis ou oito figuras periféricas, divididas entre si por sulcos radiais. A nível microscópico, a superfície dos osteodermos de G. clavipes está texturizada por um número maior de porosidades que em Holmesina sp. Um corte histológico do osteodermo de Holmesina sp. mostra uma capa superficial, integrada por fibras mineralizadas, e uma zona mais profunda de osso compacto, perfurada por canais vasculares. Em contraste, as escutas de G. clavipes revelam uma região trabecular mais ampla, com numerosas osteonas e zonas de ressorção irregulares.

Successful treatment of disseminated Aspergillosis in a leukemic child

The incidence of severe fungal infections in the immunocompromised patient with malignancies has increased in recent years. This appears to be associated to the profound periods of immunosuppression and the extended use of broad spectrum antibiotics. Aspergillosis is the second most common fungal infection reported in the immunocompromised cancer patients. In patients with advanced immunosupression, the mortality due to invasive aspergillosis approaches 100despite treatment with antifungal agents. Reports of complete or partial response to echinocandins are well demonstrated in adults, but very limited in the pediatric population. This report describes the case of a child with relapsed acute lymphoblastic leukemia (ALL) who developed cutaneous aspergillosis and subsequent multiorgan dissemination during therapeutic induction and was treated successfuly with caspofungin acetate.

Severe neutropenia in an infant with methylmalonic acidemia

Neutropenia exists when the neutrophil counts is less than 1000/mm3 in infants between 2 weeks and 1 year of age and less than 1500/mm3 beyond 1 year of age (1). Severe infections occur when the absolute neutrophil count is below 500/mm3 with perirectal abscesses, pneumonia, and sepsis being common. Granulocyte Colony-Stimulating Factor (G-CSF) produces a sustained neutrophil recovery in patients with severe neutropenia, reduces the incidence and severity of infection, and improves the quality of life. Various cytopenias, including neutropenia, thrombocytopenia and pancytopenia, have been reported in association with inborn errors of branched aminoacid metabolism such as methylmalonic, propionic and isovaleric acidemia. We report an infant with methylmalonic acidemia who presented severe neutropenia

Treatment of childhood acute immune thrombocytopenic purpura with high-dose methylprednisolone, intravenous immunoglobulin, or the combination of both.

OBJECTIVE: To compare the effectiveness of intravenous immunoglobulin (IVIG) alone, high dose methylprednisolone (HDMP) alone and the combination of IVIG and HDMP in the treatment of childhood immune thrombocytopenic purpura (ITP). BACKGROUND: Acute ITP in children is a self-limited disease with a benign course and low mortality rate. Patients with platelet count less than 20,000 x 10(9)/L are at increased risk of bleeding complications, making them candidates for treatment. METHOD: A 4 year retrospective study of 148 patients hospitalized with acute ITP was conducted to compare the effectiveness of HDMP vs IVIG vs the combination of IVIG/HDMP. Statistical methods used were descriptive statistics and variance analysis utilizing F distribution. RESULTS: The IVIG and the HDMP combination demonstrated to be superior to HDMP alone in raising the platelet count within the first 24 hours. The HDMP and IVIG combination was statistically a superior modality of treatment for patients with platelet count greater than 10,000 x 10(9)/L than was IVIG or HDMP alone. Intravenous immunoglobulin had the least effectiveness in patients with platelet count less then 10,000 x 10(9)/L within the first 24 hours. CONCLUSIONS: IVIG followed by the combination of HDMP and IVIG is the most effective therapeutic modality in rapidly increasing the platelet count to safe levels in children with acute ITP when compared to HDMP alone within the first 24 hours. For borderline low platelet count (> 10,000 x 10(9)/L) HDMP and IVIG was superior to IVIG alone.

Characterization of hemoglobin Hotel Dieu in a Puerto Rican adolescent.

PURPOSE: Hemoglobin Hotel Dieu (HbHD) is a high-oxygen affinity variant of HbA never before reported in a Hispanic patient. This Hb variant was first reported in 1981 by Blouquit et al. in a white person with erythrocytosis with a substitution in the beta 99 aspartic acid residue by glycine. METHODS: A 13-year-old Puerto Rican boy had pain in his chest, headaches, easy fatigability, and high Hb (as high as 19.1 g/dl). Protein analysis was performed by cellulose acetate, citrate agar, and isoelectric focusing electrophoresis and high-pressure liquid chromatography (HPLC), polymerase chain reaction (PCR) amplification, and DNA sequencing of the second exon of the beta gene in samples obtained from the mother, father, and the patient, and DNA fingerprinting to determine paternity. RESULTS: The variant found in the patient migrated on cellulose acetate electrophoresis to a cathodic position relative to HbF, and a band cathodal to HbA and close to HbF on isoelectric focusing electrophoresis. The patient showed an abnormal well-resolved peak on HPLC with a retention time slightly shorter than that for HbS. DNA analysis by direct sequencing of the PCR product demonstrated heterozygosity for codon 99 (GAT-->GGT) in the patient but not in either parent. DNA fingerprinting by multiplex PCR amplification of three simple tandem repeat loci showed that the patient shared alleles in all three loci with both parents, ruling out nonpaternity. CONCLUSIONS: The protein and DNA analysis indicate that the erythrocytosis is caused by the presence of HbHD in this Hispanic adolescent.

