Pakistan Pediatric Journal https://www.ppj.org.pk/index.php/ppj <p>Pakistan Pediatric Journal is, a peer reviewed, official publication of <a href="https://www.ppa.org.pk/">The Pakistan Pediatric Association</a>. It was decided in the second biennial conference of Pakistan Pediatric Association (Centre) held at Lahore in 1976 to publish the Pakistan Pediatric Journal from Lahore, Pakistan. The first issue was published in 1977 as Pakistan Pediatric Journal, ISSN No.0304-4904. Since then, the journal has been published without interruption on quarterly basis. This journal covers clinical and research work on all aspects of the health of children and adolescents. The manuscripts are categorized as original research articles, review articles, case reports, short communication and special feature articles in the field of pediatrics. The official language of Pakistan Pediatric Journal is English [American (US) English]. The journal is distributed to all members of The Pakistan Pediatric Association, medical colleges, libraries and related institutes locally and abroad.</p> <p>Prof. SMK Wasti was the first Chief Editor of the Journal and from 1977, he continued till March 1993. During his tenure, he guided and trained the team with his wisdom, experience, dedication and commitment to improve the child health services on national level. Thus he put the journal on sound footing. Prof. S M Haneef took over as the Chief editor from Prof. SMK Wasti in April 1993 and continued till December 2003. He also followed the footstep of Prof. SMK Wasti. He also by his academic and clinical expertise not only trained his team but also saw the journal to grow and sustain. Prof. Said ul Haque was appointed Chief Editor in March 2004. Prof. Said ul Haque was associated with Pakistan Pediatric Journal since 1978 in various capacities, as Senior Bibliographer; Managing Editor, and Editor before becoming the Chief Editor. He was instrumental in getting indexation in various national and international indexation agencies of repute including EMBASE/Excerpta Medica, etc. Prof. Muhammad Ashraf Sultan was associated with journal since 2004 as Managing Editor. After the death of Prof Said ul Haque in 2015 Prof. Sajid Maqbool was appointed as the Chief Editor and Prof. Humayun Iqbal Khan as the Managing Editor. Mr. Nazir Ahmad is associated with journal since 1989 as office secretary.</p> en-US Pakistan Pediatric Journal 0304-4904 The Effects Gamification of Pursed Lips Breathing on Oxygenation Status of Children Suffering from Pneumonia: A Systematic Review https://www.ppj.org.pk/index.php/ppj/article/view/502 <p>ABSTRACT<br>Objective: This review aims to determine the effects gamification of pursed lips breathing (PLB) in children with pneumonia.<br>Study Design: Systematic Review.<br>Place and Duration of Study: University of Indonesia, 6 months.<br>Material and Methods: Nine electronic databases, PubMed, Science Direct, Sage Pub, Taylor &amp; Francis, JSTOR, Springer, Google Schooler, ProQuest, and Scopus, were used to search the literature. The database search identified 691 articles, 6 of which fulfilled the inclusion criteria, including quasi-experiment. <br>Results: Most respondents were school-age and teenagers between 5 and 18 years (65%), and preschool-age (35%). In this study, respondents consisted of more boys (52%) than girls (48%) (n=160). All respondents were children who suffered from pneumonia. Subsequently, gamification for PLB includes blowing: tongue twisters; party whistles; water in bottles with straws; super bubbles; bamboo pinwheels; pinwheels, and blowing pianica. The results of gamification PLB can improve the oxygenation status of children with pneumonia by reducing respiratory rate (RR) and heart rate (HR), increasing forced expiratory volume in one second (FEV1), and increasing oxygen saturation).<br>Conclusion: using gamification of PLB can improve the oxygenation status in children with pneumonia.</p> Santun Setiawati Dessie Wanda Nur Agustini Yudan Whulanza Copyright (c) 2025 Pakistan Pediatric Journal 2025-08-16 2025-08-16 49 2 Not All Seizures Listen- Time to Change the Story https://www.ppj.org.pk/index.php/ppj/article/view/612 <p>Epilepsy is among the most common neurological disorders in children, characterized by recurrent and unprovoked seizures that can significantly impact neuro-development of a child. While many cases are manageable, 20-40% become drug resistant, demanding a shift in approach from conventional anti seizure medication to more personalized, etiology driven care. Advances in genetic testing and targeted therapies are reshaping treatment for drug resistant cases. This changing narrative emphasizes on early diagnosis, multidisciplinary care and child centered approach that prioritizes not only seizure control but developmental support and family well-being.