Cyclic Vomiting Syndrome in Infants and Children: A Clinical Follow-Up Study.

BACKGROUND: Cyclic vomiting syndrome is characterized by recurrent vomiting that is associated with increased adrenocorticotropic hormone and antidiuretic hormone levels during cyclic vomiting syndrome attacks. However, both prognosis and treatment remain unclear. We therefore evaluated the clinical features, prognosis, and effectiveness of the prophylaxis of cyclic vomiting syndrome as well as the relationship between symptoms and adrenocorticotropic hormone/antidiuretic hormone levels. METHODS: We included 31 patients with cyclic vomiting syndrome who were admitted to Teikyo University between 1996 and 2008. All patients were diagnosed with cyclic vomiting syndrome based on the criteria of the second edition of the International Headache Classification. The patients (25 of 31) were followed until 2013. RESULTS: The median overall duration of the disorder was 66 (3-179) months. Follow-up was completed for 25 patients with cyclic vomiting syndrome, of whom 44% (n = 11) developed migraine. Valproic acid, valproic acid with phenobarbital, phenobarbital, and amitriptyline were effective in nine, four, three, and one patients, respectively. Abnormally high adrenocorticotropic hormone (n = 17) and antidiuretic hormone (n = 18) levels were found among the 25 patients for whom follow-up data were available. The following correlations were significant: attack duration and adrenocorticotropic hormone levels (correlation coefficient: 0.5153, P = 0.0084) and attack duration and antidiuretic hormone levels (correlation coefficient: 0.5666, P = 0.0031). Antidiuretic hormone levels in patients with bilious vomiting were higher than in those without bilious vomiting (P = 0.048). CONCLUSIONS: Most patients with cyclic vomiting syndrome recovered completely and benefited from prophylactic therapy, although half of them developed migraines.

First case report of suspected onset of convulsive seizures due to co-administration of valproic acid and tebipenem.

OBJECTIVE: A patient presented with convulsive seizures when sodium valproate (VPA) and tebipenem pivoxil (Orapenem) were co-administered accidentally. The seizures were suspected to be caused by a reduced concentration of VPA in the blood. CASE SUMMARY: A 6-year-old boy (weight: 16 kg, at the start of treatment) began sodium valproate (valproate syrup 5%) treatment for epilepsy in February 2012. At a dose of 350 mg/day, he experienced no convulsive seizures and maintained stable symptoms for the past 9 months. In December, he was prescribed 160 mg/day tebipenem pivoxil by an otolaryngologist for inflammation of the tympanic membrane. He experienced convulsive seizures the day after beginning co-administration. The concentration of VPA in his blood at this time was 30.0 µg/mL, which was lower than the optimal blood concentration. DISCUSSION: Marked reduction of VPA concentration in the blood due to co-administration of VPA and injectable carbapenem antibiotics has been well-documented; however, this is the first report of such an interaction with tebipenem, which is an orally-administered carbapenem antibiotic. Although the mechanism of drug interaction between VPA and carbapenem antibiotics is not fully understood, it is thought that VPA blood concentrations decrease due to production of valproic acid glucuronic acid conjugates (VPA-Gluc) being promoted directly or indirectly by carbapenem antibiotics. When we assessed the patient according to the DIPS system, we calculated a score of +4 (possibility of interaction). CONCLUSIONS: The results suggest that co-administration of oral carbapenem antibiotics and VPA should be avoided.

Hypothyroidism caused by iodine deficiency and iodine levels in enteral formulas.

BACKGROUND: A 4-year-old female patient was diagnosed with hypothyroidism caused by iodine deficiency. The patient's iodine levels in serum and urine were significantly low. The iodine concentration in the enteral formula was 1.6 µg/100 kcal as measured by inductively coupled plasma mass spectrometry. The patient's iodine intake while receiving the enteral formula was calculated to be 16 µg/day, which is much lower than the recommended dietary reference intake of 80 µg for children aged 3-5 years. The purpose of this study was to assess iodine concentrations in 20 enteral nutritional formulas available in Japan in order to assess whether low iodine concentration is a characteristic of one specific formula or whether it is a more prevalent problem. METHODS: Iodine concentrations in 20 popular nutritional formulas available in Japan, for which iodine content is not indicated in the ingredient list, were analyzed with inductively coupled plasma mass spectrometry. RESULTS: Iodine concentrations were less than 5 µg/100 kcal and less than 10 µg/100 kcal in 13 and 18 enteral nutritional formulas, respectively. CONCLUSIONS: Most of the enteral nutritional formulas analyzed had low iodine concentrations. These findings suggest that iodine deficiency is a likely outcome in patients who receive these formulas for a prolonged period.

Sumatriptan as a treatment for cyclic vomiting syndrome: a clinical trial.

BACKGROUND AND OBJECTIVE: Cyclic vomiting syndrome (CVS) is associated with migraine. This study aimed to evaluate the efficacy of sumatriptan in treating CVS. METHODS: Twelve patients were enrolled in this trial. Sumatriptan was administered either subcutaneously [(age x 4 + 20)/100 x 3 mg] or by nasal spray (NS; 20 mg). Response to the treatment was classified as complete, effective, or noneffective. RESULTS: Eleven patients, who presented with 35 attacks, were treated by subcutaneous injection of sumatriptan. The treatment was responsive in 19 attacks. The efficacy of sumatriptan was high in attacks that occurred in cases with a family history of migraine compared to those without (p = .0482). Five patients were treated with sumatriptan NS for six attacks. The treatment was completely responsive in two of six attacks. We observed no adverse effects associated with sumatriptan treatment in this trial. CONCLUSION: We conclude that sumatriptan has potential efficacy in treating of patients with CVS.

