Vera E. Papochieva, Dimitrinka S. Miteva, Penka I. Perenovska and Guergana Petrova
Histiocytoses comprise a group of diverse diseases of unknown etiology with various clinical presentation and evolution. The underlying pathology is characterised by accumulation and infiltration of variable numbers of cells of the monocyte-macrophage line in the affected tissues and organs. Histiocytoses are divided into three major classes: Langerhans cell histiocytosis (LCH), non- Langerhans cell histiocytosis, and malignant histiocytic disorders. The term LCH (also known in the past as histiocytosis X) encompasses the following rare diseases: Eosinophilic Granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease, Hashimoto-Pritzker disease, in which accumulation of pathologic Langerhans cells (LCs) leads to tissue damage. LCs usually reside in the skin and ensure protection against infections by destroying foreign substances. LC accumulation is caused by antigen stimulation and inadequate immune response. Thus, clinical LCH manifestations range from isolated disease with mono- or multifocal bone lesions to disseminated multisystem disease. LCH is a rare disease, affecting mainly children and young smokers, aged 20-50 years. Lung involvement in LCH usually presents as a mono-system disease and is characterized by Langerhans cell granulomas (LCG) infiltrating and impairing the distal bronchioles. The definite diagnosis is based on lung biopsy of CAT selected LCG areas. So far, there is no an effective treatment, but the better understanding of the mechanisms involved in the pathogenesis of the disease would help in the development of effective therapeutic strategies in the future.
Guergana Petrova, Penka I. Perenovska, Dimitrinka Miteva, Radost T. Kabakchieva, Ognyan G. Brankov and Rosen S. Drebov
Pneumonia is an inflammatory lung disorder characterized by consolidation due to presence of exudates in the alveolar spaces. Most pneumonias can be effectively treated with appropriate oral antibiotics, with intravenous antibiotics being reserved for those with severe infections. We present two cases of girls admitted in our clinic with pneumonia where our conventional therapy was not sufficient. Case 1: A 15-year-old girl with cystic fibrosis, with left lobular pneumonia, for which an aggressive conservative treatment was initiated. After significant improvement, sudden detorioration and pneumothorax of the left lung occurred. She was transferred to the surgical department for intervention. Due to failure to respond to initial drainage she underwent thoracotomy and resection of the left lower lobe of the lung. The histology result confirmed gangrene. Case 2: A four-year old girl was treated for pneumonia in the right lung with aggressive intravenous antibiotic. After temporary improvement sudden deterioration was observed. The patient was transferred to the surgery department, where pulmonary gangrene was confirmed. After the lower lobe of the right lung was resected, she was discharged in good health. The careful follow up, accurate diagnosis and correct medication choice are crucial for reducing the complications of “common” pneumonia.