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Biomedical subjects

P Nicolaidou

Publications and source records attributed to P Nicolaidou.

At least 19 recordsLinked to original sources

Low vitamin D status in mother-newborn pairs in Greece.

Adequate vitamin D status during pregnancy is crucial to assure normal fetal skeletal growth and to provide the vitamin D needed for infants' stores. To determine the actual situation in Greece, we evaluated serum 25-hydroxyvitamin D (25[OH]D), calcium (Ca), phosphorus (P), alkaline phosphatase (ALP), parathyroid hormone (PTH), osteocalcin (OC), and calcitonin (CT) concentrations in 123 healthy mother-newborn pairs recruited from a public hospital of the sunny Athenian region. Blood samples were obtained from pregnant women at term and their neonates (cord blood). The study was conducted between June 2003 and May 2004. None of the mothers has been prescribed vitamin D supplements. Maternal 25(OH)D levels (16.4 [11-21.1] ng/mL) were significantly lower than umbilical venous blood concentrations (20.4 [13.9-30.4] ng/mL) (P < 0.001). A strong correlation was observed between maternal and infant 25(OH)D concentrations (r = 0.626, P < 0.001). Twenty-four (19.5%) mothers and 10 (8.1%) neonates had 25(OH)D <10 ng/mL. Pregnant women who delivered in summer and autumn reported higher levels of 25(OH)D (18.9 [12.9-23.3] ng/mL) than those who delivered in winter and spring (14.6 [10.1-18.5] ng/mL) (P = 0.006). Mothers with a darker phototype had lower levels of serum 25(OH) D than those with a fair phototype (P = 0.023). Umbilical venous blood Ca, P, OC, and CT levels were significantly higher than maternal venous blood levels (P < 0.001). PTH umbilical levels were lower than maternal levels (P < 0.001). Apparently, the abundant sunlight exposure in Athens is not sufficient to prevent hypovitaminosis D. Pregnant women should be prescribed vitamin D supplementations, and the scientific community should consider vitamin D supplementation of foods.

Adult↗

Clinical expression of co-inherited Dubin-Johnson and thalassaemic heterozygous states.

Dubin-Johnson syndrome is a worldwide prevalent familial conjugated hyperbilirubinaemia. The identification of multidrug resistance-associated protein 2 provided an understanding of the complex metabolic impairment involved in this syndrome. We report the first family with modified clinical expression of Dubin-Johnson heterozygous state due to genotypic interaction with co-inherited beta and deltabeta thalassaemia, an interaction that has never been described.

Adult↗

The effect of inhaled budesonide on adrenal and growth suppression in asthmatic children.

The present authors evaluated adrenal reserve in asthmatic children on long-term inhaled corticosteroids and whether possible adrenal suppression could be predicted by growth retardation. Low-dose synacthen test (0.5 microg x 1.73 m(-2)) was performed in 72 asthmatic children with a median age of 9.4 (range 4.2-15.7) yrs on long-term treatment (median 18 (range 6-84) months) with low-to-moderate doses (median 363 (range 127-1012) microg x m(-2)) of inhaled budesonide, as well as in 30 controls. Adrenal suppression was considered as a peak serum cortisol <495 nmol x L(-1). The current authors calculated height standard deviation score (HSDS) at the time of testing and height velocity SDS (HVSDS) in the preceding year. Mean HSDS was 0.06+/-1.3 and HVSDS was -0.9+/-2.3. Adrenal suppression was disclosed in 15 asthmatic children (20.8%). There were no differences in HSDS and HVSDS between children with and without adrenal suppression. There was no correlation between peak cortisol response and dose or duration of treatment. However, a positive relationship between HVSDS and duration of treatment was noted. These data suggest that long-term treatment of asthmatic children with low and moderate doses of inhaled budesonide may result in mild adrenal suppression that cannot be predicted by growth deceleration. The negative influence of inhaled corticosteroids on growth becomes less the longer the duration of treatment.

Administration, Inhalation↗

Congenital rickets due to maternal vitamin D deficiency in a sunny island of Greece.

