Pseudohypoaldosteronism type 2 (PHA2) is a rare inherited condition of altered tubular salt handling. It is characterized by the specific constellation of hyperkalaemic hyporeninemic hypertension, hyperchloremic metabolic acidosis and hypercalciuria. Molecular genetic testing confirms the diagnosis in the majority of cases. Thiazides constitute effective treatment. Due to its rarity, the diagnosis is often delayed. We here present two children with PHA2, who were initially treated with fludrocortisone and bicarbonate complicated mainly by exacerbation of their hypertension. Discontinuation of their previous therapy and commencement of thiazide diuretics led to normalisation of their blood pressure and electrolyte and acid-base status.
- MeSH
- Acidosis * diagnosis etiology MeSH
- Child MeSH
- Fludrocortisone therapeutic use MeSH
- Hyperkalemia diagnosis etiology genetics blood MeSH
- Hypertension * diagnosis etiology drug therapy genetics MeSH
- Sodium Chloride Symporter Inhibitors therapeutic use MeSH
- Blood Pressure MeSH
- Humans MeSH
- Pseudohypoaldosteronism * genetics diagnosis physiopathology MeSH
- Check Tag
- Child MeSH
- Humans MeSH
- Male MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Case Reports MeSH
Diabetická ketoacidóza (DKA) a hyperglykemický hyperosmolární stav (HHS) jsou nejzávažnější hyperglykemické stavy u pacientů s diabetem. V roce 2024 byla publikována po 15 letech nová mezinárodní konsenzuální doporučení zahrnující epidemiologii, patofyziologii, manifestaci i terapii těchto akutních stavů. Předkládaný článek stručně prezentuje nejdůležitější informace z těchto doporučení.
Diabetic ketoacidosis (DKA) and hyperglycemic hyperosmolar state (HHS) are the most severe hyperglycemic situations in patients with diabetes. In 2024, a new consensual recommendation on epidemiology, pathophysiology, manifestation, and therapy of these acute situations was published after 15 years. The most important information from this document is presented in the manuscript.
- MeSH
- Diabetes Mellitus MeSH
- Diabetic Ketoacidosis diagnosis etiology prevention & control therapy MeSH
- Potassium therapeutic use MeSH
- Hyperglycemic Hyperosmolar Nonketotic Coma diagnosis etiology therapy MeSH
- Insulin administration & dosage therapeutic use MeSH
- Diabetes Complications * prevention & control therapy MeSH
- Humans MeSH
- Fluid Therapy MeSH
- Check Tag
- Humans MeSH
PURPOSE: To investigate the association of diabetes mellitus and metformin use with metabolic acidosis risk after radical cystectomy (RC) and urinary diversion for bladder cancer. MATERIALS AND METHODS: This retrospective cohort study used TriNetX Research Network data. Patients undergoing RC with continent diversion or ileal conduit for bladder cancer were identified using International Classification of Diseases, 10th Revision (ICD-10) and ICD-10 Procedure Coding System (ICD-10-PCS) codes. The primary outcome was acidosis between 1 month and 3 years postsurgery. Risk ratios (RR) and odds ratios (OR) were calculated based on diabetes and metformin use, stratified by diversion type and chronic kidney disease stage. Propensity score matching balanced potential confounders. RESULTS: We identified 1,986 patients who underwent continent diversion and 11,184 who underwent ileal conduit reconstruction. In matched analyses, diabetes patients had higher acidosis risk (continent diversion: RR 1.87, 95% confidence interval [CI] 1.39-2.51; ileal conduit: RR 1.94, 95% CI 1.66-2.27). The risk was highest for diabetes patients with metformin prescription (continent diversion: RR 2.06, 95% CI 1.63-2.61; ileal conduit: RR 2.13, 95% CI 1.84-2.47). However, among patients with diabetes, metformin use did not significantly affect acidosis rates in most analyses. Continent diversion patients had higher acidosis risk than ileal conduit patients (RR 1.89, 95% CI 1.58-2.26). CONCLUSION: Diabetes significantly increases metabolic acidosis risk after RC with urinary diversion, especially in continent diversion patients. While metformin may contribute to metabolic acidosis risk, its impact appears less significant than that of diabetes. Careful monitoring and appropriate metformin adjustments are crucial in this population.
