Major Clinical Criteria Research Articles

Blood cultures and bacteraemia in an Australian emergency department: Evaluating a predictive rule to guide collection and their clinical impact.

The objective of the present study is to determine whether a predictive rule could safely reduce the number of negative blood cultures collected in an Australian ED and to assess the clinical impact of positive results from blood cultures taken in the ED. All positive blood cultures taken in the ED at a single facility were retrospectively identified for the calendar year 2012. Clinically significant bacteraemia episodes were assessed against a predictive rule using major and minor clinical and laboratory criteria gathered from medical records and pathology databases, and compared with a randomly generated sample of ED patient episode with negative blood cultures. The ED and final diagnoses and blood culture impact on clinical management were also collected. The predictive rule has a high sensitivity (98.8%) and modest specificity (48.7%), and if applied stringently would have prevented almost half of all blood cultures in our ED but missed two positives. Blood cultures altered the clinical management of 94.3% bacteraemic patients, representing 3.4% of all ED patients with blood cultures performed. High discordance (54%) between ED diagnosis and discharge diagnosis of bacteraemic patients was noted. Bacteraemia detected in the ED alters subsequent patient management. The predictive rule can be safely applied in the ED to determine need for blood culture collection. Blood cultures should not be omitted in the ED based entirely on preliminary diagnosis given the high discordance seen between ED and discharge diagnosis.

Le concept d’irritabilité chronique sévère : une dimension clinique à prendre en compte chez l’enfant et l’adolescent

IntroductionFor a decade, the concept of irritability has known a renewed interest in infant and child psychopathology. Indeed, longitudinal follow-up studies clearly highlighted their predictive value — in the short, medium and long terms — of a broad field of behavioral disorders and emotion dysregulation. This dimensional and transnosographic approach of irritability, coupled with the latest neuroscience data, points out that irritability could be the equivalent of a psychopathological marker, covering both a neurobiological, cognitive and emotional component. It is a major challenge today to better understand the developmental sequence of severe chronic irritability and its predictive influence on the etiology of mental disorders from childhood to adulthood. MethodWe briefly review here the latest current data on this topic. ResultsThe important point is that chronic and non-episodic irritability in children, associated with strong emotional sensitivity to negative events and frequent access of anger, could have a predictive value for progression to anxiety disorder or severe mood disorder but not to bipolar disorder as it was believed until now. The risk of developing a bipolar disorder would be more frequently correlated with the notion of transient and episodic irritability in a context of previous family history of bipolar disorder. ConclusionFurther studies are expected to narrow the discriminative validity of this notion of severe irritability and confirm or not its relevance as a major clinical criterion of Severe Mood Disorders in children and adolescents introduced in the last version of DSM (DSM-5).

Improving the Diagnosis of Children with 22q11.2 Deletion Syndrome: A Single-center Experience from Serbia.

The incidence of the 22q11.2 microdeletion among children who have at least two out of five major clinical criteria for 22q11.2 deletion syndrome. Prospective study. University Childrens Hospital in Belgrade, Serbia between 2005 and 2014. 57 patients with clinical characteristics of 22q11.2 deletion syndrome. Standard G-banding cytogenetic analysis was performed in all children, and the 22q11.2 genomic region was examined using fluorescence in situ hybridization (FISH). For patients with no deletion detected by FISH, multiplex ligation-dependent probe amplification (MLPA) analysis was also done in order to detect cryptic deletions of this region and to analyze other genomic loci associated with phenotypes resembling the syndrome. A selected group of patients diagnosed to have 22q11.2 microdeletion by FISH underwent MLPA testing in order to characterize the size and position of deletion. The frequency of 22q11.2 microdeletion among children with at least two of the five major characteristics of 22q11.2 deletion syndrome (heart malformations, facial dysmorphism, T-cell immunodeficiency, palatal clefts and hypocalcemia/hypoparathyroidism). Typical 22q11.2 microdeletion was detected in 42.1% of patients; heart malformation were identified in all of them, facial dysmorphism in 79.2%, immunological problems in 63.6%, hypocalcemia in 62.5% and cleft palate in 8.3%. A higher detection rate compared to one-feature criterion is obtained when at least two major features of 22q11.2 deletion syndrome are taking into consideration. The criteria applied in this study could be considered by centers in low-income countries.

