The Green River of Kentucky

Cutting a wide east-west swath from the Appalachian foothills to the heart of the western Kentucky coalfields, the Green River valley extends from below the Tennessee border in the south to the Ohio River in the north. The Green River of Kentucky presents a picture of the unity and diversity of the people living in the Green River valley. Helen Bartter Crocker finds that each generation of its people approached the river in a distinctive way. Early settlers used the river simply as it was—crooked and narrow with an unpredictable water flow, and navigable only under high-water conditions. The sons of these pioneers were interested in bringing steamboats to the valley; until they succeeded in persuading the state legislature to improve the Green River and its tributary, the Barren, by a series of locks and dams, however, volunteers would work—often up to their necks in water—until they cleared the river sufficiently to allow steamers to reach Bowling Green at high water. When the locks and dams were reopened following the Civil War, a local private corporation gained a near-monopoly of the river trade. Public outcry against this private ownership caused the federal government to take control, and through the Corps of Engineers, to undertake extensive river improvements. After the Great Depression, when trade was almost at a standstill, additional federal funds were appropriated for flood-control dams in the upper river and modern locks in the lower river to harness the valley’s industrial potential. These opened up coal barging and recreational facilities, which ensured the future economic well being of the Green River valley. Helen Bartter Crocker is assistant professor of history at Western Kentucky University.https://uknowledge.uky.edu/upk_united_states_history/1033/thumbnail.jp

Green River Steamboating a Cultural History, 1828-1931

In recent years, historians have displayed a growing interest in the cultural development of certain well-defined regions. Often a river valley inspired such a study, for example, R.E. Banta’s The Ohio, Thomas Clark’s The Kentucky and Harriette Arnow’s Seedtime on the Cumberland. These and many other river histories dealt less with the river itself than with its tendency to define and alter an area’s culture This thesis, dealing with the culture of Green River’s steamboat era, is less about the steamboat or Green River than it is about their effect on river people. It searches the area’s homes, schools, business establishments, churches, military and recreational activities from 1828 to 1931. This century is viewed through the records of four generations who know the steamboat intimately. It examines birth and death in Green River country and the sung that were sung in between

The adrenal cortex and the kidney

The adrenal cortex regulates renal function in a number of important ways; indeed, normal renal function cannot be understood without recognition of such regulation. Well-recognized examples of such regulation are the control of body fluid tonicity through regulation of urinary solute concentration-a function controlled “primarily” by vasopressin, but secondarily and importantly by the adrenal cortex-and control of body sodium-a function controlled primarily by renal tubular sodium reabsorption but regulated by sodium-retaining steroids.The kidney can regulate adrenal function by changing reabsorption of sodium and secretion of potassium, and also by release of renin. The primary target of such regulation is the secretion of aldosterone, which may be influenced by body fluid volume, potassium ion and angiotensin II.Because of these interrelationships, the pathophysiology of certain disease states may be described as aberrations in feedback loops between adrenal cortex and kidney. In this paper we will consider this “system” in some detail, and attempt to explain four disorders as examples of errors in control.In the form of “primary” aldosteronism resulting from hyperplasia of all adrenal cortical tissue, overproduction of aldosterone persists in the absence of all known stimulatory factors. In renovascular hypertension, angiotensin and aldosterone production may persist despite systemic hypertension. In the non-salt-losing form of the adrenogenital syndrome of congenital adrenal hyperplasia without treatment, failure of feedback inhibition by Cortisol may result in overproduction of adrenocorticotropic hormone (ACTH) which, in turn, may lead to overproduction of progesterone. Progesterone may cause sodium loss and overproduction of renin and aldosterone while blocking their effects. In the syndrome of juxtaglomerular hyperplasia with normal blood pressure, overproduction of renin may result from unresponsiveness of blood vessels leading to a lack of feedback inhibition by pressure rise. Under certain circumstances sodium loss can potentiate both the overproduction and the unresponsiveness. Excessive renin leads to aldosteronism and potassium loss

Syndrome of Inappropriate Antidiuretic Hormone (SIADH) Secretion Caused by Squamous Cell Carcinoma of the Nasopharynx: Case Report

