Curious Association Research Articles

On the curious association of diaphragmatic hernia and Urban-Rifkin-Davis Syndrome (Autosomal Recessive Cutis Laxa-1C): A collective review of 16 cases

Background: Autosomal recessive cutis laxa type-1C is also known as Urban-Rifkin-Davis syndrome (URDS). It is known to affect cardiopulmonary, integumentary, gastrointestinal, musculoskeletal and genitourinary systems. However, its frequent association with congenital diaphragmatic hernia has not previously been highlighted. Case Presentation: A newborn male with cutis laxa presented with respiratory distress at birth. The cause of dyspnoea was perinatal strangulation of the stomach in hiatus hernia. After surgical repair of the hernia, his respiratory distress temporarily improved but kept recurring periodically by various mechanisms in sequence namely pulmonary hypertension, tracheomalacia, pulmonary emphysema and finally he succumbed to pneumothorax. Genetic analysis revealed his skin condition as autosomal recessive cutis laxa type-1C which is also known as URDS. Exome sequencing revealed a novel frameshift mutation c.426delC (p.Cys143Alafs*41) of the LTBP4 gene in the exon 5 of Chromosome 19. Conclusion: Out of the 28 cases of URDS reported in the world literature 57% had congenital diaphragmatic hernia (CDH) and 53% of them died during infancy. Such a high incidence of CDH is not observed in other subtypes of elastic disorders. Thus, congenital diaphragmatic defects appear to be a characteristic diagnostic feature of URDS in patients with cutis laxa.

Obesity and Postoperative Cognitive Dysfunction: A Curious Association.

Postoperative cognitive dysfunction (POCD) is a medical condition that impacts cognitive function after surgery, particularly major procedures. Patients with POCD may experience physical symptoms, such as depression, anxiety, and fatigue, severely undermining their quality of life. Research establishes the connection between obesity and cognitive dysfunction since patients diagnosed with obesity are more susceptible to cognitive decline. Although obesity poses a significant risk factor for cognitive impairment, the link between obesity and POCD is still inadequately understood. Therefore, this systematic review explores the correlation between obesity and POCD by detailing potential mechanisms underlying this relationship and identifying areas for further research. Following the guidelines for systematic reviews, we conducted a literature search between August 2022 and April 2023, which identified studies with a substantial number of patients with POCD after major surgeries, including coronary artery bypass grafting, gastrointestinal procedures, cholecystectomy, and carotid endarterectomy. Our findings also demonstrated that a significant percentage of these had obesity, which was statistically significant as a risk factor for cognitive decline. Pathological processes, such as changes in vascular endothelium integrity, systemic inflammation induced by obesity, and apolipoprotein E-epsilon-4 expression, have been identified to contribute to POCD after surgery. Despite the promising results, there remains a gap in the literature. Thus, it is crucial to investigate the relationship between obesity and POCD further, uncover more potential underlying pathophysiological processes, and identify therapeutic targets. These measures would enable healthcare practitioners to prevent or reduce cognitive dysfunction associated with obesity in surgical patients.

Polycystic ovary syndrome and reproductive health of women: a curious association.

Reproductive health is a broad concept that encompasses mortality, morbidity, and quality of life associated with the reproductive system, mechanism, and incidents encountered at all ages by men and women. Orthodox Indian society finds the conversation on reproductive health to be a taboo and discourages open conversations about it. Polycystic ovary syndrome (PCOS) is a reproductive-age metabolic endocrine disorder found in females. Females suffering from PCOS are prone to reproductive, metabolic, and cardiovascular disorders. In this paper, we will systematically review about effect of PCOS on Reproductive Health of Women. The numerous electronic databases such as: BMJ, LANCET, PUBMED, Unicef Website, WHO Website and Google Scholar have been comprehensively searched for studies linked to PCOS, its various effects and effect on women's reproductive health. For additional analyses, we have reviewed reference lists of reviews and collected papers. The effects of PCOS on women's reproductive health have been verified by several scientific reports worldwide. PCOS is a hormonal condition, as per multiple reports, with the ability to lead to different outcomes. It still appears to be a common cause among females of infertility. An integral aspect of the treatment of this disease is the early diagnosis of long-term morbidities by effective screening tests. In the future, studies must concentrate on the missing holes in our growing perception of this disease. Several studies have confirmed that reproductive morbidity, including irregular uterine bleeding, abortion, miscarriage, and other risk of pregnancy during reproductive years, is associated with PCOS. PCOS is an amalgam of physiological and psychosocial dysfunction, not just an endocrine disorder.

