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ABSTRACT: Immune thrombocytopenia (ITP) in children is usually a benign, self-limiting disorder. An acute Epstein-Barr virus (EBV) infection usually causes atypical lymphocytosis and mild decrease in platelets. Severe thrombocytopenia is an extremely rare complication. Anti-D immunoglobulin has been used for treatment of ITP in Rh(D)-positive nonsplenectomized patients. Severe hemolysis and acute renal failure are extremely rare complications that may be aggravated by the presence of an acute EBV infection. It is believed that anti-D immunoglobulin triggers an unusual virus-induced immune response causing hemolysis. We present a 4-year-old girl with ITP caused by an acute EBV infection that developed acute kidney injury following treatment with anti-D immunoglobulin. The patient recovered completely from thrombocytopenia and renal dysfunction. Intravascular hemolysis and acute kidney injury are consistent with anti-D immunoglobulin mechanism of action. Pediatric patients treated with anti-D immunoglobulin for ITP should be closely monitored for signs and symptoms of hemolysis that may be aggravated by the presence of EBV infection leading to impaired renal function.
Pediatric emergency care 06/2013; 29(6):748-50. · 0.92 Impact Factor
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ABSTRACT: We report on a two-year-old boy with acute flaccid paralysis (AFP) due to West Nile neuroinvasive disease (WNND). Serum and cerebrospinal fluid serology as well as nerve-conduction studies were consistent with the diagnosis. He received intravenous immunoglobulin (IVIG). The patient showed gradual improvement and complete recovery of his muscle strength, gait and deep tendon reflexes.
The Pediatric Infectious Disease Journal 03/2013; · 3.58 Impact Factor
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ABSTRACT: Kawasaki disease is a systemic vasculitis of unknown etiology, presenting typically in infants and young children. We report a rare case of incomplete Kawasaki disease in a 15-month-old male infant presenting with symptoms mimicking retropharyngeal abscess and intermittent fever.
The Pediatric Infectious Disease Journal 12/2011; 31(4):417-8. · 3.58 Impact Factor
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Pediatrics International 08/2010; 52(4):680-1. · 0.63 Impact Factor
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ABSTRACT: Dyskeratosis congenita (DC) is a rare disease characterised by bone marrow failure and skin manifestations. Patients with DC may exhibit short stature that is not usually related to growth hormone (GH) deficiency. Replacement treatment with GH should be done cautiously as it can predispose to haematological malignancy. We present a 10-year-old boy with DC and GH deficiency.
Case Reports 01/2010; 2010.
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ABSTRACT: Primary penile tuberculosis associated with bilateral inguinal lymphadenopathy is described in a previously healthy 10-month old infant, who had been circumcised in Pakistan 4 months earlier. Mycobacterium tuberculosis was detected by acid fast stain, PCR and culture in specimens obtained from the penile ulcer and the excised inguinal lymph nodes.
The Journal of infection 01/2009; 58(1):83-5. · 4.13 Impact Factor
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ABSTRACT: Studies on the effects of sodium valproate (VPA) on thyroid hormone balance in patients with epilepsy are conflicting. The aim of this study was to prospectively evaluate the changes in thyroid profile in children with epilepsy treated with VPA monotherapy.
Serum thyroxine, free thyroxine, triiodothyronine, and thyrotropin (TSH) levels were evaluated in 30 children with epilepsy, before and at 6, 12, and 24 months of VPA monotherapy.
All children had normal thyroid function before the initiation of VPA treatment. Serum VPA concentrations remained within the therapeutic range (50-100 mg/L) during the period of study. Thyroxine and free thyroxine levels were significantly decreased, whereas TSH levels were significantly increased at 6, 12, and 24 months of VPA therapy. Triiodothyronine levels were significantly decreased only at 24 months of therapy. Thirteen children (43.3%) at 6 months, 14 children (46.6%) at 12 months, and 15 children (50%) at 24 months of treatment had TSH values greater than 5 mIU/mL. Normal serum TSH levels were restored in all 8 children examined at 3 months after withdrawal of medication.
Valproate monotherapy may cause significant alteration in thyroid profile in children with epilepsy, occurring early in the course of treatment and persisting as long as VPA is initiated. Therefore, it may be useful to measure serum thyroid hormone concentrations routinely in children with epilepsy taking VPA. Further prospective studies are required to determine the mechanisms and risk factors for development of thyroid disturbance in children treated with VPA monotherapy.
Clinical neuropharmacology 11/2008; 32(1):32-4. · 2.35 Impact Factor
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ABSTRACT: Lipid abnormalities and thyroid dysfunction have been reported in patients treated with antiepileptic drugs. The aim of this study was to evaluate prospectively the association between thyroid and lipid profile in children treated with carbamazepine (CBZ) monotherapy.
