In childhood

Absence Epilepsies are a group of epileptic syndromes typically starting in childhood or adolescence and characterized by a sudden lack of attention and mild automatic movements for some seconds to minutes. Absence epilepsies are generalized, i.e. the whole neocortex shifts into a state of sleep-like oscillations. 

EDMD is another X-linked muscular dystrophy, clinically and genetically completely distinct from DMD and BMD. Affected boys usually have onset in childhood of contractures (especially involving the Achilles tendons, elbows, and spinal muscles), humeroperoneal muscle weakness, and cardiac conduction defects, which tend to be mostly a problem in adult life and may necessitate insertion of a pacemaker. The gene for EDMD is known to map to Xq28, but this localization is... 

In childhood and adult-onset forms of AM, more moderate glycogen storage and vacuolation of muscle are seen and not all fibers are affected. Although cardiomegaly is not apparent in childhood AMD, glycogen storage is detectable histologically in heart muscle. 

Glycogenosis type VI (liver myophosphorylase deficiency) gives rise to hepatomegaly and hypoglycemia in childhood. The enzyme involved is under separate genetic control from the muscle isoform and has been assigned to chromosome 14. 

Primary hyperkalemic periodic paralysis is usually first manifest in childhood. Attacks may last for a period of a few hours to several days, and the degree of muscle damage associated with the condition appears to increase with age and frequency of attacks. Vacuolation and dilatation of the SR is the most obvious form of damage, and it increases with age. 

Vosniadou, S., Brewer, W. F. (1992). Mental models of the earth A study of conceptual change in childhood. Cognitive Psychology, 24(A), 535-585. 

Beyer K, Chung D. Schulz G. Mishoe M. Niggemann B, Wahn U, Sampson HA The role of wheat omega-5 gliadin IgE antibodies as a diagnostic tool for wheat allergy in childhood. J Allergy Clin Immunol 2008 122 419-421. 

Mastocytosis is a disorder characterized by increased numbers of mast cells in the skin, bone marrow, gastrointestinal tract, Uver, spleen, and lymph nodes [9,10]. The prevalence is unknown the incidence has been roughly estimated to be 3-7 new patients per million per year [9]. Most cases are sporadic with only a limited number (50-100) of cases with mastocytosis reported to pass from generation to generation [11], Mastocytosis presents at any age, although most cases occur during the first 2 years of life (childhood-onset) or after puberty (adult-onset) [9]. Mastocytosis in childhood often is self-limited and involves only the skin, whereas the course in patients with adult-onset disease is normally chronic and includes systemic involvement. 

This benign autosomal recessive disorder consists of conjugated hyperbilirubinemia in childhood or during adult life. The hyperbilirubinemia is caused by mutations in the gene encoding MRP-2 (see above), the protein involved in the secretion of conjugated bilirubin into bile. The centrilobular hepatocytes contain an abnormal black pigment that may be derived from epinephrine. 

Cytomegalovirus (CMV) Enveloped, icosahedral particles 150nm in diameter CMV is generally acquired in childhood as a subclinical infection. About 50% of adults carry the virus in a dormant state in white blood cells. The virus can cause severe disease (pneumonia, hepatitis, encephalitis) in immunocompromised patients. Primary infections during pregnancy can induce serious congenital abnormalities in the fetus... 

The ideal of any vaccine is to provide life-long protection to the individual against disease. Immunological memory (Chapter 14) depends upon the survival of cloned populations of small B and T lymphocytes (memory cells). These small lymphocytes have a lifespan in the body of ca. 15-20 years. Thus, if the immune system is not boosted, either by natural exposure to the organism or by re-immunization, then immunity gained in childhood will be attenuated or lost completely by the age of 30. Those vaccines which provide only poor protection against disease have proportionately reduced time-spans of effectiveness. Yellow fever vaccination, which is highly effective, must therefore be repeated at 10-year intervals, whilst typhoid vaccines are only effective for 1-3 years. Whether or not immunization in childhood is boosted at adolescence or in adult life depends on the relative risks associated with the infection as a function of age. 

Novak, Z., Nemeth, L, Gyurkovits, K., Varga, S.I. and Mat-kovics, B. (1991). Examination of the role of free radicals in bronchial asthma in childhood. Clin. Chim. Acta 201, 247-251. 

R. O., Plasma amino acids in childhood epileptic encephalopathies, Epilepsy Res., 34, 221, 1999. 

Asthma results from a complex interaction of genetic and environmental factors however, the underlying cause is not well understood. There appears to be an inheritable component, as the presence of asthma in a parent is a strong risk factor for the development of asthma in a child. This risk increases when a family history of atopy is also present.13 Approximately 50% of asthma can be attributed to atopy, and atopic asthma is more common in children than adults.3 Furthermore, atopy in childhood asthma is the strongest prognostic factor for continued asthma as an adult.1,3... 

B. Social/occupational dysfunction For a significant portion of the time since the onset of the disturbance, one or more major areas of functioning such as work, interpersonal relations, or self-care are markedly below the level achieved prior to onset (or when onset is in childhood or adolescence, failure to achieve the expected level of interpersonal, academic, or occupational achievement). 

Non-REM parasomnias have variable prevalence rates depending on patient age and different diagnoses. Sleep talking, brux-ism, sleepwalking, sleep terrors, and enuresis occur more frequently in childhood than in adulthood. Nightmares appear to occur with similar frequency in adults and children. REM behavior disorder (RBD), an REM-sleep parasomnia, has a reported prevalence of 0.5% and frequently is associated with concomitant neurologic conditions.16 Chronic RBD is more common in elderly men and may have a familial disposition. 

