A review of preclinical evidence of Cryptolepis nigrescens (Wennberg) L. Joubert. and Bruyns., Prosopsis africana (Guill. and Perr.) Taub. and Pterygota macrocarpa K. Schum. traditionally used to manage tumours in Ghana.

ETHNOPHARMACOLOGICAL RELEVANCE: Cancer stands as one of the leading causes of death worldwide according to the World Health Organization (WHO), and it has led to approximately 10 million fatalities in 2020. Medicinal plants are still widely used and accepted form of treatment for most diseases including cancer in Ghana. This review presented Cryptolepis nigrescens (Wennberg) L. Joubert. and Bruyns., Prosopsis africana (Guill. and Perr.) Taub. and Pterygota macrocarpa K. Schum. as medicinal plants that are traditionally used to treat tumour growth, amongst other diseases, in the Ashanti region of Ghana. AIM OF REVIEW: This paper aims to present a comprehensive review on the botanical description, ecological distribution, ethnomedicinal uses, phytochemical composition and ethnopharmacological relevance of C. nigrescens, P. africana and P. macrocarpa. MATERIALS AND METHODS: The review covers works published between 1962 and 2023 from various countries. Published books, thesis, scientific and medical articles on C. nigrescens, P. africana and P. macrocarpa were collected from the following databases: 'Scopus', 'Science Direct', 'Medline', 'PubMed', 'Research Gate' 'Google Scholar, and 'Springer link' using the keywords. RESULTS: Phytochemical analysis of C. nigrescens, P. africana and P. macrocarpa revealed the presence of some prominent bioactive compounds such as convallatoxin, 7,3,4-trihydroxy-3-methoxyflavanone and dioxane, respectively. Plant extracts and isolated compounds of these medicinal plants exhibited a wide range of ethnopharmacological activities including antimicrobial, anti-inflammatory, antioxidant, analgesic, cytotoxic, antimalarial, antipyretic, haematinic, hepato-protective, aphrodisiac and antihypertensive properties. CONCLUSION: The present review on C. nigrescens , P.africana and P. macrocarpa provided a credible summary of the ethnopharmacological research conducted on these medicinal plants till date. The data also highligted the potential therapeutic profiles of these plants in Ghana that could serve as foundation for future studies. Additionally, the information significantly supported the traditional and commercial use of these plants among the people.

Chemical Strike against a Dominant-Inherited MUC1-Frameshifted Protein Associated with Progressive Kidney Disease.

In a recent paper by Dvela-Levitt et al., chemical screening using an immunofluorescent assay identified a compound that caused removal of a dominant-inherited misfolded secretory protein, mucin1-frameshifted, from an intracellular location in immortalized renal epithelial cells of a patient affected with progressive medullary cystic kidney disease. This illustrates the power of chemical screening at the cellular level to address specific proteinopathies and the utility of such compounds to illuminate novel cellular pathways that can clear toxic proteins.

Roscovitine blocks collecting duct cyst growth in Cep164-deficient kidneys.

Nephronophthisis is an autosomal recessive kidney disease with high genetic heterogeneity. Understanding the functions of the individual genes contributing to this disease is critical for delineating the pathomechanisms of this disorder. Here, we investigated kidney function of a novel gene associated with nephronophthisis, CEP164, coding a centriolar distal appendage protein, using a Cep164 knockout mouse model. Collecting duct-specific deletion of Cep164 abolished primary cilia from the collecting duct epithelium and led to rapid postnatal cyst growth in the kidneys. Cell cycle and biochemical studies revealed that tubular hyperproliferation is the primary mechanism that drives cystogenesis in the kidneys of these mice. Administration of roscovitine, a cell cycle inhibitor, blocked cyst growth in the cortical collecting ducts and preserved kidney parenchyma in Cep164 knockout mice. Thus, our findings provide evidence that therapeutic modulation of cell cycle activity can be an effective approach to prevent cyst progression in the kidney.

Nystagmus And Beyond: A Rare Ocular Motility Disorder.

Joubert syndrome is a rare autosomal recessive disorder predominantly involving the cerebellar vermis and brain stem. It is characterized clinically by global developmental delay, abnormal ocular movements, hypotonia, ataxia, intellectual disability and neonatal breathing abnormalities. Due to its uncommon and unconventional presentation, its diagnosis is usually delayed. Diagnosis of this atypical disease essentially relies upon the atypical finding of the "molar tooth" sign on Magnetic Resonance Imaging (MRI). We report a case of a 5-year-old boy who presented with abnormal eye movements, regression of milestones and developmental delay. MRI investigation revealed the distinctive molar tooth sign and bat wing shaped 4th ventricle. It requires high levels of clinical suspicion and holistic approach to such children who present with delayed milestones and abnormal eye movements, to reach at early detection and diagnosis of such rare pathologies.

