Maternal age and family history are risk factors for ankylosing spondylitis.

OBJECTIVE: To investigate prevalence and gender distribution in parents of children with ankylosing spondylitis (AS). METHODS: Family history of AS (parents, uncles, and aunts), maternal age at delivery, and consecutive pregnancy number were assessed in the relatives of 40 Mexican Mestizo patients with definite AS (New York Criteria). RESULTS: We evaluated the family history of AS in 34 families of 40 AS patients; 12 with none, 4 with a paternal history (4 healthy fathers with a brother with AS) (odds ratio, OR, 1.37, p = 0.75), 15 with a maternal history of AS, (15 healthy mothers with a brother with AS) (OR 1.4, p = 0.55), and 3 with both lines (OR 0.84, p = 0.92). In these families AS was more frequent in males (29%) than in females (10%), OR 3.40 (95% confidence interval, CI: 1.43-8.29, p = 0.003). Juvenile onset was more common in the offspring of mothers with family history (72%) (OR 13.0, 95% CI: 1.68-147.48, p = 0.009). The number of first-born children with AS (18%) was similar to the later-born children (23%) (OR 1.37, 95% CI: 0.38-5.31, p = 0.78). The frequency of AS increased when the maternal age at delivery was < or = 30 years (OR 0.20, 95% CI: 0.04-0.75, p = 0.01). CONCLUSION: In Mexican Mestizo patients, there is no correlation between the risk for AS and the gender of the affected parent. However we found an association between juvenile onset and maternal family history with an increased incidence in patients with younger mothers.

Epidermal growth factor and gastrin in scleroderma/CREST syndrome

Background. The objective of this study was to determine levels of epidermal growth factor (EGF) and gastrin (GA) in saliva, serum, and urine in scleroderma (Scl) and CREST syndrome. Methods. EGF and GA levels were mesured by radioimmunoassay in saliva, serum and urine in 10 patients (51 years, median; range, 35-66 years); 9 females and 1 male with Scl, 3 females with CREST syndrome, and 18 age-and sex-matched controls, 17 females and 1 male free of any systemic inflammatory disease. Results. In serum, the EGF was lower in Scl/CREST than controls (p=0.02), while GA serum concentrations were higher in Scl/CREST (p=0.02). In urine, EGF in Scl/CREST was slightly lower than controls (p=NS) and GA concentrations were higher than controls (p=0.03). In saliva, the EGF levels in Scl/CREST were also slightly lower than controls (p=NS), while GA concentrations in both Scl/CREST and controls were not different (p=NS). Conclusions. Low concentrations of EGF in serum probably play a role in the pathogenesis of Scl/CREST. GA concentration can be increased as a consequence of the low levels of EGF because of the structural homology of this peptide with urogastrone, a GA inhibitor factor

Rhabdomyolysis in patients with acute lymphoblastic leukemia

The objective of this paper is to report 5 cases of rhabdomyolysis (RML) in patients with acute leukemia (AL). This occurred consecutively after the administration of chemotherapy, during the ensuing period of myelosuppression. Thirty-six patients with AL received, in a three-month period, 51 cycles of combined chemotherapy which included, in all of them, cytosine arabinoside (ARA-C); among them, along with myelosuppression, five experienced fever, infectious complications, gastrointestinal tract symptoms and severe myalgias. Serum creatine kinase (CK), liver function tests and a light microscopy muscle biopsy were performed on all of them. Ten - 17 days after receiving chemotherapy, five patients (4 males and 1 female) with acute lymphoblastic leukemia developed incapacitating myalgias in neck, thighs and arms. CK and/or alanine aminotransferase and spartate aminotransferase were increased 5-24 times above the normal range in four of these patients, and the muscle byopsy showed focal RML in all five. Myalgias were self-limited and lasted 4-10 days. In addition to the chemotherapy, other factors known to be capable of producing RML, such as sepsis, other medications, and dehydration were found. In conclusion, myalgias were due to focal RML produced probably by a combination of factor, particularly the chemotherapy along with dehydration due to gastrointestinal complications, infection, and the use of diverse antibioticas. The endemic nature of the finding in such a short period of time is outstanding

Disfunción ovárica en esclerosis sistémica progresiva/CREST / Ovarian disfunction in progressive systemic sclerosis/CREST

