Do Immaturity, Dehydratation or Alkalosis Predispose to Intraoperative Complications at Pyloromyotomy?

Background: Hypertrophic pyloric stenosis is the most important cause for propulsive non-bilious vomiting in infants of the first trimester. Extramucous splitting of the hypertrophic pyloric muscle is the surgical gold standard for treatment. Serious major complications of pyloromyotomy (PM) are mucosal perforation and incomplete muscle splitting. The aim of the presented study is to find out if intraoperative complications are predisposed by biometric or biochemical factors. Furthermore, we looked for the influence of the board certification of the primary surgeon. Patients and Methods: 162 infants with IHPS were operated during a six-year period (n = 150 laparoscopic operations). We had 8 major complications (4.9%): iatrogenic mucosal perforation occurred in 6 cases, and incomplete PM in 2 infants. Preoperative demographic data and data resulting from the blood acid-base- and ion-analysis were compared with data of a previously published reference group from our institution (Tr?bs RB. Open J Pediatr, 2014;4: 208-215). Results: The duration of vomiting, the grade of dehydration and the severity of blood alkalosis did not differ between both groups. Furthermore, we found no influence of the gestational age and birth weight on the occurrence of intraoperative complications. It seems that early postnatal age (p = 0.07) and low body weight at surgery (p = 0.055) may contribute to surgical problems. Board certification as a paediatric surgeon did not influence the rate of intraoperative complications. Conclusions: Laboratory data did not show any predisposition to intraoperative complications. It is assumed that small infants with early occurring symptoms carry an elevated risk for intraoperative events. Our data support the hypothesis that the rate of intraoperative complications at PM is mainly influenced by skills and experience of the surgeon.

Bartter and Gitelman syndromes:Spectrum of clinical manifestations caused by different mutations

Bartter and Gitelman syndromes(BS and GS) are inherited disorders resulting in defects in renal tubularhandling of sodium,potassium and chloride.Previously considered as genotypic and phenotypic heterogeneous diseases,recent evidence suggests that they constitute a spectrum of disease caused by different genetic mutations with the molecular defects of chloride reabsorption originating at different sites of the nephron in each condition.Although they share some characteristic metabolic abnormalities such as hypokalemia,metabolic alkalosis,hyperplasia of the juxtaglomerular apparatus with hyperreninemia,hyperaldosteronism,the clinical and laboratory manifestations may not always allow distinction between them.Diuretics tests,measuring the changes in urinary fractional excretion of chloride from baseline after administration of either hydrochlorothiazide or furosemide show very little change(< 2.3%) in the fractional excretion of chloride from baseline in GS when compared with BS,except when BS is associated with KCNJ1 mutations where a good response to both diuretics exists.The diuretic test is not recommended for infants or young children with suspected BS because of a higher risk of volume depletion in such children.Clinical symptoms and biochemical markers of GS and classic form of BS(type III) may overlap and thus genetic analysis may specify the real cause of symptoms.However,although genetic analysis is available,its use remains limited because of limited availability,large gene dimensions,lack of hot-spot mutations,heavy workup time and costs involved.Furthermore,considerable overlap exists between the different genotypes and phenotypes.Although BS and GS usually have distinct presentations and are associated with specific gene mutations,there remains considerable overlap between their phenotypes and genotypes.Thus,they are better described as a spectrum of clinical manifestations caused by different gene mutations.

Pathophysiology of Hypertrophic Pyloric Stenosis Revisited: The Use of Isotonic Fluid for Preoperative Infusion Therapy Is Supported

Background: The aim of this study was to elucidate the preoperative clinical and biochemical profile of infants with IHPS to optimize infusion therapy. Patients and Method: We retrospectively analyzed data from 56 infants who were operated for IHPS. Our study includes growth and laboratory data prior to the initiation of therapy. Results: Median duration of propulsive vomiting was 4 d;the median age was 37 d (18 - 108), and the median body weight was 3840 g (2760 -5900). Metabolic alkalosis (MAlk) with a pH of 7.45 ± 0.06 and an stHCO3- of 28.7 ± 4.5 mmol/l was found. In a subgroup of the infants, negative base excess (BE) was observed. The sodium concentration was normal or reduced (mean/median of 137 mmol/l). There was a strong negative correlation between stHCO3- and K+. The carbon dioxide partial pressure tended to increase (5.72 ± 0.84 kPa). Calculations of osmolality revealed a normal osmolarity. Hypoglycemia did not occur. The creatinine clearance according to the Schwartz formula remained at a normal level (85.3 ± 24.3 ml/min/1.73 m2). Discussion: The presented case series is characterized by a short duration of preoperative vomiting. MAlk can be classified as a chloride deficiency syndrome. It is accompanied by normo- or hyponatremic dehydration with normal osmolality. Partial respiratory compensation occurred. A normal creatinine clearance indicated good glomerular renal function. Conclusion: The presented study supports the use of an isotonic infusion fluid with a low glucose concentration for preoperative infusion therapy.

Dehydrated patient without clinically evident cause:A case report

BACKGROUND Patients affected by cystic fibrosis can present with metabolic alkalosis such as Bartter's syndrome.In this case report we want to underline this differential diagnosis and we aimed focusing on the suspect of cystic fibrosis,also in case of a negative newborn screening.CASE SUMMARY In a hot August–with a mean environmental temperature of 36℃–an 8-mo-old female patient presented with severe dehydration complicated by hypokalemic metabolic alkalosis,in absence of fever,diarrhea and vomiting.Differential diagnosis between cystic fibrosis and tubulopathies causing metabolic alkalosis(Bartter's Syndrome)was considered.We started intravenous rehydration with subsequent improvement of clinical conditions and serum electrolytes normalization.We diagnosed a mild form of cystic fibrosis(heterozygous mutations:G126 D and F508 del in the cystic fibrosis transmembrane conductance regulator gene).The trigger factor of this condition had been heat exposure.CONCLUSION When facing a patient with hypokalemic metabolic alkalosis,cystic fibrosis presenting with Pseudo-Bartter's syndrome should be considered in the differential diagnosis,even if the newborn screening was negative.

