Personality Disorders, Risky Behaviors, and Adversity: The Moderating Role of Resilience.

Individuals with personality disorders (PDs) comprise 30% to 40% of individuals receiving mental health treatment. Treatment of PDs is exceedingly difficult; therefore, research has focused on PD etiology and preventative factors. One known influence on PD etiology is adverse childhood experiences (ACEs). ACEs are associated with increased prevalence of several health risk behaviors (HRBs), including engagement in substance use, criminal, and risky sexual behavior. One protective factor, childhood resiliency, predicts lower prevalence of PDs and engagement in HRBs. We hypothesized that increased prevalence of ACEs would predict higher levels of PD symptoms and HRBs engagement. Furthermore, we predicted that childhood resiliency would moderate the relation between ACEs, PD symptoms, and HRBs. In the present study, students (N = 531) completed the Adverse Childhood Experiences survey, the Self-Administered - Standardized Assessment of Personality Abbreviated Scale, and the Childhood Youth and Resiliency Measure-28. They also responded to questions about substance use, criminal, and sexual behavior which were used to define a latent HRB variable. Structural equation modeling was conducted to examine study hypotheses. As hypothesized, we found positive relations between ACEs, PD symptoms, and our HRB latent variable. Childhood resiliency moderated both ACE and PD symptom paths. At lower levels of reported ACEs, individuals high in childhood resiliency reported fewer PD symptoms and HRBs than individuals low in childhood resiliency. At high levels of ACEs, childhood resiliency did not serve as a protective factor. Additionally, study findings suggest that childhood resiliency factors are integral for protecting against the development of these disorders.

[Complement factor I deficiency revealed by repeated systemic Streptococcus pneumoniae infection]. / Déficit homozygote en facteur I du complément révélé par une infection systémique répétée à Streptococcus pneumoniae.

Recurrent and serious otitis media, and 2 Streptococcus pneumoniae bacteraemia episodes evoked an immune system deficiency in a 6-year-old girl. Upon investigation of the complement system, CH50 activity was moderately reduced and C4 antigen level was normal contrasting with low C3 antigen level. Factor 1 was undetectable. Factor I deficiency is rare, and its diagnosis has important practical consequences. Thanks to preventive antibiotic therapy with penicillin V and vaccinations against Neisseria meningitidis and S. pneumoniae, life expectancy and quality of this child can be significantly improved.

[Ghosal haematodiaphyseal dysplasia: a new case]. / Un nouveau cas de dysplasie hématodiaphysaire de Ghosal.

Ghosal-type hematodiaphyseal dysplasia has been first described in 1986, as a steroid-dependent anemia with endosteal broadening of the long bone's diaphyses and metaphaphyses, which makes a distinction with the periosteal reaction in Camuratti-Engelmann's disease and with Caffey's disease. Extreme pallor is first noticed and leads to search for palpable thick long bones that are not always clinically obvious. The transmission of this rare entity seems to be autosomal recessive, with a common racial background from the Middle East and the Indian subcontinent. Anemia is considerably improved by steroids, which allow to avoid blood cells transfusions, but a minimum maintenance dose is necessary to keep the hemoglobin at a normal level probably during the whole life, involving iatrogen complications. The mechanism of anemia and the genetic cause of the disease are yet unknown.

Extensive phenotypic analysis of a family with growth hormone (GH) deficiency caused by a mutation in the GH-releasing hormone receptor gene.

GH secretion and release are complex phenomena depending on activation of several genes, including those encoding GH, GHRH, and its receptor (GHRH-R). The GH gene, which is the most extensively analyzed sequence in patients with familial GH deficiency (GHD), represents the main known target of mutations. To test the involvement of the GHRH-R gene in this disease phenotype, we investigated one candidate Tamoulean family originating from Sri Lanka. Two brothers, with extremely short stature (< -4 SD) and no dysmorphy, were diagnosed as having complete GHD, unresponsive to exogenous GHRH and associated with PRL levels within the lower normal range. Magnetic resonance imaging examination showed anterior pituitary hypoplasia with a normal pituitary stalk. Both patients increased their growth rate while under GH therapy. Molecular investigations revealed a homozygous GHRH-R gene mutation that introduces a stop codon at residue 72. This mutation, which predicts a severely truncated receptor lacking the seven membrane spanning domains, is identical to that recently reported in one Indian Moslem family, raising the possibility of a founder effect. There was no clear evidence for height reduction in the three heterozygous individuals studied. This observation, which underlines the phenotypic criteria associated with a loss of GHRH-R function, raises the question of the frequency of GHRH-R abnormalities among GHD patients.

Congenital insensitivity to pain with anhydrosis. Report of two unrelated cases.

