Congenital choledochal cyst in an infant with cystic fibrosis.

Congenital choledochal cyst is malformation of the biliary ductal system, which rarely occur. We describe here a 4-month old boy, who was referred to our center with respiratory distress and low level consciousness. In physical examination, a mass was detected in right upper quadrant of abdomen. Sonographic examination indicated a cystic structure representing the choledochal cyst. Further evaluation confirmed the diagnosis of cystic fibrosis in this patient. Although choledochal cyst is considered as a rare disease, it is the most frequent malformation of the extrahepatic biliary ducts, which easily could be misdiagnosed.

Osteomyelitis due to BCG vaccination.

A six month-old immunocompetent boy who received BCG at birth presented with multiple abscesses in left subaxillary region, and swelling and wound infection on the left arm. Radiographs revealed osteolytic lesion in the left humerus. A biopsy from the site revealed chronic granulomatous lesion, positive for M. bovis on tissue culture. The lesion responded to antituberculous therapy and surgical treatment. There are no sequelae after 2 years of follow-up.

Evaluation of humoral immune function in patients with bronchiectasis.

Bronchiectasis is a chronic debilitating condition characterized by abnormal dilated thick-walled bronchi. To investigate humoral immune function in bronchiectatic patients, this study was performed. Forty patients with established diagnosis of bronchiectasis, who were referred from two tertiary care pulmonology centers in Tehran, were investigated in this study. Immunoglobulin isotypes concentrations and IgG-subclasses were measured by nephelometry and enzyme-linked immunosorbent assay (ELISA) methods, respectively. All patients received unconjugated pneumococcal vaccine, and blood samples were taken before and 21 days after vaccination. Specific antibodies against whole pneumococcal antigens were measured using the ELISA method. Fifteen (37.5%) out of 40 patients were diagnosed to have defects in antibody mediated immunity including 5 (12.5%) patients with immunoglobulin class deficiency (2 with common variable immunodeficiency and 3 with IgA deficiency), 3 (7.5%) with IgG subclass deficiency and 7 (17.5%) patients had Specific antibody deficiency (SAD) against polysaccharide antigen despite normal levels of serum immunoglobulins and IgG subclasses. Our study along with several other studies confirmed that all patients with bronchiectasis should undergo thorough immunological evaluation in order to identify the presence of the underlying immunologic defect. This evaluation should include serum immunoglobulins, IgG subclasses concentrations and also determination of serum antibodies against pneumococcal antigens. Early diagnosis and appropriate treatment will prevent the subsequent complications and improve quality of life of affected individuals.

Griscelli syndrome type 2; a pediatric case with immunodeficiency.

A 3.5 month-old girl was admitted with silvery gray hair, light colored skin, recurrent diarrhea, chest infections, hepatosplenomegaly, episodes of pancytopenia, and hemophagocytosis in the bone marrow. Light microscopy of hair showed characteristic large and irregular clumps of melanin in the middle of hair shaft. Peripheral blood smear examination did not show giant granules in granulocytes. On the basis of these clinical and laboratory findings, Griscelli syndrome was diagnosed. The child succumbed to infection during an accelerated phase of the disease.

Disseminated Mycobacterium bovis infection after BCG vaccination.

The Calmette-Güerin vaccine (BCG) is administered to all the newborns in Iran in order to prevent tuberculosis. Complications of this vaccine are uncommon. We report disseminated BCG disease in 17 patients less than 10 years old. This is a retrospective study of total of 17 cases who were admitted in Children Medical Center Hospital with systemic syndrome compatible with Mycobacterium disease with evidence of histopathologic demonstration of acid-fast bacilli during 1995-2004. Fourteen cases occurred in children younger than 1 year old. Nine patients were female. Ten of the 17 total cases were associated with an immune deficiency including severe combined immunodeficiency, chronic granulomatous disease and cell mediated immune defect. Response to therapy was ineffective and 10 of them (58.8%) died. Disseminated BCG disease is an uncommon but devastating complication of vaccination that should be considered in the appropriate clinical setting. Immune-compromised infants are at greatest risk and they respond poorly to standard therapies.