Successful allogeneic bone marrow transplantation for agnogenic myeloid metaplasia in a 3-year-old boy.

PURPOSE: Agnogenic myeloid metaplasia (AMM) is a myeloproliferative disorder characterized by marrow fibrosis, extramedullary hematopoiesis, splenomegaly, and leukoerythroblastosis with abnormalities of red blood cell morphology. This is rarely encountered in children. No conventional curative therapy is known; however, allogeneic bone marrow transplantation (BMT) may eradicate the underlying stem cell abnormality with subsequent normal hematopoiesis. RESULTS: We report a 3-year-old Arab boy who had AMM and who had normal marrow hematopoiesis and markedly reduced fibrosis after high-dose chemotherapy and matched sibling BMT. CONCLUSIONS: Allogeneic BMT offers a potential cure for patients with agnogenic myeloid metaplasia. A preparatory regimen containing busulfan and cyclophosphamide appears promising for patients with the disease.

Intact splenic function in cystic fibrosis.

Patients with cystic fibrosis (CF) suffer from severe chronic pulmonary infections but rarely develop bacteremia/septicemia suggestive of an intact splenic mononuclear phagocyte function. The splenic function of 25 patients diagnosed with CF, aged 2 to 37 years, was evaluated using erythrocyte pit count by direct interference contrast microscopy. Results were compared with patients with sickle cell disease and normal individuals. All CF patients displayed normal splenic function by pit count. The mean percentage of pitted erythrocytes was 0.20 +/- 0.28 (range: 0.0% to 1.0%) versus 0.19 +/- 0.33 (range: 0.0% to 1.4%) in normal eusplenic controls. There were no episodes of bacteremia or septicemia despite recurrent acute exacerbations of chronic bacterial bronchitis and the use of central lines. We conclude that splenic function in CF is unabridged and may account for the rarity of bacteremia/septicemia in patients with CF despite the high prevalence of chronic bronchial infection in this population.

Reversible splenic hypofunction in hypertransfused children with homozygous sickle cell disease.

Functional hyposplenism, as documented by technetium 99 metastable sulfur colloid spleen scan and increased pocked erythrocyte count (also known as a pit count), is well described in children under 2 years of age with homozygous sickle cell anemia. We evaluated the clinical course and splenic function of 16 patients with sickle cell anemia (ages 3 to 20 years) on a hypertransfusion program for more than 6 months following a cerebrovascular accident. Patients were followed with simultaneous spleen scan and pitted erythrocyte count using direct interference contrast microscopy. Pit counts were taken prior to each transfusion and hemoglobin S level maintained at less than 20%. With the exception of two patients, splenic function was recovered only in those patients who were younger than 10 years of age at the time transfusion was initiated. There were no serious bacterial infections or other complications of sickle cell anemia documented in the hypertransfused group. Based on our results and the literature review, we conclude that some patients with sickle cell anemia receiving intensive hypertransfusion therapy for a cerebrovascular accident recover a normal splenic phagocytic function. Age and level at which the hemoglobin S is maintained are important factors in reestablishing splenic phagocytic function.

Childhood nasopharyngeal carcinoma.

A retrospective analysis identified 40 children and adolescents with nasopharyngeal carcinoma who were evaluated and treated at the Medical Center of Louisiana from 1948 through 1992. The male to female ratio was 1.8:1. Thirty-two patients had lymphoepithelioma and 8 had squamous cell carcinoma. Twenty-three children had primary lesions confined to the nasopharynx. In 8 patients, the tumor extended into the nasal cavity or oropharynx, and 7 patients had tumor invasion of skull or cranial nerve. Thirty-one had palpable cervical lymphadenopathy. There were no documented distant metastasis at presentation. All patients were treated with primary radiotherapy, and 9 patients also had chemotherapy containing cisplatin, fluorouracil, bleomycin, cyclophosphamide, and doxorubicin. Ten are alive 5 to 30 years from time of diagnosis (median 10 years). Distant metastasis was the initial cause of failure. Though not a controlled study, bias in the data insinuate an improved prognosis for male patients, age > 10 years, tumor limited to the nasopharynx without extension (T1-2), absence or single positive homolateral node (N0-1), and patients who received chemotherapy.

