Yeast mediates lactic acidosis suppression after antibiotic cocktail treatment in short small bowel?

During acidotic periods in a girl with a short small bowel, very high D-lactic acid concentrations were measured in blood and urine; the patient's characteristic faecal flora contained mainly lactobacilli, and during antibiotic cocktail treatment also many yeasts. In this case report we sought to understand the beneficial effect of the antibiotic cocktail. Microbiological analysis was performed in faecal samples.

Lactobacilli and acidosis in children with short small bowel.

Background: In patients with a short small bowel, D-lactic acidemia and D-lactic aciduria are caused by intestinal lactobacilli. The purpose of this study was to obtain a detailed picture of the metabolic acidosis in young children with short small bowel.

Methods: Feces, blood, and urine of children with short small bowel and acidosis were studied microbiologically and/or biochemically.

Prevalence of malnutrition in nonsurgical hospitalized patients and its association with disease complications.

The prevalence of malnutrition and its predictive value for the incidence of complications were determined in 155 patients hospitalized for internal or gastrointestinal diseases. At admission, 45% of the patients were malnourished according to the Subjective Global Assessment (physical examination plus questionnaire), 57% according to the Nutritional Risk Index [(1.5 x albumin) + (41.

Specificity of indexes of malnutrition when applied to apparently healthy people: the effect of age.

Protein-energy malnutrition is thought to be widespread in hospitalized patients. However, the specificity of indexes used to assess malnutrition is uncertain. We therefore assessed the rate of false-positive diagnoses of malnutrition when biochemical-anthropometric indexes were applied to healthy subjects.

Role of bacteria in the pathogenesis of short bowel syndrome-associated D-lactic acidemia.

Previously, we have demonstrated that short bowel syndrome (SBS) patients suffer daily from D-lactic acidemia; in these patients rather high amounts of (bacterial) D-lactate emerge in blood and urine with a circadian rhythm. The aim of this study was to establish the microbial basis of D-lactic acidemia in SBS. Therefore, faecal flora of (young and adult) SBS-patients was analysed qualitatively and quantitatively, and compared to that of controls.

Dihydropyrimidinase deficiency, a progressive neurological disorder?

A case of a child presenting with congenital abnormalities at birth is reported. The early development remained severely retarded and acquired skills minimally. The head circumference centile decreased.

The clinical phenotype of succinic semialdehyde dehydrogenase deficiency (4-hydroxybutyric aciduria): case reports of 23 new patients.

Objectives: To further define the clinical spectrum of the disease for pediatric and metabolic specialists, and to suggest that the general pediatrician and pediatric neurologist consider succinic semialdehyde dehydrogenase (SSADH) deficiency in the differential diagnosis of patients with (idiopathic) mental retardation and emphasize the need for accurate, quantitative organic acid analysis in such patients.

Patients: The clinical features of 23 patients (20 families) with SSADH deficiency (4-hydroxybutyric acid-uria) are presented. The age at diagnosis ranged from 3 months to 25 years in the 11 male and 12 female patients; consanguinity was noted in 39% of families.

2-Hydroxyglutaric aciduria: a case report on an infant with the D-isomeric form with review of the literature.

We report on an infant with D-2-hydroxyglutaric aciduria, who presented with severe seizures and developmental delay. We reviewed the literature for 2-hydroxyglutaric aciduria and found six other patients with the D-isomer and 24 patients with the L-isomer. Although the clinical spectrum of this inborn error of metabolism is variable, the clinical course of the D-form seems to be more severe than this of the L-form.

The importance of immunoglobulin-breakdown supporting the growth of bacteria in oral abscesses.

Oral bacteria play an important rôle in the causation of oro-facial abscesses. However, they can also be involved in brain, liver and lung abscesses. To persist, it is essential that these bacteria can grow on those sites.

CD4 deficiency in myelodysplastic syndrome with monosomy 7.

Unlabelled: We describe a patient with myelodysplastic syndrome with monosomy 7 presenting with a T-cell defect. He suffered from infections from the age of 10 years, when a CD4 deficiency and impaired lymphoproliferative responses in vitro were found. The only symptom of a myelodysplastic syndrome at that time was thrombocytopenia with giant platelets.

