Primary hypogammaglobulinemia (Bruton's disease) is a rare X-linked infantile immunodeficiency syndrome due to a B-cell defect. The patients suffer from acute and recurrent bacterial infections with chronic rhinosinusitis and chronic lung disorders. Immunoglobulin replacement therapy and antibiotics do not suffice in some cases, making sinus surgery to advance the drainage necessary. A 25-year-old man with hypogammaglobulinemia was treated with functional endoscopic sinus surgery and mucotomy of the turbinates. Tissue samples of the inferior turbinates were taken for histological and electron-microscopic examination. Immuno-electron-microscopic methods were carried out with antibodies against substance P and calcitonin gene-related peptide (CGRP). Morphological investigations to better understand pathophysiological changes in hypogammaglobulinemia are rare. Pathological changes in the glands and venous vessels could be demonstrated. A rich neural supply and participation of neuropeptides such as substance P and CGRP could play a role in the unspecific defense via neurogenetic inflammation in these cases.
Primary ciliary dyskinesia syndrome (PCD) is a rare, autosomal receive disorder. Kartagener's syndrome is a subgroup of the PCD with situs inversus, bronchiectasis, and sinusitis. The symptoms results from an abnormal ultrastructural morphology of the cilia such as absence of dynein arms and other changes. As a consequence ciliary motility is disturbed. A 25-year-old man was examined because he suffered from recurrent severe pneumonia and Aspergillus infections of the lungs. On electron micrographs, ciliary abnormalities including deficiency of inner and outer dynein arms, dysmorphic outer dynein arms, and disorientation of the cilia were demonstrated. The diagnosis of PCD requires electron-microscopic investigations of the ciliated mucosa. Special attention should be given to ultrastructural changes of nasal or bronchial mucosa if a young patient suffers from recurrent severe respiratory infections.
Osteoarthritis prevalence of 22% to 39% in India. OA is more common in women than men. Nearly, 45% of women over the age of 65 years have symptoms while 70% of those over 65 years show radiological evidence of OA.
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