- Food and Food Products on the Italian Market for Ketogenic Dietary Treatment of Neurological Diseases. [Review]
- NNutrients 2019 May 17; 11(5)
- The ketogenic diet (KD) is the first line intervention for glucose transporter 1 deficiency syndrome and pyruvate dehydrogenase deficiency, and is recommended for refractory epilepsy. It is a normo-c…
The ketogenic diet (KD) is the first line intervention for glucose transporter 1 deficiency syndrome and pyruvate dehydrogenase deficiency, and is recommended for refractory epilepsy. It is a normo-caloric, high-fat, adequate-protein, and low-carbohydrate diet aimed at switching the brain metabolism from glucose dependence to the utilization of ketone bodies. Several variants of KD are currently available. Depending on the variant, KDs require the almost total exclusion, or a limited consumption of carbohydrates. Thus, there is total avoidance, or a limited consumption of cereal-based foods, and a reduction in fruit and vegetable intake. KDs, especially the more restrictive variants, are characterized by low variability, palatability, and tolerability, as well as by side-effects, like gastrointestinal disorders, nephrolithiasis, growth retardation, hyperlipidemia, and mineral and vitamin deficiency. In recent years, in an effort to improve the quality of life of patients on KDs, food companies have started to develop, and commercialize, several food products specific for such patients. This review summarizes the foods themselves, including sweeteners, and food products currently available for the ketogenic dietary treatment of neurological diseases. It describes the nutritional characteristics and gives indications for the use of the different products, taking into account their metabolic and health effects.
- Nephrolithiasis in the Obese Patient. [Review]
- CUCurr Urol Rep 2019 May 18; 20(7):36
- The link between metabolic syndrome (MetS)/obesity and kidney stone disease (KSD) has gained importance over recent years due to the increasing prevalence and healthcare burden worldwide. This review…
The link between metabolic syndrome (MetS)/obesity and kidney stone disease (KSD) has gained importance over recent years due to the increasing prevalence and healthcare burden worldwide. This review analyses the literature exploring the link between MetS/obesity and KSD and the impact that obesity has on KSD management.
- Thermal effects of Ho:YAG laser lithotripsy during retrograde intrarenal surgery and percutaneous nephrolithotomy in an ex vivo porcine kidney model. [Journal Article]
- WJWorld J Urol 2019 May 16
- CONCLUSIONS: Ho:YAG laser lithotripsy bears the risk of thermal damages to the urinary tract even at low-power settings if inadequate irrigation is applied. Sufficient irrigation is mandatory to perform safe Ho:YAG laser lithotripsy. Based on the results, we developed a formula calculating the approximate ΔT for irrigation rates ≥ 30 ml/min: ΔT = 15 K × (power [W]/irrigation [ml/min]).
- Management of primary hyperparathyroidism in pregnancy: a case series. [Journal Article]
- EDEndocrinol Diabetes Metab Case Rep 2019 May 16; 2019
- Primary hyperparathyroidism (PHPT) is characterised by the overproduction of parathyroid hormone (PTH) due to parathyroid hyperplasia, adenoma or carcinoma and results in hypercalcaemia and a raised …
Primary hyperparathyroidism (PHPT) is characterised by the overproduction of parathyroid hormone (PTH) due to parathyroid hyperplasia, adenoma or carcinoma and results in hypercalcaemia and a raised or inappropriately normal PTH. Symptoms of hypercalcaemia occur in 20% of patients and include fatigue, nausea, constipation, depression, renal impairment and cardiac arrythmias. In the most severe cases, uraemia, coma or cardiac arrest can result. Primary hyperparathyroidism in pregnancy is rare, with a reported incidence of 1%. Maternal and fetal/neonatal complications are estimated to occur in 67 and 80% of untreated cases respectively. Maternal complications include nephrolithiasis, pancreatitis, hyperemesis gravidarum, pre-eclampsia and hypercalcemic crises. Fetal complications include intrauterine growth restriction; preterm delivery and a three to five-fold increased risk of miscarriage. There is a direct relationship between the degree of severity of hypercalcaemia and miscarriage risk, with miscarriage being more common in those patients with a serum calcium greater than 2.85 mmol/L. Neonatal complications include hypocalcemia. Herein, we present a case series of three women who were diagnosed with primary