Global Initiative for Asthma (GINA) guideline: achieving optimal asthma control in children aged 6-11 years.

The Global Initiative for Asthma (GINA) 2021 guidelines for asthma have been set forth with some alterations in Step 3, for children from 6-11-year-old age group. The low dose LABA-ICS, very low dose formoterol-ICS, medium dose ICS and ICS-LTRA combination were recommended in the guideline. We organized this study to draw an effective comparison between these three combinations of controller therapies in pediatric population. A retrospective study was conducted at the Aga Khan University Hospital, Karachi, Pakistan which enrolled 114 children aged 6-11 years old, from July 2021 to December 2022. These children were admitted with asthma exacerbations and were discharged on controller medications as per GINA guidelines on step 3 for control of asthma for 3 months. They were then followed for re-admission within 30 days of discharge, number of ER visits with asthma exacerbations for 1 year, number of admissions with asthma including HDU and PICU admissions, length of stay per admission for all admissions in subsequent one year. The pulmonary function test was done at 1 week follow-up in clinic after discharge and at 3 months visit post discharge. A total of 114 pediatric patients from age 6-11-year-old, were enrolled in the study period out of which 36 (31.57%), 33(28.9%) and 34 (29.82%) patients were categorized into ICS-LABA, ICS and ICS-LTRA groups respectively. ER visits were significantly low in ICS-LABA group followed by ICS-LRTA group and then ICS group (1.75±0.96 vs 2.93±1.412 vs 3.11±1.21, p<0.001). Similar statistically significant results were observed on average number of admissions per year (1.52±1.02 vs 1.96±0.84 vs 2.06±1.07, p=0.047) and number of patients needing PICU (13.88% vs 26.47% vs 39.39%, p=0.034) in these groups respectively. ICS- LABA group patients had the best values of FEV1 and FEV1/FVC ratio after pulmonary function tests at 3 months follow-up followed by ICS-LTRA and ICS group. Amongst the three options regimens for children managed at step 3 on GINA 2021 guidelines, ICS-LABA therapy helps attain optimal patient outcomes and lung functions in children with asthma followed by ICS-LTRA and ICS group respectively.

Clinical outcomes of children with acute asthma managed with intravenous magnesium sulphate outside intensive care setting.

Asthma in children constitutes a well-known respiratory condition with significant mortality. In poorly controlled asthma, multiple adjunct therapies including magnesium sulphate (MgSO4), are recommended to decrease the likelihood of intubation; however, limited evidence exists to support their routine usage in day-to-day situations. Aim of this study is to determine the outcomes of pediatric patients treated with magnesium sulphate during exacerbations of asthma admitted at a tertiary care unit. A retrospective study was conducted at The Aga Khan University Hospital, Karachi, Pakistan from January 2019 to December 2021. Patients aged 6 years to 15 years presented with acute asthma through Emergency Room (ER) having clinical respiratory score (CRS) more than five, admitted in high-dependency unit (HDU) were included in the study. Patients who were started on magnesium sulfate within 24 hours of admission were categorized in magnesium sulfate (MS) group. Patients receiving all standard acute asthma treatment but were not started on magnesium therapy within 24 hours of admission were categorized in the non-magnesium sulfate (non-MS) group. Different outcome variables were compared between the groups. A total of 110 patients with asthma were enrolled. Fifty-four patients were categorized into MS group while 56 were included in non-MS group. Fewer patients were transferred from HDU to pediatric intensive care unit (PICU) (24.07%) in MS group compared to non-MS group (42.85%), (p=0.02). In MS group, the mean number of days spent on oxygen in HDU were 2.38±0.81, while non-MS group spent more days (3.10±0.84 (p<0.01). This study demonstrates that for pediatric patients with severe asthma exacerbations, administration of IV MgSO4 (within 24 hours) is beneficial and results in fewer admissions to PICU and reduces the mean number of days spent on oxygen therapy.

Asthma exacerbations and body mass index in children and adolescents: experience from a tertiary care center.

