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Copyright of Monatsschrift Kinderheilkunde is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Monatsschrift Kinderheilkunde is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

In Germany, the "Dietary Schedule for the 1st year of life" and the "Optimised Mixed Diet" for children and adolescents serve as scientifically based and generally applicable dietary concepts throughout the period of growth and development. Vegetarian diets as the lacto-ovo-vegetarian diet (exclusion of meat, fish) and the vegan diet (exclusion of all food groups of animal origin) need to be evaluated for their potential to safely meet the high and specific requirements for growth and development. In this regard, high-quality studies are needed. In individuals on lacto-ovo-vegetarian diets, the safe supply with critical nutrients should be checked by thorough dietary history, possibly additional laboratory tests in risk situations like pregnancy, infancy and toddlerhood. Children on pure vegan diet need ongoing elaborate dietary strategies and continuous supplementation at any age, similar to nutritional management in children with metabolic disorders. A vegan diet is disadvised during all periods with intense growth and development., Competing Interests: Disclosure The authors report no conflicts of interest in this work., (© Georg Thieme Verlag KG Stuttgart · New York.)

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., (© Georg Thieme Verlag KG Stuttgart · New York.)

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.

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 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.

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.

We report a female newborn with prolonged neonatal hypoglycaemia and vomiting. Endocrinologic studies demonstrated the absence of the anterior pituitary hormones ACTH, STH, LH, FSH, and PRL while TSH basal secretion as well as TSH stimulation were normal at the age of 2 months. Magnetic resonance imaging showed aplasia of the anterior pituitary and a nodular ectopic posterior lobe. Substitution with prednisone resulted in normalization of blood glucose values with clinical improvement of the patient. At the age of 8 months the patient was started on recombinant human growth hormone due to poor growth; with 10 months decrease of thyrotropin secretion resulted in additional thyroxine replacement therapy.

A standard preterm formula was supplemented with calcium (Ca) and phosphorus (P) (F-CaP: Ca 87 mg/dl, P 43 mg/dl) and compared to the non-supplemented form (F: Ca 62 mg/dl, P 36 mg/dl). VLBW and small-for-gestational-age infants (n = 79) were included in the study which was performed to look for adverse effects and to decide about a reasonable start and duration of supplementation. In preterm infants with a birth weight lower than 1500 g and a body weight of more than 2000 g, the additional supplementation with Ca and P lead to a significant higher Ca- and P-retention without further load for the kidney. In addition, preterm infants with a body weight lower than 1500 g also had a better retention of Ca and P. Both, F-CaP and F lead to a high urinary excretion of phosphorus, a high renal net acid excretion and a relatively high activity of serum alkaline phosphatase. Anthropometric measurements did not reveal any evidence for an impaired caloric absorption due to an increased fecal fat excretion. Hypercalcemia or hyperphosphatermia was not seen. Hypercalciuria occurred in less than 5% of the samples studied. The results of this study indicate that a continuation of the supplementation with Ca and P is justified in VLBW infants with a body weight of more than 2000 g. There was no evidence for adverse effects of Ca and P supplementation in VLBW infants with a body weight lower than 1500 g, who might therefore also benefit from supplementation. Further studies are necessary to investigate unsatisfactory metabolic conditions of these children e.g. the high renal load.