Aplasia Cutis Congenita of the Scalp with Bone Defect and Exposed Sagittal Sinus in Trisomy 13 Newborn - a Case Report.

Background: Aplasia cutis congenita is a heterogeneous disorders group with a rare reported incident of 0.5 to 1 in 10,000 births. ACC can be associated with physical defects or syndrome that may help in diagnosis, prognosis and further evaluation of the patient. Trisomy 13 is one of the most common fetal life limiting diagnosis which is associated with ACC of membranous type scalp. Objective: In this article, we report cases of aplasia cutis congenita of the scalp with dura and bone defect and exposed sagittal sinus in newborn diagnosed to have trisomy 13. It emphasizes the importance of ACC associated syndrome which is having high mortality prior to surgical intervention. Case presentation: The patient was born at 35 weeks of gestation. Her physical examination revealed a newborn girl with dysmorphic facial features including widely separated eyes, downward slanting of the palpebral fissure, microphthalmia, retrognathia, and low seat ears. She had area of loss of scalp skin and skull bone with seen brain tissue and sagittal sinus were exposed that was measure 6 by 5 cm in size. Additionally, she had a clenched fist and overlapping fingers and rocker bottom feet. Laboratory investigations include basic labs and the TORCH screen was negative. On the 9th day of life, a chromosomal analysis showed a female karyotype with three copies of chromosome number 13 in all 20 metaphase cells counts. Conclusion: The patient was managed conservatively. However, a multidisciplinary team agreed on do not resuscitate with no further surgical intervention as survival rate of trisomy 13 is poor.

Disparities in oesophageal cancer risk by age, sex, and nativity in Kuwait:1980-2019.

BACKGROUND: This cross-sectional cohort study assessed the inequalities in oesophageal carcinoma risk by age, sex and nativity in Kuwait: 1980-2019. METHODS: Using oesophageal cancer incidence data from the Kuwait National Cancer Registry, relevant Kuwaiti population data and World Standard Population as a reference, age-standardized incidence rates (ASIR) (per 100,000 person-years) overall and by subcohorts were computed. The incident oesophageal cancer cases count was overdispersed with excessive structural zeros, therefore, it was analyzed using multivariable zero-inflated negative binomial (ZINB) model. RESULTS: Overall ASIR of oesophageal cancer was 10.51 (95% CI:  6.62-14.41). The multivariable ZINB model showed that compared with the younger age category (< 30 years), the individuals in higher age groups showed a significant (p < 0.001) increasing tendency to develop the oesophageal cancer.  Furthermore, compared with the non-Kuwaiti residents, the Kuwaiti nationals were significantly (p < 0.001) more likely to develop oesophageal cancer during the study period. Moreover, compared with 1980-84 period, ASIRs steadily and significantly  (p < 0.005) declined in subsequent periods till 2015-19. CONCLUSIONS: A high incidence of oesophageal cancer was recorded in Kuwait, which consistently declined from 1980 to 2019. Older adults (aged ≥ 60 years) and, Kuwaiti nationals were at high risk of oesophageal cancer. Focused educational intervention may minimize oesophageal cancer incidence in high-risk groups in this and other similar settings. Future studies may contemplate to evaluate such an intervention.

Childhood leukaemia incidence and trends in a Middle Eastern country during 1980-2014: a population-based study.

BACKGROUND: This retrospective cohort study examines the trends in childhood leukaemia age-standardized incidence rates (ASIRs) (per million person-years) using cases which were diagnosed at age 0-19 years from 1980 to 2014 and recorded in the Kuwait Cancer Control Center (KCCC) registry. METHODS: Childhood leukaemia age-specific incidence rates overall and by sub-cohorts defined by age (0-4, 5-9, 10-14, and 15-19 years), sex (male, female) and nationality (Kuwaiti, non-Kuwaiti) were computed and age-standardized. Joinpoint regression models were used to evaluate trends in childhood leukaemia ASIRs. Average annual percent change (AAPC) and its 95% confidence interval (CI) were used to interpret the observed trends. RESULTS: During the study period, 1077 childhood leukaemia cases of 32.3 million person-years were diagnosed. From 1980 to 2014, the average annual childhood leukaemia ASIR was 53.1 (95% CI 20.9, 85.2). Overall childhood leukaemia ASIRs significantly decreased on average by 6.8% per year (AAPC = -6.8; 95% CI -12.1, -1.1; p = 0.02) from 1980 to 1993, but a marginally significant increase in ASIRs from 1993 to 2014 was recorded (AAPC = 2.5; 95% CI -0.5, 5.5; p = 0.10). During the entire period, childhood leukaemia ASIRs trends significantly (p < 0.05) increased among 6 of 16 sub-cohorts, which was more pronounced among females and 10-14-year-old children. CONCLUSIONS: Overall, ASIRs significantly increased from 1993 to 2014, which specifically seems to be driven by an increase in ASIRs among females and 10-14 -year-old children. These increasing trends underscore the potential involvement of a range of exposures. Future studies on unravelling such factors may help develop preventive measures to minimize childhood leukaemia risk in this and similar settings in the region.

Chronic urinary tract infection and bladder carcinoma risk: a meta-analysis of case-control and cohort studies.

OBJECTIVE: This meta-analysis of published case-control and cohort studies sought to quantify the magnitude and direction of association between chronic UTI (defined as the infection of the urinary tract that either does not respond to treatment or keeps recurring) and risk of bladder carcinoma (BCa) (i.e., including mainly urothelial carcinoma, squamous cell carcinoma or adenocarcinoma). METHODS: A literature search was conducted using Medline, Embase, Ovid, Web of Science, Science Direct and Cochrane Library, which was supplemented with manual search of reference lists of the identified articles. Case-control and cohort studies examining UTI as a predictor of BCa risk published through June 2016 were eligible. Using random-effects models, odds ratios (OR) or relative risks (RR) from eligible studies were combined to synthesize summary effect estimates. The included studies were assessed for methodological quality and potential publication bias. Heterogeneity by study characteristics was examined by sub-group and meta-regression analyses. RESULTS: Eighteen case-control and three cohort studies published between 1963 and 2016 were eligible. Random-effects models showed that UTI was significantly associated with an increased BCa risk both in case-control studies (summary ORRE = 2.33; 95% CI 1.86, 2.92) and cohort studies (summary RRRE = 2.88; 95% CI 1.20, 6.89). The observed relationship of UTI with an increased BCa risk was independent of the study characteristics considered. No significant publication bias was detected. CONCLUSIONS: Chronic UTI was significantly and independently associated with an increased BCa risk. However, due to the presence of high between-study heterogeneity and inconsistent patterns of adjusted confounding effects, more data are needed to clarify the role of chronic UTI in causation of BCa and if established, prompt and effective treatment of UTI may minimize a substantial proportion of BCa risk.