Congenital dislocation of the hip in Western Australia. A comparison of neonatally and postneonatally diagnosed cases

Speculation that neonatally diagnosed congenital dislocation of the hip (CDH) may have a different etiology from cases diagnosed in the postneonatal period has not been examined in Australia because insufficient data have been available. A population-based study of CDH in children up to the age of two years who were born in Western Australia (WA) in 1981, 1982, or 1983 is the subject of this report. Study material comprised cases of CDH from the WA Congenital Malformations Register and denominator data (all births in WA for 1981-1983) from the Health Department of WA. From this material, rates of CDH were calculated for each of the study variables of interest. The rate of CDH was low for babies born to aboriginal mothers, and it is postulated that this may have a cultural basis, possibly in infant carrying postures. Overall, the prevalence of CDH for 1981-1983 was 6.4 cases per 1000 births, with 4.2/1000 diagnosed in the neonatal period and 2.2/1000 in the postneonatal period. Epidemiologic differences were noted between infants diagnosed in the neonatal period and those diagnosed postneonatally; rates of neonatally diagnosed CDH were higher in first births, breech presentation, and postmature infants than were rates of postneonatally diagnosed cases. Bilateral dislocation was more common (45.3%) in neonatally diagnosed cases than in postneonatally diagnosed cases (23.3%). These findings tended to support the idea that the time of diagnosis may define two distinct entities in CDH.     

Renal enlargement in the fetus and newborn with congenital diaphragmatic hernia: a refuted hypothesis

BACKGROUND/PURPOSE: Congenital diaphragmatic hernia (CDH) is one of the most frequent causes of neonatal death because of lung hypoplasia (LH). The literature mentions a relationship between renal and pulmonary development, with higher kidney weight in presence of LH. The aims of this study were to evaluate the relationship between lung and kidney weight and to test the hypothesis of renal enlargement in fetuses and newborns with CDH. METHODS: Body weight (BW), combined kidney weight (KW), and lung weight (LW) of 52 CDH cases were established; 52 morphologically normal fetuses or newborns, matched by BW, served as a control population. Comparisons were done by covariance analysis, and a P value of less than .05 was considered as significant. RESULTS: Excluding renal abnormalities, adjusted mean kidney weights were 22.0 g (+/-1.8 SE) in CDH cases and 20.5 g (+/-1.5 SE) in controls (F = 1.05; P = .308). KW to BW ratio was lower in CDH cases than in controls (P = .023). LW was significantly lower in CDH cases than in controls. CONCLUSIONS: No significant difference between KW of CDH cases and controls could be observed. The current study provides enough evidence to reject the hypothesis of renal enlargement in cases of LH and CDH.     

Low incidence of acute urinary retention in the general male population: the triumph project

OBJECTIVE: To describe the incidence of acute urinary retention (AUR) in the general male population and in a population of men newly diagnosed with lower urinary tract symptoms suggestive of BPH (LUTS/BPH). METHODS: We performed a retrospective cohort study in the Integrated Primary Care Information (IPCI) database, a GP research database in The Netherlands, during the period 1995-2000. All males, > or =45 years, without a history of AUR or radical cystectomy were included in the study. In addition, we followed a sub-cohort of men, newly diagnosed with LUTS/BPH. AUR was defined as the sudden inability to urinate, requiring catheterization. RESULTS: Amongst 56,958 males with a mean follow-up of 2.8 years, 344 AUR cases occurred (incidence rate 2.2/1000 man-years) of whom more than 40% were precipitated. AUR was the first symptom of LUTS/BPH in 73 (49%) of the 149 AUR cases that occurred in men newly diagnosed with LUTS/BPH. The risk of AUR was 11-fold higher in patients newly diagnosed with LUTS/BPH (RR 11.5; 95%CI: 8.4-15.6) with an overall incidence rate of 18.3/1000 man-years (95%CI: 14.5-22.8). CONCLUSIONS: The incidence rate of AUR is low in the general population but substantial in a population of men newly diagnosed with LUTS/BPH. The incidence rate increases with age and AUR is precipitated in approximately 40% of all cases. Within the LUTS/BPH cohort, AUR is the first presenting symptom of BPH in 50% of all AUR cases.     

