Parenteral nutrition in childhood and consequences for dentition and gingivae.

AIM: Assessment of dentition in children under parenteral nutrition, risk factors for caries, and dental developmental abnormalities. MATERIALS AND METHODS: The study involved 63 patients (aged 2.25-16.6 years), i.e. 32 subjects receiving parenteral nutrition for a mean period of 5.6±2.94 years, and 31 healthy control subjects. Oral hygiene (OHI-S, PL-I), gingival (GI), and dentition status (caries, DMFT/dmft, enamel defects, shape alterations), frequency of oral meals and frequency of cariogenic snacks consumption were evaluated. Medical records provided information on parenteral meals per week, age parenteral nutrition started, birth body mass, Apgar score, weight deficiency, and antibiotic therapy until aged 1 year. The Mann-Whitney test, chi-squared test, and Spearman rank correlation coefficient were used (p≤0.05). RESULTS: Dental developmental abnormalities occurred more often in PN subjects (71.87% vs. 25.80%). The prevalence of caries in PN (56.25% vs. 90.32%) and dmft (2.00±3.30 vs. 4.21±3.33) and DMFT (2.47±4.08 vs. 3.33±3.50) were lower. Positive caries Spearman's rank correlation coefficients: frequency of oral meals and frequency of cariogenic snacks consumption, and GI. Negative correlation coefficients: low birth body mass, antibiotic therapy, and low body mass in the first year of life. Positive dental developmental abnormality Spearman's coefficients: low birth body mass, Apgar score < 7, parenteral nutrition duration, low body mass and antibiotic therapy in the first year of life. Beta- lactam, aminoglycoside, glycopeptide and nitroimidazole treatments were related to enamel hypoplasia. CONCLUSION: Parenteral nutrition in childhood is related to the risk of dental developmental abnormalities, promoted by malnutrition and antibiotic therapy in infancy. Limiting the number of meals and cariogenic snacks, and most probably administration of antibiotics, decreases the risk of caries.

Diagnosis of gastroesophageal reflux and anti-reflux procedures among Polish children with gastrostomies: a 10-year nationwide analysis.

BACKGROUND/OBJECTIVES: To analyse the approach to diagnose gastroesophageal reflux (GER) and the qualification criteria for anti-reflux (AR) procedures in Polish children fed via gastrostomy between 2000 and 2010. SUBJECTS/METHODS: An electronic questionnaire containing questions on the demographic and clinical data of patients with gastrostomies was distributed to six Polish centres of nutritional therapy. The portion pertaining to GER included data on clinical exponents, diagnostic procedures (pH-metry, pH-impedance, scintigraphy and upper gastrointestinal (GI) series) and AR. RESULTS: In total, 348 children (M199/F149; age at gastrostomy 5.78±5.49 years) were included. Data on the diagnosis of GER and the AR criteria were available for 343 and 336 subjects, respectively. Percutaneous endoscopic gastrostomy was performed in 258/348 patients (74.1%), while surgery was performed in 80/348 patients (23%). The data from 10/348 (2.9%) cases were unavailable. At least one of the tests for GER was conducted in 177/343 (51.6%) of patients: pH-metry in 74/343 (21.6%), pH-impedance in 17/343 (5.0%), scintigraphy in 60/343 (17.5%) and upper GI series in 102/343 (29.7%). GER was reported in 114/343 cases (33.2%), and fundoplication was performed in 87 children (76.3% of patients with GER). The highest congruence between a positive test result and the decision to perform fundoplication was documented in cases of scintigraphy and upper GI series (P=0.00000 and P=0.00191, respectively). A significant increase in the prevalence of simultaneous gastrostomy and AR was observed over the decade analysed (r=0.8, P=0.009). This study revealed a centre-specific attitude towards the diagnosis of GER and the assessment of qualifications for fundoplication in Polish gastrostomy-fed children. CONCLUSIONS: The unified diagnostic algorithm of GER and the universal qualification criteria for AR procedures need to be defined for gastrostomy-fed children.

Aluminum contamination of parenteral nutrition additives, amino acid solutions, and lipid emulsions.

Contamination of parenteral nutrition solutions with aluminum may result in accumulation of this element in bones and, in premature infants, may inhibit bone calcium uptake and induce cholestasis. We measured the aluminum concentration of small-volume parenterals, amino acid solutions, lipid emulsions, and special solutions containing glucose, amino acids, electrolytes, and trace elements (standard I for children with a body weight of 3-5 kg, standard II for children with a body weight of 5-10 kg). The method used was graphite furnace atomic absorption spectrometry GTA-AAS (SpectrAA-400 Plus, Varian, PtY Ltd., Mulgrave, Australia). Quality control was run with the use of control serum (Seronorm, Nycomed, Oslo, Norway). The aluminum contents of parenterally administered solutions were: pediatric trace elements, 130 micrograms/L, and pediatric trace elements, 3000 micrograms/L; phosphorus salts: K-phosphates, 9800 micrograms/L, and Na/K phosphates, 13,000 micrograms/L; 10% calcium gluconate, 4400 micrograms/L; 6.5% amino acids, 30 micrograms/L; 10% amino acids, 120 micrograms/L; 12.5% amino acids, 121 micrograms/L; 20% lipid emulsion, 30 micrograms/L; 20% lipid emulsion, 180 micrograms/L; water-soluble vitamins, 12 micrograms/L; lipid soluble vitamins, 360 micrograms/L; standard I, 55 micrograms/L; standard II, 90 micrograms/L; The aluminum intake from parenteral nutrition was 6.6-10.8 micrograms.kg-1.d-1--a dose exceeding the safety limit of 2 micrograms.kg-1.d-1. The possible association of aluminum not only with metabolic bone disease, but also with encephalopathy, dictates caution when dealing with the pediatric population on long-term parenteral nutrition. In the absence of reliable label information, it seems proper to monitor the aluminum concentration in parenteral nutrition products and to report it in professional journals.

