Medical Specialties Commons^™

Hereditary Sensory Autonomic Neuropathy Ii, A Rare Disease In A Large Pakistani Family, Fazal M. Arain, Prem Chand

Department of Paediatrics and Child Health

Hereditary Sensory Autonomic Neuropathy II (HSAN II) is a rare genetic disorder, characterized by severe loss of pain, temperature and touch sensation. Injuries in these patients can progress to necrosis and shedding of digits and limbs. Here we report two cases of HSAN II belonging to a Pakistani family. Individual 1, a forty five year old man, had complete loss of pain sensation since birth. Self-mutilation and complication of injuries resulted in the shedding of all the digits and right foot and surgical amputation of left leg. Individual 2, a five year old girl,had delay in healing of wounds and …

Congenital Factor Vii Deficiency In Children At Tertiary Health Care Facility In Pakistan, Muhammad Matloob Alam, Bushra Moiz, Karim Abdur Rehman, Priyanka Jethwani, Zehra Fadoo

Department of Paediatrics and Child Health

This study presents the demographics, clinical spectrum, and outcome of patients with congenital factor VII (FVII) deficiency at a tertiary care center over a period of 12 years. Of the 49 patients, 27 (55%) patients were males. Consanguinity was found in 92% of the patients. The median age of symptom onset was 2.4 (interquartile range [IQR]: 1.1-6.5) years with a median age of 5.8 (IQR: 3.1-10) years at diagnosis. Life-threatening complications like intracranial bleeding (ICB) and intra-abdominal bleeding (IAB) were observed in 8 (16.4%) patients. We found that 11 (55%) of the 20 patients with FVII coagulant activity (FVIIc) 5% …

Screening For G6pd Deficiency Among Neonates With Neonatal Jaundice Admitted To Tertiary Care Center: A Need In Disguise, Kishwer Kumar, Arjumand Sohaila, Shiyam Sunder Tikmani, Iqtidar Ahmed Khan, Anila Zafar

Department of Paediatrics and Child Health

This study was conducted to determine the association of Glucose-6-Phosphate Dehydrogenase (G-6-PD) deficiency among neonates admitted with jaundice at the neonatal intensive care unit, well baby nursery and neonatal step down nursery of the Aga Khan University Hospital, Karachi, Pakistan, from January to June 2010. A total of 205 neonates following the selection criteria were included. All selected neonates have their venous blood drawn, saved in EDTA bottle and sent to laboratory of The Aga Khan University Hospital (AKUH). The laboratory results of whether G-6-PD deficiency was present or not was recorded in the proforma. G-6-PD was deficient in 19 …

Use Of N-Acetylcysteine In Children With Fulminant Hepatic Failure Caused By Acute Viral Hepatitis, Ali Faisal Saleem, Qalab Abbas, Anwarul Haque

Department of Paediatrics and Child Health

Objective: To determine the efficacy of N-acetylcysteine (NAC) in children aged > 1 month to 16 years admitted with Fulminant Hepatic Failure (FHF) secondary to Acute Viral Hepatitis (AVH) in a tertiary care center of a developing country.

Study Design: Analytical study.

Place and Duration of Study: Department of Paediatrics, The Aga Khan University Hospital, Karachi, Pakistan, from January 2007 to December 2011.

Methodology: Medical records of children (> 1 month - 16 years) with FHF admitted with AVH of known etiology who received NAC were reviewed retrospectively. Liver function tests (mean ± SD) at baseline, 24 hours after NAC and …

Effect Of Clopidogrel On Platelet Cd Markers In Normal Individuals And In Patients With Untreated Type 2 Diabetes Mellitus, Muhammad Saboor, Moinuddin, Samina Ilyas

Department of Paediatrics and Child Health

The objective of this study was to evaluate the effect of clopidogrel response in patients with untreated type 2 diabetes mellitus as compared with normal individuals. One hundred and seven subjects i.e. 32 normal and 75 patients with untreated type 2 diabetes mellitus were enrolled in this study. In the first step, normal subjects as well as diabetic patients were selected and tested for various laboratory parameters and platelets flow cytometry. In the second step, an antiplatelet drug (clopidogrel) was administered for 10 days to each individual enrolled in the study. After 10 days blood samples were collected for platelets …