Pathophysiology of congenital diaphragmatic hernia: I. Renal enlargement suggests feedback modulation by pulmonary derived renotropins--a unifying hypothesis to explain pulmonary hypoplasia, polyhydramnios, and renal enlargement in the fetus/newborn with congenital diaphragmatic hernia

Survival of newborns with congenital diaphragmatic hernia (CDH) is largely dependent on the severity of pulmonary hypoplasia (PH) present at birth. Intrathoracic compression by the herniated abdominal viscera is thought to be the primary factor involved in the pathogenesis of the PH in CDH. Humoral and/or amniotic pulmonary growth factors (PGF) have been hypothesized to play a role in normal fetal pulmonary development and may be involved in the pathogenesis of CDH as well. The hypothesis of this paper is that growth of the fetal lung is stimulated by a PGF produced by the kidneys, which is modulated by a feedback signal from the lungs, a pulmonary derived renotropin (PDR). In the fetus with CDH, the lungs may be unable to respond to PGF due to compression by the herniated abdominal viscera. Theoretically, PH associated with CDH would maximally stimulate this feedback loop to release more PDR, resulting in continual stimulation of the kidneys and renal enlargement. If such a scheme plays a role in the in utero pathophysiology of CDH, then newborns with CDH should have enlarged kidneys. To investigate this hypothesis, we reviewed 30 autopsy cases of newborns with CDH and analyzed their kidney weights versus body weights, using historical data as control. Kidney weights in CDH cases were greater than the control population in 77% of the cases; 57% of kidney weights were more than one standard deviation above control values. Adjusted group mean kidney weights were 29.8 g (+/- 1.0 SE) in CDH cases and 25.9 g (+/- 1.5 SE) in the control population (P less than .04). These data support our hypothesis and demonstrate that in newborns with CDH and morphologically normal kidneys, there is significant renal enlargement associated with CDH. The presumed mechanism of this renal enlargement, as well as its relationship to normal and aberrant pulmonary growth and regulation are discussed. If such a selective PGF exists, its therapeutic implications for fetuses and newborns with PH are considerable.     

Correlation of fetal posture and congenital dislocation of the hip

A statistical study was carried out on the incidence of CDH associated with mechanical factors in the uterus, including congenital genu recurvatum. There were 72 cases of CDH among 6559 infants (1.1 per cent). The incidence of CDH was 0.7 per cent in cephalic presentation, 2 per cent in footling presentation and 20 per cent in single-breech presentation. In another series, CDH was found in six of seven infants with congenital genu recurvatum. These findings suggest that a fetal posture with the hip flexed and the knee extended predisposes to the development of CDH.     

肝细胞肝癌CDH1基因启动子C/A单核苷酸多态性与蛋白表达的关系

目的 探讨原发性肝细胞肝癌CDH1基因启动子-160位点的C/A单核苷酸多态性(SNP)与其蛋白表达的关系.方法 以34例肝癌手术病人为对象,DNA直接测序法检测其血液标本中CDH1基因启动子-160位点C/A SNP,免疫组化法检测组织标本中CDH1的蛋白产物--上皮钙黏素(E-cadherin)的表达情况,比较分析C/A SNP与E-cadherin表达的关系.结果 E-cadherin高表达组18例(52.9%),低表达组16例(47.1%),两组的基因型出现率CC与CA,AA比较差异均有统计学意义(P<0.05),CA与AA间差异无统计学意义(P>0.05),A、C等位基因频率在两组差异有统计学意义(P<0.05).结论 CDH1基因启动子-160位点的C/A SNP在肝癌E-cadherin表达中可能发挥重要作用,且A等位基因的出现与E-cadherin表达下调相关.     

