Sulphasalazine therapy in chronic uveitis of children with chronic arthritis.

Four children with chronic arthritis (3 juvenile rheumatoid arthritis and 1 juvenile ankylosing spondylitis) and poorly controlled chronic uveitis, were given sulphasalazine (SASP) therapy for a mean period of 3.3 years. Three patients showed an excellent response, as evidenced by a reduction of inflammatory cells in the anterior chamber of the eyes and improvement of visual acuity. The response occurred after a mean of 7.7 weeks. These data suggested SASP therapy may have a role in the treatment of chronic anterior uveitis in children with chronic arthritis.

Successful bone marrow transplantation in a Chinese boy with Wiskott-Aldrich syndrome.

We describe the successful use of HLA-compatible sibling bone marrow transplantation (BMT) in a 17-month-old Chinese boy in whom Wiskott-Aldrich syndrome (WAS) was diagnosed on the basis of eczema, thrombocytopenia, recurrent otitis media and abnormal immunological tests. The conditioning chemotherapy included 2 days' oral busulfan, 40 mg/m2/6 hours, and 2 days' intravenous cyclophosphamide, 60 mg/kg/day (BU2CY2). Complete hematological chimerism was achieved 3 weeks after transplantation. Eight months after his BMT the eczema has resolved, platelet count is normal, and he no longer has frequent infections. BU2CY2 as a preconditioning regimen gave complete lymphohematopoietic engraftment in this WAS patient with no evidence of graft-versus-host disease. The excellent clinical response of this patient and the inevitable fatal outcome of WAS support the opinion that where a histocompatible donor is available, BMT at the earliest opportunity is the best option. We believe this is the first case of successful BMT in a Chinese patient with WAS.

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)

Immunochemotherapy with recombinant interleukin-2 and adriamycin in primary hepatocellular carcinoma.

Recombinant interleukin-2 (rIL-2) and adriamycin were administered systemically to treat nine patients (age 15.5-68 years, mean 48.9 +/- 15.5 years) with far advanced primary hepatocellular carcinoma. Three patients were newly diagnosed, and the remaining patients had received surgery, transcatheter arterial embolization, chemotherapy and other treatments but without improvement. rIL-2 was given at a dose of 10,000 to 30,000 units/kg every 8 hours for consecutive 9 days, and on the fifth day, a single dose of adriamycin 30 to 60 mg/m2 was administered. Four patients interrupted the immunotherapy because of severe intolerable side effects, 4 patients completed one course and the remaining one received 2 courses of treatment. Various adverse reactions were encountered, however, they subsided promptly after stopping therapy. All patients failed to respond to the regimen. Primary hepatic tumors continued to enlarge in 8 patients and remained unchanged in one, and pulmonary metastasis also increased in size and number in 4 patients. Transient decrease in serum alpha-fetoprotein was found in 6 patients. These results suggest that systemic IL-2 immunotherapy, even in combination with chemotherapy, is not effective for the treatment of far advanced hepatocellular carcinoma. However, in view of its immune amplifying effect, rIL-2 in combination with other treatment modalities may still be worth trying in early stages of hepatocellular carcinoma.

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.

Changes of allergen-specific antibodies, circulating immune complexes and restoration of polymorphonuclear leukocyte function after hyposensitization.

To explore the working mechanism(s) and the safety of long-term hyposensitization (HS) with house dust (HD), a series of studies were undertaken on 30 newly diagnosed and 30 hyposensitized asthmatic children. Twenty age- and sex-matched school children were included as control. The results showed: (1) HS was able to decrease the total serum IgE and increase the production of allergen-specific IgG blocking antibody, however, the allergen-specific IgE antibody remained nearly the same after HS for a couple of years, (2) Normal controls had allergen-specific IgG antibody but no IgE antibody, (3) Circulating immune complex concentration in the treated group did not differ significantly from the untreated group, (4) HS was able to suppress in vivo and in vitro histamine production and restore polymorphonuclear leukocyte (PMN) function in terms of Fc gamma R expression. These results suggest that HS is a specific and safe treatment, and provide solid rationale for its use in the treatment of respiratory allergic disease.

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.

Chronic granulomatous disease in two Chinese families.

Two Chinese families with X-linked chronic granulomatous disease (CGD) are reported. The first case was an 11-month-old male baby and the second a 2-month-old male baby. Both patients presented with persistent infections caused by Staphylococcus and Candida since birth. Neutrophil functions were studied in patients and a number of family members. Chemotaxis and phagocytosis were normal in every subject. Slide and spectrophotometric nitroblue tetrazolium (NBT) tests of both patients were abnormal and remained unchanged in spite of treatment with ascorbic acid, levamisole, sulfamethoxazole, trimethoprim and isoniazide. Mothers were proved to be carriers as evidenced by the presence of both normal and CGD phagocytes in the slide NBT test. During the 2-month follow-up period, the percentage of normal phagocytes from the mother of case 1 varied from 12% to 73%, which correlated with the fluctuation of spectrophotometric NBT value. The slide NBT test of the mother of case 2 was nearly normal in face of the presence of CGD phagocytes. Both carrier mothers were healthy and asymptomatic.

Interleukin 2 and lymphokine-activated killer cells in the treatment of childhood primary hepatocellular carcinoma--a preliminary report.

Recombinant interleukin-2 (RIL-2) and lymphokine-activated killer (LAK) cells were administered to 2 boys with the end-stage of primary hepatocellular carcinoma (HCC); the efficacy and toxicity were evaluated. Immunologically, the natural killer and LAK activities were enhanced. Clinically, the side effects were similar to those reported for adults but milder. This kind of treatment may be considered for children with the early stages of hepatocellular carcinoma.

Immunological defects in allergic asthmatic children and working mechanisms of hyposensitization--a review.

Patients with allergic diseases are characterized by the presence of elevated serum IgE and specific IgE antibodies against a variety of environmental allergens, especially house dust, mites and molds in Taiwan. A series of studies on allergic asthmatic children have been conducted to explore the non-immunological and immunological causes for their augmented production of IgE antibody and to explore the working mechanisms of hyposensitization. The results showed that the patients had multiple defects in their defects in their defense mechanisms, including hyper-permeability of mucosae, hyperreactivity of target organs, defective phagocyte functions, and deficient helper and suppressor T-cell functions. Hyposensitization was able to partially correct the immunological aberrations. More over, a newly found lymphokine termed "T-cell growth factor" or "interleukin 2" may be used not only as an indicator for the initiation and termination of hyposensitization, but may also provide a promising tool for the treatment of allergic diseases in the future because of its capability of enhancing and expanding allergen-specific suppressor T cells.