Clinical Profiles And Outcomes Of Children Admitted To The Pediatric Intensive Care Unit From The Emergency Department, Anwarul Haque, Naveed Ur Rehman Siddiqui, Sidra Kaleem Jafri, Mehar Hoda, Surraiya Bano, Asad Mian

Department of Paediatrics and Child Health

The aim of this study was to describe clinical profiles and outcomes of children admitted directly from the Emergency Room (ER) to the Pediatric Intensive Care Unit (PICU) of academic hospital. The medical records of all children (1 month to 16 years) admitted in PICU from ER, from January 2011 to December 2012 were reviewed. Of the 26,774 patients seen in the ER during the study period, 468 (1.7%) were admitted to the PICU which constituted about 41.5% (468/1127) of all the total PICU admissions. Sixty three percent (n=294) were under-five; males were 60.9% (285), 82.3% (385) were in medical …

Isoniazid Induced Acute Pancreatitis In A Young Girl, Ali Faisal Saleem Dr, Saba Arbab, Farah Qamar Naz

Department of Paediatrics and Child Health

Isoniazid (INH) is the mainstay of management against Mycobacterium tuberculosis. INH-induced acute pancreatitis is an uncommon association and with dearth of literature on it. We are reporting a case of an 11 years old girl who developed acute pancreatitis after 2 weeks of antituberculous therapy. An INH free regimen was started. She was discharged and followed without complications.

Haematological Causes Of Thrombocytopenia In Children At Aga Khan University Hospital, Karachi, Amna Jawaid

Department of Paediatrics and Child Health

Objective: To identify the haematological causes of thrombocytopenia in children admitted at a tertiary care hospital.
Methods: The prospective, descriptive study was carried out at the Aga Khan University Hospital, Karachi, from September 3, 2009 to March 3, 2010, and comprised children between one month and 14 years of age with platelet count less than15000. After reviewing the record, a questionnaire was filled and data was analysed using SPSS 15.
Results: Out of 108 patients in the study, 69(64%) were male and 39(36%) were female. The overall mean age was 69±46.54 months (range: 12-168 months). The mean platelet count was …

Mortality Patterns Among Critically Ill Children In A Pediatric Intensive Care Unit Of A Developing Country, Naveed-Ur-Rehman Siddiqui, Zohaib Ashraf, Humaira Jurair, Anwarul Haque

Department of Paediatrics and Child Health

Background and Aim: Advances in biomedical technology have made medical treatment to be continued beyond a point, at which it does not confer an advantage but may increase the suffering of patients. In such cases, continuation of care may not always be useful, and this has given rise to the concept of limitation of life-sustaining treatment. Our aim was to study mortality patterns over a 6-year period in a Pediatric Intensive Care Unit (PICU) in a developing country and to compare the results with published data from other countries.
Materials and Methods: Retrospective cohort study was conducted in a PICU …

Frequency And Mortality Associated With Hyperglycemia In Critically Ill Children, Sabeen Abid Khan, Mohsina Noor Ibrahim, Anwarul Haque

Department of Paediatrics and Child Health

Objective: To determine the frequency of hyperglycemia in critically ill children admitted in PICU of a tertiary care hospital of Karachi and to compare the mortality of critically ill children with and without hyperglycemia.

Study Design: Cross-sectional study.

Place and Duration of Study: Paediatrics Intensive Care Unit (PICU) of National Institute of Child Health (NICH), Karachi, from November 2011 to April 2012.

