[Two children with suspected primary vasculitis of mesenteric vessels--a case report].

We reported 2 children with suspected primary vasculitis of mesenteric vessels. Both children were admitted to our hospital with the complaints of abdominal pain, bloody stool or diarrhea. Laboratory examination simultaneously revealed leukocytosis with dominant neutrophils, positive CRP, and hypoalbuminemia. Although prothrombin time and activated partial thromboplastin time were within normal limits, the increased levels of FDP-E, D-dimer, and von Willebrand factor activity were observed, which suggested the endothelial cell activation and the coagulation/fibrinolysis system activation. Abdominal echography and CT scanning demonstrated the edematous thickening of intestinal or colon walls probably due to the vasculitic permeability changes of mesenteric artery. During the disease courses, skin rash, bleeding tendency, arthritis and proteinuria were not observed, and no autoantibodies including anti-nuclear antibody, anti-DNA antibody, and myeloperoxidase-antineutrophil cytoplasmic antibody, were detected. Taken together, we suspected these children as restricted vasculitis of mesenteric vessels. Intravenous prednisolone was administrated, and the clinical and laboratory abnormalities recovered completely within 2 weeks. Thus, we suggested that the leukocyte counts, CRP, and the determination of von Willebrand factor and coagulation/fibrinolysis study accompanied with X-ray, echography, and CT scanning will be useful for the early diagnosis of vasculitis before the pathologic and irreversible vascular damage are demonstrated.

[Why there were few isolations of tubercle bacilli in young children with pulmonary tuberculosis?].

We retrospectively evaluated the factors influencing the isolation of tubercle bacilli in 51 children under 14 years of age with pulmonary tuberculosis who were admitted to Yokohama City University Hospital from 1975 to 1998. Young children (0-6 years of age) with pulmonary tuberculosis were significantly less positive by smear and culture than elder children (7-14 years of age) with pulmonary tuberculosis. According to the Japanese Society for Tuberculosis classification of finding on chest X-ray film for pulmonary tuberculosis, the culture-positive patients with type II (cavitary lesions) were found in all, the culture-positive patients with type III (non-cavitary lesions) in 39.3%, and the culture-positive patients with type H (hilar and mediastinal lymphadenopathys) in 35.3%. Patients with cavitary lesions (type II) were significantly more positive by smear and culture than patients with non-cavitary lesions (type III + type H). Only 15.8% of the young children with pulmonary tuberculosis had received BCG vaccine and all had non-cavitary lesions (type III and type H). But, 84.6% out of the older children had received BCG vaccine and half had cavitary lesions. Taken together, the result was that there were few isolation of tubercle bacilli in young children with pulmonary tuberculosis because they had non-cavitary tuberculosis without delayed-type hypersensitivity to tubercle bacilli.

[Two infants with classical polyarteritis nodosa but not Kawasaki disease].

We experienced two infants with polyarteritis nodosa (PN). The symptoms started with high fever and skin rash, which were similar to those of Kawasaki disease (KD). However, the involvement of central nervous system and lung, such as distension of large fontanel, stridor and mild disturbance of consciousness, occurred and the systemic vasculitis resulted in hypoalbuminemia and severe generalized edema. They flared up twice or three times during the long clinical courses. Finally, both had multiple giant coronary aneurysms and lung fibrosis. The clinical courses of these patients were different from those of KD in that: (1) the severity of vasculitis, (2) the wide-spread nature of the vasculitis, and (3) the chronic and recurrent clinical course. It is very difficult to distinguish PN from severe KD in the early stage of the diseases especially in infancy. But in the cases intractable to high-dose gamma-globulin therapy and plasma exchange, it is needed to suspect PN and to induce more aggressive immunosuppressive therapy, such as methylprednisolone pulses and cyclophosphamide pulse therapy as soon as possible.

The influence of methicillin-resistant Staphylococcus aureus (MRSA) carriers in a nursery and transmission of MRSA to their households.

