Hypertriglyceridaemia in adolescents may have serious complications.

Acute pancreatitis is an often-overlooked cause of acute abdominal pain in children and adolescents. Severe hypertriglyceridaemia is an important cause of recurrent acute pancreatitis. Monogenic causes of hypertriglyceridaemia, such as familial chylomicronaemia caused by lipoprotein lipase deficiency, are more frequently encountered in children and adolescents, but remain rare. Polygenic hypertriglyceridaemia is more common, but may require a precipitant before manifesting. With the global increase in obesity and type 2 diabetes, secondary causes of hypertriglyceridaemia in children and adolescents are increasing. We report two cases of severe hypertriglyceridaemia and pancreatitis in adolescent females. Hypertriglyceridaemia improved markedly with restriction of dietary fat. An inhibitor to lipoprotein lipase was found to be the cause in one patient, while in the other limited genetic investigation excluded chylomicronaemia owing to deficiency of lipoprotein lipase, its activators and processing proteins.

Asynchronous pentobarbital-induced burst suppression with corpus callosum hemorrhage.

OBJECTIVE: We describe the electroencephalographic (EEG) findings in a 9-year-old girl, who presented with generalized tonic-clonic status epilepticus requiring pentobarbital anesthesia, and correlate these findings with clinicoradiologic evidence of a ruptured AVM with hemorrhage into the body of the corpus callosum. METHODS: EEG analysis accompanied by clinical assessment, CT and MRI scans, and cerebral angiography were performed. RESULTS: With pentobarbital coma, the EEG showed burst suppression with prominent interhemispheric asynchrony. Suppression epochs >2 s in duration and with amplitude <20 microV in all channels were identified. In 12 min of the EEG analyzed, 6 unilateral and 20 bilateral epochs occurred. Of the 20 bilateral suppression epochs, interhemispheric asynchrony of >1 s was noted at onset for 5 epochs and at offset for one. Chi-square analysis revealed an equal tendency for unilateral suppressions to occur over either hemisphere, and for suppression in one hemisphere to begin before the other. CONCLUSIONS: We conclude that the corpus callosum plays a critical role in interhemispheric synchronization of cortical neuronal electrical activity and propose that: (1) normally, the corpus callosum modulates interhemispheric synchronization of cortical inhibition; and (2) with corpus callosal disruption, cortical areas are 'released' from such synchronization.

On facts and conceptual systems: young children's integration of their understandings of germs and contagion.

Five studies argue against claims that preschoolers understand a biological germ theory of illness. In Studies 1-3, participants were read stories in which characters develop symptoms (e.g., a bellyache) caused by germs, poisons, or events (e.g., eating too much candy) and were asked whether another character could catch the symptoms from the first. Few children made judgments in terms of germs as part of an underlying causal process linking the origin of a symptom to its subsequent transmission. Some children may have reasoned simply that certain kinds of symptoms are likely to be contagious. Studies 4 and 5 undermined the claim that preschoolers understand germs to be uniquely biological causal agents. Young children did not attribute properties to germs as they did for animate beings or for plants. It is suggested that children undergo conceptual reorganization in constructing a Western adult understanding of germs.

Why dogs have puppies and cats have kittens: the role of birth in young children's understanding of biological origins.

Three studies examined young children's understanding of the biologically causal role of birth in determining animal properties and species kind identity. In Studies 1 and 2, 4- to 7-year-olds and adults were told stories in which a baby was born to an animal of one species (e.g., a horse) but was adopted and raised by an animal of another species (e.g., a cow). In Study 1, children were asked to judge which parent the baby would resemble on a set of physical properties and beliefs. The majority of children were unable to say that the baby would resemble the birth parent on physical properties but share the beliefs of the adoptive parent. These results indicate that children were not using domain-specific causal understandings to reason about the origins of these properties. In Study 2, however, when asked to explicitly predict the kind of the baby, even 5-year-olds were able to reliably judge that the baby would be of the same species kind as the birth parent rather than the adoptive parent. This result suggests that children do understand at some level that birth determines species kind. Study 3 examined further the extent to which knowledge about birth influenced children's inferences about properties. Five-year-olds were asked to judge whether a baby would share a set of physical and nonphysical properties with its mother or its father. The results showed that children who knew the factual information about where babies come from (i.e., inside mommies' tummies) were more likely to attribute the mother's properties to the baby than the father's, regardless of whether the properties were physical or nonphysical. But this finding was true only if the property of one of the parents was not inherently more desirable or true than that of the other parent. In sum, the results of these 3 studies indicate that knowledge of birth does play a role in children's inferential reasoning, even for 5-year-olds, but that that role is not domain-specific. The implications for children's understanding of biological inheritance are discussed.

