Validation of the Children's Sleep Habits Questionnaire in a sample of Greek children with allergic rhinitis.

BACKGROUND: Obstructive respiratory disorders, such as allergic rhinitis and asthma may impair sleep quality. The aim of this study is to validate the Children's Sleep Habits Questionnaire (CSHQ) for Greek children from 6 to 14 years of age. No validated tool has been developed so far to assess sleep disturbances in Greek school-aged children. METHODS: We examined the reliability and validity of the CSHQ in a sample of children with allergic rhinitis (AR) and a non-clinical population of parents of these children as a proxy measure of children's AR quality of life (QoL) as evaluated by the Pediatric Allergic Rhinitis Quality of Life (PedARQoL) questionnaire. RESULTS: The CSHQ questionnaire Child's Form (CF) had a moderate internal consistency with a Cronbach's alpha 0.671 and Guttman split-half coefficient of 0.563 when correlated with the PedARQoL (CF). There was also a moderate intraclass correlation of ICC=0.505 between the responses to both questionnaires in the two visits. The CSHQ Parent's Form (PF) had a very good internal consistency with a Cronbach's alpha of 0.928 and Guttman split-half coefficient of 0.798. There was a high intraclass correlation of 0.643 between the responses in the two visits. CONCLUSIONS: The Greek version of the CSHQ CF, but particularly the PF has proved to be a very reliable clinical instrument, which can be used in clinical trials for assessing sleep quality in school-aged children with sleep disturbances because of obstructive airway disorders, such as AR.

Assessment of IgE-mediated food allergies in children with atopic dermatitis.

BACKGROUND: Atopic dermatitis (AD) is an inflammatory disease of the skin, which is characterised by a chronic relapsing course. AIM: The aim of the study was to assign the prevalence of clinically active food allergies among a group of children between 3 months and 7 years of age, with AD. METHODS: Eighty-eight children with AD were screened for specific IgE antibodies to food proteins. All patients with AD and specific IgE antibodies to food proteins were subjected to Oral Food Challenges (OFCs) with the relevant foods. RESULTS: Food-sensitised patients with moderate levels of sIgE had clinically active food allergy to milk (39.28%) and egg (42.34%) on the basis of positive OFCs. High IgE and eosinophilia had a prevalence of almost 80% and 25%, regardless of concomitant food sensitisation and disease severity. CONCLUSIONS: In this study, clinically active food allergies were recognised in 26.13% of children with AD. Nevertheless, no association was confirmed between food sensitisation and AD severity. High IgE and peripheral eosinophilia have not been found more prevalent among children with severe AD nor among children with food sensitisation. Infants and younger children with AD should be screened for an underlying food allergy, regardless of disease severity.

Heterozygous liver transplantation for maple syrup urine disease: First European reported case.

MSUD is an autosomal recessive metabolic disorder that results from a defect in the BCKDH enzyme. This enzyme is essential for the second step in the metabolism of the branched-chain amino acids, leucine, isoleucine, and valine. Patients with MSUD are subject to severe, irreversible neurologic injury unless closely managed with a specialized metabolic formula and a diet restricted in leucine throughout their lifetime. During times of illness, patients with MSUD can suffer from severe metabolic derangement, acute cerebral edema, and untimely death. Deceased donor liver transplant restores the ability to metabolize branched-chain amino acids, even on an unrestricted diet, and prevents metabolic derangements during times of illness. We report a successful case of living donor (parental) transplant for a child with MSUD. The donor was the child's father. This approach has been controversial as parents of children with MSUD are obligate heterozygotes for the condition and have diminished levels of BCKDH activity. If effective, living-related donor transplant provides a promising alternative for deceased donor liver transplant, which often requires a prolonged waiting period and may not be feasible in areas with limited medical resources.

Safety of a thickened extensive casein hydrolysate formula.

OBJECTIVES: Cow's milk allergy (CMA) is treated in formula-fed infants with an extensive protein hydrolysate. This study aimed to evaluate the nutritional safety of a non-thickened and thickened extensively casein hydrolyzed protein formula (NT- and T-eCHF) in infants with CMA. METHODS: Infants younger than 6 mo old with a positive cow milk challenge test, positive IgE, or skin prick test for cow milk were selected. Weight and length were followed during the 6 mo intervention with the NT-eCHF and T-eCHF. RESULTS: A challenge was performed in 50/71 infants with suspected CMA and was positive in 34/50. All children with confirmed CMA tolerated the eCHF. The T-eCHF leads to a significant improvement of the stool consistency in the whole population and in the subpopulation of infants with proven CMA. Height and weight evolution was satisfactory throughout the 6 mo study. CONCLUSIONS: The eCHF fulfills the criteria of a hypoallergenic formula and the NT- and T-eCHF reduced CMA symptoms. Growth was within normal range.

Constipation in Childhood. An update on evaluation and management.

OBJECTIVE: Constipation is a frequent problitalic in childhood and may be defined as delay or difficulty in defecation that persists for longer than two weeks. It is one of the ten most frequent pathological conditions that a general paediatrician deals with. The aim of this review is to provide the general paediatrician an overview of constipation in children discussing the etiology, differential diagnosis, signs and symptoms and patient evaluation. METHODS: We provide an overview on the pathogenesis, the diagnostic approach and the managitalicent of constipation based on electronic literature searches using the best available evidence from PubMed, Medline, Google Scholar, the European Society for Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) and the North American Society of Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN). RESULTS: The most common type of constipation is functional accounting for 90-95% of all cases. In a small percentage of children, who may have an organic cause of constipation, an appropriate laboratory investigation and imagine studying is warranted. CONCLUSIONS: Functional constipation ritalicains a frequent problitalic in childhood. Treatment options differ between infants and children. italicphasis on recommended regimens for maintenance and how to reduce medication will help to improve the long-term outcome. Moreover, it is of great importance to follow constipated children closely and restart medication promptly. On treatment failure or on suspicion of organic disease the patient should be referred for further evaluation. Hippokratia 2015, 19 (1): 11-19.