The Paraneoplastic syndromes include the disorders that accompany benign or malignant tumors but are not directly related to mass effects or invasion by the primary tumor or its metastases. Neoplastic cells can produce a variety of peptides that exert biologic actions at local and distant sites and can elicit responses that cause a variety of hormonal, hematologic, dermatologic and neurologic symptoms. Almost every type of malignancy has the potential to produce hormones or cytokines or to induce immunologic responses. Lung cancers, both non-small cell and small cell, are capable of producing a variety of paraneoplastic syndromes. The majority of such syndromes are caused by small cell carcinomas, including many endocrinopathies. Syndrome of inappropriate antidiuretic hormone (SIADH) has been commonly associated with small cell carcinoma and is often seen in these patients. However, SIADH associated with squamous cell carcinoma has rarely been reported on, and the mechanism for this rare association is still unknown. We present here a case of a 77-yr-old man who developed SIADH caused by squamous cell carcinoma of the nasopharynx

A case of Bartter syndrome type I with atypical presentations

Bartter syndrome (BS) is an autosomal recessively inherited rare renal tubular disorder characterized by hypokalemic metabolic alkalosis and hyperreninemic hyperaldosteronism with normal to low blood pressure due to a renal loss of sodium. Genetically, BS is classified into 5 subtypes according to the underlying genetic defects, and BS is clinically categorized into antenatal BS and classical BS according to onset age. BS type I is caused by loss-of-function mutations in the SLC12A1 gene and usually manifests as antenatal BS. This report concerns a male patient with compound heterozygous missense mutations on SLC12A1 (p.C436Y and p.L560P) and atypical clinical and laboratory features. The patient had low urinary sodium and chloride levels without definite metabolic alkalosis until the age of 32 months, which led to confusion between BS and nephrogenic diabetes insipidus (NDI). In addition, the clinical onset of the patient was far beyond the neonatal period. Genetic study eventually led to the diagnosis of BS type I. The low urinary sodium and chloride concentrations may be caused by secondary NDI, and the later onset may suggest the existence of a genotype-phenotype correlation

Giant pulmonary artery aneurysm in a patient with vasoreactive pulmonary hypertension: a case report

<p>Abstract</p> <p>Background</p> <p>Pulmonary artery aneurysms are a rare condition, frequently associated with pulmonary hypertension. However, the evolution and treatment of this pathology is still not clear.</p> <p>Case Presentation</p> <p>The authors report a case of a 65-year old patient with pulmonary artery aneurysm associated with pulmonary arterial hypertension. Due to a positive vasoreactivity test, treatment with calcium channel blockers was started with near normalization of the right cardiac pressures. Nevertheless, after 20 months of treatment, the pulmonary artery aneurysm size remained unchanged with an associated severe pulmonary regurgitation and causing extrinsic compression of the main left coronary artery. Surgical correction was successfully performed.</p> <p>Conclusions</p> <p>This is the first case report of a pulmonary artery aneurysm described to be associated with vasoreactive pulmonary hypertension in a living patient. Although medical therapy for pulmonary hypertension was started, surgical correction of the aneurysm was executed in order to prevent its future complications.</p

Mimicry and well known genetic friends: molecular diagnosis in an Iranian cohort of suspected Bartter syndrome and proposition of an algorithm for clinical differential diagnosis.

BACKGROUND: Bartter Syndrome is a rare, genetically heterogeneous, mainly autosomal recessively inherited condition characterized by hypochloremic hypokalemic metabolic alkalosis. Mutations in several genes encoding for ion channels localizing to the renal tubules including SLC12A1, KCNJ1, BSND, CLCNKA, CLCNKB, MAGED2 and CASR have been identified as underlying molecular cause. No genetically defined cases have been described in the Iranian population to date. Like for other rare genetic disorders, implementation of Next Generation Sequencing (NGS) technologies has greatly facilitated genetic diagnostics and counseling over the last years. In this study, we describe the clinical, biochemical and genetic characteristics of patients from 15 Iranian families with a clinical diagnosis of Bartter Syndrome. RESULTS: Age range of patients included in this study was 3 months to 6 years and all patients showed hypokalemic metabolic alkalosis. 3 patients additionally displayed hypercalciuria, with evidence of nephrocalcinosis in one case. Screening by Whole Exome Sequencing (WES) and long range PCR revealed that 12/17 patients (70%) had a deletion of the entire CLCNKB gene that was previously identified as the most common cause of Bartter Syndrome in other populations. 4/17 individuals (approximately 25% of cases) were found to suffer in fact from pseudo-Bartter syndrome resulting from congenital chloride diarrhea due to a novel homozygous mutation in the SLC26A3 gene, Pendred syndrome due to a known homozygous mutation in SLC26A4, Cystic Fibrosis (CF) due to a novel mutation in CFTR and apparent mineralocorticoid excess syndrome due to a novel homozygous loss of function mutation in HSD11B2 gene. 1 case (5%) remained unsolved. CONCLUSIONS: Our findings demonstrate deletion of CLCNKB is the most common cause of Bartter syndrome in Iranian patients and we show that age of onset of clinical symptoms as well as clinical features amongst those patients are variable. Further, using WES we were able to prove that nearly 1/4 patients in fact suffered from Pseudo-Bartter Syndrome, reversing the initial clinical diagnosis with important impact on the subsequent treatment and clinical follow up pathway. Finally, we propose an algorithm for clinical differential diagnosis of Bartter Syndrome

