Mild Metabolic Acidosis Research Articles

Case report: Encephalitis with Guillain-Barré Syndrome secondary to extensively multi-drug resistant Salmonella infection

Abstract Background This case report presents a highly unusual and challenging medical case involving a 16-month-old previously healthy boy who developed encephalitis followed by Guillain-Barré Syndrome (GBS) as complications of multi-drug resistant Salmonella infection, despite an initial misdiagnosis of gastroenteritis and outpatient treatment with Cefixime. Methods The patient initially experienced an increase in stool frequency, accompanied by low-grade fevers, which was initially attributed to gastroenteritis. Outpatient treatment with oral Cefixime was started, but the child’s symptoms worsened, ultimately leading to high-grade fevers and an increase in vomiting and loose stools. In light of his clinical deterioration, he was admitted to our hospital with signs of mild metabolic acidosis and elevated inflammatory markers. During his hospitalization, the patient’s condition worsened, with drowsiness, irritability, and a generalized tonic-clonic seizure, prompting concern for brain involvement. Consequently, he underwent lumbar puncture, and neuroimaging, and received high-dose intravenous steroids. Further medical investigations including lumbar puncture and brain Magnetic Resonance Imaging (MRI), which revealed normal cerebrospinal fluid (CSF) analysis and non-enhancing white matter changes in the occipital region, respectively. In addition, a collected blood culture confirmed the presence of extensively multi-drug resistant Salmonella typhi and prompted an adjustment of antibiotics to intravenous Meropenem. Due to autonomic dysfunction, the patient was transferred to the Pediatric Intensive Care Unit (PICU) for close monitoring and specialized care. While in the PICU, he developed flaccid limb weakness and axial hypotonia. A repeat lumbar puncture was normal, but nerve conduction studies showed features of axonal polyneuropathy diagnosis, consistent with the diagnosis of GBS. To treat the encephalopathy and polyneuropathy, high-dose dexamethasone and Intravenous Immunoglobulin (IVIG) treatments were initiated, without necessitating respiratory or inotropic support. After receiving these treatments, the patient’s autonomic instability and motor strength improved, prompting his transfer back to the general pediatrics wards. He was treated with a 21-day course of IV Meropenem. Upon discharge, his motor power had returned to normal, and he displayed progress in movement transitions, including the ability to stand and walk independently, albeit with mild gait abnormalities. Results The co-occurrence of encephalitis and Guillain-Barré Syndrome due to Typhoid fever is exceedingly rare. The patient’s origin, with recent travel to Pakistan where extensively drug-resistant Salmonella typhi prevails, necessitates heightened awareness among healthcare providers. Initial misdiagnosis and inadequate antibiotics treatment may have contributed to encephalitis and polyneuropathy. High-dose dexamethasone and IVIG were pivotal in the patient’s treatment. While Typhoid fever-related neurological complications are infrequent, high-dose dexamethasone offers promise in reducing mortality and mitigating typhoid-induced encephalopathy. Importantly, IVIG is not conventionally employed in Salmonella encephalopalitis but was used due to polyneuropathy and its role in GBS management. Conclusion This exceptional pediatric case serves as a striking example of the possibility simultaneous encephalitis and Guillain-Barré Syndrome as rare complications of Typhoid fever. This case report underscores the importance of early diagnosis, prompt management with appropriate antibiotics and caution of possible neurological complications. Furthermore, it calls for further research into the role of high-dose glucocorticoids and IVIG in the management of extensively multi-drug resistant Salmonella infection and its neurological complications.

Effect of Sodium Bicarbonate Therapy on Calciprotein Particles in Patients with CKD Stages 3 and 4 and Mild Metabolic Acidosis

Effect of Sodium Bicarbonate Therapy on Calciprotein Particles in Patients with CKD Stages 3 and 4 and Mild Metabolic Acidosis

Exogenous ketosis attenuates acute mountain sickness and mitigates normobaric high-altitude hypoxemia.

Acute mountain sickness (AMS) represents a considerable issue for individuals sojourning to high altitudes with systemic hypoxemia known to be intimately involved in its development. Based on recent evidence that ketone ester (KE) intake attenuates hypoxemia, we investigated whether exogenous ketosis might mitigate AMS development and to identify underlying physiological mechanisms. Fourteen healthy, male participants were enrolled in two 29h protocols (simulated altitude of 4,000-4,500m) receiving either KE or a placebo (CON) at regular timepoints throughout the protocol in a randomized, crossover manner. Physiological responses were characterized after 15min and 4h in hypoxia, and the protocol was terminated prematurely upon development of severe AMS (Lake Louise Score ≥ 10). KE ingestion induced a consistent diurnal ketosis ([ßHB] of ~3 mM), whereas blood [ßHB] remained low (<0.6 mM) in CON. Each participant tolerated the protocol equally long or longer (n=6 or n=8, resp.) in KE. Protocol duration increased by 32% on average with KE, and doubled upon KE for severe AMS-developing participants (n=9). Relative to CON, KE induced a mild metabolic acidosis, hyperventilation, and relative sympathetic dominance. KE also inhibited the progressive hypoxemia that was observed between 15min and 4h in hypoxia in CON, while concomitantly increasing cerebral oxygenation and capillary pO2 within this timeframe despite a KE-induced reduction in cerebral oxygen supply. These data indicate that exogenous ketosis attenuates AMS development. The key underlying mechanisms include improved arterial and cerebral oxygenation, in combination with lowered cerebral blood flow and oxygen delivery, and increased sympathetic dominance.