Wireless Capsule Endoscopy Detects Meckel's Diverticulum in a Child with Unexplained Intestinal Blood Loss.

Meckel's diverticulum (MD) is the most common congenital anomaly of the gastrointestinal (GI) tract, affecting about 2% of the population. Most cases of Meckel's diverticula are asymptomatic. The diagnosis of symptomatic MD is often difficult to make. We report the case of an 8-year-old boy who presented with GI bleeding due to MD. The diagnostic difficulties after an initial negative endoscopic evaluation and the diagnostic value of the various endoscopic procedures are discussed. The patient had suffered from bright red stools for 20 h before hospital admission. GI scintigraphy with (99m)Tc-Na-pertechnetate was negative for heterotopic gastric tissue in the small bowel area. Colonoscopy performed in order to exclude Crohn's disease was also negative. He was placed on ranitidine at a dose of 6 mg/kg body weight twice daily. The patient remained asymptomatic over a period of 6 months before he was readmitted due to macroscopic rectal bleeding. Upper endoscopy and colonoscopy used to investigate the source of bleeding showed normal macroscopic findings. Radiolabeling of blood constituents with (99m)Tc on delayed imaging showed radionucleotide concentration in the ascending and transverse colon suggestive of a lesion in the ileocecal area. Further investigation with the use of wireless capsule endoscopy revealed a MD. Wireless capsule endoscopy may thus be indicated for patients with GI blood loss when other diagnostic methods, such as upper and lower endoscopy and colonoscopy, have failed to identify the source of bleeding.

Dietary interventions for primary allergy prevention in infants.

Allergy prevention remains a vexing problem. Food sensitization frequently occurs early in life and is often the first sign of future atopic disease. Therefore, interventions to prevent food allergies and the development of the atopic phenotype are best made early in life. The results of studies regarding the effects of breast-feeding and the prevention of allergy remain inconclusive. Several factors in breast milk either induce or protect against food allergies. Probiotic and prebiotic supplemented whey hydrolysate formulas need further research in order to determine the future of this intervention in the prevention of food allergies. Several dietary manipulations in infancy, such as prolonged breast feeding, maternal avoidance diets during pregnancy and lactation, the use of hypoallergenic formulas, have been proposed as ways of altering the Th1/Th2 balance in infants, with varying degrees of success. Studies have examined whether food atopy can be prevented by controlling the intake of highly allergenic foods by a high-risk infant from a variety of sources, that is, both direct ingestion and indirect ingestion through the breast milk. The previous studies showed that in high risk infants who are unable to be completely breast fed, there is evidence that prolonged feeding with a hydrolysed formula compared to a cow's milk formula reduces infant and childhood allergy and infant cow's milk allergy ,while other studies reported that an antigen avoidance diet for high risk mothers is unlikely to reduce the atopic diseases in their children substantially, and that such a diet may adversely affect maternal and/or fetal nutrition.

[Congenital muscular dystrophy with laminin-a2 deficiency in early infancy: diagnosis and long-term follow-up]. / Kongenitale Muskeldystrophie mit Merosin-(Laminin-a2)-Mangel im frühen Säuglingsalter: Diagnostik und Langzeitverlauf.

Congenital muscular dystrophy (CMD) is a heterogeneous group of neuromuscular disorders characterized by muscle weakness and hypotonia at birth or within the first few months of life. It is inherited in an autosomal recessive pattern. About half of the patients have a deficiency of the alpha-2-chain of laminin (merosin). We describe a case of congenital muscular dystrophy in an infant with laminin-a2-chain deficiency, which appeared hypotonia in early infancy. Diagnosis was made by clinical features and the histological and immunohistochemical studies on muscle biopsy.

Cornstarch thickened formula reduces oesophageal acid exposure time in infants.

BACKGROUND: Infant regurgitation is a phenomenon causing worldwide parental distress and anxiety. Parental reassurance and dietary advices regarding feeding techniques and volumes are helpful in the management. Guidelines also recommend the use of thickened formula. However, the impact of thickened feeding on the frequency of acid reflux is still a matter of debate. Therefore, we evaluated the effect of a casein predominant formula thickened with a specifically selected and treated cornstarch on the frequency and duration of acid reflux episodes. METHODS: Ninety-six formula-fed infants with a mean age of 93 days, presenting with episodes of regurgitation and vomiting occurring more than five times a day and with an abnormal oesophageal pH monitoring, were randomised to a regular infant formula (n = 45) or cornstarch thickened casein predominant formula (n = 51) for 28 days. A second pH monitoring was performed at the end of the study period (26+/-5 days). Symptoms were daily recorded in a diary by the parents for 28 days. RESULTS: At inclusion, the pH-metric parameters did not differ between the control and the intervention group. Results of pH monitoring at baseline and at the end of the study did not differ in the control group on the regular infant formula. However, in the group with the casein dominant cornstarch thickened formula, all pH-metric parameters (reflux index (% of the investigation time with a pH < 4.0), number of reflux episodes >5 min, duration of the longest reflux episode) decreased significantly. The frequency of vomiting and regurgitation did not differ between both groups at baseline, remained unchanged in the control group, but decreased significantly in the intervention group. CONCLUSION: A casein dominant formula thickened with a specifically treated cornstarch reduces oesophageal acid exposure, and reduces the frequency of clinical symptoms.