Non-deficient Group Research Articles

Potential effects of zinc on information processing in boys with attention deficit hyperactivity disorder

Purpose The aims of the present study were to investigate the relationship between plasma zinc levels and amplitudes and latencies of P1, N2, and P3 in parietal and frontal areas in children with ADHD, and to compare these zinc levels and event-related potentials (ERPs) indices with controls. Methods 28 boys with ADHD were divided into two groups according to plasma zinc levels: low zinc group ( N = 13, zinc level < 80 μg/dL) and zinc non-deficient group ( N = 15, zinc level ≥ 80 μg/dL). ERP indices from parietal and frontal brain regions were recorded in children with ADHD and in 24 normal boys by using an auditory oddball paradigm. Plasma zinc levels were measured by an atomic absorption spectrophotometer. Results The plasma zinc levels were significantly lower in both ADHD groups (means are 65.8 μg/dL in low zinc group and 89.5 μg/dL in zinc non-deficient group) than controls (mean: 107.8 μg/dL; both p values < 0.017). In ADHD compared to controls, the amplitudes of P3 in frontal and parietal regions were significantly lower, and the latency of P3 in parietal region was significantly longer (all p values < 0.017). In low zinc ADHD group compared to zinc non-deficient ADHD group, the latencies of N2 in frontal and parietal region were significantly shorter (all p values < 0.017). In addition, there was a medium but significant positive correlation between plasma zinc levels and amplitude and latency of frontal N2 wave in ADHD. Conclusions These results can suggest that plasma zinc levels might have an effect on information processing in ADHD children, and lower zinc levels seem to affect N2 wave. Since N2 wave changes may reflect a different inhibition process, further studies are warranted to investigate the effect of zinc on inhibitory process in children with ADHD, and in low zinc and non-deficient ADHD groups.

Functional results of unilateral lung volume reduction surgery in alpha1-antitrypsin deficient patients

Lung volume reduction surgery (LVRS) has been shown to improve lung function and exercise tolerance in patients with severe emphysema. Some predictors of poor outcome have been described but the role of alpha1-antitrypsin (alpha1-AT) deficiency is still not well known. The aim of this study was to analyze the results of unilateral LVRS in our center according to the alpha1-AT status. The results of LVRS in 17 deficient patients and 35 nondeficient patients were analyzed at 3-6 months and 1 year after surgery. Compared with baseline, a significant improvement of FEV1, partial pressure in arterial blood (PaO2), dyspnea score and walking distance was observed in the two groups at 3-6 months after surgery and the studied parameters remained significantly improved at 1 year in the nondeficient group. By contrast, PaO2 and walking distance returned towards baseline in the deficient group at 1 year whereas improvement of FEV1 and dyspnea score was persistent. Mean values of FEV, at baseline, 3-6 months, and 1 year were 22 +/- 6%, 29 +/- 11%, and 26 +/- 9% and 28 +/- 12%, 38 +/- 17%, and 40 +/- 17% predicted in the deficient group and in the non-deficient group, respectively. In conclusion, the functional benefit is short-lasting in alpha1-AT deficient patients after unilateral LVRS.

Chronic venous abnormalities in symptomatic and asymptomatic protein C deficiency.

Thrombophilia is a frequent medical condition associated with symptomatic deep vein thrombosis (DVT). Unlike other clinical risk factors associated with DVT, such as surgery, thrombophilia has not been demonstrated to be associated with asymptomatic venous thrombotic events. Our aim was to search for asymptomatic sequelae of DVT in a protein C (PC)-deficient family. We studied 228 individuals from a large kindred with PC deficiency and performed a systematic ultrasound examination. Among the 203 patients without a known history of venous thrombosis we found seven patients with abnormalities indicative of prior asymptomatic thrombosis: six (7.4%) in the PC-deficient group (n = 81) and only one (0.8%) in the non-deficient group (n = 122). The relative risk for these sequelae associated with PC deficiency was 9.0 (95% CI: 1.1-73.7). These data suggest that chronic venous abnormalities are frequently present and that thrombotic events in asymptomatic individuals with familial PC deficiency may be underestimated.