Adverse effects of human milk fortifier on preterm neonates in a neonatal intensive care unit of a tertiary care hospital, Karachi.

Objective: To determine adverse effects of Human Milk Fortifier on Preterm neonates in a Neonatal Intensive Care Unit. Study Design: Descriptive study. Setting: Indus Hospital NICU in Karachi. Period: Mar 2022 to September 2023. Methods: Aimed to investigate the adverse effects of HMF on 139 preterm neonates. The study excluded infants with congenital anomalies. Adverse effects, including Necrotizing Enterocolitis (NEC), metabolic acidosis, feeding intolerance, and other complications, were monitored. Results: The findings revealed that feeding intolerance was the most common adverse effect of HMF. Mild metabolic acidosis and NEC were also observed. These results are consistent with previous studies and may be attributed to the high osmolality of feeds following fortification with HMF. Despite the well-established role of HMF in promoting weight gain in preterm infants, the study underscores the importance of closely monitoring its use. Conclusion: Careful consideration of the potential risks and benefits of HMF is essential before prescribing it to preterm neonates. Future research with larger sample sizes and controlled designs is needed to validate these findings and explore strategies to mitigate the adverse effects of HMF in NICU settings.

Not all ketosis is type 1 – remember Flatbush

A 35-year-old otherwise healthy gentleman from Togo, was referred as a ‘walk-in’ to our clinic with polyuria and polydipsia, and a glycated haemoglobin (Hba1c) of 119 mmol/mol (13.1%). The patient also noted 5kg weight loss over a short span of time. He had a significant family history of Type 2 Diabetes Mellitus (T2DM). Initial blood tests revealed a blood glucose of 22.84 mmol/L, with positive ketones (1.2 mmol/L). Urinalysis showed glycosuria (1000 mg/dL) but was negative for nitrites and white cells. Renal, liver and thyroid function tests were all within normal limits. He had mild metabolic acidosis.

Negative dietary cation and anion difference supplementation of twin-bearing Merino ewes grazing pasture in late gestation did not affect lamb growth or survival.

Each year in Australia, 53% of lamb mortalities are attributed to dystocia, with subclinical maternal calcium deficiencies likely contributing to dystocia rates. A negative dietary cation and anion difference (DCAD) diet has increased circulating calcium in sheep. Therefore, this study aimed to investigate the effects of supplementing twin-bearing, grazing ewes with a negative DCAD partial mixed ration (PMR) during late gestation on ewe calcium and magnesium concentrations and subsequent lamb growth and survival. On day 120 of gestation (dG), blood samples were collected from 115 twin-bearing Merino ewes and analyzed for glucose, ketone bodies, pH, ionized calcium, and serum calcium and magnesium. On dG 130, ewes were moved into lambing paddocks and placed in the following 2 treatment groups; ewes receiving a positive DCAD PMR (DCAD = 287 mEq/kg DM; n = 58) and ewes receiving a negative DCAD PMR (DCAD = -125 mEq/kg DM; n = 57) fed as a PMR. On dG 140, a blood and urine sample were collected. The urine was tested for pH. Pasture samples were taken on dG 133 and 149 and tested for DCAD and mineral content. When a lamb was 6 to 18h old, survival, vigor score, liveweight (LW), rectal temperature, blood glucose, and body morphology were recorded. At 10 d of age, lamb LW and survival were recorded and a milk sample was collected from ewes. At 44 d of age, lamb LW and survival were recorded. The DCAD of the pastures across the 6 paddocks ranged from 598 to 893 mEq/kg DM. There were no differences in lamb survival, weight, or viability at any timepoint (P > 0.05). There were no differences in mineral status, metabolic state, or acid-base balance between the positive and negative DCAD-supplemented ewes (P > 0.05) during supplementation (dG 140). Supplementing a negative DCAD diet to ewes grazing pasture during late gestation did not improve lamb survival. The blood and urine pH of the negative DCAD-supplemented ewes indicated a mild metabolic acidosis was not reached due to the high DCAD of the pastures. Further research needs to take careful consideration of the DCAD of pasture when designing a negative DCAD supplement in order for it to be effective.

DEXMEDETOMIDINE AND MIDAZOLAM INTRAMUSCULAR SEDATION IN BROWNBANDED BAMBOO SHARKS (CHILOSCYLLIUM PUNCTATUM).

