Adverse cutaneous reactions to phenobarbital in epileptic children.

INTRODUCTION: Cutaneous adverse reaction to phenobarbital is not uncommon. According to previous studies, around 3 per cent of children taking phenobarbital have reactions. However, there has been no report in Thai children. OBJECTIVE: To study adverse cutaneous reactions to phenobarbital in children with epilepsy. PATIENTS AND METHOD: A retrospective study from medical records of epileptic children aged under 15 years diagnosed at the Department of Pediatrics, Ramathibodi Hospital, Bangkok, Thailand from January 1989 to December 1993 was done. Adverse cutaneous reactions were categorized into 3 groups according to severity. Duration from the initiation of phenobarbital to the onset of reactions and the clinical course were collected for analysis. RESULT: There were 18 children from the total of 572-retrievable medical records of children with epilepsy who had adverse cutaneous reactions. The prevalence was 3.2%. There were 5, 10 and 3 patients categorized into mild-form, moderate-form, and severe form respectively. All except one patient had the onset of cuteneous reactions within 3 weeks. No morbidity or mortality was observed in these patients. Recovery of the cutaneous reactions was obtained between 5 and 14 days in those with mild or moderate form. CONCLUSION: Adverse cutaneous reactions to phenobarbital observed in Thai epileptic children were similar to those found in previous reports. Physicians who prescribe phenobarbital must be aware of the serious adverse reactions which might occur. Early recognition of the adverse reactions and prompt intervention including discontinuation of the drug must be exercised to prevent any serious complications.

Thromboembolic complications in Thai pediatric patients.

Sixty patients with thromboembolic complications from 1987 to 1997 at the Department of Pediatrics, Ramathibodi Hospital were retrospectively studied. Twenty patients were infants and 40 patients were children and adolescents with a mean age of 18 days and 8 years, respectively. The sites of thromboembolic complications were in the central nervous system, 27.5 per cent; skin as purpura fulminans or necrotic lesions, 24.5 per cent; gangrene of the toe, finger or colon, 19 per cent; deep vein thrombosis, 16 per cent; and other sites such as heart and lungs, 13 per cent. Most of them had triggering conditions (80%) and underlying diseases (76.7%) causing thromboembolism. The low levels of either antithrombin III, protein C or protein S were found in 42 per cent (15/36). The management included administration of standard or low molecular weight heparin if not contraindicated, replacement of fresh frozen plasma 10 ml/kg twice a day and treatment of underlying and triggering conditions. The fatality rate was 15 per cent (9/60). Subsequent episodes of thromboembolism occurred in 6 patients including: pulmonary emboli in one patient with protein C deficiency who refused warfarin administration, deep vein thrombosis in 2 patients with unidentified etiology, and necrotic skin lesions in 3 patients with vasculitis who did not respond to treatment. In conclusion, a comprehensive investigation and specific treatment for patients with thromboembolic complications are emphasized in order to prevent recurring episodes.

Seizures after intracranial surgery in pediatric patients.

We retrospectively reviewed the occurrence of seizure after performing intracranial operations in children aged less than 15 years. During a 5-year period, there were 18 patients out of a total of 155 who developed one or more seizures within 1 year after operation. The majority of patients (55.6%) had the onset of seizures within 24 hours. Seventy-two per cent of the patients had partial seizures. Two patients who developed immediate postoperative seizures had sodium derangement. Eight of 10 patients who had early onset seizures had had an operation for supratentorial lesions. Among the 6 patients who developed seizures after infratentorial tumor removal, the cause of seizure was not known in 4 patients. We emphasize that prompt investigations to exclude any structural lesions and other possible causes of seizure, especially electrolyte disturbance along with appropriate antiepileptic drug administration, are important in patients who develop seizures after the operation. The recommendation for routine administration of antiepileptic drug prior to the operation has yet to be concluded.

Vigabatrin in infantile spasms: preliminary result.