Dental maturity in children of short stature-a two-year longitudinal study of growth hormone substitution

The aim of this investigation was to present the 2-year follow-up results of a longitudinal study examining the influence of growth hormone (GH) substitution on dental maturity in healthy children of short stature (height <2 SD). At baseline, the children were divided into a GH-deficient group and a GH non-deficient group, and comparisons were made with healthy controls (height between -2 SD and 2 SD) and between the short stature groups. The GH-substituted group included 24 children (8 F, 16 M) with a mean chronological age of 12.20 ± 2.40 years, whereas the GH non-substituted group included 19 children (5 F, 14 M) with a mean chronological age of 11.00 ± 2.40 years. The corresponding age- and sex-matched control groups constituted 48 and 36 children, respectively. The mean dental age in the GH-substituted group was 11.60 ± 2.70 years, compared to their healthy controls 12.40 ± 2.60 years (P < 0.05). The dental age for the GH non-substituted children was 10.20 ± 2.60 years compared to their controls 11.90 ± 2.60 years (P < 0.001). GH-substituted children show an acceleration in their dental maturity in contrast to controls, whereas in non-substituted children the acceleration is less pronounced.

Is Plasminogen Deficiency a Thrombotic Risk Factor ? – A Study on 23 Thrombophilic Patients and their Family Members

The role of plasminogen (plg) deficiency in the pathogenesis of venous thromboembolism is debated in the literature. In the present study we evaluated the prevalence of plg deficiency in our thrombophilia patients and aimed to elucidate the thrombosis risk of plg deficiency as a single defect or in combination with other defects, with special focus on APC resistance. The study cohort included 1192 consecutive patients with a history of clinically or objectively diagnosed venous and/or arterial thromboembolism and/or positive family history who were referred to our department for thrombophilia investigation from 02/1988 to 03/1997. All available family members of patients with plg deficiency were tested for plg, APC resistance and other thrombophilic defects that were established in the propositus. 23/1192 propositi were plg-deficient corresponding to an overall prevalence of 1.9%, i.e. 2.2% in patients with venous thrombosis and 1.4% in those with arterial events. Out of the 23 plg-deficient propositi, 8 showed one or multiple additional thrombophilic defects, and in 4 patients relevant circumstantial risk factors were present. Of the 53 available family members, 28 were plg-deficient including 5 with additional APC resistance, and 4 subjects had isolated APC resistance. Ten of the 53 family members had already suffered thromboembolic events, i.e. 5 (18%) in the plg-deficient group and 5 (20%) in the non-deficient group, both groups showing an almost identical median age at the time of investigation (28.9 years and 27.1 years, respectively). Based on our data, plg deficiency is a rare defect in thrombophilic patients and as a single defect it does not seem to be a strong thrombotic risk factor, as 11 of 23 propositi had additional thrombophilic defects or circumstantial risk factors, and in the family members thrombotic events were equally frequent in the plg-deficient and non-deficient subjects.

Dental maturity in children of short stature, with or without growth hormone deficiency

The aim of this investigation was to study dental maturity in healthy prepubertal children of short stature (height<-2 SD), with or without growth hormone (GH) deficiency, compared to healthy controls. The GH-deficient group (GH level<10.0 microg/l) included 29 children (11 female, 18 male) with a mean age of 10.2+/-2.2 years. The GH non-deficient group consisted of 17 children (5 female, 12 male) with a mean age of 8.5+/-2.1 years. All the children were evaluated for serum concentrations of IGF-1, alkaline phosphatase (ALP), triiodthyronin (T3), thyroxine (T4), thyroid-stimulating hormone (TSH), and fasting plasma insulin; height and bone age were also recorded. Dental maturity was determined from panoramic radiographs. The mean difference between the dental and chronological ages was -0.67+/-0.89 years in the GH-deficient group compared to 0.23+/-1.07 years in their controls: in the GH non-deficient group the difference was -0.95+/-0.82 years compared to controls 0.16+/-1.06 years in their controls. Compared to chronological age, both bone and dental age were lower in the GH-deficient and GH non-deficient groups. It is concluded that children of short stature, both GH-deficient and GH non-deficient. exhibit a delayed dental age compared to their chronological age- and sex-matched controls. A multiple stepwise regression analysis showed that the sitting height and GH level were the only significant factors associated with dental maturity.

Frequency of pregnancy-related venous thromboembolism in anticoagulant factor-deficient women: implications for prophylaxis.