Bamboo sharks are some of the most common elasmobranch species in zoos and aquaria and are frequently sedated for medical exams, treatments, and research. This study investigated the use of an IM sedation protocol of a single dose of dexmedetomidine (0.05 mg/kg) and midazolam (2.0 mg/kg) in brownbanded bamboo sharks (Chiloscyllium punctatum). Sharks were serially monitored every 5 min for heart rate, branchial beats, righting reflex, coelomic response, cloacal response, pelvic fin reflex, response to noxious stimulus, voluntary movement, and ability to swim. This sedation dose was effective at rapidly and significantly decreasing responses to tactile and noxious stimuli with minimal respiratory depression and was quickly reversible with atipamezole (0.5 mg/kg) and flumazenil (0.05 mg/kg). Sedated sharks developed a mild metabolic acidosis evidenced by a significant increase in lactic acid (mean < 0.37 mmol/L presedation, 4.2 mmol/L after reversal) and decrease in blood pH (mean 7.464 presedation, 7.277 after reversal); however, clinical intervention was not required. This protocol should be further investigated in different elasmobranch species but is promising for providing sedation for noninvasive procedures in brownbanded bamboo sharks.

A Spanish Family with Gordon Syndrome Due to a Variant in the Acidic Motif of WNK1.

(1) Background: Gordon syndrome (GS) or familial hyperkalemic hypertension is caused by pathogenic variants in the genes WNK1, WNK4, KLHL3, and CUL3. Patients presented with hypertension, hyperkalemia despite average glomerular filtration rate, hyperchloremic metabolic acidosis, and suppressed plasma renin (PR) activity with normal plasma aldosterone (PA) and sometimes failure to thrive. GS is a heterogeneous genetic syndrome, ranging from severe cases in childhood to mild and sometimes asymptomatic cases in mid-adulthood. (2) Methods: We report here a sizeable Spanish family of six patients (four adults and two children) with GS. (3) Results: They carry a novel heterozygous missense variant in exon 7 of WNK1 (p.Glu630Gly). The clinical presentation in the four adults consisted of hypertension (superimposed pre-eclampsia in two cases), hyperkalemia, short stature with low body weight, and isolated hyperkalemia in both children. All patients also presented mild hyperchloremic metabolic acidosis and low PR activity with normal PA levels. Abnormal laboratory findings and hypertension were normalized by dietary salt restriction and low doses of thiazide or indapamide retard. (4) Conclusions: This is the first Spanish family with GS with a novel heterozygous missense variant in WNK1 (p.Glu630Gly) in the region containing the highly conserved acidic motif, which is showing a relatively mild phenotype, and adults diagnosed in mild adulthood. These data support the importance of missense variants in the WNK1 acidic domain in electrolyte balance/metabolism. In addition, findings in this family also suggest that indapamide retard or thiazide may be an adequate long-standing treatment for GS.

