Diabetic ketoacidosis presenting with pneumomediastinum.
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Child↗
Biomedical subjects
Muzaffer Polat
Publications and source records attributed to Muzaffer Polat.
Severe juvenile dermatomyositis: two patients complicated with extra musculocutaneous involvement.
Here we report two patients with severe juvenile dermatomyositis (JDM) complicated with extra musculocutaneous involvement. The first case (a 10-year-old boy) had unusual initial presentation of JDM complicated with interstitial lung disease documented with high-resolution computed tomography. He had a rapidly progressive course and died in 7 weeks after the onset of the disease despite steroid and immunosuppressive treatment. The second case (a 14-year-old boy) was presented with myositis complicated with hepatitis. He also had a chronic course of JDM with unfavorable outcome. It appears that the prognosis of patients with severe JDM is related with the degree of autoimmune vasculitis on extra musculocutaneous involvement.
Adolescent↗
The effect of seasonal changes on blood pressure and urine specific gravity in children living in Mediterranean climate.
BACKGROUND: We aimed to evaluate the effects of seasonal changes on urinary specific gravity, blood pressure and urinary erythrocyte number in children living in Mediterranean climate. MATERIAL/METHODS: The study was conducted on 547 children who presented for routine follow up to healthy-child care department between January 1997 and December 2002. Age, sex, weight, height, blood pressure, urinary specific gravity and urinary erythrocyte number were recorded by retrospective evaluation of files. Then, the parameters during summer were compared with those during winter. Additionally, correlation between the blood pressure, urinary specific gravity and urinary erythrocyte number was assessed separately during summer and winter. RESULTS: Anthropometrical measurements and mean age of the patients in summer and winter groups were similar. There was no significant change in urinary specific gravity (p > 0,05), while systolic and diastolic blood pressures were significantly higher in winter (p = 0.031 and p = 0.028 respectively). Temperature and humidity levels did not change significantly among different years but mean air temperatures during summer positively correlated with time from 1997 till 2002 (r = 0.965, p = 0.002). Blood pressure and urinary specific gravity were not correlated to each other at any time. Contrarily, there was a positive correlation between urinary specific gravity and erythrocyte number in summer (p = 0.01). The number of children with hematuria and degree of hematuria did not differ significantly between summer and winter. CONCLUSIONS: Seasonal changes in Mediterranean climate do not lead to changes in hydration status or in urinary erythrocyte number in children. Therefore, the decrease in blood pressure during summer can not be attributed to the hydration status.
Adolescent↗
Electrodiagnostic pattern approach for childhood polyneuropathies.
Electrophysiologic studies play a key role in the detection and characterization of the pattern in childhood polyneuropathies. In this study, the etiologic profile of 74 children with polyneuropathy was prospectively evaluated based on the electrophysiologic studies. Five electrodiagnostic patterns were identified in the cohort: (1) acute axonal polyneuropathy (n: 32, 43%); (2) chronic axonal polyneuropathy (n: 16, 22%); (3) demyelinating motor and sensory polyneuropathy (n: 13, 17%); (4) pure sensory polyneuropathy (n: 11, 15%); (5) high-low syndrome (n: 2, 3%). Etiologic factors were identified in all of the patients with three electrodiagnostic patterns of polyneuropathy: acute axonal, pure sensory, and high-low syndrome. However, etiologic factors could not be determined in 5 (31%) children with chronic axonal polyneuropathy and in 3 (23%) children with demyelinating sensory and motor polyneuropathy. Among children with the acute axonal pattern, toxic causes were evident in 18 (56%), acute motor axonal neuropathy in 11 (35%), and acute motor sensory axonal polyneuropathy in 3 (9%). Nine (82%) patients with pure sensory polyneuropathy had diabetes mellitus. In conclusion, a thorough history and physical examination in conjunction with specific electrodiagnostic patterns might provide a cost-effective and rational differential diagnosis of childhood polyneuropathies.