膀胱移行细胞癌CDH1基因启动子C/A单核苷酸多态性与其蛋白表达关系的研究

目的探讨膀胱移行细胞癌（BTCC）中E-钙粘连素（CDH1）基因启动子-160处C/A单核苷酸多态性（SNP）与CDH1表达的关系。方法BTCC患者36例，男24例，女12例，分别取血液和膀胱肿瘤组织标本。对照组36例，为非肿瘤患者，男30例，女6例，取膀胱组织标本。PCR-限制性片段长度多态性分析法检测血液标本中CDH1基因启动子上游-160处C／ASNP。免疫组化方法检测组织CDH1的表达情况。比较2组组织CDH1表达情况，分析BTCC组C／ASNP与CDH1表达的关系。结果BTCC组和对照组CDH1阳性率分别为61％（22／36）和86％（31／36），P＜0．05。BTCC组14例CDH1表达阴性者中．AA基因型8例、AC型3例、CC型3例，A等位基因频率为68％（19／28）；22例CDH1表达阳性者中，AA型3例、AC型11例、CC型8例，A等位基因频率为39％（17／44）；2组中AA基因型发生率与AC、CC基因型比较差异均有统计学意义（P＜0．05），A等位基因频率比较差异有统计学意义（P＜0．05）。结论CDH1基因启动子-160处C／ASNP在TBCC的CDH1表达过程中可能具有重要作用，A等位基因对CDH1表达下调可能发挥主要作用。     

Colorectal carcinoma--a new threat to black patients? A retrospective analysis of colorectal carcinoma received by the Institute for Pathology, University of Pretoria.

OBJECTIVE: To compare black and white patients with colorectal carcinoma treated at Pretoria Academic and Kalafong hospitals, and to compare pathological trends of our study population with others reported in the literature. DESIGN: A retrospective study of all cases of resected colorectal carcinomas received by our department during the periods 1986-1987 (82 cases) and 1996-1997 (91 cases). To investigate variables of age, race and gender distribution in the two study populations. METHODS: Routinely stained histological sections of all relevant cases were examined. Findings regarding age, gender, population group, anatomical location of the tumour and presence of other pathological lesions were recorded. Changes in the referral population and number of surgical specimens received were also considered during statistical analysis of the study findings. RESULTS: There has been a significant increase in the number of black patients with colorectal carcinoma at our Institute. In addition, adenomatous polyps were found in 9 of our black patients (1996/97). This is significantly higher than expected from reports in the literature. This could be predictive of an increase in incidence of colorectal carcinomas in our black population. Black patients were also found to be considerably younger at age of presentation than their white counterparts. A further significant finding was a considerable increase in the number of black females under the age of 40 years from 1986/87 to 1996/97. On the other hand, the number of white females above 40 years of age decreased considerably over this time. The reason for this finding is uncertain and warrants further study.     

C.N.S. Tumors in Eastern Saudi Arabia

In Saudi Arabia, there were no attempts previously to describe a population based frequency or incidence, particularly so the age adjusted incidence of various CNS tumors. This paper presents the primary CNS tumors from a population based tumor registry over two years period, from January 1987 till December 1988. There was a total of 85 cases representing 5.4% of the total captured cases (1,568 cases of malignant tumors at all sites). The population of the Eastern Province is estimated to be 1.37 million, the Saudis forming 80% of the total population. Out of the 85 cases captured over two years, there were 64 cases diagnosed in indigenous Saudi population forming 75%. The remaining occurred in non-Saudi residents. The male/female ratio in Saudis was 1:1.1 with a slight predominance of the female, while the reverse is true in the non-Saudis (2:1). The total captured cases per annum is 43, making the incidence of primary CNS neoplasms in the Eastern Province of Saudi Arabia 3.1/100,000 of all the population and 2.9/100,000 in Saudi nationals. Comparing this incidence to the international figure, it was clear that it is far less than the incidence reported from North America and Europe, particularly in the Caucasian population, but similar to incidences reported in the Chinese, black Americans, Romanians and Yugoslavians, but certainly less than the Ashkenazi or Safari Jews, and slightly higher than the incidence reported in Japan and Southeast Asia. Malignant brain tumors of various types dominated the primary CNS neoplasms reported over these two years forming 69% of the cases and 52% of the primary brain tumors.     