UNLABELLED: A full-term male infant presented with clinical and biochemical findings consistent with the diagnosis of congenital rickets: weak muscle tone, craniotabes, episodes of tremor, hypocalcaemia, elevated serum alkaline phosphatase, secondary hyperparathyroidism, decreased 25-hydroxyvitamin D and normal 1,25-dihydroxyvitamin D serum levels. The mother's history and biochemical findings suggested nutritional vitamin D deficiency. Treatment with calcium and vitamin D resulted in the disappearance of clinical findings of rickets, normalization of the baby's biochemical profile and normal growth. It is surprising that this case occurred in an affluent setting, in the Mediterranean island of Crete, with an abundance of sunlight throughout the year. CONCLUSION: We report this case in order to emphasize the continuing occurrence of congenital rickets even in populations not considered at risk for hypovitaminosis D. A high index of suspicion is required for prompt diagnosis and treatment, thus preventing complications.

Female↗

Plasma and urine elastase alpha-1-proteinase inhibitor levels in neonatal urinary tract infection.

OBJECTIVE: To examine whether plasma or urine elastase alpha(1)-proteinase inhibitor (E-alpha(1)-PI) levels could be used as a diagnostic marker of urinary tract infection (UTI) in neonates. SUBJECTS AND METHODS: Plasma and urine E-alpha(1)-PI levels were measured by immunoassay in 23 neonates with UTI at the time of admission and 72 h after the onset of treatment and in 10 'normal' neonates (i.e. with trivial problems). Additionally E-alpha(1)-PI plasma levels were measured in 15 neonates with septicemia. RESULTS: E-alpha(1)-PI plasma levels did not differ between normal neonates and those with UTI. Urine E-alpha(1)-PI levels were significantly higher in neonates with UTI on admission compared to normal neonates. A significant decrease in urine E-alpha(1)-PI levels was noticed 72 h after the onset of treatment in all but 2 neonates in whom infection persisted. In this study, we have found that the urine E-alpha(1)-PI concentration at a cutoff level of 48 microg/l had a sensitivity of 83%, a specificity of 90%, a positive predictive value of 95% and a negative predictive value of 69% for the diagnosis of neonatal UTI. CONCLUSION: Elevated levels of E-alpha(1)-PI in urine seem to be a useful tool for the diagnosis of UTI in neonates (even in those that have already been started on antibiotics) and possibly a valuable marker for early recognition of treatment failure.

Bacteremia↗

Hypercalciuria in children with febrile convulsions.

BACKGROUND: The purpose of the present study was to investigate whether idiopathic hypercalciuria may be implicated in the pathogenesis of febrile convulsions. METHODS: We studied 38 children (22 boys) with febrile convulsions (mean (+/- SD) age 3.25 +/- 1.09 years) and 45 healthy children (28 boys) of similar age who served as controls. Twenty-four hour urine calcium and phosphate, as well as serum calcium, phosphate, alkaline phosphatase and intact parathyroid hormone (PTH) concentrations were determined. RESULTS: Hypercalciuria (urine Ca >4.0 mg/kg bodyweight per 24 h) was found in nine children with febrile convulsions (23.7%) and in three controls (6.7%). Hypercalciuric children excreted significantly more phosphate in their urine (37.0 +/- 11.6 mg/kg bodyweight per 24 h) than normocalciuric children (18.7 +/- 8.7 mg/kg bodyweight per 24 h) and controls (20.2 +/- 7.6 mg/kg bodyweight per 24 h). They also had higher serum intact PTH concentrations (49.87 +/- 15.36 pg/mL) than normocalciuric (35.39 +/- 15.67 pg/mL) and control children (28.21 +/- 14.00 pg/mL). According to the calcium-loading test, eight of nine children with hypercalciuria had the renal type of the disorder. Furthermore, hypercalciuric children had significantly more convulsive episodes (2.77 +/- 1.98) than normocalciuric children (1.86 +/- 1.24). CONCLUSIONS: Our results suggest that renal hypercalciuria may be implicated in the pathogenesis of febrile convulsions.

Calcium↗

Glomerulopathy with mesangial IgM deposits: long-term follow up of 64 children.

