Effect of wheat-grain moxibustion on Wnt/β-catenin signaling pathway in bone marrow cell in mice with bone marrow inhibition / 中国针灸

OBJECTIVE@#To observe the effect of wheat-grain moxibustion at "Dazhui" (GV 14), "Zusanli" (ST 36) and "Sanyinjiao" (SP 6) on Wnt/β-catenin signaling pathway in bone marrow cell in mice with bone marrow inhibition, and to explore the possible mechanism of wheat-grain moxibustion in treating bone marrow inhibition.@*METHODS@#Forty-five SPF male CD1(ICR) mice were randomly divided into a blank group, a model group and a wheat-grain moxibustion group, 15 mice in each group. The bone marrow inhibition model was established by intraperitoneal injection of 80 mg/kg of cyclophosphamide (CTX). The mice in the wheat-grain moxibustion group were treated with wheat-grain moxibustion at "Dazhui" (GV 14), "Zusanli" (ST 36) and "Sanyinjiao" (SP 6), 3 moxa cones per acupoint, 30 s per moxa cone, once a day, for 7 consecutive days. The white blood cell count (WBC) was measured before modeling, before intervention and 3, 5 d and 7 d into intervention. After intervention, the general situation of mice was observed; the number of nucleated cells in bone marrow was detected; the serum levels of interleukin-3 (IL-3), interleukin-6 (IL-6) and granulocyte macrophage colony stimulating factor (GM-CSF) were measured by ELISA; the protein and mRNA expression of β-catenin, cyclinD1 and C-Myc in bone marrow cells was measured by Western blot and real-time PCR method.@*RESULTS@#Compared with the blank group, the mice in the model group showed sluggish reaction, unstable gait, decreased body weight, and the WBC, number of nucleated cells in bone marrow as well as serum levels of IL-3, IL-6, GM-CSF were decreased (P<0.01), and the protein and mRNA expression of β-catenin, cyclinD1 and C-Myc was decreased (P<0.01). Compared with the model group, the mice in the wheat-grain moxibustion group showed better general condition, and WBC, the number of nucleated cells in bone marrow as well as serum levels of IL-3, IL-6, GM-CSF were increased (P<0.01, P<0.05), and the protein and mRNA expression of β-catenin, cyclinD1 and C-Myc was increased (P<0.05).@*CONCLUSION@#Wheat-grain moxibustion shows therapeutic effect on bone marrow inhibition, and its mechanism may be related to activating Wnt/β-catenin signaling pathway in bone marrow cells, improving bone medullary hematopoiesis microenvironment and promoting bone marrow cell proliferation.

Microambiente medular en la leucemia mieloide crónica: su relación con la enfermedad y la respuesta al tratamiento / Bone marrow microenvironment in chronic myeloid leukemia: Implications for disease physiopathology and response to treatment

Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm related to the presence of the BCR-ABL1 fusion gene, linked to t (9;22) (q34;q11). It is originated from an abnormal hematopoietic stem cell, which is characterized as its normal counterparts by long-term self-renewal and multi-lineage differentiation. Both leukemic and quiescent normal hematopoietic stem cells preferentially reside in the osteoblastic niche. Mesenchymal stromal cells (MSC) are located near them, playing a critical role in their regulation. Currently, with tyrosine kinase inhibitor (TKI) therapy, long term clinical responses are achieved in most CML cases. However, late treatment failures may be observed related to the persistence of leukemic stem cells. The interactions between the leukemic stem cell and the microenvironment may be responsible in part for these events. We review the interactions between the leukemic stem cell and BM stroma and its potential clinical and therapeutic implications.