Medullary thyroid cancer, papillary thyroid microcarcinoma and Sarcoidosis: a curious association

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

From scuba diving to social networks: A curious association between two small fish species, Lepadogaster candolii Risso, 1810 and Parablennius rouxi (Cocco, 1833), and Muraena helena (Linnaeus, 1758) coming from citizen science

In this work, we report for the first time the associationbetween two cryptobenthic fish, Lepadogaster candolii and Parablennius rouxi, and the Mediterranean moray, Muraena helena. The nature of these associations and the importance of citizen science and social network in marine biology are discussed. Other associations with two relatively large fishes (Conger conger and Epinephelus marginatus) emerged during the study.

Curious Associations of Atrial Septal Defect (ASD) with Cyanosis: Elucidating the Missing Links; Retrospective Descriptive Study

scholarly articles on cardiology Journal of Cardiology & Cardiovascular Therapy is an Open Access, international peer reviewed journal launched from Juniper Publishers. Cardiology is a branch of medicine deals with the disorders of heart that keeps cardiologists up to date with advances in the diagnosis and treatment of cardiovascular diseases.

The Curious Association between Hadley Circulation Intensity and the Meridional Distribution of Tropical Cyclones in the Eastern North Pacific

It has recently been shown that an interesting inverse relationship exists between the strength of the overturning in the regional boreal Hadley circulation and tropical cyclone genesis and lifetime maximum intensity latitudes in the eastern North Pacific. One of the particularly curious aspects of this association is its uniqueness to this ocean basin. We show here that this result can be understood as the outcome of an equatorward shift in the local Intertropical Convergence Zone (ITCZ) and a moderate reduction of tropical vertical shear. These two factors are not necessarily concomitant. The magnitude of the vertical shear change is low enough to suggest that the primary physical mechanism behind this inverse relationship is the equatorward shift in the ITCZ. Since a significant proportion of tropical cyclone genesis results from aggregated convective cells forming into a coherent convective vortex and being shed from the ITCZ, this shift is apparently sufficient to explain the observed association. The equatorward shift in the ITCZ is potentially the result of a relative warming of the Southern Hemisphere in the boreal tropical cyclone season. This more equatorward ITCZ, located over warmer surface waters, presumably explains the more intense Hadley circulation, despite a reduction of the large-scale boreal meridional temperature gradient. Although, these links require further research.

P.071 Novel mutations in SPG7 identified from patients with late-onset spasticity

Background: Hereditary spastic paraplegia (HSP) is a group of genetic diseases that cause progressive degeneration of the corticospinal tract. Historically, this disease was divided into two types:the classic subtype, with leg weakness and hypertonic bladder, and the complicated subtype, with features such as cerebellar ataxia or optic atrophy.Mutations in SPG7 (encoding paraplegin) leads to complicated HSP causing cerebellar ataxia, progressive external ophthalmoplegia in addition to the classical symptoms. AFG3L2 is a binding partner of paraplegin and mutations in AFG3L2 cause a similar syndrome Methods: From a neurogenetic clinic , we identified 11 patients with late-onset HSP. Sequencing of SPG7 and AFG3L2 was performed using a customised assay, and/or clinical diagnostic sequencing panels.SPG7 transcript level quantification was performed from whole blood RNA on a digital droplet qPCR system. Results: We identified 4 patients with pathogenic variants or variants of unknown significance in SPG7. No AFG3L2 mutations were identified. We provide evidence for pathogenicity for three mutations that were not previously associated with SPG7-related disease, based on their occurrence in context of the correct phenotype, and the reduction of transcript levels measured with RT-qPCR.A curious association of the heterozygous p.Gly349Ser mutation in association with an ALS-like syndrome is reported. Conclusions:SPG7 mutations sequencing has high diagnostic yield in late onset paraparesis

