Salivary osmolality and hydration status in children with cerebral palsy.

BACKGROUND: Unstimulated whole salivary parameters have been identified as potential markers of hydration status. Reduced salivary flow rate and increased salivary osmolality have been shown to be useful to identify dehydration, even when minimal loss of body water occurs. This study aimed to evaluate whether unstimulated salivary flow rate and salivary osmolality from individuals with cerebral palsy correlate with plasma and urine osmolality. METHODS: Thirty-five male and female children, aged 9-13 years old, diagnosed with cerebral palsy were compared to 27 nondisabled children (10-12 years old). Unstimulated whole saliva was collected under slight suction and salivary flow rate (ml/min) was calculated. Plasma without venostasis and urine were also collected. Salivary, plasma and urine osmolality were measured using a freezing point depression osmometer. RESULTS: Cerebral palsy children presented a reduction in salivary flow rate (50%) compared to the control group (P < 0.01). Moreover, an increase in salivary (50%), plasma (3%), and urine osmolality (20%) was also observed in the cerebral palsy children compared to the control group (P < 0.01). Salivary flow rate was negatively correlated with the salivary, plasma and urine osmolality (P < 0.01). Salivary osmolality correlated positively with plasma and urine osmolality (P < 0.01). CONCLUSION: Cerebral palsy children seem to present impaired adequate hydration status. Since the possible hypohydration condition may be reflected in saliva fluid, which could compromise the protective function exerted by saliva, the earlier this condition is identified the greater the chances of administering preventive measures. Moreover, salivary osmolality is a reliable parameter that reflects changes in plasma and urine.

A carbon column-based liquid chromatography electrochemical approach to routine 8-hydroxy-2'-deoxyguanosine measurements in urine and other biologic matrices: a one-year evaluation of methods.

8-Hydroxy-2'-deoxyguanosine (8OH2'dG) is a principal stable marker of hydroxyl radical damage to DNA. It has been related to a wide variety of disorders and environmental insults, and has been proposed as a useful systematic marker of oxidative stress. Analytic procedures for 8OH2'dG in DNA digests are well established; however, routine measurement of free 8OH2'dG in other body fluids such as urine or plasma has been problematic. This has hindered its evaluation as a general clinical, therapeutic monitoring, or environmental assessment tool. Therefore, we developed a liquid chromatography electrochemical column-switching system based on the use of the unique purine selectivity of porous carbon columns that allows routine accurate measurement of 8OH2'dG in a variety of biologic matrices. This paper describes the rationale of the system design and the protocols developed for 8OH2'dG in urine, plasma, cerebrospinal fluid, tissue, DNA, saliva, sweat, kidney dialysis fluid, foods, feces, culture matrix, and microdialysates. Concentrations in both human and animal body fluids and tissues are reported. The system performance is discussed in the context of a 1-year evaluation of the methods applied to approximately 3600 samples, using internal quality control and external blind testing to determine long-term accuracy. The methods are reliable and accurate, and therefore should prove useful in assessing the role and utility of oxidative DNA damage in aging and human illness.

3-Methyl glutaconic aciduria in Iraqi Jewish children may be misdiagnosed as cerebral palsy.

It is generally accepted that patients with cerebral palsy suffer from a static encephalopathy causing a non-progressive disorder of posture and/or movement. We describe 7 patients from 5 families who were initially diagnosed with cerebral palsy. Eventually, excessive excretion of urinary 3-methyl glutaconic acid (3-MGA) was found. The data of our 7 patients are quite similar to the clinical description of Costeff et al (1989): Jewish-Iraqi origin (7/7), consanguinity (2/7), involuntary movements (5/7), ataxia (6/7), pyramidal involvement (6/7) and optic atrophy (6/7). The cognitive functions were intact in 5/7 and 2/7 showed mild to moderate mental retardation. The mean delay in the definitive diagnosis was 9 years. Cerebral palsy-like symptoms accompanied by optic atrophy and extrapyramidal signs should call for extensive metabolic evaluation including the determination of urinary 3-MGA.