[Assessment of joint attention skill in at-risk infants during follow-up outpatient examination].

We assessed the joint attention skill of infants by a novel method. One hundred eighty infants who were discharged from the NICU of Teikyo University Hospital and subsequently brought to the outpatient clinic for follow-up examinations between 6 and 12 months of corrected age (297 examinations in total) were entered into the study. Infant were sitting on the mother's knees facing the examiner. After confirming the infant's visual axis to the examiner's eyes, the examiner looked at blocks held in the infant's visual field. When the infant looked at the blocks by perceiving the examiner's gaze, the infant was judged to have joint attention skill. Then, when the infant looked at the examiner again, the attitude was estimated as an action for reconfirmation. Sixty percent of the infants at 6 months of age showed joint attention skill, and more than 90 % of infants showed this skill at 9 months of age. At 6 months of age, boys showed that skill significantly more frequently than girls. This new method is very easy to perform, uses only small blocks and can be completed within a few minutes. Therefore, we considered this new method useful for the assessment of joint attention skill in infants during periodic outpatient health clinic examination.

Effective prophylactic therapy for cyclic vomiting syndrome in children using valproate.

This trial sought to evaluate our experience using the antimigraine prophylactic drug, use of valproate for the prophylactic management of cyclic vomiting syndrome (CVS) in children. Thirteen children diagnosed with severe CVS were enrolled. Prophylactic therapy consisted of valproate administered at a dose of 10-40 mg/kg/day. Upon enrollment in the study, all patients underwent diagnostic tests to rule out organic causes of their symptoms. Vomiting was severe enough in all patients to cause dehydration requiring hospitalization for intravenous rehydration. Nine of 13 patients did not respond to numerous previous medical therapies like propranolol, amitriptyline, cyproheptadine, phenobarbital, phenytoin, and carbamazepine. Three of 13 patients required combination therapy with valproate and phenobarbital. Of the 13 patients, two showed complete resolution of their symptoms, nine had marked improvement in their symptoms, as evidenced by infrequent attacks of reduced severity, and two failed to respond to valproate therapy. Four patients experienced relapse with a decreased dosage of valproate. Side effects associated with long-term valproate administration were not observed. Valproate appears to be effective for the prophylactic management of severe CVS, with 85% of all patients achieving at least a reduction in the frequency of attacks.

[The effect of prophylactic therapy with valproate sodium and phenobarbital in two patients with cyclic vomiting syndrome].

Cyclic vomiting syndrome (CVS) is a disorder characterized by recurrent, stereotypic episodes of incapacitating nausea, vomiting, and other symptoms, separated by intervals of comparative wellness. Associated symptoms include nausea, abdominal pain, headache, and motion sickness. Recently, CVS was categorized as a migraine. Case 1 was a girl aged 4 years and 11 months, who had frequent and severe episodes of vomiting since she was 3 years old. The diagnosis of CVS was established on the basis of clinical symptoms and laboratory data. Her electroencephalogram was normal. Prophylactic therapy using a single drug such as amitriptyline, carbamazepine, phenytoin, cyproheptadine, valproate sodium or phenobarbital was not effective. However, her recurring vomiting disappeared with prophylactic therapy using valproate sodium and phenobarbital. Case 2 was a boy aged 10 years and 7 months, who had frequent episodes of vomiting since he was 1 year and 10 months old. He had been receiving intravenous hyperalimentation therapy at home since infancy because of frequent vomiting and failure to thrive. His electroencephalogram showed no abnormality. Prophylactic therapy using a single drug such as diazepam, phenytoin, valproate sodium or phenobarbital was not effective. However, his recurring vomiting disappeared with prophylactic therapy using valproate sodium and phenobarbital. There were no adverse effects in both patients. The combination therapy with valproate sodium (20 - 26 mg/kg/day) and phenobarbital (4 - 5 mg/kg/day) was effective as a prophylactic therapy in these two patients. The combination therapy with valproate sodium and phanobarbital for prophylaxis of vomiting may be helpful in patients with intractable CVS.

[Clinical manifestations for early diagnosis of the patient with classical Menkes disease].

Early treatment reportedly prevents neurological disturbance in patients with Menkes disease. To promote early diagnosis, a questionnaire concerning symptoms during the neonatal period and at the age of 1 month was sent to the mothers of 14 patients with Menkes disease. The height, body weight and head circumference were almost normal in most of the Menkes patients. At the age of one month, all patients had abnormal hair. The mothers noticed it in 8 cases, and in the remaining 6 cases, the abnormalities were confirmed by the photos at the age of one month. At this age, the mothers noticed weak activity in 5, jaundice in 5, dyspnea in 4 cases, feeding disturbance in 3, hypothermia in 2 and eczema in 2. These results suggest that recognizing hair abnormalities at a one-month check-up may provide a clue to the early diagnosis of Menkes disease.