We examined two persistent MRSA-carrier nurses in a maternity hospital to elucidate the transmission of methicillin-resistant Staphylococcus aureus (MRSA) from healthcare providers to newborn infants and to the nurses' own families. Genotyping of the MRSA strains was performed by analyzing genomic DNA restriction length polymorphisms from pulsed-field gel electrophoresis (PFGE-RFLPs). The children of these nurses were carrying genotypically identical MRSA strains as their mother. Both MRSA carrier families remained asymptomatic over a two-year follow-up period. Eradication of nasal MRSA carriage from the two nurses resulted in declining MRSA carriage rates among infants in the nursery. Healthcare providers may become transient or persistent MRSA carriers whilst working in hospitals in which MRSA is endemic. They may then become a source of infection for patients as well as their own families. We recommend that healthcare providers should be examined for MRSA if an MRSA epidemic occurs in a hospital. The families of any such carriers should also be examined for MRSA.

[Improvement of the maintenance therapy after methylprednisolone pulse therapy--effect of prednisolone combined with immunosuppressants].

OBJECTIVES: We investigated the effect of the combination therapy of prednisolone (PSL) and immunosuppressants after methylprednisolone pulse therapy. METHODS: A protocol of PSL (15-20 mg/day) and mizoribine (150-200 mg/day) after methylprednisolone (mPSL) pulses was used for 2 years to treat 7 patients (PSL + MZB group). Cyclophosphamide (CYC) pulse therapy was added to the combined therapy in 4 patients with severe lupus nephritis. The total dose of predinisolone, and side effects were compared with those in 6 patients who were treated with PSL (30 mg/kg) alone after mPSL pulse therapy (PSL group). RESULTS: No relapses occurred in the PSL + MZB group, although all of 6 patients relapsed in the PSL Group. The total doses of PSL in the PSL + MZB group was about 70% of the PSL Group. There were two patients with Herpes-Zoster infection and one patient with liver dysfunction as side effects, with no differences in the frequency of side effects between the was groups. CONCLUSIONS: Combination maintenance therapy with prednisolone and immunosuppressants after methylprednisolone pulse therapy was effective in preventing relapse.

[Results of clinical study with epirubicin hydrochloride injectable solution in hepatoma].

A 10-center cooperative clinical study with a new formulation of epirubicin hydrochloride injectable solution (Epirubicin-RTU) was conducted in patients with hepatocellular carcinoma. Epirubicin-RTU 60 mg/m2 was injected into the hepatic artery and a three-week drug-free interval followed. Of 15 patients with hepatocellular carcinoma registered in this study, 14 patients were eligible, and they all completed the entire course. The objective was to investigate the safety of treatment with Epirubicin-RTU in 14 eligible patients. The adverse drug reactions frequently observed in these 14 eligible cases were leukopenia, neutropenia, thrombocytopenia, alopecia, and fever. They were all reversible and tolerable. With these results. Epirubicin-RTU was considered to be a safe pharmaceutical product to inject into the hepatic artery.

99mTc-DTPA-HSA uptake in a case of splenic hamartoma.

Primary splenic tumors are very rare. The authors present a case of splenic hamartoma in which positive 99mTc-DTPA-HSA uptake was recognized with ultrasound, CT, MR and angiographic images. This case showed the need to consider hamartoma as well as hemangioma in the differential diagnosis of 99mTc-DTPA-HSA uptake in splenic tumors.

[Serial computed thermography for Raynaud's phenomenon of patient with progressive systemic sclerosis (PSS)].

We reported a case with progressive systemic sclerosis, a relatively rare disease in childhood. Raynaud's phenomenon (RP) of the patient was serially monitored by computed thermography for 1 year. Although clinically apparent RP manifested only in winter season, thermographical changes were documented through all season, suggesting that RP is a constitutional sign which may emerge in winter by provocation of atmosphere. Thus, we recommend to use the words, "Raynaud's phenomenon" and " Raynaud's symptom", in an appropriate meaning. RP of the patient, however, varied depending on the time determined, which indicated that the cause of RP is not due to the irreversible tissue degenerations, but due to the reversible functional factors. Computed thermography was proved useful for detecting RP objectively, and will be valuable for estimating the effects of medications for RP.

[A case of tuberculous meningitis followed by tuberculoma with pan-hypopituitarism].

