Sachi Fujiwara

Effect of testosterone on bone density and bone metabolism in adolescent male hypogonadism

To assess the influence of gonadal steroid testosterone (T) on bone mineral status in males during puberty, we observed the response of cortical bone density and serum biochemical parameters of bone metabolism to T treatment in 12 adolescent patients with hypogonadotropic hypogonadism (11 with both gonadotropin and growth hormone deficiency and one with isolated gonadotropin deficiency). The 12 patients aged 15 to 21 years (Tanner stage I to II) were divided into two groups: group 1 (n = 6) given T treatment for 2 consecutive years, and group 2 (n = 6) without T treatment for the first year and then with T treatment for the second year. Cortical bone density measured in the radius was less than the age-matched mean value for normal subjects in all 12 patients (groups 1 and 2) at the start of the study. Bone density in group 1 increased significantly during the 2-year T treatment period, but did not increase in group 2 during the first year without T treatment, although an increase was observed during the subsequent year with T treatment. Among circulating biochemical factors such as osteocalcin, parathyroid hormone (PTH), 25-hydroxyvitamin D (25-OHD), and 1,25-dihydroxyvitamin D [1,25-(OH)2D], only osteocalcin showed an increase in response to T treatment in both groups. Levels of insulin-like growth factor-I (IGF-I) remained consistently low and did not change in any patients except one with isolated gonadotropin deficiency.(ABSTRACT TRUNCATED AT 250 WORDS)

Kawasaki disease followed by haemophagocytic syndrome

Sir: Ohga et al. [6] reported the development of haemophagocytic syndrome (HPS) in an infant during the recurrent evolution of Kawasaki disease (KD). We have recently seen a similar course of this rare association. A 1-year-old Japanese girl was admitted to our hospital with a nonexudative conjunctival injection, a polymorphous rash, and fever for 3 days. The perinatal period and past medical history were unremarkable. On admission, blood hyperleucocytosis and increased C-reactive protein (CRP) prompted treatment with antibiotics, acetylsalicyclic acid, and dipyridamole because of the possibility of a streptococcal infection. Nevertheless, fever persisted with redness of the pharynx and cervical adenopathy; when indurated oedema of hands and feet became manifest, the diagnosis of KD was made. She was given 200 mg/kg of intravenous immunoglobulin (IVIG) on days 11, 12, and 13 after admission. Although afebrile up to day 18 (see Table 1) she developed again fever afterwards, up to 40.6°C on day 24, with at the same time recurrence of skin rash, conjunctival injection, and cervical lymphadenopathy. Marked hepatosplenomegaly appeared. Blood demonstrated again hyperleucocytosis and increased levels of CRP Fig. 1 Bone marrow smear of the patient (Wright-Giemsa Stain). Arrow indicates a haemophagocytic histiocyte

Characterization of low-density lipoprotein subclasses in children☆☆☆

Low-density lipoprotein (LDL) particles are heterogeneous in density, size, and chemical composition, and this heterogeneity is thought to be genetically influenced. In the present study, plasma LDL subclasses in 248 children aged 7 to 13 years were analyzed by gradient gel electrophoresis. The prevalence of small dense LDL (SDLDL), a potent atherogenic LDL, was 9.3%, which is lower than that reported in adults. Furthermore, children with this LDL subclass showed increased body fatness and dyslipidemia, including elevated plasma triglyceride and apolipoprotein (apo) B concentrations and decreased plasma high-density lipoprotein (HDL) cholesterol and apo A-I concentrations, compared with children without this phenotype. These findings suggest that in addition to genetic factors, environmental factors that affect these cardiovascular risk factors may also influence expression of the SDLDL subclass.

Liprotein(a) Phenotypes in Japanese Children: A Cohort Study

BACKGROUND Elevated serum lipoprotein(a) [Lp(a)] concentrations have been demonstrated to be associated with cardiovascular diseases due to premature atherosclerosis. However, the association of Lp(a) phenotypes with the development of these diseases remains largely unexplored. METHODS We analyzed the population-based frequencies of serum Lp(a) phenotypes in 269 Japanese children aged 8-13 years in one community. According to the different apolipoprotein(a) [apo(a)] electrophoretic mobilities, Lp(a) was classified into seven single-band and respective double-band phenotypes. Each individual expressed a single (homozygotic) or a double band (heterozygotic). RESULTS The serum Lp(a) concentration frequency distribution was skewed toward lower levels with a mean +/- SD of 15.5 +/- 18.0 mg/dl and a median of 11.0 mg/dl. The Lp(a) phenotype frequencies revealed that the frequency of double-band phenotype expression (55%) was higher than that of single bands (44%) and that the frequency of phenotypes representative of low molecular weight apo(a) was very low (2%). The mean serum Lp(a) concentration of the double-band-expressing subjects was higher than that of subjects with the single-band phenotype (20.1 +/- 19.9 vs. 10.5 +/- 15.9 mg/dl, p < 0.01). CONCLUSIONS These findings of Lp(a) phenotypes in children seemed to differ from those in Japanese adults in another study; contrary to expectation, the predominant Lp(a) phenotypes found in children were those frequently associated with cardiovascular diseases in adults. Thus, it is speculated that children whose Lp(a) phenotypes remain unchanged during the transition to adulthood may show an increased susceptibility to cardiovascular disease, although the nutritional effects on the Lp(a) phenotypes cannot be neglected.

Graves’ disease associated with exophthalmos, cerebral ventricular dilatation and accelerated growth

A report is presented of a girl with Graves’ disease, which was diagnosed at the age of 1.7 years. The mother had no thyroid disease. The patient developed signs of hyperthyroidism shortly before her first birthday, and the most prominent manifestations were accelerated skeletal maturation and linear growth, and dilatation of the brain ventricles. The latter manifestation, which has not been reported previously, was reversible upon normalisation of thyroid function with antithyroid treatment for three years.

Spontaneous pneumothorax and mitral valve prolapse

Sir: Refererring to an earlier letter to this journal [1] we wish to report on an l l year-old boy with ~-thalassaemia major in whom Yersinia enterocolitica mesenteric adenitis was diagnosed by ultrasonography alone. The boy was admitted because of fever (39.5~ vomiting, diarrhoea and pain in the right ilia fossa of 3 days duration. Sonographic examination disclosed multiple hypo-echoic masses in the lower right abdomen which were interpreted as enlarged mesenteric lymph nodes (Fig. 1). Y. enterocolitica infection was suspected and faecal cultures obtained. Therapy with trimethoprime-sulphamethoxazole was started. The diagnosis was confirmed by the isolation of Y. enterocolitica from the stool specimen. We