Apparently increased trough levels of tacrolimus caused by acute infantile diarrhea in two infants with biliary atresia after liver transplantation

Two infants with biliary atresia who exhibited three-fold increased trough levels of tacrolimus and required reduced doses during episodes of acute infantile diarrhea within 5 months of liver transplantation are described. The cause of the increase was not explained simply by hemoconcentration as a result of significant loss of extracellular fluid during these episodes. It does highlight an important issue: that of the continuing need to carefully monitor the trough levels of tacrolimus in such infants.     

Acute heart failure and acute renal failure in Kawasaki disease

Acute renal failure and acute heart failure are rare in Kawasaki disease. We experienced two patients with Kawasaki disease who presented acute renal failure and acute heart failure. These two patients gave us an important insight into the understanding of water balance and fluid therapy in Kawasaki disease. One patient showed acute prerenal failure due to fluid exudation from the intravascular to the extravascular space, and subsequent acute heart failure. The other patient showed acute heart failure caused by fluid infusion for the treatment of dehydration. It is suggested that acute renal failure could be caused by a fluid shift from the intravascular to the extravascular space in Kawasaki disease. It is also demonstrated that the reserve of cardiac function could be decreased in patients with Kawasaki disease due to myocarditis even with normal echocardiography and chest X-rays.     

Genetic analysis of hypophosphatasia

A review is presented of the recent advances in: (i) clinical features, (ii) biochemistry and molecular biology of alkaline phosphatase, (iii) genetic defect in hypophosphatasia, and (iv) prenatal diagnosis. Despite the recent progress, the pathogenesis of hypophosphatasia is far from being elucidated. More clinical cases and further characterization of the alkaline phosphatase gene mutations are needed for better understanding of the clinical spectrum of the entity.     

Electron microscopic finding of eccrine sweat gland epithelial cells in a patient with Krabbe disease

A 13 month old boy was found to have severely reduced β-galactocerebrosidase activity suggesting infantile Krabbe disease. Clinically, the patient showed a progressive neurological deterioration with white-matter disease on radiological study. Axillary skin biopsy was performed to support the diagnosis. On electron microscopy, needle-like inclusions, which are the typical finding seen in the cytoplasm of astrocytes and Schwann cells in the classic infantile form, were present in eccrine sweat gland epithelial cells. This method is useful for diagnosis when nerve biopsy and biochemical analysis are not readily available.     

Cytomegalovirus infection during immunosuppressive therapy for diffuse parenchymal lung disease

Background and objective: Cytomegalovirus (CMV) infection is a life‐threatening condition in patients with diffuse parenchymal lung diseases (DPLDs), who are receiving immunosuppressive therapy. The aim of this study was to describe the clinical features of CMV infection and to propose a strategy for managing CMV infection in patients with DPLD who are receiving immunosuppressive therapy. Methods: A retrospective longitudinal observational study was performed on 69 patients with DPLDs (39 with acute/subacute onset, 30 with chronic onset) who were receiving immunosuppressive therapy and were positive for CMV pp65 antigen (CMV‐pp65Ag) in peripheral blood leukocytes (PBLs). Results: Clinical CMV disease and subclinical CMV antigenaemia developed in 23 and 46 patients, respectively. The cut‐off level of CMV‐pp65Ag indicating clinical CMV disease, as determined by receiver operator characteristic curve analysis, was 7.5 cells per 5 × 10⁴ PBLs. Multivariate analysis revealed that early CMV infection was associated with acute/subacute onset of underlying DPLD and with respiratory dysfunction at the commencement of immunosuppressive therapy. Multivariate analysis also suggested that the acute/subacute onset of underlying DPLD, a CMV‐pp65Ag titre of >7.5 cells per 5 × 10⁴ PBLs, and C‐reactive protein levels ≥10 mg/L indicated a poor prognosis. Conclusions: We recommend that CMV‐pp65Ag antigenaemia of >7.5 cells per 5 × 10⁴ PBLs in patients with DPLD should be treated with ganciclovir. Patients with lower levels of CMV‐pp65Ag antigenaemia should be closely monitored or treated with ganciclovir if the clinical findings suggest a poor prognosis.     

Studies of Human Erythrocyte Catalase by Fluorescent Antibody Technic The Distribution of Catalase in Acatalasia and Hypocatalasia

In order to investigate the cellular distribution of catalase in normal,hypocatalasic and acatalasic red blood cells, the fluorescent antibody labellingtechnic was employed. Sensitive anticatalase sera were produced in rabbitsby immunization with purified catalase extracted from human erythrocytes.Specificity against human erythrocyte catalase was confirmed by Ouchterlony’sdouble diffusion method.

The distribution of catalase is fairly homogeneous in normal and hypocatalasic red cells, but in acatalasic cells fluorescence due to the presence ofcatalase was not observed.

By this method the amount of catalase in hypocatalasic red cells was judgedto be between that of normal and acatalasic red cells.

Submitted on October 1, 1968 Accepted on February 13, 1969     

Catheter Ablation of Accessory Atrioventricular Connection between Right Atrial Appendage to Right Ventricle

Accessory AV Connection Between RAA and RV. A 24-year-old woman had experienced frequent attacks of orthodromic AV reciprocating tachycardia. The polarity of the delta waves suggested a right anterior or anterolateral accessory pathway. After ablation at the tricuspid annulus was unsuccessful, earliest retrograde atrial activation was recorded on the floor of the right atrial appendage, 2 cm above the tricuspid ring. Application of radiofrequency en-ergy at this site aholished accessory pathway conduction. This unusual accessory pathway, located between the floor of the right atrial appendage and the right ventricle, was amenable to radiofrequency catheter ablation from within the right atrial appendage.     

A Japanese boy with Young-Simpson syndrome

In 1987 Young and Simpson reported a child with hypothyroidism, a congenital heart disease, severe mental retardation and striking facial dysmorphism, including microcephaly, blepharophimosis, bulbous nose, thin lip, low-set ears and micrognathia. This study presents an 8-month-old boy with virtually identical features to those in Young and Simpson's original case. The patient is a sporadic case and his parents are unrelated and phenotypically normal, hence the family data corresponds to any mode of inheritance. This is the first male case reported in the world and the first in Orientals.