The prevalence and incidence of asthma continue to rise globally. Obesity has been identified as a potential risk factor for asthma exacerbations. The association between body mass index (BMI) and asthma is not well studied in some regions. This study aims to investigate the impact of BMI in pediatric asthmatic patients. This retrospective study was conducted at the Aga Khan University Hospital from 2019 to 2022. Children and adolescents with asthma exacerbation were included. The patients were classified into four groups based on their BMI: underweight, healthy weight, overweight, and obese. The demographic characteristics, medications used, predicted FEV1 measurements, asthma exacerbations per year, length of stay per admission, and the number of patients requiring High Dependency Unit (HDU) care were recorded and analyzed. Our results demonstrated that patients in the healthy weight category had the highest percentage of FEV1 (91.46±8.58) and FEV1/FVC (85.75±9.23) (p<0.001). The study found a significant difference in the average number of asthma exacerbations per year between the four groups. Obese patients had the highest number of episodes (3.22±0.94), followed by the underweight group (2.42±0.59) (p<0.01). The length of stay per admission was significantly shorter for patients with a healthy weight (2.0±0.81), and there was a statistically significant difference observed in the number of patients requiring HDU care among the four groups, as well as in the average length of stay at the HDU (p<0.001). Elevated BMI is related to an increased number of annual asthma exacerbations, a low FEV1 and FEV1/FVC, increased length of stay at admission, and increased stay in the HDU.

Immunoglobulin Ige Levels And Clinical Dynamics Of Asthma In Children And Adolescents.

Background: The involvement of IgE in type 1 hypersensitivity reactions, including asthma and allergic rhinitis, is widely recognized, and established. However, within this region, there exists a dearth of research exploring the relationship between serum IgE levels and the severity of asthma within the pediatric population. Consequently, the primary objective of this study was to compare the IgE status among children and adolescents diagnosed with asthma, and to examine its association with asthma exacerbations and hospitalizations. By investigating these parameters, we aimed to shed light on the potential role of IgE in influencing disease outcomes in this specific population. Methods: A retrospective study was conducted at The Aga Khan University Hospital from January 2015 to December 2020. Children and adolescents aged 6-18 years who were diagnosed and admitted with asthma, and who had measurement of IgE levels during asthma exacerbation were included in the study. The participants were stratified into two groups based on their IgE levels: normal IgE levels and high IgE levels. Various parameters, including the number of asthma exacerbations, emergency room (ER) visits, hospitalizations, average length of stay, as well as other clinical characteristics such as the presence of allergic rhinitis in the past and a family history of asthma were assessed. Results: A total of 122 patients were included in the study. Of them 57 patients (46.72%) were found to have high IgE levels, and 65 patients (53.28%) had normal IgE levels based on their age group. The average number of exacerbations per year was significantly greater the in high IgE group (3.6±1.09), in comparison to the normal IgE group (2.49±1.22). Conclusion: Higher serum IgE levels correlated with increased asthma exacerbations, emergency room visits, hospitalizations, and a positive family history of asthma, suggesting a potential link between IgE and asthma severity in the pediatric population of Pakistan.

Long-Term Use Of Inhaled Hypertonic Saline In Children And Adolescents With Cystic Fibrosis: Experience From A Tertiary Care Center In A Low And Middle Income Country.

Background: Long term hypertonic saline use has been found to improve mucus transport, airway hydration, and mucociliary clearance in patients with cystic fibrosis. However, the effect of hypertonic saline on the outcomes of patients with cystic fibrosis is not well established. The aim of our study was to determine the long-term use of hypertonic saline in reducing pulmonary exacerbations, length of hospital stay and pseudomonas colonization in patients with cystic fibrosis admitted for treatment at a tertiary care referral center. Methods: Retrospective cohort study was conducted on 71 patients with cystic fibrosis. Patients ranged in age between 3-18 years. All patients with two to five pulmonary exacerbations in the preceding six months were included in the study. Those who received regular inhaled 3-7% hypertonic saline twice daily during their admission and till 6 months after discharge from hospital were categorized as hypertonic saline (HTS) group. Patients who did not receive regular hypertonic saline for 6 months were included in the non-hypertonic saline (NHTS) group. Data was analyzed at the end of one year. Results: The HTS group had 37 patients whereas, the NHTS group had 34 patients. Mean number of exacerbation episodes was significantly lower in HTS group (2.18±0.84) as compared to NHTS group (3.67±0.91) (p<0.01) whereas, length of hospital stays and frequency of pseudomonas colonization did not significantly differ between the two groups (p=0.78 and p=0.12 respectively). The mean number of pulmonary exacerbations also significantly reduced from 3.11±1.07 to 2.18±0.84 p-value <0.01 in the HTS group over the follow-up period of one year. Conclusion: : Long term hypertonic saline therapy is beneficial in patients with cystic fibrosis in preventing pulmonary exacerbations and subsequently reducing morbidity.