PURPOSE: To evaluate the efficacy of vigabatrin in the treatment of infantile spasms in Thai children. PATIENTS & METHOD: From March 1996 to May 1998, patients aged under 2 years presenting with infantile spasms at Ramathibodi Hospital were initiated with vigabatrin 35-50 mg/kg/day in two-divided doses. The dosage was escalated by 25 mg/kg weekly until spasms ceased or the maximum dose of 130 mg/kg was reached. RESULTS: There were 20 patients enrolled. The ages ranged from 3 to 23 months (mean 7.6 months). They were categorized as 4 cryptogenic and 16 symptomatic. Infantile spasms were completely controlled in 12 patients (60%). Six patients (30%) had at least 50 per cent reduction of seizure frequency. There were 2 patients whose seizure frequencies and severity were not altered. Only one patient whose infantile spasms partially responded to vigabatrin developed orofacial dyskinesis which disappeared after discontinuation of vigabatrin. Five patients had their vision evaluated which was unremarkable. Based on parental global evaluation, there was an increase in alertness, cheerfulness and interaction to the environment and stimulation in 8 out of 15 patients who were still taking vigabatrin and responded to treatment. CONCLUSION: Vigabatrin is effective for infantile spasms. A long-term follow-up of these patients is necessary to evaluate its efficacy and side-effects.

Temporal lobectomy for intractable complex partial seizures in pediatric patients.

Fourteen children, 7 boys and 7 girls, who failed conventional medical treatment for complex partial seizures underwent anterior temporal lobectomy at Ramathibodi Hospital, Bangkok, Thailand, from July 1993 to June 1998. The mean age at onset of patients was 6.7 years old and the mean duration of seizures before surgery was 6.4 years. The age of patients at surgery ranged from 8 to 22 years old. These patients had had limited presurgical evaluation which included video-electroencephalography (EEG), magnetic resonance imaging (MRI) and single photon emission computed tomography (SPECT). All patients demonstrated concordant among clinical symptoms, EEG, MRI and SPECT on the same side of the temporal lobe. Ten and 4 patients had unilateral and bilateral temporal lobe lesions respectively. The operations were done on the left in 10 patients and on the right in 4 patients. Seizure free after 1/2-5 years of follow-up was obtained in 70 per cent of patients which included 9 of 10 patients with unilateral temporal lesion and 1 of 4 patients with bilateral temporal lesions. The rest of the patients showed marked reduction of seizures except for one patient with bilateral lesions in whom only 50-70 per cent reduction was obtained. Marked improvement of behaviour was also observed in 70 per cent of patients. Resected brain specimens revealed mesial temporal sclerosis, gliosis, tumors and cavernoma in 6, 5, 2 and 1 patients respectively.

Recurrent acute cerebellar ataxia of childhood following nonspecific respiratory tract infection.

Acute cerebellar ataxia in childhood following viral infection is a self-limited disease. The disease with recurrent course has rarely been reported. At the Department of Pediatrics, Ramathibodi Hospital, three children with recurrent episodes of acute cerebellar ataxia following nonspecific viral infection were encountered. The age at onset of each patient was 2 years, 18 months and 2 years old. The clinical symptoms were similar and improved rapidly after gluco-corticoid was given. All patients recovered without residual deficit. Six, 5 and 3 recurrent attacks of similar illness were noted in each patient respectively after the first episode. However, no further attack occurred after the age of 5 years and the age of last follow-up was 17, 16 and 14 years old respectively. The pathogenesis of the recurrent episodes is uncertain. The abnormal immunological response is postulated.

Guillain-Barre syndrome in Thai children: retrospective analysis of the clinical and outcome prior to intravenous immune globulin era.

The authors retrospectively reviewed Guillain-Barre syndrome (GBS) in 48 Thai children over a period of 20 years from 1970 to 1989. The clinical presentations of this syndrome were compared to those reported in previous studies of children in Western and Asian countries. Antecedent infection, including respiratory tract infection and nonspecific viral infection, in this group of patients is similar to previous studies in children. Cranial nerve involvement found in this study (45.8%) was higher than that in other studies. The outcome of GBS in Thai children in this study was not different from other reports even without IVIG administration. The authors emphasize that respiratory and supportive care are important in managing patients suffering from this syndrome.

