Successful treatment of virus-associated haemophagocytic syndrome in adults by cyclosporin A supported by granulocyte colony-stimulating factor

Two young adult patients with typical virus-associated haemophagocytic syndrome (VAHS) were treated with cyclosporin A and granulocyte colony-stimulating factor (G-CSF). Clinical symptoms such as high fever and malaise disappeared rapidly with concurrent haematological improvement in both patients. The serum levels of interleukin-6 (IL-6), soluble IL-2 receptor, tumour necrosis factor and macrophage-CSF were all elevated before treatment but that of G-CSF was not. The dramatic effect of cyclosporin A observed implies that it efficiently and rapidly suppresses the cytokine storm caused by dysregulated T cells in VAHS. In addition, G-CSF may promote haematological recovery without syndrome regression. We believe that the combination of cyclosporin A and G-CSF may be effective, at least in selected patients with VAHS. Further studies are required to confirm its role as first-line therapy for adult patients with VAHS.     

Long-Pulse Pulsed Dye Laser Delivered with Compression for Treatment of Facial Lentigines

BACKGROUND AND OBJECTIVE: The 595-nm long-pulsed dye laser (LPDL) has been used for the treatment of vascular lesions, and although it is well absorbed by blood, it is also well absorbed by melanin. To utilize this device for the treatment of facial lentigines, we attached a glass window to the tip of the laser's handpiece, allowing compression of the skin during treatment. This prospective study aims to evaluate the efficacy and complications of using a LPDL delivered with compression for the treatment of facial lentigines in Asian persons. MATERIALS AND METHODS: Fifty-four Asian patients with facial lentigines were enrolled in this study. The laser settings included fluences between 9 and 13 J/cm(2) and a constant pulse duration of 1.5 ms. Cryogen spray cooling was not used. RESULTS: Thirty-eight patients showed excellent results, 14 patients showed good results, and 2 patients showed fair results. Hyperpigmentation was seen in 1 patient. CONCLUSION: LPDL delivered with the compression method is effective in the treatment of facial lentigines in Asian patients, and the side effect profile is minimal. The compression technique allows the traditional "vascular" LPDL to be used for treating a variety of pigmented lesions.     

Focal nodular hyperplasia in an adolescent with glycogen storage disease type I with mesocaval shunt operation in childhood: a case report and review of the literature

As patients with glycogen storage disease type I survive longer, cases with hepatic tumor have been increasingly documented. A 16 year old boy with glycogen storage disease type I was evaluated for multiple liver tumors. He was diagnosed on clinical features at 9 months of age and underwent a mesocaval shunt operation at 5 years of age. The biopsy of one of the masses showed focal nodular hyperplasia. This is uncommon in patients with glycogen storage disease type I, compared to those with adenoma or malignant hepatic tumor. The association of a portacaval shunt with focal nodular hyperplasia is significant compared to other tumors. An environment of high estrogen concentration or sex hormone binding globulin accompanied by shunt operation may cause focal nodular hyperplasia to develop in the liver of patients with glycogen storage disease type I.     

Different kinetics of antibody responses between IgA and IgG classes in nasopharyngeal secretion in infants and children during primary respiratory syncytial virus infection

The secretory antibody responses in 34 infants and children (20 days-17 months old) with lower respiratory tract disease following primary respiratory syncytial virus (RSV) infection were determined using a sensitive tissue culture enzyme linked immunosorbent assay. None of the patients in the acute phase showed IgA antibody responses. In contrast significant IgG antibody responses which were thought to be maternally derived were observed in infants younger than 2 months of age. In the convalescent phase sample, significantly high IgA antibody responses were observed in all patients except one, and there was no significant difference in magnitude of antibody activity between patients younger than 8 months and patients older than 8 months. However, IgG antibody responses in infants younger than 8 months were significantly lower than in subjects 8 to 17 months old. Notably, infants younger than 2 months developed no significant IgG antibody activity in the convalescent phase. These observations suggest that the antibody activity which contributes to recovery from primary infection by RSV in younger infants may be IgA rather than IgG class antibodies. These observations also suggest that the presumptive immunosuppression mediated by maternally derived antibodies may predominantly influence the IgG antibody response rather than the development of local IgA antibody activity.     