Use of systemic steroids during pulmonary exacerbations in children and adolescent with Cystic fibrosis.

OBJECTIVE: To determine the role of systemic steroids in cystic fibrosis patients and its effects on pulmonary exacerbation in children and adolescents. METHODS: The retrospective cohort study was conducted at the Aga Khan University Hospital, Karachi, and comprised data from January 2015 to December 2019 of cystic fibrosis patients aged 3-18 years hospitalised with pulmonary exacerbations. The patients were divided into systemic steroid group A and non-systemic steroid group B. Patients in group A received parenteral steroids during acute exacerbation of cystic fibrosis in the first two weeks of admission, while those in group B did not receive systemic steroids. Length of hospital stay and number of days on oxygen support were compared between the groups. Data was analysed using SPSS 22. RESULTS: Of the 124 patient charts evaluated, 84(67.7%) were included; 40(47.6%) in group A and 44(52.4%) in group B. There were no significant differences between the groups related to age, age at diagnosis, weight, height, and pulmonary exacerbations (p>0.05). Group A had significantly fewer days on oxygen support compared to group B (p<0.001), but there was inter-group difference in mean length of hospital stay (p=0.53). CONCLUSIONS: Systemic steroid usage during hospitalisation for acute exacerbation of cystic fibrosis was associated with decreased duration of oxygen requirement with standard treatment.

Age Related Characteristics Of Children And Adolescent With Henoch Schönlein Purpura And Systems Involvement: An Experience From Tertiary Care Center.

BACKGROUND: Henoch-Schönlein Purpura (HSP) is the most common vasculitis among children and adolescent characterized by skin, joints, renal and gastrointestinal involvement. There is different presenting feature of Henoch-Schönlein Purpura (HSP) and systemic involvement may vary at a certain age group. METHODS: This was a ten-year retrospective cohort study done at a tertiary care hospital of Pakistan conducted from 2011-2020. Patients admitted with the diagnosis of Henoch- Schönlein Purpura (HSP) and in accordance with inclusion criteria were divided into groups based on their age, gastrointestinal symptoms and renal symptoms with the objective to compare the clinical features and investigations of Henoch-Schönlein Purpura (HSP) patients. Younger age group had patients age 7 years and less while the other group included patients who were older than 7 years. pvalue <0.05 was considered as significant and SPSS 23 was used to analyzed the data. RESULTS: Total 104 patients diagnosed with Henoch-Schönlein Purpura (HSP) were studied. Henoch-Schönlein Purpura (HSP) was more prevalent in males with an increased frequency during autumn and winter. Purpuric rash was present in more than 90% of the patients involved. Joint swelling was significantly (p=0.029) more common in the younger age group (73.3%) while renal involvement was seen more frequent in the older age group (57.1%) (p=0.002). Renal symptoms were less commonly seen in patients with GI involvement. There was no significant difference in platelet count, WBC count and ESR levels among any of the groups. CONCLUSIONS: Age related difference in presentation help us to anticipate more renal involvement in older children and adolescent likewise joint involvement is more commonly seen in younger children.

Association of vitamin D levels and asthma exacerbations in children and adolescents: Experience from a tertiary care center.

