[Occurrence of the mycotoxin ochratoxin a in breast milk samples from Germany]. / Zum Vorkommen des Mykotoxins Ochratoxin A in Muttermilchproben aus Deutschland.

INTRODUCTION: Breast milk is the best form of nutrition early in life, yet it may contain contaminants which were ingested by mothers. Ochratoxin A (OTA) is a well-known nephrotoxin with carcinogenic properties and a frequent food contaminant. Ingested OTA is partly excreted with human milk and studies conducted in different countries have shown a wide range of OTA concentrations. The aim of this study was to assess the exposure of infants to OTA by analysing breast milk samples from 2 German areas. METHODS: Breast milk samples were obtained from 90 mothers who had signed an informed consent sheet. The previously validated analytical method (LOD=10 ng/L, LOQ=30 ng/L) involves liquid-liquid extraction and analysis by HPLC with tandem mass spectrometric detection. A preliminary risk assessment was done using the TDI approach. CONCLUSION: More than 50% of the collected 90 milk samples contained detectable OTA levels. Overall, the average concentration in milk from -Dortmund (24.4 ± 21.1 ng/L (n=30), range:<10-100 ng/L) were significant higher than those measured in the Hannover cohort (14.4 ±1 5.1 ng/L (n=60), range: <10-78 ng/L). The OTA levels of 13 samples were measured with concentrations≥ LOQ. The burden of breast milk in different lactation stages, differentiated by colostrum, transitional milk and mature milk, did not differ in the 2 samples collectives Dortmund and Hannover. The infants' exposure was assessed by calculating their OTA intake via human milk. These results were then compared to the recently re-evaluated Tolerable Daily Intake (TDI) of 3 ng/kg body weight/day. In 29% of the cases (with 26 milk samples), the TDI of 3 ng/kg body weight/day was exceeded.In summary, infant exposure to OTA with human milk in Germany is usually low compared to several other countries. Given that in some cases the TDI is exceeded, further efforts to regulate OTA levels in food with the aim of reducing the contamination should be made to minimize the exposure of lactating women to OTA.

Lung function at age 18-25 years: a comparison of different reference value systems.

The anthropometrical data of the Caucasian population have significantly changed within the last five decades. The European Community for Coal and Steel (ECCS) assumes a plateau phase and recommends the entry of 25 years old for calculation of reference values in this age range. The question arises if the commonly used reference recommendations for lung function of the ECCS can still be accepted. In the present study standardized spirometric lung function tests were performed by pneumotachography, recording lung volumes and flows (MasterScreen Pneumo, CareFusion, Höchberg) in asymptomatic nonsmoking subjects (202 females, 201 males), aged between 18 and 26, according to the ATS/ERS criteria. The results were compared with the reference recommendations of ECCS, SAPALDIA, LuftiBus, and Bochum (only males). All absolute lung function values showed a correlation (p< 0.05) with height. With respect to FVC and FEV(1), SAPALDIA and Bochum reference values were comparable and close to a 100 (range 97.6-101.4) %pred, whereas both ECCS and LuftiBus showed higher values (range 103.6-109.9%pred). The FEV(1)/FVC ratio was close to a 100 (range 97.6-101.7) %pred in all reference systems, whereas flows showed a wide variability between the reference systems (77.1-114.6%pred), single flows (e.g., 96.9-114.2%pred for MEF(50)) and males/females (males: 93.6-114.6%pred; females: 77.1-107.9%pred). We conclude that SAPALDIA reference values for FVC and FEV(1) should be used, as they better represent lung function in the age group. ECCS and LuftiBus reference values are appreciably (4-10%) lower. Differences between reference systems were less important for the FEV(1)/FVC ratio and lung flows.

PUFA and LC-PUFA intake during the first year of life: can dietary practice achieve a guideline diet?

