Red Blood Cell Alloimmunization in Pediatric group with Beta Thalassemia: A Five-Year Experience.

Beta-thalassemia is one of the most frequently occurring hematological disorders in [Removed for blinded peer-review]. Regular blood transfusion is required in almost all cases for management. However, this is associated with significant major complications like red blood cell (RBC) alloimmunization. This retrospective cross-sectional is conducted to evaluate the RBC alloimmunization frequency in children with beta-thalassemia aged between 6 months and 16 years in [Removed for blinded peer-review]. Antibody screening was performed using the Dia clon3 cell antigen panel. If the screening came back positive, a detailed panel was created for the identification of specific antibody. In our sample, the frequency of RBC alloimmunization was found in 22 (26.19%) patients. Of these 22 patients, the Rhesus system was found in most patients 17 (77.3%), followed by Kell 5 (22.7%). RBC alloimmunization was significantly associated with a family history of a blood disorder and splenectomy.

Paediatric Bickerstaff brainstem encephalitis: A rare case report.

Bickerstaff Brainstem Encephalitis, a rare variant of Guillain- Barre Syndrome has an estimated prevalence of eight per 100 million individuals. It presents with the classic triad of ataxia, ophthalmoplegia and altered consciousness. We report the case of a 10-year-old child who presented with low grade fever, hypersomnia and inability to walk. Central nervous system examination revealed ophthalmoplegia and multiple cranial nerve palsies. However, CSF examination showed lack of albuminocytological dissociation with no previous history of respiratory or gastrointestinal tract infection. Unavailability of Anti-GQ1b antibodies led to a diagnosis based on suggestive clinical features, abnormal MRI signals and prompt response to corticosteroid administration. Intravenous Methyl Prednisolone in a dose of 30mg/kg/day was administered for 10 days followed by oral Prednisolone 2 mg/kg/day. After complete recovery the patient was discharged, Prednisolone was tapered gradually and eventually discontinued after four months.

Electrolyte Imbalance in Children With Severe Acute Malnutrition at a Tertiary Care Hospital in Pakistan: A Cross-Sectional Study.

Background Malnutrition is a significant public health concern and a leading contributor to the global burden of children's diseases, affecting 50 to 150 million children under the age of five years worldwide. Globally, undernutrition accounts for approximately 33% of the deaths among under-fives. South Asia alone contributes to 50% and 38.8% of the world's population of wasted and stunted children, respectively. In Pakistan, malnutrition is the leading cause of childhood mortality, accounting for nearly 35% of all deaths under five years of age. Severe acute malnutrition (SAM), the most severe form of malnutrition, is often associated with electrolyte imbalances. This study aimed to determine the frequency of electrolyte imbalance in children with SAM admitted at a tertiary care hospital. Methods This cross-sectional study includes 184 patients with SAM aged between 6 and 60 months, who were admitted at the inpatient Department of Pediatrics, Dr. Ruth K. M. Pfau, Civil Hospital, Karachi, Pakistan, from January 17, 2017 to July 16, 2017. Weight and length/height were measured, and weight-for-height was calculated. Children were labeled to have SAM when weight-for-height was below -3 standard deviation (SD). Blood samples for serum electrolytes were drawn and sent to the lab. Descriptive statistics were calculated, and stratification was performed using the chi-square test. A p-value of ≤0.05 was considered statistically significant. Results  The mean age of participants was 22.63 ± 12.71 months. Of the 184 patients with SAM, 172 (93.5%) patients had electrolyte imbalance. Hypokalemia was present in 79.9%, whereas hypocalcemia, hyponatremia, and hypomagnesemia were present in 71.7%, 48.9%, and 13.6%, respectively. Post-stratification results showed a significant association of electrolyte imbalance with gender (p = 0.005) and educational status of parents (p = 0.001). Conclusions Electrolyte disturbances are common in SAM. Serum electrolytes of every malnourished child admitted should be assessed and corrected to avoid fatal outcomes. We suggest that more research with better study designs should be conducted to develop policies and strategies for successfully combating malnutrition in Pakistan. In the meantime, we recommend adopting national guidelines for the management of acute malnutrition to reduce morbidity and mortality.

Parental Refusal to Lumbar Puncture: Effects on Treatment, Hospital Stay and Leave Against Medical Advice.