BACKGROUND: The aim of the present study was to investigate to what extent IgM nephropathy in children with minimal change nephrotic syndrome (MCNS) and diffuse mesangial hypercellularity (DMH) evolves to focal segmental glomerulosclerosis (FSGS). METHODS: Tissues from renal biopsies were examined by light microscopy (LM), immunofluorescence (IF) and, in four cases, by electron microscopy (EM). From a total of 352 nephrotic children, 121 had renal biopsy results as steroid dependent or resistant. A diagnostic renal biopsy was also performed in 331 children with non-nephrotic proteinuria and/or hematuria. A second renal biopsy was performed in 16 children whose renal function was impaired during the follow up. The clinical course of IgM-positive children was compared with that of IgM-negative children. RESULTS: Of the 121 nephrotic children with renal biopsy, 85 were MCNS. Twenty were IF positive mainly for IgM, six of whom (30%) presented evolution to FSGS, while of the remaining 65 IF-negative children, only three (4.6%) presented evolution to FSGS. Of the total 331 children with non-nephrotic proteinuria and/or hematuria, 139 were diagnosed as IgA--IgG nephropathy, 44 had positive IF for IgM and 148 were IF negative. Of the 44 children IF positive for IgM, seven (15.9%) presented evolution to FSGS, while none of the 148 IF-negative children presented evolution to FSGS. The follow-up time for all children ranged from 1 to 14 years. CONCLUSIONS: Of IgM nephropathy patients with MCNS and DMH, a significant percentage develop impaired renal function, due to the evolution of FSGS, as revealed by repeat biopsy during long-term follow up.

Adolescent↗

Erythema nodosum in children: a prospective study.

BACKGROUND: The studies of series of children with erythema nodosum (EN) are limited and mostly retrospective. OBJECTIVE: We evaluated the epidemiology, etiology, clinical manifestations, course, and prognosis of EN in children. METHODS: Thirty-five children with EN (17 boys, 18 girls; mean age, 8.79 years) have been studied. Four excluded children proved, on biopsy, to have leukocytoclastic vasculitis (n = 3) or eosinophilic cellulitis (n = 1). RESULTS: In 27 of the 35 children (77%), the etiology of EN was established by laboratory investigations. In 25 children the causative factor of EN was an infectious agent (including beta-hemolytic streptococcus [n = 17], and Mycobacterium tuberculosis [n = 2]), whereas in 2 patients, EN was associated with Crohn's disease in one and Hodgkin's disease in the other. In 8 of the 35 children (23%) the etiology of EN remained undetermined. The mean duration of the rash was 11.5 days. Recurrences were noted in only 2 children (1 episode in 1 child and 3 episodes in the other). CONCLUSION: Currently the most common provoking agent of EN in children in Greece is beta-hemolytic streptococcus. However, Mycobacterium tuberculosis should still be considered as a cause of the disorder. Also, the course of EN is benign and recurrences are exceptional.

Adolescent↗

Hodgkin's disease in a child with sickle cell disease treated with hydroxyurea.

Hydroxyurea (HU) is an oral drug that ameliorates the clinical course of sickle cell anemia by increasing the levels of fetal hemoglobin and decreasing the adhesion of red cells to endothelium. Although HU has minimal short-term toxicity, few data are available about the long-term safety and the potential risk for carcinogenesis or leukemogenesis. An 8-year-old child with sickle cell/beta 0-thalassemia who received HU treatment for painful crises is described. Six months after the initiation of the HU treatment he developed Hodgkin's disease, lymphocyte predominance subtype. Chemotherapy induced a complete remission. After discontinuation of chemotherapy the painful crises recurred and bone marrow transplantation was decided at the age of 12 years. Two years after the bone marrow transplantation, the child is in complete remission without painful crises. Although the authors suggest that the development of Hodgkin's disease is a coexisting event, questions arise about the safety of HU treatment in childhood.

Anemia, Sickle Cell↗

Atrial natriuretic peptide in children with idiopathic hypercalciuria.