The role of vitamin D as an immunosuppressant and anti-inflammatory has been studied previously for different pathologies in different populations globally. Relationships between serum vitamin D levels and its effect on asthma exacerbations in the adolescent asthma population are not well studied in this region. Therefore, this study was conducted to determine the vitamin D status in pediatric and adolescent asthma patients, and its association with asthma exacerbations. A retrospective study was conducted at The Aga Khan University Hospital from 2016 to 2020. Children and adolescents who were diagnosed and admitted with acute asthma exacerbations and who had at least one measurement of 25 hydroxy-vitamin D (25 OHD) were included in the study. Serum vitamin D levels were documented for enrolled patients and their past 2-year data was analyzed for asthma exacerbations, mean length of stay per admission, and admission plus length of stay at High Dependency Unit. 114 patients were included in the study. 41 patients (35.96%) were found to be vitamin D deficient, 38 patients (33.3%) were vitamin D insufficient, and 35 patients (30.7%) were labeled as vitamin D sufficient. The average number of exacerbations per year was significantly high in vitamin D deficient group (2.82±1.11) in comparison with insufficient (2.05±0.92) and sufficient groups (1.37±0.59) (p<0.001). Vitamin D deficiency is related to an increased number of annual asthma exacerbations, length of stay per admission, and admission into High Dependency Unit (HDU).

Role of inhaled antibiotics in children and adolescents with cystic fibrosis: Experience from the tertiary care center.

Introduction: Cystic fibrosis (CF) remains under-diagnosed in Pakistan. CF population has increased tendency for Pseudomonas aeruginosa (Pa) infection and it is one of the leading causes of mortality. Utilizing inhaled antibiotics (IAs) for the treatment of Pa infection has been well established in the literature. There is limited data available on CF in Pakistan, especially regarding the efficacy of IAs. The aim of this study is to investigate the role of IAs on Pa infection in children and adolescents with CF. Methodology: CF patients enrolled between January 2012 and December 2019 were selected as part of this retrospective cohort study. CF patients from 2 to 18 years of age who cultured Pa on any respiratory sample and who had never been Pa-free in at least two sputum cultures in the previous 12 months were included. Patients were divided into an IA group and a noninhaled antibiotic (NIA) group based on the treatment they received. Follow-up was done between 3 and 6 months posttherapy on Pseudomonas growth in the sputum. The number of pulmonary exacerbations were documented for 6 months follow-up. Results: Eighty-one children with CF were enrolled during the study period, of which 39 were in the IA group and 42 were in the NIA group. There was no significant difference in their demographics and initial clinical characteristics. The mean pulmonary exacerbations after 6 months were lower in the IA group as compared to the NIA group (1.102 ± 0.50 vs. 2.45 ± 0.89: P = 0.001). Follow-up between 3 and 6 months showed greater Pseudomonas colonization in the IA group versus the NIA group (53.84% vs. 92.85%: P = 0.001). Conclusion: IAs in combination with airway clearance therapy and oral or IV antibiotics are an effective regimen for children with CF.

Risk factors leading to pulmonary exacerbation in patients with cystic fibrosis: A systematic review.

OBJECTIVE: To ascertain major risk factors associated with pulmonary exacerbation and pulmonary function decline in cystic fibrosis. METHODS: The systematic review was conducted at Aga Khan University, Karachi, in September 2018, and comprised electronic search of PubMed, Ovid, Science Direct and Cumulative Index of Nursing and Allied Health Literature databases of studies conducted from January 1990 to September 2018 which were categorised into 3 sets; 1990-98, 1999-2007 and 2008-18. Studies included for review focussed on articles with pulmonary exacerbation as the health outcome indicator, and had diagnosis of cystic fibrosis as the inclusion criteria, while risk factors were the exposure terms used in the search process. References in bibliographies of the included studies were also systematically searched for relevant documents. RESULTS: Of the 60 studies obtained, 31(51.7%) were selected; 2(6.45%) from 1990-98, 7(22.58%) from 1999-2007 and 22(70.96%) from 2008-18. Overall, 17(54.83%) were cohort studies, 7(22.5%) were cross-sectional studies, 3(9.6%) were case-control studies, 3(9.6%) were randomised controlled trials and 1(3.2%) was systematic review and meta-analysis. In terms of major risk factors, genetic mutations were cited by 4(12.9%) studies, infections and inflammatory biomarkers by 15(48.4%), nutritional deficiencies by 9(29%) and geographical and socioeconomic status by 3(9.6%) studies. CONCLUSIONS: Early identification and recognition of risk factors associated with pulmonary exacerbation can have an explicit impact on its management, leading to decreased morbidity and mortality burden in cystic fibrosis cases.

