Rectal carcinoma in a young female patient with peutz-jeghers syndrome: a case report

To report a case of rectal cancer in a patient with Peutz-Jeghers syndrome [PJS]. A 20-year-old woman with intermittent bloody stool of 4 months was admitted for examination. Gastroendoscopy revealed multiple polyps involving the stomach, small intestine, colon and a rectal adenocarcinoma. A diagnosis of PJS was made based on intestinal polyps with characteristic pathology and melanotic macules on the lips. After surgery and chemotherapy upon follow-up at 8 months, the patient did not have any signs of recurrence. This case showed that rectal carcinoma should be considered for young patients with PJS

Mechanisms of all-trans retinoic acid-induced differentiation of acute promyelocytic leukemia cells.

Retinoic acids (RA) play a key role in myeloid differentiation through their agonistic nuclear receptors (RAR alpha/RXR) to modulate the expression of target genes. In acute promyelocytic leukemia (APL) cells with rearrangement of retinoic acid receptor a (RAR alpha) (including: PML-RAR alpha, PLZF-RAR alpha, NPM-RAR alpha, NuMA- RAR alpha or STAT5b-RAR alpha) as a result of chromosomal translocations, the RA signal pathway is disrupted and myeloid differentiation is arrested at the promyelocytic stage. Pharmacologic dosage of all-trans retinoic acid (ATRA) directly modulates PML-RAR alpha and its interaction with the nuclear receptor co-repressor complex, which restores the wild-type RAR alpha/RXR regulatory pathway and induces the transcriptional expression of downstream genes. Analysing gene expression profiles in APL cells before and after ATRA treatment represents a useful approach to identify genes whose functions are involved in this new cancer treatment. A chronologically well coordinated modulation of ATRA-regulated genes has thus been revealed which seems to constitute a balanced functional network underlying decreased cellular proliferation, initiation and progression of maturation, and maintenance of cell survival before terminal differentiation.

The effect of immunotherapy on bronchial hyperresponsiveness in asthmatic children.

Bronchial hyperresponsiveness (BHR) to methacholine were evaluated in 47 asthmatic children before and after allergen-specific immunotherapy (IT) by using the forced oscillation method. Eighty-seven percent (13/16) of BHR-negative patients had good clinical response after 1-year immunotherapy while there were only 45% (14/31) in the BHR-positive asthmatic children (p < 0.02). In the BHR-positive group, the relationship between clinical response and the change of nonspecific bronchial sensitivity was further analyzed. In those of good clinical response (IT responder), the tolerance dose of methacholine was significantly increased from 0.78 +/- 0.71 to 4.11 +/- 4.65 mg/ml (p < 0.05), and bronchial sensitivity increased from 1.14 +/- 1.42 U to 7.55 +/- 9.55 U (p < 0.02). In those with no clinical improvement (IT non-responder), there were no significant changes in either methacholine tolerance dose or bronchial sensitivity. With respect to other parameters, such as Grs, PD35, and SGrs, the differences between before and after immunotherapy were similar in both the IT responders and IT non-responders. These results suggest that asthmatic children with different bronchial sensitivity had different responses to immunotherapy and the clinical improvement after immunotherapy is significantly related to the improvement of bronchial hyperresponsiveness.

The change of allergen-specific IgG subclass antibodies during immunotherapy in mite-sensitive asthmatic children.

Fifty-six Dermatophagoid farinae (D.f)-sensitive asthmatic children were hyposensitized by D.f-crude extract for two years. Serum total IgG subclass antibodies and D.f-specific IgE and IgG subclass antibodies were measured by ELISA before and after 2 years of treatment. The results showed that 1) After two years of treatment, there were significantly higher levels of total serum IgG1 in both responder and non-responder groups than those before treatment (p less than 0.01). The responder group also had significantly higher values of total IgG2 and IgG4 after immunotherapy (IT) (p less than 0.05), but not in the non-responder group. 2) The serum levels of D.f-specific IgG3 and IgG4 antibodies in responder group increased significantly after IT (p less than 0.05). On the contrary, the D.f-specific IgE and IgG1 IgG1 in the responder group were significantly lower than those before IT. No signi- in the responder group were significantly lower than those before IT. No signi-body titres before and after IT was found in non-responder group. 3) There was a significant correlation between the total IgG4 and D.f-specific IgG4 antibody (r = 0.634, p less than 0.01). The correlation coefficient was 0.634. No correlation was found between the other IgG subclass antibodies and D.f-specific IgG subclass antibodies. 4) Correlations between the levels of D.f-specific IgE and IgG subclass antibodies were highly significant both in IT-responder and non-responder groups. There was a significant correlation between the levels of D.f-specific IgG1 and IgG4 in non-responders, while no relationship was observed in the responder group.(ABSTRACT TRUNCATED AT 250 WORDS)

