A girl with hyperglycinemia of nonketotic type was presented. The liver biopsied from the patient was studied for glycine metabolism. It was found that the yield of 14C02 from glycine-l-14C and the rate of 11C incorporation into serine from glycine-1 -14 IC as well as glycine-2 14C were extremely low in the patient's liver than in control livers, while the patient's liver showed normal activities of serine-hydroxymethylase and serine-dehydratase.hese findings indicate that the primary lesion of hyperglycinemia of nonketotic type is a defect in the glycine cleavage reaction which gives rise to the formation of CO2, methylene-tetrahydrofolate and ammonia from glycine.

Increasing experience was associated with better outcomes, but to a lesser degree than in previous reports. Our findings suggested that the risks associated with the learning curve process were appropriately mitigated.

The ventricular performance is dependent on the drainage effect of rotary blood pumps (RBPs) and the performance of RBPs is affected by the ventricular pulsation. In this study, the interaction between the ventricle and RBPs was examined using the pressure-volume (P-V) diagram of the ventricle and dynamic head pressure-bypass flow (H-Q) curves (H, head pressure: arterial pressure minus ventricular pressure vs. Q, bypass flow) of the RBPs. We first investigated the relationships in a mock loop with a passive fill ventricle, followed by validation in ex vivo animal experiments. An apical drainage cannula with a micro-pressure sensor was especially fabricated to obtain ventricular pressure, while three pairs of ultrasonic crystals placed on the heart wall were used to derive ventricular volume. The mock loop-configured ventricular apical-descending aorta bypass revealed that the external work of the ventricle expressed by the area inside the P-V diagrams (EW(Heart) ) correlated strongly with the area inside dynamic H-Q curves (EW(VAD)), with the coefficients of correlation being R² = 0.869 ∼ 0.961. The results in the mock loop were verified in the ex vivo studies using three Shiba goats (10-25 kg in body weight), showing the correlation coefficients of R² = 0.802 ∼ 0.817. The linear regression analysis indicated that the increase in the bypass flow reduced pulsatility in the ventricle expressed in EW(Heart) as well as in EW(VAD) . Experimental results, both mock loop and animal studies, showed that the interaction between cardiac external work and H-Q performance of RBPs can be expressed by the relationships "EW(Heart) versus EW(VAD) ." The pulsatile nature of the native heart can be expressed in the area underneath the H-Q curves of RBPs EW(VAD) during left heart bypass indicating the status of the level of assistance by RBPs and the native heart function.

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Defective transport of imino acids and glycine in the intestine was demonstrated in a third case of prolinuria, basing upon the increased fecal excretion of imino acids and glycine after the oral loading with these amino acids.In preceding papers of ours,1,2 two patients were reported of 'prolinuria', which was characterized by a defect in renal tubular transport of proline and