A clinical and pathological study of congenital biliary atresia.
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Biomedical subjects
Publications and source records attributed to T Miyano.
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Hepatic portoenterostomy (Kasai operation) for the patient with biliary atresia (BA) can restore the bile flow in approximately 80% of children operated on before 60 days of life [1]. However, in terms of long-term survival, according to a recent nationwide survey among the major pediatric centers in Japan, only 325 of 2013 patients had more than 10 years' survival, and only 157 patients (7.8%) remained jaundice-free with normal liver function [2]. About 20% of BA cases without jaundice are generally able to survive for long periods; and most of those patients have portal hypertension or abnormal liver function [3-5]. As the results of liver transplantation have improved, controversy has arisen over the optimal care of these children [4, 6, 7]. Some investigators have claimed that transplantation is the favored primary therapy for most patients with BA [8]. We are thus at a turning point concerning the primary therapy of BA, which makes it necessary to determine the exact indications for the Kasai portoenterostomy and the timing of liver transplantation. This paper describes our strategy for the optimal treatment of BA patients based on our 117 patients who have had various form of portoenterostomy.
In order to clarify the pathogenesis of rickets in preoperative patients with extrahepatic biliary atresia, we evaluated baseline serum 25-OHD and 1,25(OH)2D levels and correlated serum 25-OHD levels with increase in age and season of birth in 16 preoperative patients. Further, parenteral vitamin D2 tolerance tests were performed in 5 cases. Serum 25-OHD and 1,25(OH)2D levels were significantly lower than those in 15 normal controls. There was a negative correlation between the serum 25-OHD levels and increase in age. The patients born during the winter had lower serum 25-OHD concentrations than those born in summer. The mean value of increased 25-OHD levels after the parenteral vitamin D2 tolerance tests did not differ from that of 6 controls. Since there was no impairment of vitamin D 25-hydroxylation, the reduction in serum 25-OHD may therefore be mainly due to disturbed intestinal vitamin D absorption. It was also concluded that season of birth and increase in age are pathogenic factors in the etiology of rickets in preoperative patients with extrahepatic biliary atresia.
We report a case of the papillonodular type of cystic partially differentiated nephroblastoma (CPDN), an extremely rare renal neoplasm that occurs in newborns and infants. The papillonodular type of CPDN is a variant of the conventional form of CPDN. MRI clearly demonstrated the gross pathologic features, distinguishing it from other renal multilocular cystic tumors.
This is a case report of immediate acute pulmonary edema following the intravenous administration of Stronger Neo-Minophagen C (glycyrrhizin) and Chlor-Trimeton (chlorpheniramine maleate). The patient was a 15-year-old Japanese boy who had a previous history of surgery for right testicular tumor and adverse reactions to contrast media. The patient complained of severe headache, nausea, and vomiting just before the end of intravenous administration of these drugs, which were being given to prevent an adverse reaction to contrast enhanced CT. The symptoms disappeared within a few minutes, but chest CT examination performed immediately after the onset of the adverse reaction showed ill-defined consolidations with air bronchogram, especially in the anterior portion of both lungs. One day later, the abnormalities coalesced and poorly marginated patchy opacities developed. A week later, the abnormal densities disappeared. CT findings suggested acute pulmonary edema, especially in the anterior portion of both lungs. Thus CT examination was useful to detect focal pulmonary edema even in a patient with no particular respiratory symptom.