[Show abstract][Hide abstract] ABSTRACT: Advances in medical science have enabled many children with chronic diseases to survive to adulthood. The transition of adult patients with childhood-onset chronic diseases from pediatric to adult healthcare systems has received attention in Europe and the United States. We conducted a questionnaire survey among 41 pediatricians at pediatric hospitals and 24 nurses specializing in adolescent care to compare the perception of transition of care from pediatric to adult healthcare services for such patients.
Three-fourths of the pediatricians and all of the nurses reported that transition programs were necessary. A higher proportion of the nurses realized the necessity of transition and had already developed such programs. Both pediatricians and nurses reported that a network covering the transition from pediatric to adult healthcare services has not been established to date.
It has been suggested that spreading the importance of a transition program among pediatricians and developing a pediatric-adult healthcare network would contribute to the biopsychosocial well-being of adult patients with childhood-onset chronic disease.
[Show abstract][Hide abstract] ABSTRACT: Magnesium is a critical cofactor in numerous enzymatic reactions. Diabetic patients and obese subjects are often reported to have intracellular magnesium ([Mg(2+)](i)) deficiency. We studied the change of [Mg(2+)](i) in obese children and children with type 2 diabetes mellitus (DM2) after educational intervention or treatment.
A total of 25 subjects were included: 13 with simple obesity (10 male, 3 female; mean age 16±8 years, intervention period 1.0±0.6 years), 12 with DM2 (8 male, 4 female; mean age 15±3 years, medication period 1.1±0.7 years), and 16 controls (8 male, 8 female; mean age 17±7 years). By using a fluorescent probe, mag-fura-2, we examined the basal and insulin-stimulated [Mg(2+)](i) of platelets in the blood. Plasma leptin, ghrelin, adiponectin, and resistin levels were determined with the use of enzyme-linked immunosorbent assay (ELISA).
Mean basal [Mg(2+)](i) was lower in the obesity (160±65 μmol/L) and DM2 groups (140±30 μmol/L) compared with the control group (330±28 μmol/L). The elevated [Mg(2+)](i) after insulin stimulation was also lower in these two groups (420±140 μmol/L, and 330±70 μmol/L, respectively) compared with the control group (690±270 μmol/L). In the DM2 group, the basal [Mg(2+)](i) was significantly increased after treatment, while in the obesity group, stimulated [Mg(2+)](i) was increased after intervention.
Platelet [Mg(2+)](i) increased after intervention in children with obesity or DM2.
[Show abstract][Hide abstract] ABSTRACT: The WHIM syndrome is a rare immunological disorder characterized by warts, hypogammaglobulinemia, infections, and myelokathexis. We hypothesized that immunological or genetic mechanisms may link WHIM syndrome and type 1 diabetes. We report that the young girl with WHIM syndrome developed diabetes and transient hypothyroidism. A nonsense mutation (C-->T) truncating the CXC chemokine receptor 4 (CXCR4) C-terminal cytoplasmic tail domain occurred at nucleotide position 1000(R334X) of the CXCR4 gene in one allele of the patient was identified, and the person was diagnosed as having WHIM syndrome. Recent observation suggested that the CXCR4, a G-protein-coupled receptor with a unique ligand, CXCL12, might be involved in the pathogenesis for type 1 diabetes. Taken into consideration the concurrent prevalence of the two disorders and the speculated common pathogenesis associated with the CXCR4, our patient may enable us to understand the genetic damage related to accelerated apoptosis.
[Show abstract][Hide abstract] ABSTRACT: Many epidemiologic studies have disclosed that restricted fetal growth has been associated with an increased risk of insulin resistance in adulthood. We studied the relationship of intracellular magnesium [Mg2+]i in cord blood platelets to adipocytokine and birth size. The subjects were 20 infants with small for gestational age (SGA) and 45 infants with appropriate for gestational age (AGA). By using a fluorescent probe, we examined [Mg2+]i of platelets in the cord blood. Cord plasma insulin, IGF-I, ghrelin, adiponectin, plasminogen activator inhibitor-1 (PAI-1), and leptin levels were determined with the use of ELISA. Mean [Mg2+]i was lower in the SGA than in the AGA groups (p < 0.001). Adiponectin and IGF-I were also lower in the SGA than in the AGA, whereas PAI-1 was higher in the SGA. [Mg2+]i was significantly correlated with birth weight, birth length, and adiponectin. Birth weight was also correlated with cord plasma IGF-I, adiponectin, and leptin. Quantitative insulin sensitivity check index (QUICKI) was lower in the SGA group than in the AGA group. [Mg]i and adiponectin were correlated with QUICKI in all subjects. [Mg]i, as well as leptin and IGF-I, reflect the extent of fetal growth. Decreased [Mg2+]i may be involved in the underlying processes to insulin resistance.
