Diagnosis and management of splenic injury following colonoscopy: algorithm and case series.

BACKGROUND: Splenic injury following colonoscopy is a rare yet life-threatening complication. These injuries are often associated with delayed diagnosis and may require invasive intervention. We sought to study the emergent presentation associated with splenic injury post-colonoscopy and to suggest a new treatment algorithm. METHODS: Six cases of splenic injury following colonoscopy were collected from three medical centers. Data regarding patient medical history, clinical presentation, laboratory and imaging findings and clinical management were recorded. A systematic PubMed/MEDLINE search was performed. Non-English-language publications and publications dating earlier than 2010 were excluded. An emergency department trauma-based management algorithm was designed according to the identified publications and review of the available trauma literature. RESULTS: The mean age was 65.3 years and the male-to-female ratio was 1:5. Five of the cases presented within 24 h of the colonoscopy complaining of severe abdominal pain. Hemodynamic instability was noted in four patients who presented with tachycardia (105-130), hypotension and/or a rapid drop in hemoglobin levels. All of the patients underwent initial resuscitation and a computerized abdominal tomography scan. Four of them required emergent splenectomy. No mortality or major morbidity was reported following the hospitalization. CONCLUSIONS: Although very rare, splenic injury during colonoscopy is an acute, severe and possible fatal complication. Patients may present with a rapid clinical deterioration and hemodynamic instability. Physicians should be familiar with the practical management of this surgical emergency and the treatment options available.

[Selective mutism].

Selective mutism is an uncommon disorder in young children, in which they selectively don't speak in certain social situations, while being capable of speaking easily in other social situations. Many etiologies were proposed for selective mutism including psychodynamic, behavioral and familial etc. A developmental etiology that includes insights from all the above is gaining support. Accordingly, mild language impairment in a child with an anxiety trait may be at the root of developing selective mutism. The behavior will be reinforced by an avoidant pattern in the family. Early treatment and followup for children with selective mutism is important. The treatment includes non-pharmacological therapy (psychodynamic, behavioral and familial) and pharmacologic therapy--mainly selective serotonin reuptake inhibitors (SSRI).

Multicenter long-term follow-up of children with idiopathic West syndrome: ACTH versus vigabatrin.

BACKGROUND AND PURPOSE: Long-term follow-up of children with idiopathic West syndrome (WS) treated with adrenocorticotropic hormone (ACTH) or vigabatrin. METHODS: Records of 28 normal magnetic resonance imaging (MRI) WS cases were reviewed for seizure development and cognitive outcome in relation to treatment type and lag. RESULTS: Average age at disease onset was 5.5 months, and average lag time to treatment was 25 days. Fourteen patients were treated with ACTH (eight early and six late), and 14 with vigabatrin (without delay). Response rates were 88% for ACTH and 80% for vigabatrin. Short-term outcomes for seizure cessation and electroencephalography normalization were identical between the groups. In the long-term, early ACTH treatment was better than the rest combined. Average follow-up time was 9 years. A normal cognitive outcome was achieved in 100% of the early-ACTH group, 67% of the late-ACTH group and 54% of the vigabatrin group (P = 0.03). Seizures subsequently developed in 54% of the vigabatrin group, in 33% of the late ACTH group, and 0% of the early ACTH group (P < 0.05). CONCLUSIONS: Idiopathic WS with normal MRI is associated with a good cognitive outcome. Early ACTH treatment, administered within 1 month, yields a better cognitive and seizure outcome than vigabatrin or late ACTH.

Random perturbation: a potential aid in treatment of children with cerebral palsy.

