Ototoxicity of baby oil in a chinchilla animal model.

OBJECTIVE: Baby oil is commonly used to soften ear wax in order to aid its removal. The aim of this study was to determine the potential ototoxicity of baby oil. METHOD: A prospective controlled animal study was conducted using ten chinchillas with normal hearing function. Each had bilateral myringotomies. One ear was randomly assigned to receive 1 ml of baby oil while the other ear received an equal volume of 0.45% NaCl. Distortion product otoacoustic emissions (DPOAEs) and Auditory Brainstem Response (ABR) measurements were recorded at baseline (post myringotomy and pre application of product) and on days 5 and 15 after application. Two months after application of baby oil, the cochleae were processed for light microscopy and qualitative comparisons were made between the cochleae of both control and experimental ears. RESULTS: There was no statistically significant difference in DPOAE between experimental and control ears at 5 and 15 days after treatment. ABR results did not reveal ototoxicity at days 5 and 15 post treatment. None of the animals developed facial paralysis or any signs of vestibular toxicity. There were no overt mucosal changes in the middle ear of the ears exposed to baby oil compared to the control ears. Light microscopy showed comparable features in the organ of Corti, stria vascularis, spiral ligament and the spiral ganglion cells of both groups of cochleae. CONCLUSION: Baby oil did not produce ototoxic effects when applied ototopically in chinchillas with non-intact tympanic membrane.

Reversible cardiomyopathy caused by an uncommon form of congenital adrenal hyperplasia.

A child with 11, beta-hydroxylase deficiency, an unusual variant of congenital adrenal hyperplasia, presented with dilated cardiomyopathy. Congestive heart failure and dilatation of ventricles resolved with hydrocortisone replacement therapy. This case highlights the importance of complete history and physical examination of children with dilated cardiomyopathy.

Prevalence of mental retardation among children in Saudi Arabia.

This survey determined the prevalence and regional distribution of mental retardation among children in Saudi Arabia. Data were analysed from a population-based national survey conducted during 1996-99, in which 60,630 children aged up to 18 years were screened using IQ tests and questionnaires completed by physicians. The prevalence of mental retardation was 8.9 per 1000 children, a rate similar to that reported in other countries. Moderate or severe retardation was classified in 70.9% of these children. Of the mentally retarded children in the 0-18 years age range, 83.2% were not attending school. Special educational programmes are needed to improve the quality of life of mentally retarded children.

Prevalence of mental retardation among children in Saudi Arabia

This survey determined the prevalence and regional distribution of mental retardation among children in Saudi Arabia. Data were analysed from a population-based national survey conducted during 1996-99, in which 60,630 children aged up to 18 years were screened using IQ tests and questionnaires completed by physicians. The prevalence of mental retardation was 8.9 per 1000 children, a rate similar to that reported in other countries. Moderate or severe retardation was classified in 70.9% of these children. Of the mentally retarded children in the 0-18 years age range, 83.2% were not attending school. Special educational programmes are needed to improve the quality of life of mentally retarded children

Nontraumatic brain hemorrhage in children: etiology and presentation.

The clinical and radiographic findings of 68 children and adolescents with nontraumatic intraparenchymal brain hemorrhage were analyzed retrospectively. There were 43 boys and 25 girls, and the average age was 7.1 years (range, 3 months to 18 years). The most common presenting symptom was a combination of headache or vomiting (40 cases, or 58.8%). Hemiparesis was the major presenting sign in 11 (16.2%) of the children, seizures occurred in 25 (36.8%) patients, and 6 (8.8%) children were irritable. Only 2 (2.9%) children were comatose at presentation. One or more risk factors for hemorrhage were found in 61 (89.7%) of 68 children. A third (23 cases, or 33.8%) had an arteriovenous malformation or fistula; altogether 29 (42.6%) children had some type of congenital vascular anomaly. Hematologic or coagulation disorders were present in 22 (32.4%) patients, and 9 (13.2%) patients had brain tumors. Hemorrhage could not be attributed to systemic hypertension in any child. The likelihood of establishing the cause of bleeding was greater when evaluation included cerebral angiography (97.3% versus 80.4% without angiography). Half (34 cases, or 50.0%) of the patients regained normal neurologic function. Six (8.8%) patients died, either directly or partly as a consequence of the hemorrhage. The remaining patients had various neurologic sequelae, including 17 (25.0%) with hemiparesis, 5 (7.4%) with aphasia, 7 (10.3%) with epileptic seizures, and 3 (4.4%) with hydrocephalus. More detailed follow-up studies are needed to obtain more information about the frequency of cognitive sequelae.

