Childhood acute disseminated encephalomyelitis: an Egyptian pilot study.

Describing the variable clinical features, laboratory findings, neuroimaging findings, and treatments given to children who presented with ADEM and following them up both clinically and radiologically. 21 patients were recruited: 14 new cases, and 7 old ones presenting over the preceding 5 years (retrospective review of existing data). 11 males and 10 females, with a mean age of 4.4 years ± 2.7 SD, were included. All new patients were subject to full history, examination and a panel of investigations including MRI of the brain. Treatment was given in the form of pulsed methyl prednisolone or intravenous immunoglobulin (IVIG), followed by clinical and radiological follow-up every 3 months as needed. 11 cases occurred in spring, 8 post vaccine, of which 5 were after oral polio vaccine (OPV). MRI was done for all 21 patients and was abnormal in all of them, CT was done in only 10 patients as was normal in 9. Hyponatremia was seen in 11 patients. All patients who received corticosteroids showed prompt improvement. 6 out of 10 patients who received IVIG first failed treatment. Of the 17 treated patients, 10 had no sequelae and 10 had total lesion resolution on MRI at 3 months, versus 1 and 0 patients, respectively, in the untreated group. We found a disproportionately large number of post vaccination cases, especially after OPV. The association of ADEM with hyponatremia needs further study. MRI is central to diagnosis. Outcome is much better with treatment with steroids being far superior to IVIG. Excess use of IVIG should be discouraged.

Assessment of oral health status of children with epilepsy: A retrospective cohort study.

BACKGROUND: Epileptic children are liable to experience oral health problems either due to the disease itself or medications administered to control the condition. AIM: We aimed to investigate caries experience, gingival health and oro-facial traumatic injuries in a sample of epileptic Egyptian children. DESIGN: A retrospective cohort study was conducted from September 2016 to April 2017 using data from medical records in Children Hospital at Ain Shams University. Dental examination was performed for 100 epileptic children and 80 healthy subjects who matched in age, gender, and socio-economic status. Caries experience was measured using the decayed, missing and filled teeth (DMFT or dmft) index and gingival index (GI) of Loe and Sillness was used to determine gingival health status. Oro-facial injuries were assessed using the WHO classification of trauma. Quantitative data were presented as mean, SD, and 95% CI values. Qualitative data were presented as frequencies and percentages and the significance level was set at P ≤ 0.05. RESULTS: The mean ± SD GI for epileptic children was significantly higher (1.16 ± 0.42) than that of healthy children. (1.01 ± 0.11) Healthy subjects had significantly lower mean dmf index scores 2.2 ± 2.6 compared to epileptic subjects where mean dmf scores were 4.1 ± 2.1. No significant difference, however, was detected between the two groups regarding caries experience in permanent dentition or incidence of oro-facial injuries. CONCLUSIONS: Epileptic children are highly burdened with gingival problems and liable to develop dental caries especially in primary dentition. Oral traumatic injuries, however, are not common complications if seizure attacks are well controlled.

Negative Impact of Prolonged Antibiotics or Persistent Diarrhea on Vitamin K1 Levels in 2-24 Weeks aged Egyptian Infants.

BACKGROUND: To evaluate the hazard of prolonged antibiotic therapy and/or persistent diarrhea on vitamin K1 (VK1) level and bleeding profile in infants (2-24 weeks). METHODS: A one-year case-control study, conducted at Ain Shams University, Egypt. 338 infants (2-24 weeks) were recruited and divided into 3 groups (1:1:3 ratios); group A (n=67) patients who received antibiotics for ≥10 days, group B (n=67) who had persistent diarrhea ≥ 14 days and group C (n=204) age- and gender- matched infants who had not either received antibiotics nor had diarrhea. All subjected to clinical assessment, bleeding history and had their complete blood count (CBC), PT and PTT, liver transaminases and VK1 level assayed. RESULTS: There was a significant increase in frequency of VKDB (vitamin K deficiency bleeding) and abnormal bleeding profile in cases than control group. There was significant negative correlation between VK1 level and duration of diarrhea, length of antibiotics used and bleeding profile. Antibiotic usage has hazardous effect on VK1 level in those with diarrhea; more patients were receiving antibiotic in those with persistent diarrhea and VKDB (N=55) than those with persistent diarrhea and normal VK1 (N=12). The longer duration of antibiotic therapy the lower level of VK1. Combining cephalosporin/penicillin therapy and/or diarrhea, in particular, had an impact on VK1 level. CONCLUSION: VKDB, a preventable cause of life-threatening hemorrhage, is still a major health problem in Egyptian infants, where persistent diarrhea and misuse of antibiotics are prevalent, necessitate a booster dose of VK in those high risk infants.

Role of plasma amino acids and urinary organic acids in diagnosis of mitochondrial diseases in children.

BACKGROUND: Diagnostic difficulty in mitochondrial diseases (MD) results not only from the wide spectrum of symptoms and signs but also from the absence of a reliable screening or diagnostic biomarker. AIM: To investigate the likelihood of MD in patients with symptoms and signs impressive of MD through quantitative measurement of plasma amino acids, and urinary organic acids. METHODS: Twenty patients with symptoms and signs suggestive of MD were further evaluated by quantitative plasma amino acids and urinary organic acids assay and neuroimaging. RESULTS: Plasma amino acid results revealed elevation of alanine in 11, glycine in five, and proline in two patients. Abnormal urinary organic acid analysis was present in six patients; increased urinary lactate (20%), dicarboxylicaciduria (15%), and urinary ketone bodies (10%). Upon enrollment our patients scored as possible MD according to the MD scoring system. At the end of the study, five patients still scored as possible MD, eight patients as probable MD, and seven patients as definite MD. All patients with definite MD had elevated serum lactate. In three patients, elevated urinary lactate was the only abnormality. Alanine was elevated in all patients with definite MD, whereas proline was elevated in only one. Magnetic resonance imaging of the brain showed atrophic changes in one patient and bilateral basal ganglia hyperintensity in another. CONCLUSION: Urinary organic acids and quantitative plasma amino acids can help in the diagnosis of MD, especially when the economic burden and absence of specialized centers limits the diagnosis.