Thyroid function was evaluated in 18 epileptic children, previously reported with CBZ-induced changes in serum lipid profile, before and at 6, 12 and 24 months of CBZ monotherapy.
All children had normal thyroid function before the initiation of CBZ treatment. During CBZ therapy thyroid dysfunction, with increased thyrotropin (TSH) and decreased thyroxine (T4), free thyroxine (FT4) and triiodothyronine (T3) was found, while, significant association was revealed between serum low-density lipoprotein cholesterol (LDL-C) and TSH levels at 6 (r=0.469; p=0.043) and 12 (r=0.730; p=0.001) months of treatment.
Lipid abnormalities may be associated with thyroid hormone disturbance in children treated with CBZ monotherapy. Since thyroid dysfunction and hypercholesterolemia are both associated with a higher atherosclerotic risk special attention and further studies are needed in epileptic patients treated with CBZ monotherapy.
European Journal of Paediatric Neurology 12/2007; 11(6):358-61. · 2.12 Impact Factor
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ABSTRACT: Congenital cataracts-facial dysmorphism-neuropathy syndrome (CCFDN, MIM: 604168), is a recently delineated neurogenetic disease causing recurrent episodes of rhabdomyolysis; prevention and early diagnosis of rhabdomyolysis should be part of the clinical management of the disease.
European Journal of Pediatrics 08/2007; 166(7):747-9. · 1.88 Impact Factor
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ABSTRACT: The case of a 13-year-old girl with primary Epstein-Barr virus (EBV) infection and concomitant cholestatic hepatitis, which initially presented as acute acalculous cholecystitis (AAC), is described. The diagnosis of AAC was documented by clinical and ultrasonographic criteria, whereas acute EBV infection was confirmed serologically. AAC may develop during the course of acute EBV infection, especially in patients with cholestatic hepatitis.
Journal of Pediatric Surgery 02/2007; 42(1):E11-3. · 1.45 Impact Factor
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Epilepsia 02/2007; 48(1):205-6. · 3.96 Impact Factor
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ABSTRACT: Plasma total homocysteine (p-tHcy), serum folate (s-F), serum vitamin B-12 (s-B12) and plasma pyridoxal-5'-phosphate (p-PLP) were measured in epileptic children before and after a 20-week period of sodium valproate (group A, n=32) and carbamazepine (group B, n=20) monotherapy. P-tHcy significantly increased in both groups, s-F and s-B12 significantly increased in group A, while s-F and p-PLP significantly decreased in group B. Our study showed an early effect of antiepileptic drug treatment on homocysteine metabolism.
Epilepsy Research 11/2006; 71(2-3):229-32. · 2.29 Impact Factor
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ABSTRACT: To investigate by a prospective, self-controlled method, whether early treatment with sodium valproate (VPA) monotherapy has some effect on serum total amylase and particularly on its pancreatic isoenzyme and lipase activities in epileptic children. Serum total amylase, pancreatic amylase and lipase activities have been evaluated in 23 epileptic children, before and at 6 and 12 months of VPA monotherapy. All children remained without clinical symptoms of pancreatitis during the period of study. Serum pancreatic amylase activities were significantly decreased at 6 and 12 months of treatment with VPA, whereas serum total amylase and lipase activities did not show any significant changes at 6 or 12 months of treatment. Non-pancreatic isoenzyme activities of amylase were significantly higher at 6 and 12 months of treatment. Three patients (13%) had slightly elevated serum total amylase levels at 6 and 12 months of treatment. There was no significant correlation of serum pancreatic amylase levels or non-pancreatic isoenzyme levels of amylase with serum VPA levels at 6 and 12 months of treatment. Non-pancreatic amylase activities, probably derived from salivary glands, may be increased in children treated with VPA monotherapy. Measurement of serum pancreatic amylase and/or serum lipase activities is indicated in patients with increased serum total amylase levels but without clinical symptoms of pancreatitis and, furthermore, in patients with symptoms suggesting dysfunction of pancreas, in order to avoid unnecessary discontinuing of VPA.
Brain and Development 11/2006; 28(9):572-5. · 2.12 Impact Factor
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ABSTRACT: The aim of this study was to investigate by a prospective, self-controlled method, whether treatment with carbamazepine (CBZ) and sodium valproate (VPA) monotherapy may alter serum lipoprotein (a) [Lp(a)] concentrations in epileptic children.
Serum Lp(a) concentrations have been determined in 18 epileptic children before and at 6, 12 and 24 months of treatment with CBZ monotherapy and in 30 epileptic children before and at 6, 12 and 24 months of treatment with VPA monotherapy. Serum total cholesterol, triglycerides, high-density lipoprotein cholesterol, low-density lipoprotein cholesterol, apolipoproteins A-I and B concentrations and serum concentrations of biochemical markers of liver and renal function were also measured in the study participants.