A dysbalanced immune system in cryptogenic Lennox-Gastaut syndrome.

In children with cryptogenic Lennox-Gastaut syndrome we found a functionally impaired humoral immune response to a primary antigen (haemocyanin), despite signs of a triggered immune system consisting of elevated IgG concentrations. This combination of immunological findings, considered to be the expression of a dysbalanced-triggered as well as functionally impaired-immune system, has also been described in an auto-immune disease like systemic lupus erythaematodes in humans, and in genetically epilepsy-prone rats. The interactions between the immune system and the nervous system in Lennox-Gastaut syndrome will be discussed.

D-lactic acidemia and aciduria in pediatric and adult patients with short bowel syndrome.

D-Lactate produced by abundant intestinal lactobacilli during acidotic episodes in short bowel (SB) patients is commonly regarded as a main factor in the pathogenesis of SB syndrome-associated (D-lactic) acidosis. Since we had observed that gram-positive bacteria, mainly lactobacilli, were abundant even in the absence of acidosis, we studied serum concentrations and urinary excretions of D- and L-lactate in young and adult SB patients, especially during nonacidotic periods. Serum L-lactate and urinary L-lactate excretion were similar in adults and children.

Altered immunoglobulin concentrations and light chain ratios in juvenile onset mixed connective tissue disease.

Immunological disturbances may result in altered immunoglobulin concentrations and kappa/lambda light chain (kappa/lambda) ratios. We measured the kappa/lambda ratios of total serum immunoglobulins and of polyclonal IgG, A, and M separately as well as concentrations of these immunoglobulins in fourteen patients with juvenile onset mixed connective tissue disease. When comparing the patient group with a reference group the mean serum IgG and IgA concentrations were respectively 2.

Degradation of immunoglobulin G by periodontal bacteria.

Several subgingival microorganisms were tested for their ability to utilize human immunoglobulin G (IgG) as a substrate for growth. This was done using a protein-free chemically defined medium, supplemented with IgG. Stimulation of growth was observed for Capnocytophaga ochracea, Porphyromonas asaccharolytica, Porphyromonas endodontalis, Porphyromonas gingivalis, Prevotella intermedia, Prevotella oralis, Lactobacillus catenaforme and Streptococcus intermedius.

Light chain ratios and concentrations of immunoglobulins G, A, and M in childhood common acute lymphoblastic leukemia.

The light chain ratios and the concentrations of immunoglobulin G (IgG), IgA, and IgM were measured before, during, and after antileukemic therapy in 10 patients with common acute lymphoblastic leukemia. The concentrations of IgG, IgA, and IgM decreased substantially during treatment but recovered slowly after cessation of the therapy. The light chain ratios were not systematically affected, but at diagnosis the kappa/lambda ratios of total serum Igs, IgG, and in particular IgM were somewhat lower in the patient group compared with an age-matched reference group.

Antibody deficiency and isolated growth hormone deficiency in a girl with Mulibrey nanism.

A combination of humoral immunodeficiency and isolated growth hormone deficiency was observed in a girl with Mulibrey nanism. The humoral immunodeficiency consisted of subnormal concentration of serum IgG, in particular IgG2 and IgG4, and low concentration of serum IgM. Serum IgA and IgD were elevated, IgE was absent.

Variants of Nijmegen breakage syndrome and ataxia telangiectasia.

Ataxia telangiectasia (AT) is an autosomal recessive disorder characterized by telangiectasia, progressive ataxia, sinopulmonary infections and a combined immunodeficiency (usually consisting of IgA deficiency, IgE deficiency, IgG2 and IgG4 deficiency and a disturbed T cell immunity). The alpha-fetoprotein level is elevated. Cytogenetic studies reveal a very specific chromosome instability with multiple chromosome 7 and/or 14 rearrangements (preferential breakpoints 14q32, 14q12, 7q35 and 7p12).

Light chain ratios and concentrations of serum immunoglobulins in children with epilepsy.

Serum immunoglobulin G, A and M concentrations and their respective kappa/lambda (kappa/lambda) light chain ratios were studied in 26 children with epilepsy. Fifteen had cryptogenic West syndrome or Lennox-Gastaut syndrome and 11 had other forms of childhood epilepsy. The results were compared to the data of a reference group of healthy children.