- MeSH
- Acidosis * etiology epidemiology chemically induced MeSH
- Cystectomy * adverse effects methods MeSH
- Urinary Diversion * adverse effects methods MeSH
- Hypoglycemic Agents * adverse effects therapeutic use MeSH
- Middle Aged MeSH
- Humans MeSH
- Metformin * adverse effects therapeutic use MeSH
- Urinary Bladder Neoplasms * surgery MeSH
- Postoperative Complications * etiology MeSH
- Retrospective Studies MeSH
- Aged MeSH
- Check Tag
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
- Comparative Study MeSH
Chronic diarrhea is a significant challenge in clinical practice because of its high prevalence and various causes. Comprehensive clinical assessment and stepwise laboratory approach are crucial for an accurate diagnosis. This report presents a case of an adult woman who experienced chronic watery diarrhea, complicated by renal impairment and multiple electrolyte imbalances, including hypokalemia, hypophosphatemia, and metabolic acidosis. The diagnosis of a vasoactive intestinal polypeptide-secreting tumor (VIPoma) with liver metastases was confirmed by elevated serum levels of a vasoactive intestinal polypeptide (VIP) and imaging findings of a pancreatic mass with multiple hepatic lesions. Preoperative management, including fluid rehydration, electrolyte correction, and somatostatin analog therapy, significantly improved her clinical symptoms. Subsequent surgical tumor removal and radiofrequency ablation of the hepatic lesions resulted in complete resolution of symptoms and normalized VIP levels. This case emphasizes the importance of early recognition of this rare tumor in patients with chronic diarrhea to improve clinical outcomes.
- MeSH
- Chronic Disease MeSH
- Vipoma * complications diagnosis MeSH
- Middle Aged MeSH
- Humans MeSH
- Liver Neoplasms secondary complications MeSH
- Pancreatic Neoplasms * complications MeSH
- Diarrhea * etiology MeSH
- Vasoactive Intestinal Peptide blood MeSH
- Check Tag
- Middle Aged MeSH
- Humans MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Case Reports MeSH
AIMS: Refractory out-of-hospital cardiac arrest (r-OHCA) in patients with pulmonary embolism (PE) is associated with poor outcomes. The role of extracorporeal cardiopulmonary resuscitation (ECPR) in this patient group is uncertain. This study aims to analyse clinical course, outcomes, and the effect of an invasive procedure, including ECPR, in a randomized population. METHODS AND RESULTS: A post hoc analysis of a randomized controlled trial (Prague OHCA study) was conducted to evaluate the effect of ECPR vs. a standard approach in r-OHCA. A subgroup of patients with PE-related r-OHCA was identified, and procedural and outcome characteristics, including favourable neurological survival, organ donation, and complications, were compared to patients without PE. Pulmonary embolism was identified as a cause of r-OHCA in 24 of 256 (9.4%) enrolled patients. Patients with PE were more likely to be women [12/24 (50%) vs. 32/232 (13.8%); P < 0.001] and presented more frequently with an initial non-shockable rhythm [23/24 (95.8%) vs. 77/232 (33.2%); P < 0.001], as well as more severe acidosis at admission [median pH (interquartile range); 6.83 (6.75-6.88) vs. 6.98 (6.82-7.14); P < 0.001]. Their favourable 180-day neurological survival was significantly lower [2/24 (8.3%) vs. 66/232 (28.4%); P = 0.049], but the proportion of accepted organ donors was higher (16.7 vs. 4.7%, P = 0.04). CONCLUSION: Refractory out-of-hospital cardiac arrest due to PE has a different presentation and inferior outcomes compared to other causes but may represent an important source of organ donations. The ECPR method did not improve patient outcomes.