Alagille syndrome: clinical perspectives.

Alagille syndrome is an autosomal dominant, complex multisystem disorder characterized by the presence of three out of five major clinical criteria: cholestasis with bile duct paucity on liver biopsy, congenital cardiac defects (with particular involvement of the pulmonary arteries), posterior embryotoxon in the eye, characteristic facial features, and butterfly vertebrae. Renal and vascular abnormalities can also occur. Inter- and intrafamilial variabilities in the clinical manifestations are common. We reviewed the clinical features and management as well as the molecular basis of Alagille syndrome.

Ankle-brachial index predicts change over time in functional status in the San Diego Population Study

Peripheral artery disease (PAD) affects millions of people, both in the U.S. and worldwide. Even when asymptomatic, PAD and the ankle-brachial index (ABI), the major clinical diagnostic criterion for PAD, are associated with decreased functional status and quality of life, as well as mobility impairment. Whether the ABI or change in the ABI predicts decline in functional status over time has not been previously assessed in a population-based setting. Participants were 812 non-Hispanic white, African American, Hispanic, and Asian men and women from the San Diego Population Study (SDPS) who attended a baseline examination (1994-1998), and follow-up clinic examination approximately 11years later. The Medical Outcomes Study 36-Item Short Form (SF-36) was obtained at both the baseline and follow-up examinations, and the summary performance score (SPS) at the follow-up examination. Associations of the baseline ABI and clinically relevant change in the ABI (<-0.15 vs ≥-0.15) with change in SF-36 scores over time were assessed using growth curve models, a type of mixed model that accounts for within participant correlation of measurements over time, and using linear regression for SPS. Models were adjusted for baseline age, sex, race/ethnicity, body mass index, ever smoking, physical activity, hypertension, diabetes, and dyslipidemia. Mean± standard deviation (SD) for the baseline ABI was 1.11± 0.10, and 50.8± 9.0 for the baseline Physical Component Score (PCS), 50.1± 9.5 for the baseline Mental Component Score (MCS), and 11.2± 1.9 for the SPS at the follow-up examination. In fully adjusted models, each SD lower of the baseline ABI was significantly associated with an average decrease over time of 0.6 (95% confidence interval [CI],-1.1 to-0.1; P= .02) units on SF-36 PCS. Each SD lower of the baseline ABI was also significantly associated with an average decrease over time of 1.2 units (95% CI, -2.3 to-0.2; P= .02) on the SF-36 physical functioning subscale, and a decrease of 1.3 units (95% CI, -2.3 to-0.3; P=.01) on the SF-36 energy/vitality subscale in fully adjusted models. Baseline ABI was not significantly associated with change in the SF-36 MCS over time, or the SPS at the follow-up examination. Change in the ABI was not associated with SF-36 PCS, MCS, or the SPS. In this multiethnic population of healthy middle-aged community-living men and women, we showed that participants with a lower baseline ABI had declines in functional status over 11years. Findings suggest that small differences in the ABI, even within the normal range, may identify subclinical lower extremity PAD, which in turn may help to identify individuals at risk for declining functional status with age.

Stress and Psychological Factors in the Variable Clinical Phenotype of Hereditary Angioedema in Children: A Pilot Study

Hereditary angioedema due to C1 inhibitor (SERPING1) deficiency (C1-INH-HAE) is characterized by unpredictable and variable attacks of cutaneous and mucosal edema carrying a major impact on daily life. While stress and psychological factors are usually suggested as trigger factors of HAE, they have not been sufficiently investigated. Our aim is to study, for the first time in a group of pediatric patients, the role of psychological stress and emotion regulation competence in the variability of attacks of C1-INH-HAE. The study involved 12 children aged 6–14 with a diagnosis of C1-INH-HAE [a diagnosis of C1-INH-HAE must be based on presence of 1 major (1–3) clinical criterion and 1 laboratory criterion] as well as their parents in the following: (1) a qualitative analysis of parent interviews on disease variability and (2) a quantitative evaluation of the psychological profile, perceived stress levels, and emotion regulation competence of the young patients. In the interviews, 91% of parents believe emotion...

