Immune deficiencies in chronic intestinal pseudo-obstruction

Aim: Chronic intestinal pseudo-obstruction has been associated with urinary disorders, myopathy, and ophthalmoplegia in adults and cholelithiasis in children. We observed a high percentage of total-parenteral-nutrition-dependent patients with pseudo-obstruction and recurrent infections requiring gammaglobulin infusions. Methods: AH records for 23 children with chronic intestinal pseudo-obstruction (10 females and 13 males, mean age 9.8 y ± 4.9 y, range 4–24 y) referred for a nutritional evaluation from 1992 to 1995 were reviewed. Chronic intestinal pseudo-obstruction was diagnosed by clinical, radiographic findings and antroduodenal manometry. Intestinal full-thickness biopsies were performed in seven children. Results: Hypogammaglobulinemia was diagnosed in 18 patients (78%): 16 patients had various immunoglobulin deficiencies and 2 had selective antibody deficiency. Intravenous gammaglobulin was administered in 14 patients. Other medical conditions affecting the children are summarized as follows: autonomic dysfunction in 10 patients (43%), recurrent hypoglycemia in 9 (39%), asthma in 9 (39%), cholecystitis in 7 (30%), low serum carnitine level in 6 (26%), urinary dysfunction in 6 (26%), pancreatitis in 5 (22%), behavioral problems in 5 (22%), myopathy in 2 (9%), idiopathic thrombocytopenia in 2 (8%), velopharyngeal insufficiency in 1 (4%), oculocutaneous albinism in 1 (4%), Pierre-Robin syndrome in 1 (4%), and protein C deficiency in 1 (4%). Munchausen syndrome was suspected in two patients. Conclusions: Chronic intestinal pseudo-obstruction appears to be associated with immune deficiencies. It is unclear if the immune deficiencies, intestinal pseudo-obstruction, and the other medical conditions have a common underlying etiology. Repeated infections may be due to impaired immune function in children with chronic intestinal pseudo-obstruction. We recommend screening for immune deficiencies in children with chronic intestinal pseudo-obstruction.     

Clinical case review: A method to improve identification of true clinical and radiographic pneumonia in children meeting the World Health Organization definition for pneumonia

Background

In 2001, two hexavalent vaccines were licensed in Italy (Hexavac^®, Infanrix Hexa^®), and since 2002 were extensively used for primary immunization in the first year of life (at 3, 5, 11/12 months of age). In 2005, the market authorization of Hexavac^® was precautionary suspended by EMEA, because of doubts on long-term protection against hepatitis B virus. The objectives of this study were to evaluate the persistence of antibodies to anti-HBs, in children in the third year of life, and to investigate the response to a booster dose of hepatitis B vaccine.

Methods

Participant children were enrolled concomitantly with the offering of anti-polio booster dose, in the third year of life. Anti-HBs titers were determined on capillary blood samples. A booster dose of hepatitis B vaccine was administered to children with anti-HBs titers < 10 mIU/ml, with the monovalent precursor product of the previously received hexavalent vaccine. HBsAb titers were tested again one month after the booster.

Results

Sera from 113 children previously vaccinated with Hexavac^®, and from 124 vaccinated with Infanrix Hexa^® were tested for anti-HBs. Titers were ≥ 10 mIU/ml in 69% and 96% (p < 0,0001) respectively. The proportion of children with titers ≥ 100 mIU/ml did also significantly differ among groups (27% and 78%; p < 0,0001). Post-booster, 93% of children achieved titers ≥ 10 mIU/ml, with no significant difference by vaccine group.

Discussion

Fifteen months after third dose administration, a significant difference in anti-HBs titers was noted in the two vaccine groups considered. Monovalent hepatitis B vaccine administration in 3-year old children induced a proper booster response, confirming that immunologic memory persists in children with anti-HBs titers < 10 mIU/ml. However, long-term persistence of HBV protection after hexavalent vaccines administration should be further evaluated over time.     

Deletions of the cyclin-dependent kinase inhibitor genes p16INK4A and p15INK4B in non-Hodgkin's lymphomas

The tumor suppressor genes p16INK4A and p15INK4B map to the 9p21 chromosomal locus and are either homozygously deleted or mutated in a wide range of human cancer cell lines and tumors. Although chromosome 9 abnormalities have been described in non-Hodgkin's lymphomas (NHLs), to date, the mutational status of these genes has not been determined for these malignancies. A total of five cell lines and 75 NHLs were examined for homozygous deletions or point mutations in the coding regions of both the p15 and p16 genes using Southern blot and/or polymerase chain reaction-single-strand conformation polymorphism analyses. Homozygous deletions of either the p16 gene or both the p15 and p16 genes were observed in one diffuse large B-cell lymphoma cell line and two uncultured lymphomas consisting of one large B-cell and one mixed T-cell lymphoma. In contrast, point mutations were not detected in either the cell lines or lymphomas. These results indicate that the rate of alterations in the p15 and p16 genes is low for lymphomas, but loss of p16 and/or p15 may be involved in the development of some lymphomas.     

Negative ANA-IIF in SLE patients: what is beyond?

Clinical Rheumatology - The antinuclear antibody (ANA) test has high sensitivity in diagnosing and classifying systemic lupus erythematosus (SLE). To describe the immunological pattern of SLE...     

Bicycle-related injuries requiring hospitalization in the United Arab Emirates

ObjectivesTo study the anatomical distribution, severity, and outcome of bicycle-related injuries requiring hospitalization in Al-Ain city, United Arab Emirates in order to improve preventive measures.MethodsAll patients with bicycle-related injuries who were admitted to Al-Ain Hospital or who died after arrival were studied. Data were prospectively collected over a period of six years (October 2001–October 2007). Demography of patients, Glasgow Coma Scale (GCS), Injury Severity Score (ISS), hospital stay, and mortality were analysed.ResultsThere were 130 patients (126 males). Mean (SD) age was 27.1 (14.5) years. 17.7% were United Arab Emirates (UAE) nationals. None of the patients was wearing a helmet. The percentage of UAE nationals of less than 15 years old was significantly higher (65.2%) than non-UAE nationals (14.3%) (p < 0.0001, Fisher's Exact Test). The most common mechanism of injury for UAE nationals was falling from a bicycle (73.7%) whilst for non-UAE nationals was hitting a moving vehicle (66.7%). 96 (73.9%) patients had head and face injuries whilst 91 patients (70%) had extremity injuries. On arrival to the hospital, the median (range) ISS was 4 (1–41) and the median (range) GCS was 15 (3–15). The median (range) of total hospital stay was 4 (1–95) days. 17 patients (13.1%) were admitted to the Intensive Care Unit. Two patients died because of head injury (overall mortality was 1.5%).ConclusionsThe majority of hospitalized injured cyclists in our study were low income adults using cycling as a cheap transportation method. Compulsory helmet use by bicycle riders and subsidising helmet cost should be adopted so as to reduce morbidity and mortality of bicycle-related injuries.