GH Research Society. Consensus guidelines for the diagnosis and treatment of growth hormone (GH) deficiency in childhood and adolescence summary statement of the GH Research Society. J Clin Endocrinol Metab 2000 85(ll) 3990-3993. 

Thrombotic thrombocytopenic purpura (TTP) is a severe systemic disorder characterized by the thrombi formation within the circulation that result in the platelet consumption and subsequent thrombocytopenia. The inherited sub-type is chronic and relapsing and generally occurs in childhood. Acute idiopathic TTP, which occurs in adults, is more common and harder to treat. The estimated annual incidence of TTP is 3.7 cases per million.33... 

The proportion of ALL in patients older than age 60 years constitutes between 16% and 31% of all adult leukemias. Treatment of adults largely has followed the conventional chemotherapeutic regimes used in childhood ALL. However, the intensification regimens common in childhood are not suitable for this population because of their associated toxic-ities in older patients. The adverse prognostic factor, the Philadelphia chromosome, occurs in 15% to 30% of adults and thus is more common in the over 60 age group.17 Based on the experience achieved in CML, the use of imatinib, a potent inhibitor of the Ph+-associated BCR-ABL tyrosine kinase, is becoming a common practice for these older adults. Results show that the combination of imatinib with conventional chemotherapy has improved remission rates compared with the use of conventional chemotherapy alone,... 

Goldstein DA, McGuigan MA, Ripley BD. 1988. Acute tricresylphosphate intoxication in childhood. Human Toxicol, 7 179-182. 

D. Hariparsad, N. Wilson, and C. Dixon, Oral tar-trazine challenge in childhood asthma Effect on bronchial reactivity, Clin. Allergy, 14, 81 (1984). 

Purine nucleoside phosphorylase (PNP) deficiency engenders a combined immunodeficiency and neurologic abnormalities and is usually fatal in childhood (G4). Patients with PNP deficiency have profound lymphopenia and a small thymus with poorly formed Hassall corpuscles. Lymphocyte enumeration shows markedly decreased numbers of T cells and T-cell subsets, with normal percentages of B cells. Point mutations and a splicing mutation have been identified in some PNP-deficient patients (H4). 

Hohman and Carothers had much in common. Bachelors of almost the same age, they were former Midwesterners who spoke German and loved classical music. Hohman, who coined the term depression, believed that many adult problems originated in childhood. 

An analysis of eight cross-sectional and/or prospective studies which reported tooth lead and PbB levels of the same children found considerable consistency among the studies (Rabinowitz 1995). The mean tooth lead levels ranged from under 3 to over 12 pg/g. In a study of 63 subjects, dentin lead was found to be predictive of concentrations of lead in the tibia, patella, and mean bone lead 13 years after tooth lead assessment in half of them (Kim et al. 1996b). The authors estimated that a 10 pg/g increase in dentin lead levels in childhood was predictive of a 1 pg/g increase in tibia lead levels, a 5 pg/g in patella lead levels and a 3 pg/g increase in mean bone lead among the young adults. 

Children s Susceptibility. Many of the known health effects that have been associated with low level lead exposure have been detected in children who experienced lead exposures both in utero and postnatally. Considerable uncertainty remains about the relative contribution of in utero and postnatal exposures to the development of health outcomes that are expressed later in childhood. This information is important for distinguishing those health outcomes that might be mitigated during the post-natal period from those that must be mitigated by limiting in utero exposure. Considerable uncertainty also remains... 

Chisolm JJ Jr. 1968. The use of chelating agents in the treatment of acute and chronic lead intoxication in childhood. J Pediatr 73 1-38. 

Gersberg RM, Gaynor K, Tenczar D, et al. 1997. Quantitative modeling of lead exposure from glazed ceramic pottery in childhood lead poisoning cases. International Journal of Environmental Health Research 7(3) 193-202. 

Kim R, Hu H, Rotnitzky A, et al. 1996b. Longitudinal relationship between dentin lead levels in childhood and bone lead levels in young adulthood. Arch Environ Health 51(5) 375-382. 

Lyngbye T, Hansen O, Grandjean P, et al. 1988b. Traffic as a source of lead exposure in childhood. Sci Total Environ 71 461-467. 

Needleman HL, Schell A, Bellinger D. et al. 1990. The long-term effects of exposure to low doses of lead in childhood. An 11-year follow-up report. N Engl J Med 322 83-88. 

O Flaherty EJ. 1995a. Physiologically based models for bone-seeking elements. V. Lead absorbtion and disposition in childhood. Toxicol Appl Pharmacol 131 297-308. 

Ong CN, Phoon WO, Law HY, et al. 1985. Concentrations of lead in maternal blood, cord blood, and breast milk. Archives of Disease in Childhood 60 756-759. 

Sachs HK, Moel DI. 1989. Height and weight following lead poisoning in childhood. American Journal of Diseases and Children 143 820-822. 

Seto DSY, Freeman JM. 1964. Lead neuropathy in childhood. Am J Dis Child 107 337-342. 

Asher MI, Keil U, Anderson HR et al. International Study of asthma and allergies in childhood (ISAAC) rationale and methods. Eur Respir J 1995 8 483-491. 

The International Study of Asthma and Allergies in Childhood (ISAAC) Steering Committee. Worldwide variation in prevalence of symptoms of asthma, allergic rhinoconjunctivitis, and atopic eczema ISAAC. Lancet 1998 351 1225-1232. 

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