Management Of Childhood Steroid Dependent Nephrotic Syndrome With Cyclophosphamide - An Experience At Ayub Teaching Hospital, Abbottabad.

BACKGROUND: This study has been done in children with Steroid dependent nephrotic syndrome (SDNS) to check for the response to cyclophosphamide and relapse on follow up for one year after completion of treatment. METHODS: This study was conducted over two years and nine months. Patients were taken as steroid dependent when there were two consecutive relapses occur on steroids tapering or within two weeks of stopping treatment. Children of either sex between ages of 1-14 years, diagnosed case of SDNS were included in this study. Renal biopsy was not done in any patient. After achieving remission with oral steroids, cyclophosphamide was given after calculation of maximum cumulative dose 168 mg/kg for 8 - 12 weeks along with oral steroids. Follow up done every two weeks till completion of treatment for response and adverse effects and thereafter for one year. RESULTS: There were 31 patients, 23 (74.2%) male and 8 (25.8%) females. Age ranged from 1.5 years to 11 years with mean age 5.44±2.39 years. There was full response to cyclophosphamide as none of patient had proteinuria on cyclophosphamide therapy. After completion of cyclophosphamide course, four patients (12.9%) relapsed on follow up while 87.9% remain in complete remission. Only one female patient (3.23%) had adverse effect in form of hair fall and she recovered after completion of treatment. None of patient showed any other adverse effect including haematuria. CONCLUSIONS: Cyclophosphamide is an effective therapy in management of childhood SDNS with minimum adverse effects in medium term.

An Outcome Of Conservative Management Of Primary Nocturnal Enuresis In Children.

BACKGROUND: Primary nocturnal enuresis is one of the common problems in children. Mostly parents are concern for this condition in children and also children are depressive from this condition. The main stay of treatment is the training of child. The objective of this study was to look for the outcome of conservative management of primary nocturnal enuresis. METHODS: This study was done in OPD of paediatrics department. Patients aged five year or more were included in the study. Patient age, weight, sex, blood pressure, family history in siblings and parents, number of wet days/week, recorded on specific proforma along with renal function tests. Patients were advised fluid restriction after evening and micturition before sleep and after 2-3 hours of sleep. Follow up was done after six months to observe for the impact of habit change. Data has been analysed by SPSS 20 and results are taken significant with p-value <0.05. RESULTS: Out of 81 patients, 41 were male and 40 females. Age ranged from 5 to 14 years and mean age was 8.2±2.35 years. There were 11.1% parents who had primary nocturnal enuresis during childhood and in 29.6% siblings, history was positive. Follow up at 6 months, 58% patients improved while 42% showed no improvement. There was significant relationship between evening fluid restriction, micturition before and after sleep with improvement at 6 months with p-value of 0.010, <0.001 and 0.002 respectively. CONCLUSIONS: Conservative management is the effective intervention in children as parents should be emphasized for habit change.

Outcome Of Balloon Valvuloplasty In Children With Pulmonary Stenosis - Single Center Experience.

BACKGROUND: Pulmonary valve stenosis (PS) is common congenital heart disease in children and patient can present with cyanosis, chest pain, dyspnea and failure with severe form. The objective of this study was to enlighten the short outcome of balloon pulmonary valvuloplasty in children with severe pulmonary stenosis. METHODS: This cross sectional observational study was done in paediatric cardiology department of Lady Reading Hosptial, Peshawer form June 2019 to December 2020 over 1.5 years. Children aged 6 months to 16 years of either sex diagnosed as case of severe stenosis with doming pulmonary valve and having symptoms of chest pain and dysnea on exeration and pregradient pressure of 64 mm Hg or more on echocardiography were included. Patients were interviened with ballon-valvuloplasty. Outcome was taken as discharge from hospital with complications after procedure if any. Patients were followed up for 3 months. Data including age, sex, pre cath echocardiography, cath pulmonary valve annulus, post ballooning pulmonary valve (PV) gradient, PV gradient on echocardiography after intervention and follow up gradient on echocardiography at three months was documented. Data was analyzed by SPSS 20. Chi square test and paired T - Test was applied where required. Results were taken as significant with p value <0.05. RESULTS: There were 51 patients, 35 (68.6%) male and 16 (31.4%) female. Mean age was 8.35±4.93 years. Mean pre cath gradient across the pulmonary valve on echocardiography was 109.14±31.44 mm Hg. Post intervention mean pressure gradient across PV was 32.41±11.49 mm Hg. Pulmonary valve annulus on echocardiography before intervention ranged from 7 to 25 mm with mean of 14.67±3.79 mm. There was no complication in majority (82.4%) of patients. Mild PR was in 5 (9.8%) patients. There was significant relationship between pre and post intervention pressure gradient across PV valve with p value of <0.001. CONCLUSIONS: Balloon pulmonary valvuloplasty in one of the safest intervention for PS in children with few complications.

Mucoepidermoid Carcinoma - Rare Presentation Of Childhood Chronic Respiratory Symptoms In 10 Years Old Girl.

Mucoepidermoid carcinoma is the rarest presentation in children arising from bronchial glands with low potential for malignancy as most of children have history of recurrent chest infections. We present the case of ten years old girl presenting with history of recurrent respiratory infections in past admitted for fever, cough and respiratory distress. HRCT showed soft tissue in lumen of trachea at level of carina extending into right main bronchus along with collapse of apical segment of right upper lobe and right middle and lower lobes collapse consolidation. Surgery was done and histopathology showed low grade mucoepidermoid carcinoma with clear histopathology of regional lymph nodes. Follow up of patient done for two years. Informed consent was taken from patient prior to writing of this case report.

Childhood Idiopathic Steroid Resistant Nephrotic Syndrome, Different Drugs And Outcome.

BACKGROUND: The management of steroid resistant nephrotic syndrome (SRNS) is quite difficult in paediatric patients. Not only the remission is difficult but also these patients are at risk of progression to end stage renal disease (ESRD). The goal of treatment is either to achieve complete remission or even partial remission as it is the most important predictor of disease outcome. METHODS: This study was conducted at The Children's Hospital, Lahore from February 2014 to May 2015. The SRNS patients of either sex between ages of 1-12 years were included with histology showing mesangioproliferative glomerulonephritis (MesangioPGN), focal segmental glomerulosclerosis (FSGS) or minimal change disease (MCD). Patients were given different immunosuppressant drugs and steroid 30 mg/m2 alternate day therapy on case to case basis and kept on regular follow up to check for response and adverse effects. RESULTS: Total of 105 patients included, 63 (60%) male and 42 (40%) female patients. The age ranges from 1.08 to 12 years, mean age of 6.53 years and SD of ±3.17. Tacrolimus was the most common drug used 43 (41%) patients followed by cyclosporine in 38 (36.2%) patients, while Mycophenolate mofetil (MMF) was prescribed in 21 (20%) patients. Complete response was in 96 (91.4%) initially while partial response was seen in 8 (7.6%) patients. On follow up, 92 (87.6%) patients showed complete response and partial response was in 5 (4.7%) patients. Cushingoid features and hypertrichosis were the most common adverse effect seen. CONCLUSIONS: Steroid resistant nephrotic syndrome can be managed well with various immunosuppressant drugs and steroids but treatment should be individualized according to clinical presentation, disease histology and cost/social factors.