Sotos syndrome (cerebral gigantism): a clinical and radiological study of 14 cases from Saudi Arabia.

Fourteen children (of Arab ethnic origin) with Sotos syndrome are described. They were referred to King Khalid University Hospital, Riyadh between July 1992 and June 1997. Their phenotypic characteristics were compared with established diagnostic criteria. There was a male:female ratio of 1.3:1 and a high rate of consanguinity (36%) among parents. At birth, 54% were large and about one-third showed increased height and occipitofrontal head circumference (OFHC). The neonatal histories revealed respiratory and feeding problems in 21%, followed later by delayed motor milestones and speech development in 57%. During childhood, weight, height and OFHC increased further to > 97th centile in 71%, 71% and 93%, respectively. A seizure disorder affected 43%, and 75% had mental retardation (IQ < 70). A non-specific EEG abnormality was found in half of those with seizures. Cranial CT/MRI showed ventricular dilatation in 15% and one patient had corpus callosum dysgenesis. Abdominal ultrasound revealed hydronephrosis in two patients. Radiological cephalometric measurements showed relative prognathism in cases of Sotos syndrome compared with controls (p = 0.003). The study highlights the importance of considering Sotos syndrome in children who present with psychomotor delay.

Results of 546 Blalock-Taussig shunts performed in 478 patients.

Between 1983 and 1995, 546 Blalock-Taussig shunt procedures were performed in 472 patients: 128 (23.0%) were classical shunts, 90 of them on the same side as to the aortic arch, and 418 (77.0%) were modified shunts, 182 on the same side of the arch. At the time of surgery, 78 patients were aged below one week, 270 from one week to 12 months, and 198 patients were over one year of age. The mean pre-operative arterial saturation (71.7% +/- 16.5%) was significantly increased to 83% +/- 17.9% immediately after the procedure (p=0.017). The overall hospital mortality rate was 2.9% (16/546), with rates of 2.3% (3/128) for the classical, and 3.1% (13/418) for the modified shunts (p=not significant). The rate was significantly higher, however, for classical shunts when the pulmonary arterial diameter was less than 4 mm (15.4% versus zero; p=0.047), though this relationship was reversed for modified shunts (zero versus 3.6%; p=0.338). Early mortality was significantly influenced by the age at surgery, 5/78 (6.4%) in patients aged below 1 week, 3.7% between 1 week and 1 year, and 0.5% over 1 year (p=0.019). Early mortality was also significantly increased in patients weighing 3kg or less, 8/156 (5.1%), versus 3/303 (1.0%), p=0.037. Overall, 51 shunts failed (9.3%), 10 early and 41 late. Early failure was significantly increased in patients weighing 3kg or less, 8/156 (5.1%) versus 3/303 (1.0%), p=0.016. The overall early failure rate was 1.4% (3/215) when heparin was administered intra-operatively and for 48 hours postoperatively, in contrast to an early failure rate of 3.4% (7/203) when heparin was not used (p=0.294). Overall rates of failure during follow-up were 9.1% (17/188) in heparinized patients versus 13.6% (24/177), (p=0.173) in non-heparinized patients. Failure of classical shunts was 10.2% (13/128), compared with 6.7% (28/418) for modified shunts (p=0.195). Failure was more common overall if the pulmonary arterial diameter was less than 4 mm, 14.7% (9/61), as opposed to 8.7% (26/300) when the diameter was 4 mm or greater, (p=0.144). Administration of aspirin during follow-up after the modified shunt procedure reduced failure from 11% (18/163) to 6.7% (10/150), p=0.176. Classical or modified Blalock-Taussig shunts, either on the same side or opposite to the aortic arch, can be performed on patients of any age with minimum postoperative complications and low operative mortality. The use of intra- and post-operative heparin appears to reduce the overall rate of failure, and the administration of aspirin during follow-up appears to reduce failure of modified Blalock-Taussig shunts.

The spectrum of child abuse presenting to a university hospital in Riyadh.