Spectrum Of Pulmonary Exacerbation In Children And Adolescent With Cystic Fibrosis And Physicians' Practices: Experience From Tertiary Care Center From Lower-Middle Income Country.

BACKGROUND: Pulmonary exacerbations (PEx) are major contributor of significant morbidity and mortality in CF patients. Managing PEx needs standardization and without standard local practice guidelines there will be significant variation in practice in managing these children. The aim of this study is to analyse the clinical management of PEx in our setup and to document variation in practices among physicians. METHODS: Children and adolescents ≤18 years with CF pulmonary exacerbations admitted at high dependency unit (HDU) or wards were included in the study. Frequencies of different intravenous antibiotic combinations were documented along with use of different inhaled antibiotics and inhalation therapy. Practices of different physician were further studied with regards to use of systemic steroids, oral azithromycin and inhaled antibiotics. One way ANOVA was used to assess differences between physicians' practices. RESULTS: Fifty-seven patients were selected according to the inclusion criteria for 114 different exacerbations. Mean pulmonary exacerbation (PEx) for a patient (events/person-year) over five years was 3.16±1.41 per year and average length of stay was 5.7±4.4 days. Combination of intravenous ceftazidime and amikacin was the most frequently used regimen (28.07%). Five different physicians dealing with majority of the exacerbations (n=74) were studied further. Variability among consultants was significant in using systemic steroids (21.42-92.30%), use of maintenance oral azithromycin (0- 80%) and inhaled antibiotics (0-86.6%). CONCLUSIONS: Significant variation exists in practices of physicians dealing with CF PEx. Variability observed in our study will definitely provide openings for local CF experts to come up with standardized inpatient exacerbation guidelines.

Vitamin D status and pulmonary exacerbations in children and adolescents with cystic fibrosis: Experience from a tertiary care center.

BACKGROUND: The function of Vitamin D in preventing inflammation and infection has been studied previously for different pathologies in different populations globally. Relationships between serum Vitamin D levels and its effect on pulmonary exacerbations in the cystic fibrosis (CF) population are not well studied in our part of the world. Therefore, we aimed to ascertain the Vitamin D status in pediatric and adolescent CF patients and its association with pulmonary exacerbations. MATERIALS AND METHODS: A retrospective study was conducted at The Aga Khan University Hospital from 2015 to 2018. Patients of CF with sweat chloride value >60 mmol/l and who had at least one measurement of 25 hydroxy Vitamin D (25 OHD) were included in the study. Annual serum Vitamin D levels were documented for enrolled patients and their past 1-year data were analyzed for pulmonary exacerbations, average length of stay, and tracheal/airway colonization with organisms. RESULTS: 69 patients were included in the study. 28 patients (40.57%) were found to be Vitamin D deficient, 22 patients (31.88%) were Vitamin D insufficient and 19 patients (27.53%) were labeled as Vitamin D insufficient. The average number of exacerbations per year was significantly high in Vitamin D deficient group (3.71 ± 0.96) in comparison with insufficient (3.18 ± 1.09) and sufficient groups (2.26 ± 0.93) (P < 0.001). CONCLUSION: Vitamin D deficiency is related to an increased number of annual pulmonary exacerbations and pseudomonas infections.

Role Of Long-Term Intermittent Use Of Oral Azithromycin On Pulmonary Exacerbations In Cystic Fibrosis Children.