Intoxicação aguda por Pteridium arachnoideum e Pteridium caudatum em bovinos e distribuição das plantas em Mato Grosso / Acute poisoning by Pteridium arachnoideum and Pteridium caudatum in cattle and distribution of the plants in Mato Grosso

São descritos os aspectos epidemiológicos, clínicos e patológicos de um surto de intoxicação por Pteridium arachnoideum e Pteridium caudatum em bovinos no Estado de Mato Grosso. A distribuição dessas plantas no Estado, a intensidade de invasão de pastagens e alguns fatores associados à invasão das pastagens por Pteridium spp. são também descritos. Pteridium spp. foi observada em 83 propriedades de nove municípios de Mato Grosso e deste total, amostras de 22 propriedades foram coletadas para identificação taxonômica. Em 22 propriedades identifica-se P. arachnoideum e em duas dessas havia também P. caudatum. O desmatamento e a realização de queimadas parecem estar relacionados com a invasão de Pteridium spp. nas pastagens. Na propriedade em que ocorreu a doença, 306 bovinos foram introduzidos em uma pastagem formada por Brachiaria brizantha intensamente invadida por P. arachnoideum e P. caudatum e desses 22 bovinos adoeceram e morreram. Os principais sinais clínicos foram febre alta, apatia, fadiga, fraqueza e decúbito. Na necropsia havia graus variáveis de hemorragias em vários órgãos e cavidades e infartos ocasionais. Microscopicamente, a alteração mais importante consistiu em aplasia severa da medula óssea vermelha. Os achados epidemiológicos, clínicos e patológicos associados à identoificação taxonômica da planta fundamentam o diagnóstico de intoxicação aguda por P. arachnoideum e P. caudatum em bovinos. O crescente desmatamento e a frequente utilização de queimadas em Mato Grosso podem contribuir para que esta doença venha a ser uma importante fonte de prejuízos para a pecuária do Estado.

Epidemiological features, clinical signs and pathological findings of a spontaneous acute poisoning by Pteridium arachnoideum and Pteridium caudatum in cattle in the State of Mato Grosso, Brazil, are described. The plant distribution, magnitude and some aspects about the pasture invasion by P. arachnoideum and P. caudatum in the State are also described. Pteridium spp. were found in 83 farms from nine counties and 22 of them were submitted to taxonomic identification. P. arachnoideum was found in 22 farms and in two farms also P. caudatum. Deforestation and burning appear to be associated with pasture invasion by Pteridium spp. On the farm where the poisoning occurred 306 cattle were introduced into a pasture of Brachiaria brizantha intensely invaded by P. arachnoideum and P. caudatum. Twenty two cattle were poisoned by the plants and died. The main clinical signs were hemorrhages, high fever, apathy, fatigue, weakness and recumbency. Gross findings included variable degrees of hemorrhages in several organs and cavities with occasional infarcts. The most consistent histological finding was marked bone marrow aplasia. Epidemiological, clinical and pathological findings associated with the taxonomic identofication of the plant support the diagnosis of acute poisoning by P. arachnoideum and P. caudatum in cattle. The increasing rate of deforestation and frequent burning in Mato Grosso can contribute to this disease which becomes a major cause of economic losses to livestock production in the State.

Evaluation of (anti)genotoxic activities of Phyllanthus niruri L. in rat bone marrow using the micronucleus test

Phyllanthus niruri L. (Euphorbiaceae), known as "quebra-pedra" (Portuguese for "stonebreaker"), is an herb used for kidney disorders. In light of its frequent use by the population, the present study aimed to investigate the genotoxic, antigenotoxic and cytotoxic activities of a standardized P. niruri extract in bone marrow rats. Three groups of 12 animals were treated daily by gavage over a period of 30 days, with 50, 150 or 250 mg/kg of P. niruri extract aqueous solution. The control group (n = 12) received tap water. At the end of treatment (day 31), groups were divided into two minor subgroups (n=6/group) and received cyclophosphamide (50 mg/kg, i.p.) or saline 0.9% (i.p.). After 24 hours, we evaluated the frequency of micronucleated polychromatic erythrocytes for each animal (MNPCE) at 1000 PCE. Cytotoxicity was evaluated with the PCE/NCE ratio (NEC = normochromatic erythrocytes). General toxicity was assessed during treatment using the parameters of body weight gain, ration and water consumption. The dry extract did not provoke changes in body weight, weight gain, ration and water intake or changes in the frequency of MNPCE or cytotoxicity in bone marrow. We propose that the P. niruri extract used here showed no genotoxic, antigenotoxic and cytotoxic activities under the experimental conditions.