Like father, like son: young children's understanding of how and why offspring resemble their parents.

4 studies investigated the broad claim that preschoolers understand biological inheritance. In Study 1, 4-7-year-old children were told a story in which a boy was born to one man and adopted by another. The biological father was described as having one set of features (e.g., green eyes) and the adoptive father as having another (e.g., brown eyes). Subjects were asked which man the boy would resemble when he grew up. Preschoolers showed little understanding that selective chains of processes mediate resemblance to parents. It was not until age 7 that children substantially associated the boy with his biological father on physical features and his adoptive father on beliefs. That is, it was not until age 7 that children demonstrated that they understood birth as part of a process selectively mediating the acquisition of physical traits and learning or nurturance as mediating the acquisition of beliefs. In Study 2, subjects were asked whether, as a boy grew up, various of his features could change. Children generally shared our adult intuitions, indicating that their failure in Study 1 was not due to their having a different sense of what features can change. Studies 3 and 4 replicated Study 1, with stories involving mothers instead of fathers and with lessened task demands. Taken together, the results of the 4 studies refute the claim that preschoolers understand biological inheritance. The findings are discussed in terms of whether children understand biology as an autonomous cognitive domain.

Assessment and psychotherapeutic intervention for an HIV-infected preschool child.

Pediatric acquired immunodeficiency syndrome (AIDS) is becoming more common. Moreover, human immunodeficiency virus (HIV) positive status in multiple family members is common and complicates disease management. Practitioners treating these children are often unaware of the effect of the virus on the child's psychological, cognitive, and emotional functioning. In addition, children with AIDS frequently come from families facing pressing social problems, including homelessness, poverty, and drug addiction. HIV-positive children thus confront such diverse issues as the deterioration of developmental skills, social ostracism, and the possibility of imminent death, placing them in a socioemotional crisis. This paper presents a comprehensive psychotherapeutic intervention for such children.

Intracranial EEG monitoring in Landau-Kleffner syndrome associated with left temporal lobe astrocytoma.

A 3 1/2-year-old boy developed partial complex seizures with right-sided motor activity, occasionally secondarily generalized at age 18 months. Initial EEG showed left-sided focal epileptiform discharges. Seizures became refractory to antiepileptic drugs (AEDs). At age 3 years, there was severe language deterioration consistent with Landau-Kleffner syndrome (LKS). At that time, an EEG showed almost continual generalized spikes and polyspikes, worse during sleep. Video-EEG showed generalized tonic seizures associated with generalized EEG ictal activity. Magnetic resonance imaging (MRI) showed a cystic and solid lesion of the left hippocampal and parahippocampal gyri without surrounding edema. Subdural strip electrodes under the left temporal and overlying the left frontal lobes demonstrated interictal spikes simultaneously in all recording contacts. Ictal EEG activity occurred in the temporal electrodes 0.2-1 s before appearing in the frontal electrodes. After left temporal lobectomy with subtotal resection of a low-grade astrocytoma, he had an immediate marked decrease in seizures. In 1 month he was seizure-free, and in 6 months had no further seizures and markedly improved language. These findings provide further evidence that left temporal structural epileptogenic lesions may contribute significantly to the pathophysiology of LKS.

Aphasia as the sole manifestation of simple partial status epilepticus.

Aphasia due to simple partial status epilepticus is rare, particularly in the absence of a seizure history. No previous report describes acute aphasia as the sole clinical manifestation of EEG-monitored status epilepticus, with prompt resolution with treatment. We report a 45-year-old man with a left temporal glioblastoma who acutely developed a global aphasia, during which an EEG revealed continual repetitive sharp waves emanating from the left hemisphere. After injection of i.v. diazepam, the EEG seizure activity ceased, and the patient's language output returned to preseizure levels.

A diffuse infiltrative CD8 lymphocytosis syndrome in human immunodeficiency virus (HIV) infection: a host immune response associated with HLA-DR5.