Two unrelated female cases of congenital insensitivity to pain with anhydrosis are presented. The first case was born from consanguineous parents. In both cases, onset of manifestation was observed in infancy with automutilation and recurrent fever. Both were mentally retarded. They underwent a peripheral nerve biopsy respectively at 3 and 33 years. A dramatic loss of unmyelinated fibers was observed in both cases. Myelinated fibers were also moderately reduced in number, especially those of smallest diameter; this loss was more marked in the second patient who was adult when the peripheral nerve was studied. Clusters of regenerating myelinated fibers were seen in both cases. Such histological observations might suggest a slowly progressive disorder. The cases are discussed together with previous reports dealing with congenital insensitivity to pain.

Human immunodeficiency virus-1 infection in neonates: correlation of plasma and cellular viremia and clinical outcome. French Pediatric Cohort Study Group.

Among human immunodeficiency virus-1 (HIV-1) vertically infected children, two patterns of disease progression have been observed: about 25% develop a severe immunodeficiency within the first 2 years of life; the rest experience a slower progression, like adults. We have assessed infectious viral burden in infected neonates through the French National Prospective Study. Plasma and cell-associated viremia were assayed by endpoint-dilution cultures in samples from 46 infants followed prospectively from birth. Plasma and cell-associated viral burden were found to be significantly higher in rapid progressing infants than in non-progressing infants in the first months of life: before the age of 2 months, between 2 and 4 months of age and by the age of 6 months. Moreover, among the non-progressing children, the infectious viral burden before the age of 4 months was predictive of the viral burden measured after the age of 12 months. In conclusion, this work demonstrates that infectious viral load is a reliable predictive marker for rapid progression to AIDS in infants and could be useful for initiating antiretroviral therapy.

[Arthropathies following the administration of pefloxacin to an adolescent with mucoviscidosis]. / Arthropathies consécutives à l'administration de péfloxacine chez un adolescent atteint de mucoviscidose.

We report the occurrence of knee joint effusion with prolonged functional impairment in a 17-year old boy with cystic fibrosis treated with pefloxacin for recurrent lower respiratory tract infections caused by Pseudomonas aeruginosa. Because the quinolone pefloxacin fairly often induces joint disease in pediatric age groups, we advocate restricting its use to those patients whose growth is completed. In growing individuals, it seems reasonable to use second generation quinolones only in infections caused by resistant organisms and to avoid exerting the joints during treatment.

A Weaver-like syndrome with endocrinological abnormalities in a boy and his mother.

A boy and his mother had dysmorphic features and accelerated growth of prenatal onset suggestive of the Weaver syndrome. Both had endocrinologic abnormalities. The boy had very low, hGH, which did not respond to stimulation. The mother had low, non-stimulate hGH hyperprolactinemia with secondary amenorrhea and galactorrhea. This is the first report of a mother to son transmission of the condition.

[Opsomyoclonus syndrome in children. A new case. Review of the literature (110 cases)]. / Syndrome opso-myoclonique de l'enfant. Nouvelle observation. Revue de la littérature (110 cas).

The authors report a new case of infantile myoclonic encephalopathy with opsoclonus and neuroblastoma in a 14 months-old infant. Some immunologic abnormalities were found at the initial course of the disease. The review of 110 cases of "dancing-eyes syndrome" permit them to specify clinical features and prognosis but cannot explain the pathogenesis of this rare disease.

[Salmonella panama responsible for meningitis secondary to febrile gastroenteritis in a 3-month-old infant]. / Salmonelle panama responsable d'une meningite secondaire à une gastro-entérite fébrile chez un nourrisson de trois mois.

The authors report a case of meningitis following enteritis due to Salmonella Panama (group D). The severity of Salmonella infection is especially correlated with hematogenous dissemination, through a transitory lack of intestinal immunity. The limited efficacy of antibiotics in salmonellosis is responsible for an ambiguous preventive attitude towards salmonella meningitis.

[Long-term results of palliative operations and complete cure of tetralogy of Fallot]. / Résultats à long terme des interventions palliatives et de la cure complète dans la tétralogie de Fallot.

714 cases of Fallot's tetralogy have been encountered over the last thirty years. A closed heart operation was undertaken in 472 patients. The mean age at operation was 5,1 years. In 87% of cases, a Blalock-Taussig anastomosis was carried out. The operative mortality was of the order of 3%. The operative risk was almost three times as large before the age of two years as after. The patients benefit by a demonstrable improvement of function after this operation, but it does not protect them from the complications of the condition (cervica abscess, Osler's disease, cerebro-vascular accidents) or from death. Open heart surgery was carried out in 403 patients. 161 as a primary procedure, and 242 after an initial palliative operation. The mean age at operation was 13.3 years. The operative mortality was 15%. The functional improvement was striking, and the patients appeared to be protected from the usual sequelae of the condition. On the other hand they cannot always be considered as cured: 16 died some time after the operation; 37% had pulmonary incompetence; 15% had a residual BSD; 8% had a block of both bundles, and 3% had a definitive atrio-ventricular block.