BACKGROUND: An appropriate supply of n-3 long-chain polyunsaturated fatty acids (LC-PUFAs) during early childhood may enhance cognitive development. Little attention is paid to the fatty acid (FA) supply during the complementary feeding period. We examined the polyunsaturated fatty acids (PUFAs) and LC-PUFAs pattern in dietary practice of two study groups and evaluated the results against the present Dietary Guidelines in Germany. METHODS: The food consumption and FA pattern of dietary practice in subjects from two prospective studies (n=102 and n=184, respectively) at the age of 3, 6 and 9 months was assessed by weighed diet records, and changes during the first year of life were compared with the food-based dietary guidelines for the first year of life. RESULTS: Dietary practice in the complementary feeding period was clearly dominated by commercial food products. The FA composition in dietary practice was different from the Guideline Diet and the ratio of n-6/n-3 PUFAs was less favorable. Consumption of breast milk or formula was still of major importance for the intake of LC-PUFAs in the complementary feeding period. CONCLUSION: LC-PUFAs are predominantly provided by breast milk and formula during the first year of life and consequently decrease when milk consumption decreases. For compensation, commercial complementary food might come closer to the Guideline Diet by lowering the n-6/n-3 PUFA ratio through appropriate vegetable oil along with an increase in total fat content up to the legal limit.

Modification of dietary polyunsaturated fatty acids via complementary food enhances n-3 long-chain polyunsaturated fatty acid synthesis in healthy infants: a double blinded randomised controlled trial.

OBJECTIVE: To study the effect of modified polyunsaturated fatty acid (PUFA) profiles of complementary food on long-chain (LC) PUFA composition in healthy infants. DESIGN: Double blinded, randomised, controlled intervention trial. SETTING: Dortmund, Germany. PATIENTS: Free-living sample of healthy term infants. METHODS: Participants were randomly assigned within the first 2 months of life. During the intervention period from 4 to 10 months, the control group (n = 53) received commercial complementary meals with corn oil (3.4 g/meal) rich in n-6 linoleic acid (LA), the intervention group (n = 49) received the same meals with rapeseed oil (1.6 g/meal) rich in n-3 alpha-linolenic acid (ALA). Fatty acid intake was assessed from dietary records throughout the intervention period. Fatty acid proportions (% of total fatty acid) in total plasma were analysed before and after the intervention. RESULTS: Plasma fatty acid profiles did not differ between the intervention and control groups before the intervention. During the intervention, the only difference in fatty acid intake between the intervention and control groups was a higher intake of ALA in the intervention group, 21% deriving from study food and a lower ratio of LA/ALA (10.7 vs 14.8). At the end of the intervention, the plasma proportions of total n-3 fatty acids and of n-3 LC-PUFA, but not of ALA, were higher and the ratios of n-6/n-3 fatty acids were lower in the intervention group. CONCLUSIONS: Feasible dietary modifications of the precursor fatty acid profile via n-3 PUFA-rich vegetable oil favoured n-3 LC-PUFA synthesis in the complementary feeding period when LC-PUFA intake from breast milk and formula is decreasing.

Spirometry in preschool children: time has come for new reference values.

Lung function measurements play an essential role in early diagnosis and monitoring of bronchial asthma in children. For clinical evaluation, measurements are commonly compared to reference values. However, these reference values are calculated based on measurements performed in groups of mostly older children and young adults two or three decades ago. In the present, cross-sectional study, lung function measurements were performed in 518 children (241 boys and 277 girls; mean age 6.0+/-0.3 years) at a regular medical check prior to school enrollment. Spirometry was done using the MasterScreen IOS (Cardinal Health, Wurzburg). We recorded forced vital capacity (FVC), forced expiratory volume in one second (FEV(1)), maximal expiratory flow (PEF), and maximal expiratory flow at 75, 50, and 25% of vital capacity (MEF(75), MEF(50), MEF(25)). We found that FEV(1) and FVC corresponded to reference values (101.0+/-14.9% and 95.4+/-13.6%, in boys and girls, respectively). In maneuvers satisfying ATS/ERS criteria (T(E) >1 sec), forced expiratory (parameters (PEF, MEF(50)) reached only 68.9+/-13.6 and 75.9+/-26.6% of reference values, in boys and girls, respectively). There was no significant correlation of lung function parameters to BMI. In conclusion, the hitherto reference values largely overestimate the maximal flow rates of preschool children performing a forced spirometry with T(E) >1 sec. At the age of 6, forced expiratory flow values are not (yet) impaired by an increased BMI. Standardized spirometry starting in preschool children allows closely evaluating the individual development of lung function during follow-up measurements.