Phyllanthus niruri L. (Euphorbiaceae), conhecida como "quebra-pedra", é uma planta medicinal utilizada frequentemente pela população no tratamento de problemas renais. Foram avaliadas as atividades genotóxicas, antigenotóxicas e citotóxicas de um extrato padronizado dessa espécie em ratos. Três grupos de doze animais foram tratados durante trinta dias, por gavagem, com 50, 150 ou 250 mg/kg/dia de solução aquosa do extrato de P. niruri e um grupo controle (n=12) recebeu água destilada pela mesma via. No final do tratamento os grupos foram divididos em dois subgrupos (6 animais/grupo) e receberam uma dose única de ciclofosfamida (50 mg/kg, i.p.) ou de solução salina 0,9% (i.p.). Após 24 horas, a frequência de eritrócitos policromáticos micronucleados (EPCMN) foi avaliada em 1000 EPC. A citotoxicidade foi avaliada pela relação entre eritrócitos policromáticos e normocromáticos (EPC/ENC) e a toxicidade geral foi avaliada através dos parâmetros de ganho de peso corporal, consumo de ração e ingestão hídrica. O extrato seco não provocou alterações significativas no peso corporal, ganho de peso e consumo de ração em relação ao grupo controle, nem alterações na frequência de EPCMN ou citotoxicidade em medula óssea. Dessa maneira, pode-se concluir que P. niruri não apresentou atividades genotóxica, antigenotóxica e/ou citotóxica nas condições experimentais executadas.

Resposta osteogênica sistêmica no estímulo medular ósseo / Osteogenic systemic response at bone marrow stimulation

OBJETIVO: Avaliar a influência da resposta osteogênica sistêmica, causada pelo estímulo da medula óssea à distância, na consolidação de falha óssea. MÉTODO: 36 coelhos adultos jovens foram divididos aleatoriamente em três grupos (A, B, C) e submetidos à ostectomia do rádio direito, com retirada de 4mm de fragmento ósseo. Os animais do grupo A foram submetidos ao estímulo da medula óssea pela sua ablação do fêmur esquerdo. Os animais do grupo B foram submetidos ao estímulo da medula óssea pela introdução de fio de Kirschner com 1,5mm de espessura, no interior do canal medular femoral esquerdo. Os animais do grupo C foram utilizados como grupo controle. Foram realizadas radiografias semanais até a 4ª semana pós-operatória, quando os animais foram sacrificados. Foi realizado estudo histomorfométrico do calo ósseo formado no local da ostectomia. As radiografias foram avaliadas para análise da evolução da consolidação óssea. RESULTADOS: Os grupos que sofreram estímulo medular à distância tiveram menor número de células ósseas, comparativamente ao grupo controle. No estudo radiográfico não houve diferença na evolução da consolidação entre os grupos. CONCLUSÃO: O estímulo da medula óssea à distância influenciou desfavoravelmente a consolidação de falha óssea em coelhos.

OBJECTIVE: To assess the influence of systemic osteogenic response caused by remote stimulation of bone marrow in a bone gap union. METHOD: 36 young adult rabbits were employed. The animals were randomly divided into 3 groups (A, B, C) and submitted to ostectomy of the right radius, removing 4mm of bone. The animals on Group A had their bone marrow stimulated by ablation on the left femur. Animals on Group B had their bone marrow stimulated by introducing a 1.5mm-thick Kirschner wire into the shaft of the left femur. The animals on Group C served as controls. X-ray images were taken on a weekly basis until the 4th post-surgical week, when the animals were sacrificed. Histomorphometric study of the bony callus formed at the ostectomy site was conducted. The x-ray images were evaluated in order to analyze the evolution of bone union at the ostectomy site. RESULTS: The groups with remote bone marrow stimulation had a smaller number of bone cells as compared to the control group. On radiographic studies, no difference in terms of evolution of union was evident between the groups. CONCLUSION: Remote stimulation of bone marrow had an unfavorable influence on bone gap union in rabbits.

