Successful therapy with tonsillectomy plus pulse therapy for the relapse of pediatric IgA nephropathy treated with multi-drugs combination therapy.

Immunoglobulin A nephropathy (IgAN) is the most common form of chronic glomerulonephritis worldwide.　In Japan, the treatment for use as an initial therapy was established in Guidelines for the Treatment of Childhood IgA nephropathy; however, no rescue therapy for recurrent or steroid-resistant pediatric IgAN was established.　We report here a 15-year-old boy with severe IgAN, who was treated with combination therapy involving prednisolone, mizoribine, warfarin, and dilazep dihydrochloride for 2 years.　The response to the combination therapy was good and both proteinuria and hematuria disappeared.　The pathological findings at the second renal biopsy were improved and PSL was discontinued.　However, due to nonadherence to the treatment regimen and tonsillitis, macrohematuria and an increase of proteinuria were again observed and the pathological findings at the third renal biopsy showed clear deterioration.　The patient was, therefore, diagnosed with recurrent IgAN.　Tonsillectomy plus methylprednisolone pulse therapy (TMP) was performed as a rescue therapy for the recurrence of severe IgAN.　Both the proteinuria or hematuria subsequently disappeared, and no proteinuria or hematuria has been observed and kidney function has remained normal during a 5-year follow-up.　The patient experienced no severe side effects associated with the drug regimens.　In conclusion, our case suggests that TMP may be an effective and useful rescue therapy for recurrent IgAN after multi-drug combination therapy.

Efficacy of recombinant human soluble thrombomodulin for childhood hemolytic uremic syndrome.

Recombinant human soluble thrombomodulin (rhTM) is a promising therapeutic natural anticoagulant and is used clinically for the treatment of disseminated intravascular coagulation (DIC). Herein is reported the cases of two HUS children treated with rhTM. The patients were diagnosed as having typical HUS on the basis of thrombocytopenia, hemolytic anemia, acute renal failure, and the detection Escherichia coli 0157. I.v. rhTM was started as an anti-coagulant drug. At 2 days after the first treatment in both patients, fibrin/fibrinogen degradation products and d-dimer levels were significantly decreased, and there was a subsequent slight improvement in thrombocytopenia, and a decrease in serum lactate dehydrogenase level. Urinary protein excretion gradually diminished and a decrease in serum creatinine level was observed. The patients did not require dialysis therapy. The present results suggest that rhTM may be a safe and effective treatment for DIC complicated with HUS in children.

[Spinal anesthesia for cesarean section in a patient with multiple sclerosis].

Spinal anesthesia is thought to be contraindicated for patients with multiple sclerosis (MS). We describe the case of a patient with MS who was administered spinal anesthesia for cesarean section. A 29-year-old woman (weight 55.8 kg, height 154 cm) with MS underwent an urgent cesarean section in the 39th week of her pregnancy for fetal malpresentation. Although the patient had experienced repeated relapses of MS thrice since she was 19, she had remained in remission since the age of 27, and did not have significant neurological disability. A 27 G needle (pencil type) was used for arachnoid puncture, and 0.5% hyperbaric bupivacaine (2.0 ml) was administered to the subarachnoid space at the L3-4 interspace. After confirming that the sensory blockade after spinal anesthesia had spread to T4, cesarean section was performed. For the subsequent 19 months, no remission of MS was recognized. Thus, spinal anesthesia does not seem to be contraindicated for patients with MS in remission state.

Development of common variable immunodeficiency in IgA- and IgG2-deficient patients with systemic lupus erythematosus.

BACKGROUND: There have been few reports on children who developed common variable immunodeficiency (CVID) in association with immunoglobulin A (IgA) and IgG2 deficiencies and systemic lupus erythematosus (SLE). CASE-DIAGNOSIS/TREATMENT: Our patient experienced nephrotic syndrome and acute respiratory distress syndrome (ARDS) caused by influenza A/H1N1 virus infection at 5 years of age. A diagnosis of IgA and IgG2 deficiency and SLE was made on the basis of severe proteinuria, hematuria, hypocomplementemia, high anti-DNA antibody and antinuclear antibody (ANA) titers, and malar rash. However, these clinical signs and symptoms and laboratory features disappeared after the administration of methylprednisolone pulse therapy and prednisolone. For the 5 years following the initial treatment for SLE, the patient experienced a number of infections and had a low serum total IgG level; she was eventually diagnosed with CVID. The administration of intravenous immunoglobulin (IVIG) was required to prevent subsequent infections, and no relapse of SLE was observed. CONCLUSION: We report the development of CVID in an IgA- and IgG2-deficient patient with SLE on the basis of multiple episodes of infection. To prevent the development of CVID in IgA- and IgG2-deficient patients with SLE, it is important to prevent immune dysregulation by the avoidance of infections through the use of IVIG therapy.

Successful therapy with tonsillectomy for severe ISKDC grade VI Henoch-Schönlein purpura nephritis and persistent nephrotic syndrome.

Henoch-Schönlein purpura (HSP) is a systemic disorder characterized by leukocytoclastic vasculitis involving the capillaries and the deposition of IgA immune complexes. Renal involvement is the principal cause of morbidity and mortality in children with HSP. We report here a 13-year-old girl with Henoch-Schönlein purpura nephritis (HSPN) of International Study of Kidney Disease in Children (ISKDC) grade VI and persistent nephrotic syndrome despite receiving conventional therapy, such as prednisolone, methylprednisolone and urokinase pulse therapy and plasmapheresis (PP). The patient was treated with tonsillectomy, which subsequently decreased proteinuria, induced the disappearance of microscopic hematuria, and improved renal pathological findings. A regimen of methylprednisolone and urokinase pulse therapy plus PP with tonsillectomy may be an effective and useful therapy for some children with severe HSPN children of ISKDC grade VI and persistent nephrotic syndrome.

Mechanism of growth inhibitory effect of cape aloe extract in ehrlich ascites tumor cells.

Cape aloe (Aloe ferox Miller) has been a herb well known for its cathartic properties and has also been used popularly as a health drink (juice, tea and tonic) in the United States and in Europe. Cape aloe extract also has been reported to possess several pharmacological effects, such as anti-inflammatory, anti-bacterial, anti-fungal and protective effect against liver injury. However, the investigations on an anti-tumor activity in cape aloe extract are very few and subsequent mechanisms have not been well elucidated. In this study, we examined the effect of the selective growth inhibitory activity of cape aloe extract and found that the cape aloe extract, especially the dichloromethane (CH(2)Cl(2)) extract, caused a dose-dependent growth inhibitory effect in Ehrlich ascites tumor cells (EATC), but not in mouse embryo fibroblast (NIH3T3) cells, which was used as a normal cell model. Furthermore, the CH(2)Cl(2) extract caused an accumulation of cells in the G1 phase and a decrease of cells in the S and G2/M phase of the cell cycle and inhibited DNA synthesis in a dose-dependent manner. In addition, other results suggest that cell cycle arrest and inhibition of proliferation in EATC by the CH(2)Cl(2 )extract are associated with decreased retinoblastoma protein (Rb) phosphorylation.