#6212 SEVERE HYPERCHLOREMIC METABOLIC ACIDOSIS WITH SGLT-2 INHIBITORS IN PATIENTS WITH URINARY DIVERSION

Abstract Background and Aims Urinary diversion after cystectomy using autologous intestinal segments has been the gold standard treatment of benign and malignant diseases of urinary tract. The most frequent metabolic abnormalities is hyperchloremic metabolic acidosis, due to ammonium absorption alongside chloride gain and bicarbonate excretion in the bowel conduit. Sodium-glucose co-transporter-2 (SGLT2) inhibitors are a class of antihyperglycemic agents that recently revolutionized the paradigm of chronic kidney disease. It exerts its effect by preventing the reabsorption of filtered glucose from the tubular lumen, acting on the SGLT2 proteins in the renal proximal convoluted tubules, and thus promoting a greater urinary glucose excretion. The authors described 2 patients with bowel conduit and mild hyperchloremic metabolic acidosis, presented with severe metabolic acidosis short after starting SGLT2 inhibitors. Method Consultation of medical records. Results Case 1: 62-year-old male with an orthotopic neobladder since 2005, type 2 diabetes and nonspecific interstitial pneumonia. In 2018 arterial blood gas (ABG) was: pH 7.45, pO2 81, pCO2 28, HCO3- 19.3, Cl 107, AG 7.7 (normal albumin), K+ 3.25. In February 2022 he was sent to the emergency department (ER) from a Pneumology appointment, after an ABG showed an aggravated hyperchloremic metabolic acidosis: pH 7.25, pCO2 27.5, HCO3- 12, Cl 122, Na+ 143, K+ 3.74, AG 9, lactate 0.69, glucose 261, ketone bodies negative); blood workup added acute kidney injury (AKI), urea 81, creatinine 1.6. The patient medical history revealed: administration of 5 cycles of cyclophosphamide (7 months before) and the introduction of empagliflozin 10mg id, 10 months prior to this event, as an addition to Metformin 1000mg id. A progressive metabolic normalization was observed after the SGLT2 inhibitor was withdrawn and intravenous hydration. Case 2: A 63-year-old male with an ileal conduit since 2018 and type 2 diabetes. He had chronic renal disease since 2021 with serum creatinine 1.5-2. He presented with a 3-day history of lethargy and anorexia. He had started dapagliflozina 10mg id 10 months prior to this event. At admission the patient had normal vital signs, glasgow coma scale 15 and no major signs at observation. ABG showed severe hyperchloremic metabolic acidemia (pH 6.98, pCO2 20.5, pO2 124.4, HCO3- 4.7, Na 129, Cl 120, AG 4.3, K+ 3.54, glucose 200, lactate 0.5) alongside AKI, urea 209 and creatinine 2.8 and spot urine without ketone bodies. He was admitted for a short period to the intensive care unit, for intravenous sodium bicarbonate and hydration, on top of SGLT2 inhibitor suspension, with progressive clinical and metabolic resolution. Conclusion Chronic hyperchloremic metabolic acidosis is a frequent and known complication of urinary diversion, due to the reabsorption of solutes by the intestinal mucosa because of the presence of urine (ammonium and chloride absorption and bicarbonate excretion) and diarrhea and volume depletion due to reduced sodium absorption in the gout. Euglycemic Ketoacidosis is a well-known complication of SGLT2 Inhibitor therapy and was not this case as both patients had normal anion gap. Hyperchloremic acidosis as been rarely described but not so severe as we presented. Although both patients had AKI, was not severe. We hypothesize that SGLT2 inhibitors, could exacerbate the chronic hyperchloremic metabolic acidosis by increasing urinary sodium and glucose excretion, that could exacerbate volume depletion, diarrhea and hyperglycemia (and subsequent volume depletion). This are the first 2 cases reported of severe hyperchloremic metabolic acidosis in patients with orthotopic neobladder and SGLT2 inhibitors treatment. The physiopathology of this presentation needs to be studied.

The association of dietary acid load (DAL) with estimated skeletal muscle mass and bone mineral content: a cross-sectional study

Background & AimsDietary patterns that promote mild metabolic acidosis may have a negative effect on bone and muscle, and a high dietary acid load (DAL) may be detrimental to skeletal muscle mass and bone mineral content. However, the association between skeletal muscle mass and bone mineral content with dietary acid load has not been consistently reported in previous studies. The objective of the study was to evaluate the association of potential renal net acid load (PRAL) and net endogenous acid production (NEAP) with bone mineral content and skeletal muscle mass in pre-menopause women with overweight or obesity in Iran.MethodThree hundred and ninety women with a body mass index (BMI) of 25 were included in this cross-sectional study. We used a validated 147-item semi-quantitative food frequency questionnaire (FFQ) for evaluating the dietary intake. Based on the dietary data, potential renal net acid load (PRAL) and net endogenous acid production (NEAP) were calculated. Muscle mass and bone mineral content were estimated by a bioelectrical impedance analyzer (BIA).ResultsAfter controlling for potential confounders, we discovered a significant linear relationship between PRAL (β = -0.027, 95%CI = -0.049 to -0.004, P = 0.02) and NEAP (β = -0.05, 95%CI = -0.097 to -0.003, P = 0.03) and skeletal muscle mass index. However, there was no significant difference between SMM and BMC across PRAL and NEAP tertiles.ConclusionPRAL and NEAP were found to be inversely related to skeletal muscle mass index among overweight/obese women. Further research is required to establish whether this relationship is important for musculoskeletal health in these populations.

Troublesome Teeth, Search for Stones – Enamel-Renal Syndrome

Background: Enamel-renal syndrome (ERS), is a rare autosomal recessive disorder involving family with sequence similarity 20 member A (FAM20A) gene. This unique syndrome is characterized by severe enamel hypoplasia, intrapulpal calcification, nephrocalcinosis, or nephrolithiasis. This case report highlights the early presentation and incidental detection of chronic kidney disease (CKD) in a young child with enamel defects. Clinical Description: A 10-year-old girl, with no significant past or family history, presented with delayed tooth eruption and abnormal shaped teeth. She was detected to have generalized gingival hyperplasia and multiple unerupted teeth. Suspecting amelogenesis imperfecta, she was screened for coexisting systemic conditions. On evaluation, abdominal ultrasound demonstrated bilateral nonobstructive nephrolithiasis. Further renal workup done showed an abnormal creatinine (stage 2 CKD) and mild metabolic acidosis. Reduced urinary citrate excretion with no evidence of hypercalciuria was noted on extensive urine assessment. Genetic testing revealed a novel pathogenic variant in FAM20A, confirming the diagnosis of ERS. Management: The child was initiated on citrate supplements, salt restriction, and adequate hydration. She was advised of dental interventions, including pulp therapy and full-coverage restoration of decayed teeth. The family was counseled about the need for close monitoring of growth, renal function, and progression of nephrolithiasis. Conclusion: Prompt screening for renal associations in oro-dental and systemic disease must be undertaken to ensure early detection of kidney disease and timely institution of appropriate treatment. In children diagnosed to have kidney involvement, the importance of regular follow-up with clinical, biochemical, and imaging modalities, even during adulthood, must be emphasized.