&nbsp;</p> Javeria Alvi Copyright (c) 2025 Pakistan Pediatric Journal 2025-08-25 2025-08-25 49 2 Efficacy and Safety of Racecadotril in Management of Acute Diarrhea in Severely Malnourished Children Under 5 Years https://www.ppj.org.pk/index.php/ppj/article/view/503 <p><strong>Objectives</strong></p> <p>To evaluate the efficacy and safety of Racecadotril in the management of acute diarrhea in severe acute malnourished children.</p> <p><strong>Study design</strong></p> <p>Single-blinded randomized control trial</p> <p><strong>Place and duration of study</strong></p> <p>The study was conducted at the Children's Hospital and the Institute of Child Health Multan from January 1, 2019, to December 31, 2019.</p> <p><strong>Materials and Methodology</strong></p> <p>This clinical trial evaluates the efficacy and tolerance of Racecadotril in the management of acute diarrhea in children suffering from severe acute malnutrition. This was designed as a single-blinded, randomized control trial from January to December 2019 at the Children's Hospital, Multan. There were 174 children enrolled in this study, aged between 6 months and 60 months. The patients were divided into two random groups. The patients in Group A were administered standard diarrheal management according to IMNCI. IV fluids, zinc supplements, and Racecadotril were given, but Group B received all the treatments except Racecadotril. The patients were followed for stool consistency, frequency, and hydration status for 72 hours.</p> <p><strong>Result </strong></p> <p>The results indicated that both groups were not significantly different in the first 24 hours, but in the third dose after 72 hours, Group A showed dramatic improvement, as 80% of them became better hydrated with minimal diarrhea, contrasted with 70% in the Racecadotril group. On the Bristol Stool Scale and Mann-Whitney U test, the Racecadotril group showed significant recovery when the p-value was less than 0.05 with no side effects.</p> <p><strong>Conclusion </strong></p> <p>The study concludes that Racecadotril is a relatively safe adjunct treatment for SAM children with diarrheal diseases, especially after the first 72 hours of treatment.</p> <p>&nbsp;</p> Saadia Khan Haroon Gul Khan Ghulam Abbas Qazi Reema Arshad Tehseen Ikram Muhammad Shakir Copyright (c) 2025 Pakistan Pediatric Journal 2025-08-16 2025-08-16 49 2 An Outcome-Based Comparative Analysis Between Variants of Guillain Barre Syndrome Undergoing Plasmapheresis https://www.ppj.org.pk/index.php/ppj/article/view/549 <p><strong>ABSTRACT:</strong>&nbsp;</p> <p><strong>OBJECTIVE:</strong> To compare functional outcomes among Guillain Barre Syndrome (GBS) variants, including acute demyelinating inflammatory neuropathy (AIDP) and acute motor axonal neuropathy (AMAN) treated with Plasmapheresis.&nbsp;</p> <p><strong>STUDY DESIGN</strong>: Prospective, single-center, comparative analysis&nbsp;</p> <p><strong>PLACE &amp; DURATION OF STUDY</strong>: Pediatric Neurology department and critical care unit of Children Hospital Lahore within one-year duration&nbsp;from January 2024 to January 2025.</p> <p><strong>MATERIALS &amp; METHODS</strong>: All patients diagnosed with GBS who were eligible for plasmapheresis were enrolled in the study after informed consent. The outcome was measured as shifting to ICU or death due to plasmapheresis as well as functional outcome at discharge and after 3 months, compared among AIDP and AMAN variants of Guillain barre syndrome.&nbsp;</p> <p>RESULTS: In this study, we examined 31 cases of Guillain-Barré Syndrome (GBS). Among these, 10 cases (32.3%) were diagnosed with Acute Inflammatory Demyelinating Polyneuropathy (AIDP), while 21 cases (67.7%) were diagnosed with Acute Motor Axonal Neuropathy (AMAN). The mean age of children diagnosed with AIDP was 6.55 years (±3.97), whereas the mean age of children in the AMAN group was 8.02 years (±2.75). All children in both groups were transferred to the wards without complications (100%), and no children required ICU admission due to plasmapheresis. In the AMAN group, hypotension occurred in 2 (0.95%), urticaria in 3 (14.3%), and muscle cramps in 2 (9.5%) during plasmapheresis. In the AIDP group, 1 (10%) child experienced lightheadedness. &nbsp;</p> <p>CONCLUSION: Plasmapheresis is a reliable and beneficial treatment choice for pediatric Guillain-Barré Syndrome (GBS), promoting notable functional improvement in both AIDP and AMAN cases. Although AMAN was the more common subtype in our group, the outcome for both types were similar.