Genetic background of congenital chloride diarrhea in high-incidence populations: Finland, Poland, and Saudi Arabia and Kuwait.

Congenital chloride diarrhea (CLD) is an inherited intestinal disorder caused by mutations in the down-regulated in adenoma gene. In Finland, the disease is prevalent because of a founder effect, and all but one of the CLD-associated chromosomes carry the same mutation, V317del. In Poland, another area with a high incidence of CLD, as many as seven different mutations have been detected so far. A third known cluster of CLD, around the Persian Gulf, has not been genetically studied. We studied the allelic diversity of CLD in Poland, in Saudi Arabia and Kuwait, and in three isolated families from North America and Hong Kong. Altogether, eight novel mutations were identified, making a total of 19 known CLD gene mutations. The Polish major mutation I675-676ins was found in 47% of the Polish CLD-associated chromosomes. Haplotype analysis and clustering of the I675-676ins mutation supported a founder effect and common ancestral origin. As in Finland, a major founder effect was observed in Arab patients: 94% of the CLD-associated chromosomes carried a nonsense mutation, G187X, which occurred in either a conserved ancestral haplotype or its derivative. Our data confirm that the same locus is mutated in all cases of CLD studied so far. In Poland, a relatively common founder mutation is likely to highlight a set of rare mutations that would very rarely produce homozygosity alone. This suggests that mutations in the CLD locus are not rare events. Although the disease is thought to be rare, undiagnosed patients may not be uncommon.

Clustering of private mutations in the congenital chloride diarrhea/down-regulated in adenoma gene.

An inherited defect in intestinal anion exchange, congenital chloride diarrhea (CLD), was recently shown to be caused by mutations in the down-regulated in adenoma (DRA) gene. A three base pair deletion resulting in the loss of an amino acid valine (V317del) in the predicted CLD/DRA protein was shown to be responsible for all CLD cases in a Finnish founder population. Two additional mutations, H124L and 344delT, were found in Polish CLD patients. Here, we screened for additional mutations in a set of 14 CLD families of Polish, Swedish, North American, and Finnish origin using primers that allowed mutation searches directly from genomic DNA samples. We found eight novel mutations in the CLD/DRA gene. The mutations included two transversions, one transition, one insertion, and four small deletions. Of 11 sequence alterations detected so far, nine lie clustered in three short segments that are 49 bp, 39 bp, and 65 bp in size, respectively. These short segments span only 6.7% of the total cDNA length, suggesting functional importance or mutation-prone DNA regions of the corresponding CLD/DRA protein domains.

Disorders of magnesium homeostasis in the course of liver disease in children.

Magnesium deficiency can develop in patients with acute or chronic liver disease as a result of low dietary magnesium intake, low intestinal absorption or renal magnesium loss caused by natriuretic drugs. The aim of this study was to evaluate magnesium homeostasis in 39 children: 10 with acute liver failure due to Amanita phalloides poisoning. 14 with chronic liver diseases without cholestasis, and 15 with chronic liver diseases with cholestasis. Serum magnesium and fractional and 24 h urinary magnesium excretion were measured in all the children. Magnesium retention after intravenous infusion was also evaluated. Tissue magnesium deficit was found in 30 per cent of children with acute or chronic liver disease.

[Autoimmune enteropathy--difficulties in diagnosis and treatment]. / Biegunka autoimmunologiczna--trudnosci w diagnostyce i leczeniu.

We describe a 42-month-old child with protracted diarrhoea that began at 6 months of age. Severe secretory diarrhoea persisted despite therapy with exclusion diets, total parenteral nutrition, chemotherapeutics, antisecretory drugs. The diagnosis of autoimmune enteropathy with total villous atrophy and anti-enterocyte antibodies was established at 16 months of age. Prednisone therapy induced a clinical remission. After dose reduction, clinical relapse occurred, complicated with HCV infection with elevated serum alanine aminotransferase activity. Increasing the prednisone dose did not result in clinical improvement. Treatment with cyclosporine induced clinical remission. After 10 months cyclosporine therapy is still continued and the boy is doing well.

[Familial diarrhea exemplified by two children]. / Biegunka rodzinna na przykladzie dwojga dzieci.

Two cases (siblings) are presented of children with diarrhoea since neonatal period and early infancy, of secretory character. The aetiological factor could not have been established and effective treatment could not have been instituted.