Symptomatic hypogammaglobulinemia in infancy and childhood – clinical outcome and in vitro immune responses

BACKGROUND: Symptomatic hypogammaglobulinemia in infancy and childhood (SHIC), may be an early manifestation of a primary immunodeficiency or a maturational delay in the normal production of immunoglobulins (Ig). We aimed to evaluate the natural course of SHIC and correlate in vitro lymphoproliferative and secretory responses with recovery of immunoglobulin values and clinical resolution. METHODS: Children, older than 1 year of age, referred to our specialist clinic because of recurrent infections and serum immunoglobulin (Ig) levels 2 SD below the mean for age, were followed for a period of 8 years. Patient with any known familial, clinical or laboratory evidence of cellular immunodeficiency or other immunodeficiency syndromes were excluded from this cohort. Evaluation at 6- to 12-months intervals continued up to 1 year after resolution of symptoms. In a subgroup of patients, in vitro lymphocyte proliferation and Ig secretion in response to mitogens was performed. RESULTS: 32 children, 24 (75%) males, 8 (25%) females, mean age 3.4 years fulfilled the inclusion criteria. Clinical presentation: ENT infections 69%, respiratory 81%, diarrhea 12.5%. During follow-up, 17 (53%) normalized serum Ig levels and were diagnosed as transient hypogammaglobulinemia of infancy (THGI). THGI patients did not differ clinically or demographically from non-transient patients, both having a benign clinical outcome. In vitro Ig secretory responses, were lower in hypogammaglobulinemic, compared to normal children and did not normalize concomitantly with serum Ig's in THGI patients. CONCLUSIONS: The majority of children with SHIC in the first decade of life have THGI. Resolution of symptoms as well as normalization of Ig values may be delayed, but overall the clinical outcome is good and the clinical course benign

Remote links: Redesigning maternity care for Aboriginal women from remote communities in Northern Australia - A comparative cohort study.

OBJECTIVE: to compare the quality of care before and after the introduction of the new Midwifery Group Practice. DESIGN: a cohort study. SETTING: the health centers (HCs) in two of the largest remote Aboriginal communities (population 2200-2600) in the Top End of the Northern Territory (NT), each located approximately 500km from Darwin. The third study site was the Royal Darwin Hospital (RDH) which provides tertiary care. METHODS: a 2004-06 retrospective cohort (n=412 maternity cases) provided baseline data. A clinical redesign of maternity services occurring from 2009 onwards focused on increasing Continuity of Carer, Communication, Choice, Collaboration and Co-ordination of Care (5Cs). Data from a 2009-11 prospective cohort (n=310 maternity cases) were collected to evaluate the service redesign. Outcome measures included indicators on the quality of care delivery, adherence to recommended antenatal guidelines and maternal and neonatal health outcomes. FINDINGS: statistically significant improvements were recorded in many areas reflecting improved access to, and quality of, care. For example: fewer women had <4 visits in pregnancy (14% versus 8%), a higher proportion of women had routine antenatal tests recorded (86% versus 97%) and improved screening rates for urine (82% versus 87%) and sexual tract infections (78% versus 93%). However, the treatment of conditions according to recommended guidelines worsened significantly in some areas; for example antibiotics prescribed for urine infections (86% versus 52%) and treatment for anaemia in pregnancy (77% versus 67%). High preterm (21% versus 20%), low birth weight (18% versus 20%) and PPH (29% versus 31%) rates did not change over time. The out of hospital birth rate remained high and unchanged in both cohorts (10% versus 10%). CONCLUSION: this model addresses some of the disparities in care for remote-dwelling Aboriginal women. However, much work still needs to occur before maternity care and outcomes are equal to that of non-Aboriginal women. Targeted program interventions with stronger clinical governance frameworks to improve the quality of care are essential. A complete rethink of service delivery and engagement may deliver better results