Teratologic Congenital Dislocation of the Hip: Report of Two Cases

The incidence of teratologic dislocation of the hip is about 0.04 per thousand. Teratologic CDH is usually described together with other anomalies, such as arthrogryposis.

Quite different opinions about the diagnostic criteria are found in the literature; some of these are reviewed in this report. Two cases of teratologic CDH with no other anomalies are also reported.

Computer tomography was used to confirm reduction in plaster when conventional radiologic examination gave ambiguous results.     

Congenital dislocation of the hip in Finland: An epidemiologic analysis of 1035 cases

The incidence of congenital dislocation of the hip (CDH) and some birth characteristics of the population in Uusimaa county in southern Finland were investigated during the years 1966 through 1975. A total of 1035 babies with CDH was born during the time under review. This was 0.68 per cent of liveborns in the area. In 920 babies, the diagnosis was made during the first month of life, corresponding to 0.61 per cent of liveborns and 89 per cent of all children with CDH. The annual variation was great. The monthly variation of CDH in girls was significant, with a peak in June-July, differing from other investigations. Contrary to results of several other studies, the birth weight of the affected babies was normal. The sex distribution, number of first-born babies, side of the dislocation and associated calcaneovalgus foot were in accordance with findings in most other investigations.     

浅表性膀胱移行细胞癌复发与CDH1基因启动子C/A编码区单核苷酸多态性的关系

目的探讨CDH1基因启动子160C/A编码区单核苷酸多态性(cSNP)和浅表性膀胱移行细胞癌(STCCB)的复发之间的关系。方法健康对照组50例,无复发STCCB组33例,复发STCCB组28例,其中复发组按复发后有无临床分期和病理分级的升高分为进展组(10例)和非进展组(18例);PCRRFLP技术检测各组样本CDH1基因启动子160位点基因型。结果复发STCCB组该位点A等位基因频率(0.66)高于无复发STCCB组(0.48),P<0.05,差异有统计学意义;携带A等位基因的STCCB患者治疗后复发的危险性高于C等位基因携带者,OR值3.54,95%CI为1.52～5.73。结论CDH1基因启动子160A等位基因与浅表性膀胱移行细胞癌的复发密切相关。     

Adverse outcome of congenital diaphragmatic hernia is determined by diaphragmatic agenesis, not by antenatal diagnosis

BACKGROUND/PURPOSE: The authors studied their congenital diaphragmatic hernia (CDH) cases retrospectively to ascertain if classical CDH and diaphragmatic agenesis (DA) have separate clinical manifestations, whether antenatally diagnosed cases behave differently from those not diagnosed antenatally, and if antenatal diagnosis before 25 weeks carries a worse prognosis. METHODS: The authors performed a retrospective review of 23 infants with CDH treated at their institution between January 1996 and March 1999. The patients were divided into 3 groups that were analyzed: DA and classical CDH, antenatally diagnosed and nonantenatally diagnosed, and antenatally diagnosed before 25 weeks and after 25 weeks. RESULTS: There were 8 cases of DA and 11 cases of classical CDH. Four infants died without operation and could not be classified. Neonates with DA had significantly longer mean duration of preoperative stabilization compared with classical CDH (5.25+/-2.76 days v 1.36+/-1.0 days) and postoperative mechanical ventilatory support (15.7+/-3.0 days v 4.9+/-3.0 days). Fifty percent of DA patients died; all classical CDH patients survived. Twelve cases were diagnosed antenatally, 6 before 25 weeks' gestation. Antenatally diagnosed cases had no statistically significant difference in mortality rates from those not diagnosed antenatally; 50% of those diagnosed before 25 weeks survived. CONCLUSIONS: DA cases require more preoperative preparation and postoperative ventilation and have a bad prognosis compared with classical CDH. Antenatal diagnosis of CDH does not convey a different prognosis. Fifty percent of CDH patients with antenatal diagnosis before 25 weeks survive.     

