Efficacy of probiotic in perennial allergic rhinitis under five year children: A randomized controlled trial.

OBJECTIVE: To determine the efficacy of probiotic (Lactobacillus Paracasei, LP-33) and compare it with cetirizine for the treatment of perennial allergic rhinitis in under five year's children. METHODS: The randomised clinical trial was conducted at Kharadar General Hospital, Karachi, from Dec 2016 to Nov 2017. Children aged 6 to 60 months, clinically presented with allergic rhinitis were included in the study. Total 212 children, randomized into intervention group A (received probiotic LP-33) and control group B (received cetirizine) for six weeks, were included in the analysis. Baseline allergic rhinitis symptoms (rhinorrhea, sneezing, nasal blocking, coughing, feeding & sleeping difficulties) were assessed after two and six weeks follow up and correlated both groups by using Pearson chi-square test. A p value of <0.05 were considered statistically significant. RESULTS: Total 212 children were analysed, out of them 113 (53.3%) were male. Mean age of study participant was 26 ± 16.64 months and mean body weight was 10.1 ± 3.26 Kg. More than 95% cases have shown significant improvement in their baseline symptoms (rhinorrhea, sneezing, nasal blocking, coughing, feeding difficulties and sleeping difficulties) in both intervention (L-33 Probiotic) and control (Cetirizine) groups. Statistically there was no difference in effectiveness of probiotic and cetirizine treatment for perennial allergic rhinitis (P > 0.05). CONCLUSIONS: Probiotic (LP-33) was equally effective as cetirizine in under five year's children for the treatment of perennial allergic rhinitis. Probiotic has additional benefit to treat allergic rhinitis without causing any major side effect in children reported by the study.

Clinical Efficacy Of Lactase Enzyme Supplement In Infant Colic: A Randomised Controlled Trial.

OBJECTIVE: To determine the efficacy of lactase enzyme supplement in infant colic. METHODS: The double-blind randomised clinical trial was conducted from November 2014 to June 2017 at Kharadar General Hospital, Karachi, and comprised infants aged 0-6 months with infant colic, excessive crying lasting at least 3 hours a day on at least 3 days a week for at least 3 weeks. The subjects were randomised into intervention group A which received lactase enzyme Colibid, and placebo group B. Five drops of intervention preparation were received by all the infants before each feed for two weeks. Confidentiality of active agent and placebo was maintained through drug codes. The duration of crying was recorded at baseline then after first and second weeks of intervention. The two groups were compared with level of significance set at p<0.05. RESULTS: There were 104 subjects with 52(50%) in each of the two groups Overall, 50(48.1%) were boys. At baseline, all (100%) the subjects had infant colic or excessive crying. After two-week intervention, significant improvement was seen in the duration of crying in group A 45(86.5%) compared to group B 31(59.6%) (p<0.05). CONCLUSIONS: Significant improvement was seen in the duration of crying in infants who received lactase enzyme supplement..

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.

Posterior mediastinal branchial cleft cyst: an unusual site.

A two and a half year old Kenyan girl presented with recurrent chest infections and difficulty in swallowing. Her clinical and laboratory workup was suggestive of lower respiratory tract infection for which she received a course of antibiotics; however, she remains symptomatic after the management of her suspected diagnosis. Therefore, further radiological workup including a chest CT scan and barium study were performed. This showed a homogeneous mass surrounded by ascending and descending aorta in the posterior mediastinum that was compressing the middle esophagus. She had a posterolateral thoracotomy which revealed a cystic mass with smooth surfaces (5x5 cm) in the posterior mediastinum. Histopathology showed branchial cleft cyst predominately lined by stratified squamous epithelium, with lymphocytes predominance. A final diagnosis of posterior mediastinal branchial cleft cyst was made. She was discharged home and remained well at follow-up.

Perception and practices of breastfeeding of infants 0-6 months in an urban and a semi-urban community in Pakistan: a cross-sectional study.

OBJECTIVE: To investigate mother's perception and practices about breastfeeding and their socio-demographic correlate in infants equal to or less than 6 months. METHODS: A cross-sectional study was carried out on 200 mother-infant pairs who visited the health care centers, Bilal Colony (semi-urban) and the Aga Khan University (urban), for their well baby follow-ups and vaccination using convenient sampling. Frequencies and percentages were computed and Chi-square was used to find associations between socio-demographics of mothers and their perception and practices about breastfeeding. RESULTS: Exclusive breastfeeding was reported by about 54% of the mothers. Thirty-five percent of the mothers gave prelacteal feed, 14% discarded colostrum and 43% woke up their infant to feed if time had exceeded 2 hours. Majority of the females were aware of the advantages (92%) and the disadvantages (85%) of breastfeeding. However, the awareness of positive feedback relationship of milk production and sucking was lacking and breast feeding was considered to cause weakness in mothers. CONCLUSION: Despite the efforts of health policy makers, the results show a situation that is not improving. Women were aware of the advantages and disadvantages of breast and bottle feeding but a disparity was observed between their perception and practices.

