RESUMO
Objective: to report a rare case of newborn male presented with right anophthalmia and left unilateral complete cleft lip and palate
RESUMO
Bart's syndrome is defined as congenital localized absence of skin, oral mucosal lesions and dystrophic nails associated with epidermolysis bullosa. It is an inherited condition as autosomal dominant with complete penetrance but variable expression. We report a rare case of a near term male newborn born to non-consanguineous parents who presented with congenital absence of skin in scalp, face, trunk and extremities. He has dystrophic and deformed fingernails with multiple areas of blistering and erosions. Clinical appearance and skin biopsy was sufficiently distinct to suggest the diagnosis of Bart syndrome
RESUMO
To show that intravenous immunoglobulin is safe and effective alternative treatment in newborn with iso-immune hemolytic jaundice. This study was carried out in neonatal unit in King Hussein Medical Center [KHMC] and Queen Alia Hospital during two years period of all cases who presented with jaundice either RH or ABO iso-immune jaundice in the first day of life. All patients received phototherapy and intravenous immunoglobulin. There were 91 patients with iso-immune jaundice admitted to the neonatal unit in their first day of life. Eighty five patients out of 91 [93.4%] were due to ABO incompatibility. Six patients out of 91 [6.6%] were due to RH incompatibility. Direct coombs test was positive in all cases of RH incompatibility and in 16 cases of ABO incompatibility. Intravenous immunoglobulin [0.5g/kg] was given to all patients over four hours every 24 hours for three doses. Exchange transfusion was done for one patient only [1%]. Blood transfusion was given for 22 patients out of 91 [24%]. Hospital stay was around 3-4 days in majority of cases. Intravenous immunoglobulin in newborn with ABO or RH hemolytic jaundice is effective in reducing hemolysis, serum bilirubin level and the need for exchange transfusion