RESUMEN
Two firstborn, breast-fed infants (delivery at home) were admitted to the hospital in a critical state of hypernatraemic dehydration. Case 1, a boy aged 13 days, had suffered 1220 g loss of weight since birth (31%), his serum sodium concentration was 180 mmol/l. Case 2, a girl aged 7 days, had lost 610 g since birth (18%); her serum sodium level was 159 mmol/l. In both cases poor professional support of lactation and lack of weight control had resulted in unnoticed severe malnutrition. After slow rehydration recovery was uneventful. Closer monitoring of babies' weight, e.g. twice a week, is advocated especially for breast-fed firstborns in the early weeks of life.
Asunto(s)
Lactancia Materna/efectos adversos , Deshidratación/etiología , Hipernatremia/etiología , Trastornos Nutricionales/etiología , Femenino , Humanos , Hipernatremia/sangre , Hipernatremia/diagnóstico , Recién Nacido , Masculino , Tamizaje Neonatal/normas , Países Bajos , Trastornos Nutricionales/sangre , Trastornos Nutricionales/diagnóstico , Sodio/sangre , Pérdida de PesoRESUMEN
We report on a four generation family with Greig cephalopolysyndactyly syndrome. The clinical variability of the malformations of hands and feet is described. A review of the literature is given, with emphasis on the frequency of clinical signs of the Greig cephalopolysyndactyly syndrome. Polydactyly of the hands and feet are possibly an underestimated feature in Greig syndrome. We suggest that radiographs of the hands and feet should be performed in every patient, especially those with broad thumbs. The resemblance of preaxial polydactyly type 4 and crossed polydactyly with Greig syndrome is discussed. These disorders may be allelic.