We measured plasma atrial natriuretic peptide (ANP) levels in 30 children with idiopathic hypercalciuria (IH) and 19 normal controls (NC). A calcium (Ca) loading test was performed in all patients to determine the type of IH. Subsequently plasma ANP, cAMP and renin activity (PRA), serum total and ionized Ca, intact parathyroid hormone, aldosterone, and 1,25-dihydroxyvitamin D as well as urine Ca, cAMP, and electrolytes were determined in all subjects. The mean (SD) plasma ANP levels were significantly lower in patients with renal hypercalciuria (RH) [21.4 (4.8) pg/ml] than in those with absorptive hypercalciuria (AH) [26.8 (7.6) pg/ml, P<0.05] and NC [27.6 (6.6) pg/ml, P<0.001]. PRA was significantly lower in AH [2.9 (1.3) ng/ml per hour] than in RH patients [7.8 (6.8) ng/ml per hour, P<0.01] and in NC [6.8 (4.6) ng/ml per hour, P<0.005]. Serum aldosterone values were significantly lower in AH [14.5 (11.4) ng/dl] than in RH patients [25.4 (14.1) ng/dl, P<0.05] and in NC [32.6 (20.5), P<0.001]. The lower plasma ANP levels in RH than in AH patients and in NC may be due to Ca depletion. The lower PRA and serum aldosterone levels in AH than in RH patients and in NC may be attributed to Ca excess.

Aldosterone↗

Plasma immunoreactive endothelin levels in children with cystic fibrosis.

UNLABELLED: Plasma immunoreactive endothelin levels were determined in 31 children and adolescents with cystic fibrosis and it was examined whether these levels correlated with the severity of the disease. The study comprised 16 cystic fibrosis patients (mean (SD) age 13.0 (4.9) y) with impaired lung function (Group A), 15 cystic fibrosis patients (11.2 (5.5) y) with unimpaired lung function (Group B) and 28 healthy controls (10.6 (4.3) y) (Group C). The selection and classification of patients into groups was based on criteria including the grade of finger-clubbing, the Brasfield chest radiograph score and spirometric and arterial blood gas values. In all subjects, plasma immunoreactive endothelin, atrial natriuretic peptide, renin, serum aldosterone levels and serum and urine electrolytes were measured. CONCLUSIONS: Plasma endothelin levels were significantly higher in Group A (range 2.5-8.4 pg/ml, median 3.2 pg/ml) than those in Group B (1.3-3.8 pg/ml, median 2.0 pg/ml, p < 0.001) and Group C (1.5-3.5 pg/ml, median 2.5 pg/ml, p < 0.001), whereas they did not differ between groups B and C. They correlated positively with the severity of finger-clubbing, heart rate, arterial blood PCO2, plasma atrial natriuretic peptide levels and serum aldosterone levels and negatively with the arterial blood PO2, forced vital capacity (FVC), forced expiratory volume in 1 sec (FEV1) and the Brasfield chest radiograph score. In multivariate regression analysis PO2 was the only independent factor found to significantly affect plasma endothelin levels. In conclusion, plasma immunoreactive endothelin levels are increased in cystic fibrosis patients with impaired pulmonary function and are related to the severity of the disease.

Adolescent↗

Idiopathic intracranial hypertension in children.

We studied prospectively the etiology, clinical presentation, and outcome of idiopathic intracranial hypertension in 36 patients (20 boys and 16 girls) aged 3.5 months to 14 years. The etiology was identified in 28 (77.7%) patients. The most common predisposing factor was middle-ear infection, followed by obesity. Of the 36 cases seen, 26 presented with the classic picture of headaches, papilledema, and elevated cerebrospinal fluid pressure; 8 children had intracranial hypertension in the absence of papilledema and 2 had fundoscopic evidence of papilledema with normal cerebrospinal fluid pressure initially. In four children resolution of intracranial hypertension occurred with removal of the causative agent or appropriate treatment of the underlying condition. In 8 of 17 patients intracranial hypertension resolved with acetazolamide therapy and in 22 of 24 patients it resolved with corticosteroids in combination with acetazolamide. Subnormal visual acuity, narrowing of visual fields, or both were present on the initial examination in 10 patients; 2 of them, who presented with loss of vision, have permanent visual impairment. Four additional patients presented a transient, mild impairment of visual acuity during treatment. Our study confirms the wide clinical spectrum and the difficulties encountered in diagnosis of idiopathic intracranial hypertension. A wide variety of etiologic associations should be investigated to provide definitive therapy. Loss of visual function is the only serious complication.

Adolescent↗

DNA degradation in the kidney of folic acid-treated guinea pigs.