Abstract 02: American Heart Association (AHA)’s Life’s Simple 7 and Risk of Lower Extremity Peripheral Artery Disease (PAD): The Multi-Ethnic Study of Atherosclerosis (MESA)

Background: Approximately 8.5 million Americans are affected by lower extremity PAD, which is associated with a substantially greater risk of cardiovascular (CV) events, mortality and functional decline. In 2010, the AHA initiated new CV health metrics (Life’s Simple 7) to monitor the goal of significantly improving CV health by the year 2020. The extent to which Life’s Simple 7 may be associated with risk of PAD or change in the ankle brachial index (ABI), the major clinical diagnostic criterion for PAD, has not been established. Methods: MESA is a population-based prospective cohort of 6814 Caucasian, African-American, Hispanic and Chinese men and women from six US field centers. The baseline exam occurred in 2000-02. Life’s Simple 7 at baseline included AHA definitions of poor, intermediate and ideal health behaviors (diet, body mass index, smoking, physical activity) and health factors (blood pressure, glucose, cholesterol), and was modeled continuously on a 0-14 point scale, with a higher score indicating better CV health. The scale was also categorized into overall inadequate (0-7points), average (8-11) and optimum (12-14) CV health. Incident PAD was defined as an ABI≤0.90 at Exam 3 (2004-05) or Exam 5 (2010-12), removing participants both with ABI&gt;1.40 and prevalent PAD at baseline. Cox models were used for incident PAD analyses and change in the ABI over time was assessed using mixed models. All models were adjusted for age, sex and race/ethnicity. Results: The mean±SD Life’s Simple 7 score was 8.4±2.1, with 33.8% of participants classified as inadequate, 59.6% average, and 6.6% classified as having optimum CV health. Adjusted rates of PAD per 1000 person-years were 7.3 for inadequate, 3.2 for average and 1.1 for optimum CV health. Each point higher on the Life’s Simple 7 scale was associated with a 20% lower risk of incident PAD (95% CI (0.76-0.85); p&lt;0.001). Compared to inadequate CV health, participants with average and optimum health had a 55% lower risk (95% CI (0.37-0.56), p&lt;0.001) and an 85% lower risk (95% CI (0.06-0.38); p&lt;0.001) of incident PAD, respectively. Each point higher on the scale was associated with a higher average ABI over a median follow-up time of 9.4 years (0.006 (95% CI (0.005, 0.007), p&lt;0.001). Compared to those with inadequate health, participants with average and optimum health maintained a 0.021 (95% CI (0.016, 0.027, p&lt;0.001) and a 0.030 (95% CI (0.018, 0.041, p&lt;0.001) greater average ABI over the follow-up period, respectively. Conclusions: Maintaining average or optimum CV health results in a substantially reduced risk of incident PAD and also in maintaining a higher average ABI over time, even when accounting for age, sex and race/ethnicity. Encouraging improvement in health behaviors, and treatment to achieve and maintain better levels of CV health metrics, may contribute to decreasing the rate of PAD.