Introduction Lumbar puncture (LP) is an effective method in the diagnosis and management of central nervous system infections. Refusal to LP is associated with severe consequences. This study aims to examine the impact of parental LP refusal on treatment, the length of hospital stay, and the frequency of patients leaving against medical advice (LAMA). Method A cross-sectional study was conducted at the pediatric department of Civil Hospital, Karachi, from June 2018 to November 2019. All hospitalized patients suspected to have a central nervous system disease, which requires LP, were enrolled. Patients were followed for the duration of antibiotic and antiviral therapy, length of hospital stay, and LAMA. Results A total of 220 patients participated in the study, with the median age of nine (2-47) months. There were 113 (51.1%) males. The median length of hospital stay was 10 (4-14) days. The comparison of parental LP refusal with the length of hospital stay showed a significant difference (p-value <0.001) in the number of days of treatment among patients who received vancomycin (p-value =0.008) and meropenem (p-value =0.012). A significant association of parental LP refusal was also observed with meningoencephalitis and meningitis as provisional diagnosis (p-value =0.006). In particular, LAMA and death were found significantly higher among parents who refused LP (p-value <0.001).  Conclusion LP refusal has a significant effect on the treatment, hospital stay, and disposition outcomes. A large number of parents who declined the procedure left against medical advice or suffered grave medical consequences. Parental education addressing their concerns and beliefs, while explaining the indications, and need for performing LP can help effectively overcome this issue.

Frequency and Associated Factors of Parental Refusal to Perform Lumbar Puncture in Children with Suspected Central Nervous System Infection: A Cross-sectional Study.

Objective Lumbar puncture (LP) is a useful procedure which is performed for both diagnosis and treatment of numerous conditions affecting children and adults. The purpose of this study was to determine the frequency and cause of increased parental refusal to perform LP in the pediatric population. Method A cross-sectional study was conducted from January 2018 to June 2019 at the Civil Hospital, Dow University of Health Sciences, pediatric department, Civil Hospital, Karachi. Over the 18-month time period, a total of 215 patients who had indications of LP were selected from the in-patient pediatrics department; the age range was between newborn to 12 years of age. The mode of research was a questionnaire and interview-based method that was conducted with guardians of minor patients to understand the extent of their knowledge and awareness about the LP procedure as well as its complication and the role of culture, education background, and financial status of the families which may lead to an increased likelihood of refusal. Result The frequency of LP refusal amongst the 215 families of the patients that were interviewed was found to be 32.6%. Mean age of the respondents was 30.98 years. The decision for LP was not significantly affected by the subjects' gender (p=0.1), by the religious communities to which the families belonged (p=0.9), their ethnicities (0.52), or by the families' financial status (p=0.4). It was observed that when indications for performing LP were appropriately explained, there was a significantly greater number of consents given as compared to when they were not made clear (p=0.009). Explaining the complications of the procedure did not considerably impact the decision for refusal of the procedure (p=0.1). The multi-variable logistic regression analysis model was applied to determine the likelihood of variables affecting refusal of LP and the logistic regression model was found to be statistically significant, χ2 (8) = 38.2 p < 0. 001. Conclusion Lack of knowledge about the LP procedure and fear of ramification plays a conspicuous role in the denial of LP procedure by the guardians of minor patients. A better, simpler approach using standardized consent forms by the doctors may lead to the removal of the information gaps and can provide a better understanding about the concerned risks, the primary indications, and the benefits of this procedure to the guardians.

Factor XIII deficiency with intracranial haemorrhage.

A 5-year-old girl presented to paediatric emergency with fever and seizures for a short duration. At first, meningitis was suspected and management was started empirically. There was no improvement in the clinical condition of the patient and investigations revealed spontaneous intracranial haemorrhage (ICH) secondary to factor XIII deficiency. The child was transfused cryoprecipitate and managed conservatively for ICH. She became asymptomatic and was kept on monthly cryoprecipitate transfusions. This case report summarises factor XIII deficiency in ICH which was not suspected initially, but diagnosed later on after CT scan head and factor XIII assay. This report also highlights events occurring during its management.

Frequency and features of nocturnal enuresis in Pakistani children aged 5 to 16 years based on ICCS criteria: a multi-center cross-sectional study from Karachi, Pakistan.

BACKGROUND: Nocturnal enuresis (NE) is a common symptom in children worldwide. International Children's Continence Society (ICCS) defines enuresis as either mono-symptomatic, NE with lower urinary tract symptoms and NE with co-morbid conditions. The objectives of this study were to determine the frequencies and types of NE and associated symptoms and conditions in children aged 5 to 16 years based on ICCS criteria. METHODS: A multi-center cross sectional study was conducted between November 2012 and December 2013 in the primary care clinics of four hospitals in Karachi. Children aged five to fifteen years were included through consecutive sampling. Informed consent was obtained from the parents and a pre-coded semi-structured questionnaire was used to obtain the information. Data was entered on SPSS version 20.0 and multivariable logistic regression analysis was used for data analysis. RESULTS: Out of 429 children aged between five and sixteen years, 243(56.9%) were boys and the remaining 186(43.1%) were girls. One hundred and eighty three children (43%) had nocturnal enuresis (NE). Forty four (10.3%), had mono-symptomatic NE, 57(31.1%) had associated lower urinary tract symptoms (NE-LUTS), whereas 30 (16.3%) had NE with a co-morbid condition. Fifty two (28.4%) NE's had at least one of both LUTS and a co-morbid condition. Out of the 246(57%) non-enuretic's, 31(12.6%) had a LUTS, 95(38.6%) had a co-morbid condition and 57(23.2%) had at least one of both LUTS and a co-morbid condition. The remaining 63 (25.6%) were symptom free. Increased voiding frequency, urgency, dysuria, suprapubic pain and daytime incontinence were the LUTS significantly associated with NE. Co-morbid conditions significantly associated with NE included constipation, congenital defects, developmental delay, and learning and sleep problems. CONCLUSION: Although NE can be an only symptom, it is often associated with lower urinary tract symptoms like dysuria, urgency, suprapubic pain, and daytime incontinence. Children presenting with NE often have co-morbid conditions like constipation, urinary tract infection, sleep disorders, and developmental delay. Many children presenting with these conditions as the primary complaint may also have NE. It should be addressed as unrecognized and untreated NE can cause additional morbidity and distress.