- MeSH
- Cardiopulmonary Resuscitation * methods MeSH
- Humans MeSH
- Extracorporeal Membrane Oxygenation * methods MeSH
- Pulmonary Embolism * etiology complications MeSH
- Retrospective Studies MeSH
- Out-of-Hospital Cardiac Arrest * etiology therapy MeSH
- Check Tag
- Humans MeSH
- Male MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Randomized Controlled Trial MeSH
Diabetická ketoacidóza je život ohrožující stav, související s onemocněním diabetes mellitus a zároveň hlavní příčinou mortality dětí s diabetem. Je asociována ve většině případů s diabetem 1. typu, vzácně se může objevit u pacientů s diabetem 2. typu. Vedoucími příznaky jsou dehydratace, metabolická acidóza, hyperglykemie a ketonurie, v těžších případech Kussmaulovo dýchání, peritonismus, zvracení a těžká porucha vědomí. Nejčastější příčinou smrti je edém mozku. Laboratorně je jako těžká diabetická ketoacidóza hodnocen stav s pH pod 7,1, a koncentrací bikarbonátů pod 5 mmol/l. V dětském věku se poměrně často diabetes poprvé manifestuje právě diabetickou ketoacidózou, bez předchozí pozitivní osobní anamnézy. Neléčená diabetická ketoacidóza je smrtelná. Velmi nebezpečná může být záměna za jinou diagnózu nebo neadekvátní terapie.
Diabetic ketoacidosis is a life threatening event, associated with diabetes mellitus. Diabetic ketoacidosis is the main reason of mortality in pediatric diabetic population. It is usually associated with the first type of diabetes mellitus, however, it can rarely appear in patient with diabetes type two, too. Dehydration, metabolic acidosis with Kussmaul breathing, hyperglykaemia and ketonuria are the leading symptoms. In severe cases peritonism, vomiting and severe consciousness disorder or even coma can occur. The most frequent reason of death is brain edema. The value of pH lower than 7,1 and sodium bicarbonate concentration lower than 5 mmol/l are considered severe ketoacidosis. Diabetic ketoacidosis is a frequent primary presentation of diabetes mellitus in childhood with negative case history. Unrecognized and untreated ketoacidosis may lead to death. Missdiagnosis of diabetic ketoacidosis or inadequate therapy may also lead to severe complications.
Based on many reports, an unmistakable link probably exists between diabetes mellitus and COVID-19. A major predisposing factor determining severity and mortality of COVID-19 is diabetes mellitus, diabetic patients were shown to be at higher risk for developing severe COVID-19 disease than non-diabetics; many recent studies reported a striking prevalence of DM in those diagnosed with COVID-19. Accordingly, antidiabetic drugs can possibly impact the clinical course and / or the outcome of this infection, either by alleviating diabetes-associated symptoms, minimizing its complications, or by mitigating or aggravating COVID-19 disease by effects independent from their direct antidiabetic effects. Several antidiabetic drug classes were shown to have varying effects, like blocking viral entry to cells, as well as having immunomodulatory, anti-inflammatory, antifibrotic, or cardioprotective effects; such effects could prove beneficial for COVID-19 patients. On the other hand, some antidiabetic agents may have adverse effects that aggravate patients' condition like hypoglycemia, fluid retention, increased weight or lactic acidosis, which require special consideration in patient management. Some of the drugs were found in observational studies to either reduce mortality from COVID-19 or pose no harm, but more solid evidence from clinical trials is still lacking.
- MeSH
- COVID-19 * etiology complications MeSH
- Hypoglycemic Agents adverse effects MeSH
- Diabetes Complications * MeSH
- Humans MeSH
- Metformin adverse effects MeSH
- Drug-Related Side Effects and Adverse Reactions MeSH
- Risk Factors MeSH
- Sulfonylurea Compounds adverse effects MeSH
- Thiazolidinediones adverse effects MeSH
- Check Tag
- Humans MeSH
- Publication type
- Review MeSH
D-laktátová acidóza reprezentuje zriedkavú formu metabolickej acidózy, ktorá sa vyskytuje najčastejšie u pacientov so syndrómom krátkeho čreva. Ide o závažnú, niekedy až život ohrozujúcu komplikáciu. Príčinou je akumulácia D-laktátu v organizme, ktorý vzniká v nadmernom množstve fermentáciou nevstrebaných sacharidov mikrobiotou hrubého čreva. Predominantne býva postihnutý nervový systém, z čoho vyplýva aj klinická manifestácia. V klinickom obraze dominuje široká škála nešpecifických neurologických príznakov. Ochorenie sa môže niekedy manifestovať somnolenciou až kómou. Z aspektu laboratórnej diagnostiky ochorenie charakterizuje ťažká metabolická acidóza so zvýšenou aniónovou medzerou. V tejto kazuistike prezentujeme ojedinelý prípad 54-ročnej ženy s Crohnovou chorobou a syndrómom krátkeho čreva, ktorá bola v krátkom čase opakovane hospitalizovaná pre recidívu ťažkej metabolickej acidózy so závažnou poruchou vedomia. Na základe zhodnotenia anamnestických údajov, klinického obrazu a laboratórnych vyšetrení bola pacientke diagnostikovaná D-laktátová acidóza. V diskusii rozoberáme jednotlivé kroky, ktoré viedli k tejto diagnóze a porovnávame našu skúsenosť s údajmi vo svetovej literatúre.