Methodology: One hundred fifty critically ill children admitted to PICU were included. Patients who had fasting blood sugar levels more than 126 mg/dl within 48 hours of admission were included in the hyperglycemic group. The normoglycemic and hyperglycemic …

Diagnostic Accuracy Of Who Verbal Autopsy Tool For Ascertaining Causes Of Neonatal Deaths In The Urban Setting Of Pakistan: A Hospital-Based Prospective Study, Sajid Bashir Soofi, Shabina Ariff, Ubaidullah Khan, Ali Turab, Gul Nawaz Khan, Atif Habib, Kamran Sadiq, Zamir Suhag, Zaid Bhatti, Imran Ahmed, Rajiv Bhal, Zulfiqar Ahmed Bhutta

Department of Paediatrics and Child Health

Background: Globally, clinical certification of the cause of neonatal death is not commonly available in developing countries. Under such circumstances it is imperative to use available WHO verbal autopsy tool to ascertain causes of death for strategic health planning in countries where resources are limited and the burden of neonatal death is high. The study explores the diagnostic accuracy of WHO revised verbal autopsy tool for ascertaining the causes of neonatal deaths against reference standard diagnosis obtained from standardized clinical and supportive hospital data.
Methods: All neonatal deaths were recruited between August 2006 –February 2008 from two tertiary teaching …

Diagnosis And Management Of Preeclampsia In Community Settings In Low And Middle-Income Countries, Rehana A. Salam, Jai K. Das, Anum Ali, Soumyadeep Bhaumik, Zohra S. Lassi

Department of Paediatrics and Child Health

Hypertensive disorders of pregnancy contribute significantly to maternal mortality and morbidity. Preeclampsia belongs to the spectrum of hypertensive disorders of pregnancy and if undiagnosed and/or untreated leads to fatal consequences for both the mother and the baby. Early detection and prevention of preeclampsia is limited by uncertainty in the knowledge about its etiopathogenesis. While much work has been done in establishing clinical guidelines for management of preeclampsia in the hospital or tertiary care settings, there is considerable lack of work in the domain of evidence-based guidelines for screening, identification and management of preeclampsia at the community-level. The article reviews these …

Abdominal Lymphonodular Cryptococcosis In An Immunocompetent Child, Mehjabeen Zaidi, Sonia Qureshi, Sadia Shakoor, Saira Fatima, Fatima Mir

Department of Paediatrics and Child Health

We describe our experience with an apparently immunocompetent child presenting with pyrexia of unknown origin without focal signs. Investigations revealed lymphadenopathy at lung hila, mesentery, and porta hepatis. The child had received at least two months of empiric antituberculous therapy (ATT) before she came to us. A CT-guided biopsy revealed granulomatous inflammation. PAS stain showed yeasts which stained blue with Alcian blue, suggesting C. neoformans.

Impact Of Withholding Breastfeeding At The Time Of Vaccination On The Immunogenicity Of Oral Rotavirus Vaccine--A Randomized Trial, Asad Ali, Abdul Momin Kazi, Margaret M. Cortese, Jessica A. Fleming, Sungsil Moon, Umesh D. Parashar, Baoming Jiang, Monica M. Mcneal, Duncan Steele, Zulfiqar Bhutta

Department of Paediatrics and Child Health

Background: Breast milk contains anti-rotavirus IgA antibodies and other innate immune factors that inhibit rotavirus replication in vitro. These factors could diminish the immunogenicity of oral rotavirus vaccines, particularly if breastfeeding occurs close to the time of vaccine administration.
Methods: Between April 2011 and November 2012, we conducted an open label, randomized trial to compare the immunogenicity of Rotarix (RV1) in infants whose breastfeeding was withheld one hour before through one hour after vaccination with that in infants breastfed at the time of vaccination. The trial was conducted in the peri-urban area of Ibrahim Hyderi in Karachi, Pakistan. Both groups …

Outcome Of Pediatric Procedural Sedation & Analgesia In A Tertiary Care Hospital In Pakistan, Humaira Jurair, Amyna Bhimani, Anwar-Ul-Haque

Department of Paediatrics and Child Health

Background and Objective: Procedural sedation and analgesia (PSA) is pharmacologically induced state which allows patients to tolerate painful procedures while maintaining protective reflexes. It is the standard of care but there is limited data from Pakistan. Our objective was to assess the safety of the procedural sedation and analgesia in pediatric population at a tertiary care setting.
Methods: A retrospective notes and record review was conducted at the Aga Khan University Hospital, Karachi over 4 years from April 2010 to August 2014. Patients were between ages 6 months to 16 years and were in low risk category. The combination of …