&nbsp;</p> Ahmad Bilal Tipu Sultan Azeem Ashfaq Arshad Mehmood Javeria Raza Alvi Zia ur Rehman Copyright (c) 2025 Pakistan Pediatric Journal 2025-08-16 2025-08-16 49 2 Comparison Between 0.9% Normal Saline and 0.3 % Hypertonic Saline Nebulization in Acute Bronchiolitis in Terms of Mean Length of Hospital Stay. https://www.ppj.org.pk/index.php/ppj/article/view/541 <p><strong>Introduction: </strong>Hypertonic saline draws fluid&nbsp;&nbsp; from&nbsp;&nbsp; epithelium&nbsp;&nbsp; reducing edema and preventing necrosis that is helpful in healing epithelium. It may play an immunomodulator role by suppressing inflammatory markers released by neutrophils.</p> <p>&nbsp;</p> <p><strong>Objectives: </strong>To compare the outcome when nebulized with 3% hypertonic saline as compared to 0.9% normal saline in the terms of mean length of stay in hospitalized children suffering from bronchiolitis.</p> <p><strong>Study design: </strong>Randomized controlled trial.</p> <p><strong>Settings: </strong>Department of Paediatric Medicine, Ghurki trust teaching&nbsp;&nbsp; hospital, Lahore.</p> <p><strong>Study duration: </strong>29<sup>th</sup> December 2023 to 28<sup>th</sup> June 2024.</p> <p><strong>Materials &amp; Methods: </strong>A total of 234 (117 in each group) children of age &lt;24 months, both genders presenting with bronchiolitis were included. Group A was nebulized with 4 ml 3% hypertonic saline every 6 hours and Group B with 4 ml normal &nbsp;&nbsp;&nbsp;&nbsp;saline every 6 hours. Length of hospital stay was noted in hours after discharging the patient according to outcome criteria.</p> <p><strong>Results: </strong>The mean age of patients in group A was 14.72 ± 4.33 months and in group B was 15.0 ± 4.59 months. Mean length of hospital stay in Group A (hypertonic saline) was 1.83 ± 1.13 days while in Group B (normal saline) was 3.37 ± 1.39 days (p-value = 0.0001).</p> <p><strong>Conclusion: </strong>This study concluded that mean length of hospital stay is less in patients nebulized with 3% hypertonic saline in treatment of Bronchiolitis as compared to 0.9% normal saline.</p> Abida Parveen Saima Naureen Rizwan Waseem Tayyaba Noor Tooba Malik Bushra Shaheen Copyright (c) 2025 Pakistan Pediatric Journal 2025-08-16 2025-08-16 49 2 Genotype phenotype variability and genetic spectrum of inherited neuropathies in children https://www.ppj.org.pk/index.php/ppj/article/view/548 <p><strong>Abstract</strong></p> <p><strong>Objective</strong></p> <p>To characterize the clinical, electrophysiological, and genetic spectrum of inherited neuropathies (INs) in a pediatric cohort, emphasizing genotype-phenotype correlations and the role of whole-exome sequencing (WES) in genetic diagnosis.</p> <p><strong>Study Design</strong></p> <p>Cross-sectional observational study.</p> <p><strong>Place and Duration of Study</strong></p> <p>Pediatric Neurology Department, Children’s Hospital and University of Child Health Sciences, Lahore, Pakistan, from January 2017 to January 2023.</p> <p><strong>Materials and Methods</strong></p> <p>Twenty-eight pediatric patients (≤18 years) with genetically confirmed inherited neuropathies were enrolled. Clinical, electrophysiological, and genetic assessments were conducted. Nerve conduction studies classified neuropathies into axonal, demyelinating, or mixed subtypes. Whole-exome sequencing (WES) was performed in collaboration with UCL Institute of Neurology, London. Statistical analysis was performed with SPSS version 26 and p-value of 0.05 was considered significant.</p> <p><strong>Results</strong></p> <p>The cohort had a median symptom onset at 4 years (IQR: 2–6 years), with 96.4% consanguinity. Muscle atrophy (35.7%) and pes cavus (21.4%) were common. Electrophysiology classified neuropathies into demyelinating (28.6%), axonal (14.3%), and mixed (46.4%) subtypes. WES yielded a 68.29% diagnostic rate, with GAN and PDXK mutations (14.3% each) most common.</p> <p><strong>Conclusion</strong></p> <p>This study highlights the genetic heterogeneity of inherited neuropathies and the importance of WES for genetic diagnosis and targeted management, particularly in high-consanguinity populations.