An immunohistochemical study of placental syncytiotrophoblasts in neonatal hemochromatosis.

INTRODUCTION: Neonatal hemochromatosis (NH) is a rare neonatal disorder that results in liver cirrhosis with hemosiderin deposition in the liver and other organs, similarly to hereditary hemochromatosis. Excess iron is transferred from the mother to fetus through the placenta in NH. We examined the expression of iron metabolism-related substances in placental syncytiotrophoblasts (STB) by immunostaining to clarify how the transfer of iron through STB increases in NH. METHODS: Immunostaining was performed using formalin-fixed, paraffin-embedded sections of placentae from three NH cases, four gestational age-matched controls, and, depending on the antibody examined, five to seven full-term controls. The reactivity of immunostaining was assessed by averages of scores assigned by 3 researchers. RESULTS: On the microvillar surface of STB, the reactions of the antibodies against transferrin receptor 1 (TFR1), transferrin, ferritin, hepcidin, ferroportin, divalent metal transporter-1 (DMT1), hephaestin, and HFE were stronger in NH than in controls. In the cytoplasm, the reactions of antibodies against TFR1, transferrin, ferritin, hepcidin, DMT1, hephaestin, HFE, and ZIP 14 were stronger in NH than in gestational age-matched controls. Among these reactions, those of anti-TFR1 antibody on the surface of STB in NH was especially marked. DISCUSSION: In the placenta of NH, increases in expressions of TFR1, transferrin, and ferritin of which those of TFR1 were especially marked, reflect increased iron influx from the mother to fetus. The hepcidin observed on the surface and in the cytoplasm of STB of NH is suggested to be from the mother, possibly to compensate for the decreased fetal liver-derived hepcidin.

[Importance of anesthetic management for the patients with systolic anterior motion after mitral valve plasty].

Systolic anterior motion (SAM) after mitral valve plasty (MVP) occurs at an incidence of 1 to 4%. The management is related to this condition. Three patients developed SAM just after MVP. In the first patient, a 51-year-old man, volume loading and methoxamine were employed, which elevated arterial pressure, but led to a severer catastrophe. This suggested the indication of mitral valve replacement to the surgeons. The second patient, a 75-year-old woman, underwent re-annuloplasty after the first MVP because of incomplete correction. Before the separation from the second CPB, continuous dopamine infusion was started because we feared that the long CPB time would have impaired LV function. The TEE showed the catastrophe, but it disappeared just after the discontinuation of the dopamine infusion and the administration of propranolol and norepinephrine. The last patient was a 72-year-old woman. For the catastrophe, propranolol was given and an immediate improvement followed with TEE showing no evidence of the catastrophe. The goal for anesthetic management of the catastrophe after MVP is to decrease the hyperdynamic ventricular contraction. We managed SAM by volume loading and discontinuation of beta-stimulants and/or administration of beta-blockers.