Serum Lp(a) concentrations were significantly increased at 6, 12 and 24 months of CBZ and VPA monotherapy. There were no significant correlations between serum Lp(a) and serum lipids, lipoproteins, apolipoproteins, concentrations of biochemical markers of liver and renal function or antiepileptic-drugs concentrations.
Children who receive CBZ or VPA monotherapy may have significant and persistent increase in serum lipoprotein (a) concentrations, occuring early in the course of therapy. It may be useful to measure serum Lp(a) concentrations routinely in epileptic children taking these antiepileptic drugs, especially in those that are already at higher atherosclerotic risk.
Epilepsy Research 09/2006; 70(2-3):211-7. · 2.29 Impact Factor
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ABSTRACT: The etiology of transient erythroblastopenia of childhood (TEC) remains unknown, although an association with viral infections has been proposed. The authors describe a 3.5-year-old girl with classic TEC concomitantly with human parvovirus B19 (HPV) infection. The infection was evident by detection of HPV genome in the blood and the bone marrow by polymerase chain reaction. Viral genome was no longer detected when the TEC resolved clinically. The patient was immunocompetent and the anemia has not recurred. To the authors' knowledge, this is one of the few documented cases of classic TEC attributable to HPV infection.
Journal of Pediatric Hematology/Oncology 07/2005; 27(6):333-6. · 1.16 Impact Factor
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ABSTRACT: The purpose of this study was to investigate, by a prospective, self-controlled method, whether early treatment with carbamazepine monotherapy can alter bone metabolism in ambulatory epileptic children with adequate sun exposure, based on the determination of total serum alkaline phosphatase and its bone isoenzyme activities. Serum total alkaline phosphatase and its bone, liver, and intestinal isoenzyme activities were evaluated in 22 epileptic ambulatory children (13 males and 9 females, aged from 5 to 12 years) before and at 3, 6, and 12 months of carbamazepine monotherapy. Serum concentrations of other biochemical markers of bone and liver metabolism, such as calcium, phosphorus, magnesium, gamma-glutamyltransferase, alanine aminotransferase, aspartate aminotransferase, and lactate dehydrogenase, were also measured in the study participants before and at 6 and 12 months of treatment. Carbamazepine was prescribed at normal dosages (16.4-20 mg/kg/day). Serum total alkaline phosphatase activities were significantly increased at 3 (P = .000), 6 (P = .024), and 12 (P = .037) months of treatment; serum bone alkaline phosphatase activities at 3 (P = .000), 6 (P = .008), and 12 (P = .017) months of treatment; and serum liver alkaline phosphatase activities at 3 (P = .000), 6 (P = .049), and 12 (P = .008) months of treatment, whereas serum intestinal alkaline phosphatase isoenzyme activity was significantly increased only at 3 months of treatment (P = .035). Serum gamma-glutamyltransferase activities were also significantly increased at 6 (P = .000) and 12 (P = .000) months of treatment. No significant changes in the concentrations of serum calcium, phosphorus, magnesium, alanine aminotransferase, aspartate aminotransferase, and lactate dehydrogenase were noted at 6 and 12 months of treatment. There was a significant correlation between serum gamma-glutamyltransferase activities and serum total alkaline phosphatase activities (r = .689, P = .000 at 6 months; r = .493, P = .020 at 12 months), bone alkaline phosphatase activities (r = .700, P = .000 at 6 months; r = .466, P = .029 at 12 months), and liver alkaline phosphatase activities (r = .427, P = .047 at 6 months; r = .425, P = .048 at 12 months). These findings indicate that ambulatory children who receive carbamazepine monotherapy, even when residing in a country with adequate sunlight, can have their bone metabolism altered early in the course of treatment, as indicated by the elevated activities of serum bone alkaline phosphatase isoenzyme. This early alteration in bone metabolism is probably due to the hepatic enzyme-inducing character of carbamazepine.
Journal of Child Neurology 07/2005; 20(6):513-6. · 1.75 Impact Factor
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ABSTRACT: Serum total amylase and lipase activities have been determined in epileptic patients treated with polytherapy using enzyme-inducing anticonvulsant drugs; however, to our knowledge, serum total amylase, pancreatic amylase and lipase activities have not previously been determined in patients receiving carbamazepine monotherapy. The purpose of this study was to investigate by a prospective, self-controlled method, whether early treatment with carbamazepine monotherapy may alter serum total amylase, pancreatic amylase and lipase concentrations of epileptic children.
