Guillain- barre syndrome in children; clinicolaboratory features, electrodiagnosis and prongnosis

Guillain - barre syndrome [GBS] is an acute post- infectious immune mediated peripheral neuropathy with a highly variable clinical course and outcome[1]. There are several factors claimed to affect the outcome as age, electrophysiologic type, levels of proinflammatory cytokines, and previous diarrhea[2]. This study was performed to evaluate the effect of these previous factors on the outcome of GBS. Fifty patients with GBS were prospectively studied and the following factors were evaluated; age of onset of the disease, days from onset of the disease to nadir level, days required to improve GBS Score from nadir level by one, severity of the disease at nadir, prior diarrhea, bulbar affection, autonomic dysfunction, parasthesia, respiratory failure, need for mechanical ventilation, electrophysiologic classification, Tumour necrosis factor [TNF] alpha in serum on admission and one week after IVIG therapy. Outcome was evaluated using the functional grading scale [FGS] of Hughes[3]. Prognosis of GBS in children is generally good with complete recovery of all patients after one year from the onset of the disease without residual neurologic deficit; however severity of the disease at nadir was increased by older age of onset, axonal type on electrophysiology, prior diarrhea, and higher levels of TNF alpha in serum. Guillan barre syndrome in children carry good prognosis as regard the long term outcome, however there is some risk of mortality and morbidity during the short term outcome. Both long and short term outcomes are improved by younger age of onset, demyelinating nature, and use of IVIG therapy

Serum adiponectin in newborns and in one and two years old infants: relationship with body weight, leptin and insulin

Adiponectin is the only adipose-specific hormone that, despite its exclusive production by adipose tissue, is reduced in obesity and is inversely correlated with leptin levels in adults. It plays a critical role in the control of energy balance in adult life. However, its functions and relations to other hormones are not yet fully understood in infants. The aim of this study is to assess adiponectin levels in newborns at birth, one and two years of life and to define its association with weight, serum leptin and insulin. Serum adiponectin, leptin and insulin levels were investigated in 48 newborns [24 small for gestational age [SGA] and 24 appropriate for gestational age [A GA]]. Infants aged one year [n=20] and two years [n=20] were also included in this study. The levels of serum adiponectin were significantly decreasing while serum leptin and insulin were significantly increasing at one and two years. Serum adiponectin was correlated positively with weight [r=0.509, p<0.001] and leptin [r=0.355, p<0.05] but not correlated with serum insulin in all newborns. At one and two years adiponectin was correlated positively with insulin [r=0.444, p<0.05 and r0.448, p<0.05 respectively]. The correlation between adiponectin and weight at one year [r=-0.056, p=0.816] and between it and leptin at two years [r=-0.171, p=0.471] changed into negative but did not reach a statistically significant level. Serum leptin was correlated positively with weight in all newborns [r=0.935, p<0.001]. At two years it was positively correlated with weight [r=0. 721, p<0.001] and insulin [r=0.641, p<0.01]. Serum adiponectin and leptin levels were significantly lower in SGA than AGA infants [30.08 +/- 9.54 micro/ml and 4.33 +/- 3.20 microg/L, p<0.001 respectively] but there was no difference in serum insulin. In conclusion, adiponectin is higher in newborns than at one and two years. The change in correlation of adiponectin with weight and leptin from positive in newborns to negative in adults might occur at the first 2 years of life

Benign covuIsions with acute gastroenteritis in infants and young children

Seizures in diarrheic children may be due to several etiologies. However seizures may occur in the absence of an obvious cause which is called benign convulsions with gastroenteritis [BCWG]. The aim of this study is to describe the frequency, clinical characters and outcome of benign seizures with gastroenteritis in children attending Assiut University Children Hospital and its possible relationship to some pathogenic organisms. 62 patients with acute gastroenteritis and convulsions were recruited for the study and 35 children with acute gastroenteritis without convulsions were included as a control group They were 34 male and 28 female. The clinical data and electroencephalogram were recorded. Stool specimens from patients and controls were tested for Shigella spp, E. coil 0157. Stool radioimmunoassay for rotavirus was done. Follow up was done after recovery from seizure for one year in pediatric neurology outpatient clinic. The number of patients presented with seizures was 62 patients [34 boys and 28 girls]. Causes of convulsions could be detected in 29[46.8%] of the cases. in 33 cases [53.2%] of all studied patients no obvious causes for convulsions could be detected so they were considered as having BCWG. They were 11 girls and 22 boys. Rotavirus particles were present in 15 [45.5%] of BCWG cases and these cases had younger age, associated with more frequent motions, watery diarrhea, vomiting, thirst, more frequent seizures, earlier onset of seizures to onset of diarrhea and more frequent focal seizures. None of these patients showed recurrence of afebrile seizures later on and their developmental milestones remained normal during the one year follow up period. Benign convulsions with gastroenteritis are frequently associated with rotavirus infection, usually carry excellent prognosis and no need for prolonged antiepileptic therapy