BACKGROUND: Child abuse is prevalent worldwide, although it is often underreported. we describe the pattern of child abuse and neglect presenting to the emergency room of our hospital, the sociocultural changes which brought this about, and suggest ways to deal with this emotionally sensitive issue. PATIENTS AND METHODS: Thirteen cases of child abuse and neglect were seen in the emergency room of King Khalid University Hospital over a period of one year from July 1996 to June 1997. There were four cases of non-accidental injury, three of which had serious injury. There were three cases of sexual abuse, four cases of neglect, resulting in the death of one child and severe emaciation in another. There was one suspected case of Munchausen syndrome by proxy, and one case of child labor with neglect. CONCLUSION: Public awareness of the problem of child abuse has increased, and recent media reports reflect the significance accorded to the issue. As more information is obtained on this subject and policies and guidelines are set in place, efforts at reporting and preventing physical and psychological trauma will gather momentum.

The Hemopump as a left ventricular assist device in pediatric applications: initial Canadian applications.

Three children aged 11 months, and eight and nine years were supported with a Hemopump as a potentially life saving measure for circulatory failure. Left ventricular assist time ranged from 10 to 32 h. None of the three patients could be successfully weaned from the device because of complications of bleeding, arrhythmia or neurological insult. Despite poor outcomes, each patient demonstrated important hemodynamic stabilization with the device. The Hemopump is suggested as a potentially life saving treatment modality for selected pediatric patients who have critical left ventricular failure.

Hemiconvulsion-hemiplegia-epilepsy syndrome. A clinical, electroencephalographic and neuroradiological study.

Six patients (4 boys and 2 girls) with hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome are described. They had prolonged seizures, lasting from 30 min to 12 h, at ages 1-4 years. These took the form of hemiconvulsion in three of the children and generalized tonic-clonic seizures in the others, being preceded by hemifacial twitching or head and eye deviation in two. They were followed by hemiplegia, which cleared with time in five patients, apart from subtle pyramidal tract signs. One child had spastic quadriparesis, choreiform movements, contracture deformities and severe mental retardation following repeated status epilepticus. Subsequent epilepsy developed in five patients and was satisfactorily controlled with carbamazepine and/or phenobarbitone. Cerebral hemiatrophy was documented in all patients by cranial computed tomography and/or magnetic resonance imaging. Single photon emission computed tomography (done in 4 patients) showed ipsilateral hypoperfusion (of the damaged hemisphere). Electroencephalography showed ipsilateral slowing and low voltage of background activity. Epileptiform discharges were found on the ipsilateral side in two cases and the contralateral side (the undamaged hemisphere) in one.

Childhood neuromuscular disorders: a decade's experience in Saudi Arabia.

The study describes the pattern of childhood neuromuscular disorders seen in a decade (1982-1992) at King Khalid University Hospital, Riyadh, Saudi Arabia. Similar data are scanty outside Europe and North America, and lacking in Saudi Arabia. Eighty-four children (< or = 16 years) were assigned to an entity of neuromuscular disease following review of the clinical, biochemical and neurophysiological data, and after re-examination of the histological and histochemical features of the muscle biopsies. Of the 84 ascertained cases, 40 (48%) had different forms of muscular dystrophy (MD), 26 (31%) had one of the various types of spinal muscular atrophy (SMA) and two (2.4%) hereditary motor and sensory neuropathy type I. The rest were miscellaneous cases including one (1.2%) with dermatomyositis. Of the dystrophies, severe childhood autosomal recessive muscular dystrophy (SCARMD) was more prevalent (30%) than Duchenne type (25%), conforming with observations from North African countries known to have a high incidence of consanguineous marriages. Family history of other cases of SCARMD included three males and three females, one of whom died at 15 years, and consanguinity was evident in 63%. Congenital MD, inherited in an autosomal recessive pattern, was also common (30%). A history of consanguinity was present in 55%. Of the 26 cases of SMA, type I (Werdnig-Hoffman disease) was the most prevalent (69%). Consanguinity was ascertained in 65% of SMA families and histories revealed another 14 affected siblings. Autosomal recessive forms seem to constitute the bulk of neuromuscular disorders in Saudi Arabia.

Rett syndrome in Saudi Arabia: report of six patients.