Efeitos da lidocaína hiperbárica em concentrações crescentes, administrada no espaço subaracnóideo, sobre a medula e meninges: estudo experimental no cão / Effect of the hyperbaric lidocaína in increasing concentrations, managed in the subaracnóideo space, on the marrow and meninges: experimental study in the dog

Em estudo experimental em ratos, a injeçao de lidocaina subaracnoidea a partir da concentraçao de 7,5 porcento, resultou em mudanças neurofuncionais e histopatologicas. O proposito deste estudo foi investigar, em animais vivos as possiveis alteraçoes clinicas e histologicas desencadeadas por injeçoes de lidocaina hiperbarica nas concentraçoes de 5 porcento, 7,5 porcento e 10 porcento, administradasno espaço subaracnoideo de caes. Quarenta animais foram randomizados em quatro grupos que receberam por via subaracnoidea: G1 glicose a 7,5 porcento e 10 porcento, administradas no espaço subaracnoideo de caes. Quarenta animais foram randomizados em quatro grupos que receberam por via subaracnoidea: G1 glioce a 7,5 porcento, G2 lidocaina hiperbarica a 5 porcento, G3 lidocaina hiperbarica a 7,5porcento e G4 lidocaina hiperbarica a 10 porcento. A punçao subaracnoidea foi realizada no espaço intervetebral L6-L7. O volume da solução injetada foi de 1 ml. Os animais foram sacrificados apos sete dias de observaçao em cativeiro.As porçoes lombar e sacral da medula foram removidas para exame histologico, por microscopia optica. Os caes pertencentes aos grupos G1 e G2, ao apresentaram alteraçoes clinicas e histologicas na medula e meninges. Foi observado tres casos em G3, de necrose em focosno tecido medular mas, necrose abrangendo toda a superficie da medula em somente um dos animais e, a despeito dos achados histologicos, um destes caes permaneceu clinicamente normal. No G4, sete caes apresentaram alteraçoes clinicas e histologicas. As mudanças histologicas encontradas variaram de areas de necrose restritas a algumas regioes da medula a necrose em faixa abrangendo toda a superficie medular. Observamos diminuiçao de força muscular nas patas posteriores em seis animais, sendo que em tres deles houve associação em relaxamento de esfincter anal, e em um dos animais, a diminiçao do tonus esfincteriano foi a unica alteraçao clinica obervada. A neurotoxicidade da lidocaina hiperbarica espinhal em concentraçoes supra-clinicas determinou alteraçoes histopatologicas, restritas ao tecido nervoso da medula espinhal. A extensão e gravidade das lesoes foram intimamente relacionadas com a concentração da lidocaina. Lesoes brandas causadas por concentraçoes mais baixas de lidocaina podem nao manifestar alteraçoes clinicas.

Hyperfibrotic myelodysplasia: case report with response to steroid therapy / Mielodisplasia hiperfibrótica: relato de caso com resposta à terapia com corticoesteróides

A fibrose de medula óssea é encontrada em algumas doenças hematológicas clonais, incluindo síndromes mieloproliferativas, leucemias agudas e síndromes mielodisplásicas. Nas síndromes mielodisplásicas, uma nova entidade clinicopatológica com acentuado aumento das fibras de reticulina tem sido sugerida, e o termo "síndrome mielodisplásica hiperfibrótica" é usado para sua definiçäo. A biópsia de medula óssea mostra aumento das fibras de reticulina e hiperplasia megacariocítica com displasia. O diagnóstico diferencial com mielofibrose primária é difícil, e casos híbridos podem ocorrer. Pacientes com síndromes mielodisplásicas hiperfibróticas que respondem à terapia com corticosteróide têm sido descritos. Na maioria dos casos a remissäo é apenas hematológica, mas resoluçäo da fibrose de medula óssea já ocorreu em um paciente. Planejamento: Relato de caso. Relato: Paciente do sexo masculino, 62 anos de idade, apresentouðse, em junho de 1995, com história de seis meses de letargia e dispnéia. Ao exame, mostrouðse pálido, sem hepatoesplenomegalia. A concentraçäo de hemoglobina foi de 3g/dl, com acentuada anisocitose, mas sem "células em lágrimas". Os aspirados de medula óssea mostraramðse "secos", enquanto a biópsia revelou hipercelularidade e fibrose grau IV, obliterando a arquitetura habitual. Os megacariócitos estavam em número aumentado, com morfologia anômala, reagindo com anticorpos antifator VIII e CD31. Com a introduçäo de prednisona 1mg/kg em junho de 1996, os sintomas reduziram, a hemoglobina elevouðse a fibrose cedeu para grau II, tornandoðse independente de transfusões até janeiro de 1999. Entäo, a hemoglobina caiu para 6g/kl e, com a introduçäo de prednisona, houve pronta elevaçäo da hemoglobina

