A case of Candida septic arthritis with rice body formation in a 2-month-old infant.

We present a case of rice body formation in the left knee joint of a 2-month-old infant affected by Candida albicans septic arthritis which has never been reported before. Rice body formation has been described in association with rheumatoid or tuberculous arthritis and is very rare in Candida arthritis. After three weeks of therapy with amphotericin B administered intravenously, the infant recovered fully from infection. Septic arthritis is a serious cause of morbidity and for proper evaluation and treatment fungal septic arthritis should be included in the differential diagnosis.

Wide spectrum of congenital anomalies including choanal atresia, malformed extremities, and brain and spinal malformations in a girl with a de novo 5.6-Mb deletion of 13q12.11-13q12.13.

A 2 ½-year-old girl with multiple congenital anomalies and a de novo 5.6-Mb deletion on chromosome 13q12.11-13q12.13 is reported. She showed choanal atresia, scalp aplasia cutis, mild dysmorphic features, severe malformation of the hands and feet, Sylvian aqueductal stenosis, hydrocephalus, small cerebellum with pointed cerebellar tonsils, cervical, lumbar and sacral clefting, single central incisor and mild developmental delay. The girl's anomalies were compared with: (A) one boy reported by each of Der Kaloustian et al. [2011] and Tanteles et al. [2011] with similar, albeit smaller, 2.1 to 2.9 Mb deletions in which the abnormalities consisted of mild facial dysmorphism, mild malformations of the fingers and/or toes, and developmental delay; (B) one girl reported by Friedman et al. [2006] with similar, albeit larger, 5.7 Mb deletion with mild developmental delay and haematological abnormalities; (C) one girl reported by Slee et al. [1991] with a deletion of band q12.2 in chromosome 13, who had Moebius syndrome with facial dysmorphism, high arched palate, micrognathia, and small tongue with no abnormalities of the extremities; and (D) seven additional individuals recorded in the DECIPHER 6.0 database who all had dysmorphic features and developmental delay plus a spectrum of clinical manifestations including deafness, ataxia/oculomotor apraxia, spasticity, small testes, and mild fingers' anomalies. The deleted region hereby reported encompassed 34 known genes, including GJA3, GJB2, and GJB6, which are responsible for autosomal recessive deafness, FGF9, which plays crucial roles in embryonic neurological development, and ATP8A2, which causes a cerebellar ataxia and disequilibrium syndrome.

An extra mouth in a newborn: a unique case of facial duplication.

An accessory mouth is a very rare condition, with approximately 15 cases reported in the literature to date. We describe another unique case and discuss its possible embryopathogenesis.

Primary dietary prevention of food allergy.

OBJECTIVE: To present research and clinical evidence on the use of primary dietary prevention in food allergy management. DATA SOURCES: We conducted MEDLINE searches for pertinent articles published between January 1986 and October 2001 with use of the following keywords or phrases: prevention and diet and allergy, food allergy and prevention, and dietary prevention and food allergy or allergens. Also included are information and commentary reflecting the authors' cumulative clinical experience in an allergy unit of a city hospital. RESULTS: We define as "proactive" those strategies centered on "success factors," such as the early postnatal environment, prolonged breast-feeding, and use of formula and probiotic supplementation, in contrast to earlier "prohibitionist" approaches to prevention of food allergy. These two approaches are not antagonistic and may even be synergistic. We introduce this distinction in light of epidemiologic evidence and out of concern about compliance and the quality of life for patients. CONCLUSIONS: Inasmuch as nutritional and immune maturation are implicated, the prohibitionist measures that are most effective in primary prevention of food allergy are exclusive breast-feeding for at least 6 months (for lifelong immunity and other benefits), delayed (after the sixth month) introduction of solid foods, and on-indication use of "hypoallergenic" formulas. Whether proactive strategies can be contemplated remains a debatable issue. Evidence for and against the scientific use of probiotics as well as microbiologic, epidemiologic, and clinical data are discussed. Review of published epidemiologic studies and randomized clinical trials is essential before planning dietary intervention or prevention.

Management of bovine protein allergy: new perspectives and nutritional aspects.

OBJECTIVES: Awareness of the considerable incidence of bovine protein allergy in infancy makes it necessary to eliminate cow's milk antigens from the diet in special cases. The primary objective of this review is to discuss the nutritional aspects and appropriate use of hypoallergenic formulas as a substitute for cow's milk formulas and to present new alternative feeding modalities in this field. DATA SOURCES: Related articles in PubMed (National Library of Medicine) were reviewed. This review is a synthesis of these sources along with the discussions with experts in this field and the expert opinion of the authors. RESULTS: Soy protein-based and hydrolyzed milk protein formulas are the most commonly used alternative protein sources in the case of bovine protein allergy. Despite the adequacy of their nutritional values, there are still some problems to be solved regarding these formulas. In addition, a considerable percentage of the infants with bovine protein allergy may also present allergenic reactions to soy proteins as well as to hydrolyzed milk proteins. Thus, there still exists chaos in the selection of the most appropriate formula for infants sensitized to cow's milk protein. Rice protein-based formulas, probiotics, and prebiotics are expected to be new effective alternatives. CONCLUSIONS: Bovine protein allergy constitutes an important place in childhood food allergies. Soy protein-based and hydrolyzed protein formulas have some disadvantages and risk of allergenic activity that is not to be underestimated. So, substitution of cow's milk protein with an alternative protein source in sensitized infants has become an art rather than a science. Rice protein, prebiotics, and probiotics appear to be valuable alternatives giving hope for the future.