Bone mineral density in precocious puberty.
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Biomedical subjects
Publications and source records attributed to K Yabuta.
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We report the case of a 4-year-old boy with a postoperative hypothalamic tumor, who exhibited unusual water and electrolyte disturbance. This developed as a late manifestation during the course of central diabetes insipidus (DI), which started when the patient was 2 years old. Clinically, hyponatremia and DI appeared alternately within 1 day. The hyponatremia (lowest value Na+ 115 mmol/I) was associated with afebrile convulsions. Assessment of fluid status revealed that the patient had a reduced capacity for arginine vasopressin(AVP) secretion (partial DI), which was not physiologically regulated and which was not concomitantly sufficient to produce maximally concentrated urine and allow the production of maximally diluted urine. This defective osmoreceptor function in association with the previously existing reduced capacity for AVP release seemed to be responsible for the fluid disturbance in the patient. The administration of nasal 1-desamino-8D-arginine vasopressin (DDAVP) only when urine output was increased, instead of regular administration at a fixed time, prevented both worsening of hyponatremia and development of DI.
Kawasaki disease (KD) is an acute febrile illness of early childhood. Although the epidemiology of KD suggests an infectious agent, the cause still remains unknown. Intense immune activation during the acute disease has been well documented. Quantitative determination of soluble CD23 in serum can serve as an index of macrophage/monocyte or B-cell activation. To further characterize the immunological profile in KD, we investigated whether soluble CD23 levels in serum increase during the acute disease. In addition, we compared soluble CD23 levels in 33 patients with acute KD with levels in ten patients each with measles, rubella, infectious mononucleosis, and scarlet fever to determine if marked elevations in soluble CD23 were unique to acute KD. Patients with KD, rubella and infectious mononucleosis, but not patients with measles or scarlet fever, had increased soluble CD23 levels in serum during the acute stage, as compared to age-matched control subjects (P < 0.01). These data suggest infection with Epstein-Barr virus and rubella and acute KD are all characterized by B-cell and macrophage/monocyte activation.
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We performed a retrospective study of infants with unilateral multicystic dysplastic kidney (MCDK) in order to investigate the associated urological abnormalities. We examined the records of seven patients, in five of whom a diagnosis had been made prenatally using ultrasonography. Our investigation focused on the associated urological abnormalities, particularly on the contralateral side, and the results of voiding cystourethrography (VCUG). Four of the seven patients (57%) had urological abnormalities other than MCDK: three exhibited vesicoureteral reflux (VUR) of the contralateral side including one patient who also had an ipsilateral ectopic ureter, and the fourth patient had a ureterocele of the ipsilateral side. The results indicate that contralateral VUR, was the most common abnormality associated with MCDK. Two infants had high-grade VUR and underwent anti-reflux surgery soon after the diagnosis. Both have remained free of recurrent urinary tract infection.
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Fifteen patients were examined before and immediately after balloon angioplasty with a 4.3F, 30 MHz rotational tip intravascular ultrasound system. In 12 (80%) patients, 13 procedures could be analyzed because of sufficient image quality. The lesions for intravascular ultrasound (IVUS) study consisted of pulmonary arterial stenosis in 4 patients, Blalock-Taussig shunt stenosis in 3, coarctation of the aorta in 2, subclavian artery stenosis in 1, renal artery stenosis in 1, and ductus arteriosus in 1. Three distinctive morphologic types were identified: Group I had arterial stretching (3 patients); group IIa had superficial tearing (3); group IIb had deep intimal-medial tearing (5); group III had dissection (2). In the narrowest site, minimal and maximal luminal diameters, luminal area before and after balloon angioplasty were 3.5 +/- 1.8 mm vs 4.5 +/- 2.5 mm, 4.1 +/- 2.1 mm vs 5.4 +/- 3.5 mm, and 49.8 +/- 30.2 mm vs 88.3 +/- 45.2 mm2, respectively. The recoil value of group IIb with appropriate balloon angioplasty was approximately 0.3. IVUS may be an useful modality for evaluating the morphologic mechanism of dilatation after balloon angioplasty.
