[Leukocyte adhesion deficiency syndrome and report of three cases]

Leukocyte adhesion deficiency syndrome [LADS] is a rare inherited immunodeficiency in which the frunction of leukocytes, particularly the phagocytes are disturbed. In the present study, we report three cases with LADS [2 male infants and 1 female child] who had a history of delay in separation of umbilical cord. One infant admitted for skin infection and two patients admitted in hospital following bacterial and fungal infections in nose and finger. All cases had a remarkable leukocytosis, neutrophilia and decreased of CD 11 and CD 18. LADS is a congenital syndrome causes recurrent bacterial and fungal infections. Usually all patients have a history of delayed separation of umbilical cord

Evaluation of Auditory Brainstem Response [ABR] in neonatal hyperbilirubinemia

Jaundice is a common problem in newborns and clinical presentations resulting from early stages of hyperbilirubinemia toxicity. ABR is useful test to recognize the primary brain stem encepholapathy. This study has been carried out with the objective of evaluating the ABR in recognizing hearing disorders in newborns suffering from Jaundice. In this study, we describe ABR obtained in 60 full term newborns, with birth weight more than 2500 gr, and Bilirubin concentration Between 18-54mg/dl without immune hemolysis and hearing disorder risk factor, from march 2005 until January 2006. ABR was carried out before commencing therapy, While first ABR was abnormal, repeated ABR was done after treatment exactly after 3 months. In this study, sixty newborns were evaluated by ABR for detecting early hearing disorder. Mean weight were 3000 +/- 250 gr, Gestational age 38 +/- 1 weekes, bilirubin concentration 36 mg/d.l +/- 9 [18-54 range]. 17 patients had abnormal ABR pretreatment, and 12 patients done normal ABR second evalution after treatment [Bili: 26 +/- 3], 5 patients had persistent abnormal [mean Bili: 43 +/- 5]. ABR abnormalities includes latencies wave I,III,V and loger duration interpeaks I-V,I-III and III-V. [Mean values of ABR latencies when compared with a group of normal babies as a control group were significantly increased before treatment, but returned to normal value after treatment [p=0.001]. Our study proved that newborns with serum bilirubin ranging from 18 to 54 mg/dl would be increase in ABR waves I,III,V and also increase in interpeak I-III,III-V,I-V that may be irreversible in those infants with bilirubin more than 32 mg/dl