BACKGROUND AND PURPOSE: The motor behaviour of children with cerebral palsy (CP) can be viewed in terms of a stable mode with very little flexibility that prevents adaptation to tasks. We hypothesized that the use of random perturbations (RP) would weaken excessive stability, introduce flexibility and enhance the effects of physical treatment. The objective was to evaluate the contribution of RP to gross motor function and mechanical efficiency (MEg) during intensive physiotherapy in children with CP. METHODS: A convenience sample of 20 children with CP (mean age 8.2, range: 5.9-12.9 yrs) were matched by age and GMFCS level, and randomly assigned to structured intensive treatment (SIT) or to SIT + RP groups. Groups received one month of daily treatment. RP was applied by engine-induced random passive cycling for upper and lower limbs for up to 10 min in a 90-min treatment session. Gross Motor Function Measure (GMFM)-66 and gross mechanical efficiency (MEg) during stair climbing (MEg) were measured before and after treatment. RESULTS: GMFM-66 scores increased by about 1.0 in both groups. However, external work and MEg increased significantly more in SIT + RP than SIT. The increase in MEg in SIT + RP was independent of the level of motor function at baseline. CONCLUSION: The addition of RP in treatment of children with CP may have weakened previously established stereotypical motor patterns and introduced flexibility, thereby improving mechanical efficiency of a complex motor task. RP may enhance the effects of intensive treatment.

Hyperglycemia is associated with simultaneous alterations in electrical brain activity in youths with type 1 diabetes mellitus.

OBJECTIVE: To assess the association between hyperglycemia and electrical brain activity in type 1 diabetes mellitus (T1DM). METHODS: Nine youths with T1DM were monitored simultaneously and continuously by EEG and continuous glucose monitor system, for 40 h. EEG powers of 0.5-80 Hz frequency bands in all the different brain regions were analyzed according to interstitial glucose concentration (IGC) ranges of 4-11 mmol/l, 11-15.5 mmol/l and >15.5 mmol/l. Analysis of variance was used to examine the differences in EEG power of each frequency band between the subgroups of IGC. Analysis was performed separately during wakefulness and sleep, controlling for age, gender and HbA1c. RESULTS: Mean IGC was 11.49 ± 5.26 mmol/l in 1253 combined measurements. IGC>15.5 mmol/l compared to 4-11 mmol/l was associated during wakefulness with increased EEG power of low frequencies and with decreased EEG power of high frequencies. During sleep, it was associated with increased EEG power of low frequencies in all brain areas and of high frequencies in frontal and central areas. CONCLUSIONS: Asymptomatic transient hyperglycemia in youth with T1DM is associated with simultaneous alterations in electrical brain activity during wakefulness and sleep. SIGNIFICANCE: The clinical implications of immediate electrical brain alterations under hyperglycemia need to be studied and may lead to adaptations of management.

Severe periodic febrile myalgia in infancy due to carnitine palmitoyltransferase deficiency.

A 7 1/2-yr-old girl suffered, since early infancy, severe recurrent myalgia during periodic attacks of fever, vomiting and pharyngitis. Neither myoglobinuria nor exercise-induced muscle pain was present. She was found to have carnitine palmitoyltransferase deficiency (CPTD) in leukocytes, fibroblasts and muscle. This case exemplifies the importance of looking for an associated metabolic etiology of recurrent febrile myalgia even in the absence of myoglobinuria.

Renal and absorptive hypercalciuria: a metabolic disturbance with varying and interchanging modes of expression.