Examination, prevention and treatment of congenital dislocation of the hip in the newborn infant--experience over an eighteen-year period

On the basis of the authors' experience during the past 18 years, clinical data on CDH in the newborn were studied and summerized as follows. The use of diaper with hip-flexed position decreased not only the incidence of click, but also the frequency of CDH in infancy, indicating their effectiveness for the prevention of CDH. It is important to know whether the hips are dislocated or dislocatable at the original examination, when one decides on indication and initiation of treatment. The affected hips should be treated when "dislocated state" is found and the instability persists for 1 or 2 weeks or when "dislocatable state" is found and the instability persists for 2 or 3 weeks. Treatment with harness is useful for the newborn with CDH. The use will not cause any complication. Development of infants with CDH following after newborns with CDH is influenced by postnatal environmental factors as well as late diagnosis.     

Incidence and outcomes of patients with congenital diaphragmatic hernia and pulmonary sequestration

BackgroundBronchopulmonary sequestration (BPS) has long been identified in patients with congenital diaphragmatic hernia (CDH), however the reported incidence in the literature varies widely and is not based on large series.MethodsVersion 4 of the Congenital Diaphragmatic Hernia Study Group (CDHSG) included questions specifically identifying BPS-associated cases. The data were prospectively collected between 2015 and 2020. Clinical characteristics and outcomes for CDH+BPS patients were compared to patients without BPS using univariate and multiple regression analyses.ResultsOut of 2118 total patients, 72 had a pulmonary sequestration (3.4%). The amount of CDH+BPS with high-risk (CDHSG type C and D) defects was significantly greater than those without BPS (68.0% vs 49.3%, respectively; p = 0.001). The need for ECLS (35/72, 48.6%) and overall mortality (21/72, 29.2%) was significantly higher in CDH+BPS. When corrected for hernia size, cardiac and chromosomal anomalies, the need for ECLS (OR 2.2, p = 0.004) and mortality (OR 2.0, p = 0.015) was significantly greater in CDH+BPS.ConclusionsThis is the largest series to look at the incidence of BPS in children with CDH. They are associated with larger defect sizes, a greater need for ECLS and higher mortality than those patients without BPS.     

Soft tissue collagen in congenital dislocation of the hip. Biochemical studies of the ligamentum teres of the femur and the hip joint capsule

Collagens of the ligamentum teres of the femur and the hip joint capsule of 14 patients with congenital dislocation of the hip (CDH) were studied biochemically. Collagen was a major component of these tissues. Solubility of collagen was reversely related to age except in two cases. Collagen of both ligamentum teres of the femur and hip joint capsule was composed of type I, III and V collagens except in three cases. In these three cases with nevus or general joint laxity, two additional collagenous components were also found. The ratios of type III collagen to type I collagen were increased in CDH except in one case with short stature. These abnormalities of collagen metabolism could be the underlying cause of CDH and other clinical symptoms in these patients.     

A population-based study of congenital diaphragmatic hernia: impact of associated anomalies and preoperative blood gases on survival.