hyperparathyroidism in pregnancy. Case 1 was diagnosed with multiple endocrine neoplasia type 1 (MEN1) in pregnancy and required a bilateral neck exploration and subtotal parathyroidectomy in the second trimester of her pregnancy due to symptomatic severe hypercalcaemia. Both case 2 and case 3 were diagnosed with primary hyperparathyroidism due to a parathyroid adenoma and required a unilateral parathyroidectomy in the second trimester. This case series highlights the work-up and the tailored management approach to patients with primary hyperparathyroidism in pregnancy. Learning points: Primary hyperparathyroidism in pregnancy is associated with a high incidence of associated maternal fetal and neonatal complications directly proportionate to degree of maternal serum calcium levels. Parathyroidectomy is the definitive treatment for primary hyperparathyroidism in pregnancy and was used in the management of all three cases in this series. It is recommended when serum calcium is persistently greater than 2.75 mmol/L and or for the management of maternal or fetal complications of hypercalcaemia. Surgical management, when necessary is ideally performed in the second trimester. Primary hyperparathyroidism is genetically determined in ~10% of cases, where the likelihood is increased in those under 40 years, where there is relevant family history and those with other related endocrinopathies. Genetic testing is a useful diagnostic adjunct and can guide treatment and management options for patients diagnosed with primary hyperparathyroidism in pregnancy, as described in case 1 in this series, who was diagnosed with MEN1 syndrome. Women of reproductive age with primary hyperparathyroidism need to be informed of the risks and complications associated with primary hyperparathyroidism in pregnancy and pregnancy should be deferred and or avoided until curative surgery has been performed and calcium levels have normalised.
- Risk factors for development of urinary tract infection in children with nephrolithiasis. [Journal Article]
- JPJ Paediatr Child Health 2019 May 14
- CONCLUSIONS: The age at diagnosis, the presence of a metabolic risk factor and size of stone are significant risk factors for UTI in children with nephrolithiasis. The patients with idiopathic hypercalciuria, metabolic risk factor and age at diagnosis under 2 years have increased risk of recurrence of UTI.
- Hereditary Hypercalcemia Caused by a Homozygous Pathogenic Variant in the CYP24A1 Gene: A Case Report and Review of the Literature. [Case Reports]
- CRCase Rep Endocrinol 2019; 2019:4982621
- CONCLUSIONS: CYP24A1 gene mutations should be considered in cases of PTH-independent hypercalcemia, once that more common causes (hypercalcemia of malignancy, granulomatous diseases, and vitamin D intoxication) have been ruled out.
- Evaluation of the efficacy of Phyllanthus niruri standardized extract combined with magnesium and vitamin B6 for the treatment of patients with uncomplicated nephrolithiasis. [Journal Article]
- MPMed Pharm Rep 2019; 92(2):153-157
- CONCLUSIONS: The current treatment had the highest efficacy in achieving stone-free status for patients with calculi ≤ 3 mm, located in the middle or upper calyx. A higher duration of the treatment might show improved results.
- Distal renal tubular acidosis: genetic causes and management. [Review]
- WJWorld J Pediatr 2019 May 11
- CONCLUSIONS: dRTA is a multifactorial disease leading to several clinical manifestations. Clinical and laboratory alterations can be corrected by alkali replacement therapy.
- Kidney stone compositions and frequencies in a Norwegian population. [Journal Article]
- SJScand J Urol 2019 May 09; :1-6
- CONCLUSIONS: Frequencies of kidney stone types in this Norwegian population are mainly in accordance with other studies, except a large increase in UA stones over four decades, partly caused by a particularly low frequency of UA stones in the old study, a decreased carbonate apatite frequency over four decades, and an unaltered brushite frequency. Also, in contrast to other studies, a relatively small and non-significant male UA stone predominance was found.
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- Nephrocalcinosis and Nephrolithiasis in X-Linked Hypophosphatemic Rickets: Diagnostic Imaging and Risk Factors. [Journal Article]
- JEJ Endocr Soc 2019 May 01; 3(5):1053-1061
- CONCLUSIONS: NC was more prevalent than NL. The main metabolic factor was hyperphosphaturia, and intensive phosphate treatment appears to be a worsening factor for kidney calcification.