Hemimegalencephaly with intractable epilepsy: A case report

Isolated Hemimegalencephaly (iHME) is a rare form of congenital malformation of cortical development.It is characterized by enlargement of all or part of one cerebral hemisphere. It typically presents with intractable seizures, mental retardation, developmental delay, contralateral hemiparesis and hemianopia. The patient was a five and half month\u27s old baby girl who presented first with focal seizures at 10th day of life. No other physical or behavioral abnormality was noted. However, Initial EEG showed excessive sharp EEG transients more over the right hemisphere, repeated EEG showed spikes, polyspikes, sharps and slow wave discharges predominately over right hemisphere. MRI brain showed asymmetric enlargement of the right cerebral hemisphere, suggestive of hemimegalencephaly. Initial treatment with anti-epileptics was successful in controlling the seizures but later on the seizures became intractable even on polytherapy. Identification of this and similar cases of iHME can help us better understand this disorder and its associated symptoms and eventually help us develop better treatment options for it

Teaching paediatric critical care medicine to paediatric residents

Critical care training during paediatric residency provides an ideal opportunity to learn and refine the skills needed in the early recognition and prompt treatment of the acutely ill paediatric patients. Paediatric critical care medicine is a relatively young sub-specialty in Pakistan. The aim of this study is to describe our experience of teaching paediatric residents in paediatric critical care medicine during paediatric intensive care unit rotation. Our paediatric critical care teaching curriculum for residents is based on the spectrum of our common critical care problems along with basic principal of critical care. The clinical rotation in our paediatric intensive care unit is very dynamic, thrilling, enjoyable and provides a lot of learning opportunities. During the rotation, the residents were exposed to all major critical care illnesses in infants and children. We use four traditional models of learning in our Paediatric Intensive Care Unit (PICU): bedside rounds, direct patient care, didactic learning and self-study. Our curriculum enhances the resident\u27s educational and clinical experience of paediatric intensive care medicine

Neurodevelopment outcome of neonates treated with intraventricular colistin for ventriculitis caused by multiple drug-resistant pathogens-A case series

Background: Multiple-drug-resistant Gram-negative bacteria (MDR-GNB)-associated neonatal ventriculitis is a life-threatening complication that needs timely diagnosis and effective treatment with broad-spectrum antimicrobials in critical-care settings. Inadequate penetration of antibiotics through the blood-brain barrier also demands an intraventricular (IVT) route of administration. This study reports mortality and neurodevelopmental sequelae of neonates till 18 months of age, who received IVT-colistin for treating MDR-GNB associated ventriculitis. Methods: In a case series of seven neonates with ventriculitis due to MDR-GNB at NICU of Aga Khan University Hospital, Pakistan, between June 2015 and 2018, we reviewed IVT-colistin therapy in critically ill neonates. Treatment outcomes were assessed based on clinical sign\u27s resolution and MDR-GNB eradication in subsequent CSF cultures. Neurodevelopmental outcomes were evaluated at 18 months after discharge. Results: The average birth weight was 1.38 kg (range: 1.02-1.5 kg), and the average gestational age was 30.7 weeks (ranged: 26-34 weeks). All neonates reported colistin-sensitive MDR-GNB in CSF, five with Acinetobacter baumannii, and polymicrobial CNS infection was found in two patients (one due to Klebsiella pneumonia and A. baumannii and one due to K. pneumonia and Escherichia coli). All neonates received IVT colistin and concomitant intravenous meropenem, and five of them also received intravenous colistin. One neonate died. At the 18-month assessment, only one neonate had cerebral palsy and hydrocephaly and 50% had seizure disorders. Conclusion: Practicing intraventricular antibiotics in the neonatal population is challenging but may be used successfully, especially to overcome the limitation of poor penetration through the blood-brain barrier

Pharmacist-directed vancomycin therapeutic drug monitoring in pediatric patients: A collaborative-practice model

Background: Therapeutic drug monitoring (TDM) of Vancomycin (VCM) is required to prevent inappropriate dosage-associated bacterial resistance, therapeutic failure, and toxicities in pediatrics. Anecdotal experience and studies show that many healthcare institutions confront barriers while implementing TDM services, this study aimed to assess a pharmacist-directed VCM-TDM service for optimizing patient care in our institution.Materials and methods: Patients aged 1 month-18 years who received intravenous VCM were included in this quasi-experimental study. The pre-implementation phase (March-June 2018) consisted of retrospective assessment of pediatric patients, the interventional phase (July 2018 to February 2020) included educational programs and the post-implementation phase (March-June 2020) evaluated the participants based on pharmacist-directed VCM-TDM services as a collaborative-practice model including clinical and inpatient pharmacists to provide 24/7 TDM services. Outcomes of the study included the mean difference in the number of optimal (i) prescribed initial VCM doses (primary) (ii) dosage adjustments and (iii) VCM-sampling time (secondary). After ethical approval, data were collected retrospectively.Results: A hundred patients were there in each phase. The number of cases who were correctly prescribed initial VCM doses was significantly higher in the post-implementation phase, mean difference of 0.22, [95% CI (0.142-0.0.358), p \u3c 0.0001]. Patients who had correct dosage adjustments in the post-implementation phase also had higher statistical significance, mean difference of 0.29, [95% CI (0.152-0.423), p \u3c 0.05]. More correct practices of VCM-levels timing were observed in the post-implementation phase, mean difference of 0.15, [95% CI (- 0.053-0.264), p = 0.079].Conclusion: This study showed the significant role of pharmacist-directed TDM services to optimize the correct prescribing of initial VCM doses and dose adjustments