“This curious association of objects”: Dickens's Treatment of Chair-Transported Characters in <I>Dombey and Son</I> and <I>Bleak House</I>

“This curious association of objects”: Dickens's Treatment of Chair-Transported Characters in <I>Dombey and Son</I> and <I>Bleak House</I>

Head and neck mucoepidermoid carcinoma: a curious association with second primary malignancy.

We noticed that a significant proportion of our patients with head and neck mucoepidermoid carcinoma (HNMEC) had second primary malignancies. To our knowledge, such an association has never been described. The aim of our study is to elucidate the association between second primary malignancies and HNMEC. Case series with chart review. Tertiary referral center. We included all patients with histologically proven HNMEC managed in the Department of Surgery, Queen Mary Hospital, from January 2003 through December 2013. Medical records were retrospectively reviewed and analyzed. Fifty-seven patients with HNMEC were identified. Fourteen (24.6%) had second primary malignancies. The commonest second primary malignancy was nasopharyngeal carcinoma, followed by carcinoma of the thyroid. Second primary malignancies developed before HNMEC in 7 patients, with a mean interval of 196 months. Five patients had second primary malignancies after development of HNMEC, with a mean interval of 65 months. Two patients had synchronous second primary malignancies. Clinical patterns of patients with HNMEC with and without second primary malignancies were compared. Major salivary glands were more frequently affected among patients with second primary malignancies, while minor salivary glands were more frequently affected among those without secondary primary malignancies (P = .032). Development of second primary malignancy was not found to affect the survival. About one-fourth of patients with HNMEC had a second primary malignancy. Major salivary glands were more frequently affected among patients with second primary malignancies. Development of second primary malignancy did not affect survival.

Unearthing the Mechanism of Prebiotic Nitrile Bond Reduction in Hydrogen Cyanide through a Curious Association of Two Molecular Radical Anions

HCN is clearly associated with the prebiotic chemical evolution of life. It has been known for decades that the radiolysis of HCN solutions produces sugars, amino acids and nucleobases. Remarkably, recent experimental studies have shown that the photolytic reduction of aqueous HCN by a photoredox reagent [Cu(CN)3](2-) specifically yields sugars, which are the essential building blocks of RNA. Although a mechanistic understanding of such reductions with solvated electrons is poor, the general consensus is that they involve neutral free radicals. We show herein through the use of electronic structure studies and molecular simulations that the reduction of the nitrile bond of HCN is initiated through the formation of a molecular dipole-bound anion from the photoredox reagent. Our theoretical studies show how HCN binds to the photoexcited reagent and then extracts an electron from the reagent and is ultimately detached as a dipole-bound anion. The dipole-bound anionic form of [HCN](-) can easily convert into a solvated valence-bound form of [HCN](-). After the formation of solvated [HCN](-), an extraordinary chemical event ensues through a counter-intuitive coupling of two valence-bound anions to form a solvated molecular dianionic intermediate, [HCN]2(2-). Finally, a proton-coupled electron transfer occurs within the dianionic entity to complete the reduction. This mechanistic scenario is applicable to the reduction of other prebiotic nitrile species and avoids neutral radical-based pathways, thereby preventing the proliferation of reactive species and preserving chemical selectivity. Furthermore, we show how such similar nitrile reduction pathways operate to yield the sugar precursors.

Curious association between Plasmodium vivax malaria and nontraumatic acute subdural hematoma.