Absence of associative motor learning and impaired time perception in a rare case of complete cerebellar agenesis.

Primary cerebellar agenesis (PCA), a brain disease where the cerebellum does not develop, is an extremely rare congenital disease with only eleven living cases reported thus far. Studies of the PCA case will thus provide valuable insights into the necessity of cerebellar development for controlling and modulating cognitive functions of the brain. In this follow-up study, we further investigated the performance of associative learning and time perception of a 26-year-old female complete PCA case. We assessed whether delayed eyeblink conditioning (EBC), which represents prototypical associative motor learning function of the cerebellum, could be partially compensated by the extracerebellar brain regions in complete absence of the cerebellum. We also assessed whether the cerebellum, a critical brain region for millisecond-range interval timing, is essential for perception of the second-range time interval. Twelve neurotypical age-matched individuals were used as controls. We found that although the complete PCA patient had only mild to moderate motor deficits, she was unable to perform the delayed EBC even after 1-week of extensive training. Additionally, the PCA patient also performed poorly during time reproduction experiments in which she overproduced the millisecond-range time intervals, while underproduced the second-range time intervals. The PCA patient also failed to perform the temporal eyeblink conditioning with a 5 s fixed interval as the conditioned stimulus. These results indicate that the cerebellum is indispensable for associative motor learning and involved in timing of sub-second intervals, as well as in the perception of second-range intervals.

Soy Protein Alleviates Hypertension and Fish Oil Improves Diastolic Heart Function in the Han:SPRD-Cy Rat Model of Cystic Kidney Disease.

Abnormalities in cardiac structure and function are very common among people with chronic kidney disease, in whom cardiovascular disease is the major cause of death. Dietary soy protein and fish oil reduce kidney disease progression in the Han:SPRD-Cy model of cystic renal disease. However, the effects of these dietary interventions in preventing alterations in cardiac structure and function due to kidney disease (reno-cardiac syndrome) in a cystic kidney disease model are not known. Therefore, weanling Han:SPRD-Cy diseased (Cy/+) and normal (+/+) rats were given diets containing either casein or soy protein, and either soy or fish oil in a three-way design for 8 weeks. Diseased rats had larger hearts, augmented left ventricular mass, and higher systolic and mean arterial blood pressure compared to the normal rats. Assessment of cardiac function using two-dimensional guided M-mode and pulse-wave Doppler echocardiography revealed that isovolumic relaxation time was prolonged in the diseased compared to normal rats, reflecting a diastolic heart dysfunction, and fish oil prevented this elevation. Soy protein resulted in a small improvement in systolic and mean arterial pressure but did not improve diastolic heart function, while fish oil prevented diastolic heart dysfunction in this model of cystic kidney disease.

Dietary flax oil rich in α-linolenic acid reduces renal disease and oxylipin abnormalities, including formation of docosahexaenoic acid derived oxylipins in the CD1-pcy/pcy mouse model of nephronophthisis.

The CD1-pcy/pcy mouse model of nephronophthisis displays reduced renal docosahexaenoic acid (DHA) levels and alterations in renal cyclooxygenase and lipoxygenase oxylipins derived from n-6 fatty acids. Since dietary flax oil ameliorates disease progression, its effect on renal fatty acids and oxylipins was examined. Sixteen weeks of feeding resulted in reduced disease progression and enrichment of renal phospholipid α-linolenic acid (ALA) and eicosapentaenoic acid, reduction in arachidonic acid (AA), but no change in linoleic acid (LA) or DHA. In diseased kidneys, flax oil feeding mitigated the elevated levels of renal cyclooxygenase derived oxylipins formed from AA and the lowered lipoxygenase and cytochrome P450 derived oxylipins formed from ALA and DHA. Increased DHA oxylipins occurred with flax feeding despite not altering DHA levels. Dietary flax oil may therefore reduce disease progression via mitigation of oxylipin abnormalities. This study also provides evidence of in vivo ALA conversion to DHA in amounts necessary to restore DHA oxylipin levels.