ACTH-Bestimmungen im Plasma aus dem Bulbus cranialis venae jugularis

Der Anstieg der Corticosteroninkretion in das Nebennierenvenenblut frisch hypophysektomierter Ratten diente zur Bestimmung von ACTH-Spiegeln in 1 ml nativen, menschlichen Plasma. Normale ACTH-Plasmaspiegel sind sowohl bei Punktion der Vena cubitalis als auch des Bulbus cranialis venae jugularis durch diese Methode nicht oder nur ungenau zu erfassen. Bei Patienten mit pathologisch erhöhten ACTH-Spiegeln in der Vena cubitalis sind die ACTH-Spiegel im Bulbus cranialis venae jugularis signifikant höher. Es ließ sich eine Beziehung zwischen ACTH-Spiegel in der Peripherie (Vena cubitalis), Differenz der ACTH-Spiegel zwischen Bulbus cranialis venae jugularis und Vena cubitalis und biologischer Halbwertszeit von endogenem ACTH aufstellen. Nach den Ergebnissen der Bestimmung von ACTH-Spiegeln bei Nebennierengesunden läßt sich folgern, daß die biologische Halbwertszeit von endogenem ACTH größer als 4 min sein muß. Bei Patienten mit erhöhten ACTH-Spiegeln ließ sich die biologische Halbwertszeit von endogenem ACTH größenordnungsmäßig mit ca. 40 min berechnen. Bei diesen Patienten betrug die mittlere tägliche ACTH-Inkretion ca. 100 E.ACTH-contents of 1 ml specimens of human plasma were assayed by measurement of increases of corticosterone output in the adrenal vein of acutely hypophysectomized rats. This procedure is not sensitive enough to measure normal ACTH-levels acurately, neither when blood was drawn from the bulbus cranialis venae jugularis, nor from the vena cubitalis. In patients having pathologically elevated ACTH-levels, the ACTH-content of plasma is significantly higher in the bulbus cranialis venae jugularis than in peripheral venous blood. An equation is presented formulating the relation of peripheral ACTH-levels, differences of ACTH-levels between bulbus cranialis venae jugularis and vena cubitalis, and of the biological halflife of endogenous ACTH. On the basis of the results of the determinations of socalled normal ACTH-levels it can be concluded, that the biological halflife of endogenous ACTH is longer than 4 min. From the data of patients with elevated ACTH-levels a halflife of approximately 40 min and a mean ACTH-secretion of approx. 100 units per day could be calculated

Bartter- and Gitelman-like syndromes: salt-losing tubulopathies with loop or DCT defects

Salt-losing tubulopathies with secondary hyperaldosteronism (SLT) comprise a set of well-defined inherited tubular disorders. Two segments along the distal nephron are primarily involved in the pathogenesis of SLTs: the thick ascending limb of Henle’s loop, and the distal convoluted tubule (DCT). The functions of these pre- and postmacula densa segments are quite distinct, and this has a major impact on the clinical presentation of loop and DCT disorders – the Bartter- and Gitelman-like syndromes. Defects in the water-impermeable thick ascending limb, with its greater salt reabsorption capacity, lead to major salt and water losses similar to the effect of loop diuretics. In contrast, defects in the DCT, with its minor capacity of salt reabsorption and its crucial role in fine-tuning of urinary calcium and magnesium excretion, provoke more chronic solute imbalances similar to the effects of chronic treatment with thiazides. The most severe disorder is a combination of a loop and DCT disorder similar to the enhanced diuretic effect of a co-medication of loop diuretics with thiazides. Besides salt and water supplementation, prostaglandin E2-synthase inhibition is the most effective therapeutic option in polyuric loop disorders (e.g., pure furosemide and mixed furosemide–amiloride type), especially in preterm infants with severe volume depletion. In DCT disorders (e.g., pure thiazide and mixed thiazide–furosemide type), renin–angiotensin–aldosterone system (RAAS) blockers might be indicated after salt, potassium, and magnesium supplementation are deemed insufficient. It appears that in most patients with SLT, a combination of solute supplementation with some drug treatment (e.g., indomethacin) is needed for a lifetime