The study of bronchial hyperresponsiveness in asthmatic children by forced oscillation technique.

We have studied the bronchial hyperresponsiveness (BHR) of children with normal controls and asthma by methacholine inhalation challenge, using a forced oscillation method. Four parameters, respiratory conductance (Grs), bronchial responsiveness (PD35Grs), bronchial sensitivity (Dmin) and reactivity (SGrs) were studied. There were three patterns of dose-response curves identified in this study, which were significantly correlated to the clinical severity of asthma. (r = 0.846, p less than 0.001, Spearman's rank correlation). There were significant negative correlations between control Rrs (Rrs cont.) and age (r = 0.514, p less than 0.001) or body height (r = 0.685, p less than 0.001). Positive correlations between SGrs and subjects' age (r = 0.457, p less than 0.001) and body height (r = 0.496, p less than 0.001) were also noted. In the normal controls, Dmin and PD35Grs were over 25 units and 50 units, respectively. The Grs for normal children was statistically higher than that of asthmatic children (p less than 0.05). In the asthmatic children, there were significant differences among all subgroups in PD35Grs (p less than 0.001) and Dmin (p less than 0.01). In summary, the bronchial provocation test using the forced oscillation technique is simple, fast and easy to be applied to children. In addition to being capable of investigating BHR, it may offer valuable information for the clinical diagnosis and treatment of asthmatic children.

The effect of immunotherapy on the in vitro productions of histamine, prostaglandin E2 and leukotriene C4 in asthmatic children.

In order to elucidate the working mechanisms of immunotherapy (IT), the in vitro productions of histamine, prostaglandin E2 (PGE2) and leukotriene C4 (LTC4) were studied in 18 newly diagnosed and 20 hyposensitized (greater than 2 yr) asthmatic children. All were sensitive to house dust and dust mites. (D. pteronyssinus). Ten age-matched normal children were included as control. Polymorphonuclear (PMNs) and mononuclear (MNCs) leukocytes were separated by density gradient centrifugation and dextran sedimentation. PMNs (2 x 10(7) cells/ml) and MNCs (2 x 10(7) cells/ml) were stimulated with mite allergen (10 micrograms/ml) and calcium ionophore A23187 (1 microgram/ml) for 15 minutes. The plasma and culture supernatant (sup) histamine levels and sup PGE2 and LTC4 were measured by RIA. The results showed; 1) When compared to new patients, the treated patients had much lower plasma and sup histamine (p less than 0.001), no matter whether PMNs and MNCs were stimulated with allergen or A23187 and the normals had the lowest histamine level among 3 groups; 2) LTC4 in A23187-stimulated sup was lower in treated patients (p less than 0.05); 3) The PGE2 in allergen-stimulated sup was markedly increased in treated patients as compared to new patients (p less than 0.01) and the PGE2 in sup of normals was also much higher than that of new patients. Thus, immunotherapy is able to reverse the abnormal secretory pattern of inflammatory mediators of allergic patients, and this change may account, partly, for its clinical effectiveness.

Selective IgG subclass deficiencies in patients with recurrent sinopulmonary infections: report of two cases.

Two patients with recurrent sinopulmonary infections and normal total serum immunoglobulin levels were found to have selective deficiencies in IgG subclasses. The serum of one patient contained abnormally low IgG2 and IgG4; and the other was deficient in IgG4. Both patients responded to the treatment with high dose intravenous immunoglobulin. The experiences on these two cases strongly suggest that IgG subclasses should be checked in patients with recurrent sinopulmonary infections in face of normal total immunoglobulins.