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Biomedical subjects

T Kuribayashi

Publications and source records attributed to T Kuribayashi.

At least 91 records · Page 5Linked to original sources

Hypokalemic myopathy associated with 17 alpha-hydroxylase deficiency: a case report.

We studied a patient with hypokalemic myopathy associated with 17 alpha-hydroxylase deficiency. An 18-year-old high school student, who appeared to be a girl with poorly developed secondary sex characteristics, had generalized muscle weakness. The cause of muscle weakness proved to be hypokalemic myopathy confirmed by clinical findings and muscle biopsy. Endocrinologic study demonstrated 17 alpha-hydroxylase deficiency with male pseudohermaphroditism. The metabolic abnormality of this patient was corrected by the administration of glucocorticoid. The possibility of this rare disease has to be considered when we examine a patient who has hypokalemic myopathy associated with hypogonadism.

Adolescent↗

Successful pregnancy in an acromegalic patient during 2-Br-alpha-ergocryptine (CB-154) therapy.

Foeto-placental function and hormone levels in the maternal, foetal and amniotic compartments have been investigated in an acromegalic woman who was treated with 20 mg bromocriptine/day throughout gestation. Bromocriptine therapy during pregnancy had no effect on urinary oestriol excretion and plasma levels of unconjugated oestriol, progesterone, human placental lactogen, cystine aminopeptidase and heat-stable alkaline phosphatase. The maternal and foetal (cord )blood and amniotic fluid showed prolactin levels of 3.8, 6.5 and 1700 ng/ml, respectively, in the 39th week of pregnancy during bromocriptine therapy. Compared with data from normal pregnancies, these values demonstrated that bromocriptine or an active metabolite crossed the term placenta to suppress prolactin secretion from the foetal pituitary gland and that the prolactin level in amniotic fluid was scarcely affected by the drug. Maternal, foetal and amniotic fluid growth hormone levels were 27.0, 33.0 and 3.8 ng/ml, respectively, thus indicating that dopamine agonists suppress growth hormone only in acromegalic patients, and not in normal babies. Bromocriptine had no effect on thyroid-stimulating hormone concentrations in maternal, foetal and amniotic compartments.

Acromegaly↗

[Two-dimensional echocardiographic evaluation of the right ventricular wall in hypertension and hypertrophic cardiomyopathy].

The thickness and motion of the right ventricular wall (RVW) were studied with two-dimensional echocardiography from a subxiphoid approach in 20 normal subjects (N Group), 86 patients with hypertension (HT Group) and 20 patients with hypertrophic cardiomyopathy (HCM Group). Comparison was made between the patients with and without echocardiographic RVW hypertrophy in each patient group in regard to the thickness of the interventricular septum (IVS) and the left ventricular posterior wall (LVPW) as well as the motion of the RVW and cardiac catheterization data. 1. RVW hypertrophy was visualized in 39.5% of the HT Group and in 40% of the HCM Group. There was no difference in the thickness of RVW between the two groups. 2. A localized hypertrophy and an abnormal motion of RVW were obtained in the HCM Group, but not in the HT Group. 3. RVW hypertrophy in the HT Group was seen in patients with marked IVS hypertrophy, while that in the HCM Group was not related to IVS hypertrophy. 4. Pulmonary arterial pressure remained normal in each group. Therefore, RVW hypertrophy did not appear to be the result of pressure overload. 5. Right atrial mean pressure and right ventricular end-diastolic pressure were elevated only in HCM patients with RVW hypertrophy. We conclude that there is a different mechanism in the mode of production of RVW hypertrophy between patients with hypertension and those with hypertrophic cardiomyopathy.

Cardiac Volume↗

Congenital dyserythropoietic anemia type I: report of a pair of siblings in Japan.

This is the first case of two siblings with congenital dyserythrobpoietic anemia (CDA) type I to be reported from a Japanese family. Both of these cases showed characteristic morphological aberration of erythroid precursors, ineffective erythropoiesis, and negative acidified serum test. The ultrastructural study of erythroblasts also revealed characteristics quite compatible with CDA type I.

Anemia, Dyserythropoietic, Congenital↗