An epidemiological analysis of acute flaccid paralysis in Khuzestan Province, southwest Iran, from 2006 to 2010 / 한국역학회지

OBJECTIVES: Investigations into the epidemiology of acute flaccid paralysis (AFP) are an essential strategic component of the Global Poliomyelitis Eradication Initiative of the World Health Organization (WHO), and are part of the certification process for polio eradication worldwide. This is an epidemiological report of AFP incidence in children less than 15 years old in southwest Iran. METHODS: This was a retrospective cohort study, carried out based on WHO guidelines, in which we reviewed non-polio AFP cases recorded from January 2006 to December 2010 in different regions of Khuzestan Province, southwest Iran. In this study, the records of all children under 15 years old with AFP were evaluated. RESULTS: During a 5-year period, 137 cases of AFP were reported (incidence rate, 2.21 per 100,00 children <15 years old). More than 50% (73 of 137) of the cases were boys, and 52.6% (72 of 137) were under 5 years of age, with a mean age of 5.39±3.98 years. The incidence of AFP was significantly higher in older children (p=0.001). The most common cause of paralysis was Guillain-Barré syndrome (117 of 137). None of the cases were diagnosed with acute poliomyelitis. CONCLUSIONS: In this study, we found that the incidence rate of AFP in the region was almost in agreement with the expected incidence of AFP in children less than 15 years old; therefore, the AFP surveillance program in Khuzestan Province is satisfactory in terms of reliability and effectiveness. Nevertheless, routine vaccination against polio and ensuring that patients with AFP receive follow-up are essential for eradicating polio.

SMN1 and NAIP genes deletions in different types of spinal muscular atrophy in Khuzestan province, Iran

Spinal muscular atrophy [SMA] is the second most common lethal autosomal recessive disease. It is a neuromuscular disorder caused by degenerative of lower motor neurons and occasionally bulbar neurons leading to progressive limb paralysis and muscular atrophy. The SMN1 gene is recognized as a SMA causing gene while NAIP has been characterized as a modifying factor for the clinical severity and age at disease onset in SMA patients [SMA subtypes]. The relationship between NAIP deletion and type of SMA remains to be clarified; we investigated this gene alteration in all types of SMA patients. Molecular analysis was performed on fifty patients with a clinical diagnosis of SMA in Khuzestan province. In addition to common PCR-RFLP analysis for exon 7 and 8 of SMN1 gene, as an internal control we analysed NAIP deletion with PCR of exon 5 of this gene in a multiplex PCR with exon 13 of it. Homozygous-deletion frequency rate for the telomeric copy of SMN [SMN1] exon 7 in all types [type I, II, III] of SMA was approximately 90% and the frequency of deletion in exon 7 and 8 together in all types estimated about 70%. Moreover NAIP gene was deleted in about 60% of these patients and this shows deletion in 91% of type I SMA patients. The correlation between NAIP-deletion and SMN1 mutation showed a high frequency rate. In this study, high frequency of NAIP gene deletion in all type of disease shows the importance role of it in disease pathogenesis. High frequency of NAIP deletion in SMA type I, also shows the importance of the gene in type and severity of disease so it may be a modifier factor in severity of disease

frequency of urinary tract infection among children with febrile convulsion

This study was conducted to determine the frequency of urinary tract infection [UTI] among children with febrile convulsion [FC]. We analyzed the hospital records of 137 children who had been admitted to the pediatric ward from March 2004 to February 2007 because of FC. Information such as age, sex, developmental status, type of FC, family history of seizure, urine sampling method, and the results of antibiograms were recorded. The age distribution of 137 patients [82 boys, 55 girls] was as follows: 1-6 months of age, 1 infant [0.7%]; 6-12 months, 21 infants [15.3%]; 1-3 years, 75 [54.8%]; 3-5 years, 30 [21.9%]; and more than 5 years, 10 [7.3%]. Three out of the 82boys and 6 out of the 55 girls had UTI [3.7% vs. 10.9%, total, 6.6%]. The age distribution of these 9 patients was as follows: 1-6 months, 1 patient [11.1%]; 7-12 months, 5 [55.6%]; and 1-3 years, 3 [33.3%]. The relative incidence of UTI was 6.6%. The most common organisms causing infections were Escherichia coli in 8 and Proteus spp., in 1 patient [88.8% vs. 11.1%]. Simple FC was seen in all 9 patients with UTI. In this study, the relative frequency of UTI among children with FC was 6.6% and this frequency was higher that the incidence of UTI in girls and boys [3-5% and 1%, respectively]. Therefore, we recommend that UTI should be considered as an important cause of FC in children

case report of a survival from Ekiri syndrome

Lethal toxic encephalopathy of shigellosis [Ekiri syndrome] is a rare complication of the shigella infection presented with fever, severe toxicity, seizure and diffuse brain edema, coma and death in the absence of dehydration and is nearly always lethal. This report is about a child who has survived the Ekiri syndrome. A three-year old child was admitted to the emergency ward because of fever, one attack of generalized tonic-clonic seizure, drowsiness and diarrhea with no signs of dehydration. The differential diagnosis was meningitis, shigellosis and atypical febrile convulsion. All test results for blood, urine, stool and cerebro-spinal fluid samples were normal except for serum sodium [119 mmol/1] and stool exam [many RBCs]. He was treated with anticonvulsants because of the recurrent seizures and ceftriaxone with probability of shigellosis. The emergency brain CT scan showed diffuse brain edema. After 48 hours the consciousness was improved. Stool culture showed the growth of Shigella flexneri. Second brain CT scan revealed a dramatic decrease of the brain edema. Because neurological symptoms are among the extra intestinal complications of the shigellosis, it is recommended to put this disease in the list of differential diagnosis of unexplained neurological signs in endemic areas and consider the specific medications in the treatment programs

Primary amebic meningoencephalitis

This is the first case description of primary amebic meningoencephalitis [PAM] in an Iranian child. The patient was an eighteen months old boy referred to the hospital with signs and symptoms of acute meningitis. Cerebrospinal fluid [CSF] examination indicated pleocytosis with predominance of neutrophils, low sugar and high protein. Gram staining and culture of CSF for bacteria were negative. Microscopic examination of CSF revealed the motile amebae with pseudopodia. In Giemsa staining of CSF, the trophozoites of amebae were observed. The initial response to a six-week treatment course with amphotericin B, rifampin and chloramphenicol was satisfactory, but in the follow-ups the patient regressed neurologically. Although PAM is a rare and fatal disease, it should be differentiated from bacterial meningitis and treatment must be promptly carried out