BACKGROUND: In previous studies, the oral calcium loading and deprivation test has been used to distinguish between children with renal (fasting) hypercalciuria (RH) and absorptive hypercalciuria (AH). OBJECTIVE: We evaluated the long-term clinical course of 30 children with idiopathic hypercalciuria and investigated the influence of urinary sodium excretion, as a reflection of its intake, on urinary calcium excretion. METHODS: Thirty normocalcemic, normophosphatemic children (21 boys and 9 girls) with urinary calcium to creatinine ratios greater than 0.57 mmol/L/mmol/L ( > 0.21 mg/dL/mg/dL on the three consecutive examinations participated in this study. They were divided according to their responses to calcium deprivation and loading into AH (16 patients) and RH (14 patients). RESULTS: When restudied 3 to 7 years later, 6 of the 16 children with AH were normocalciuric and three demonstrated characteristics compatible with RH. The remaining seven patients maintained their initial AH pattern. Of the 14 children with RH, four were normocalciuric and four demonstrated AH. The remaining six children maintained their initial RH pattern. A significant positive correlation was observed between urine sodium and calcium excretion in children with AH or RH. Children who were normocalciuric at the second study had significantly lower values of urine sodium excretion when compared with those in whom hypercalciuria persisted. CONCLUSIONS: We suggest that AH and RH constitute a continuum. The change in characteristics observed during the second study suggests that any attempt to divide these patients into two physiologically distinct subtypes may be artificial. The main factor influencing urinary excretion of calcium in our patients seemed to be sodium intake.

Inhibition of human red blood cell glutathione reductase by valproic acid.

Glutathione reductase (GR) one of the enzymes of the glutathione redox cycle, plays a salient role in maintaining appropriate cellular levels of reduced glutathione. The enzyme in human red blood cells is inhibited in vitro by the anticonvulsant drug valproic acid (VPA). The inhibition is dose-dependent, reversible, uncompetitive and does not depend on the redox state of the enzyme. VPA also inhibits red blood cell GR activity in children being treated with the drug. The level of serum VPA correlates significantly with the suppression of GR activity.

Gunshot wounds in brains of children: prognostic variables in mortality, course, and outcome.

A retrospective study of 51 children presenting with craniocerebral gunshot lesions was carried out to identify predictors of outcome. The patients ranged in age from 2 months to 17 years, with a mean of 14.5 years. The outcome was good in 20 patients, and seven and four were moderately and severely disabled, respectively. Twenty patients died. Statistical analysis showed prognostic significance of the admission Glasgow Coma Score (GCS), computerized tomographic findings of intraventricular hemorrhage and midline shift, and metabolic abnormalities, including hypokalemia and hyperglycemia. These prognostic factors may have implications regarding counseling of families, utilization of resources, and organ transplantation.

Hypertension and abdominal pain: uncommon presentation after exposure to a pine caterpillar.

UNLABELLED: The pine caterpillar Thaumatopoea wilkinsoni is found in pine woods all over Israel. Contact with its hair usually results in local reactions. Systemic reactions after contact with caterpillar hairs are known in other caterpillar species, but have been described only once after contact with T. wilkinsoni. We describe a group of adolescents who were exposed to T. wilkinsoni while camping in a pine wood. Three of them were referred to an emergency department. They had severe pruritus, pain and edema at the contact sites, with papular and urticarial rashes. Two of the patients had abdominal pain and one patient had hypertension for several hours. The hypertension resolved spontaneously. CONCLUSION: Skin eruptions are the most common manifestations of T. wilkinsoni contact, however, although systemic manifestations are rare, abdominal pain and hypertension may occur.

Asymmetric crying facies and associated congenital anomalies: prospective study and review of the literature.

Congenital asymmetric crying facies, a minor congenital anomaly due to absence or hypoplasia of the depressor anguli oris muscle on one side of the mouth, is associated at times with major congenital anomalies, most commonly in the cardiovascular system. In a prospective study of 5532 infants born at the Assaf Harofeh Medical Center, Israel, during 12 months (January to December 1998), 17 infants (an incidence of 0.31%) had asymmetric crying facies. One of the affected infants had ventricular septal defect and another infant had VATER (vertebral defects, imperforate anus, tracheoesophageal fistula, and radial and renal dysplasia) syndrome. No noxious obstetric perinatal factors could be identified. Family history was unremarkable in all cases. Diagnostic work-up performed in all of the affected infants failed to reveal an additional congenital malformation. Asymmetric crying facies is a minor isolated finding in most of the cases; however, a thorough search for other congenital malformations, especially of the cardiovascular system, should be performed.

Bone mineral density and turnover in children with attention-deficit hyperactivity disorder receiving methylphenidate.

