Osteomielitis crónica multifocal recurrente / Chronic recurrent multifocal osteomyelitis

La osteomielitis crónica multifocal recurrente se considera actualmente una variante de un trastorno autoinflamatorio infrecuente, que requiere un alto índice de sospecha clínica para efectuar un adecuado abordaje diagnóstico y terapéutico. Presentamos un caso clínico típico, aportando datos sobre las hipótesis etiopatogénicas que se manejan actualmente en esta entidad (AU)

Chronic recurrent multifocal osteomyelitis is currently considered a variant of a rare autoinflammatory disorder, which requires a high index of clinic suspicion for suitable diagnostic and therapeutic approach. We present a typical case, providing data on etiopathogenetic hypothesis currently handled in this entity. The early detection of these malformations can prevent the appearance of chronic lung diseases and can allow for the best therapeutic approach. Although most of the cases do not require treatment, some of them require specific and more aggressive handling (AU)

[Pneumonectomy in cystic fibrosis]. / Neumonectomía en fibrosis quística.

We present the case of a 13-year-old boy with cystic fibrosis (CF) who developed severe right-sided lung infection with formation of abscess and localized bronchiectasis. The boy's lung disease was complicated by nephrotic syndrome and secondary amyloidosis. Unilateral pneumonectomy was performed, producing significant clinical improvement with a remarkable increase in quality of life which has lasted to the present date, 15 years later. Most patients with CF develop lung disease, which is the main cause of adult mortality in this population. Lung transplantation is currently considered the treatment of choice in severe bilateral lung disease in CF. However, in severe unilateral lung disease such as localized bronchiectasis, surgical resection of the affected lobe or lung is still a worthwhile option as a rescue therapy for patients who are at high risk of dying while waiting for lung transplantation.

Neumonectomía en fibrosis quística / Pneumonectomy in cystic fibrosis

Se presenta un niño de 13 años diagnosticado de fibrosis quística que desarrolló una infección grave del pulmón derecho con formación de abscesos y bronquiectasias localizadas. La evolución de su enfermedad se complicó con un síndrome nefrótico y amiloidosis secundaria. Se practicó neumonectomía derecha con lo que se consiguió una gran mejoría clínica y un aumento significativo en su calidad de vida hasta el momento actual, 15 años más tarde. Muchos pacientes con fibrosis quística desarrollan enfermedad pulmonar. Esta es la principal causa de mortalidad en la edad adulta. Hoy día, el trasplante pulmonar se considera el tratamiento de elección en la enfermedad pulmonar grave bilateral. Sin embargo, en los casos de lesiones graves unilaterales, como bronquiectasias localizadas, la resección quirúrgica del lóbulo o pulmón afectado es todavía una opción terapéutica para pacientes con alto riesgo de fallecimiento en la lista de espera para trasplante pulmonar (AU)

[The development of lipid and anthropometric parameters in the treatment of pre-pubertal obese patients]. / Evolución de parámetros lipídicos y antropométricos en el tratamiento de pacientes obesos prepúberes.

OBJECTIVE: Relationships have been found between obesity and changes in serum lipid levels which may lead to antherogenic lipid profiles. There are few studies treating obesity and showing the possibility of improving lipid abnormalities in prepubertal obese patients. PATIENTS AND METHODS: One hundred and three obese patients ranging from 5.3 to 9.9 years old have been studied. Anthropometric (height, weight, body mass index-BMI-, and subscapular and triceps skinfolds) and lipid (total and lipoprotein cholesterol, triglycerides, apoprotein A1 and B) parameters were measured both at the beginning of the study and after six months of diet treatment. Patients were included in three groups according to their response to treatment: good response (GR), no response (NR) and bad response (BR), if the difference in BMI was less than 1 point (< 1), between -1 and +1 and more than 1 point (> 1), respectively. RESULTS: Forty-one patients (39.8%) were noncompliant. Of the remaining sixty-two, 41 (66%) had a GR, 16 (25.8% NR and 5 (8%) BR. The obese patients showed a significantly lower HDL-C (38.8 +/- 15.6 vs 48.3 +/- 10.2 mg/dl, p < 0.01) in comparison to the control group. GR patients showed a statistically significant increase in HDL-C (48.2 +/- 9.1 vs 54.9 +/- 12.0 mg/dl, p < 0.01) and apoprotein A1 (126.7 +/- 17.3 vs 136.0 +/- 17.4 mg/dl, p < 0.05) levels at the end of the study. There was no statistically significant difference in serum lipid levels in the other two groups. There was no statistically significant correlation between serum lipid levels and anthropometric parameters. The difference between final minus initial value in both lipid and anthropometric parameters were not correlated. Sixteen patients (17.7%) had pathological serum lipid levels, 15 of them showing hypertriglyceridemia, that disappeared in GR patients. CONCLUSIONS: Prepubertal obese patients show alterations in lipid profiles and this is not correlated with anthropometric parameters. The improvement in these anthropometric parameters improve the lipid profile. Initial pathologic serum lipid levels in GR patients become normalized.

[An alpha 1-antitrypsin aerosol in the treatment of cystic fibrosis]. / Aerosol de alfa-1-antitripsina en el tratamiento de la fibrosis quística.

Alpha-1-antitrypsin (a-1AT) is a natural inhibitor of the elastase that is released physiologically by neutrophils in the lung. As a result of the increased neutrophil degranulation secondary to chronic epithelial inflammation in cystic fibrosis patients with chronic infections by Pseudomonas aeruginosa, there are larger amounts of elastase in airway secretions. This results in the a-1AT concentration being insufficient to inhibit the destructive proteolytic degradation, culminating in a chronic epithelial burden and a worsening of the cystic fibrosis pulmonary disease. In this preliminary study, we have evaluated the results obtained from the sputum of 4 cystic fibrosis patients treated with a-1AT (Prolastina, Bayer) in aerosol. The levels of a-1AT, neutrophil elastase, antineutrophil elastase activity, IgG, albumin and clinical parameters were measured. The concentration of sputum a-1AT was increased when compared to the same patient after 8 days with treatment (we compared means with Student's t-test and p < 0.05 was considered significant). We did the same with the impairment of neutrophil elastase, although we found no significant results. Nevertheless, antineutrophil elastase activity increased (p < 0.05). These results encourage us to continue the same treatment for a longer period of time to prevent pulmonary disease in CF subjects.