</p> Arshad Mehmood SHAILA ALI AHMED BILAL JAVERIA RAZA ALVI SAMEEN QURESHI TIPU SULTAN Copyright (c) 2025 Pakistan Pediatric Journal 2025-08-16 2025-08-16 49 2 Extensive Drug Resistant Typhoid in Pediatric Age Group https://www.ppj.org.pk/index.php/ppj/article/view/373 <p>&nbsp;</p> <p>Abstract:<br>Objectives: To determine the frequency of extensive drug resistant (XDR) Typhoid in pediatric age group in Peads unit IV Bolan Medical Complex Hospital Quetta.<br>Materials and Methods: This study was conducted in the Department of Pediatrics Unit-II Bolan medical college hospital Quetta. Detailed history and physical examination of children with suspected enteric fever was carried out. Using aseptic measures 3-5 ml of venous blood was collected into BactecPeds Plus culture bottles before initiation of therapy. Antimicrobial susceptibility testing was performed. Isolates resistant to ciprofloxacin, co-trimoxazole, chloramphenicol, ampicillin and ceftriaxone were deemed as extensively drug resistant positive. Later, results were analyzed by senior resident which again were checked by consultant before putting the data onto Proforma. Afterwards, data analysis procedure was started as soon as required sample data was achieved. All the demographic details and study results were recorded on a predesigned data collection proforma.<br>Results: A total of 137 patients with mean length of hospital stay of 7.51+2.01 days were enrolled. Out of total 79 (58%) were males and 58 (42%) were female. The frequency of blood culture positive to Samonela Typhi in our study was found to be 66%. Moreover, the overall patients found with extensively dug resistance were 35 % out of 137. There was significant relationship found between blood culture positivity and extensive drug resistance. Out of 91 positive blood cultures, 48 were extensive drug resistance respectively.<br>Conclusion: The occurrence of XDR typhoid infection secondary to antimicrobial resistance has also put a huge impact on our population. Azithromycin is a convenient choice based on cost of therapy and availability in oral form. However, there is a remarkable response observed on treatment with Meropenem along with Azithromycin in XDR typhoid fever. Typhoid conjugate vaccine (TCV) vaccination can significantly decrease the burden of typhoid and may also impact antimicrobial resistance.</p> <p>&nbsp;</p> Shabana akber Habibullah babar Waqas younus Copyright (c) 2025 Pakistan Pediatric Journal 2025-08-16 2025-08-16 49 2 Assessment of timely diagnosis practices in pediatric rheumatology: A survey of Current Trends and Challenges in Pakistan https://www.ppj.org.pk/index.php/ppj/article/view/535 <p><strong>Background: </strong>Paediatric rheumatic illnesses <strong>comprise</strong> a spectrum of inflammatory disorders. These ailments <strong>persist</strong> as the leading cause of acquired disability in children. Pediatric rheumatology is still deemed a puzzling field of pediatric healthcare, particularly among underdeveloped regions. This study aimed to address key aspects of pediatric rheumatology care in Pakistan through a multi-dimensional framework. It emphasized diagnostic practices including evaluation of challenges in diagnosis, and identifying the referral systems.</p> <p><strong>Study Design:</strong> &nbsp;Cross sectional Survey</p> <p><strong>Place and Duration of study:</strong> It is multicentered survey, From October 2024 to December 2024.</p> <p><strong>Methods:</strong> This cross-sectional study used an online survey and convenience sampling to recruit 213 pediatricians and healthcare professionals in Pakistan. A structured questionnaire assessed PR diagnosis, referral patterns, and barriers. Participants received the survey link via social media and email, with data analyzed using SPSS version 26, employing descriptive statistics and chi-square tests.</p> <p><strong>Results: </strong>Most respondents (68.5%) encountered pediatric rheumatology cases occasionally, and 85.4% collaborated with specialists, though only 5.6% were very familiar with management guidelines. Referral practices were largely driven by challenges, with 56.8% referring when stuck during management and 37.1% referring before treatment. Key barriers to prompt diagnosis included inadequate training (70.4%), low public awareness (61.0%), and a shortage of specialized rheumatologists (58.2%). Familiarity with guidelines varied by educational background, with most FCPS-qualified respondents being somewhat familiar, but no significant association between familiarity and education was found.