Factores predictivos de infiltración a médula ósea en niños con enfermedad de Hodking del Instituto Nacional de Pediatría / Predictive factors if bone marrow infiltration in children with Hodgkin's disease of the Instituto Nacional de Pediatría. Mexico

La biopsia de médula ósea ha sido esencial para la estadificación de pacientes con enfermedad de Hodgkin (EH). El presente estudio tiene como objetivo conocer si existen factores predictivos, clínicos, radiológicos y de laboratorio que permitan al oncólogo pediatra decidir de manera objetiva en qué pacientes debe o no realizar biopsia de médula ósea o debe de seguir siendo aparte del abordaje diagnóstico básico. Se incluyeron a los pacientes de uno u otro sexo con diagnóstico histopatológico de enfermedad de Hodgkin. Se consideraron como factores predictivos la variedad histológica, la presencia o ausencia de síntomas B, el estadio al momento del diagnóstico sin tomar en cuenta la infiltración a la médula ósea según la estadificación de Ann Arbor. Se realizó biometría hem tica completa, DHL y fosfatasa alcalina. Como variable dependiente se realizó biopsia de médula ósea bilateral a todos los pacientes posterior a la estadificación inicial, la médula ósea fue evaluada en el departamento de patología. Se formaron dos grupos de correlación entre la afección o no a la médula ósea y los factores predictivos estableciendo la significancia estadística de estas últimas tomando en cuenta el error alfa de .05. Para las variables continuas se realizó el Análisis con prueba de t y para las variables ordinales y nominales prueba exacta de Fisher. Se analizaron 109 pacientes. Los síntomas B, el estadio avanzado, variedad histología, depleción linfocítica, los niveles Hb al momento del diagnóstico y los niveles de fosfatasa alcalina mostraron tener una fuerte asociación con el resultado positivo de la biopsia de médula ósea. El estadio clínico se modificó en 12 pacientes posterior a las biopsias, del estadio III se estatificaron como IV sin embargo no hubo un caso en que un estadio temprano I o II se modificara, esto no tuvo repercusión en el tratamiento ya que el manejo para ambos estadios avanzados es el mismo. No se encontró asociación con género, cuenta leucocitaria o plaquetaria. De acuerdo a lo reportado y en estudios previos, la biopsia de MO podría estar limitada a pacientes con determinadas características clínicas como estadios clínicos III y IV así como en los pacientes con presencia de síntomas B, o con variedad histológica de depleción linfocítica, niveles bajos de Hb o fosfatasa alcalina elevada, lo que podría asegurar evitar realizar un procedimiento invasivo doloroso al paciente con EH que no tenga estas características

Long-term outcome of 25 children and adolescents with severe aplastic anemia treated with antithymocyte globulin

Severe aplastic anemia (SAA) is probably an immune-mediated disorder, and immunosuppressive therapy is recommended for patients with no available donor for bone marrow transplant. Between October 1984 and November 1987, 25 consecutive children and adolescents with SAA with no HLA-compatible marrow donor received equine antithymocyte globulin (ATG) (15 mg kg-1 day-1) for 10 days. The patients were evaluated 6 weeks, 6 months, and 12 months after starting ATG treatment. Thereafter, patients were evaluated yearly until July 1998. Median age was 10 years (range, 1.5-20 years), granulocyte counts on referral ranged from 0.032 to 1.4 x 10(9)/l (median 0.256 x 10(9)/l), and 12 patients had granulocyte counts < 0.2 x 10(9)/l. At a median follow-up of 9.6 years (range, 8.6-11.8 years), 10 patients (40 percent) remained alive with good marrow function. No morphologic evidence of hematological clonal disorders has been observed, although two patients probably have acquired clonal chromosomal abnormalities (trisomy 8 and del(6)q21, respectively). Responses to ATG were observed between 6 weeks and 6 months from the start of treatment in 60 percent of evaluable patients. The response rate was not different in patients whose granulocyte count at diagnosis was < 0.2 x 10(9)/l, or in those who were < 10 years of age. This study supports the view that, when compared with supportive measures, ATG is an effective treatment for children or adolescents with SAA. Although these results are inferior to those reported for marrow transplantation or more intensive immunosuppressive regimens, these patients who responded to ATG are long-term survivors with stable peripheral blood counts and a low rate of relapse.