Adolescent↗
Vigabatrin caused rapidly progressive deterioration in two cases with early myoclonic encephalopathy associated with nonketotic hyperglycinemia.
Vigabatrin, a structural analogue of gamma-aminobutyric acid (GABA), is used for the treatment of generalized and partial seizures in infants. The drug inhibits the GABA transaminase and elevates the GABA concentration in the brain. Here we present the vigabatrin experience in two patients with early myoclonic encephalopathy owing to nonketotic hyperglycinemia (glycine encephalopathy). Both patients had early infantile seizures characterized by fragmentary myoclonic jerks associated with burst-suppression pattern on electroencephalography. Nonketotic hyperglycinemia was diagnosed with elevated cerebrospinal fluid and plasma glycine levels. The seizures were initially thought to be infantile spasms, and vigabatrin (50 mg /kg/day) was started for the treatment of seizures. Rapidly progressive deterioration was noticed after a few days. Acute encephalopathy associated with sleepiness and respiratory failure developed. Vigabatrin produced acute encephalopathy, which regressed in a few days after vigabatrin was stopped in the first patient. However, in the second case, despite the discontinuation of vigabatrin, there was no recovery of general conditions. Our observations in two cases indicate the risk of using vigabatrin in patients with nonketotic hyperglycinemia. The elevated GABA concentration in the brain can enhance the encephalopathy, together with the elevated levels of glycine. (J Child Neurol 2006;21:82-84).
Anticonvulsants↗
Central core disease: atypical case with respiratory insufficiency in an intensive care unit.
Central core disease is a rare congenital myopathy characterized by formation of typical cores in myofibrils. We report an atypical case of central core disease with respiratory insufficiency in the late stage of congenital myopathy. A 13-year-old girl was admitted to the intensive care unit with the diagnosis of respiratory distress syndrome. Ventilatory support was initiated. After 2 weeks of follow-up, the Division of Pediatric Neurology was consulted owing to the failure to wean her from the ventilator. Clinical and electromyographic features were in favor of primary muscle disease. Muscle biopsy revealed typical cores in type 1 muscle fibers, which were diagnostic for central core disease. This case was presented to emphasize that patients with respiratory distress who cannot be weaned from the ventilator should be evaluated for central core disease with an atypical presentation.
Adolescent↗
Cerebrospinal fluid interleukin-6 levels in patients with West syndrome.
Elevated cytokine response has been reported in patients with epileptic seizures. The objective of this study was to investigate the possible role of interleukin-6 (IL-6) in the pathogenesis of infantile spasms in West syndrome (WS). We measured IL-6 levels in cerebrospinal fluid (CSF) obtained from the newly diagnosed patients with WS. Twelve patients with WS (Group I) were classified as symptomatic WS (Group IA) in eight and as cryptogenic WS (Group IB) in four. The results were compared with control groups including patients with tonic-clonic seizures associated with two different kind of inflammation of central nervous system; Group IIA (infection): bacterial meningitis/encephalitis and Group IIB (trauma): post-traumatic seizures. There was no statistically significant difference between the mean values of CSF IL-6 levels in patients with WS (2.95 +/- 2.31 pg/ml) and those of subgroups of WS (Group IA: 2.26 +/- 2.01 pg/ml and Group IB: 4.33 +/- 2.52 pg/ml). Both control groups had highly increased IL-6 levels in CSF (Group IIA: 193.05 +/- 185.52 pg/ml and Group IIB: 112.74 +/- 167.44 pg/ml) than those of the patients with WS. Elevated IL-6 response in patients with tonic-clonic seizures associated with inflammation of central nervous system might be due to the seizures themselves or related to the underling etiology (infection or trauma). However, no elevated IL-6 response was found in patients with infantile spasms.
Child, Preschool↗
Premedication during micturating cystourethrogram to achieve sedation and anxiolysis.