We reported a case who suffered from tuberculous meningitis at 10 months of age, and progressed to basal tuberculoma despite intensive drug therapy with isoniazid, rifampin, and streptomycin. Pan-hypopituitaliam due to basal tuberculoma was effectively replaced by the administration of anti-diuretic hormone (DDAVP) and levothyroxine sodium. Basal tuberculoma was finally removed by surgical operation. Histopathological examination of the tuberculoma revealed Mycobacterium tuberculosis and Langhans giant cells. During the 6 years after the operation, her growth rate was found to be retarded, and the administration of human growth hormone was started. Remarkable catch-up growth was demonstrated. We like to emphasize that infantile tuberculosis, mostly a result of intafamilial transmission, may manifest meningitis in the early phase of the disease, and it sometimes progresses to basal tuberculoma unresponsive to anti-mycobacterial drug therapy.

[Coronary arteritis of Kawasaki disease unresponsive to high-dose intravenous gammaglobulin successfully treated with plasmapheresis].

Kawasaki disease (KD) is characterized by marked activation of immune system and generalized vasculitis including coronary arteritis, and the intravenous gammaglobulin therapy is recommended as the first line of the choices. However, in some fulminant cases the coronary arteritis progresses rapidly and extensively in the early phase of the disease despite of the aggressive high-dose gammaglobulin administration. Recent observations indicated that the vasculitis begins at the endothelial cells which are activated by several inflammatory cytokines and are adhered to by cytokine-activated cytotoxic immune cells. Thus, it is important for the prevention of coronary arteritis and the resultant coronary aneurysm to reduce the inflammatory cytokines in the plasma. We reported here a 4-year-old girl with coronary arteritis which began on the 3rd day of the disease. The patient was fulfilled the revised criteria of KD at the hospital admission. As the initial intravenous gammaglobulins (400 mg/kg/day for 3 consecutive days and the subsequent 1 g/kg single intravenous infusion) revealed ineffective, which was judged by echocardiography in the persistence of brightness of the slightly widened coronary artery, and by laboratory data, we conducted therapeutic approach of plasmapheresis (replacement of plasma with 5% albumin in saline) on 8-10th days of KD. Fever was down to normal range soon after the first plasmapheresis, and after the second pheresis CRP was reduced, and finally on the 10th day echocardiography demonstrated marked improvement of inflamed coronary artery. The precise mechanism of the effective plasmapheresis is to be investigated, but it is possible that the removal of inflammatory cytokines from the plasma ceased the subsequent endothelial cell damage and coronary arteritis.(ABSTRACT TRUNCATED AT 250 WORDS)

[Clinical and cytological features of acute myelogenous leukemia with 8; 21 chromosome translocation].

Eight cases of acute myelogenous leukemia with (8; 21) translocation were reported. As recently reported, they showed following features: M2 morphology in FAB classification (all 8 patients), abnormal granulocyte maturation, i.e. large granules and pseudo Pelger-Huet forms (5), Auer rods (8), occasional eosinophilia (2), frequent loss of one sex chromosome (5), the low neutrophil alkaline phosphatase activity (5), and tumor formation (one). Both CD13 and CD33 antigens were expressed on smaller number of leukemic cells than the other AML (M2) cells, whereas CD34 and HLA-DR antigens were expressed on higher number of cells. Interestingly CD19 antigen was detected on a small to large population of tumor cells from four out of six patients. Despite the high remission rate, many of them relapsed within one year. More intensive postinduction and maintenance therapy should be considered for those patients.

Involvement of bcl-2 gene in Japanese follicular lymphoma.

A t(14;18) (q32;q21) chromosome translocation is closely associated with the follicular lymphoma, which is prevalent in the United States, and the t(14;18) causes the juxtaposition of a bcl-2 gene on chromosome 18 with an immunoglobulin heavy-chain gene locus on chromosome 14. Genomic DNAs from 30 Japanese patients with follicular lymphoma were examined for the molecular features by Southern blot hybridization. Using probe b for the major breakpoint cluster region of a bcl-2 gene, the rearrangements were detected in eight patients. Six of the eight patients had breakpoints located within the major breakpoint region, while two had breakpoints outside this cluster region but within the region of the 7.5-kb SstI fragment containing the probe b sequence. In two patients, pFL-2 probe detected the bcl-2 gene rearrangements that occurred near or within the minor breakpoint cluster region. These ten patients had a rearranged JH-containing fragment that migrated with the rearranged bcl-2 fragment. In the other 20 patients, these two chromosome 18-specific DNA probes did not detect the bcl-2 rearrangements. Compared with studies performed in the United States, the statistical analysis indicates a significant difference in frequency of the bcl-2 gene rearrangements near or within the major breakpoint cluster region (P = 0.0027) and the minor breakpoint cluster region (P = 0.029). However, the distribution difference of these events was not significant.