Pharmacological impact on loop gain properties to prevent irregular breathing.

Theory predicts respiratory instabilities at elevated system loop gain (G), determined by such factors as ventilatory CO(2) sensitivity, set-point PCO(2), and metabolic rate. In anesthetized rabbits, the effects on G of carbonic anhydrase (CA) inhibitors and of different sodium/proton exchanger type 3 (NHE3) inhibitors were studied. Acetazolamide significantly reduced G by 42.0 +/- 9.3% and methazolamide by 35.0 +/- 9.5% (each n = 7, P<0.01). Irrespective of the substance, NHE3 inhibition reduced G by 33.0 +/- 7.8% (n = 10, P<0.01) at 35.5 +/- 1.6 mmHg PaCO(2) (mean +/-SE), but not at lower arterial CO(2) levels (n=5). Since high baseline PCO(2) coincides with elevated brainstem NHE3 mRNA expression, this may also account for a higher risk of sleep apnea (or even occurrence of sudden infant death). Therefore, NHE3 inhibitors may gain similar therapeutic importance in the treatment of irregular breathing as CA inhibitors. Generally, effective treatment should aim at a low system loop gain, by reducing respiratory chemosensitivity, improving blood gases and preventing low metabolic rates.

Patterns of intrathoracic gas volume and airway resistance in children with asthma.

Airway resistance (Raw) decreases with an increase of lung volume (ITGV) during body-growth. In asthmatic subjects, an increase of Raw may be modified by hyperinflation. In this study thirty five asthmatic children underwent histamine challenges with the monitoring of changes of Raw and ITGV. Control measurements (after withhold of spasmolytic medication) showed increased values of ITGV and Raw with high interindividual variability. Histamine challenge resulted in a further increase of both ITGV and Raw in 18 patients (normal pattern). Twelve patients showed an increase predominantly of Raw (obstructive pattern), 5 patients an increase predominantly of ITGV (hyperinflatory pattern). On provocation and after bronchospasmolysis, all subjects presented the expected inverse relation between ITGV and Raw. The variability of ITGV-Raw patterns in children with asthma may agree with the concomitantly established role for vagal reflex mechanisms in prolonged inspiratory diaphragmatic innervation during experimentally induced bronchoconstriction in animals. In children with asthma the ITGV-Raw pattern may point to different risk profiles.

[Congenital intestinal lymphangiectasia: a rare differential diagnosis in hypoproteinemia in infants]. / Kongenitale intestinale Lymphangiektasie: Eine seltene Differerenzialdiagnose hypoproteinämischer Odeme im Säuglingsalter.

BACKGROUND: Congenital intestinal lymphangiectasia is a rare disease in childhood, which may already cause protein-losing enteropathy in newborns. PATIENT, METHODS AND RESULTS: This is a case report of an infant with generalized edema and protein-losing enteropathy, in whom intestinal lymphangiectasia was diagnosed at the age of two months. Following repetitive intravenous albumin und gamma globulin infusions, the elimination of long-chain fats from the diet and the substitution with medium-chain triglycerides (MCT) led to an improvement of the protein-losing enteropathy. CONCLUSION: In newborns with low level of serum protein and edema protein-losing enteropathy caused by congenital lymphangiectasia might be considered as a differential diagnosis.

Mild dehydration: a risk factor of broncho-pulmonary disorders?