Estudio de la incidencia de tumores raquimedulares tratados en el Servicio de Neurocirugía Hospital San Juan de Dios set. 1994 - mar. 1998 y revisión del tema / Study is a retrospective review of spinal cord tumors, it`s incidence and treatment at San Juan de Dios Hospital, that underwent surgery betwen september 94 to march 98

El presente trabajo es un estudio retrospectivo sobre la incidencia de presentación de tumores raquimedulares en pacientes tratados en el servicio de Neurocirugía del Hospital San Juan de Dios durante cuarenta y dos meses (8 setiembre de 1994 a marzo de 1998). Se incluyeron veintiséis pacientes: dieciocho (18) de sexo masculino y ocho (8) femeninas, se logró determinar los tipos de tumores más frecuentes y si eran de origen primario o metastásico, su histopatología; en relación con su localización a nivel raquídeo 46,1 por ciento fueron extraudrals y 53,9 intradurales. Se logró conocer las manifestaciones clínicas y síndromes clínicos al igual que los tratamientos realizados para cada caso en particular y los métodos de diagnósticos disponibles y empleados en estos pacientes y así obtener un modelo de tratamiento integral para que los futuros pacientes de nuestro servicio sean manejados en forma ideal

Histologic and histochemical studies on the bone marrow, spleen and liver in experimentally-induced chronic renal failure in the rat

Albino rat has been used as an animal model for adenine induced chronic renal failure. Animals were fed a diet containing 0.75% adenine in a dose of 250-350 mg/kg body weight/day for one month. Histologic and histochemical changes of bone marrow, spleen and liver were investigated. Bone marrow smear showed a hypocellularity in the from of a marked decrease of different stages of development of RBCs, leucocytes and megakaryocytes. This was associated with a marked increase in acid phosphatase enzyme activity and a decrease in succinic dehydrogenase enzyme activity. Numerous mast cells were also observed. With the spleen, lymphocytic depletion was observed in the periarteriolar lymphatic sheath, the rest of lymphatic follicle and the red pulp. Macrophage number was apparantely increased in the red pulp. Haemosiderin granules showed a marked increase in the red pulp. Like with the bone marrow, an increase in activity of acid phosphatase enzyme and a decrease in succinic dehydrogenase enzyme activity were noticed in the red pulp. In the liver, hepatocyte vacuolation and increased number of von Kupffer cells were noticed. An increase in acid phosphatase enzyme activity and a decrease in succinic dehydrogenase enzyme activity were observed. There was a patchy depletion of glycogen granules

Analysis of 3494 bone marrow examinations in a referralhospital: indications and interpretations

To characterize the indications of performing bone marrow examination and the frequent diagnoses encountered in a referral hospital in Riyadh, Saudi Arabia and to correlate the indications with the yield of specific pathologic diagnosis. The bone marrow records in the Hematology section of the Department of Pathology at King Khalid University Hospital over a 12-year period from May 1983 - May 1995 were reviewed. The records were reviewed for age, sex, main indication for performing bone marrow examination and primary bone marrow diagnosis. There were 3494 bone marrow examinations, 2375 [68%] were from adults and 1016 [29%] were from children. The M: F ratio was 1.5:1. The three most common indications were evaluation of postchemotherapy bone marrow in acute leukemia 26.2%, suspected infiltration by lymphoma or carcinoma in 10.3% and thrombocytopenia in 9.9%. The three most common specific diagnoses encountered were acute leukemia 27.3%, malignant lymphoma 13% and multiple myeloma 8.3%

Bone marrow involvement in Hodgkin's disease: the significance of non-infiltrative changes

