Right-sided congenital diaphragmatic hernia and myelomeningocele: a rare association

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

Effectiveness of simple strategies in reducing multidrug resistant blood stream infections in neonatal intensive care unit of tertiary care hospital in Karachi, Pakistan

To report reduction in transmission of multidrug resistant organisms from the neonatal intensive care unit after the implementation of simple risk-reduction strategies. Using a pre-and-post design, the study was carried out from June 2010 to December 2011 at the neonatal intensive care unit of Aga Khan University Hospital, Karachi, which is 12-bed, level III facility. The intervention comprised hand washing certification for all staff, use of chlorhexidine instead of povodine iodine for skin preparation, use of non-sterile gloves for diaper change, implementation of barrier nursing for clinically-suspected and culture-proven infections, provision of separate intubation and central line trolley for each room and limiting the use of umbilical catheters to 7 days. Data is reported for 3-month pre-intervention period, one-month implementation phase, and for 3-month post-intervention phase. Data for 12 months post-implementation is reported to show sustainability. The average pre intervention rates of bloodstream infections due to extended spectrum beta lactamase, Acinetobacter, Pseudomonas and methicillin resistant staphylococcus aureus were 4.7, 3.3, 1.2 and zero respectively. The average number of admissions during the 3 phases was almost similar [49, 46 and 53 respectively]. There was sustained reduction in rates for all organisms 12 months after the intervention period. Nosocomial transmission of multi drug resistant organisms within the neonatal intensive care unit can be effectively reduced by adopting simple strategies

Short-term neonatal outcome in late preterm vs. term infants

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

Clinical and biochemical characteristics of children with juvenile idiopathic arthritis

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

Pneumothoraces in a neonatal tertiary care unit: case series

Neonatal pneumothoraces are associated with high mortality Prompt recognition to minimize its complications is paramount for ultimate outcome of these babies, A retrospective case series study was carried out at Aga khan University Hospital, from January 2010 to December 2010 to determine the etiology and outcome of neonates with pneumothorax in a neonatal tertiary care unit, Ten neonates diagnosed radiologically with pneumothoraces were included. M: F ratio was 1:2.3, Birth weight ranged from 1750-3600 grams with a mean of 2100 grams. The occurrence of pneumothoraces was 50% on the left side, 20% on right, and 30% were bilateral. Primary etiology included pneumonia and sepsis [30%], hyaline membrane disease [20%], meconium aspiration syndrome [20%] and congenital diaphragmatic hernia [10%], Spontaneous pneumothoraces were present in 20% of cases. In our study, the incidence of neonatal pneumothoraces was 2.5/1000 births compared to 10-15/1000 in Denmark, 10-20/1000 in Turkey and 6.3/1000 from Vermont Oxford Group Despite the small number of cases, one incidental finding was the occurrence of pneumothorax, which declined in elective cesarean section after 37 weeks gestation i.e., 1.3 of 1000 births. Mortality was 60% determined mainly by the primary etiology and other co-morbid conditions. The study showed a higher number of mortality cases [60%]. Although, it was difficult to draw a conclusion from the limited number of cases, there may be a benefit on neonatal respiratory outcome to be obtained by better selection of mothers and by waiting until 37 weeks before performing elective cesarean section. Adequate clinician training in soft ventilation strategies will reduce the occurrence of pneumothoraces

Salmonella cholecystitis: atypical presentation of a typical condition

Salmonella cholecystitis is a rare but important complication of Salmonella typhi infection. We are reporting an 11 years old female child who presented with complaints of high-grade fever, jaundice and right sided abdominal pain [Charcot's triad]. Her examination showed tender hepatomegaly. Initial blood results revealed high white cell counts with left shift, deranged liver function tests. Abdominal ultrasonography revealed distended gallbladder with minimal layer of sludge seen within its lumen along with streak of pericholecystic fluid. Blood culture grew Salmonella typhi. She was successfully treated with intravenous ceftriaxone.

Icterus neonatorum in near-term and term infants an overview

Neonatal jaundice is the yellowish discoloration of the skin and/or sclerae of newborn infants caused by tissue deposition of bilirubin. Physiological jaundice is mild, unconjugated [indirect-reacting] bilirubinaemia, and affects nearly all newborns. Physiological jaundice levels typically peak at 5 to 6 mg/dL [86 to 103 micro mol/L] at 72 to 96 hours of age, and do not exceed 17 to 18 mg/dL [291-308 micro mol/L]. Levels may not peak until seven days of age in Asian infants, or in infants born at 35 to 37 weeks' gestation. Higher levels of unconjugated hyperbilirubinaemia are considered pathological and occur in a variety of conditions. The clinical features and management of unconjugated hyperbilirubinaemia in healthy near-term and term infants, as well as bilirubin toxicity and the prevention of kernicterus, are reviewed here. The pathogenesis and aetiology of this disorder are discussed separately

Neonatal hypothermia among hospitalized high risk newborns in a developing country

To estimate the frequency of neonatal hypothermia and to identify the risk factors associated with it. This cross sectional survey study was conducted from March 2008 to November 2008 in the Neonatal Intensive Care Unit, Department of Women and Child Health, Aga Khan University Hospital, Karachi, Pakistan. Data was collected with a pre tested data collection tool. Three hundred neonates were included in the study. SPSS v17.0 was used for descriptive analysis. Of the 300 neonates admitted to the neonatal unit, 144 [49.5%] developed hypothermia. The frequency of hypothermia was found to be significantly higher in low birth weight [LBW] 58.1% and preterm 64.2% babies. There is a high frequency of neonatal hypothermia in our setup which is on the higher side of the range reported in comparable regions. LBW and prematurity were independent risk factors as was the external environment at the time of delivery

Juvenile dermatomyositis

Juvenile dermatomyositis [JDM] is an important subtype of dermatomyositis characterized by inflammation of muscle, skin and gastrointestinal tract. A 14-year-old girl, with a history of fever, joint pain, easy fatigability and a rash since the age of 3 years is described. Physical examination, laboratory evaluation, electromyography [EMG] and muscle biopsy were suggestive of a chronic inflammatory process involving the muscles, most likely dermatomyositis. The report highlights the importance of a muscle biopsy as the gold standard for diagnosing dermatomyositis

Asthma in children management issues for family doctors

Asthma is still one of the leading causes of morbidity in children. Despite the improved understanding in the disease pathogenesis and availability of the different classes of drugs, the incidence of emergency visits due to acute exacerbations and admission rates due to frequent and uncontrolled disease is fairly high. Management of bronchial asthma in children is quite different to that of adults. Although there are universal guidelines available for the management of childhood asthma, there is still confusion especially among the family physicians who are largely involved in the management of the children, both in acute exacerbations as well as in long term prevention. This article aims to simplify all the management issues for family physicians in concurrence with the available asthma management guidelines