Attention-deficit hyperactivity disorder (ADHD) is the most common behavior disorder among children; methylphenidate is a drug frequently prescribed for the control of its symptoms. One of the potential side effects of methylphenidate that concerns parents is its impact on the growth of children, since the mechanism by which methylphenidate might influence growth is not known. As linear growth is associated with an increase in bone mineral density and turnover, this study was undertaken to evaluate bone mineral density by dual photon absorptiometry and bone turnover by measuring serum bone-specific alkaline phosphatase and the urinary deoxypyridinoline excretion rate in children treated with methylphenidate for 1 to 2 years as compared to a control group. There were no significant differences in bone mineral density at either the lumbar spine or femoral neck in the study group (0.662 +/- 0.04 and 0.735 +/- 0.07 g/cm2, respectively) as compared to the controls (0.675 +/- 0.05 g/cm2 and 0.734 +/- 0.07 g/cm2, respectively). Furthermore, there were no significant differences in serum bone-specific alkaline phosphatase in the study group (58 +/- 22 U/L) as compared to the control children (71 +/- 34 U/L) or in urinary deoxypyridinoline in the study group (34 +/- 38 nM/mM), as compared to the control group (27 +/- 12 nM/mM). In conclusion, our data do not support a significant effect of methylphenidate on bone mineral density turnover in children when used for 1 to 2 years.

Heliox use in the treatment of acute hyperammonemia.

The objective of this study was to evaluate a new method for the treatment of acute hyperammonemia with a helium-oxygen mixture (heliox). We conducted a prospective, randomized, controlled study of male Sprague-Dawley rats. Experimental hyperammonemia was induced by 7 days of a high-ammonia diet. Subsequently, the animals were randomly divided into two groups: the study group treated with heliox breathing for 24 hours and a control group breathing room air for 24 hours. A prospective, randomized, controlled laboratory animal study was conducted at an animal research facility. The baseline plasma ammonia level was 9.49 +/- 10.96 micromol/L. After 7 days of a high-ammonia diet, the plasma ammonia level rose to 31.53 +/- 8.86 micromol/L. There was a significant statistical difference between the plasma ammonia level following 24 hours of heliox therapy (23.14 +/- 13.97 micromol/L) and the ammonia level in the control group (42.31 +/- 24.25 micromol/L) (P < .05). Heliox breathing was found to be an efficient treatment modality for decreasing plasma ammonia levels in an animal model. Further studies are required to evaluate its potential application in the treatment of patients with hyperammonemia.

Nocturnal acute laryngospasm in children: a possible epileptic phenomenon.

Respiratory difficulties are not uncommon during epileptic activity in all age groups. Laryngospasm, as an isolated manifestation of epileptic disorder, is a rare phenomenon described previously in only two patients. We report our experience with five children in whom nocturnal laryngospasm was the only clinical manifestation of their epileptic disorder. All children underwent extensive workup and the diagnosis was made by sleep-deprived electroencephalography (two cases) and sleep study (three cases). All patients were treated with carbamezapine with prompt resolution of their laryngospasm.

Abnormal visual evoked potentials in children with "Alice in Wonderland" syndrome due to infectious mononucleosis.

Visual illusions characterized by distortion of form, size, reciprocal position of objects, movement, or color, labeled as "Alice in Wonderland" syndrome, were discussed in children with infectious mononucleosis, as well as in other clinical conditions, such as migraine, epilepsy, use of certain hallucinogenic drugs, etc. The purpose of our study was to investigate for the first time visual evoked potential results in children with "Alice in Wonderland" syndrome associated with infectious mononucleosis. Five children with "Alice in Wonderland" syndrome associated with infectious mononucleosis underwent visual evoked potential studies during and after their clinical symptoms. Visual evoked potential results during the disease demonstrated statistically significant high amplitudes of P100-N145 in all children compared to the control group. A few weeks later, repeated studies after the resolution of the complaints were normal. Since the same findings can be observed in patients with migraine, we postulate that a common pathophysiologic underlying abnormality, which can cause transient focal decreased cerebral perfusion, could be involved in the disease process of these two conditions.