</p> <p><strong>Conclusion: </strong>The findings highlight critical barriers to prompt diagnosis of pediatric rheumatological conditions, including limited availability of multidisciplinary teams, a shortage of specialized pediatric rheumatologists, low public awareness of pediatric rheumatic diseases, insufficient awareness among healthcare providers, and inadequate training for pediatricians.</p> <p><strong>Background: </strong>Paediatric rheumatic illnesses <strong>comprise</strong> a spectrum of inflammatory disorders. These ailments <strong>persist</strong> as the leading cause of acquired disability in children. Pediatric rheumatology is still deemed a puzzling field of pediatric healthcare, particularly among underdeveloped regions. This study aimed to address key aspects of pediatric rheumatology care in Pakistan through a multi-dimensional framework. It emphasized diagnostic practices including evaluation of challenges in diagnosis, and identifying the referral systems.</p> <p><strong>Study Design:</strong> &nbsp;Cross sectional Survey</p> <p><strong>Place and Duration of study:</strong> It is multicentered survey, From October 2024 to December 2024.</p> <p><strong>Methods:</strong> This cross-sectional study used an online survey and convenience sampling to recruit 213 pediatricians and healthcare professionals in Pakistan. A structured questionnaire assessed PR diagnosis, referral patterns, and barriers. Participants received the survey link via social media and email, with data analyzed using SPSS version 26, employing descriptive statistics and chi-square tests.</p> <p><strong>Results: </strong>Most respondents (68.5%) encountered pediatric rheumatology cases occasionally, and 85.4% collaborated with specialists, though only 5.6% were very familiar with management guidelines. Referral practices were largely driven by challenges, with 56.8% referring when stuck during management and 37.1% referring before treatment. Key barriers to prompt diagnosis included inadequate training (70.4%), low public awareness (61.0%), and a shortage of specialized rheumatologists (58.2%). Familiarity with guidelines varied by educational background, with most FCPS-qualified respondents being somewhat familiar, but no significant association between familiarity and education was found.</p> <p><strong>Conclusion: </strong>The findings highlight critical barriers to prompt diagnosis of pediatric rheumatological conditions, including limited availability of multidisciplinary teams, a shortage of specialized pediatric rheumatologists, low public awareness of pediatric rheumatic diseases, insufficient awareness among healthcare providers, and inadequate training for pediatricians.</p> Shazia Rizwan Sobia Qamar Samia Naz Sobia Shahalam Rabeya Rehman Copyright (c) 2025 Pakistan Pediatric Journal 2025-08-16 2025-08-16 49 2 Relationship between Lactate levels and Shock Index in pediatric patients with sepsis at a tertiary care Hospital in Karachi, Pakistan. https://www.ppj.org.pk/index.php/ppj/article/view/498 <p>Background &amp;amp; Objectives: Septic shock is common in children and is linked to high rates of<br>mortality and morbidity. Early identification of severe sepsis can improve outcomes. The Shock<br>Index (SI), which is the ratio of heart rate (HR) to systolic blood pressure (SBP), could serve as<br>a valuable noninvasive indicator of hemodynamic instability; however, it has been inadequately<br>studied in pediatric populations. The objective of this study was to assess the potential of SI as<br>an early prognostic marker for septic shock in children.<br>Methods: A cross-sectional study was conducted on children aged 1 to 60 months who<br>presented with septic shock at the pediatric intensive care unit of Civil Hospital Karachi. Heart<br>rate (HR) and systolic blood pressure (SBP) were recorded for Shock Index (SI) calculation,<br>along with lactate concentrations, at 0, 2, 4, and 6 hours after admission. The outcome (death or<br>survival) was documented for all patients. We calculated the Pearson correlation coefficient (r)<br>between SI and lactate levels and assessed the area under the curve (AUC) to determine the<br>sensitivity and specificity of both SI and lactate levels in predicting mortality.