Previous investigators agree on the increased DNA synthesis and destruction of tissues caused by folic acid (FA) administered parenterally. This study aims to clarify whether DNA degradation due to the destruction of cells and nuclei precedes DNA synthesis following FA administration. Forty guinea pigs were divided into four groups: group 1, contained 10 controls; in group 2, ten animals received intraperitoneally 300 mg/kg of body wt FA; in group 3, ten animals received FA and 12 h later frusemide intramuscularly in a dose of 7 mg/kg body wt; and finally in group 4, ten animals received frusemide as in group 3. FA produced necrosis of the epithelial cells of the convoluted tubules as the detection of the beta-aminoisobutyric acid end product of DNA and thymine catabolism indicated. Frusemide administered in group 3 had a favourable effect on the acute renal failure induced by FA.

Acute Kidney Injury↗

Insulin dependent diabetes mellitus (IDDM) and autoimmune thyroiditis in a boy with a ring chromosome 18: additional evidence of autoimmunity or IDDM gene(s) on chromosome 18.

A 4 year 3 month old boy with insulin dependent diabetes mellitus (IDDM), autoimmune thyroiditis, slight mental retardation, facial dysmorphism, and a de novo ring chromosome 18 (deletion 18q22.3-18qter) is described. This unique association of defects could represent a chance association. Alternatively, the clinical features could be the result of the chromosomal aberration. If so, one could speculate that a gene or genes on chromosome 18 might act as a suppressor or activator of the autoimmune process by itself or in concert with other IDDM loci.

Blood Chemical Analysis↗

Cat scratch disease in Greece.

An indirect fluorescent antibody test for Bartonella henselae, B quintana, and B elizabethae was performed in all 18 children who presented to our paediatric outpatient clinic with cat scratch disease over a six year period. Serum samples were taken on admission, after 15 days, and after six months. Diagnosis was confirmed in 15 patients (83%) and was based on seroconversion or a fourfold change of the antibody titre to B henselae in 12 patients and on a single high titre (> 128) in three patients. Lymphadenopathy was present in all patients, erythema nodosum in one, osteomyelitis in one, hepatitis in one, transverse myelitis in one, and liver or spleen granulomata, or both, in three patients. Cat scratch disease developed in autumn or winter in 12 patients. All had a history of physical contact with a cat. Our study shows that our clinical suspicion was accurate in the diagnosis of cat scratch disease in a high percentage of patients presenting to a hospital and that indirect fluorescent antibody testing for B henselae is a useful diagnostic tool.

Adolescent↗

Family pattern of idiopathic hypercalciuria and its subtypes.

PURPOSE: We determined the mode of inheritance of idiopathic hypercalciuria and its subtypes. MATERIALS AND METHODS: We evaluated 40 children with symptomatic idiopathic hypercalciuria and 129 of their first-degree relatives (80 parents and 49 siblings). In hypercalciuric individuals in families with at least 2 affected members the type of idiopathic hypercalciuria was determined by the calcium loading test. RESULTS: Of the 40 affected children 19 (47.5%) had 1 or more affected first-degree relatives (23 of 80 parents and 2 of 49 siblings). In all 44 affected members of the 19 hypercalciuric families (19 index cases, 23 parents and 2 siblings) the type of idiopathic hypercalciuria was determined (absorptive in 38 and renal in 6). Study of the pedigree of the 19 families showed that idiopathic hypercalciuria appears to be transmitted as an autosomal dominant trait. With only 1 exception the subtype of disease was specific for members of the same family. CONCLUSIONS: Idiopathic hypercalciuria has a familial or sporadic pattern. In the familial pattern an autosomal dominant inheritance is present. The type of the disease is identical in affected members of the same family. The absorptive subtype is more frequent.

Calcium↗

Arthritis and vasculitis during the incubation period of varicella.

Arthritis and vasculitis are very rare complications of varicella occurring almost exclusively after the eruption of the characteristic rash. We report two children, aged three and seven years, who developed vasculitis and polyarthritis (patient No. 1) and arthritis (patient No. 2) 13 and 9 days, respectively, before the onset of their typical varicella. Arthritis and vasculitis so early in the incubation period of varicella have not been described previously. In all previously reported cases the arthritis occurred after or together with the onset of varicella and only in one instance preceded the exanthem by 2 days. Although this may have been an unfortunate coincidence, an association between varicella zoster virus and these manifestations should be seriously considered. Clinicians confronted with such cases must be cautious and search for varicella infection, especially in times of epidemics.

Arthritis↗