Effect of 5 weeks of oral acetazolamide on patients with pulmonary vascular disease: A randomized, double-blind, cross-over trial

BackgroundThe carbonic anhydrase inhibitor acetazolamide stimulates ventilation through metabolic acidosis mediated by renal bicarbonate excretion. In animal models, acetazolamide attenuates acute hypoxia-induced pulmonary hypertension (PH), but its efficacy in treating patients with PH due to pulmonary vascular disease (PVD) is unknown. Methods28 PVD patients (15 pulmonary arterial hypertension, 13 distal chronic thromboembolic PH), 13 women, mean±SD age 61.6±15.0 years stable on PVD medications, were randomised in a double-blind crossover protocol to 5 weeks acetazolamide (250mg b.i.d) or placebo separated by a ≥2 week washout period. Primary endpoint was the change in 6-minute walk distance (6MWD) at 5 weeks. Additional endpoints included safety, tolerability, WHO functional class, quality of life, arterial blood gases, and hemodynamics (by echocardiography). ResultsAcetazolamide had no effect on 6MWD compared to placebo (treatment effect: mean change [95%CI] -18 [-40 to 4]m, p=0.102) but increased arterial blood oxygenation through hyperventilation induced by metabolic acidosis. Other measures including pulmonary hemodynamics were unchanged. No severe adverse effects occurred, side effects that occurred significantly more frequently with acetazolamide vs. placebo were change in taste (22/0%), paraesthesia (37/4%) and mild dyspnea (26/4%). ConclusionsIn patients with PVD, acetazolamide did not change 6MWD compared to placebo despite improved blood oxygenation. Some patients reported a tolerable increase in dyspnoea during acetazolamide treatment, related to hyperventilation, induced by the mild drug-induced metabolic acidosis. Our findings do not support the use of acetazolamide to improve exercise in patients with PVD at this dosing. ClinicalTrials.gov IdentifierNCT02755298

ODP336 Nephrogenic Diabetes Insipidus and Fanconi Syndrome Induced by Ifosfamide in a Patient with Femur Osteosarcoma. A Case Report

Abstract Background Fanconi syndrome and diabetes insipidus are rare adverse effects of ifosfamide. They usually present with high cumulative doses of this medication. They are mainly related to type II proximal renal tubular dysfunction. These complications typically present with severe polydipsia and polyuria accompanied by glycosuria, proteinuria, and some electrolyte abnormalities including hypokalemia, hypophosphataemia, and non-anion gap metabolic acidosis. Clinical case: A 15-year-old male with, a diagnosis of metastatic osteoblastic osteosarcoma in the right distal treated with ifosfamide three years prior to this hospitalization, was admitted to the emergency department complaining of 3 episodes of vomiting, 6 days history of increase volume of the right lower limb and fever. On admission, blood pressure of 80/50 mm Hg, heart rate of 110 beats / minute, respiratory rate of 30 breaths / minute, axillary temperature of 38.3 ° C. It was evident phlogosis at level of the proximal third of the right lower limb. CBC revealed pancytopenia (WBC 5 x10 9 L, Hb 6.8 g/dL, platelets 25×10 3 /mm 3) and ABG and CMP were compatible with mild metabolic acidosis normal AG (pH 7.285, K 1.9 meq / L, Na 156 meq / L, lactate: 0.9 meq / L, HCO3: 17 meq / L). A diagnosis of sepsis was made and the patient was started on meropenem 2g IV every 8 hours, vancomycin 1 g IV every 12 hours, filgastrim 120 ug/daily and one unit of packed RBC. Patient improved after 5 days over the course of his hospitalization. However, on the 18 th day after his hospital admission, he developed a new episode of polyuria, associated with hypocalcemia (7 mg/dL), hypokalemia (2 mEq/L), hypomagnesemia (0.95 mg/dL), hypophosphatemia (0.9 mg/dL) and metabolic acidosis (pH 7.2, bicarbonate 15 mEq/L). Urine analysis revealed glucosuria and proteinuria. Desmopressin test did not showed increase urinary osmolarity thus nephrogenic diabetes insipidus and Fanconi syndrome were diagnosed due to the multiple laboratory abnormalities and sign/symptoms. Patient received electrolyte replacement and ifosfamide was stopped from his chemotherapy regimen. After this, patient recovers and he was discharge at 25 th day of his admission. Conclusion : Ifosfamide causes tubular cell dysfunction leading to both nephrogenic diabetes insipidus and Fanconi syndrome. These complications might event present several years after receiving this chemotherapeutic agent. Full electrolyte repletion and hydration are the gold standard for management The present case report emphasizes the importance of the prevention of nephrotoxicity associated with ifosfamide, since its presentation could increase poor outcomes in patients receiving chemotherapy regimens that include ifosfamide. Presentation: No date and time listed