<br>Results: This study enrolled 153 children admitted with septic shock, with an almost equal<br>distribution of males and females. The mortality rate was 63.4%, with significant differences<br>observed in survival rates across age groups, particularly between 1-2 years and 2-4 years<br>(p=0.049). Gastroenteritis was the most common diagnosis, while myocarditis was more<br>prevalent in deceased patients (p&amp;lt;0.0001). A strong correlation between serum lactate levels<br>and Shock Index (SI) was found (r=0.910 to 0.942, p&amp;lt;0.001), with both markers showing high<br>sensitivity and specificity for predicting mortality across various time intervals. The Shock<br>Index demonstrated strong diagnostic performance across all age groups, making it a reliable<br>prognostic tool for pediatric septic shock.<br>Conclusions: In our cohort of children with septic shock, the Shock Index (SI) proved to be a<br>clinically significant and easily calculated predictor of mortality. It may provide a more accurate<br>assessment of hemodynamic status than heart rate (HR) and systolic blood pressure (SBP)<br>alone, enabling earlier detection of severe sepsis.<br>KeyWords: sepsis, severe sepsis, septic shock, shock index, prognostic, lactate level</p> Aleena Ayesha Saleem Waseem Ahmed Jamalvi Muzamil Shabana Ejaz Samra Midhat Farooq Malik Copyright (c) 2025 Pakistan Pediatric Journal 2025-08-16 2025-08-16 49 2 To evaluate the efficacy and safety of ketogenic diet in patients of refractory epilepsy https://www.ppj.org.pk/index.php/ppj/article/view/263 <p><strong>OBJECTIVE</strong> <strong>:</strong></p> <p>To evaluate the efficacy and safety of ketogenic diet in patients of refractory epilepsy.</p> <p><strong>STUDY DESIGN:</strong></p> <p>Prospective interventional study.</p> <p><strong>PLACE AND DURATION OF STUDY</strong>:</p> <p>The study was conducted at Pediatric Neurology Department. The University of Child Health and The Children Hospital Lahore from Jan 2020 - Dec 2021. Study was conducted after approval of synopsis.</p> <p><strong>MATERIAL AND METHOD:</strong></p> <p>All children with refractory epilepsy of age 6 months to 18 years and both genders were included in the study while Children of refractory epilepsy with associated hepatic, renal or cardiac issues, patients of hyperlipidemia and those who refused to take ketogenic diet after explaining the benefits and side effects of the diet were excluded from the study</p> <p><strong>Results: </strong>Out of 50 patients, there was male predominance (64%). In 48 % patients the seizure burden reduced to 90%from the baseline. 72 % presented with good compliance.&nbsp; 60% patients presented with no complications. Among the rest, constipation was the most common complication. Good seizure control is associated with good compliance and good education status of parents.</p> <p><strong>Conclusion:</strong></p> <p>Ketogenic diet is a good modality for the treatment of refractory epilepsy and its effectiveness is associated with good compliance resulting in better control of seizure burden.</p> <p><strong>Key words</strong>: ketogenic diet, refractory epilepsy</p> Natasha Ghani Ghani Shaila Ali Shumaila Rafiq Zia Ur Rehman Tipu Sultan Copyright (c) 2025 Pakistan Pediatric Journal 2025-08-16 2025-08-16 49 2 Factors Affecting the Outcomes of Activities of Daily Living (ADLs) Based Intervention on Spastic Hemiplegic Cerebral Palsy https://www.ppj.org.pk/index.php/ppj/article/view/467 <p><strong>ABSTRACT</strong></p> <p><strong>&nbsp;</strong><strong>Objective: </strong>To observe the effects of different factors on ADLs-based intervention in spastic hemiplegic cerebral palsy.</p> <p><strong>Place and Duration of the Study</strong>: This observational study was conducted at the Department of Developmental and Behavioral Pediatrics; Children Hospital Lahore, PSRD (Pakistan Society of Rehabilitation for Disabled), and Private Clinical Setups. This study was completed in 6 months.</p> <p><strong>Materials and Methods:</strong> A total of 37 were taken into observation to fill out the questionnaire. The patients taken in this study were all spastic hemiplegic cerebral palsy, with under-going interventions based on ADLs technique.</p> <p><strong>Results:</strong> The result shows that the Education and financial status of the parents, availability of tools, and time to travel to the therapy center have an almost insignificant impact on the undergoing therapy. While the working status of mothers, level of impairment, and family history of the disease has a moderate effect. Vaccination course, time per session, number of sessions per week, Regularity of sessions, involvement and compliance of the patient, comorbidities, and observation of the parents have a significant impact on ADLs-based intervention.