Sir, A 65-year-old male was brought to the erectile dysfunction with acute onset of altered consciousness level. Relatives informed that the patient was having high-grade fever 4 days back that lasted for 2 days. On the day of admission, the patient suddenly lost his consciousness following an episode of vomiting. Relatives denied any history of abnormal limb movements or frothing from mouth or any facial deviation. Repeated questioning of all the available relatives was negative for history of head trauma (even trivial). On examination, he was afebrile with heart rate 76/min, regular, blood pressure 130/80 mmHg. He was stuporous, Glasgow coma scale-E1 M5 V1 with bilateral equal size (2 mm) pupils reacting to light. There was a paucity of movement in the right side with right-sided extensor plantar reflex. There was no hepatosplenomegaly. Respiratory, cardiovascular, and skin examinations were unremarkable. Computed tomography head showed acute subdural hematoma involving left cerebral convexity with 7 mm midline shift [Figures ​[Figures11 and ​and2].2]. Laboratory investigations revealed leukopenia (3400/mm3) with normal differential count, thrombocytopenia (25,000/mm3) with normal hemoglobin level. Blood urea nitrogen and serum creatinine level were mildly elevated (41 and 1.6 mg/dl, respectively). Liver function test, electrocardiography, and chest X-ray did not reveal any abnormality. Peripheral blood film (both thick and thin film) showed Plasmodium vivax trophozoites. Rapid test for malaria (lactate dehydrogenase based antigen detection test, First sign) was also positive for P. vivax. Dengue serology (NS1, IgG, and IgM dengue) was negative. Patient was intubated and placed on mechanical ventilation. He was started on intravenous artesunate and clindamycin as per WHO guideline and was planned for decompressive craniotomy following platelet transfusion. Only the following day, the patient could be taken up for surgery following eight random donor and one single donor platelet transfusion. Left frontotemporoparietal craniotomy, evacuation of the hematoma and cranioplasty was done [Figure 2]. In the following days in the intensive care unit patient's blood film became negative for malaria parasites and platelet count normalized. However, his sensorium never showed improvement-he remained comatose throughout the postoperative course. On day 16 the family decided to take him home against medical advice. Figure 1 Acute subdural hematoma over left cerebral convexity Figure 2 Postcraniotomy Increasing number of severe manifestations reported in the literature contributed to the increasing interest in P. vivax infection recently. In an observational study of 40 cases of severe vivax malaria, Kochar et al., described different complications, including ARDS, acute kidney injury, thrombocytopenia, hepatic dysfunction, and cerebral malaria.[1] Mixed infection with Plasmodium falciparum species was ruled out by PCR method in all these cases. Falciparum malaria complicated by subdural hematoma has been described in the literature.[2,3] All these cases had associated thrombocytopenia. To the best of my knowledge, this is the first case reported in the literature of a case of vivax malaria associated with acute nontraumatic subdural hematoma.

A novel microhabitat for parasitic copepods: A new genus of Ergasilidae (Copepoda: Cyclopoida) from the urinary bladder of a freshwater fish

An endoparasitic copepod is reported from the urinary bladder of a fish for the first time. Endoparasitic copepods on fish hosts are extremely rare and the impact of colonization of this novel microhabitat on the biology of the parasite is discussed. This curious association was reported from two different host families of Neotropical freshwater fishes, Erythrinidae and Cichlidae, collected from the Cristalino River, a tributary of the Araguaia River, in Brazil. The copepod is fully described using light and scanning electron microscopy. Urogasilus brasiliensis n. g., n. sp. represents a new genus and species of the family Ergasilidae and can be distinguished from other genera by its unique tagmosis, in which the fourth and fifth pedigerous somites and the genital double-somite are all fused to form an elongate trunk. The anal somite is the only free abdominal somite present. The pattern of leg segmentation is also unique, with legs 1 to 3 each having a 2-segmented endopod and leg 4 reduced to a single seta. The discovery of ovigerous female ergasilids in the urinary bladder of a fish is novel and this discovery represents a good model for further studies on the adaptations to an endoparasitic lifestyle.