BACKGROUND: Pulmonary exacerbation is the most common acute event occurring in a patient with cystic fibrosis and Pseudomonas aeruginosa is the most commonly involved organism. Azithromycin has antimicrobial and immunomodulatory effects on the lungs and our study aimed to determine the role of long-term intermittent use of oral azithromycin on pulmonary exacerbations in children with cystic fibrosis. METHODS: A retrospective cohort study was conducted from January 2012 to December 2016 at a tertiary care hospital, Aga Khan University Hospital, Karachi. The criteria for enrolment included cystic fibrosis patients aged 3-18 years who were classified into two groups based on their antibiotic use. The Azithromycin group included those CF patients who were on three days per week oral Azithromycin (10 mg per Kg per day) for 6 months. The non-azithromycin group included CF patients who were not on long term oral azithromycin. Our primary outcome was to assess the reduction in the number of exacerbations. RESULTS: Sixty-three patients with a mean age (10.06±3.80) and mean pulmonary exacerbations of (3.67±1.58) in the 6 months before enrolment were included in our study. Out of these, 30 patients were included in the azithromycin group and 33 patients in the non-azithromycin group. Overall, 180 exacerbations were documented during the study period. The one-way ANOVA (F (1,61) =8.033, p<0.05) demonstrated a statistically significant difference in the mean number of exacerbations between the azithromycin (2.70±1.72) and non-azithromycin group (3.81±1.40) however, the mean length of stay between the groups was not significant (p=0.582). P. aeruginosa was found to be the most predominant colonizer of the airways. CONCLUSION: Long term low dose azithromycin therapy is beneficial in young patients with cystic fibrosis. It is an effective prevention strategy for pulmonary exacerbations.

Paediatric Inflammatory Bowel Disease: Clinical Presentation and Disease Location.

OBJECTIVE: To determine different clinical presentationsand disease location demarcatedby upper and lower gastrointestinal endoscopyand relevant histopathologyin children diagnosed with inflammatory bowel disease (IBD). METHODS: This is 5 years (2010 to 2015) retrospective studyconducted at the Aga Khan University Hospitalenrolling65admitted children between 6 months to 15years from either gender, diagnosed with IBD on clinical presentation, endoscopy and biopsy. Different clinical presentations at the time of diagnosis were noted in different categories of the disease. All patients underwent upper and lower (up to the terminal ileum) endoscopy with multiple punch biopsies and histologic assessment of mucosal specimens. All endoscopies were done by paediatric gastroenterologists at endoscopy suite of the hospital and all specimens were reported by the pathology department. ESPGHAN revised criteria for the diagnosis of inflammatory bowel disease in children and an adolescent was used to standardize our diagnosis. Extent of disease on endoscopy and relevant histopathology of the biopsy samples were noted at the time of diagnosis. Data was summarized using mean, standard deviation, numbers and percentages for different variables. RESULTS: Total 56 children were enrolled according to inclusion criteria. There were 34children (61.53%) diagnosed with ulcerative colitis (UC), 10 patients (16.92%) had Crohn'sDisease (CD) and 11 (21.53%) patients were labeled as Indeterminate colitis (IC). Mean age at onset of symptoms was10.03±2.44 and mean age at diagnosis was11.10±2.36. Abdominal pain (80%) and chronic diarrhea (70%) were common symptoms in CD whereas bloody diarrhea (79.41%) and rectal bleeding(64.70%)were common presentation in UC. Patients diagnosed with indeterminate colitis(IC) had similar clinical features as in UC patients. Only 7% patients had some extra-intestinal features in the form of joint pain and/or uveitis. Aspartate aminotransferase level (95.18 ±12.89) was relatively high in patients withCD in comparison with other categories of IBD. Endoscopic findings and relevant histopathology of biopsy samples in UC showed 65% patient had pan-colitis and 13 % with disease restricted to rectum only whereas in CD 70% patient had disease in ileo-colon and only 10 % had involvement of ileum at the time diagnosis. CONCLUSION: Patients with UC dominated in our cohort. The most common clinical presentation in UC was bloody diarrhea and rectal bleeding and patients with CDhad abdominal pain and chronic diarrhea as predominant clinical features. Extraintestinal features were uncommon in our cohort. In endoscopic findings, pan-colitis was the mostfrequentfinding in UC and ileo-colonwas common location in CD. IC and UC shared common clinical features and disease location on endoscopy.

Clinical and laboratory profile of children with Cystic Fibrosis: Experience of a tertiary care center in Pakistan.