</p> <p>&nbsp;<strong>Conclusions:</strong> Patients with more educated parents, better financial status, non-working status of the mother, complete vaccination, more duration of session, more number and regular session, complete involvement of patient with good compliance, having no other comorbidities and family history of disease, low level of impairment, availability of tools, observation of professional and less time to travel shows better results during ADLs based intervention.</p> <p><strong>Keywords:</strong> ADLs (Activities of daily living), sessions, Cerebral palsy.</p> <p>&nbsp;</p> Amna Kamal Ahmed Kamal Khan Copyright (c) 2025 Pakistan Pediatric Journal 2025-08-16 2025-08-16 49 2 Stress and Compliance with A Home Exercise Program Among Caregivers of Children with Disabilities https://www.ppj.org.pk/index.php/ppj/article/view/414 <p><strong>Objective:</strong></p> <p>We sought to identify the relationship between caregiver stress and compliance with home programs in caregivers of children with disabilities.</p> <h5>Methods:</h5> <p>In 2023, a population-based study was conducted in Karachi, Pakistan. Participants aged 20 years to 50 years and older were selected according to convenience sampling strategy.100 caregivers of children with disabilities responded to a survey to determine their level of compliance with a prescribed home physical therapy program and the level of caregiver stress. Compliance was defined as the degree to which caregivers followed the prescribed frequency of the home program.</p> <h5>Results:</h5> <p>According to the results, it was reported that the caregivers showed noncompliance with the home programs being prescribed by the physical therapists. The data analysis through linear regression revealed a strong and significant relationship between the caregiver’s non-compliance with the home program and the level of stress they stated. Correlation coefficients showed a strong and significant relationship between domestic problems and noncompliance with home programs. This reveals that the home program noncompliance increased due to the caregiver and family problems increment.</p> <h5>Conclusion:</h5> <p>Caregivers of children with disabilities experience stress that should be addressed by the therapists to maximize compliance with home programs.</p> Faiza Hakeem Aqsa Faiz Mubushara Afzal Bahadur Kanwer Copyright (c) 2025 Pakistan Pediatric Journal 2025-08-16 2025-08-16 49 2 Carnitine-acylcarnitine translocase deficiency (CACTD) in an infant who presented with convulsions in Pediatrics Department of Punjab Rangers Teaching Hospital (PRTH), Lahore https://www.ppj.org.pk/index.php/ppj/article/view/469 <p>Carnitine-acylcarnitine translocase deficiency (CACTD) is an autosomal disorder of mitochondrial oxidation of fatty acids, occurring due to mutation in gene named SLC25A20. Due to this deficiency, the carnitine cycle is disrupted which is responsible for providing energy during fasting states and expenditure of high energy levels. Its deficiency causes hypoglycemia and hyperammonemia affecting functions of brain, liver, and heart, causing muscle weakness. An 8-month-old baby girl presented with the complaint of two episodes of generalized tonic-clonic convulsions, lasting for 5-10 seconds, which were self-resolving, without cyanosis or frothing from mouth. There was also complaint of loose stools. On detailed examination and lab diagnosis it was found that the patient had CACTD. The patient was given cornita syrup i.e. carnitine supplement along with iron supplement. Also, the mother was counselled to feed the baby frequently and not to keep baby hungry more than 4 hours.</p> Faiqa Fazal Ayesha Fazal Copyright (c) 2025 Pakistan Pediatric Journal 2025-08-16 2025-08-16 49 2 An Abdominal mass in a child with PICA; Rapunzel syndrome. A case report https://www.ppj.org.pk/index.php/ppj/article/view/490 <p><strong>ABSTRACT:</strong></p> <p>Rapunzel syndrome is an unusual and rare presentation of gastric trichobezoar that can extends from stomach to small gut or beyond. This case report is about a 6-year-old girl who presented with complaint of severe abdominal pain and vomiting in OPD of pediatric gastroenterology, General hospital, Lahore. There was a palpable mass in epigastric region along with pallor. A trichobezoar was diagnosed on esophagogastroduodenoscopy that was extending to the colon and was removed surgically. After treatment and cognitive behavioral therapy symptoms have resolved completely.