Predictors And Outcome Of Tetanus In Newborns In Slum Areas Of Karachi City: A Case Control Study, Arjumand Sohaila, Yasir Shafiq, Shazia Azim, Benazir Baloch, Ali Syed Muhammad Akhtar, Shiyam Sunder Tikmani, Nick Brown

Department of Paediatrics and Child Health

Background: Tetanus in newborns, is an under-reported public health problem and a major cause of mortality in developing countries. This study aimed to determine the predictors and outcome of tetanus in newborn infants in the slums of Bin-Qasim town, Karachi, Pakistan.
Methods: We conducted a case-control study at primary health care centers of slums of Bin-Qasim town, area located adjacent to Bin Qasim seaport in Karachi, from January 2003 to December 2013. Cases were infants aged ≤30 days with tetanus, as defined by the World Health Organization. Controls were newborn infants aged ≤30 days without Tetanus, who were referred for …

Can Routine Screening And Iron Supplementation For Iron Deficiency Anemia In Nonsymptomatic Pregnant Women Improve Maternal And Infant Health Outcomes?, Anoosh Moin, Zohra S. Lassi

Department of Paediatrics and Child Health

Pregnant women have an increased need for iron that might not be met with diet alone. Due to physiologic anemia and population differences, no set criteria for defining iron deficiency anemia (IDA) are available globally. Serum ferritin and transferrin levels are often used to guide therapy by clinicians. Studies have reported an association between poor iron status and negative health outcomes such as low birth weight, premature birth, and perinatal death for women and their infants, although the evidence is weak.

Risk Factors Associated With Birth Asphyxia In Rural District Matiari, Pakistan: A Case Control Study, Farhana Tabassum, Arjumand Rizvi, Shabina Ariff, Sajid Bashir Soofi, Zulfiqar Ahmed Bhutta

Department of Paediatrics and Child Health

Background: During the past two decades there has been a sustained decline in child mortality; however, neonatal mortality has remained stagnant. Each year approximately 4 million babies are born asphyxiated resulting in 2 million neonatal deaths and intrapartum stillbirths. Almost all neonatal deaths occur in developing countries, where the majority is delivered at homes with negligible antenatal care and poor perinatal services.
Objectives: To identify socio-demographic and clinical risk factors associated with birth asphyxia in Matiari District of Sindh Province, Pakistan.
Method: A matched case control study was conducted in Matiari District with 246 cases and 492 controls. Newborn deaths …

Multi-Drug Resistant Gram Negative Infections And Use Of Intravenous Polymyxin B In Critically Ill Children Of Developing Country: Retrospective Cohort Study, Naveed-Ur-Rehman Siddiqui, Farah Naz Qamar, Humaira Jurair, Anwarul Haque

Department of Paediatrics and Child Health

Background: Patients in pediatric intensive care Units (PICU) are susceptible to infections with antibiotic resistant organisms which increase the morbidity, mortality and cost of care. To describe the clinical characteristics and mortality in patients with Multi-Drug Resistant (MDR) gram negative organisms. We also report safety of Polymyxin B use in these patients.
Methods: Files of patients admitted in PICU of Aga Khan University Hospital, from January 2010 to December 2011, one month to 15 years of age were reviewed. Demographic and clinical features of patients with MDR gram negative infections, antibiotic susceptibility pattern of isolates, discharge disposition and adverse effects …

Vascular Ehlers-Danlos Syndrome: A Rare Disorder Presenting With Focal Convulsions, Shakeel Ahmed, Syed Rehan Ali, Naila Nadeem, Muhammad Hamid

Department of Paediatrics and Child Health

Vascular Ehlers-Danlos Syndrome (VEDS), previously called Ehlers-Danlos syndrome type-IV, is a heterogeneous group of heritable connective tissue disorders characterized by thin, translucent skin, easy bruising, arterial, intestinal, and/or uterine fragility. There is large vessel involvement that leads to arterial rupture often preceded by aneurysm, arteriovenous fistulae, or dissection. Noninvasive imaging studies such as CT angiography and MR angiography are preferred as diagnostic studies for this condition. We are reporting a 4 years old girl who was presented with right sided unilateral convulsions and hypertension. CT angiogram showed stenosis with post-stenotic dilatation of coeliac and superior mesenteric arteries. There were extensive …