Several expert committees recommend a high fluid intake in patients with chronic bronchitis and asthma. Is there a relationship between fluid intake or hydration status and broncho-pulmonary disorders like bronchitis and asthma? First, basic physiologic mechanisms like regulation of lung fluid balance and water transport at pulmonary surfaces were analyzed, in order to characterize the role of local hydration status in lung and airways. Second, making use of the computer-based literature searches (PubMed), evidence for a role of hydration status in complex physiological and pathophysiological conditions of lungs and airways like perinatal lung adaptation (PLA) (in prematures), mucociliary clearance(MC) and asthma was categorized. The movement of fluid between the airspaces, interstitium, and vascular compartments in the lungs plays an important physiological role in the maintenance of hydration and protection of the lung epithelium and significantly contributes to a proper airway clearance. PLA is characterized by a rapid change from fluid secretion to fluid absorption in the distal respiratory tract, with the literature data confirming a critical role of the epithelial sodium channel. Only few studies have investigated the effect of different fluid input regimens on PLA in prematures. MC relies on the interaction between epithelial water fluxes, mucus secretions, and ciliary activity. Whereas animal data show that drying of the airway epithelium decreases MC, few clinical studies investigating the effect of local or systemic hydration on MC have led to ambiguous results. Asthma (A) is characterized by chronic airway inflammation and episodic airway obstruction. Data in animals and humans indicate an association between exercise-induced-A and conditioning (humidity and heat exchange) of inspired air. However, epidemiological studies (children and adults), investigating the role of fluid (and salt) input in the etiology of the disease as well as studies analyzing different markers of hydration status during asthmatic attacks have so far led to conflicting results. Some expert groups recommend sufficient hydration as a complementary A-therapy. Analysis of basic physiological mechanisms in lungs and airways clearly demonstrates a critical role for water transport and local hydration status. In broncho-pulmonary diseases, however, analysis of the complex pathophysiological mechanisms is difficult. Thus, we still need more studies to confirm or refute mild dehydration or hypohydration as a risk factor of broncho-pulmonary disorders.

Renal excretion of calcium and phosphorus in premature infants with incipient late metabolic acidosis.

BACKGROUND: Premature infants receiving alimentation with cow milk-based formulas run a considerably high risk of incipient late metabolic acidosis, an early stage developing of manifest late metabolic acidosis. Is bone metabolism involved in pathophysiologic mechanisms characterizing this early stage of retention acidosis? METHODS: Urinary ionography was performed in 10 premature infants with spontaneous development of incipient late metabolic acidosis (indicated by urine pH < 5.4 on 2 consecutive days) and 10 pair-matched premature infants with normal values of urine pH; both groups were receiving full oral nutrition with the same standard formula. Moreover, in 37 premature infants with incipient late metabolic acidosis who were randomly allocated to oral therapy with 2 mmol. kg(-1). d(-1) of either NaHCO 3 or NaCl over a period of 7 days, urinary excretion of calcium and phosphorus was assessed on day 1 and day 7. RESULTS: Incipient late metabolic acidosis was accompanied by increased phosphaturia in premature infants receiving full oral nutrition. Seventeen premature infants receiving NaCl therapy (19 treatment periods) showed increased calciuria from day 1 to day 7, whereas, in 20 premature infants receiving NaHCO 3 therapy (23 treatment periods), calcium or phosphorus excretion in urine did not increase. CONCLUSIONS: The data of urinary calcium and phosphorus excretion in premature infants support the hypothesis that bone mineralization may already be impaired in the early stage of incipient late metabolic acidosis.

X-linked anhidrotic ectodermal dysplasia with immunodeficiency is caused by impaired NF-kappaB signaling.