BACKGROUND/PURPOSE: Although neonatal care has improved over the past 20 years, mortality rate with congenital diaphragmatic hernia (CDH) remains 50% to 60%, possibly reflecting differences in management or selection biases. The authors determined the incidence, outcome, effect of coexisting anomalies, and prognostic indicators for neonates with CDH in a single inborn population older than 13 years. METHODS: Forty-three neonates with CDH, those symptomatic within the first 6 hours of life, were identified using a validated neonatal database and diagnosis coding data from medical records among 180,643 live inborn neonates delivered at Parkland Memorial Hospital between 1983 and 1995. Charts were reviewed for prenatal history, demographic variables, presence of coexisting malformations, preoperative arterial blood gases, surgical findings, and outcome. Survival to hospital discharge was the primary outcome variable. RESULTS: The incidence of CDH was 1 in 4,200 live births; overall survival rate was 51%. Thirty-two (74%) neonates underwent surgical repair, often at less than 8 hours of life; postoperative mortality rate was 31%. Eighteen (42%) had coexisting major anomalies or chromosomal abnormalities. Eighty percent of neonates with isolated CDH survived, whereas 89% with CDH and associated defects died. Nonsurvivors had lower birth weights and Apgar scores, were more acidotic, and had more severe respiratory compromise. When best preoperative pH was > or = 7.25 or PaCO2 < or = 50 mm Hg, 80% of neonates survived. CONCLUSION: In this inborn population-based review of neonatal CDH between 1983 and 1995, the best predictors of survival were the presence or absence of other anomalies and the best preoperative PaCO2 and pH.     

Unequal burden of head and neck cancer in the United States

BACKGROUND: Black Americans are adversely affected by many types of malignancies. METHODS: We reviewed data from the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) program to evaluate racial disparities in head and neck cancer incidence, mortality, and survival. RESULTS: Head and neck cancer incidence is greater in the black population and peaks at a younger age. The incidence disparity is decreasing over time and is less for cancers of the oral cavity/pharynx (OCP) than for cancers of the larynx. The disparity in survival after diagnosis is substantial for both sites and is increasing over time because of improvement in survival for the white population, but not for the black population. Some, but not all, of the survival disparity is due to more advanced stage at the time of diagnosis within the black population. The age-adjusted mortality rate for black men is approximately twice the rate for white men. CONCLUSION: Black Americans clearly bear a greater burden from head and neck cancer. The underlying causes are largely unknown, but are most likely due to a complex interplay of differences in access to health care, quality of medical care, biologic/genetic factors, incidence of comorbid conditions, exposure to carcinogens, diet, and cultural beliefs. Prospective studies are needed to define the relative importance of these factors and to inform intervention strategies.     

Pitfalls of early diagnosis and treatment of congenital dislocation of the hip joint

The efficiency of the routines for early diagnosis and treatment of congenital dislocation of the hip joint (CDH) practiced in the 1960s in the city of G?teborg were reviewed. Between 1961 and 1970 there were 65,875 live births in G?teborg. Eighty-five percent of the estimated number of cases of CDH were diagnosed during the first month of life. Of the children "missed" at the primary screening on the maternity wards, 75% (15/20) were premature babies and/or had been delivered during weekends. In all, 475 newborn children were judged to have unstable hips (dislocated or dislocatable hips), corresponding to an incidence of 7.2 per thousand live births. In 19 children, abduction treatment with braces commenced during the first 10 days of life failed to prevent dislocation. Eighteen of these children were treated primarily with a Frejka pillow and one with a von Rosen splint. A history of CDH among relatives was obtained in 47% of these 19 children including four of five children who had developed pathological hips after supplementary treatment with a hip spica cast and/or surgery. Radiological examination at the age of 1-4 months was helpful in revealing failure of early abduction treatment to prevent dislocation, which in most cases was difficult to detect by clinical examination at this early age.     

Congenital dislocation of the hip and its relation to swaddling used in Turkey.

A screening program was initiated and performed at five hospitals in Konya, Turkey from 1988 to 1990. Four thousand one hundred seventy-three infants aged 3-24 months were examined. With this study, we hoped to determine the incidence of and contributing factors to the etiology of congenital dislocation of the hip (CDH) in the central region of Turkey. The overall CDH incidence was 1.34%. CDH occurred about three times more often among girls than boys (40 girls vs. 16 boys). No infant with CDH had been delivered by Cesarean section or breech presentation. No teratologic CDH was detected. The relationship between CDH and swaddling (bundled in extension and adduction) was statistically significant.