Comprehensive treatment of advanced neuroblastoma involving autologous bone marrow transplant

Encouraging results are reported with high-dose chemotherapy and total body irradiation followed by autologous bone marrow transplantation in the treatment of advanced neuroblastoma. However, relapse remains a significant problem. We used high-dose chemotherapy, surgery, intraoperative radiation and an autologous bone marrow transplant treated in vitro to remove tumor cells followed by 13-cis-retinoic acid to treat 36 children with advanced neuroblastoma. This comprehensive treatment appears to improve the survival rate of patients with advanced neuroblastoma, including those with N-myc amplification and bony involvement. The disease-free survival rate was 66% (95% confidence interval, 49–84%) at 3 years. All patients who received 13-cis-retinoic acid developed cheilitis, but no bone marrow depression occurred in these patients. Five patients developed hemolytic uremic syndrome (HUS) post-transplant. This may have been related to the procedure used for total body irradiation. Patients who had their kidneys shielded during this procedure did not develop this syndrome. Patients who received local irradiation at the primary site showed no evidence of relapse in this region, indicating that such therapy may help to prevent a relapse. These data suggest a high rate of 3 year disease-free survival with this treatment strategy. The nonrandomized nature of the study and use of multiple modalities precludes analysis of the specific contribution of each.     

Distinctive portal venographic pattern in patients with sclerotherapy resistant oesophageal varices

Abstract  We performed prophylactic sclerotherapy in 350 patients with 'high risk' oesophageal varices (F2 or F3 with a moderate or severe red colour sign). Of these patients, eight exhibited sclerotherapy resistance (i.e. no significant reduction in the size of varices after five sessions of sclerotherapy). Thus, the prevalence of sclerotherapy resistant varices was 2%. Of 350 patients, 97 underwent haemodynamic investigation before sclerotherapy. This group consisted of seven patients with sclerotherapy resistant varices and 90 patients with non-resistant varices. Portal pressure, assessed by portal venous pressure gradient, was similar in these two groups (21.5±4.8 vs 19.8±5.0 mmHg, respectively; NS). However, the prevalence of the 'pipe-line' form of variceal feeding pattern (a large dilated left gastric vein running up the oesophagus) was higher in patients with resistant varices than in those with non-resistant varices (100 vs 3%, respectively; P <0.01) and the diameter of the left gastric vein was larger in patients with resistant varices than in those with non-resistant varices (12.4±2.0 vs 7.8±2.3 mm, respectively; P <0.01). Moreover, the extravariceal portosystemic shunt was poorly developed in patients with resistant varices compared with non-resistant varices (0 vs 52%, respectively; P <0.05). We conclude that the pipe-line pattern, fed by a large left gastric vein and associated with poorly developed extravariceal portosystemic shunt, is a distinctive portal venographic feature of sclerotherapy resistant varices.     

Moment analysis of hepatic local disposition of allopurinol and oxipurinol: metabolism kinetics from allopurinol to oxipurinol in the rat isolated perfused liver

Abstract— Drug metabolism in the liver was examined by the rat isolated perfused liver using the single-pass bolus-input technique. The test compounds, allopurinol and its metabolite oxipurinol, were independently introduced into the liver from the portal vein, and the concentration profiles in the venous outflow were monitored and kinetically analysed by moment theory. The recovery ratios of allopurinol and oxipurinol after the individual administration of each drug were estimated to be 0·17 (±0·08 s.d.) and 1·03 (± 0·02 s.d.), respectively. The outflow recovery ratio of oxipurinol as the metabolite after allopurinol administration was estimated to be 0·80 (±0·07 s.d.). These results indicate that the combined outflow recovery of the precursor and the metabolite after allopurinol administration is almost 100% in the rat liver.     

Respiratory inhibition after crying in infants

BACKGROUND: Among full-term neonates, the authors discovered infants who showed respiratory inhibition after crying which involved a marked decrease in SpO2. The infants were found to present increased echogenicity or a cyst in a cranial region termed the ganglionic eminence, or to have a subependymal cyst. The authors prospectively examined the relationship between respiratory inhibition after crying and these changes to examine the prevention and treatment of the episode. METHODS: The authors conducted cranial ultrasonography to screen 381 full-term neonates who showed no abnormalities at birth and whose parents requested ultrasonographic screening of the head, followed by polygraphy of infants who showed increased echogenicity or a cyst in ganglionic eminence, or had a subependymal cyst. The authors similarly conducted polygraphy for 50 neonates without cranial ultrasound abnormalities; the former constituted the control group. Respiratory inhibition was defined to be central apnea immediately after crying with a decrease in SpO2 to <60%. RESULTS: Among 381 neonates examined, 104 showed cranial ultrasound abnormalities; 60 of the 104 neonates indicated respiratory inhibition after crying. Oxygenation failed to improve the episode in 17 neonates with severe respiratory inhibition. However, theophylline alleviated the episode, and SpO2 no longer decreased to <60%. Theophylline was discontinued successfully by 6 months after birth, while 50 neonates in the control group showed no respiratory inhibition after crying. CONCLUSION: Respiratory inhibition after crying which involved a marked decrease in SpO2 was observed in full-term neonates who showed no abnormalities after birth. These neonates could be screened by cranial ultrasonography.