Antenatal renal pelvic dilatation and foetal outcomes - review of cases from a tertiary care center in Karachi, Pakistan

Objective: To determine the incidence of antenatal renal pelvic dilatation to evaluate antenatal resolution/ progression and post-natal outcome. Methods: This retrospective study was conducted at the Aga Khan University Hospital, Karachi, and comprised data of all women found with renal pelvic dilatation in antenatal scans between January 2011 and December 2013. A cut-off of 5mm was used to diagnose renal pelvic dilatation. Renal pelvic dilatation was categorised into three groups: mild (5-6mm in second trimester and 5-9mm in third trimester), moderate (7-10mm in second trimester and 10-15 in third) and severe (more than 10mm in second trimester and more than 15mm in third trimester). Results: Of the 13,337 scans, renal pelvic dilatation was found in 111(0.8%) cases. The overall mean maternal age was 28.5 ± 4.2 years. Renal pelvic dilatation was unilateral in 52(46.8%) and bilateral in 59(53.2%) cases. Post-natal scan was done in 61(55%) cases at the discretion of the neonatologist. A pathological finding was diagnosed in post-natal scan in 19(17.7 %) cases. Pelvi-ureteric junction obstruction was found in 6(5.4%) neonates, all in the severe renal pelvic dilatation category. Conclusion: The incidence of renal pelvic dilatation was low and the outcomes were normal in majority of cases

Skeletal manifestations of scurvy: A case report from dubai

Introduction: Nutritional deficiencies are rarely reported in developed countries. We report a child of Pakistani origin brought up in Dubai who developed skeletal manifestations of scurvy due to peculiar dietary habits. Case Presentation: A 4.5 year old boy presented with pain and swelling of multiple joints for three months and inability to walk for two months. Dietary history was significant for exclusive meat intake for the preceding two years. On examination the child\u27s height and weight were below the 5th percentile for his age. He was pale and tachycardic. There was significant swelling and tenderness over the wrist, knee and ankle joints, along with painful restriction of motion. Basic blood workup was unremarkable except for anemia. However, X-rays showed delayed bone age, severe osteopenia of the long bones, epiphyseal separation, cortical thinning and dense zone of provisional calcification, suggesting a radiological diagnosis of scurvy. The child was started on vitamin C replacement therapy. Over the following two months, the pain and swelling substantially reduced and the child became able to walk. Repeat X-rays showed improvement in the bony abnormalities. Conclusion: Although scurvy is not a very commonly encountered entity in the modern era, inappropriate dietary intake can lead to skeletal abnormalities which may be confused with rickets. A high index of suspicion is thus required for prompt diagnosis of scurvy in patients with bone and joint symptoms

Meropenem-induced pancytopenia in a preterm neonate: A case report

Background: A post-marketing surveillance study has reported an association between meropenem use and the incidence of hematologic abnormalities, including leukopenia, thrombocytopenia, hemolysis, and neutropenia, but the precise incidence in neonates is unknown. Here, we report meropenem-induced pancytopenia in a preterm neonate.Case presentation: A preterm newborn Pakistani received intravenous meropenem 40 mg/kg every 8 hours to treat Klebsiella pneumoniae in blood cultures and suspected meningitis. The baby developed severe thrombocytopenia, with a platelet count of 22 × 103 cells/mm3, low hemoglobin level of 9.7 g/dl, and low absolute neutrophil count (ANC) of 816 cells/mm3 on days 3, 14, and 17 of meropenem therapy, respectively. Based on the blood culture and institutional guidelines, meropenem treatment was continued with monitoring and supportive care for a total of 19 days. After discontinuation of meropenem, the baby was monitored continuously for hematological changes, and low counts persisted for 3 days. ANC improved to \u3e 1500 cells/mm3 on the fourth day, and the platelet count reached \u3e 150 × 103 cells/mm3 for the first time on the seventh day of meropenem discontinuation. All subsequent complete blood count (CBC) reports showed improving trends. The baby was discharged on the 48th day of life (DOL), with follow-up monitoring of CBC. The baby was kept on iron supplements, and hemoglobin level of 11.2 g/dl was observed on the 59th DOL.Conclusion: Neonatal pancytopenia may lead to serious health complications; therefore, clinicians and pharmacists need to vigilantly monitor CBC in this vulnerable population, even when administering meropenem in septic doses for the recommended duration

Clinical profile and predictors of mortality in neonates born with non-immune hydrops fetalis: Experience from a lower-middle-income country