S2079 Not Just Another Case of Diarrhea: A Case of Vibrio Cholera During COVID-19 Era

Introduction: Vibrio cholerae is a gram negative, comma-shaped, toxin producing bacteria, which causes large volumes of watery stool leading to dehydration, hypovolemic shock, and metabolic acidosis. Stool culture with PCR is the gold standard for diagnosis. It is pertinent that if cholera is a differential, treatment with isotonic oral rehydration and antibiotics are not delayed. When traveling to endemic or epidemic areas vaccination should be considered. Here we discuss a case of Vibrio cholera infection diagnosed in Farmington, Connecticut in a patient with recent travel back from Pakistan in mid-April 2022. Case Description/Methods: 61-year-old female with no relevant past medical history presented to the hospital complaining of fatigue and 3 days of profuse, watery, dark diarrhea occurring 8 times daily, starting the day she returned home to Connecticut after traveling in Pakistan. She drank primarily store-bought bottled water but reported some cooking with tap water. She was lethargic and hypovolemic with a diffusely tender abdomen and hyperactive bowel sounds. Lab work was pertinent for leukocytosis, AKI, hypokalemia, and mild metabolic acidosis. Hemoccult stool, Clostridium difficile toxins A&B, Salmonella, Shigella, and fecal leukocyte count were negative. Her course was complicated by altered mental status, worsening AKI, hypokalemia, and metabolic acidosis requiring a bicarbonate drip. Eight days into her admission stool study resulted positive for cholera (Figure 1). Her metabolic derangements were aggressively treated, and her symptoms improved. Discussion: During the COVID-19 pandemic and ongoing travel restrictions, cholera is not typically the first infection thought of for patients with diarrhea and fatigue. Still, we are seeing cases rise in locations where cholera case-fatality rates were previously dropping in a pre-pandemic COVID-era, thought to be from reduced health promotion activities.1 In 2019, the Center for Disease Control and Prevention reported a total of 14 cases of Vibrio cholera within the United States,2 most from overseas travel. Thus, it is important to keep a broad differential diagnosis regardless of location even during a time of travel restrictions.Figure 1.: Vibrio culture on thiosulfate-citrate-bile salts-sucrose (TCBS) agar (left) and on blood agar (right).

Changes of Acid-Base Variables in Dairy Cows with Chronically Implanted Fetal and Maternal Catheters during Late Gestation and Calving.

Simple SummaryA fetal catheterization is an efficient tool allowing longitudinal in vivo studies on hormonal and metabolic changes, including fetal blood gases and acid-base changes. These surgical techniques made it possible to take blood samples daily under aseptic conditions to determine arterial and/or venous blood samples for acid-base variables (like pH, blood gas tensions: partial pressure of carbon dioxide and partial pressure of oxygen, oxygen saturation, bicarbonate concentration, total carbon dioxide, and base excess). All these examinations may contribute to a better understanding of the physiological changes that occur during calving, which may help reach a significant reduction in losses caused by perinatal mortality, which is still high today.The objective of the present study was to evaluate the changes in maternal and fetal arterial acid-base variables withdrawn from catheterized dams and fetuses during the last days before and during calving. The average gestation length in nine cows with chronically catheterized fetuses was 285 ± 10 (SD) days. The arterial acid-base variables of a catheterized dam and fetus were very stable during late gestation. Four newborn calves showed small differences between prenatal and postnatal pH values (−0.035). At the same time, pCO2 values started to increase significantly (p = 0.02), indicating a shift towards physiological respiratory acidosis during calving. The partial pressure of oxygen and oxygen saturation values showed some non-significant improvements immediately after birth, while the other acid-base parameters did not differ. The remaining five newborn calves showed a significant decrease in arterial blood pH (p < 0.01) and BE (p = 0.01), while pCO2 tended to be higher (p = 0.06), indicating a shift towards physiological respiratory and metabolic acidosis, while the other acid-base parameters hardly differed. It is essential to mention that physiological (n = 2) and mild metabolic acidosis (n = 2) developed gradually in four newborn calves during the second stage of calving, lasting about ≤ 2 h. In contrast, in the remaining newborn calf the physiological metabolic acidosis developed during the last 3 min of birth because immediately before birth, the BE value was 0.4 mmol/L. After birth, it was −5.4 mmol/L. The results indicate that the acid-base variables may start to move gradually in the direction of expressed respiratory and metabolic acidosis only after appearing the amniotic sac and fetal feet in the vulva during the second stage of labor; therefore, it is essential to complete obstetrical assistance in time.

The Correlation of Cord Arterial Blood Gas Analysis Results and Apgar Scores in Term Infants Without Fetal Distress.