Effect of Low-Dose Sodium Bicarbonate Supplementation on Intermittent Endurance Performance

In this study, we investigated the effect of sodium bicarbonate (NaHCO3) supplementation at a dose of 0.2 g&middot;kg&minus;1 40 min before an exercise on intermittent endurance performance and gastrointestinal distress. The participants were six healthy male students of a university. The Yo-Yo intermittent recovery test level 2 (Yo-Yo IR2) was used for the evaluation of intermittent endurance. The participants ingested water (Cont) or NaHCO3 independently at three timings. After drinking water, the Yo-Yo IR1 was performed for 2 min as warm-up. After resting for 5 min, the Yo-Yo IR2 was initiated. The measurement items were exercise distance, nutrition intake, biochemical test results, and blood gas analysis findings. The participants who ingested NaHCO3 had a 14% extended distance than those who ingested Cont, and 2/3 of those who took NaHCO3 showed a better motor performance. No significant difference was observed between the participants who took Cont and NaHCO3 in terms of pH level at baseline, and the NaHCO3 level was significantly higher during pre- and post-exercise (p 3 in terms of bicarbonate ion level at baseline, and the NaHCO3 level was significantly higher during pre- and post-exercise (p 3 at a low dose (0.2 g&middot;kg&minus;1), their exercise ability during an intermittent endurance test has improved. Furthermore, when digestive absorption is considered, eating a meal in advance may be able to suppress the onset of gastrointestinal distress.

Biochemical Syndrome in Adult Pyloric Stenosis, a Clue to Diagnosis in Emergency Department

Characteristic biochemical changes have been documented in patients presenting with the symptoms and signs of gastric outlet obstruction. These changes usually consist of hypochloremic, hypokalemic metabolic alkalosis and hypocalcaemia with or without renal failure. The aim of this report is to highlight these biochemical abnormalities again. Moreover, we suggest conversely that the presence of these changes should prompt the clinician for further evaluation to rule out gastric outlet obstruction especially in the emergency department. Early intervention resulted in dramatic improvement in patient’s clinical and biochemical status.

Clinical research on curative effect of treatment on patients with gouty renal stone by hydration-alkalization therapy

Objective:Study the clinical effect of intravenous hydration and alkalization therapy on uric acid nephrolithiasis.Methods: Sixty-five patients with 86 stones admitted to Department of Endocrinology and Urinary Nephropathy from December 2015 to December 2017 in our hospital were divided into control group (n= 31) and Treatment group (n = 34) with at random. Both groups were given basic treatment with low purine diet and the control group was treated with oral sodium bicarbonate tablets + oral rehydration 1000 mL / d. The treatment group was treated with hydration alkalosis treatment by 0.9% saline 500 mL + 5% sodium bicarbonate injection of 60 mL + 10% potassium chloride injection 10 mL intravenous infusion, 2 times/d. Two groups were treated for a course of treatment (10 d), respectively, detecting relevant laboratory parameters and imaging studies before and after treatment for assessing the effectiveness of treatment.Result: The total effective rate of the treatment group was 86.67%, which was significantly better than the control group (4.88%).Conclusion: It is a significant effect that Intravenous hydration alkalosis therapy for the treatment of uric acid nephrolithiasis. The diameter of less than 0.5 cm stones disappears, and the diameter of 0.5-0.8 cm stone was significantly reduced. As it can improve stone related renal insufficiency with low cost, it is widely recommended in clinical.

Bartter's syndrome:clinical findings,genetic causes and therapeutic approach

Backgound Bartter's syndrome(BS)is a rare group of salt losing tubulopathies due to the impairment of transport mecha-nisms at the thick ascending limb of the Henle's loop.Data sources Literature reviews and original research articles were collected from database,including PubMed and Scopus.Results According to the time of onset and symptoms,BS can be classified into antenatal and classic BS.Molecular studies have identified different subtypes of BS.BS types Ⅰ,Ⅱ and Ⅲ are caused by mutations on genes encoding the luminal Na^(+)-K^(+)-2Cl^(-) co-transporter,the luminal K+ channel ROMK,and the basolateral chloride channel ClC-Kb(CLCNKB),respectively.Loss-of-function mutations of Barttin CLCNK type accessory beta subunit cause BS type Ⅳa.Simultaneous mutations of CLCNKB and CLCNKA cause BS type Ⅳb.BS type Ⅴ consists in a novel transient form characterized by antenatal presentation due to mutations in the MAGE family member D2.Severe gain-of-function mutations of the extracellular calcium sensing receptor gene can result in an autosomal dominant condition of BS.Main clinical and biochemical alterations in BS include polyuria,dehydration,hypokalemia,hypochloremic metabolic alka-losis,hyperreninemia,high levels of prostaglandins,normal or low blood pressure,hypercalciuria and failure to thrive.Treatment focuses mainly at correcting dehydration and electrolyte disturbances and in measures to reduce polyuria,including the use of nonsteroidal anti-inflammatory medications to control excessive renal prostaglandin E2 production.Conclusions Early diagnosis and treatment of BS may prevent long-term consequences such as growth failure,nephrocal-cinosis and end-stage renal disease.