Quiste renal hiperdenso: presentación de un caso / Hyperdense renal cyst: a case report

Parejo al aumento de las exploraciones radiológicas ha ocurrido el escubrimiento de quistes renales que, en ocasiones, no cumplen los criterios de benignidad o malignidad. Dentro de esta lesiones se encuentran los quistes hiperdensos, los cuales constituyen un reto para todos los responsables del diagnóstico y tratamiento de estos pacientes. Por tales razones se decidió la presentación del caso de una paciente de 23 años de edad, con antecedentes de sicklemia, que acudió al cuerpo de guardia del Hospital General Universitario Gustavo Aldereguía Lima, por sufrir dolor lumbar en lado izquierdo, de tipo cólico, que no se aliviaba con analgésicos. Mediante estudio por tomografía axial computarizada, simple y con contraste endovenoso, se le diagnosticó un quiste renal hiperdenso tipo II, según la clasificación de Bosniak(AU)

Increased radiological tests have facilitated the discovery of kidney cysts, which sometimes do not meet clear criteria for benignity or malignancy. Among these lesions, the hyperdense cysts pose a challenge to all those responsible for their diagnosis and treatment. For such reasons we decided to present the case of a 23-year-old female patient with a history of sickle cell anemia, who attended the emergency department of the Gustavo Aldereguía Lima University General Hospital due to colic-like pain in the left lumbar region unresponsive to analgesics. She was diagnosed with category II renal cyst according to Bosniak classification using computed tomography with and without intravenous contrast(AU)

Comorbidity profile of poliomyelitis survivors in a Chinese population: a population-based study.

Previous reports of comorbid conditions in poliomyelitis survivors mainly focused on some disease categories, such as respiratory diseases, gastrointestinal diseases, psychiatric diseases, neurological diseases and cancer. Data regarding a wide spectrum of medical comorbidities in patients with poliomyelitis is still sparse. This study aimed to investigate and profile the wide range of comorbidities among the survivors of paralytic poliomyelitis in a Chinese population. In total, 2,032 paralytic poliomyelitis patients were selected as the study group and the comparison group consisted of 10,160 randomly selected enrollees. The comorbidities for analysis were based on a modified version of the Elixhauser Comorbidity Index. Conditional logistic regression analyses were computed to investigate the risk of comorbidities for these two groups. As compared to controls, patients with paralytic poliomyelitis had significantly higher prevalence of hypertension, ischemic heart disease, hyperlipidemia, congestive heart failure, cardiac arrhythmias, peripheral vascular disorder, stroke, paralysis, migraines, Parkinson's disease, rheumatoid arthritis, ankylosing spondylitis, pulmonary circulation disorders, chronic pulmonary disease, liver disease, peptic ulcers, hepatitis B or C, deficiency anemias, depression, and lymphoma. Most of the differences are of clinical interest, ORs often being between 2 and 3. No significant difference between poliomyelitis patients and controls was observed in the prevalence of SLE, tuberculosis, alcohol abuse and drug abuse. Our findings demonstrate that survivors of paralytic poliomyelitis in Taiwan are at higher risk of having multiple medical comorbidities although some potential confounding factors including educational level, marital status, obesity and physical activity are not available in our database. The pattern is generally consistent with previous observations from Western populations. Nevertheless, we found several novel associations which have rarely, if ever, been reported previously.

Percutaneous treatment of simple renal cysts with n-butyl cyanoacrylate and iodized oil.

PURPOSE: The aim of this study was to demonstrate the efficacy of n-butyl cyanoacrylate (NBCA) and iodized oil mixture in treatment of simple renal cysts. MATERIALS AND METHODS: Twenty-three patients with 27 symptomatic or large ( > 3 cm) simple cysts were treated by computed tomography (CT)-guided percutaneous aspiration and injection of NBCA and iodized oil mixture. The patients (17 men and 6 women, mean age, 57.2 +/- 10.5 years) were treated with as out-patients. The volume of the treated cysts was calculated with periodic noncontrast enhanced CT examinations 1 and 6 months after the procedure. The procedure was considered successful during follow-up when the diameter of the cyst was measured less than 50% of the initial diameter in CT . RESULTS: The sclerotherapy was technically successful in all patients. The diameter of the cysts ranged between 3.5 and 8 cm (mean, 5.6 +/- 1.3 cm), and 1 and 3.4 cm (mean, 2.1 +/- 0.6 cm) before and after sclerotherapy, respectively (P < 0.001). Average diameter reduction was 62.5% during the follow-up period. The mean follow- up lasted 9.1 months (6-14 months). Flank pain resolved in 20 of 23 symptomatic patients (87%). In three patients, the symptoms decreased slightly. At follow-up CT, the procedure was successful in 25 of 27 cysts (93%). We did not observe any complications related to the procedure. CONCLUSION: Percutaneous sclerotherapy with NBCA and iodized oil mixture was found to be a simple, safe, effective, well tolerated alternative technique for management of simple renal cysts.