The vascular type of Ehlers–Danlos syndrome

Ehlers–Danlos syndrome (EDS) type IV (vascular type of EDS) is a rare inherited autosomal dominant connective-tissue disorder caused by a mutation in the procollagen III gene (the COL3A1 gene). Patients with this syndrome are prone to rupture of arteries and hollow body organs. Among all types of EDS, type IV involves ~5–10% of cases. Vascular complications may develop in any anatomical region; large and medium-sized arteries are affected most frequently. Typical complications include dissection of the vertebral and carotid arteries at the extra- and intracranial levels, carotid-cavernous fistulas, and aneurysms. The diagnosis is based on major and minor clinical criteria and can be confirmed by laboratory tests: by detecting a quantitative and qualitative disruption of type III collagen synthesis by fibroblast culture or identifying the mutation in the COL3A1 gene. Invasive diagnostic techniques and surgical intervention should be used in case of life-threatening complications. Today, there is no specific technique for treating EDS. Our findings demonstrate that vascular complications in patients with EDS type IV were reduced by using β-blocker celiprolol. Inhibitors of the renin–angiotensin system and the agents lowering the concentration of transforming growth factor-beta open up new prospects for conservative treatment of this pathology and improve the future outlook.

Validation of diagnostic criteria for genital tubercusosis in cases of infertility

Objective: Mycobacterial infections of the genital tract have been implicated in the etiology of female infertility. However it is almost impossible to get a tissue diagnosis due to inaccessibility of the genital organs involved. Hence a need was felt to put forward and validate certain clinical diagnostic criteria for Genital Tuberculosis in cases of infertility. Methods: Certain major and minor clinical criteria were selected and definitive diagnostic criteria to diagnose Genital tuberculosis was proposed. Those diagnosed as Genital Tuberculosis were exhibited six months of ATT. Results: 120 infertile couples were enrolled in the study. Out of 120 couples, 61(50.8%) were diagnosed as Genital Tuberculosis. Out of the 61 patients, 20(32.8%) conceived spontaneously while on ATT. Conclusion: The high conception rate in this study is most probably due to the fact that these are the patients who had early TB endometritis which carries a better prognosis as compared to those who have TB salpingitis who have a poor prognosis in terms of conception rate.

Small Bowel Polyposis Without Diagnosis

A 19 year old male presents with nausea and vomiting. He is treated for gastroenteritis but returns with intussusception and small bowel obstruction that is managed conservatively. Two months later the intussusception recurs with 30 pound weight loss and hematochezia. An exploratory laparotomy is performed and a segment of jejunum with carpeting polyps is resected. He denies a history of cognitive delay and mucocutaneous freckling. On physical exam he is well-developed with a head circumference of 62cm. He has normal abdominal, skin, genital and neurologic exams. Upper endoscopy shows H. pylori gastritis and a stricture at the surgical anastomosis which is dilated. A colonoscopy and capsule endoscopy reveal only lymphoid follicles in the terminal ileum. Pathology from the jejunal resection is significant for hamartomatous polyps containing gastric type tissue. The patient's clinical condition is most consistent with Cowden Syndrome/PTEN Hamartoma Syndrome. He undergoes genetic testing that reveals a variant of uncertain significance (VUS) in the phosphatase and tensin homolog (PTEN) gene. Cowden syndrome is a rare genetic condition that carries an increased risk for multiple cancers. Our patient had two major clinical criteria, macrocephaly (>97th percentile) and hamartomatous intestinal polyps. Approximately 85% of individuals with an operational diagnosis of Cowden syndrome have autosomal dominant mutations identified in the PTEN gene on chromosome 10, which acts as a negative regulator of cell cycle pathways including mTOR and PI3K-AKT. Mutations can be deleterious or a VUS. Our case is unique in that the polyps architecturally resembled Peutz-Jeghers Syndrome (PJS) with an arborizing lamina propria and a lack of significant inflammation or dysplasia. However, the polyps were almost entirely composed of gastric foveolar and pyloric type mucosa, which is highly atypical in small bowel PJS. Furthermore, there have been few reports of small bowel hamartomas associated with Cowden syndrome, and none involving the jejunum to our knowledge. The patient also did not have any classic mucocutaneous findings or family members with a similar phenotype. Up to 44% of CS/PTHS cases arise from de novo PTEN mutations. It is not yet known if his VUS impacts protein function and/or predisposes to increased cancer risk. It is also unclear if the H. pylori infection contributed to his polyposis, however polyp regression after antibiotic therapy is reported.Figure 1Figure 2Figure 3

