Publications by authors named "Klee P"

Purpose: While most small renal masses (SRM) < 4 cm have an excellent prognosis following resection, the impact of adverse T3a pathologic features on oncologic outcomes of SRMs remains unclear. We sought to compare clinical outcomes for surgically resected pT3a versus pT1a SRMs at our institution.

Materials And Methods: We retrospectively reviewed records of patients who underwent radical or partial nephrectomy (RN, PN) for renal tumors <4 cm at our institution between 2010 and 2020.

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Over the past decade, the efficiency of photovoltaic devices based on CHNHPbI have dramatically increased. This has driven research efforts in all areas, from the discovery of materials to film processing to long-term device stability studies. Here, we report the synthesis and structure of three new "zero dimensional" organic-inorganic metal halides which use the meta-xylylenediammonium (MXD) cation: MXDPbI, MXDBiI, and (MXD)BiBr·2HO.

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Unrestrained ketogenesis leads to life-threatening ketoacidosis whose incidence is high in patients with diabetes. While insulin therapy reduces ketogenesis this approach is sub-optimal. Here, we report an insulin-independent pathway able to normalize diabetic ketogenesis.

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  • Lactate levels indicate oxygen delivery issues, and elevated lactate (hyperlactatemia) in children after heart surgery may predict poorer outcomes.
  • A study in a pediatric critical care unit involved 93 patients aged 6 months to 16 years, who underwent surgery with cardiopulmonary bypass, assessing metabolic markers and oxygen extraction at various points.
  • Results showed that 62% had hyperlactatemia 4 hours after admission, linked to high blood glucose levels, but no significant relationship with oxygen extraction, suggesting different underlying mechanisms at play.
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Background: Perrault syndrome is a rare recessive and genetically heterogeneous disorder characterized by sensorineural hearing loss in males and females and gonadal dysgenesis in females. Mutations in seven different genes have been identified: HARS2, HSD17B4, CLLP, C10orf, ERAL1, TWNK and LARS2. To date, 19 variants have been reported in 18 individuals with LARS2-Perrault syndrome.

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Globally it is estimated that over 1 million children and adolescents have Type 1 diabetes with large variations in incidence between different contexts. Health systems need to provide a variety of elements to ensure appropriate diabetes care, such as service delivery; healthcare workforce; information; medical products and technologies; financing and leadership and governance. Describing these elements between Geneva, Switzerland, a high-income country with high spending on healthcare and a large density of doctors, and low- and middle-income countries this article aims to highlight the global inequality of diabetes care.

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  • * In a study of 1,209 youth with diabetes in Lithuania, 40.7% of antibody-negative patients screened for genetic variants were found to have MD, with the highest prevalence in infants and decreased percentages in older age groups.
  • * The research revealed an overall MD prevalence of 3.5% and identified likely pathogenic variants in 11 additional genes, while effective treatment changes were successful for over 50% of eligible patients, particularly those with high C-peptide levels.
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Background: A previous randomised controlled trial (RCT) by our group found that robotic assisted unicompartmental knee arthroplasty (UKA) surgery was significantly more accurate than conventional instrumentation. The aim of this study was to determine whether a low-cost novel PSI design could deliver the same level of accuracy as the robot in the same time efficient manner as conventional instruments.

Methods: Thirty patients undergoing medial UKA took part.

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Background: Prevention of type 1 diabetes mellitus (T1DM)-related complications is dependent on metabolic control. The recommended glycated hemoglobin (HbA1c) values <7.5% (58.

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Background: The study aimed to assess accuracy, satisfaction and usability of a flash glucose monitoring system (FGM) in children and adolescents with type 1 diabetes mellitus (T1DM) attending a diabetes summer camp.

Methods: Sixty-six children and adolescents with T1DM aged 6 to 17 years participating in a 7-day medically supervised summer camp were enrolled. Capillary blood glucose (BG) and flash glucose (FG) values were measured simultaneously at breakfast, lunch, and dinner and for any given FG value <72 mg/dL (<4.

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Background: Cystic fibrosis-related diabetes (CFRD) is the most frequent extrapulmonary complication of cystic fibrosis (CF).

Methods: We report the first combined pancreatic islet-lung-liver transplantation in a 14-year-old adolescent. CFTR was analyzed by Sanger sequencing.

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Objectives: Hyperglycemia after cardiac surgery and cardiopulmonary bypass in children has been associated with worse outcome; however, causality has never been proven. Furthermore, the benefit of tight glycemic control is inconsistent. The purpose of this study was to describe the metabolic constellation of children before, during, and after cardiopulmonary bypass, in order to identify a subset of patients that might benefit from insulin treatment.

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Type 1 diabetes (T1DM) results from destruction of most insulin-secreting pancreatic β-cells. The persistence of β-cells decades after the onset of the disease indicates that the resistance of individual cells to the autoimmune insult is heterogeneous and might depend on the metabolic status of a cell at a given moment. The aim of this study is to investigate whether activation of nicotinic acetylcholine receptors (nACh-Rs) could increase β-cell resistance against the adverse environment prevailing at the onset of T1DM.

