RESUMO
Congenital diaphragmatic hernia [CDH] is a rare birth defect with a prevalence of < 0.5 per 1,000 live births. Majority of these defects are left-sided as most studies suggest that frequency of right-sided CDH was 10% of the total. The association of CDH with myelomeningocele [MMC] is extremely rare; as in Sweed's study of 116 consecutive cases of CDH, the incidence of associated MMC with CDH was stated as 4.3%. There has been one previous case report of leftsided CDH, MMC and hydrocephalus prenatally diagnosed; but to the best of authors' knowledge, this is the first reported case of the above constellation with a right-sided CDH diagnosed prenatally
RESUMO
To determine the short-term neonatal outcomes in late preterm infants [LPI's] as compared to term infants and their association with maternal risk factors. A case control, descriptive study. The Aga Khan University Hospital, Karachi, Pakistan, from January to December 2009. The study included 326 late preterm babies [defined as those born between 34 0/7 to 37 6/7 weeks of gestation] and equal number of term control babies at the Aga Khan University Hospital, Karachi, Pakistan. Data, including obstetric history, maternal complications, neonatal morbidities, etc., was retrieved from patients' medical records. The data was compared with the control group for complications, fetal morbidity and maternal morbidity. Late preterm infants constituted 10.6% of all deliveries and 77% of all live preterm births during the study period. Respiratory distress syndrome [RDS] [16.5% vs. 0.3%, p < 0.001], growth retardation [24.8% vs. 4%, p < 0.001], hyperbilirubinemia requiring phototherapy [37.9% vs. 11%, p < 0.001], and sepsis [4.9% vs. 0.3%, p < 0.001] were found to be the major morbidities in the study group. The need for resuscitation was 12.7 times higher in the study group as compared to the term babies [21.4% vs. 1.2%, p < 0.001]. NICU admissions in the study group were also higher [18.8% vs. 2.4%, p < 0.001]. Hypertension [12.5% vs. 1.5%, p < 0.001], diabetes [12.5% vs. 9.2%, p < 0.001], antenatal history of UTI [1.5% vs. 0.3%, p < 0.001], and prolong rupture of membrane [8.9% vs. 4%, p < 0.001] were significant maternal morbidities in the late preterm group. The late preterm group had greater morbidity, compared to term neonates. Prior awareness of the morbidities associated with late preterm babies is helpful for the health care providers to anticipate and manage potential complications in late preterm infants
RESUMO
To determine the clinical and biochemical characteristics of children with Juvenile Idiopathic Arthritis [JIA] at a tertiary care centre in Karachi, Pakistan. A descriptive study. Paediatric Rheumatology Clinic of The Aga Khan University Hospital [AKUH], Karachi, from January 2008 to December 2011. Clinical and laboratory profile and outcome of children less than 15 years of age attending the Paediatric Rheumatology Clinic of the Aga Khan University, Karachi with the diagnosis of Juvenile Idiopathic Arthritis according to International League against Rheumatism were studied. These children were classified into different types of JIA; their clinical and laboratory characteristics, response to therapy and outcome was evaluated. Sixty eight patients satisfying the criteria of International League against Rheumatism [ILAR] for Juvenile Idiopathic Arthritis were enrolled during the study period of four consecutive years, their age ranged from 9 months to 15 years. Mean age at onset was 6.45 +/- 4.03 years while mean age at diagnosis was 7.60 +/- 3.93 years. Polyarticular was the most predominant subtype with 37 [54%] patients, out of these, 9 [24%] were rheumatoid factor positive. An almost equal gender predisposition was observed. Fever and arthritis were the most common presenting symptoms, with only 2 patients presenting with uveitis. The clinico-biochemical characteristics of JIA at the study centre showed a pattern distinct with early onset of disease, high frequency of polyarticular type and a higher rheumatoid factor [QRA] and ANA positivity in girls