Efficacy and adverse effects of oral chelating therapy (deferasirox) in multi-transfused Pakistani children with ß-thalassemia major.

OBJECTIVE: To determine the efficacy and adverse effects of oral chelation therapy (deferasirox) in multi-transfused ß-thalassemia major patients visiting pediatric thalassemia clinic in Civil Hospital Karachi. METHODS: This prospective study was conducted at pediatric thalassemia clinic of Civil Hospital Karachi. Hundred multi-transfused ß-thalassemia patients registered in the clinic for oral iron chelation therapy were included in the study. Information regarding clinical and laboratory parameters including abdominal pain, jaundice, serum ferritin, creatinine and serum transaminase levels were recorded on a Performa and data was analyzed through SPSS 16. RESULTS: Hundred patients were stratified into two age groups, 54% were below and 46% were above nine year. Majority were males, 62% and 38% were females. Abdominal pain 41%, nausea 31%, vomiting 15%, jaundice 15% and elevated serum creatinine 11.5% were frequently observed clinical adverse effects in this study. Serum glutamic pyruvic transaminase (SGPT) level was statistically significant compared with initial visit and six months after optimal chelation therapy (p=0.030). Although Serum ferritin was decreased but it was not statistically significant (p=0.929). CONCLUSION: Deferasirox is an effective oral chelation agent for ß-thalassemia major patients. Most common adverse effects of the drug are abdominal pain, nausea, vomiting, and elevation of liver enzymes.

Stunting and micronutrient deficiencies in malnourished children.

OBJECTIVE: To determine the frequency of stunting and micronutrient deficiencies in malnourished children admitted in a tertiary care hospital. METHODS: It was a cross-sectional study conducted at Paediatric department of Civil Hospital Karachi from January 2007 to December 2008. Patients aged 6 to 60 months, admitted in the ward were assessed for nutritional status and stunting according to the WHO classification of malnutrition, mild (weight for height ratio between -1SD to -2SD) moderate (-2SD TO-3SD) and severe (less than -3SD of NCHS/WHO reference values) malnourished children were included in the study. All the patients were subjected to clinical examination, which included height/length, weight and clinical signs of micronutrient deficiencies. Laboratory investigations were done to confirm the clinical diagnosis of iron deficiency anaemia and rickets. The collected data was analyzed by Statistical Program SPSS version 15. Frequencies and percentages were computed to present all categorical variables. Quantitative variables such as age was presented by mean +/- SD. RESULTS: A total of 150 patients were enrolled in this study. Their mean age was 24.4 +/- 13.8 months (range 6 to 60 months). Of all, 63 (44%) patients were severely stunted, 44 (29%) had moderate stunting, 27 (18%) had mild stunting while only 16 (10.7%) had normal stature. Severely malnourished (< -3SD) were 119 (79%) patients, 30 (20%) patients had moderate malnutrition (-2SD to -3SD). Forty two percent severely malnourished children also had severe stunting. Anaemia was the most common micronutrient deficiency seen in 117 (78%) patients, out of these 88% had iron deficiency anaemia, Rickets was found in 54(36%) patients. Vitamin A deficiency was present in 21 (14%) cases. Other miscellaneous micronutrient deficiencies were zinc and B-complex deficiency in 42 (28%). CONCLUSION: Malnutrition and associated micronutrient deficiencies were frequently found in children between 13 to 24 months of age. Stunting was commonly present between 25 to 36 months of age, it was associated with severe malnutrition in 42% cases. Anaemia is the most common micronutrient deficiency in malnourished patients, it was observed in 78% of cases whereas 44% of severely stunted patients had coexisting rickets.

Hereditary prothrombin deficiency.

Hereditary prothrombin deficiency is one of the rare congenital coagulation defects. We report a case of 4 months old child who initially presented at 11/2 month of age with high-grade fever, generalized convulsions and brownish aspirate through nasogastric tube, diagnosed and managed as meningitis and sepsis. He was readmitted at 4 months of age with bruises over legs. Coagulation profile was suggestive of common pathway defect. Further evaluation revealed absent prothrombin level while other factors were within normal limits.