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  • Diabetic striatopathy is a recognized complication of diabetes primarily seen in adults, with very few cases reported in children.
  • This case study details a teenager diagnosed with diabetic striatopathy experiencing hemichorea-hemiballism alongside symptoms like weight loss and increased thirst and urination.
  • The patient's glycemia control led to quick recovery, despite the presence of confirmed striatal lesions visible through MRI, and the study also explores potential underlying mechanisms of this condition.
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  • Transient and permanent neonatal diabetes mellitus (NDM) are rare conditions that occur in infants, typically diagnosed within the first 6 months of life and linked to single gene defects rather than type 1 diabetes.
  • * Genetic analysis revealed a novel variant in the HNF4A gene in a very low birth weight neonate who developed diabetes shortly after birth but achieved normal blood sugar levels after one month of insulin treatment.
  • * The study concludes that the unique HNF4A variant associated with transient NDM may have been influenced by an additional variant in the KCNJ11 gene and the infant's premature birth.
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Cell-to-cell communication mediated by gap junctions made of Connexin36 (Cx36) contributes to pancreatic β-cell function. We have recently demonstrated that Cx36 also supports β-cell survival by a still unclear mechanism. Using specific Cx36 siRNAs or adenoviral vectors, we now show that Cx36 downregulation promotes apoptosis in INS-1E cells exposed to the pro-inflammatory cytokines (IL-1β, TNF-α and IFN-γ) involved at the onset of type 1 diabetes, whereas Cx36 overexpression protects against this effect.

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  • * Researchers studied the effects of IUGR in rat pups using two methods: exposure to synthetic glucocorticoids and prenatal undernutrition, assessing their physiological and molecular changes early in life.
  • * Findings showed that both groups of IUGR pups experienced significant metabolic issues like hyperglycemia and altered insulin responses, highlighting early defects that could lead to future metabolic disorders.
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  • SRY gene on the Y chromosome is crucial for sex determination, and its mutations account for 10-15% of cases of 46,XY gonadal dysgenesis (GD), but are not commonly linked to mixed GD.
  • A novel SRY point mutation was discovered in a patient with mixed GD characterized by specific hormonal and histological findings in the gonads, including a streak gonad and a dysgenetic testis.
  • The SRY mutation led to uneven activation in the gonads, suggesting that local factors might influence how SRY functions or is expressed.
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Aim: ATP-sensitive potassium channels are important regulators of insulin secretion. They consist of four sulphonylurea receptor (encoded by ABCC8) and four inwardly rectifying protein (encoded by KCNJ11) subunits. Activating ABCC8 mutations lead to decreased insulin secretion and to diabetes.

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Type 1 diabetes develops when most insulin-producing β cells of the pancreas are killed by an autoimmune attack. The in vivo conditions modulating the sensitivity and resistance of β cells to this attack remain largely obscure. Here, we show that connexin 36 (Cx36), a trans-membrane protein that forms gap junctions between β cells in the pancreatic islets, protects mouse β cells against both cytotoxic drugs and cytokines that prevail in the islet environment at the onset of type 1 diabetes.

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The advent of multicellular organisms was accompanied by the development of short- and long-range chemical signalling systems, including those provided by the nervous and endocrine systems. In turn, the cells of these two systems have developed mechanisms for interacting with both adjacent and distant cells. With evolution, such mechanisms have diversified to become integrated in a complex regulatory network, whereby individual endocrine and neuro-endocrine cells sense the state of activity of their neighbors and, accordingly, regulate their own level of functioning.

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Diabetes develops when the insulin needs of peripheral cells exceed the availability or action of the hormone. This situation results from the death of most beta-cells in type 1 diabetes, and from an inability of the beta-cell mass to adapt to increasing insulin needs in type 2 and gestational diabetes. We analyzed several lines of transgenic mice and showed that connexins (Cxs), the transmembrane proteins that form gap junctions, are implicated in the modulation of the beta-cell mass.

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Fetal adverse environment, such as insufficient maternal nutrition, placental insufficiency and stress, alters organ development and leads to poor fetal growth, also called intrauterine growth retardation (IUGR). IUGR is associated with an increased risk of perinatal mortality and morbidity as well as late-onset metabolic diseases, such as obesity, diabetes and hypertension in adulthood. In the rodent model, IUGR can be induced by fetal caloric restriction, fetal protein restriction, by exposure to high levels of glucocorticoids or by restricted placental blood supply.

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The emergence of pancreatic islets has necessitated the development of a signalling system for the intra- and inter-islet coordination of beta cells. With evolution, this system has evolved into a complex regulatory network of partially cross-talking pathways, whereby individual cells sense the state of activity of their neighbours and, accordingly, regulate their own level of functioning. A consistent feature of this network in vertebrates is the expression of connexin (Cx)-36-made cell-to-cell channels, which cluster at gap junction domains of the cell membrane, and which adjacent beta cells use to share cytoplasmic ions and small metabolites within individual islets.

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