Objetivo. Estudiar la función ovárica en esclerosis sistémica progresiva (ESP)/CREST. Metodología. Se estudiaron 13 mujeres con ESP/CREST y 18 controles sanas, subdivididas en menstruantes y menopáusicas y analizadas desde los puntos de vista reumatológico, endocrinológico y ginecológico. Resultados. La ESP inició a edad más temprana que el CREST (p=0.02). Ocho enfermas menstruantes (Grupo 1) tuvieron opsomenorrea y amenorrea y 2 infertilidad. En las pacientes ESP/CREST menopáusicas (Grupo 2), 3 tuvieron menopausia temprana. En el Grupo 1, la hormona folículo estimulante (HFE) y la prolactina (PRL) en suero resultaron elevadas (p=NS) y el estradiol (E2) sérico disminuido en la fase preovulatoria respecto de las controles (p<0.05). En el Grupo 2 la HFE y la luteinizante estuvieron elevadas en suero (p<0.04 y p<0.03, respectivamente) y el E2 fue bajo (p=NS) respecto de las controles. El Grupo 1 tuvo inmunoglobulinas séricas (p=0.0001 y 0.004) y velocidad de sedimentación globular (VSG, p=0.016) mayores que las controles. En el grupo de ESP/CREST hubo correlación entre E2 e IgA (p<0.001) y los niveles de PRL e IgM (p<0.001), que fueron diferentes a los de las controles. Conclusión. En pacientes con ESP/CREST existe disfunción ovárica, con problable participación de E2 y PRL como intermediarias de la inflamación y/o de la respuesta inmunológica

Storage histiocytes and hemophagocytosis: a common finding in the bone marrow of patients with active systemic lupus erythematosus

We report the frequency of the finding of storage and hemophagocytic histiocytes in the bone marrow of patients with systemic lupus erythematosus with one or more hemocytopenias. The study was performed on bone marrows of ten patients with systemic lupus erythematosus during an episode of hemocytopenia. Four patients were not receiving any treatment and six had been takin oral prednisone (3.5 ñ 1.5 mg/day) for the previous 6 months. Hemocytopenia found were lymphocytopenia in five, thrombocytopenia in three, and neutropenia and anemia in two each. The bone marrow had variable cell content; megakaryocytes, the myeloid:erythroid ratio, as well as lymphocyte, plasma cell, and reticular cell contents were usually increased. Seven bone marrow samples showed storage distiocytes (sea-blue histiocytes and other histiocytes that contained unidentified intracytoplasmic material). These same samples revealed histiocytes phagocytosing erythoblasts, erythrocytes, polymorphonuclear neutrophils, lymphocytes in all seven patients was related to a decrease in serum complement and with lupus disease activity as mesured with the SLEDAI index (X ñ SD = 2.1 ñ 1.5). The SLEDAI score of the remaining three patients, who had no histiocytes in their bone marrow, was 0, 0, and 1, respectively. the present study reveals that the bone marrow in patients with systemic lupus erythematosus and peripheral hemocytopenia contains storage and hemophagocytic histiocytes and the significance of these cells is discussed

Tomografía computada de las articulaciones sacroiliacas y esternoclaviculares en la espondilitis anquilosante juvenil y del adulto

Se empleó la tomografía computada (TC) con el fin de detectar la afección articular temprana de las sacroiliacas (SI) y de la esternoclavicular (EC) en 68 pacientes consecutivos con espondilitis anquilosante (EA) con una evolución de 14.5 ñ 2.7 años clasificándose según la edad de inicio y el sexo en: espondilitis juvenil (EJ) 19 pacientes (p), espondilitis de inicio en el hombre adulto (EHA) 33 p y espondilitis de inicio en la mujer adulta (EMA) 16 p. Para su análisis se empleó la prueba de Fisher. Resultados: La TC de SI fue anormal en 67/68 casos mostrando cambios erosivos desde los 8 meses de iniciada la enfermedad en adelante. En 1/49 hombres y 4/19 mujeres se detectó hipodensidad intraarticular compatible con gas (-13 UH), líquido (+19 UH) o grasa (+232 UH) (p< 0.001). Las EC estuvieron anormales en 27/66 (41 por ciento) de los casos B27 + (en 2 no se practicó TC por problemas técnicos), observándose disminución del espacio articular y erosiones sin anquilosis de inicio y predominio clavicular. Por grupos las TC de SI mostró alteraciones más tempranas en la EHA y en loe EJ que en la mujer (p< 0.001) con un predominio de las imágenes hipodensas en la mujer sobre los otros grupos (p< 0.001). Las alteraciones de las EC fueron más frecuentes y tempranas en la EJ que en la de los adultos (p< 0.001). Estos datos muestran diferente evolución tomográfica de la enfermedad entre los 3 grupos