Cardiovascular changes in patients with type I diabetes with and without autonomic dysfunction

Diabetic complications - either microvascular or macrovascular - represent a major cause of morbidity and mortality in diabetic patients[1]. Cardiovascular autonomic neuropathy is one of the complications of type I diabetes mellitus. It may lead to life discomfort or even it may be the direct cause of death in diabetic patients. Early detection of cardiovascular autonomic neuropathy is of major clinical interest that could lead to a more intensive supervision of diabetic patients.[2] Cardiovascular autonomic neuropathy induces different functional cardiac changes, especially a reduction in left ventricular contractility and changes in ventricular repolarization.[3] This study was designed to estimate the prevalence of cardiovascular autonomic neuropathy among a group of children with type I diabetes mellitus, to assess the cardiac function in the same group of patients by echocardiography and to relate the abnormalities to the duration of diabetes, glycemic control and other risk factors

Transforming growth factor beta and other angiogenic factors in hematologic malignancies of childhood

Angiogenesis, a process of new blood vessels formation from an existing vasculature, has been blamed for growth, dissimination and metastasis of solid tumors. Little is known about angiogenesis and angiogenesis- related molecules in hematologic malignancies. Transforming growth factor-beta [TGF- beta] and platelet-derived growth factor-C [PDGF-C] are considered as indirect angiogenic factors in tumorigenesis. Gangliosides and hyaluronan are components of cell membrane that modulate cell membrane signal transduction of multiple growth factors. The aim of this study is to assess serum levels of TGF- beta, PDGF-C, Gangliosides, and hyaluronan in children with newly diagnosed malignancies including 15 cases with acute lymphoblastic leukemia [ALL], 15 cases with non Hodgkin lymphoma [NHL], and 10 cases with neuroblastoma. Ten matchable apparently healthy children were included as controls. Results of the study showed that cases as a whole had significantly higher levels of TGF-beta, PDGF-C, gangliosides and hyaluronan than controls. Furthermore cases with hematologic malignancies as well as those with neuroblastoma had significantly higher levels of the studied parameters than controls. On comparing the different groups of cases with each other, it was found that cases with ALL and NHL had significantly higher level of TGF-beta than cases with neuroblastoma whereas the latter group had significantly higher level of gangliosides than ALL and NHL groups and higher level of hyaluronan than ALL group. Cases of NHL and neuroblastoma with advanced disease had significantly higher levels of the studied parameters than the rest of cases. In conclusion the angiogeneic factors TGF-beta, PDGF-C, gangliosides, and hyaluronan are raised in children with ALL and NHL as well as in those with neuroblastoma. Their levels are significantly higher in severer cases with advanced stages of malignancy. The estimation of their levels therefore not only points to the severity but also help to predict progress in these cases

Febrile convulsions: risk factors and therapeutic options

Febrile convulsions [FCs] are the most common cause of seizure disorder in childhood, occurring in 2-5% of children.[1] The aim of the present study is to evaluate if there is any clinical or electroencephalographic findings that exist as predictives of the risk of recurrence of febrile convulsions [FC]. The study included 118 children [73 boys and 45 girls] with febrile convulsions who were evaluated clinically and by EEG. The patients received various types of prophylaxis to prevent recurrence of FC and patients were followed up for 2 years. It was observed that both intermittent oral diazepam and continous sodium valproate prophylaxis equally induced significant reduction in the recurrence of FC in patients receiving prophylaxis than patients without prophylaxis. There was significantly higher recurrence risk of FC associated with younger age of onset, positive family history of FC, complex features of FC [as focal, prolonged or recurrence in clusters] early onset of FC to onset of fever, occurrence of FC at lower grades of fever and higher frequency of febrile illnesses. EEG findings have no relation to recurrence of FC

Effect of oral stabilized oxygen on patients with fallot's tetralogy

Fallot's tetralogy is a common congenital cyanotic heart disease affecting 0.2-0.3 per 1000 live births.[1] This study was done to evaluate the effect of oral stabilized oxygen therapy on patients with Fallots tetralogy. The present work included 15 children [10 boys and 5 girls] with an age range from one month to 8 years diagnosed as Fallot's tetralogy. The patients received oral stabilized oxygen for one month, Blood gases were done before, one, two and four weeks while the patient on oral oxygen therapy and two weeks after stoppage of oral oxygen. Echocardiography, complete blood count and hematocrit values were done before starting and after one month while the patient was on oral oxygen therapy. It was found that there was marked rise In SaO[2], and PaO[2] while the patients were on oral oxygen therapy than before initiation and after stoppage of oral oxygen therapy but without any echocardiographic changes, indicating that these changes in blood oxygenation are merely due to gastrointestinal absorption of oxygen. There was highly significant reduction in the level of hemoglobin, red cell count, and Hematocrit after than before oral oxygen therapy