Triple diagnosis in carcinoma of the breast: a replacement for open biopsy.

Using the combination of clinical diagnosis, mammography and fine-needle aspiration cytology (FNA) as a "triple diagnosis" to guide management of carcinoma of the breast, we retrospectively reviewed 46 patients with FNA diagnosis of carcinoma of the breast in Ramathibodi Hospital from 1993 to 1995. Pathological diagnosis was available in 36 cases and used as a gold standard. Clinical diagnosis alone was accurate in 28 of 36 cases. Mammography was available in 24 cases of which the diagnosis was accurate in 22 cases. Triple diagnosis was available in 19 cases and all were accurate. Using this approach and review of the literature, we suggest that triple diagnosis could replace an open biopsy in diagnosis of carcinoma of the breast.

Accuracy of fine needle aspiration cytology in the evaluation of peripheral lymphadenopathy.

We conducted an audit of the lymph node aspirates received from January 1996 to December 1996 of 541 patients sent to the Cytology Division, Department of Pathology, Ramathibodi Hospital by their clinicians. The aim of this retrospective study was to determine the pattern of diseases that commonly present with peripheral lymphadenopathy and to evaluate the accuracy of Fine Needle Aspiration Cytology (FNAC) in the diagnosis of lymph node diseases. An excisional biopsy sample of lymph node was available in 233 (43%) cases for comparison to the histopathology. The predominant lesion was benign which included necrotizing granulomatous lymphadenitis (NGL), reactive changes (RC) and suppurative lymphadenitis (SL). The predominant malignant lesion was metastatic squamous cell carcinoma. The accuracy for NGL, SL and RC were 69 per cent, 75 per cent and 95 per cent, respectively. The accuracy for metastatic disease was 97 per cent. The specificity and sensitivity of FNAC were 99 per cent and 94 per cent, respectively. An excisional biopsy should be done in case of doubt to clarify the pattern of RC.

Strokes in Thai children : etiology and outcome.

In Asian countries, specific etiology and outcome of stroke in children are rarely reported. During January 1979 to December 1997, 68 children with stroke, admitted to the Department of Pediatrics, Ramathibodi Hospital, Bangkok were reviewed for etiology and outcome; 38 patients (22 males) had ischemic stroke and 30 (20 males) had hemorrhagic stroke. Severe headache, vomiting, disturbance of consciousness and papilledema were prominent presentations of hemorrhagic stroke. Bleeding from vascular anomalies of the brain (AVM) was the most common etiology of hemorrhagic stroke while septic and non-septic emboli from congenital and acquired heart diseases were the most common cause of ischemic stroke. The mortality rate was 7% and 9% in hemorrhagic and ischemic strokes, respectively. Complete recovery was observed in 52% of cases of hemorrhagic stroke but in only 26% of ischemic stroke. Hemiparesis was the most common residual neurological deficit with higher occurrence in ischemic stroke.

Sensorineural hearing loss in children recovered from purulent meningitis: a study in Thai children at Ramathibodi Hospital.

Hearing loss is one of the serious complications of bacterial meningitis. Conventional audiometry and auditory brainstem response (ABR) methods were used to detect this complication in eighteen children who recovered from bacterial meningitis treated at Ramathibodi Hospital from January 1983 to December 1987. Six patients (33%) were found to have persistent bilateral sensorineural hearing loss. Among them, the causes of meningitis were: Hemophilus influenzae (3 patients), Streptococcus pneumoniae (1 patient), Streptococcus agalactiae (1 patient) and Escherichia coli (1 patient). Various clinical and demographic factors were examined in relation to the hearing loss, but no significant correlation was observed. Since meningitis often affects small children and makes conventional audiometry tests difficult, ABR was found to be a more effective method for testing this group of patients.