We have tried to elucidate the significance of so called non-infiltrative changes in order to find their place in the staging procedure particularly in countries where facilities for elaborate clinical staging are not available. Seventy nine out of 88 patients were classified into 3 groups depending upon the histological findings in their bone marrow trephine biopsies. Bone marrow in Group-I [n = 20] patients was essentially normal. The established criteria of bone marrow involvement were fulfilled in Group III patients [n = 25]; while bone marrow in Group-II patients [n = 34] showed non-infiltrative changes. The clinical presentation, peripheral blood parameters and LDH levels of the 3 groups of patients were compared. There was progressive anaemia, neutrophilic leucocytosis and increase in ESR from Group I to III. The change was statistically significant when Group I was compared with Group II or Group III but non-significant when Group II was compared with Group III. It is, therefore, postulated that both Groups II and III reflect the bone marrow involvement although the changes observed in Group II do not satisfy the previously established criteria for this purpose

Síndrome de Shwachman / Shwachman syndrome

Antecedentes: El síndrome de Shwachman es considerado como la segunda causa de insuficiencia pancreática en niños, se acompaña de disfunción de la médula ósea así como de alteraciones esqueléticas. Objetivo: Conocer e identificar las manifestaciones clínicas, de laboratorio y gabinete en los pacientes con síndrome de Shwachman. Material y métodos: Se estudió a paciente femenino de un año cuatro meses realizándole historia clínica tomándose para el estudio peso y talla, percentilas y examen físico. Exámenes de laboratorio: biometría hemática, hemoglobina fetal, ferrocinética, de terminación de vitamina B12 y folatos, aspirado de médula ósea, pruebas de absorción intestinal, coproparasitoscópicos y coprocultivos, coproantígeno para Giardia, transaminasas séricas, perfíl viral para hepatitis, electrólitos en sudor. Gabinete: Radiografía de huesos largos, cadera, ultrasonido abdominal y tomografía axial computarizada pancreática. Biopsia hepática e intestinal. Resultados: En la paciente se corroboró la presencia de insuficiencia pancreática, alteración de la médula ósea, alteraciones óseas compatibles con el síndrome de Shwachman.

Osteopetrosis-a survey of 17 cases

This is a retrospective survey of 17 children with osteopetrosis admitted in paediatrics ward from 1990 to 1995. Their clinical data, radiographs, laboratory data and histories were analyzed. All of them were male with age range from 1 month to 5 years. 80% of patients had a history of parental consanguinity. All of them had moderate to severe anaemia and failure to thrive as their presenting symptoms. Other predominant associated features were stuffy nose with depressed nasal bridge [95%], hepatosplenomegaly [90%] and increase in size of head [80%]. Neurological complications such as visual loss [80%] and deafness [40%] were also observed. Not a single fracture was observed. The diagnosis of osteopetrosis was made by chance, radiologically. Treatment in all patients was symptomatic. No curative treatment was available. Mortality was high due to repeated infections and bleeding. The possibility of a new subtype of malignant recessive osteopetrosis is discussed

Trasplante autólogo de médula ósea y tratamiento in vitro en leucemia aguda no linfoblástica / Autologous bone marrow transplant in the treatment in vitro in acute nonlymphoblastic leukemia

Se reporta un paciente de 4 años y 9 meses de edad con el diagnóstico de leucemia aguda no linfoblástica (LANoL) M4 el cual presentó el antecedente de una recaída medular. Al momento de ser referido a nuestro departamento se encontró en remisión completa, clínica y hematológica. Previa evaluación por aparatos y sistemas se sometió el paciente al programa de trasplante autólogo de médula ósea (TAMO) y dado el diagnóstico, se trata la médula ósea in vitro con quimioterapia además de administrar megalodosis (terapia ablativa) de quimioterapia sistémica. Se describe el tratamiento in vitro de la médula ósea y el procedimiento tanto de evaluación para viabilidad celular antes y después del procesamineto de congelación. Por otro lado se señala la evolución del paciente, observando que la recuperación medular aconteció dentro del periodo descrito en la literatura. Este es el primer paciente pediátrico en México con LANoL sometido a TAMO con tratamiento in vitro de la médula ósea y con éxito terapéutico. Se discuten en términos generales tanto el tratamiento del paciente como el manejo de la médula ósea en el laboratorio. Actualmente el paciente se encuentra a más de 9 meses del trasplante en remisión completa y en excelente estado general. Se concluye que este procedimiento puede constituir en instituciones hospitalarias de tercer nivel otra opción de tratamiento, siempre y cuando la selección del paciente sea la correcta