Elevated level of creatine phosphokinase in newborn: Clinical significance and association with congenital muscle diseases.

OBJECTIVES: To establish the normal Creatine phosphokinase (CPK) range in newborns of all 3 modes of delivery and prove that high CPK level in neonates is not specific a indicator for muscular pathology. METHODS: This is a prospective cohort study that is conducted in King Abdulaziz Medical City and King Abdullah Specialized Children Hospital in Riyadh and included 504 term neonates who were born between March 2021 and August 2021. Two hundred and fifty three were males and 251 were females. Data and consents were managed and collected using 2 coordinators. RESULTS: Duration of the second stage of labor, age on the first CPK test and fetal gestational age were significantly correlated with CPK values (r=0.197, r=-0.234, r=0.274, respectively). The normal ranges for each delivery type were 334 U/L-2667U/L in normal spontaneous vaginal delivery, 265U/L-1182U/L in elective cesarean section, and 223U/L-3082 U/L in emergency cesarean section. CONCLUSION: The CPK was elevated in all neonates in all 3 modes of deliveries. An elevated levels of CPK in neonates is not a specific indicator for any congenital muscular pathology.

Effect of melatonin in children with neurodevelopmental disabilities and sleep disorders.

Objectives: The aim of this study is to assess the efficacy and side effects of melatonin use in a population of children with neurodevelopmental disabilities who had sleep disorders. Methods: This is a cross-sectional study conducted in the pediatric neurology clinic at King Abdulaziz Medical City. A designed questionnaire was given to the parents to inquire about the sleep characteristics of their children before and after using melatonin. The patients' demographic data were collected and different parameters before and after starting melatonin were compared. Categorical variables were summarized and reported in terms of frequency and percent (n%). Continuous variables were reported in terms of mean and standard deviation. Results: A total of 23 patients were enrolled in our study, of which 15 (65.22%) were male. The mean age was 5.83 ± 3.07 years. For melatonin dose, 9 (39.13%) received 1 mg, 8 (34.78%) received 2 mg, and 6 (26.09%) received over 3 mg. Regarding melatonin duration of use, 7 (30.43%) received melatonin for 0 to 6 months, 7 (30.43%) received it for 7 to 12 months, and 9 (39.13%) received it for over a year. Significant differences were observed in time taken to fall asleep (P =0.046), the number of times the child woke up at night (P =0.071), total sleep time within 24 hours (P =.011), and time taken to wake up (P =.007), while no significant difference was observed in the number of naps taken during the daytime (P =.801). There were no major side effects reported. Conclusion: Melatonin had a significant impact on total sleep time and quality during the pre and post assessment of children with neurodevelopmental disabilities and sleep disorders.

Pediatric intracranial hypertension. Experience from 2 Tertiary Centers.

OBJECTIVE: To review the experience of 2 tertiary centers in Saudi Arabia with intracranial hypertension (IH) in the pediatric population. METHODS: We retrospectively reviewed and analyzed pediatric patients diagnosed with IH from June 2002 to May 2017 in 2 institutes. RESULTS: We identified 53 patients (30 females and 23 males) with a mean age of 7 years at the time of presentation. Among them, 41 patients were younger than 12 years, and 12 were older. Obese and overweight patients constituted 27.00% (n = 14) of all cases, 8 (66.7%) of whom were older than 12 years. The most common presenting feature was papilledema followed by headache. Vitamin D deficiency, which constituted the most common associated condition, was identified in 12 (22.6%) patients. Acetazolamide was the treatment option in 98.11% of patients, and only 5.7% underwent surgical interventions. The length of follow-up ranged from 6 months to 8 years. CONCLUSION: Intracranial hypertension is rare in children and commonly seen in overweight females older than 12 years similar to adults. Patients younger than 12 years tend to develop secondary IH. More studies are needed to characterize the clinical presentation and guide the management plan.

A novel chronic childhood sensory predominant neuropathy.

Chronic childhood neuropathies are predominantly hereditary in origin. Specific distinct clinical entities have been described, however, occasionally children with an unusual clinical phenotype are encountered in practice. We describe four children (3 males, 1 female) of Lebanese Moslem descent all sharing a common great-great-grandparent pairing with such a novel clinical spectrum. The three males were first cousins, each the product of a different parental consanguineous (i.e., parents second cousins) mating, whereas the female was a third cousin to each of the males whose parents were also second cousins. Each child presented early in life with developmental delay with associated hypotonia and areflexia. All had a sensorineural hearing loss documented and two of the patients were dysmorphic in facial appearance. Nerve conduction studies highlighted a sensory axonal neuropathy and sural nerve biopsy undertaken in two patients confirmed an axonal neuropathy. Detailed genetic and metabolic testing was negative. Followed into later childhood, each child continued to manifest motoric impairment, unsteadiness, areflexia, and cognitive disability. These children appear to provide evidence for a novel autosomal recessive inherited chronic predominantly sensory neuropathy that shares core clinical features with observed variability in associated symptoms.

Pediatric migraine equivalents: occurrence and clinical features in practice.

Migraine equivalents of infancy, childhood, and adolescence are recognized periodic, paroxysmal syndromes without associated headache that are thought to be migrainous in etiology. Five such equivalents are presently recognized. Their clinical features and relative frequency in ambulatory pediatric neurology practice have not been well documented. Utilizing a comprehensive, standardized computer database, the occurrence of these migraine equivalents in a single pediatric neurology practice together with their observed clinical features were documented over an 8-year period. Of a total of 5,848 patients in the database, of whom 1,106 were migraineurs, 108 patients (1.8% of total, 9.8% of migraineurs) were identified to have migraine equivalents. The following distribution among migraine equivalents was observed: benign paroxysmal torticollis 11 (10.2% of patients with migraine equivalents), benign paroxysmal vertigo 41 (38%), abdominal migraine/cyclical vomiting 20 (18.5%), acephalgic migraine 31 (28.7%), and acute confusional migraine 5 (4.6%). In each type, with the exception of benign paroxysmal torticollis and acute confusional migraine, females clearly predominated, and in all types a strong positive family history of migraine was elicited (68%-100%). There was variation in the age of onset of a particular equivalent with considerable overlap observed. Coexisting more typical migraines were observed in from 10% (benign paroxysmal torticollis) to 70% (abdominal migraines/cyclical vomiting) of the cases. In conclusion, pediatric migraine equivalents occur with relative frequency in ambulatory practice, possessing discrete clinical features that have a clear relationship to more typical migrainous phenomena.