Antigens, antibodies and immune complexes in cerebrospinal fluid of patients with cerebral gnathostomiasis.

Methods for the detection of antigens, antibodies and immune complexes in the cerebrospinal fluid (CSF) of patients with neurological manifestations suggestive of cerebral gnathostomiasis were developed, in the hope that they may be useful in confirming the diagnosis of Gnathostoma spinigerum infection. Gnathostoma antigens were determined by a sandwich enzyme linked immunosorbent assay (ELISA) using antibodies from rabbits immunized with the excretory/secretory (ES) antigens obtained from the in vitro supernatant fluid in which the third-stage G. spinigerum larvae were maintained. With a biotin streptavidin procedure, the presence of G. spinigerum antigens as low as 2 ng in one ml of CSF could be detected. An indirect ELISA was used for the quantitation of IgG antibodies in the paired serum and CSF of these patients. A complement consumption method was used for the detection of immune complexes in the concentrated CSF specimens. Of the 11 patients with clinical signs and symptoms suggestive of having G. spinigerum infection involving the central nervous system, only one patient had antigens detected in the CSF and in this one patient no antibody could be demonstrated. One other patient had immune complexes in her CSF. All remaining patients had IgG antibodies demonstrable in the CSF specimens. These data suggest that the detection of IgG antibodies in CSF is more reliable than the other two methods in confirming the diagnosis of cerebral gnathostomiasis.

Brain tumors in children at Ramathibodi Hospital.

Brain tumors remain the second most common neoplasm of childhood, however, there is no adequate study of this disease in Thai children. From the year 1971 to 1987, 468 children under 15 years of age were admitted to the Department of Pediatrics, Ramathibodi hospital with the diagnosis of brain tumor. Four hundred and forty-four patients had primary intracranial tumors, of these, 17 were excluded due to inadequate information and the remaining 427 patients were studied. Male to female ratio was 1.3:1, the age ranged from one day old to 14 years old. The duration of illness before admission ranged from a few days to 4 years, with over 70 per cent within 3 months. Headache, vomiting and papilledema were the three most common symptoms and signs. Supratentorial and infratentoral tumors were seen in 209 and 216 patients respectively, craniopharyngioma was the most common supratentorial tumor (51/209) and medulloblastoma was the most common infratentorial tumor (95/216), however, malignant astrocytoma is the overall most common tumor type. Early diagnosis and treatment were needed for good outcome.

Maple syrup urine disease: case report of 2 Thai infants.

Maple syrup urine disease (MSUD) is a rare inborn error of metabolism characterized by typical urine odor. The deficiency of branched-chain ketoacid decarboxylase enzyme is responsible for the clinical abnormalities. The classical disease usually manifests in the neonatal period with lethargy, refused feeding, seizures and death. Since 1984, at the Department of Pediatrics, Ramathibodi hospital, 2 patients with classical MSUD have been seen. The parents of one patient were relatives and already had 2 affected but undiagnosed daughters. Both patients had strong urine odor which was described as the odor of boiled Chinese herbal medicine. The first child died at 4 months old and the second was severely retarded at one year old. The importance of early diagnosis and genetic counselling are emphasized.

Moyamoya disease: a report of four cases of Thai children.

Moyamoya disease is a cerebrovascular disorder characterized by occlusion of the internal carotid arteries with cerebral angiographic finding of smoke-like collateral vessels. Typical moyamoya blush can also be seen in immediate enhanced CT scan of the brain. The disease is rare in non Japanese. The present communication reports four Thai children aged 3-12 years, presented at the Department of Pediatrics, Ramathibodi hospital with recurrent hemiparesis. In one patient, brain biopsy was almost carried out. The disease should be suspected in every child with recurrent cerebral ischemic episodes. Cerebral angiogram and immediate enhanced CT scan are the two most useful investigation procedures. Either of them should be selected first for diagnosis before other unnecessary invasive investigations are done.