D-lactic acidosis represents a rare form of metabolic acidosis that occurs most commonly in patients with short bowel syndrome. This is a serious, sometimes life-threatening complication. The cause is the accumulation of D-lactate in the body, which is formed in excessive amounts by fermentation of unabsorbed carbohydrates by the intestinal microbiota. The nervous system is predominantly affected, which also results in clinical manifestations. The clinical picture is dominated by a wide range of non-specific neurological symptoms. The disease can sometimes manifest as somnolence to coma. From the aspect of laboratory diagnostics, the disease is characterized by severe metabolic acidosis with an increased anion gap. In this case report, we present a unique case of a 54-year-old woman with Crohn's disease and short bowel syndrome who in a short time was repeatedly hospitalized for recurrence of severe metabolic acidosis with severe impaired consciousness. Based on the evaluation of anamnestic data, clinical picture and laboratory tests, the patient was diagnosed with D-lactic acidosis. In the discussion we discuss the individual steps that led to this diagnosis and compare our experience with data in the world literature.
- MeSH
- Acidosis, Lactic * diagnosis etiology therapy MeSH
- Crohn Disease complications MeSH
- Lactic Acid metabolism MeSH
- Middle Aged MeSH
- Humans MeSH
- Brain Diseases, Metabolic complications MeSH
- Critical Care MeSH
- Consciousness Disorders MeSH
- Short Bowel Syndrome * complications MeSH
- Treatment Outcome MeSH
- Check Tag
- Middle Aged MeSH
- Humans MeSH
- Female MeSH
- Publication type
- Case Reports MeSH
Metformin je perorálním antidiabetikem první volby s příznivým vlivem na průběh diabetes mellitus 2. typu a s pozitivním ovlivněním zvýšeného kardiovaskulárního rizika diabetiků. Metformin je s ohledem na tíži renálního poškození předepisován v redukované dávce či zcela kontraindikován z důvodu možné akumulace metforminu a rizika rozvoje laktátové acidózy. Tato komplikace zůstává dodnes rizikem zejména pro geriatrické pacienty s omezenými tělesnými rezervami a s četnými chronickými onemocněními, jejichž deterioraci může přivodit jinak banální inzult. Na tento fakt by měl pomýšlet každý ošetřující lékař a umět správně rozhodnout o vhodnosti léčby metforminem. V následujícím článku prezentujeme čtyři pacienty metabolické jednotky intenzivní péče naší kliniky, kteří laktátovou acidózu v souvislosti s užíváním metforminu prodělali.
Metformin is an oral antidiabetic drug of first choice with a positive effect on type 2 diabetes mellitus control as well as protective action on cardiovascular system. With respect to renal impairment, metformin should be prescribed in reduced doses or is even contraindicated due to high risk of metformin accumulation and/or lactic acidosis development. Till this day, this complication remains a risk especially in geriatric patients with reduced reserve capacity, who often suffer from numerous chronic diseases which can deteriorate due to a minor event. This fact should be remembered by every doctor, who should also be able to decide whether to terminate metformin therapy or not. In this article we present case studies of four patients of metabolic ICU treated with metformin-associated lactic acidosis.
- Keywords
- laktátová acidóza,
- MeSH
- Acidosis * etiology therapy MeSH
- Diabetes Mellitus drug therapy MeSH
- Middle Aged MeSH
- Humans MeSH
- Metformin * adverse effects MeSH
- Drug-Related Side Effects and Adverse Reactions MeSH
- Renal Insufficiency etiology therapy MeSH
- Aged, 80 and over MeSH
- Aged MeSH
- Treatment Outcome MeSH
- Check Tag
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged, 80 and over MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Case Reports MeSH