Mild form of acute herpes simplex encephalitis in childhood.

We describe two patients, aged 3.5 years and 15 years, with a mild form of herpes simplex encephalitis (HSE). The disease was characterized by convulsions and lymphocytic pleocytosis in the cerebrospinal fluid (CSF). Involvement of herpes simplex virus (HSV) was established by antibody measurements in serum and CSF. Recovery was complete with no antiviral drug administration. It appears that scrutinized serological work-up would widen our concept of mild forms of HSE, with a better prognosis and complete recovery.

Pseudotumor cerebri as a presenting symptom of acute sinusitis in a child.

Pseudotumor cerebri is a clinical syndrome characterized by increased intracranial pressure in the absence of an intracranial tumor. It is most frequently diagnosed in obese young women, but it is also reported in children of all age groups, including infants. A variety of medical conditions have been suggested as possible etiologic factors, including several infectious diseases. This study presents a child with pseudotumor cerebri as the only presenting symptom of acute frontal sinusitis. The possible association between these two conditions should be investigated in cases of pseudotumor cerebri to enable the appropriate treatment.

Type IV 3-methylglutaconic (3-MGC) aciduria: a new case presenting with hepatic dysfunction.

We describe a new patient with type IV 3-methylglutaconic aciduria who presented with a clinical picture simulating a primary hepatic disorder subsequently followed with progressive neurologic impairment and an magnetic resonance imaging picture of Leigh syndrome.

Interleukin-1beta levels in serum and cerebrospinal fluid of children with febrile seizures.

We compared interleukin-1beta (IL-1beta) levels in peripheral blood and cerebrospinal fluid (CSF) of children with febrile seizures with those of children with febrile illnesses without seizures (control). Twenty patients were included in the study, 10 with febrile seizures and 10 with febrile illness not complicated by seizures (control). Blood and CSF were obtained after the patients' admission to the hospital. IL-1beta levels were measured by a specific radioimmunoassay method. No significant differences were observed between mean IL-1beta levels in CSF (4.15 +/- 1.5 pg/ml) and blood (2.92 +/- 2.6 pg/ml) of the patients with febrile seizures group as compared with those in CSF (3.2 +/- 3.2 pg/ml) and blood (3.44 +/- 3.16 pg/ml) in the control group. These results do not support the hypothesis that increased production of IL-1beta or increased diffusion of IL-1beta through the blood-brain barrier is involved in the pathogenesis of febrile seizures in children.

Parkinsonian syndrome complicating systemic lupus erythematosus.

Two girls with florid extrapyramidal parkinsonism complicating systemic lupus erythematosus (SLE) are reported. One patient (15 years old) presented with extreme rigidity, irritability, and mutism initially diagnosed as acute psychosis. Examination revealed severe extrapyramidal akinetic mutism, along with marked restlessness. CT and MRI imaging of the brain were unremarkable. EEG revealed moderate generalized disturbance of background activity. 99mTc-HmPAO SPECT cerebral scanning detected decreased regional cerebral blood flow at the basal ganglia. Dopamine-agonist drugs led to complete recovery after 3 months, along with normalization of EEG and SPECT alterations. The second patient (16 years old) was assessed for progressive bradykinesia and apathy impeding her active daily activities, and she was suspected to have developed depression. Neurologic assessment revealed a parkinsonian syndrome that was less severe than that of the first patient. The EEG showed mild disturbance of background activity, and 99mTc-HmPAO SPECT demonstrated impaired regional cerebral blood flow over the basal ganglia. A parkinsonian extrapyramidal syndrome complicating SLE should therefore be taken into account in any patient with SLE presenting with marked behavioral alterations, rigidity, or akinetic mutism.