Objective:This study aimed to evaluate the necessity of cord arterial blood gas analysis in cases without fetal distress and normal Apgar score.Materials and Methods:The cord arterial blood gas analysis and the 1- and 5-minute Apgar scores data of 1438 cases were evaluated. Newborns with fetal distress, neonates requiring cardiopulmonary resuscitation in the delivery room, congenital anomalies, severe and moderate acidemia (pH ≤7.1 at cord arterial blood gas analysis), and pre- and post-term newborns are excluded. Following cord arterial blood gas analysis, threshold values were accepted as abnormal pH <7.2, base excess ≥ −6 mmol/L, lactate ≥ 5 mmol/L, bicarbonate < 18 mmol/L, and partial pressure of carbon dioxide ≥ 50 mmHg. We evaluated the correlation between cord arterial blood gas analysis and 1- and 5-minute Apgar scores.Results:There was a significant correlation between both 1- and 5-minute Apgar scores and cord arterial blood gas analysis values such as pH, lactate, and partial pressure of carbon dioxide (P < .001). In addition, a significant correlation was found between the 5-minute Apgar score of <7 and some cord arterial blood gas analysis abnormal threshold values (pH, bicarbonate, base excess) (P < .001). We found that some patients with mild acidemia had 1- and 5-minute Apgar scores of ≥7 in 1.9% and 2% of cases, respectively.Conclusion:The 5-minute Apgar score of 7 or higher may not be sufficient to verify the well-being of a newborn. Relying only on the Apgar scores may create the risk of missing some newborns with mild metabolic acidosis. The necessity of routine cord arterial blood gas analysis should be considered in prospective studies even if there are no signs of fetal distress and Apgar score ≥7.

NOVEL MUTATION IN THE CYP11B2 GENE IN AN INFANT WITH CONGENITAL HYPOALDOSTERONISM

We report a 4-monthboy infant withisolated congenital hypoaldosteronism due to a novel mutation in CYP11B2 gene who presented with, failure to thrive, profound hyponatremia, hyperkalemia and mild metabolic acidosis, which managed byintravenous saline, oral saline, resoniumandcaloriesformula. In infants with severe electrolyte disturbance,it is important to rule out CAH and consider other rare form of isolated hypoaldosteronism and pseudohypoaldosteronism, since prompt and appropriate treatment will correct the associated electrolytes abnormalities.

Methanol Intoxication After Suicidal Ingestion of Liquid Rodenticides: A Report of Two Cases.

Methanol is highly toxic to humans. Although methanol poisoning is not uncommon in developing countries, poisoning caused by ingestion of commercial products containing undeclared methanol has rarely been reported. Herein, we describe two patients who experienced methanol poisoning after ingestion of liquid rodenticides. A 39-year-old woman attempted suicide by ingesting liquid rodenticide which contained bromadiolone. She developed high anion gap metabolic acidosis and coagulopathy. Methanol poisoning was confirmed 20 hours later. She received oral ethanol therapy and hemodialysis. Vitamin K1 was also administered. She did not develop any hemorrhage or visual impairment and was discharged after 11 days. The rodenticide sample was tested and found to have a methanol concentration of 324 g/L. In another case, a 62-year-old man ingested the same brand of rodenticide. Laboratory data showed mild metabolic acidosis with an increased osmol gap, suggestive of methanol poisoning. He received hemodialysis and eventually recovered without sequelae. Liquid rodenticide may contain methanol as a solvent. Ingestion of a methanol-containing commercial product without a clear label can result in a considerable delay in diagnosis and management. Methanol poisoning should be considered for patients who present with unexplained metabolic acidosis following exposure to liquid rodenticides or other liquid commercial products.

Case Report: A Case of Encephalopathy Presenting the Lentiform Fork Sign on MRI in a Diabetic Dialysis Patient

Basal ganglia lesions showing an expansile high signal intensity on T2-weighted MRI are termed the lentiform fork sign. This specific finding is mainly observed in diabetic patients with uremic encephalopathy with metabolic acidosis, although there are also reports in patients with ketoacidosis, dialysis disequilibrium syndrome, intoxication, and following drug treatment (e.g., metformin). A 57-year-old Japanese man on chronic hemodialysis for 4 years because of diabetic nephropathy was admitted to our hospital for relatively rapid-onset gait disturbance, severe dysarthria, and consciousness disturbance. Brain T2-weighted MRI showed the lentiform fork sign. Hemodialysis was performed the day before admission, and laboratory tests showed mild metabolic (lactic) acidosis, but no uremia. Surprisingly, metformin, which is contraindicated for patients with end-stage kidney disease, had been prescribed for 6 months in his medication record, and his sluggish speaking and dysarthria appeared gradually after metformin treatment was started. Thus, the encephalopathy was considered to be related to metformin treatment. He received hemodialysis treatment for 6 consecutive days, and his consciousness disturbance and dysarthria improved in 1 week. At the 8-month follow-up, the size of the hyperintensity area on MRI had decreased, while the mild gait disturbance remained. Considering the rapid onset of gait and consciousness disturbance immediately before admission, diabetic uremic syndrome may also have occurred with metformin-related encephalopathy, and resulted in the lentiform fork sign, despite the patient showing no evidence of severe uremia on laboratory data.