STUDY OBJECTIVE: To describe the clinical, immunologic, and immunogenetic features of a diffuse infiltrative lymphocytic disorder resembling Sjögren syndrome in persons infected with human immunodeficiency virus (HIV). DESIGN: Clinical case study. SETTING: University-affiliated hospitals and outpatient clinics. PATIENTS: Consecutive sample of 17 patients. MEASUREMENTS AND MAIN RESULTS: All of the 17 patients had bilateral parotid gland enlargement; 14 had xerostomia and 6 had xerophthalmia. Of the 17 patients, 14 had generalized lymphadenopathy, 10 had histologically proved lymphocytic interstitial pneumonitis, 4 had neurologic involvement, and 3 had lymphocytic infiltration of the gastrointestinal tract. Gallium scanning in all of 11 tested patients showed abnormal salivary gland uptake. Minor salivary gland biopsies showed more than 2 lymphocytic foci per 4 mm2 tissue in all of 11 tested patients, the infiltrate consisting predominantly of CD8 cells. Fifteen patients had circulating CD8 lymphocytosis; the principal phenotype of these cells was CD8+ CD29+. Rheumatoid factor and antinuclear antibodies were infrequent, and none of the patients had anti-Ro/SS-A or anti-La/SS-B antibodies. HLA-DR5 was significantly more frequent in the black patients (10 of 12) compared with controls (13 of 45). Only one patient developed an opportunistic infection during 544 patient-months of study, and none has died of AIDS. CONCLUSIONS: A distinct syndrome primarily characterized by parotid gland enlargement, sicca symptoms, and pulmonary involvement occurs in HIV infection. This disorder is associated with CD8 lymphocytosis and the presence of HLA-DR5, and appears to be a genetically determined host immune response to HIV.

An abused psychotic preadolescent at risk for Huntington's disease.

An abused 10-year-old girl with a family history of Huntington's disease developed incapacitating abdominal pain with concomitant behavioral symptomatology suggestive of dementia. The pseudoneurologic nature of her symptoms was clarified through exhaustive evaluation and did not appear to be that of early-onset Huntington's disease. Assessment included pediatric, psychiatric, neurologic, and gynecologic examination; extensive radiologic and laboratory tests; and chronobiology studies. Successful treatment necessitated the integration of numerous therapeutic modalities including dynamically oriented psychotherapy, psychopharmacologic intervention, physical therapy, behavior modification, and electroconvulsive therapy.

Progressive dyskinesia due to internal cerebral vein thrombosis.

A 9 1/2-year-old boy developed a progressive dyskinesia 7 years after an angiographically demonstrated internal cerebral vein thrombosis. CT revealed bilateral thalamic lesions, more severe on the side contralateral to the movement disorder, without striatal involvement. This is a rare example of survival after internal cerebral vein thrombosis in a child and demonstrates that movement disorders may be delayed consequences of childhood stroke.

White-centered retinal hemorrhages: a sign of intracranial hemorrhage.

White-centered retinal hemorrhages (so-called "Roth spots") were seen in an 11-year-old girl with spontaneous intraventricular and subarachnoid hemorrhage from a ruptured "berry" aneurysm without underlying systemic disorder.

Sleep state, apnea and bradycardia in pre-term infants.

Respiratory pattern and heart rate were studied in 28 pre-term infants and four full-term infants during the first weeks of life. Sleep state was identified by standard neurological and EEG criteria. Apneic spells lasting 10 seconds or more were most frequent in the least mature infants, and their frequency tended to diminish with increasing maturity. When these apneic attacks were related to sleep state they were found to occur more frequently during non-REM sleep in infants of 30 to 33 weeks gestation. This was the only statistically significant difference found. No over-all pattern relating sleep state to frequency of apnea could be detected for individual infants. It was also difficult to relate the occurrence of bradycardia to apnea in terms of sleep state or gestational age. It is concluded that no simple relationship between sleep state, apnea and bradycardia exists and that a complex interrelation between central and peripheral factors is involved in these events.

Anticonvulsivant-induced depression of clotting factors in children.

A few neonates born to mothers receiving anticonvulsant drugs during pregnancy have shown defects in vitamin K dependent clotting factors with or without clinical bleeding. Experimentally, phenytoin (diphenyl hydantoin, DPH) has induced clotting defects in cats and inhibited production of clotting factors in rat liver slices. Phenobarbital has produced similar but milder defects. Anticonvulsants have been observed to produce clotting defects in 9 children, 2 weeks to 8 years in age. Elevated levels of phenytoin or other anticonvulsants, or a combination of anticonvulsants were measured in the children. Six patients were on drug combination including two or more of the following: phenytoin, phenobarbital, primidone, carbamazepine, diazepam, ethosuximide. Clotting defects included: elevated prothrombin time, elevated partial thromboplastin time, diminished factors V, VII or X. All children had neurologic symptoms of anticonvulsant toxicity, but the only hematologic problems were oozing from venipuncture sites and increased bruising in 3. All patients were on normal diets and had normal liver function tests. By lowering the level of anticonvulsants, clotting factors returned toward normal. Elevated levels of anticonvulsants can potentially produce clotting defects in neonates and young children.