Acute Liver Failure in an Adult, a Rare Complication of Alagille Syndrome: Case Report and Brief Review

Alagille syndrome (AS) is an autosomal-dominant, multisystem disorder affecting the liver, heart, eyes, skeleton, and face. The manifestations are predominantly pediatric. Diagnosis is based on findings of a paucity of bile ducts on liver biopsy combined with ≥3 of 5 major clinical criteria. Orthotopic liver transplantation (OLT) is the only option for treating patients who developed liver failure, portal hypertension, severe itching, and xanthomatosis. It is difficult to establish clear criteria for OLT; indications are controversial because of the wide variety of clinical symptoms and the multisystem involvement. Generally, AS-associated liver disease is never an acute illness. We report the case of a 28-year-old woman with AS who underwent urgent OLT for acute liver failure. At 24 months posttransplant, the patient is in good clinical condition and with normal hepatic and renal function.

Functional and Structural Alterations of Cardiac and Skeletal Muscle Mitochondria in Heart Failure Patients

The fundamental mechanisms involved in the genesis and progression of heart failure are not clearly understood. The present study was conducted to analyze the cardiac mitochondrial involvement in heart failure, the possible parallelism between cardiac and skeletal muscle and if there is a link between clinical symptoms and mitochondrial damage. Left ventricle and pectoral biopsies were obtained from patients with heart failure (n: 21) and patients with inter-auricular communication as the unique diagnosis for surgery (n: 6). Mitochondria were isolated from these tissues and studied through electron microscopy, spectrophotometry to measure the activity of respiratory complex III and immunohistochemistry to determine the presence of reactive oxygen species. More than 90% of cardiac and skeletal muscle mitochondria presented structural and functional alterations in relation to an increment in the reactive oxygen species production, even in patients without the presence of any clinical Framingham criteria. We demonstrated some parallelism between cardiac and skeletal muscle mitochondrial alterations in patients with heart failure and that these alterations begin before the major clinical Framingham criteria are installed, pointing to mitochondria as one of the possibly responsible factors for the evolution of cardiac disease.

Abstract SY02-01: Regulators of in situ to invasive breast carcinoma progression.

Abstract A major clinical diagnostic criterion that differentiates in situ from invasive breast carcinoma is the presence of a continuous myoepithelial cell layer and basement membrane. In pre-invasive lesions such as DCIS, a relatively intact myoepithelial cell layer and basement membrane are still observed, but we have found that the molecular profiles of DCIS-associated myoepithelial cells are distinct from those from normal breast. The mechanisms underlying the loss of myoepithelial cells during the in situ to invasive breast carcinoma transition are unknown. We hypothesized that the differentiation of breast epithelial progenitors into myoepithelial cells is progressively inhibited by signals from tumor epithelial cells and cells composing the microenvironment such as fibroblasts and leukocytes. As a consequence, the myoepithelial cell layer is progressively lost and eventually disappears resulting in progression to invasion. We have been testing this hypothesis by characterizing myoepithelial cells from normal human breast tissues from nulliparous and parous women, including BRCA1 and BRCA2 mutation carriers. To dissect the functional properties of different types of myoepithelial cells, we have been using the MCFDCIS xenograft model. Our results will not only improve the current understanding of the role of myoepithelial cells but also have the potential to provide new prognostic markers to predict risk of invasive progression in DCIS. Furthermore, our results may help identify new targets for chemoprevention aimed at decreasing the risk of invasive breast tumors. Citation Format: Kornelia Polyak. Regulators of in situ to invasive breast carcinoma progression. [abstract]. In: Proceedings of the 104th Annual Meeting of the American Association for Cancer Research; 2013 Apr 6-10; Washington, DC. Philadelphia (PA): AACR; Cancer Res 2013;73(8 Suppl):Abstract nr SY02-01. doi:10.1158/1538-7445.AM2013-SY02-01