Approaches To Improve Quality Of Care (Qoc) For Women And Newborns: Conclusions, Evidence Gaps And Research Priorities, Zulfiqar Ahmed Bhutta, Rehana A. Salam, Zohra S. Lassi, Anne Austin, Ana Langer

Department of Paediatrics and Child Health

Despite progress in recent years, an estimated 273,500 women died as a result of maternal causes in 2010. The burden of these deaths is disproportionately bourne by women who reside in low income countries or belong to the poorest sectors of the population of middle or high income ones, and it is particularly acute in regions where access to and utilization of facility-based services for childbirth and newborn care is lowest. Evidence has shown that poor quality of facility-based care for these women and newborns is one of the major contributing factors for their elevated rates of morbidity and mortality. …

Frequency Of Cardiorenal Syndrome Type-I In Hospitalized Children With Acute Heart Failure In A Tertiary-Care Hospital, Mehjabeen Zaidi, Arshalooz Jamila Rehman, Anwarul Haque, Saleem Akhtar, Prem Kumar Maheshwar

Department of Paediatrics and Child Health

Objective: To determine the frequency of cardiorenal syndrome in hospitalized children with acute heart failure.

Study Design: Descriptive study.

Place and Duration of Study: Paediatric Intensive Care Unit, The Aga Khan University Hospital, Karachi, from December 2010 to December 2011.

Methodology: Sixty eight (68) children with acute heart failure fulfilling the selection criteria were evaluated for worsening of renal function (WRF). Serum creatinine was done at baseline and repeated at 72 hours to see the worsening of renal function. Estimated serum creatinine clearance was calculated by Schwartz formula.

Results: Mean age of patients was 43.6 ± 55.2 months. There were …

Empyema Thoracis In Children: Clinical Presentation, Management And Complications, Ali Faisal Saleem, Abdul Sattar Shaikh, Reema Sajjad Khan, Fazal Khan, Ahmad Vaqas Faruque, Muhammad Arif Mateen Khan

Department of Paediatrics and Child Health

Objective: To determine the etiology, clinical manifestation, management (medical and surgical) and complications of children with empyema thoracis in a tertiary care hospital from Karachi, Pakistan.

Study Design: Descriptive, analytical study.

Place and Duration of Study: Department of Surgery, The Aga Khan University Hospital, Karachi, from January 1996 to December 2010.

Methodology: Medical records of admitted children aged > a month to 15 years with discharge diagnosis of empyema thoracis and data was collected on demographic features, clinical manifestation, management and complications. Children managed medically were compared with those managed surgically by using interquartile range and median comparison. Mann-Whitney U test …

Tackling The Existing Burden Of Infectious Diseases In The Developing World: Existing Gaps And The Way Forward, Zulfiqar Ahmed Bhutta, Rehana A. Salam, Jai K. Das, Zohra S. Lassi

Department of Paediatrics and Child Health

This series evaluates the effectiveness of community-based interventions (CBIs) to prevent and control infectious diseases of poverty (IDoP). Evidence from our reviews suggests that CBIs and school-based delivery platforms are effective in averting risk behaviors and reducing the disease burden. Co-implementation of interventions through existing community-based programs including immunization campaigns, antenatal care and maternal and child health programs have the potential to scale-up interventions for IDoP. Future research should focus on the process of developing and implementing efficient community-based programs through a comprehensive approach, and to gauge the effectiveness of various existing delivery models in order to improve morbidity and …

Subacute Sclerosing Panencephalitis: Clinical And Demographic Characteristics, Arshad Rafique, Nida Amjad, Prem Chand, Syed Sohail Zahoor Zaidi, Muhammad Suleman Rana, Khalid Ahmed, Shahnaz Ibrahim

Department of Paediatrics and Child Health

Objective: To determine the clinical and demographic characteristics of children diagnosed with Subacute sclerosing panencephalitis (SSPE).

Study Design: Case series.

Place and Duration of Study: The Aga Khan University Hospital, Karachi, from January 2000 to June 2012.