It has been reported that women with an inherited deficiency of antithrombin, protein C, or protein S have an increased risk for developing venous thromboembolic disease during pregnancy and the postpartum period. However, because the available data on risk are flawed, it is difficult to define a rational, efficacious, and safe policy about prophylaxis for venous thromboembolism in these women. To determine the frequency of venous thromboembolism during pregnancy and the postpartum period in women with heritable deficiencies of anticoagulant factors. Retrospective cohort study. University outpatient clinics in the Netherlands and Italy. 129 otherwise asymptomatic female family members of patients with a history of venous thromboembolism and an established deficiency of antithrombin, protein C, or protein S. Medical history, with specific attention to episodes of venous thromboembolism and obstetric history, was taken. The anticoagulant factor status of the study participants was determined. If a patient had an episode of venous thromboembolism, subsequent pregnancies in that patient were not analyzed. Of the 129 women who participated in the study, 60 had anticoagulant factor deficiency and 69 did not. In the nondeficient group, 198 pregnancies occurred; 1 of these (0.5%) was complicated by an episode of venous thromboembolism during the postpartum period. In the deficient group, 169 pregnancies occurred; 7 of these (4.1%) were complicated by an episode of venous thromboembolism during the third trimester of pregnancy (2 pregnancies [1.2%]) and the postpartum period (5 pregnancies [3.0%]). The risk for venous thromboembolism was increased eightfold in deficient women compared with nondeficient women (hazard ratio, 8.0 [95% CI 1.2 to 184]). Anticoagulant factor-deficient women have an increased risk for venous thromboembolism during pregnancy and the postpartum period. Although data from an appropriate randomized clinical trial are lacking, the frequency of venous thromboembolism seen in deficient women might justify the use of anticoagulative prophylaxis during the third trimester of pregnancy and the postpartum period.

Recurrent aphthous ulceration: vitamin B1, B2 and B6 status and response to replacement therapy

An evaluation of the thiamine, riboflavin and pyridoxine (vitamin B1, B2 and B6) status of 60 patients with recurrent mouth ulcers was performed. Seventeen patients (28.2%) were found to be deficient in one or more of these vitamins. Replacement therapy of these vitamins was given to a study group of deficient patients and a non-deficient group for one month. At the end of therapy and after a follow-up period of 3 months, only those patients who had a B complex deficiency had a significant sustained clinical improvement in their mouth ulcers. Vitamin B1, B2 and B6 deficiencies should, therefore, be considered as another possible precipitating factor in recurrent aphthous ulceration.

Increased plasma lipid peroxidation in riboflavin-deficient, malaria-infected children

Plasma lipid peroxides were measured as malonyldialdehyde (MDA) by the thiobarbituric acid (TBA) method in 75 children suffering from Plasmodium falciparum malaria. Their riboflavin status was assessed by measuring erythrocyte glutathione reductase activation coefficients (EGRACs), and values greater than 1.40 were regarded as indicating biochemical deficiency. Plasma MDA was higher (p less than 0.001) in patients than in control subjects; the concentrations were 3.65 +/- 0.70 and 1.77 +/- 0.45 mumol/L (means +/- SD), respectively. The riboflavin-deficient group had higher plasma MDA values (3.98 +/- 0.70 mumol/L) than did the nondeficient group (3.30 +/- 0.68 mumol/L, p less than 0.001). Plasma MDA concentrations correlated with EGRACs (r = 0.46, p less than 0.01) in the patients. It is proposed that riboflavin deficiency restricts regeneration of reduced glutathione making the parasitized erythrocytes more vulnerable to destructive lipid peroxidation and increasing plasma lipid hydroperoxides.

Short-term effects of marginal vitamin B deficiencies on immune parameters in healthy young volunteers

Twenty-four healthy, male volunteers (age 20–30 years) participated in a 14-week double blind study to determine the influence of marginal vitamin B-1, B-2 and/or B-6 deficiency on humoral (IgG, IgA, IgM, IgE, IgD) and cellular (total lymphocyte count, OKT3/Cd3, OKT4/Cd4, OKT8/Cd8, OKT9/Cd71, OKT10/Cd38, OKT11/Cd2, Leu1/Cd5, Leu7/Cd57, Leu11/Cd16, IL-2 receptor/Cd25, BA1/Cd24, B4/Cd19 and OKIa/HLA-Dr positive lymphocytes) immunity as well as on acute- phase reactants (C3, C4, C3 pro- activator and α-1-acid glycoprotein, α-1-antitrypsin, haptoglobin, ceruloplasmin, transferrin, prealbumin and C- reactive protein). The diet consisted of conventional, mixed solid foods, deficient in vitamin B-1 (0.43 mg/d), B-2 (0.94 mg/d) and B-6 (0.39 mg/d). The volunteers were either or not supplemented with one or more of the vitamins B-1, B-2 and B-6, thus constituting 8 experimental groups. Although at the end of 11 weeks of depletion statistically significant differences (p<0.05 or p<0.025) between the deficient and non-deficient groups were found for some immune status parameters, we concluded that these differences do not imply physiologically important effects.

Clinical improvements of specific seminal deficiencies via intercourse with a seminal collection device versus masturbation

In the present study of 50 patients, it was determined that those with seminal deficiencies in masturbated samples showed greater improvement in semen parameters with I-SCD use than the nondeficient groups with which they were compared. The use of I-SCD in oligospermia and OTA Syndrome is therefore indicated, both diagnostically and therapeutically. The subjective rating of sexual stimulation elicited when I-SCD was used was far superior than with masturbation. It is believed the success of I-SCD is due, in part, to this greater degree of sexual stimulation, presumably by added loading of the vas deferens prior to ejaculation.