The molecular basis of X-linked recessive anhidrotic ectodermal dysplasia with immunodeficiency (EDA-ID) has remained elusive. Here we report hypomorphic mutations in the gene IKBKG in 12 males with EDA-ID from 8 kindreds, and 2 patients with a related and hitherto unrecognized syndrome of EDA-ID with osteopetrosis and lymphoedema (OL-EDA-ID). Mutations in the coding region of IKBKG are associated with EDA-ID, and stop codon mutations, with OL-EDA-ID. IKBKG encodes NEMO, the regulatory subunit of the IKK (IkappaB kinase) complex, which is essential for NF-kappaB signaling. Germline loss-of-function mutations in IKBKG are lethal in male fetuses. We show that IKBKG mutations causing OL-EDA-ID and EDA-ID impair but do not abolish NF-kappaB signaling. We also show that the ectodysplasin receptor, DL, triggers NF-kappaB through the NEMO protein, indicating that EDA results from impaired NF-kappaB signaling. Finally, we show that abnormal immunity in OL-EDA-ID patients results from impaired cell responses to lipopolysaccharide, interleukin (IL)-1beta, IL-18, TNFalpha and CD154. We thus report for the first time that impaired but not abolished NF-kappaB signaling in humans results in two related syndromes that associate specific developmental and immunological defects.

PC-1 nucleoside triphosphate pyrophosphohydrolase deficiency in idiopathic infantile arterial calcification.

Inogranic pyrophosphate (PPi) inhibits hydroxyapatite deposition, and mice deficient in the PPi-generating nucleoside triphosphate pyrophosphohydrolase (NTPPPH) Plasma cell membrane glycoprotein-1 (PC-1) develop peri-articular and arterial calcification in early life. In idiopathic infantile arterial calcification (IIAC), hydroxyapatite deposition and smooth muscle cell (SMC) proliferation occur, sometimes associated with peri-articular calcification. Thus, we assessed PC-1 expression and PPi metabolism in a 25-month-old boy with IIAC and peri-articular calcifications. Plasma PC-1 was <1 ng/ml by enzyme-linked immunosorbent assay in the proband, but 10 to 30 ng/ml in unaffected family members and controls. PC-1 functioned to raise extracellular PPi in cultured aortic SMCs. However, PC-1 was sparse in temporal artery lesion SMCs in the proband, unlike the case for SMCs in atherosclerotic carotid artery lesions of unrelated adults. Proband plasma and explant-cultured dermal fibroblast NTPPPH and PPi were markedly decreased. The proband was heterozygous at the PC-1 locus, and sizes of PC-1 mRNA and polypeptide, and the PC-1 mRNA-coding region sequence were normal in proband fibroblasts. However, immunoreactive PC-1 protein was relatively sparse in proband fibroblasts. In conclusion, deficient extracellular PPi and a deficiency of PC-1 NTPPPH activity can be associated with human infantile arterial and peri-articular calcification, and may help explain the sharing of certain phenotypic features between some IIAC patients and PC-1-deficient mice.

Nutrition, acid-base status and growth in early childhood.

Optimal growth is only possible in a well-balanced "inner milieu". Premature infants are especially vulnerable for disturbances of acid-base metabolism with a predisposition to metabolic acidosis due to a transient disproportion between age-related low renal capacity for net acid excretion (NAE) and an unphysiologically high actual renal NAE on nutrition with standard formulas. During a 50 month period, 452 low birth-weight infants were screened for spontaneous development of incipient late metabolic acidosis (ILMA), an early stage during the development of retention acidosis, characterized by maximum renal acid stimulation (MRAS, urine-pH < 5.4) on two consecutive days but still compensated systemic acid-base status. Compared with controls, patients with ILMA showed higher serum creatinine values, an increased urinary excretion of sodium, aldosterone and nitrogen, but only slightly lower blood pH (7.38 vs 7.41) and base excess (-2.8 vs. 0.2 mmol/l) with respiratory compensation (PCO2 35 vs 37 mm Hg). Patients with altogether 149 episodes of ILMA were subsequently randomly allocated to either treatment with NaHCO3 2 mmol/kg/d for 7 days or no special therapy in protocol I, or NaHCO3 vs NaCl each 2 mmol/kg/d for 7 days in protocol II. Patients of protocol I with persistent MRAS for 7 days showed lowest weight gain and a tendency for a further increase in urinary aldosterone and nitrogen excretion. NaCl supplementation (protocol II) seemed to promote weight gain without affecting either impaired mineralization or suboptimal nitrogen retention. Patients with alkali therapy under both protocols showed normal weight gain and normalization of hormonal stimulation, mineralization (protocol II) and nitrogen assimilation. Modification of the mineral content of a standard preterm formula decreased renal NAE to the low level seen on alimentation with human milk and reduced the incidence of ILMA in preterm and small-for-gestational-age infants to 1%. The data show that ILMA is associated with impaired growth. Activation of secondary homeostatic mechanisms (extracellular volume contraction, depletion of disposable net base pools) might be important for impaired growth. Production of new formulas for reduced renal NAE could be an effective general preventive measure to reduce the clinical importance of one component of mixed acid-base disorders in early childhood.