Prevalence and correlates of anemia and uncontrolled anemia in chronic hemodialysis patients--the Campania Dialysis Registry.

BACKGROUND: This study investigated prevalence and correlates of anemia and uncontrolled anemia in chronic hemodialysis patients. METHODS: A cross-sectional analysis was performed on registry data for 2,746 chronic (>6 months) hemodialysis patients aged 25-84. Data collection included years of dialysis, hours of dialysis/wk, disease causing hemodialysis, body mass index (BMI), erythropoietin (EPO) treatment, hemoglobin, markers of viral hepatitis, serum albumin, calcium, and phosphorus. RESULTS: Prevalence was 88.7% for anemia (hemoglobin <11 g/100 mL and EPO treatment at any Hb level), 39.4% for uncontrolled anemia (hemoglobin<11 g/100 mL). Gender, years of dialysis, hereditary cystic kidney disease (HCKD), and low BMI (<24 kg/m2) were independent correlates of anemia (P<0.001). Gender, HCKD, low BMI, serum albumin and calcium were independent correlates of uncontrolled anemia (P<0.05). An interaction was found between age (not correlated with anemia and uncontrolled anemia) and the association of gender with uncontrolled anemia (P<0.05). EPO doses were higher in patients with high prevalence of uncontrolled anemia than in patients with low prevalence (i.e., women vs men, other diseases vs HCKD, low vs not-low BMI, P<0.01). Gender, years of dialysis, HCKD, BMI, serum albumin, and calcium were independent correlates of the hemoglobin/EPO dose ratio in patients on EPO treatment (P<0.05). CONCLUSION: Anemia and uncontrolled anemia are more frequent in hemodialysis patients with shortterm dialysis, diseases other than HCKD, low BMI, and female gender. Gender effect was lower in elderly patients. Uncontrolled anemia was also associated with low serum albumin and calcium, suggesting that these parameters are indices of EPO resistance.

[Congenital and genetic related causes of end-stage renal disease--data from Polish Registry of Renal Rreplacement Therapy in Children 2000-2004]. / Genetycznie uwarunkowane i wrodzone choroby nerek prowadzace do schylkowej niewydolnosci nerek--dane z polskiego rejestru dzieci leczonych nerkozastepczo (2000-2004).

UNLABELLED: One of the objectives of Polish Registry of Renal Replacement Therapy in Children established on 31st Dec. 2000 was to collect complete data on etiology of end stage renal disease (ESRD) in polish children. MATERIAL AND METHODS: Data on 469 patients (251 boys, 218 girls) aged 0-22 years treated with renal replacement therapy (RRT) at 13 pediatric dialysis units in Poland from 2000 to 2004 were analyzed. The mean age at start of dialysis was 10 years and 3 months. Renal diseases were defined according to EDTA coding system. Data is presented for the whole group, in 5-year age groups and separately for both sexes. RESULTS: Congenital and genetic renal diseases were the cause of ESRF in 56% of the polish population of children and adolescents on RRT. 39% of causes were acquired diseases, 5% remained unidentified. Congenital and genetic causes dominated in children < 5 years of age (71%). They accounted for 49%, 61% and 45% of causes in the consecutive 5-year age groups. The most numerous group of congenital diseases leading to ESRF were uropathies 37% and 25% of causes in the consecutive age groups. In boys the most frequent uropathy was obstructive uropathy (25%), the majority caused by posterior urethral valves. In girls the most frequent uropathies were reflux nephropathy (10%) and nephropathy secondary to neurogenic bladder (9%). Uropathies were followed by renal hypo-dysplasia without urinary tract anomalies (11%) and cystic diseases (10%). CONCLUSIONS: Congenital kidney anomalies and genetic diseases are the leading cause of end-stage renal disease in children up to 15 years of age.