[Glutaric aciduria type I]. / Aciduria glutárica tipo I.

We report three patients with glutaric aciduria type I. The biochemical diagnosis of two cases was revealed by determination of free glutaric acid in urine, by using the CG/EM method. In the third patient, however, these levels were only slightly increased and the diagnosis was attained by the determination of total glutaric acid and glutaryl-carnitine. Serum carnitine levels were decreased in two cases. Clinical symptoms of this type of organic acidemia are highlighted by an acute or subacute presentation with signs of dysfunction of the neostriatum, simulating a cerebral paralysis with extrapyramidal signs. Homozygous patients have been reported with the same biochemical and enzymatic activity findings, but these patients were neurologically asymptomatic throughout life. Other features suggestive of the disease are macrocephaly associated with a widening of the subarachnoid spaces. Riboflavin and carnitine administration to these patients seems to prevent new bouts of neurological dysfunction.

Urinary levels of taurine in mentally retarded children.

Urinary taurine levels were estimated in 29 mentally retarded children. These levels were then compared with those of normal healthy children. The urinary taurine levels were significantly higher in the mentally retarded subjects. There is probably an intriguing relationship between taurine levels and mental retardation.

[Clinical results of using Nakom in diseases of the nervous system]. / Klinicheskie rezu'ltaty primeneniia Nakoma pri zabolevaniiakh nervnoi sistemy.

Clinical investigation was performed and dopamine metabolism (renal catecholamine excretion) studied in 29 children and 5 adults with various forms of CP, 5 myasthenic children and 1 child with Strümpel disease treated with Nakom. The therapy was judged expedient in 20% of the cases of pronounced muscular rigidity and dystonia with decreased dopamine excretion. The drug proved ineffective in atonic, astatic, severe hyperkinetic arms, and in cases with severe psycho-organic signs.

[Pathogenesis of hyperkinesis in children]. / O patogeneze giperkinezov u detei.

Pheylalanine metabolism was studied in 56 children with various forms of hyperkinesias. It was found that in the development of slow and fast hyperkinesias a certain role belongs to dihydroxyphenylalanine (DOPA). It is probable, that in patients with Turett's syndrome the synthesis of DOPA is increased while in patients the excretion of phenylacetylglutamine was found to be disturbed: it was decreased in the patients with the fast and increased in the children with the slow hyperkinesias. Phenylalanine load led to a lowering of the DOPA level in the patients with Turett's syndrome; an intensification of the synthesis of phenylacetylglutamine and diminution of the intensity of hyperkinesias. L-glutamine load resulted in detoxication of the toxic phenylalanine metabolites which inhibited the DOPA synthesis, as well as in a short-time increase in the phenylacetylglutamine excretion and a moderation of tonic hyperkinesias that manifested by athetosis and dystonia. All this points out that in the development of various forms of hyperkinesias a certain role belongs to amino acid metabolites.

Glutaric aciduria in progressive choreo-athetosis.

The clinical symptoms in a 10-year-old girl with progressive dystonic cerebral palsy are described. The biochemical findings were dominated by large amounts of glutaric acid in the urine. The disorder is caused by impairment of the degradation of glutaryl-CoA. A survey is given of the clinical and biochemical symptoms, based on the five cases reported so far. It is concluded that patients with progressive dystonic palsy should be examined for disorders in the metabolism of organic acids.

Glutaric aciduria: clinical and laboratory findings in two brothers.

In two siblings with dystonic cerebral palsy the urinary metabolic profiles of organic acids were dominated by glutaric acid, a metabolite not normally present in urine. The exretion of glutaric acid amounted to several grams per day. The urinary excretion of beta-OH-glutaric acid and glutaconic acid was also enhanced. Imparied metabolism of glutaryl-CoA by leukocytes indicates that the patients suffer from an inborn error of lysine, tryptophan, and hydroxylysine metabolism. A defective oxidation of glutaryl-CoA to crotonyl-CoA, probably due to a deficiency of glutaryl-CoA dehydrogenase, is consistent with these findings.