Pediatric Research 12/2007; 62(6):700-3. · 2.67 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: We report the case of a 7-yr-old girl with Turner syndrome, ulcerative colitis (UC) and coarctation of the aorta. The diagnosis of Turner syndrome was made in early infancy (karyotype analysis 45, X). Growth hormone treatment was started at 3 yr and 2 mo of age. From the age of 4 yr and 5 mo, the patient suffered from persistent diarrhea with traces of blood and intermittent abdominal discomfort. As these symptoms gradually deteriorated, she was referred to our clinic at the age of 7 yr for further evaluation. Barium enema showed aphtha and loss of the fine network pattern in the descending colon and rectum. An endoscopic examination showed ulceration, edema, friability, and erythema beginning in the rectum and extending up to the splenic flexure of the descending colon. The histology of the descending colon area showed severe stromal infiltration of inflammatory cells. These endoscopic findings and the histological findings were consistent with UC. Thus, based on these findings, the patient was diagnosed as having UC. Mesalazine therapy was initiated at this time. The patient is currently being treated with mesalazine (1,000 mg/day) and abdominal symptoms and bloody diarrhea have disappeared. GH therapy was not interrupted during the therapy for UC. Retrospectively, growth hormone improved growth velocity (9 cm/year) during the first year of treatment, however from the age of 4 yr, growth velocity decreased (4-5 cm/yr) in spite of the GH treatment.
Patients with Turner syndrome and gastrointestinal symptoms should be investigated for inflammatory bowel diseases. Growth velocity is useful for evaluating the presence of inflammatory bowel diseases and other systemic diseases.
[Show abstract][Hide abstract] ABSTRACT: We followed up a girl with primary aldosteronism for 8 y, which was diagnosed at 6 y of age when she was referred to us for evaluation of heart murmur and growth failure. The diagnosis of bilateral adrenal hyperplasia was made by selective adrenal venous sampling. Following potassium supplement, her retarded growth was corrected dramatically, and she attained a normal adult height. Puberty developed normally and menarche occurred at 12 y of age. Blood pressure was also controlled adequately. Myocardial hypertrophy associated with aortic damage was noted at 13 y of age. Chronic renal failure developed with proteinuria and enlarged renal cysts. CONCLUSION: Serum electrolytes should be included in the evaluation of children with impaired growth. Although primary aldosteronism is a rare occurrence in children, the condition appears to deserve special attention not only from the viewpoint of growth failure and hypokalaemia but from the occurrence of late organ damage to the kidney and heart.
[Show abstract][Hide abstract] ABSTRACT: Magnesium (Mg(2+)) has an important role in insulin action, and insulin stimulates Mg(2+) uptake in insulin-sensitive tissues. Impaired biologic responses to insulin are referred to as insulin resistance. Diabetic patients and obese subjects are reported to have intracellular magnesium ([Mg(2+)](i)) deficiency. Many epidemiologic studies have disclosed that restricted fetal growth has been associated with increased risk of insulin resistance in adult life. We studied the relationship of [Mg(2+)](i) in cord blood platelets to birth weight. The subjects were 19 infants who were small for gestational age (SGA) and 45 who were appropriate for gestational age (AGA). By using a fluorescent probe, mag-fura-2, we examined the basal and insulin-stimulated [Mg(2+)](i) of platelets in the cord blood. Cord plasma insulin-like growth factor-1 (IGF-1)and leptin levels were determined with the use of enzyme-linked immunosorbent assay (ELISA). Birth weight was correlated with cord plasma IGF-1 (P < .001) and leptin (P < .005). Mean basal [Mg(2+)](i), but not plasma Mg(2+), was lower in the SGA than in the AGA group (291 +/- 149 micromol/L v 468 +/- 132 micromol/L, P < .001). The basal [Mg(2+)](i) was significantly correlated with the birth weight (P < .001) as well as birth length (P < .001). At 60 seconds after stimulation with insulin, there was no significant difference in stimulated [Mg(2+)](i) between the SGA and AGA groups. Although the SGA group had low [Mg(2+)](i), the platelets had good potentiality to compensate for low [Mg(2+)](i). [Mg(2+)](i) reflects the extent of fetal growth. Decreased [Mg(2+)](i) in SGA might underlie the initial pathophysiologic events leading to insulin resistance.
[Show abstract][Hide abstract] ABSTRACT: Magnesium, the second most abundant intracellular divalent cation, is a cofactor of many enzymes involved in glucose metabolism. Magnesium has an important role in insulin action, and insulin stimulates magnesium uptake in insulin-sensitive tissues. Impaired biological responses to insulin is referred to as insulin resistance. This review was designed to reach a better understanding of the mechanism involved in the correlation between magnesium and insulin resistance. Intracellular magnesium concentration is low in type 2 diabetes mellitus and in hypertensive patients. In patients with type 2 diabetes an inverse association exists between the plasma magnesium and insulin resistance due to intracellular changes. The suppressed intracellular magnesium concentration may result in defective tyrosine kinase activity and modify insulin sensitivity by influencing receptor activity after binding or by influencing intracellular signaling and processing. Intracellular magnesium deficiency may affect the development of insulin resistance and alter the glucose entry into the cell. CONCLUSIONS: Magnesium is required for both proper glucose utilization and insulin signaling. Metabolic alterations in cellular magnesium, which may play the role of a second messenger for insulin action, contribute to insulin resistance.