Evaluating a Pseudo-Wilson Disease Patient: A Previous Undescribed Disease Association of Hallervorden-Spatz Syndrome (HSS) with Anti-m2 Positivity

Purpose: Hallervorden-Spatz Syndrome is a rare neurodegenerative disease with 2 types of clinical presentation: early and late onset. In this subtype, psychiatric, pyramidal and extrapyramidal signs are present. A 56 years old female patient was referred to us to evaluate a possible Wilson’s disease, because of liver function tests abnormalities (ALT and AST 2N; alk phos 1,5 N, GGT 8N) associated to recent psychiatric disturbances (mainly depressive type) and extrapyramidal signs (discrete spasticity, dysarthria and dystonia). No family history of neurological or autoimmune disorders was recorded; she was not taking any medication. She underwent extensive work up revealing high levels of anti-mithocondrial antibodies (>1/640) with positive anti-M2. Cholesterol was 240 mg/dl and tryglicerides were 8 times above normal. Liver biopsy showed panlobular steatosis with scarce fi brosis and no infl ammation, namely on portal triads. Copper in serum, urine and liver measurements were normal. MRI showed bilateral symmetric hyperintense signal changes in globus pallidus, with surrounding hypointensity on T2-weighted images, the characteristic “eye-of-the-tiger” sign. Th erapy was initiated to treat dyslipidemia, including weight loss, and aft er 6 months there has been a progressive normalization of liver tests (except GGT) and no worsening on the discrete neurological signs. To our knowledge it is the fi rst description of this curious association between an iron metabolism disorder and a possible secondary copper accumulation disease (primary biliary cirrhosis).

Incomplete Carney triad--a review of two cases

A curious association of three rare tumours was described by Carney in 1977. 'Carney's triad' characteristically includes multifocal pulmonary chondroma, gastric stromal sarcoma and extra-adrenal paraganglioma. Patients may exhibit complete or incomplete expression of the triad. Carney acknowledged that, of 79 patients, only 17 possessed all three tumours. We report here two patients with incomplete expression of Carney's triad.

Potential ZnS fossilization of gastropods (Middle Jurassic claystones from Central Poland)

The rich fossil fauna in the Middle Jurassic claystones that crop out in the Krakow-Czestochowa Upland is extensively replaced by sulfide minerals, mainly pyrite. Sphalerite (ZnS) is rare and restricted to the internal casts of gastropods, often together with framboidal and euhedral pyrite and calcite. Scanning electron microscopy-energy dispersive spectrometer study was undertaken to explain this curious association. The results show that although direct infilling of the carbonate shell, similar to processes occurring during pyritization, is probable, it does not explain all textures observed. We propose that the carbonate shells were initially infilled by calcite and iron sulfides and sphalerite subsequently replaced the calcite casts. Preferential occurrence of ZnS in gastropods could result from accumulation of higher concentration of zinc during the organisms' life. After death, this Zn was introduced into the carbonate making gastropods more prone to ZnS replacement. Formation of ZnS casts was probably a late diagenetic event as zinc content of the surrounding sediment does not appear to influence sphalerite formation.

Illegal Armed Groups and Local Politics in Colombia

This paper studies the factors associated with the presence of guerrilla, paramilitary, and drug trafficking groups in the municipalities of Colombia. Statistical analysis shows three kinds of results. Some of these tend to corroborate explanations that are commonly accepted in Colombia, while others defy deeply rooted beliefs. As a result, some rather curious associations emerge. The availability of energy resources appears to be a crucial factor of attraction for guerrilla and paramilitary groups, but not for drug traffickers. The conflict also seems to affect both the municipalities' ability to impose taxes, as well as the latter's composition. Against conventional wisdom, drug traffickers appear to have the closest ties to the political sphere. This group shows the greatest capacity to influence civil society, has the most corrosive effect on elections, and exerts the greatest influence on the composition of municipal expenditures. A very popular explanation of violence in Colombia posits a strong and positive association between economic conditions, the so-called objective causes, and the armed conflict. The data does not support the arguments that poverty is responsible for it. In contrast, the data suggest that the age of the population, a purely demographic factor, is determinant. The effect of other social variables is more ambiguous. Curiously, the existence of communications infrastructure is the best indicator of the conflict's geography. The administrative disorganization in public expenditures also appears to be related to the influence of armed groups. However, on an almost anecdotal level, an office for municipal planning is positively associated with the influence of drug trafficking.