Comparison of oral versus injectable vitamin-D for the treatment of nutritional vitamin-D deficiency rickets.

OBJECTIVE: To assess the safety and acceptability of a single dose of vitamin-D versus the efficacy of injectable Vitamin-D versus oral vitamin-D. STUDY DESIGN: Case control. PLACE AND DURATION OF STUDY: It was carried out at the Department of Paediatrics, Kharadar General Hospital, Karachi, from August 2003 to April 2004. METHODOLOGY: Children of the age of 6 months to 3 years with clinical, biochemical and radiological evidence of vitamin- D deficiency rickets were included. The history, clinical examination, complete blood picture, serum calcium. Phosphorus, alkaline phosphatase and X-ray of wrist joint were done. The children were divided into two groups A and B. Group A was given oral vitamin-D and group B was given intramuscular injection of vitamin-D on the first day and then they were followed for two more visits at 30 and 90 days with clinical, biochemical and radiological examinations to assess the outcome. RESULTS: There were 50 confirmed cases of rickets in each group. The mean age was 10.9+5.1 months and 14.7+8.1 months in group A and B respectively. In these children, clinical features were weakness, difficulty in walking, frontal bossing, ribcage deformity and widening of wrist were seen. After one dose of vitamin-D (cholecalciferol), there was appreciable gain of weight and height and raised levels of alkaline phosphatase became normal during follow-up. Radiological florid rickets and non-florid rickets in both groups healed clinically during follow-up period. Oral and injectable forms of vitamin-D (cholecalciferol) were effective but injectable form was shown to be statistically significant. There were no undesirable side effects and both forms of treatment were well-tolerated.

Incomplete Kawaski disease: are we missing it?

Kawasaki disease, also known as mucocutaneous lymph node syndrome or infantile polyarteritis nodosa is an acute febrile vasculitis of unknown etiology with a predilection for coronary arteries and potential for aneurysm formation. In Incomplete Kawasaki disease, children with fever lack the sufficient number of criteria to fulfill the epidemiologic case definition and are diagnosed when coronary artery disease is detected. We present a case report of a one and a half years old girl who came with features of incomplete Kawasaki disease, high grade fever, irritability, history of conjunctivitis and cracking of lips. She was investigated and had a platelet count of 902 x 10(9)/L, ESR was 71 mm/hr and CRP was also raised to 12.8 mg/l. Cardiac evaluation and echocardiography was done which showed dilated coronary arteries >3mm on the left side and 4mm on the right side with early aneurysmal changes. She was treated with immunoglobulin and aspirin and improved.

Familial chylomicronemia in a nine months old infant.

Familial chylomicronemia syndrome is a rare disorder of lipoprotein metabolism due to familial lipoprotein lipase or apolipoprotein C-II deficiency or the presence of inhibitors to lipoprotein lipase. It manifests as eruptive xanthomas, acute pancreatitis, and lipaemic plasma due to marked elevation of triglyceride and chylomicrons levels. We report a rare case of familial chylomicronemia in a 9-month-old infant, who was diagnosed after his plasma was incidentally found to be milky. Lipid profile showed familial chylomicronemia (Type 1 Hyperlipidemia). The infant was started on a low fat diet and advised a regular follow-up.

A family with acute intermittent porphyria.

Porphyrias are inherited defects in heme metabolism that result in excessive secretion of porphyrins and porphyrin precursors. Porphyrias can be classified into acute, (neuropsychiatric), cutaneous and mixed forms. There are seven main types of porphyrias; acute intermittent porphyria and plumboporphyria are predominantly neuropsychiatric; congenital erythropoietic porphyria, porphyria cutanea tarda and erythropoietic protoporphyria have predominantly cutaneous manifestations and hereditary coproporphyria and variegate porphyria are classified as mixed as they both have neuropsychiatric and cutaneous features. They cause life-threatening attacks of neurovisceral symptoms that mimic many other acute medical and psychiatric conditions. Lack of clinical recognition often delays effective treatment, and inappropriate diagnostic tests may lead to misdiagnosis and inappropriate treatment. Although the specific enzyme and gene defect have been identified, diagnosis and treatment of these disorders present formidable challenges because their signs and symptoms mimic other common conditions. We present a case report of a 13 years old girl who suffers from acute intermittent porphyria and the family tree showing all members who suffer from it.