</p> <p>Trichobezoar is a rare entity but must be kept as a differential diagnosis in a patient with abdominal pain and palpable mass.</p> Hooria Rehman Hassan Suleman Muhammad Shahid Rizwan Gohar Waheeh ur Rehman Rehman Sidra Rasheed Copyright (c) 2025 Pakistan Pediatric Journal 2025-08-16 2025-08-16 49 2 Case Report Congenital Erythropoietic Porphyria (Gunther Disease): A Rare Case of Photosensitivity and Erythrodontia https://www.ppj.org.pk/index.php/ppj/article/view/566 <p data-start="314" data-end="761">Porphyria comprises a group of metabolic disorders caused by enzyme deficiencies in the heme biosynthetic pathway, leading to the accumulation of porphyrins. Congenital erythropoietic porphyria (CEP), also known as Gunther disease, is a rare autosomal recessive condition resulting from a deficiency of uroporphyrinogen III cosynthase (UROS). It manifests as painful photosensitivity, erythrodontia, and chronic hemolytic anemia.</p> <p data-start="763" data-end="1178">We present a case of a 17-month-old boy with reddish discoloration of urine, erythrodontia, and a painful sclerodermatous rash. The diagnosis was primarily clinical and confirmed by elevated urinary levels of uroporphyrin and coproporphyrin. Management included sun protection, vitamin D supplementation, cimetidine, and beta-carotene. While the patient showed improvement, dyspigmentation and scarring persisted.</p> <p data-start="1180" data-end="1319">This case underscores the importance of early diagnosis and intervention in metabolic disorders like CEP to prevent severe complications.</p> <p data-start="1321" data-end="1429"><strong data-start="1321" data-end="1334">Keywords:</strong> porphyria, uroporphyrin III synthetase, sclerodermatous rash, reddish discoloration of urine</p> Urwa Khalil Tarifa Illiyas Fizza Agha Copyright (c) 2025 Pakistan Pediatric Journal 2025-08-16 2025-08-16 49 2 A A Rare Case of Human Herpesvirus 7 and Adenovirus Meningoencephalitis https://www.ppj.org.pk/index.php/ppj/article/view/568 <p><span style="font-weight: 400;">Encephalitis is characterized by altered mental status and additional neurological criteria. Although commonly linked to herpesviruses, its association with human herpesvirus 7 (HHV-7) and adenovirus in immunocompetent individuals is rare. We present the case of an 11-year-old boy admitted with fever, seizures, and altered level of consciousness. Initial cerebrospinal fluid (CSF) analysis indicated pleocytosis with elevated protein levels. Brain magnetic resonance imaging (MRI) showed mild cortical swelling and hyperintense signals. NeuroPCR identified HHV-7 and adenovirus in the CSF. Empirical treatment with corticosteroids, ceftriaxone, vancomycin, and acyclovir was initiated. As the patient’s condition deteriorated, acyclovir was replaced with ganciclovir, resulting in significant neurological improvement. He was discharged with continued recovery over four months. This case highlights the importance of early viral identification and targeted antiviral therapy in encephalitis management. Further research is needed to understand the pathogenic role of HHV-7 and adenovirus in immunocompetent individuals.</span></p> Shawal Sajid Hira Nawaz Murtaza Ali Gova Uzma Siddique Afshan Asif Aasma Kayani Copyright (c) 2025 Pakistan Pediatric Journal 2025-08-25 2025-08-25 49 2 The he Use of Early Oral Corticosteroid for Henoch-Schönlein Purpura: A Case Report from Rural Primary Health Care https://www.ppj.org.pk/index.php/ppj/article/view/200 <p>Henoch-schönlein purpura (HSP) is a frequent kind of vasculitis juvenile population. This case report narrated the use of early oral corticosteroid in HSP. A 13 year-old girl came with non-itchy rash on all extremities&nbsp; and bilateral knee pain since 3 weeks before. Physical examinations and laboratory tests were performed. The diagnosis of HSP was made. The patient was prescribed with oral prednisone, analgesics, and antacids. Musculoskeletal symptom was resolved within two days and the purpura slowly disappeared within a week. No standardization of prescribing early oral corticosteroid in HSP yet. Initiation of oral corticosteroid might improve the symptoms rapidly.</p> Indah Sagitaisna Putri Bastomy Eka Rezkita Steven Irving Farida Indriawati Copyright (c) 2025 Pakistan Pediatric Journal 2025-08-16 2025-08-16 49 2