Significance of extra 18q- chromosome in Japanese t(14;18)-positive lymphoma.

Karyotype evolution of t(14;18)-positive lymphoma was studied in 13 Japanese patients. The extra 18q- chromosome, found in six of ten patients with complex karyotypes, was the most common change subsequent to a t(14;18)(q32;q21) chromosome translocation. The additional change was interpreted as being a duplication of an 18q- derived from a t(14;18). The six patients had transformed histology of follicular small cleaved cell lymphoma or diffuse large cell lymphoma, and five of them had extranodal expansion associated with a poor prognosis. These findings indicate that the extra 18q-, together with other chromosome abnormalities, is closely associated with the advanced grade disease of t(14;18)-positive lymphoma, and the extra chromosome is evolutionally comparable with the second Philadelphia (Ph1) chromosome often found in the blastic phase of chronic myelocytic leukemia carrying a t(9;22)(q34;q11). In addition, since the extra 18q- is rarely found in American patients with t(14;18)-positive lymphoma, there appears to be a difference in the karyotype evolution between Japanese and American patients.

[Pharmacokinetics of arbekacin in dogs. I. Serum concentration and urinary excretion].

A new aminoglycoside antibiotic, arbekacin (HBK) was intramuscularly and intravenously administered to dogs in order to study its pharmacokinetics in comparison to amikacin (AMK). The results obtained are summarized as follows. Serum concentrations of HBK were well correlated with dose levels. The dose-serum concentration relationship with HBK was similar to other aminoglycoside antibiotics. Biological half-lives of HBK and AMK were both about 1 hour in dogs. This was also similar to other aminoglycoside antibiotics. There was no significant difference in peak serum concentrations between 1 hour intravenous infusion and intramuscular injection of HBK at 2 mg/kg in dogs. Repetitive administration of HBK to dogs at 2 mg/kg twice a day for 14 days did not affect its serum concentration and biological half-life. Urinary excretion of HBK in dogs in 24 hours after administration accounted for about 80-90%.

[Pharmacokinetics of arbekacin in dogs. II. The distribution of arbekacin in tissues].

A new aminoglycoside antibiotic, arbekacin (HBK) was intramuscularly and intravenously administered to Beagle dogs at a dose of 10 mg/kg to study its distribution into various tissues. The results obtained are summarized as follows. Biological half-lives of HBK in serum after intramuscular and intravenous injection were 1.15 hours and 1.00 hour after administration, respectively. These half-lives were similar to the results obtained in previous studies. Maximum concentrations of HBK in tissues and biological fluids after intramuscular injection were the highest in kidney followed by vagina, serum, urinary bladder, uterus, ovarium, lung, parotid gland, trachea, tonsil, gall bladder, pericardiac fluid, thymus, muscle, heart, liver, mandibular gland, bile, aqueous humor, pancreas, cerebrospinal fluid and brain, in this order. Maximum concentrations were found at 4 hours in trachea, aqueous humor and cerebrospinal fluid, and at 2 hours in pericardiac fluid. In other tissues and biological fluids, they were obtained at 0.5-1 hour. Maximum concentrations in tissues and biological fluids after 1 hour intravenous injection were the highest in kidney followed by serum, ovarium, vagina, lung, parotid gland, urinary bladder, uterus, spleen, thymus, pericardiac fluid, gall bladder, trachea, tonsil, heart, liver, pancreas, muscle, mandibular gland, bile, aqueous humor, cerebrospinal fluid and brain in this order. Maximum concentrations were found at 4 hours in trachea, cerebrospinal fluid and bile, and at 2 hours in pericardiac fluid. In other tissues and biological fluids, they were obtained at the end of the infusion. Maximum concentrations in these biological fluids after both intramuscular and intravenous injection were similar to serum concentrations except kidney, trachea, and other biological fluids.(ABSTRACT TRUNCATED AT 250 WORDS)