Minimal-invasive approach to study pulmonary, metabolic and renal responses to alimentary acid-base changes in conscious rabbits.

BACKGROUND: Systemic acid-base balance is maintained by the complex interplay of renal and pulmonary control functions and metabolic adaptations, whereby intake and mineral composition of feed are important factors. AIM OF THE STUDY: It was intended to explore the role of alimentary acid-base load and carbonic anhydrase activity for regulatory responses of renal, pulmonary or metabolic origin in rabbits as typical herbivores. METHODS: Sixty-eight conscious male rabbits (about 3.5 kg) were kept in a metabolic cage, to determine daily water intake, urine excretion and food consumption. Different groups were fed either alkali-rich rabbit standard pellets, or modified rabbit chow with low Ca++-content, or a special diet with very low alkali content, or standard food together with a low oral dose (about 20 mg x kg(-1) x d(-1)) acetazolamide. Samples from the central ear artery were analyzed for blood gases (PaO2, PaCO2), pHa, base excess (BE) and actual bicarbonate (HCO3a-). The metabolic CO2 production (VCO2 STPD) was determined, to calculate alveolar ventilation (VA BTPS). Anaerobically collected urine was analyzed for pHu and for concentrations of bicarbonate/carbonate (HCO3-/CO3--), ammonium (NH4+), and phosphate. RESULTS: 1) Systemic BE was not affected by alimentary alkali load, either varied spontaneously by standard food intake or by the low-Ca++ diet, and decreased only slightly on the low-alkali diet, but distinctly upon carbonic anhydrase inhibition. 2) Under all conditions of alimentation, PaCO2 was closely correlated with BE without a detectable set-point, the normal-range variability of BE being sufficient to elicit corresponding changes in VA. In contrast, acetazolamide led to much lower values of PaCO2 than predicted by the reference PCO2/BE relationship, being primarily caused by significant reductions in VCO2 (> 20%). 3) Prior to other systems, renal base excretion, normally being high on species-adapted standard chow, closely followed any variation of alimentary alkali load and approached zero upon the low-alkali diet. It was, however, not significantly influenced by carbonic anhydrase (CA) inhibition on alkalirich alimentation. CONCLUSIONS: Blood acid-base balance in rabbits is maintained over a wide range of alimentary alkali load by effective adaptation of renal base excretion, independent of CA activity. Ventilatory pH control is perpetuated even in the normal range of BE, provided metabolic rate is not impaired, e. g., by CA inhibition. These results may help one understand the different manifestations of acid-base disorders in body fluids under clinical conditions.

[3-hydroxy-3-methylglutaraciduria (case report of a female Turkish sisters with 3-hydroxy-3- methylglutaryl-Coenzyme A lyase deficiency]. / 3-Hydroxy-3-methylglutaracidurie (Fallbeschreibung eines weiblichen türkischen Geschwisterpaares mit 3-Hydroxy-3-methylglutaryl-Coenzym A Lyase Mangel).