Replacement of aprotinin by ɛ-aminocaproic acid in infants undergoing cardiac surgery: consequences for blood loss and outcome

Once aprotinin was no longer available for clinical use, ε-aminocaproic acid (EACA) and tranexamic acid became the only two options for antifibrinolytic therapy. We compared aprotinin and EACA with respect to their blood-sparing efficacy and other major clinical outcome criteria in infants undergoing cardiac surgery. We retrospectively analysed data from a large consecutive cohort of infants (n=227) aged 31-365 days undergoing primary cardiac surgery requiring cardiopulmonary bypass encompassing the transition from aprotinin to EACA (aprotinin n=88, EACA n=139); all other aspects including the medical team and departmental protocols remained unchanged. The primary outcome was postoperative blood loss measured as chest tube output (CTO). Secondary outcome parameters were transfusion requirements, reoperation due to bleeding, renal, vascular, and neurological complications, and in-hospital mortality. CTO was significantly higher in the EACA patients {aprotinin 18 (13-27) ml kg(-1) 24 h(-1), EACA 23 (15-37) ml kg(-1) 24 h(-1) [mean (inter-quartile range)], P=0.001}, but transfusion requirements and donor exposures were not significantly different. A sensitivity analysis strengthened our finding that the increased blood loss in the EACA group was attributable to lower efficacy of EACA. There were no significant differences in the other clinical outcome measures. CTO was lower in aprotinin-treated patients. Nonetheless, EACA remains a suitable substitute without measurable differences in other clinical outcome criteria.

Empirical versus preemptive antimycotic therapy in terms of outcome benefit

Novel treatment strategies for invasive candidiasis (IC) are constantly emerging. Nevertheless, difficulties in diagnosis pose a challenge on their reliability, efficacy and safety. We have previously developed and approbated in our ICU an algorithm for empirical antimycotic therapy, combining the most significant risk factors for IC with three major clinical criteria for persistent nonbacterial sepsis [1]. On the other hand, preemptive therapy, based on identification of mycotic antigens and/or anti-mycotic antibodies in serum, is regarded as more reliable, even though it is known for its low sensitivity. The aim of the current study was to compare and evaluate the possible outcome benefit of our protocol implementation versus detection of galactomanan in patient's serum as a trigger for antimycotic treatment initiation.

Sleep Abnormalities in Chronic Fatigue Syndrome/Myalgic Encephalomyelitis: A Review

Chronic fatigue syndrome/myalgic encephalomyelitis (CFS/ME) is a chronic, disabling illness that affects approximately 0.2% of the population. Non-restorative sleep despite sufficient or extended total sleep time is one of the major clinical diagnostic criteria; however, the underlying cause of this symptom is unknown. This review aims to provide a comprehensive overview of the literature examining sleep in CFS/ME and the issues surrounding the current research findings. Polysomnographic and other objective measures of sleep have observed few differences in sleep parameters between CFS/ME patients and healthy controls, although some discrepancies do exist. This lack of significant objective differences contrasts with the common subjective complaints of disturbed and unrefreshed sleep by CFS/ME patients. The emergence of new, more sensitive techniques that examine the microstructure of sleep are showing promise for detecting differences in sleep between patients and healthy individuals. There is preliminary evidence that alterations in sleep stage transitions and sleep instability, and other physiological mechanisms, such as heart rate variability and altered cortisol profiles, may be evident. Future research investigating the etiology of non-restorative sleep in CFS/ME may also help us to undercover the causes of non-restorative sleep and fatigue in other medical conditions.