Methodology: A retrospective analysis was done, regarding medical charts of 43 children under the age of 16 years with a discharge diagnosis of SSPE. Demographic and clinical characteristics were recorded. Results were expressed as percentages.

Results: Most of the 43 patients were male (72%). The average age at presentation was 8.7 years with average duration of symptoms being 100.6 days. History of …

Readmission To Paediatric Intensive Care Unit: Frequency, Causes And Outcome, Muhammad Rehan Khan, Prem Kumar, Sumaira Iram, Anwarul Haque

Department of Paediatrics and Child Health

Readmission to intensive care units is considered to be an important quality indicator in ICU settings. This study was carried out at the paediatric intensive unit (PICU) and step down units of paediatric ward at the Aga Khan University Hospital, Karachi, Pakistan, to assess the frequency, common causes and outcome of patients readmitted in PICU within 48 hours after discharge from unit. During the study period, 1022 patients were admitted in PICU, out of which 24 (2.34%) patients required readmission. Male to female ratio was 1.2:1. The mean length of stay on paediatric floor before readmission was 24 hours. Fifteen …

Acute Cerebellitis With Hydrocephalus, Nida Amjad, Anwarul Haque, Khalid Ahmed

Department of Paediatrics and Child Health

We report a case of an 8 years old child who presented with sudden onset of headache and vomiting. He had broad-based gait and intention tremors on admission. MRI brain revealed isointense signals on T1-weighted imaging and hyperintense signals on T2-weighted imaging. Cerebellar swelling was also identified with significant mass effect obliterating the fourth ventricle. CT head showed prominent third and lateral ventricles. He was treated with high dose corticosteroids and required an external ventricular drain (EVD) insertion. He made an uneventful recovery and suffered no neurologic deficit. The clinical and radiologic findings in this boy were consistent with cerebellitis …

Early Recovery In Post Varicella Transverse Myelitis, Prem Chand, Shahnaz Brahim, Syed Sohail Zaidi, Nida Amjad

Department of Paediatrics and Child Health

A two and a half year old Kenyan girl presented with recurrent chest infections and difficulty in swallowing. Her clinical and laboratory workup was suggestive of lower respiratory tract infection for which she received a course of antibiotics; however, she remains symptomatic after the management of her suspected diagnosis. Therefore, further radiological workup including a chest CT scan and barium study were performed. This showed a homogeneous mass surrounded by ascending and descending aorta in the posterior mediastinum that was compressing the middle esophagus. She had a posterolateral thoracotomy which revealed a cystic mass with smooth surfaces (5 x 5 …

Short-Term Neonatal Outcome In Late Preterm Vs. Term Infants, Anila Haroon, Syed Rehan Ali, Shakeel Ahmed, Humaira Maheen

Department of Paediatrics and Child Health

Objective: 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.

Study Design: A case control, descriptive study.

Place and Duration of Study: The Aga Khan University Hospital, Karachi, Pakistan, from January to December 2009.

Methodology: The study included 326 late preterm babies (defined as those born between 340 ⁄7 to 376 ⁄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 …

Immune Responses To Vi Capsular Polysaccharide Typhoid Vaccine In Children 2 To 16 Years Old In Karachi, Pakistan, And Kolkata, India, R. Leon Ochiai, M. Imran Khan, Sajid B. Soofi, Dipika Sur, Suman Kanungo, Young Ae You, Atif Habib, Shah Muhammad Sahito, Byomkesh Manna, Camilo J. Acosta, Mohammad Ali, Sujit K. Bhattacharya, Zulfiqar A. Bhutta, John D. Clemens

Department of Paediatrics and Child Health

The geometric mean concentration (GMC) and the proportion maintaining a protective level (150 enzyme-linked immunosorbent assay (ELISA) units [ELU]/ml) 2 years following a single dose of 25 μg of injectable Vi capsular polysaccharide typhoid vaccine was measured against that of the control hepatitis A vaccine in children 2 to 16 years old in cluster randomized trials in Karachi and Kolkata. The GMC for the Vi group (1,428 ELU/ml) was statistically significantly different from the GMC of the control hepatitis A vaccine group (86 ELU/ml) after 6 weeks. A total of 117 children (95.1%) in the Vi group and 9 (7.5%) …