Infantile spasms: clinical profile and treatment outcomes

Background and Objective: Infantile spasm [IS] is one of the severe epileptic encephalopathies which affect children in early two years of life. Our objective was to determine the clinical profile, etiology and outcome of treatment in children with infantile spasms attending tertiary care hospital at Karachi, Pakistan

Methods: This is retrospective study of 36 patients out of 94 registered as IS, aged three months to two years, managed and followed up at Aga Khan University Hospital, Karachi, from 2010 to 2015. Data of all children with IS was collected from case record. Details including clinical observations, lab investigations, anti-epileptic medications and treatment outcome was collected and analyzed. Patients who received treatment for six weeks to document response were included. The treatment response was categorized as complete response, partial response [>50% improvement] and no response. Data was analyzed on SPSS using descriptive statistics

Results: Thirty-six patients [38.29%] with IS fulfilled eligibility criteria. The mean +/- SD age at presentation was 4.6 +/- 2.1 months. Male to female ratio was 2:1. Consanguinity and developmental motor delay was observed in 66.6% and 89% respectively. Symptomatic etiology was predominant [61%] and hypoxic ischemic insult [32%] was the commonest underlying cause. EEG and MRI were diagnostic tools whereas metabolic studies were not helpful. Multiple antiepileptic drugs were used for seizure control and vigabatrin was the most frequently used [88%] drug. Short term treatment response was not different in idiopathic or symptomatic infantile spasms

Conclusion: Majority of patients had symptomatic infantile spasms and generalized tonic clonic along with myoclonic jerks were predominant seizure types. EEG and MRI were diagnostic in most of cases. Multiple AEDs were required to control seizures and VGB was most common drug [88%] used. Treatment outcome was not different in idiopathic and symptomatic groups

Comparison of three formulae for estimation of glomerular filtration rate in severely malnourished children at tertiary care facility

Objectives: First objective was to compare eGFR by Updated Schwartz [US] and Simple Height Independent [SHID] formula with Original Schwartz [OS] in children with Severe Acute Malnutrition [SAM]. The second objective was to compare eGFR in children below and above two years

Methods: This analytic study on estimation of GFR was based on retrospective data collected from 78 children with SAM at Nutritional Rehabilitation Unit from October 2014 - March 2015. Glomerular filtration rate was calculated using serum creatinine [S. Cr] and height in Original Schwartz, US and by age in SHID equation and compared with OS as standard. Data was analyzed using descriptive statistics

Results: There were 78 children in this study. Males were 39[50%]. Mean age of patients was 18+/-15.53 months with 62[79.48%] /=90ml calculated by US compared to OS [0.025] and by SHID with OS [0.04] in children below two years and no difference in children above two years. But there was no difference in other categories of eGFR calculated by either of formula in both age groups

Conclusion: We found a significant difference in eGFR in ranges above 90 ml/min/1.73 m[2] by US compared to OS as well as by SHID with OS in children below two years and no difference in children above two years. Also, there was no difference in GFR categories below 90 ml/min /1.73 m[2] calculated by either of formula in both age groups. So, we may conclude that either of formula can be used in clinical practice for eGFR in mild to severe renal dysfunction in severely malnourished children

Cyclosporine versus cyclophosphamide in childhood nephrotic syndrome

Objective: To determine the response of Cyclosporine versus Cyclophosphamide in childhood nephrotic syndrome

Place and duration: Nephrology Unit, National Institute of Child Health, Karachi, from April - September 2012

Study design: Prospective Comparative study

Methods: 158 patients aged 6 months to 15 years with either steroid resistant [SR] or steroid dependent nephrotic syndrome [SDNS] were randomly assigned to receive either cyclosporine [CS -arm] or cyclophosphamide [CP-arm ] for 3 months along with alternate day prednisolone. Treatment response and side effects were monitored clinically and by laboratory tests [spot urine protein-creatinine ratio in both arms, serum creatinine in CS-arm and complete blood counts in CP-arm]. Outcome was defined after 12 weeks as complete remission [CR], partial remission and resistance. Data including demographics, type of NS, treatment response and adverse effects were collected and analyzed on SPSS-16

Results: There were 79 patients in each arm. Mean age in both arms was almost identical [6.8+/-3.9 and 6.9+/-3.7 years]. Among 158, 87[55%] were SD and 71[45%] were SRNS. Majority [78.5%] in CS-arm achieved CR compared to 34.2% in CP- arm. This is highly significant [p value <0.001]. Partial remission was observed in 19% of CS -arm compared to 48% in CP-arm and 2.5% were resistant in CS - arm compared to 17.7% in CP -arm. Hypertrichosis [5%], hypertension [3.7 %], gum hyperplasia[3.7%], nephrotoxicity[2.5%] were observed in CS -arm, whereas bone marrow suppression [7.5%] , alopecia and infections [2.5% each] were noted in CP- arm

Conclusions: Cyclosporine was more effective in inducing remission [78.5%] as compared to cyclophosphamide [34.2%] in childhood NS