Auditory Neuropathy/Dyssynchrony in Biotinidase Deficiency

Biotinidase deficiency is a disorder inherited autosomal recessively showing evidence of hearing loss and optic atrophy in addition to seizures, hypotonia, and ataxia. In the present study, a 2-year-old boy with Biotinidase deficiency is presented in which clinical symptoms have been reported with auditory neuropathy/auditory dyssynchrony (AN/AD). In this case, transient-evoked otoacoustic emissions showed bilaterally normal responses representing normal function of outer hair cells. In contrast, acoustic reflex test showed absent reflexes bilaterally, and visual reinforcement audiometry and auditory brainstem responses indicated severe to profound hearing loss in both ears. These results suggest AN/AD in patients with Biotinidase deficiency.

Lactose-free compared with lactose-containing formula in dietary management of acute childhood diarrhea

Few reports are available on some benefits, such as shortened duration of diarrhea and better weight gain, for lactose-free over lactose-containing formula in acute childhood diarrhea. We evaluated the effects of lactose-free formula in dietary management of acute diarrhea in formula-fed children. This controlled-clinical trial was conducted on formula-fed children, aged 1 to 24 months, referring with acute non-bloody diarrhea [

relationship between zinc deficiency and febrile convulsion in Isfahan, Iran

Febrile convulsion [FC] is a common cause of seizure in young children, with an excellent prognosis. In addition to genetic predisposition and infections, FCs are generally thought to be induced by metabolic and elemental changes during fever such as Zinc [Zn] deficiency. Regarding the high prevalence of febrile convulsions and the role of Zn deficiency, we investigated the role of Zn in FC patients in Isfahan, Iran. In a controlled cross sectional study, 90 patients aged 9 months to 5 years were studied in a period of 12 months. They were assigned to three groups. Thirty patients were included in the Febrile Seizure group, thirty febrile children without convulsion or previous history of convulsion were included in the febrile group and thirty afebrile healthy ones were enrolled as controls. Venous blood was obtained and Zn concentration in serum was measured using Graphite Furnance Atomic Absorbance Spectrophotometering [GF-AAS]. Patients and the control groups had no difference in either mean age or sex distribution. No significant relationship was observed between serum Zn level and age or sex among patients in the FC group and two other control groups. Our findings showed that Zinc level was significantly lower in the febrile seizure group compared to two other groups. We tried to categorize various conditions in a more practical form. Also, Zinc is in close relationship with socioeconomic level of the individuals which was well considered in the current survey

Infection rate of cryptosporidium parvum among diarrheic children in Isfahan

Cryptosporidiosis is an important enteric parasitic infection among infants and children in developing countries with significant morbidity and mortality especially among immuno-suppressed individuals. The aim of the present study was to estimate the prevalence of enteric cryptosporidiosis in children presenting with diarrhea in Isfahan. This cross-sectional study was conducted from August 2007 to June 2008 in three university hospitals in Isfahan. Children aged 1 month to 10 years presenting with acute or persistent diarrhea were selected consecutively. The oocyst of C. parvum was investigated in stool specimens using a modified acid-fast staining method. During the study period, 606 children [mean age 42.4 +/- 30.0 months, 58.1% female] were recruited. Acute and persistent diarrhea was present in 422 [69.6%] and 184 [30.4%] of the children, respectively. Twenty eight [4.6%] specimens were oocyst positive. The prevalence of Cryptosporidium infection was significantly higher in children with persistent diarrhea compared to children with acute diarrhea [12.5% vs 1.2%; P<0.001]. Most of the infected children were under 5 years of age [89.2%], however, the age difference between infected and non-infected children was not statistically significant. Also, there was no significant difference between infected and non-infected children in gender. The prevalence of Cryptosporidiosis in children presenting with persistent diarrhea is considerable and we suggest routine stool examination for Cryptosporidium in this group of children

Comprehensive assessment of the relative frequency of sleep disorders in migraine and non migraine in 6- to 14-year-old children

Headaches are common during childhood. In addition, sleep disorders are common problems in children, too. Although it is commonly thought that headache and sleep problems are related, there is not enough evidence to confirm this relationship. Finding evident relations between these problems would help practitioners a lot to make earlier diagnosis and plan treatment modalities for both problems as soon as possible. This study aimed to assess the relative frequency of sleep disorders in migraine and non migraine children. In a cross sectional study, 148 children were enrolled in migraine [60] and non migraine [88] groups. They were aged 6 to 14 years. Migraine group consisted of patients who had definite migraine according to HIS [International Headache Society] criteria. Ten sleep problems [snoring, nightmares, sleep walking, sleep talking, bedtime struggle, bruxism, sweating during sleep, excessive daytime sleepiness, insomnia in early or mid night and sleep apnea] were compared between 2 groups. Fisher exact and chi square tests were used for making comparisons. We found relationships between night sleep and daytime headaches. Some of these relationships were easy to explain but for some others, finding complicated explanations are necessary. Our findings showed that bruxism, sleep walking, early and midnight insomnia was significantly higher in migraine children. There was a common etiology for headache and sleep disorders. Also, parents and migraine children were well familiar with the effect of relaxation on decreasing migraine episodes. They provided appropriate facilities for night sleep for this reason. We found relationships between night sleep and daytime headaches. Some of these relationships were easy to explain but for some others, finding complicated explanations are necessary