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.

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.