Micturating cystourethrogram (MCUG) is an imaging technique indicated in the diagnosis and follow-up of many diseases. We investigated the reliability and the efficacy of midazolam and chloral hydrate in sedation and anxiolysis during micturating cystourethrogram. Fifty-three children of similar ages (39 girls, 14 boys, mean age of 5.8+/-3.5 years) were randomized to midazolam (n=17), chloral hydrate (n=18) and control groups (n=18). Oral midazolam 0.6 mg/kg or chloral hydrate 25 mg/kg or saline were administered to the study groups 15-30 min prior to the urinary catheterization. Brietkopf and Buttner, Frankl and Houpt scales and Spielberger's State Anxiety Inventory and parent's impressions were used to assess the level of sedation and anxiety. The Brietkopf and Buttner classification of emotional status and Houpt behavior rating scale demonstrated a significantly better emotional status and sedation in the midazolam group when compared to controls (P=0.01 and P=0.018, respectively). The catheterization was described as a more unpleasant and distressing event by the parents of the control and the chloral hydrate groups when compared to the parents of the midazolam group (P<0.05). Bladder capacity and frequency of detection of residual urine were not statistically different between the three study groups (P>0.05). Vital signs did not change significantly in any child. Sedation with midazolam does not have adverse effects on the results of micturating cystourethrogram, while it reduces the discomfort in children undergoing this radiological technique.
Anti-Anxiety Agents↗
T-cell subsets and interleukin-6 response in Rasmussen's encephalitis.
To evaluate the immunopathogenesis in Rasmussen's encephalitis, peripheral lymphocyte subsets and interleukin-6 analysis were performed in three patients. Magnetic resonance spectroscopy and diffusion-weighted magnetic resonance imaging were performed to assess neuronal injury in the affected hemisphere. Before initiation of immune therapy, percentage of cytotoxic T cells was found to be increased in peripheral blood obtained from patients compared with a group of age-matched normal control subjects. During follow-up, percentage of cytotoxic T cells returned to the normal ranges only in one patient who had an early functional hemispherectomy. All three patients had significantly increased interleukin-6 concentration in cerebrospinal fluid and serum compared with the mean values of patients with acute viral encephalitis. The magnitude of interleukin-6 response in the patients correlated with the neuronal loss and atrophy on magnetic resonance spectroscopy and diffusion-weighted magnetic resonance imaging studies. The patient, who had a fulminant course and an early hemispherectomy, had higher interleukin-6 concentration in cerebrospinal fluid and serum than those of the other two. Detection of an increased percentage of cytotoxic T cells in peripheral blood supports the presence of a T cell-mediated inflammatory pathogenesis in Rasmussen's encephalitis. However, elevated interleukin-6 response might reflect the magnitude of the inflammatory process in the affected hemisphere.
Adolescent↗
Lymphocyte subsets in Bell's palsy: immune pathogenesis and outcome prediction.
The aim of this prospective study is to define the prognostic significance of lymphocyte subset analysis in children with Bell's palsy. Lymphocyte subgroup analysis in peripheral blood was performed in 17 children with Bell's palsy by using flow cytometry. Before a standard protocol of corticosteroid treatment, patients were categorized into two groups for facial nerve impairment on the basis of the clinical findings: Group 1 (mild to moderate impairment), 7 patients; and Group 2 (severe impairment), 10 patients. Outcome of the patients was evaluated at the end of 3 months follow-up and categorized as satisfactory recovery (n = 12) or unsatisfactory recovery (n = 5). Decreased percentages of B cells (CD19) and T helper/inducer (CD4) subsets were measured in patients with Bell's palsy compared with age-matched healthy control patients. Patients with severe impairment had significantly lower percentages of CD4 and CD19 subsets, whereas patients with mild to moderate impairment had only decreased percentage of CD19 subsets. There was no statistically significant difference in the percentage of lymphocyte subsets between the patients with satisfactory and unsatisfactory recovery. These results provide additional support for cell-mediated immunopathogenesis in patients with Bell's palsy, without any prognostic significance for the outcome.
Adolescent↗