Short-term functional outcome in children with arthrogryposis multiplex congenita after multiple surgeries at an early age

Moutasem M Obeidat,1 Ziad Audat,1 Wadah Khriesat21Department of Orthopedic Surgery, 2Department of Pediatrics, Jordan University of Science and Technology – King Abdullah University Hospital, Irbid, JordanPurpose: The purpose of this study is to report our short-term functional outcome for 14 children with arthrogryposis multiplex congenita (AMC) who underwent multiple surgical procedures at an early age.Methods: During the period 2002–2010, 14 children (11 males and three females) with AMC underwent multiple surgical procedures to treat deformities of the lower and upper limbs. About 81 procedures were performed, at a rate of 5.9 procedures per child. The mean age at the last follow-up was 5.9 years. The average follow-up period was 3.6 years (range, 1.5–6 years). The functional outcome assessment included walking ability and the activities of daily living for the upper limb function.Results: At the last follow-up visit, six (43%) children (four males, two females) with a mean age of 8.3 years (range, 4–15) were independent walkers. Three children (males) with a mean age of 3.5 years (range, 2.5–5) were able to walk, but with support. One child (male), 3 years old, was a household ambulator. Three children (two males, one female) with a mean age of 4.2 years (range, 2.5–6) were nonfunctional ambulators. The last child (male) was nonambulatory at the age of 5 years. Activities of daily living were severely affected in the nonambulatory child. One child in the nonfunctional ambulators group had limitations in the activities of daily living; however, upper limb function was not affected in the remaining 12 children.Conclusion: We believe that aggressive surgical treatment using multiple operations at an early age can improve the short-term functional and clinical outcomes of children with AMC.Keywords: deformity, multiple surgeries, functional outcom

Intravenous immunoglobulin to treat hyperbilirubinemia in neonates with isolated Glucose-6-Phosphate dehydrogenase deficiency

Background Glucose-6-phosphate dehydrogenase deficiency alone or concomitant with ABO isoimmunisation is a widespread indication for neonatal exchange transfusion. Aims To evaluate the effectiveness of Intravenous Immunoglobulin in the treatment of neonatal hyperbilirubinemia due to glucose-6-phosphate dehydrogenase deficiency. Methods A retrospective cohort study was conducted between 2006 and 2014 at the Jordan University of Science and technology. The medical records of 43 infants admitted to the neonatal intensive care unit for isolated glucose-6-phosphate dehydrogenase deficiency hemolytic disease of the newborns were reviewed. Patients were divided into two groups. Group I, a historical cohort, included newborns born between 2006 and 2010, Treatment included phototherapy and exchange transfusion. Group II included newborns born between 2011 and 2014, where, in addition to phototherapy, intravenous immunoglobulin was administered. The duration of phototherapy and number of exchange transfusions were evaluated. Results Of 412 newborns that were admitted with neonatal hyperbilirubinemia, Glucose-6-phosphate dehydrogenase deficiency was present in 43. Of these, 22, did not receive intravenous immunoglobulin and served as a control group. The other 21 newborns received intravenous immunoglobulin. There was no difference in the demographic characteristics between the two groups. Infants in the control group were significantly more likely to receive exchange blood transfusion than infants in the immunoglobulin treatment group, but were significantly less likely to need phototherapy. Conclusion Intravenous immunoglobulin is an effective alternative to exchange transfusion in infants with glucose-6-phosphate dehydrogenase deficiency hemolytic disease of the newborn. It is suggested that intravenous immunoglobulin may be beneficial as a prophylaxis for infants with hyperbilirubinemia

Infant Resuscitation Practices of Midwives in a Developing Country

Background: Effective basic newborn resuscitation is an important strategy to reduce the incidence of birth asphyxia and associated newborn outcomes. Outcomes for newborns can be markedly improved if health providers have appropriate newborn resuscitation skills. Purpose: To evaluate the skills of midwives in newborn resuscitation in delivery rooms in Jordan. Methods: Data were collected from observation of 118 midwives from National Health Service hospitals in the north of Jordan who performed basic newborn resuscitation for full-term neonates. A structured checklist of 14 items of basic skills of resuscitation was used. Descriptive statistics were used to analyze the data. Results: The results highlighted the lack of appropriate performance of the 8 necessary skills at birth by midwives. About 17.8% of midwives had performed the core competencies at birth (ie, assessing breathing pattern/crying, cleaning airways) appropriately and met the standard sequence. Less than half of midwives assessed skin color (40.7%) and breathing pattern or crying (41.5%) appropriately with or without minor deviations from standard sequences. Of the 6 skills that had to be performed by midwives at 30 seconds up to 5 minutes after birth, 4 skills were not performed by about one-quarter of midwives. Implications for Practice and Research: The midwives' practices at the 2 hospitals of this study were not supported by best practice international guidelines. The study showed that a high proportion of midwives had imperfect basic newborn resuscitation skills despite a mean experience of 8 years. This highlights the critical need for continuing medical education in the area of basic newborn resuscitation. The results highlight the need for formal assessment of midwives' competence in basic newborn resuscitation. National evidence-based policies and quality assurance are needed to reflect contemporary practice