Comprehensive molecular analysis demonstrates type V collagen mutations in over 90% of patients with classic EDS and allows to refine diagnostic criteria

Type V collagen mutations are associated with classic Ehlers-Danlos Syndrome (EDS), but it is unknown for which proportion they account and to what extent other genes are involved. We analyzed COL5A1 and COL5A2 in 126 patients with a diagnosis or suspicion of classic EDS. In 93 patients, a type V collagen defect was found, of which 73 were COL5A1 mutations, 13 were COL5A2 mutations and seven were COL5A1 null-alleles with mutation unknown. The majority of the 73 COL5A1 mutations generated a COL5A1 null-allele, whereas one-third were structural mutations, scattered throughout COL5A1. All COL5A2 mutations were structural mutations. Reduced availability of type V collagen appeared to be the major disease-causing mechanism, besides other intra- and extracellular contributing factors. All type V collagen defects were identified within a group of 102 patients fulfilling all major clinical Villefranche criteria, that is, skin hyperextensibility, dystrophic scarring and joint hypermobility. No COL5A1/COL5A2 mutation was detected in 24 patients who displayed skin and joint hyperextensibility but lacked dystrophic scarring. Overall, over 90% of patients fulfilling all major Villefranche criteria for classic EDS were shown to harbor a type V collagen defect, which indicates that this is the major--if not only--cause of classic EDS.

Botulinum toxin injections into salivary glands to decrease oral secretions in CHARGE syndrome: Prospective case study

CHARGE syndrome is a genetic disorder caused by a mutation in the CHD7 gene on chromosome 8. Major clinical diagnostic criteria for this heterogeneous disorder include ocular coloboma, choanal atresia/stenosis, characteristic external and internal ear abnormalities, and cranial nerve abnormalities. Patients with CHARGE syndrome often have dysphagia and are at high risk for aspiration of both upper and lower gastrointestinal secretions. The following case-report describes the use of Botulinum toxin A (Botox) to reduce excess salivary secretions in a ventilator dependant infant who would have required a tracheotomy. Thereafter, Botox was used regularly (4-5 months) to decrease the salivary secretions. This case-report is unique in that it describes the intermittent and prospective use of Botox to reduce excess salivary secretions and prevent the resulting aspiration-related complications in an infant with CHARGE syndrome.

The Factors Influencing Decision Making on Children’s Psychiatric Hospitalization: A Retrospective Chart Review

Indications and criteria for psychiatric hospitalization(PH) continue to be questioned and assessed. We investigated the major clinical criteria affecting child psychiatrists' decision to recommend hospitalization. Records of 80 children (aged 7–13 years) treated at the Geha Mental Health Center's (GMHC's) outpatient clinic were reviewed.The patients' files were divided into three groups: admitted to PH (n = 20), candidates for PH who, ultimately, were not hospitalized (n = 20) and 40 patients for whom PH was nota consideration. Three groups of criteria were chosen to evaluate the decision for PH (type and severity of the psychiatric disorders, family's level of distress and parental capacity to contain the child). These criteria were rated quantitatively by using CGI-S. Highly significant inverse association was found between the level of parental capacity to contain the child and the decision to hospitalize in a psychiatric pediatric institution. The correlations between severity of the psychiatric disorders and family's level of distress was also found significant, but to a lesser extent.Parental capacity to contain the child, with its various components, is a major factor in the decision making process,when referring children to PH. This is the first study,demonstrating a strong association between parental-capacity components and the rate of hospitalization.

Gorlin-Goltz syndrome

Gorlin-Goltz syndrome is an uncommon autosomal dominant inherited disorder, which is characterized by multiple odontogenic Keratocysts and basal cell carcinomas, skeletal, dental, ophthalmic, and neurological abnormalities, intracranial ectopic calcifications of the falx cerebri, and facial dysmorphism. Pathogenesis of the syndrome is attributed to abnormalities in the long arm of chromosome 9 (q22.3-q31) and loss or mutations of human patched gene (PTCH1 gene). Diagnosis is based upon established major and minor clinical and radiological criteria and ideally confirmed by deoxyribo nucleic acid analysis. We report a case of a 9-year-old girl presenting with three major and one minor feature of Gorlin-Goltz syndrome. Radiologic findings of the syndrome are easily identifiable on Orthopantomogram, chest X-ray, and Computed tomography scans. These investigations prompt an early verification of the disease, which is very important to prevent recurrence and better survival rates from the coexistent diseases.