Treatment of acute idiopathic thrombocytopenic purpura with high-dose methylprednisolone and immunoglobulin.

In childhood idiopathic thrombocytopenic purpura (ITP), both intravenous high-dose steroids and immunoglobulin treatments have been demonstrated to raise platelet counts reliably and in most cases within 72 h, when used as separate therapeutic modalities. However, until now, the preferred emergency management of life-threatening complications in children with ITP has been immediate splenectomy. Since steroids and immunoglobulin create a partial splenic dysfunction, through different mechanisms, we investigated whether combined treatment with both drugs could produce a rapid platelet count increase comparable to that of splenectomy. Eleven patients, ages 4 months to 6 years, with a diagnosis of acute ITP were entered into this pilot study. Treatment consisted of intravenous high-dose methylprednisolone (20 mg/kg in 30 min) followed by intravenous gamma globulin (Gamimune-N, 1 g/kg over 5 h). The combined therapy resulted in rapid increments in the platelet counts of all patients within the 24-hour period. At 12-h, in particular, 9/11 patients had platelet counts of 30 x 10(9)l or more. We conclude that this combined therapy provides a prompt rise in platelet counts to a safe and hemostatic level and may offer a viable alternative for emergency splenectomy and its associated morbidity/mortality in many cases of childhood ITP.

[Podalic version in multiparae. Vaginal or cesarean?]. / Parto en podálica en multíparas. Vaginal o cesárea?

A review of maternal and perinatal results of multiparous patients with breech presentation was done during two period; two years before and two years after establishing the rule of cesarean section for all multi-parous with breech presentation. Seventy cases were considered at the first period in which 47.1% were vaginal delivery, proportion that significantly decreased (15%) at the second period. No significant differences on maternal results by delivery form were found and the same was found in perinatal results at comparing vaginal delivery and cesarean section. A different situation was reported studying only elective cesarean section, in which a significant decrease of severe neonatal depression was found comparing to vaginal delivery (24.2% and 0%). Because of rapid recovery there were no differences at fifth minute. As a conclusion, it appears as an exaggeration the option of cesarean section for all multiparous with breech presentation. Secondly, the adequate selection of cases to resolve by vaginal delivery may not produce adverse maternal and perinatal results.

Spleen function in children with sickle B+ thalassemia.

Splenic function in patients with sickle B+ (SB+) thalassemia has been poorly documented. We evaluated the clinical course and splenic function in 12 children with SB+ thalassemia with simultaneous technetium sulfur colloid spleen scans and determination of pitted erythrocytes by direct interference contrast microscopy (DICM). All patients displayed normal uptake of radiocolloid. Mean percentage of pitted erythrocytes was 2.2% compared to 0.06% in 10 normal eusplenic controls and 13.8% in 10 sickle cell patients. In this group of children, who were carefully monitored for 136 patient years, there was no episode of bacteremia/sepsis, and a low prevalence of vaso-occlusive episodes. The slight increase in percentage of pitted erythrocytes of SB+ thalassemia patients does not seem to herald any clinically relevant loss of splenic function. SB+ thalassemia children younger than 10 years of age who do not seem to present a higher risk of invasive bacterial infections than eusplenic children, should receive conservative treatment for isolated febrile episodes and should not be submitted to prophylactic penicillin.

Transient expression of trisomy 21 and monosomy 7 following cyclosporin A in a patient with aplastic anemia.

We present a patient with severe idiopathic aplastic anemia with no previous chromosomal abnormalities who developed trisomy 21 and monosomy 7 during treatment with intravenous (i.v.) cyclosporine. The abnormal karyotype disappeared when the drug was changed to the oral form. This cytogenetic aberration, previously unreported in association with cyclosporine, may reflect either a direct drug effect or the emergence of a hidden myelodysplastic cell clone subject to preferential survival during immunosuppression.

Successful treatment of disseminated Fusarium infection in an immunocompromised child.

We report the first know case of disseminated fungal infection due to Fusarium proliferatum in a bone marrow transplant recipient to our knowledge. Fusarium was cultured from the blood, a paranasal sinus, and necrotic skin lesions. The isolate was sensitive to amphotericin B and on further sensitivity testing, synergy was demonstrated using rifampin in combination with amphotericin B. The patient had this infection while she was receiving alternate-day amphotericin, rifampin, and 5-flucytosine (5-FC) therapy. The infection was documented within 48 h of discontinuing daily granulocyte transfusions, which she had received for 3 weeks. The 5-FC was discontinued when sensitivities showed the organism resistant. After 6 weeks of treatment she showed complete remission of the infection, although neutrophil counts remained below 0.25 X 10(9)/L. From this case and from a review of the literature, it appears that synergic antifungal agents combined with leukocyte transfusions may be beneficial in the successful treatment of fusariosis in the compromised host.