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)
Anti-allergic drugs such as sodium cromoglycate are known to be effective for the treatment of ulcerative colitis (UC). Because leukotriene (LT) production is considered to play an important role in the pathophysiology of UC, we examined the effect of the anti-allergic drugs, azelastine and tranilast, and the 5-lipoxygenase inhibitor, AA861, on LTB4 and LTC4 production by isolated rat colonic mucosa treated with the calcium ionophore, A23187. Preincubation of colonic mucosa with AA861 (10(-4) M) or azelastine (10(-4) M) significantly reduced AA23187-induced LTB4 (p < 0.01 and p < 0.05, respectively) and LTC4 (p < 0.01) production. Pretreatment with tranilast (10(-3) M) also significantly reduced A23187-induced LTC4 production (p < 0.05). These findings suggest that azelastine and tranilast can inhibit LT production in colonic mucosa and may be beneficial in the treatment of patients with UC.
OBJECTIVE: The objective of our study was to describe the efficacy of corticosteroids for ventricular tachycardia in four children with structurally normal hearts in whom endomyocardial biopsy revealed histologic changes of lymphocytic myocarditis. PATIENTS: The four patients had unexplained ventricular tachycardia. Three dysrhythmias were sustained, and one was inducible by exercise. Patient ages ranged from 4 months to 12 years. Three of the four patients had no symptoms. In two of them, ventricular tachycardia was identified by mass screening for heart disease. Two patients received oral steroids and two received pulse steroid therapy. RESULTS: In all four patients, significant underlying diseases were not found by noninvasive evaluation. Right ventricular endomyocardial biopsy revealed abnormal histologic findings of chronic lymphocytic myocarditis in all patients. Steroid therapy was effective in all four patients, two of whom received methylprednisolone pulse therapy. CONCLUSIONS: We conclude that unexplained ventricular tachycardia may be the only manifestation of clinically silent myocarditis. Steroid therapy should therefore be considered if conventional antiarrhythmic medication is not effective and histologic findings confirm the presence of lymphocytic myocarditis.
Various allergic diseases have been described in patients with systemic lupus erythematosus (SLE). In the present study, we evaluated the prevalence of allergic disease and serum IgE levels in 36 children of 26 mothers with SLE. None of the subjects had any rheumatic symptoms. There was at least one type of allergic disease in 28 (78%) of the 36 children of mothers with SLE, as compared with 30% in Japanese control children (P < 0.01). The prevalence of atopic dermatitis and bronchial asthma was higher in children of mothers with SLE (64% and 28%) than in controls (19% and 9%) (P < 0.01, respectively). Fourteen of the 36 subjects (39%) had higher levels of serum IgE than those of normal range for age-matched healthy Japanese children with no atopic family disease in the immediate family history, and these children had atopic disease. Among the 14 children with high serum-IgE levels, seven had neither immediate nor remote family history of atopic disease, while the others had an immediate family history of atopic disease. We think that genetic factors may influence the presence of allergic disease in children of mothers with SLE, and that the increased serum IgE levels in children of SLE mothers with no allergic family history may be a part of subclinical immunologic abnormalities related to SLE.
OBJECTIVE: To investigate the diagnostic value of anti-neutrophil cytoplasmic antibodies (ANCA) in the diagnosis of ulcerative colitis (UC) in Japanese children. METHODOLOGY: Serum samples from 23 children with UC (17 Japanese, 6 non-Japanese), 27 children with Crohn's disease (CD) (10 Japanese, 17 non-Japanese), 10 children with other diarrhoeal diseases, and 33 normal, healthy adult volunteers were assayed for ANCA using an indirect immunofluorescence technique. RESULTS: ANCA were detected in 6/17 (35%) UC patients and 0/10 (0%) CD patients in Japanese children, and in 3/6 (50%) UC patients and 3/17 (18%) CD patients in non-Japanese children. The difference in prevalence between Japanese and non-Japanese children with UC was not statistically significant (P > 0.05). ANCA were not found in other diarrhoeal patients and volunteers. CONCLUSIONS: Although ANCA have been reported to be useful in the diagnosis of UC in adults, they may be of limited use in Japanese children. This might reflect the heterogeneity of UC.
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