OBJECTIVE: To determine the clinical presentation, diagnostic investigations and laboratory workup done in admitted children with cystic fibrosis at Aga Khan University Hospital Karachi, Pakistan. METHODS: This is athree years retrospective study from January 2013 to December 2015 conducted at The Aga Khan University Hospital Karachi Pakistan, enrolling admitted patient from birth to 15 years of either gender, diagnosed with CF on the basis of clinical features and positive sweat chloride test. Different clinical presentations were noted including initial presentations. Sweat chloride values more than 60mmol/L were labeled as positive and consistent with diagnosis of CF. Available Delta F-508 mutation analyses were noted. Relevant laboratory and radiological investigations including sputum culture and HR-CT chest findings were documented. Results were analyzed using SPSS version 20. RESULTS: Total 43 children were selected according to the inclusion criteria. Chronic cough (69.76%) was the most common initial clinical presentation. Mean age at onset of symptoms was 14.41± 26.18 months and mean age at diagnosis was 47.20 ± 45.80 months Respiratory features were most common in our cohort including chronic productive cough (90.71%), recurrent bronchopneumonia (72.09%) and asthma like presentation (44.19%) with wheezing and cough. 86% patients presented with failure to thrive. Gastroenterological features including steatorrhea were seen in 55.81% patients and 44.19% patients had abdominal distension. Mean sweat chloride value in our population was 82.70± 22.74. Gene analysis for Delta F-508 was identified in 12 (27.90%) patients. Bronchiectatic pulmonary changes on HRCT were seen in 18 patients (41.86%). Pseudomonas grew in 12 patients (27.90%) in sputum cultures at the time of diagnosis. CONCLUSION: Respiratory presentations predominate in CF children followed by gastrointestinal features. Nearly half of our patient had bronchiectatic changes on CT scan chest and more than quarter had pseudomonas colonization in the airways at the time of diagnosis. Delta F-508 mutation was found to be uncommon in our study population. There is significant delay in diagnosing patients with CF.

Spectrum of Celiac disease in Paediatric population: Experience of Tertiary Care Center from Pakistan.

OBJECTIVE: To determine clinical features and relevant laboratory investigations of patient with celiac disease (CD) and comparing classical celiac disease (CCD) with Non-diarrheal celiac disease (NDCD). METHODS: This is a five years retrospective study conducted at The Aga Khan University Hospital Karachi, Pakistan from January 2010 to December 2015, enrolling children from one year to 15 years of either gender diagnosed as celiac disease in accordance with revised ESPGHAN criteria. Biopsy samples with grade 2 or more on Modified Marsh Classification were considered as consistent with celiac disease. Celiac patients were categorized into Classical celiac disease (with Chronic Diarrhea) and non-diarrheal celiac disease (Atypical celiac) and their clinical features and relevant laboratory investigations were documented. RESULTS: Total 66 patients were selected with celiac disease according to inclusion criteria, 39 (59.09%) patients were labeled as CCD and 27 (40.91%) patients were labeled as NDCD. Marsh grading 3a and above were more marked in CCD as compared to NDCD. Mean titer for Tissue transglutaminase antibodies (TTG) were higher in CCD group in comparison to NDCD group. In CCD, the most common clinical presentations were abdominal distension whereas in NDCD, the most remarkable features were recurrent abdominal pain (62.9%). Frequency of failure to thrive is significantly high in CCD (82.05%) but patients merely with short stature were more common in NDCD (33.3%). Refractory anemia was present in 66.6% patients in NDCD group and 41.1% patients in CCD group. 74.3% patients in CCD group were vitamin D deficient whereas 85% patient had vitamin D deficiency in NDCD group (p= 0.03). CONCLUSION: NDCD is not uncommon in our population. Recurrent abdominal pain, failure to thrive or patients only with short stature and refractory anemia are prominent features in NCDC group whereas abdominal distension, failure to thrive and recurrent abdominal pain were noticeable features in CCD. High grade histopathology and raised antibodies titer is hallmark of CCD. Vitamin D deficiency is almost equally present in both groups.

Liddle's Syndrome.

Hypertension in paediatric age group is commonly secondary to a known cause. It is crucial to identify the cause of hypertension and treat it before development of any associated complications to prevent morbidity and mortality. Paediatric Hypertension is one of the important clinical finding in a child with certain clinical syndrome. We are presenting a case of a 10 month old child presenting with hypertension and hypokalaemia, after excluding all identifiable causes and her positive response to therapy, that is amiloride, along with supportive biochemical data she was diagnosed as a case of monogenic type of hypertension known as Liddle's syndrome.