IgG4-related disease presenting with acute kidney injury and tubulointerstitial nephritis.

Immunoglobulin G-4 related disease (IgG4-RD) is an immune-mediated, inflammatory disease that often involves multiple organ systems. IgG4-RD can be classified according to the organs involved. Type 1 IgG4-RD is related to acute pancreatitis and sclerosing cholangitis. Disease manifestation is also seen in the retroperitoneal region, pelvic organs, and orbital space. Here we describe a rare case of IgG4-RD causing isolated acute kidney injury.

Referral Profile of Developmental Disabilities at a Tertiary Care Hospital in a Resource-limited Country.

The burden and the spectrum of neuro-developmental disabilities are well-established in the developed countries, however, they continue to be a challenge in the Low Middle-Income Countries. The aim of this study was to present the referral profile and trends of developmental disabilities in the child development centre of The Agha khan University, from 2012 to 2021. All children (1 month to 18 years), who had undergone diagnostic evaluations for suspected neurodevelopmental disabilities were included. A total of 1340 children were evaluated at the centre. The mean age of the children at the presentation was 5.4 ± 3.0 years. Male to female ratio was 3:1. Autism Spectrum Disorder (ASD) was the most common diagnosis (n=446, 33.3%). This study highlights a considerable burden and an increasing trend of children being referred for suspected developmental disabilities in Pakistan. Key Words: Developmental disabilities, Referral, Pakistan, Autism spectrum disorder.

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.

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.

Use of antioxidants supplementation on developmental outcomes in children with Down syndrome-A systematic review and meta-analyses.

BACKGROUND: The background of this study is to evaluate the published literature on the use of antioxidants in improving developmental outcomes in children with DS. METHODS: The systematic review included interventional studies (randomized controlled trials [RCTs] and quasi-RCTs [q-RCTs]) of children aged 0 to 18 years diagnosed with DS who received antioxidants to improve developmental outcomes. Studies were excluded if they were interventional studies with non-random allocation or lack of control group or non-interventional studies including observational studies, systematic and narrative review articles, editorials and commentaries. Studies were also excluded if data from participants with DS were included as part of a larger group of participants (e.g., children with intellectual disability) and the data could not be separated for subgroup analysis, or if outcomes did not assess developmental domains (e.g., reported biochemical markers only). The review included children with other conditions associated with developmental disability, only when data for children with DS were separately reported. RESULTS: Eleven RCTs and q-RCTs fulfilled the eligibility criteria with 683 participants with Down syndrome ranging in age from newborns to 17 years. Nine studies did not show any statistically significant clinical benefit of using antioxidants. Three studies were included in a meta-analysis comparing changes in the effect sizes (post and pre-intervention) in Global Developmental Quotient (GDQ) between the intervention and control groups. CONCLUSION: This review concludes that it is likely that the results are valid and suggest that the use of Antioxidant has no improvement in GDQ for children with DS. It is also unclear if any benefit exists for other developmental domains.

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.

Making an objective diagnosis of tetanus-utility of a simple neurophysiological test.

Tetanus remains a significant cause of mortality especially in the developing world. Early diagnosis and institution of treatment is critical to prevent fatal complications. The diagnosis is made on clinical grounds, which may sometimes be difficult, especially in case of localised tetanus. Being able to diagnose tetanus objectively is invaluable in such cases. In this regard, masseter inhibitory reflex (MIR) is a simple neurophysiological test that can be performed at the bedside. Herein, we report a case of craniocervical tetanus that was objectively diagnosed using MIR and adequately treated.

Factors associated with patient visits to the emergency department for asthma therapy in Pakistan.

BACKGROUND: Acute asthma is a chronic condition affecting people of all ages around the world and hence, is one of the leading causes of emergency department (ED) visits and hospital admissions globally. Most of them are related to poor patient practices and a weak healthcare system. The aim of our study was to assess the reasons for the increased usage of the ED by asthmatic patients in Pakistan. METHODS: A cross-sectional study was conducted on 600 asthmatic patients reporting to the ED of Civil Hospital Karachi over a 6-month period. The consenting patients were given a questionnaire to fill and the following data was collected: demographic information, duration of the disease, medications prescribed the, frequency of and reasons for outpatient clinic and ED visits for issues related to asthma. RESULT: According to our results most of the participants visited the ED to obtain a nebulized bronchodilator (90 %) or oxygen (79.5 %). Moreover, 44.8 % of the people visited the ED to get treatment without any delay and 24.0 % considered that the severity of asthma does not allow the patient to wait for clinic visits. Strikingly, 92.8 % claimed that inhaled corticosteroid therapy treatment should be stopped when patients feel better. Irregular follow ups with clinics, low education about asthma and an education level higher than a Bachelors degree were the most important factors associated with three or more ED visits per year, p values = 0.0289, 0.0110 and 0.0150 respectively. CONCLUSION: This study identifies several preventable risk factors responsible for recurrent visits to the ED by asthmatic patients in Pakistan.