Riñón multicístico: evaluación del riñón contralateral / Multicystic kidney: an evaluation of the contralateral kidney

El riñon multicístico (RMC) se asocia a malformación del riñon contralateral en porcentajes variables. Con el propósito de determinar la prevalencia y tipo de malformaciones asociadas, y la evolución de la funsión renal, se estudiaron de manera retrospectiva 34 niños con diagnóstico de RMC entre 1 día y 13 meses de edad con ecotomografía renal, cintigrama renal Tc99 DMSA y uretrocistogrfía miccional. Se encontró malformación contralateral en 11/34 pacientes (32 por ciento) siendo las más frecuentes la estenosis pieloureteral y el reflujo vesicoureteral. En 5 pacientes el seguimiento demostró signos de displasia renal y en 1 hipertensión arterial. No hubo malignización. Se concluye que la malformación del riñon contralateral es de alta frecuencia, por lo que siempre debe realizarse el estudio completo con ecotomografía renal, cintigrama renal Tc99 DMSA y urectrocistografía. Debe además efectuarse control seriado del cercimiento y funsión renal, para la detección y manejo precoz de la insuficiencia renal

Creatine supplementation increases renal disease progression in Han:SPRD-cy rats.

The growing use of creatine as a potential ergogenic aid among active individuals has raised concern regarding its effects on the kidney, particularly among those individuals with compromised renal function. The object of this study is to investigate the effects of oral creatine supplementation in an accepted animal model of renal cystic disease. Han:Sprague-Dawley (SPRD)-cy rats with cystic kidney disease were administered a creatine supplement at a loading dose of 2.0 g/kg of diet for 1 week, followed by 5 weeks during which the dose was one fifth this amount, mimicking typical human consumption on a body-weight basis. Cystic kidney disease progression was assessed by measuring kidney size and fluid content and determining cyst scores. Renal function was assessed by measuring serum urea and creatinine concentrations and creatinine clearance. Creatine supplementation resulted in greater cyst growth and worsened renal function in the Han:SPRD-cy rat, evidenced by greater kidney weights (2.87 +/- 0.08 versus 2.61 +/- 0.09 g/100 g of body weight; P: = 0.0365), renal fluid contents (89.22 +/- 0.41 versus 87.38 +/- 0.48 g/100 g of kidney weight; P: = 0.0057), cyst scores (0.49 +/- 0.02 versus 0.40 +/- 0.03; P: = 0.0167) and serum urea concentrations (23.96 +/- 0.92 versus 20.65 +/- 1.06 mmol/L; P: = 0.0230), and lower creatinine clearances (0.125 +/- 0.098 versus 0.162 +/- 0.011 mL/min/100 g of body weight; P: = 0.0159). These results indicate that creatine supplements may exacerbate disease progression in an animal model of cystic renal disease. Although systematic research of the effects of creatine supplementation in humans with compromised renal function is awaited, it follows that creatine should be used with particular caution in individuals with or at risk for renal disease.

[The percutaneous x-ray endoscopic surgery of simple kidney cysts]. / Perkutannaia rentgenoéndoskopicheskaia khirurgiia prostykh kist pochki.

An endoscopical method of treating solitary renal cysts (RC) > 5 cm in diameter has been used in the Research Institute of Urology since 1989. The method implies percutaneous puncture of the cyst controlled by x-ray television or ultrasound, dilatation of the cystostomy fistula, cystoscopy followed by resection or dissection of the cyst wall with a cold knife or electrosurgically. A total of 38 patients with large solitary cysts have been treated. A 6-12-month follow-up revealed no recurrences. Complications of the surgery were not reported. The method is easy to accomplish, effective and well tolerated.

Combined tubular dysfunction in medullary cystic disease.

A patient with medullary cystic disease presented with a combined tubular dysfunction, including severe salt wasting, renal tubular acidosis types I and IV, and marked aldosterone resistance. High-dose mineralocorticoid treatment partially corrected the defect in potassium excretion and did not affect natriuresis. Plasma aldosterone level was more than 30 times the upper normal level and was decreased but not normalized by captopril administration and volume expansion. The severe hemodynamic and metabolic consequences of these defects were corrected by renal transplantation.

[Therapeutic results of ultrasonically-guided kidney cyst punctures]. / Therapeutische Ergebnisse der ultraschallgeführten Nierenzystenpunktion.

The behaviour of renal cysts following puncture was studied in 62 patients on whom a simple diagnostic puncture had been performed and on fourteen patients who, in addition, had some of their own blood injected into the cyst. Twenty-one patients (34%) of the first group and eight patients (57%) of the second group showed definite reduction in the size of the renal cyst after an average period of observation of fifteen months. In 7%, the cyst disappeared completely following puncture. Parapelvic cysts showed much less tendency to disappear than did cortical cysts. The results of injecting autologous blood are comparable with those of injecting lipid soluble contrast media as a sclerosing agent. Before surgical removal of a symptomatic renal cyst is contemplated, puncture and evacuation of the cyst with autologous blood injection is recommended.