3-Hydroxy-3-methylglutaric aciduria is a rare inborn error of metabolism, caused by reduced enzyme activity of the intramitochondrial 3-hydroxy-3-methylglutaryl-CoA lyase. We describe two turkish sisters with this disease. In the older sister clinical symptoms with lethargy, convulsions, metabolic acidosis, hypoglycemia and hyperammonemia lead to the diagnosis. The younger sister was diagnosed prenatally. The clinical course of our patients is compared with those reported in the literature with respect to clinical symptoms, differential diagnosis and therapeutic regimens.

[Trisomy of the short arm of chromosome 10p; description of a female patient with de novo duplication 10p11.2-15]. / Trisomie des kurzen Arms von Chromosom 10p; Beschreibung einer Patientin mit de novo Duplikation 10p11.2-15.

Trisomy 10p is a rare chromosomal syndrome, characterized by craniofacial abnormalities, malformations of organs and skeleton, and impaired psychomotor development. In most of the cases partial trisomy 10p results of a balanced translocation or inversion, the mother being carrier of the structural abnormality. Only eight of 63 patients with trisomy 10p found in a literature survey present a de novo trisomy. 17 cases show a pure trisomy 10p without an associated deficiency of any other chromosome segment. We report a female patient with an interchromosomal de novo duplication 10p11.2-->15, demonstrating typical clinical signs like craniofacial abnormalities, oral cleft, club foot, seizures, and a severe delay of psychomotor development.

[Polysaccharide specific humoral immunodeficiency in ectodermal dysplasia. Case report of a boy with two affected brothers]. / Polysaccharid-spezifischer humoraler Immundefekt bei ektodermaler Dysplasie. Fallbericht über einen Jungen mit zwei betroffenen Brüdern.

We report a now three year old male patient with ectodermal dysplasia and a polysaccharide specific humoral immunodeficiency. Immunological investigations showed compromised production of IgA, IgM, and IgG2. Isohaemagglutinins still were not detectable at the age of three years. Repeated vaccination with polyvalent pneumococcal polysaccharide vaccine did not result in production of specific antibodies. Two brothers showed clinical signs of ectodermal dysplasia. The elder brother died from pneumococcal sepsis at the age of 3 years. The younger brother suffers from chronic inflammatory gastrointestinal disease with ulcerations in all parts of the gastrointestinal system. Thus, a possible association between polysaccharide specific humoral immunodeficiency and ectodermal dysplasia may be considered.

Modified cow's milk formula with reduced renal acid load preventing incipient late metabolic acidosis in premature infants.

BACKGROUND: Premature infants receiving alimentation with cow's milk formulas are at a considerably high risk of developing incipient late metabolic acidosis, an early stage in the development of manifest late metabolic acidosis. Is it possible to reduce this risk by modification of the composition of a standard formula? METHODS: The mineral composition of a cow's milk preterm formula A was modified (formula B) with the aim of reducing the alimentary load to that of human milk. 160 premature infants were fed either mother's milk (n = 50) or the modified formula B (enriched with sodium and potassium) (n = 110), and their urine pH was tested twice a week. Randomly collected subgroups of infants were studied in detail for nutrient balances. The results were compared with earlier observations of 282 premature infants fed either mother's milk (n = 28) or the standard formula A (n = 254). RESULTS: Incipient late metabolic acidosis was observed in nine of 78 premature infants receiving mother's milk, 53 of 254 premature infants receiving the standard formula A, and only one of 110 premature infants fed the modified formula B. Net acid excretion was 0.58 mmol/kg/day in 11 premature infants receiving alimentation with the modified formula B compared with 1.73 mmol/kg/day in 23 premature infants fed formula A. This reduction was mainly due to an increased alkali excess (sodium + potassium-chloride) in intake and urine. CONCLUSIONS: Reduction of renal acid load with the modified formula B had a preventive effect on the rate of development of incipient late metabolic acidosis in premature infants.