Sleep-wake patterns in children with intrauterine growth retardation

The purpose of this study was to characterize the sleep patterns of children with intrauterine growth retardation, known to be at risk for neurodevelopmental disorders, and seek a possible correlation with behavior, concentration, and attention problems. The sleep patterns of 26 children with intrauterine growth retardation aged 4 to 7 years were compared with those of 47 control children using activity monitors (actigraphs). In addition, data were collected from the parents regarding sleep habits, behavior, concentration, and attention. Children with intrauterine growth retardation aged 4 to 7 years were found to have a tendency toward poorer quality of sleep than their matched controls. This inclination was statistically significant only for one sleep measure, the true sleep time. A tendency toward increased fragmentation of sleep, prolonged waking, and decreased sleep efficiency, although not statistically significant in this study, was demonstrated. Our results showed that 58% of the children with intrauterine growth retardation, compared with 40% of the children in the control group, could be defined as "poor sleepers" (sleep efficiency lower than 90% or three or more waking episodes per night). This disturbed sleep profile is probably an integral part of the neurodevelopmental profile typical of these at-risk children. No significant correlations were found between sleep quality and behavior, concentration, and attention problems.     

Pseudotumor cerebri manifesting as stiff neck and torticollis

Stiff neck and torticollis are significant signs of neurologic disease. Nuchal rigidity is often associated with meningitis, subarachnoid hemorrhage, and posterior fossa tumor. Torticollis may be encountered in inflammatory disorders, such as cervical lymphadenitis, or it can be a sign of spinal cord syrinx or of central nervous system neoplasm. We report on three prepubertal children in whom stiff neck and torticollis were the presenting signs of pseudotumor cerebri. In all, the removal of 6-7 mL of cerebrospinal fluid led to prompt relief of symptoms and signs. We suggest that in the presence of unexplained stiff neck or torticollis in children, the optic discs should be examined to exclude pseudotumor cerebri.     

Unprovoked seizures after complex febrile convulsions

Children with complex febrile convulsions bear a higher risk of developing epilepsy than children with simple febrile convulsions. Complex febrile convulsions are defined by the presence of prolonged seizures, partial seizures and multiple seizures occurring during the same day. The aim of this study is to delineate the relative significance of each of the three criteria defining complex febrile convulsions. Fifty-seven out of 477 children (12%) admitted for febrile convulsions had complex febrile convulsions and normal neurological examination. Follow-up was available for 48 (84%) of them. Thirteen of these 48 (27%) had epilepsy at follow-up. The mean age of seizure onset among the patients with subsequent afebrile seizures was significantly lower than the rest (10.8 months versus 16.8 months). The patients with partial febrile convulsions showed a trend toward a higher risk (45%) of developing epilepsy than the patients with multiple febrile convulsions (21%).     

Methylenetetrahydrofolate reductase deficiency: importance of early diagnosis

Methylenetetrahydrofolate reductase deficiency is the most common inborn error of folate metabolism and should be suspected when homocystinuria is combined with hypomethioninemia. The main clinical findings are neurologic signs such as severe developmental delay, marked hypotonia, seizures, microcephaly, apnea, and coma. Most patients present in early life. The infantile form is severe, with rapid deterioration leading to death usually within 1 year. Treatment with betaine has been shown to be efficient in lowering homocysteine concentrations and returning methionine to normal, but the clinical response is variable. We report two brothers with methylenetetrahydrofolate reductase deficiency: the first was undiagnosed and died at 8 months of age from neurologic deterioration and apnea, while his brother, who was treated with betaine from the age of 4 months, is now 3 years old and has developmental delay.     

Reversible deafness caused by biotinidase deficiency

We present a child with complete biotinidase deficiency who developed bilateral sensorineural deafness without a response to a maximal stimulus of 90 dB in brainstem acoustic-evoked response. After treatment with 20 mg biotin daily, a repeated brainstem acoustic-evoked response demonstrated an improved hearing threshold of 65 dB, and the child began to talk. The case is a rare example of reversible hearing loss caused by to biotinidase deficiency and highlights the need for immediate replacement therapy once the diagnosis is established.     

Successful treatment of rheumatic chorea with carbamazepine

Carbamazepine has been used successfully in the treatment of different movement disorders and was recently reported to be effective for nonhereditary chorea. In view of the significant side effects associated with the drugs currently used to treat chorea, we sought to further evaluate the efficacy of carbamazepine in children with rheumatic chorea. The study was prospective and included 10 children with chorea (eight females and two males; age range = 7-16 years) referred to our Pediatric Rheumatology Clinic between 1995 and 1999. Nine had rheumatic fever and one had antiphospholipid antibody syndrome that later evolved to systemic lupus erythematosus. All were treated with carbamazepine. Improvement was evident within 2-14 days of initiation of low doses of carbamazepine (4-10 mg/kg daily). The plasma drug levels were 2.8-8.2 microg/mL (therapeutic antiepileptic range = 8-12 microg/mL). The chorea disappeared within 2-12 weeks. The duration of treatment was 1-15 months. No side effects were observed. Recurrence was observed in three patients who received a second trial of carbamazepine with a good response. We suggest that carbamazepine may serve as a first-line treatment for rheumatic chorea.     

Geographic variation in groundwater iodine and iodine deficiency in Israel, The West Bank and Gaza

BACKGROUND: Iodine deficiency during pregnancy and infancy is the world's most common preventable cause of mental retardation. Previous studies have shown a high incidence of goiter and low groundwater iodine concentrations in northern Israel. OBJECTIVE: We examined the relationship between low groundwater iodine and iodine deficiency in pregnant women and schoolchildren. SUBJECTS AND METHODS: We measured the urinary iodine excretion of school-children in the West Bank and Gaza and rural and urban pregnant women in Western Galilee (an area known to have low groundwater iodine concentrations). We also measured iodine concentrations in groundwater in various locations in the West Bank and Gaza. RESULTS: Lower urinary iodine excretion was found among pregnant Arab women living in rural Western Galilee (101+/-7 microg iodine/g creatinine). 20% of them excreted <50 microg I/g creatinine. This is relatively less than found among pregnant Jewish women living in cities in the same area (154+/-13 microg I/g creatinine). Low iodine concentrations (<5 microg/l) were found in groundwater in the Nablus, Ramallah, Bethlehem highlands, as compared to normal concentrations in the lowland districts of the West Bank and Gaza. In a cohort of 728 schoolchildren aged 8-10, 10% (range 8-13%) of children from areas of low groundwater iodine had low levels of urinary iodine excretion, as compared to only <5% of those from districts with groundwater iodine concentrations >10 microg/l. CONCLUSIONS: Lower concentrations of groundwater iodine are related to low urinary iodine excretion in Israel, the West Bank and Gaza.     

East and South East Asian Ethnicity and Moderate-to-Severe Endometriosis

Study Objective

To investigate ethnic differences for moderate-to-severe endometriosis.

Retrofitting implant overdenture attachments: A clinical report

The remake of implant-overdenture prostheses on preexisting implants can present the clinician with a challenge, especially when the prosthetic components used initially cannot be replaced. The difficulty of remaking the prosthesis may be further increased by implant attachments designed to be cemented to the implant itself--a feature that complicates future replacement. This clinical report describes the restoration of worn implant attachments using retrofit components.