Serum total amylase, pancreatic amylase and lipase activities have been determined in 18 epileptic children before and at 6 and 12 months of treatment with carbamazepine monotherapy. Serum gamma-glutamyltransferase activities were also determined.
Serum total amylase concentrations were significantly increased at 6 months of treatment (p=0.034), and serum nonpancreatic amylase concentrations were significantly increased at 6 (p=0.016) and 12 months of treatment (p=0.039), whereas serum pancreatic amylase and lipase concentrations did not significantly change at 6 or 12 months of treatment with carbamazepine monotherapy. Furthermore, serum gamma-glutamyltransferase concentrations were significantly increased at 6 (p=0.000) and 12 months of treatment (p=0.000) with carbamazepine monotherapy. There was no significant correlation between serum nonpancreatic amylase concentrations and serum gamma-glutamyltransferase or carbamazepine concentrations at 6 and 12 months of treatment with carbamazepine monotherapy.
These findings indicate that nonpancreatic amylase concentrations may be increased in patients treated with carbamazepine monotherapy. Therefore, measurement of serum pancreatic amylase and lipase concentrations is suggested in epileptic patients receiving carbamazepine monotherapy with symptoms suggesting pancreatic dysfunction, so that unnecessary discontinuing of treatment with carbamazepine should be avoided.
Clinica Chimica Acta 01/2005; 350(1-2):175-80. · 2.54 Impact Factor
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ABSTRACT: Hypoalbuminemia has been reported in patients with severe disability and epilepsy and in patients with epilepsy treated with short-term sodium valproate (VPA) therapy; however, serum albumin concentrations have not previously been determined in otherwise healthy patients with epilepsy and receiving long-term VPA monotherapy.
Serum albumin concentrations were determined in 26 ambulatory children with epilepsy before and at 6, 12, and 24 months of VPA monotherapy. Serum total protein concentrations and serum concentrations of other biochemical markers of liver and renal function such as alanine aminotransferase, aspartate aminotransferase, gamma-glutamyltransferase, and creatinine concentration were also measured in the study participants before and at 6, 12, and 24 months of treatment.
Serum albumin concentrations were reduced at 6 months of treatment (P = 0.007). Serum alanine aminotransferase concentrations were significantly increased at 6 (P = 0.034) and 12 months of treatment (P = 0.046), whereas serum aspartate aminotransferase concentrations were significantly increased at 6 (P = 0.002) and 12 months of treatment (P = 0.002). There were no significant correlations between serum albumin and the other parameters at 6 months of treatment.
Ambulatory children who receive VPA monotherapy may have early but transient decrease in serum albumin concentrations. Further studies are needed to address this issue and to determine the possible clinical implications and the mechanisms involved in VPA-mediated decrease in serum albumin concentrations.
Clinical Neuropharmacology 30(3):145-9. · 2.17 Impact Factor
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ABSTRACT: Ornithine transcarbamylase deficiency, an X-linked disorder, is the most common inherited urea cycle defect. Previous reports have documented the existence of several different mutations that can, partly at least, explain the phenotypic variability of the disorder. We describe the only male with T343K mutation, which also is present in his mother. We underline the disorientation of the beginning of clinical presentation; the patient became ill when fruits were added to his diet.
Pediatric Pathology and Molecular Medicine 22(2):153-7.
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ABSTRACT: Hyperuricemia has been shown to be related to cardiovascular morbidity and mortality. There is controversial data about the effect of sodium valproate (VPA) monotherapy on serum uric acid concentrations. The purpose of this study was to investigate by a long-term, prospective method, whether treatment with VPA monotherapy may alter serum uric acid concentrations and liver function tests in ambulatory epileptic children.
Serum uric acid concentrations were determined in 28 ambulatory epileptic children before and at 6, 12 and 24 months of VPA monotherapy. Serum concentrations of biochemical markers of liver and renal function, such as alanine aminotransferase (ALT), aspartate aminotransferase (AST), lactate dehydrogenase (LDH), gamma-glutamyltransferase (gamma-GT) and creatinine (Cr) were also measured before and at 6, 12 and 24 months of VPA monotherapy. Serum VPA concentrations remained within the therapeutic range (50-100 mg/L) during the period of study.
No statistically significant changes in serum uric acid concentrations were found at 6, 12 or 24 months of treatment. Serum ALT concentrations were significantly increased at 6 and 12 months of treatment, AST concentrations at 6 and 12 months of treatment and LDH concentrations at 12 months of treatment.
VPA monotherapy does not have a significant effect on serum uric acid concentrations in ambulatory epileptic children. Further studies are needed to definitively address whether it would be useful for physicians to routinely check for elevated serum uric acid levels in children treated with VPA.
European Journal of Paediatric Neurology 10(5-6):237-40. · 2.12 Impact Factor