Six girls (five Saudis and one Sudanese) aged between 3.5 and 12 years demonstrated the classic features of Rett syndrome (RS), including developmental regression with dementia, loss of acquired speech and hand function, and stereotypic hand movements. Myoclonic seizures were observed in two of them. Electroencephalography showed diffuse slowing in three cases generalized epileptic discharges in one, and normal findings in two. Cranial computed tomography revealed normal findings, except in the 12-year-old girl who had mild bilateral frontal lobe atrophy. Except for mildly elevated blood ammonia in three patients, associated with slightly elevated blood lactate in two of them, results of neurometabolic screening tests were normal. There are, as yet, no laboratory markers pathognomonic for RS. Hence, adherence to current diagnostic criteria is essential. To prevent unnecessary and costly investigations, especially in developing countries, wider knowledge of the clinical features of the syndrome is warranted.

Transient mutism following posterior fossa surgery in children.

We report 3 patients, ages 5, 9 and 13 years, with mutism following posterior fossa surgery (PFS). All presented with headache of 10-180 days duration, excellent premorbid learning performance and paucity of neurologic signs. Radiographic studies demonstrated large posterior fossa tumor occupying the fourth ventricle, and hydrocephalus, leading to gross total resection of the tumor. Within 24-48 hr, all patients were mute. None had problems with swallowing and coughing. All were able to nod "yes or no," follow commands, point to body parts, and make their wants known by gestures. Jaw, gag, snout and palmomental reflexes were normal. CT scan and MRI Brain showed no cerebral hemispheric lesions. All children regained speech although dysarthric, within 5-12 weeks. Twenty-two previously reported cases are similar to ours. We conclude that this post-surgical mutism syndrome is unique and must be differentiated from aphemia, Broca's aphasia, hysterical mutism and elective mutism. The cerebellum, in a way not yet understood, does play a role in speech production.

Growth hormone and IGF1 profile in short children with osteopetrosis.

Osteopetrosis is commonly associated with short stature. To identify the cause, 8 children with osteopetrosis and short stature were studied. One showed evidence of renal tubular acidosis, none showed evidence of anaemia, and all were clinically and biochemically euthyroid. Growth hormone profile was assessed using night sampling (8 patients), growth hormone provocative testing using insulin-induced hypoglycaemia (6 patients), and L-dopa-propranolol (8 patients). The mean nocturnal growth hormone values taken at 60 and 90 min after onset of sleep and at 4 a.m. were 5.5, 12.8, and 11.5 mu/L respectively. The peak stimulated growth hormone mean values with the insulin-induced hypoglycaemia (glucose 1.6-3.0 mmol/L, mean = 2.2) was 14 mu/L (range 7.3-24.5 mu/L) and with the L-dopa-propranolol was 25.6 mu/L (range 12.3-49 mu/L). IGF1 levels taken at 0 and 120 min of insulin-induced hypoglycaemia (0 min for L-dopa-propranolol), and at 120 min of L-dopa-propranolol, showed normal values for age. We conclude that: (1) growth hormone profiles in these children are normal; (2) tissue unresponsiveness to growth hormone and/or IGF1 is not likely to be the cause of short stature in children with osteopetrosis; and (3) osteopetrosis per se is not an indication for assessment of growth hormone status.

Chorea as a presentation of herpes simplex encephalitis relapse.

Three infants, ages 3 months to 3 years, presented with chorea as the initial manifestation of herpes simplex encephalitis (HSE) relapse. Patient 2, treated with repeated 10 day courses of 30 mg/kg/day of acyclovir, had no clear improvement in neurological status. Patient 1, treated with a repeated 10-day course, improved only to have another HSE relapse 4 years later. Patient 3 clearly improved soon after a 3-week course of acyclovir at conventional dosages. A fourth patient (Patient 4) who relapsed with chorea after what was thought to be HSE, and who did not respond to repeated acyclovir treatment, was negative for herpes simplex virus indicators on brain biopsy and DNA testing. We recommend treating all patients suffering from HSE with a minimum 3-week course of acyclovir at 30-35 mg/kg/day in 3 divided doses.

Antecedents and outcome of simple and complex febrile convulsions among Saudi children.

Of 215 Saudi children seen with their first febrile convulsion (FC) at the King Khalid University Hospital, Riyadh, between January 1984 and December 1988, the index FC was simple for 133 children and complex for the remaining 82. History of adverse antecedent factors, particularly perinatal asphyxia, birth injuries and pre-existing neurological deficits, were significantly more associated with complex FC, as was occurrence of first FC before the age of 12 months. Recurrence of FCs and development of epilepsy were also more common among the group of children with complex FC. Complex FCs were less benign in the present study population than has been reported in some Western studies.