Introduction: Hydrops fetalis (HF) is a life-threatening condition in which a fetus has an abnormal collection of fluid in the tissue around the lungs, heart, abdomen, or under the skin. Based on its pathophysiology, it is classified into immune and non-immune types. With the widespread use of anti-D immunoglobulin, non-immune HF has become more common, with an incidence of one in 1,700-3,000 live births. A multitude of fetal diseases with various causes can lead to non-immune HF. Due to the recent advances in prenatal diagnostic and therapeutic interventions together with improved neonatal intensive care, the diagnosis and subsequent management of HF have been refined. However, HF is still associated with a high mortality rate. A recent assessment of the literature found that there is a lack of data on prognostic variables in neonates with HF from low- and middle-income countries. In light of this, we sought to establish the etiologic causes, predictors of mortality, and eventual fate of newborns born non-immune HF at the Aga Khan University Hospital, Karachi during the 10-year period spanning January 2009-December 2019 in this retrospective analysis.Methodology: For this study, we collected data from the computerized database and patient record files at the hospital on all infants with non-immune HF. Demographic data, postnatal interventions, clinical and laboratory findings, outcomes, and the results of comparison between HF patients who died and those who survived were analyzed.Results: The incidence of non-immune HF at our hospital was 0.62/1,000 live births during the period under study, with 33 newborn babies diagnosed with non-immune HF from a total of 53,033 live-born deliveries. An etiologic factor was discovered in 17 (51.5%) neonates with non-immune HF while 16 (48.4%) were classified as those with unidentified etiology. The most common causes were cardiovascular and genetic syndromes, which resulted in 100% mortality. The overall mortality rate was 67%. The need for mechanical ventilation, surfactant therapy, and prolonged hospitalization were identified as independent risk factors of mortality.Conclusion: Our study proves that the need for mechanical ventilation [moderate to severe hypoxic respiratory failure (HRF)] and prolonged hospitalization are strong predictors of poor outcomes in neonates with non-immune HF. Therefore, severe hydrops causing significant mortality can be anticipated based on the patients\u27 respiratory status and the need for escalated oxygen support

Retinopathy of prematurity: an evaluation of existing screening criteria in Pakistan

Abstract AIM: To evaluate if broadening the criteria for retinopathy of prematurity (ROP) screening to include babies with gestational age ≥32 weeks and/or birth weight ≥1500 g, would have an impact on the number of babies diagnosed as having ROP. METHODS: A prospective cohort study was carried out at the Aga Khan University Hospital, Karachi, Pakistan. Infants with gestational age ≤35 weeks or birth weight ≤2000 g born in this hospital from May 2010 to December 2012 were screened for the presence of ROP 4-6 weeks after birth. Subsequent examinations were performed at intervals based on the findings of initial eye examinations. Infants diagnosed as having ROP were treated with argon laser therapy. Neonatal risk factors were also assessed. Cumulative incidence of ROP was calculated for babies falling within and outside current screening criteria. Multivariate logistic regression analysis was performed to examine the predictors of ROP. RESULTS: A total of 301 infants were screened: 27 (9%) babies developed ROP, of which 19 had stage 3 ROP or worse. None of the babies falling outside the current screening criteria developed ROP. The incidence of ROP in the infants meeting the current screeningcriteria was 11.5%. Multivariate logistic regression analysis showed that only gestational age (adjusted relative risk 0.774, 95% CI 0.603 to 0.994) was independently associated with the development of ROP. CONCLUSIONS: In our population ROP was not seen to occur in infants older than 32 weeks gestational age and/or weighing more than 1500 g

Intravenous vs intravenous plus aerosolized colistin for treatment of ventilator-associated pneumonia - A matched case-control study in neonates

Background: Recently intravenous (IV) and aerosolized (ASZ) colistin have been used for treating ventilator-associated pneumonia (VAP) due to colistin susceptible multidrug-resistant Gram-negative bacteria (MDR-GNB). Colistin has limited lung penetration. We compared the efficacy and safety of IV-alone versus IV+ASZ-colistin for treating VAP in neonates.Methods: This retrospective matched case-control-study was performed at NICU of the Aga Khan University Hospital, Pakistan between January 2015 and December 2018. Sixteen neonates with MDR-GNB associated VAP received IV-ASZ-colistin and were matched for date of birth, gestational age, birth weight, Apgar score, antenatal steroid history, disease severity and duration of mechanical ventilation with 16 control neonates who received IV-colistin alone.Results: Both groups had similar MDR-GNB isolates and Acinetobacter baumannii (78%) was the most common pathogen. No colistin-resistant strain was isolated. Duration of IV-colistin and concomitant antibiotics use was significantly (p \u3c 0.05) shorter in IV-ASZ-colistin group. Significantly (p \u3c 0.05) higher clinical cure and microbial eradication, along with lower ventilatory requirements, mortality rate, and colistin induced nephrotoxicity and electrolyte imbalance was observed in IV-ASZ-colistin group.Conclusions: With better lung penetration, ASZ-colistin offers effective and safe microbiological and clinical benefits as adjunctive or alternate treatment of VAP due to colistin susceptible MDR-GNB in neonates