Sizing Up Shylock's Name Again

AbstractWhence the name of the Hebrew moneylender Shylock? Although Gross has a chapter title relating to this, the name sounds basically more British than anything else. Indeed, the dramatist's father was himself a lender of money and often considered a Catholic recusant, so Shylock's name connects with that of another such recusant, Shacklock, a name with curious associations with Sherlock. Suggestions of the German scheu and Scottish loch with Shy-lock are also possible. The name may also have connections with John Florio.

Intracranial meningiomas associated with glial tumours: A review based on 54 selected literature cases from the literature and 3 additional personal cases

The simultaneous occurrence of meningioma and glioma is extremely rare. Three new cases and 54 adequately described in the literature are analyzed. Clinical diagnosis may be difficult due to discrepancy between clinical and radiological findings. Unexpected clinical deterioration following removal of a tumour and relapse simulating recurrence may occur. The introduction of CT technology does not seem to have offered the expected contribution to the early diagnosis of these coincidental lesions, at least before the introduction of the newer generation scanners or MRI. While removal of both tumours in one session yielded the best results, surgery for the sole glioma appeared to be associated with an unacceptably high mortality. Although several aetiopathogenetic hypotheses have been suggested for explaining this curious association, coincidental meningioma and glioma are most likely to be different primary brain tumours occurring randomly in the same individual.

Alcoholic Liver Disease: Pathologic, Pathogenetic and Clinical Aspects

Alcoholic liver disease includes steatosis, alcoholic hepatitis and cirrhosis. Other liver diseases of genetic origin, but with a curious association with alcohol intake, are hemochromatosis and porphyria cutanea tarda. The attribution of chronic hepatitis to alcohol intake remains speculative, and the association may reflect hepatitis C infection. Hepatic injury attributed to alcohol includes the changes reported in the fetal alcohol syndrome. Steatosis, the characteristic consequence of excess alcohol intake, is usually macrovesicular and rarely microvesicular. Acute intrahepatic cholestasis, which in rare instances accompanies steatosis, must be distinguished from other causes of intrahepatic cholestasis (e.g., drug-induced) and from mechanical obstruction of the intrahepatic bile ducts (e.g., pancreatitis, choledocholithiasis) before being accepted. Alcoholic hepatitis (steatonecrosis) is characterized by a constellation of lesions: steatosis, Mallory bodies (with or without a neutrophilic inflammatory response), megamitochondria, occlusive lesions of terminal hepatic venules, and a lattice-like pattern of pericellular fibrosis. All these lesions mainly affect zone 3 of the hepatic acinus. Other changes, observed at the ultrastructural level, are of importance in progression of the disease. They include widespread cytoplasmic shedding, and capillarization and defenestration of sinusoids. Progressive fibrosis complicating alcoholic hepatitis eventually leads to cirrhosis that is typically micronodular but can evolve to a mixed or macronodular pattern. Hepatocellular carcinoma occurs in 5 to 15% of patients with alcoholic liver disease. The clinical syndrome of alcoholic liver disease is the result of three factors--parenchymal insufficiency, portal hypertension and the clinical consequences of extrahepatic damage produced by alcohol. At the several phases of the life history of alcoholic liver disease, the individual factors play a different role. The clinical manifestations of alcoholic steatosis are mainly extrahepatic in origin. Those of alcoholic hepatitis reflect mainly parenchymal insufficiency and those of cirrhosis are mainly those of portal hypertension. Alcoholic liver injury appears to be generated by the effects of ethanol metabolism and the toxic effects of acetaldehyde, perhaps the immune responses to alcohol- or acetaldehyde-altered proteins, and questionably enhanced by viral hepatitis. Alcoholic hepatitis may be mimicked histologically, and to a varying degree clinically, by a number of conditions (obesity, diabetes, several drug-induced injuries, jejunoileal bypass, and related "shortcircuiting" of the bowel). Perhaps the most important facet of the hepatotoxicity of alcohol is its enhancement of the effects of a number of other hepatotoxic agents, among which acetaminophen is the prime example.