Magnesium research: official organ of the International Society for the Development of Research on Magnesium 07/2004; 17(2):126-36. · 1.38 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: We evaluated the usefulness of measurements of the inferior vena cava (IVC) diameters on abdominal echograms as an indicator of changes of venous return in subjects with orthostatic intolerance (OI) induced by simulated microgravity. We performed a standing test and recorded the IVC diameters on abdominal echograms in 12 subjects placed on a 20-day 6 degrees head-down-tilt bed-rest experiment. We found that different patterns of changes in IVC diameter occurred in the standing test on day 10 of the experiment; in five subjects with a marginal decrease in pulse pressure, IVC diameters in the upright position were markedly decreased compared with those in the supine position. In five subjects with feelings of discomfort, the IVC diameters in the upright position distended or did not decrease from those in the supine position. These results suggested that the changes in IVC diameter on the standing test indicated the presence of various types of hemodynamic responses of OI caused by simulated microgravity. In this study, we also evaluated changes in body-water compartments by conducting multifrequency bioelectrical impedance analysis. Longitudinal data analysis showed that the total body-water-to-fat-free mass and extracellular fluid-to-fat-free mass ratios decreased during the experimental period and recovered thereafter, and that the ratio of intracellular fluid to fat-free mass decreased during the experiment. No significant difference in changes in body-water compartments was seen among subjects with different patterns of changes in IVC diameters. Measurement of IVC diameter was useful to estimate hemodynamic changes in subjects with OI.
Journal of Applied Physiology 07/2004; 96(6):2179-86. · 3.48 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: A 10-year-old girl presented left ventricular failure 1 month after the onset of hemolytic uremic syndrome (HUS) caused by an Escherichia coli O157 infection and was diagnosed as having dilated cardiomyopathy. Thallium myocardial scintigraphy showed normal perfusion, but no myocardial uptake of iodine-123-( R, S)-15-( p-iodophenyl)-3-methylpentadecanoic acid ((123)I-BMIPP) was observed. We analyzed the CD36 expression in platelets and monocytes by using a flowcytometer, and she turned out to have CD36 deficiency type I. CONCLUSION: Some patients may be predisposed to myocardial damage in the presence of CD36 deficiency. It is necessary to clarify the etiological significance of the relationship between cardiac impairment and CD36 deficiency in children.
European Journal of Pediatrics 05/2003; 162(4):264-6. · 1.91 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Magnesium (Mg(2+)), the second most abundant intracellular cation, is a critical cofactor in numerous enzymatic reactions. By using a fluorescent probe, mag-fura-2, we examined the basal levels and changes in intracellular Mg(2+)([Mg(2+)](i)) of platelets in diabetic and obese children. Under the basal condition, the platelet [Mg(2+)](i) of both type 1 and type 2 diabetes mellitus (DM) and the obesity groups was significantly lower than the values in the nondiabetic control group (377 +/- 62 micromol/L, 312 +/- 72 micromol/L, 373 +/- 35 micromol/L v 594 +/- 62 micromol/L, respectively, P <.05). [Mg(2+)](i) was increased after the stimulation with 100 microU/mL of insulin. After 60 seconds of insulin stimulation, the value of [Mg(2+)](i) was lower in the type 1 DM group compared with the control group (729 +/- 85 micromol/L v 1,078 +/- 67 micromol/L, P <.005). The increase in percentage over the resting [Mg(2+)](i) was higher in the type 2 DM group than in the stimulated control group (222% +/- 51% v 98% +/- 18 %, P <.05), although the stimulated [Mg(2+)](i) did not reach the level of the control group. The diabetic patients and obese subjects have [Mg(2+)](i) deficiency. In the type 2 DM and obese groups, platelets responded well to insulin. In children under insulin-resistant states, [Mg(2+)](i) decreases before the poor reactivity to insulin occurs in platelets. Decreased [Mg(2+)](i) might underlie the initial pathophysiologic events leading to insulin resistance and abnormality of platelet coagulation.
[Show abstract][Hide abstract] ABSTRACT: Free intracellular Mg2+([Mg2+]i) can potentially integrate the signals from hormones, cellular metabolism and organismal ion homeostasis and affect the activities of ion channel and other effectors. Interest in [Mg2+]i has been heightened by recent reports that small changes in [Mg2+]i in the physiological range can significantly modulate important cellular functions. In addition, a variety of new evidence shows that [Mg2+]i instantaneously changes following stimulation with various biologically active substances. These observations suggest that [Mg2+]i may act as a second messenger.
Magnesium research: official organ of the International Society for the Development of Research on Magnesium 07/2000; 13(2):139-46. · 1.38 Impact Factor