Growth and growth hormone therapy of children treated for leukaemia.

A total of 37 children (24 male, 13 female) who had been treated for leukaemia with chemotherapy and 24 Gy cranial irradiation, and who were disease free for at least 18 months, were commenced on somatrem at a mean of 7.6 years (range, 4.8-12.1 years) after leukaemia diagnosis because of growth rate below the 25th centile for bone age. Peak GH response to provocation (exercise, arginine, insulin hypoglycaemia) was less than 20 milliunits/litre in 27 children (deficient group) and 20 milliunits/litre or more in 10 children (non-deficient group). The mean height SD decrease from diagnosis of leukaemia to commencement of somatrem was 1.98, 86% of the children decreasing by more than 1 SD. Those who were tall for age at leukaemia diagnosis and females were more severely affected. Mean (+/- SD) height velocity increased on somatrem from 2.7 +/- 1.1 to 6.6 +/- 2.2 cm/year during the first 6 months (n = 25), and to 6.0 +/- 1.7 cm/year during the first 12 months (n = 19). No difference in growth response was seen between the sexes or between the deficient and non-deficient groups. Catch-up growth occurred for the first 6 months only. It is concluded that children with a low growth rate after treatment of leukaemia should be considered for GH therapy irrespective of the results of GH provocative tests.

Respiratory symptoms and pulmonary function in 871 miners according to Pi phenotype: a longitudinal study.

Pi phenotype was determined and alpha 1 protease inhibitor (alpha 1 Pi) was measured in 871 iron-ore miners examined twice at five years interval. A questionnaire on respiratory symptoms was administered and lung function tests: spirometry (VC, FEV1.0), measurements of the residual volume (RV/TLC) and of the CO diffusion (FuCO), were carried out by the same medical and technical team, using the same apparatus. There were no differences in age, length of employment or smoking habits among the three Pi Phenotype groups: M (90.03%), MS (7.1%), MZ (2.8%). The prevalence of clinical symptoms at the initial survey and the incidence of symptoms between the 2 surveys were not related to Pi Phenotype groups. However a significantly steeper decline of FEV1.0/VC was observed in the alpha 1 Pi partially deficient groups MS and MZ (-3.9%) compared with the non-deficient group (-1.8%). No significant difference was observed for VC, RV/TLC and FuCO. This finding suggests that an intermediate deficiency of alpha 1 Pi may be related to a slight but statistically significant impairment of a lung function in occupational conditions.

The prolactin response to metoclopramide in growth hormone deficient adolescent males.

The growth hormone (GH) and prolactin (PRL) responses following administration of 10 mg i.v. metoclopramide (MCP) ('Maxolon'--Beecham Pharmaceuticals) have been examined in thirty adolescent males with short stature and delayed puberty, either of whom have isolated growth hormone deficiency. The results demonstrate that peak GH and PRL levels following stimulation were highly significantly correlated when all subjects were analysed together (r = 0.61, P < 0.001) but when the responses of the GH deficient and non-deficient groups of subjects were analysed separately no significant correlation was obtained (P > 0.1), this reflected the fact that the PRL response to MCP was significantly lower in the GH deficient group (P < 0.0002). In addition two boys who demonstrated biochemical GH deficiency prior to puberty only, showed PRL responses similar to boys with normal GH reserves. The results suggest that the PRL response to MCP may be of value in the assessment of stimulation tests of GH reserve, as well as in the selection of patients for treatment with GH.

Profil clinique et fonctionnel pulmonaire d’homozygotes (PiZ) déficitaires en alpha&lt;sub&gt;1&lt;/sub&gt; antitrypsine

A group of 6 males with severe alpha1-antitrypsin deficiency, underwent clinical and pulmonary function evaluation. Findings were compared to those in a group of males with different degrees of airflow obstruction, comparable ages and tobacco consumption, but with normal serum levels of alpha1-antitrypsin. The deficient group was characterized by: (1) a relatively early appearance of symptoms; (2) disturbed lung scans, mostly in the basal zones; (3) radiological evidence, in most cases, of pulmonary emphysema with, in particular, bullae in the lower lung zones; (4) hypoxemia without hypercapnia and a decreased TCO/VA, and (5) a more or less severe reduction of maximal expiratory flows largely, but not exclusively due to a decrease in lung elastic recoil. Clinical and functional parameters did not permit a clear distinction between the deficient and non-deficient groups.