Hyponatremia, Metabolic Acidosis, and Abnormal Newborn Screen in a Preterm Neonate.

A 2,250-g male neonate is born at 34 weeks of gestation via spontaneous vaginal delivery to a 30-year-old gravida 1, para 0 woman after presentation to a community hospital with uterine contractions and spontaneous rupture of membranes. The mother receives 1 dose of ampicillin, 1 dose of betamethasone, and magnesium before delivery. At delivery, the neonate is vigorous and crying. His Apgar scores are 8 and 9 at 1 and 5 minutes after birth, respectively, with no abnormalities noted on physical examination. In the first couple of hours after birth, he develops persistent oxygen desaturations and is started on continuous positive airway pressure.He is admitted to the NICU for prematurity and low birthweight. Admission examination demonstrates nondysmorphic features, no respiratory distress with good air exchange, regular heart rate and rhythm without murmur, soft and flat abdomen, patent anus, normal external male genitalia, unremarkable neurologic findings, and pink skin color.Chest radiography shows mild diffuse ground-glass reticular densities. He is placed on a stock solution of parenteral nutrition via a peripheral intravenous catheter. Antibiotics are started because of the mother’s unknown group B Streptococcus status and preterm labor. Initial surveillance of electrolytes demonstrates sodium, potassium, calcium, phosphorus, and glucose levels within normal limits. On day 4 after birth, the neonate continues on nasal canula for tachypnea, receiving parenteral nutrition, and advancing gavage feeds of maternal breast milk or formula. A capillary blood gas measurement shows mild metabolic acidosis, with a pH of 7.28, Pco2 32 mm Hg (4.26 kPa), and bicarbonate 19 mEq/L (19 mmol/L).On day 6 after birth, repeat surveillance of electrolytes shows a sodium concentration of 126 mEq/L (126 mmol/L) and potassium of 6.7 mEq/L (6.7 mmol/L). Glucose concentration is 53 mg/dL (2.94 mmol/L). Blood gas continues to show mild metabolic acidosis with a pH of 7.27, Pco2 31 mm Hg (4.12 kPa), and bicarbonate 19 mEq/L (19 mmol/L). Results of the state newborn screening demonstrate an elevated 17-hydroxyprogesterone (17-OHP) of 221 nmol/L (normal <80 nmol/L) on dried blood spot testing, or 14,586 ng/dL (normal <5,280 ng/dL) as serum equivalent. A confirmatory serum 17-OHP level is sent that same day. The pediatric endocrinology service is consulted for the concerning laboratory results.The neonate is transferred to a tertiary care pediatric hospital for further management. He is started on hydrocortisone and fludrocortisone replacements along with sodium chloride supplementation. Repeat serum 17-OHP before treatment is greater than 50,000 ng/dL, and the actual diagnosis of classic congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21-OHD) is made.The neonate continues to appear well with normal vital signs, including blood pressure. He is breathing room air, receiving full enteral feeds, and working on oral feeds with the occupational therapist. Parenteral nutrition has been discontinued.The differential diagnosis for hyponatremia and hyperkalemia in a neonate includes CAH, pseudohypoaldosteronism, inappropriate parenteral nutrition composition, and exogenous diuretic use.CAH is a group of autosomal recessive disorders encompassing enzyme deficiencies in the adrenal steroidogenesis pathway and is the most common cause of primary adrenal insufficiency in children. The classic form of CAH affects 1 in 15,000 neonates and is commonly identified through newborn screening, (1) with screening mandated across the United States since 2008. (2) Most cases of CAH are caused by 21-OHD, which is marked by an elevated serum 17-OHP. In the steroidogenic pathway, 17-OHP is the precursor in the enzymatic reaction that is catalyzed by 21-OHD, and is the primary analyte tested in newborn screening for CAH. If treatment is not initiated early, neonates may present in acute salt-wasting adrenal crisis with vomiting, hypotension, and severe dehydration, electrolyte abnormalities, and hypoglycemia. (1)(3)(4)(5) Female neonates with classic CAH also present with virilization of external genitalia at birth.False-positive newborn screening results for CAH are common in preterm neonates, related to the fact that healthy preterm neonates have higher overall levels of 17-OHP compared with term neonates. (6) Thus, many screening programs have established reference ranges that are based on weight and gestational age.For neonates with classic CAH, therapy should be initiated soon after birth to reduce the potentially life-threatening sequelae of CAH. Classic CAH is treated with steroid hormone replacements including hydrocortisone (to replace cortisol) and fludrocortisone (to replace aldosterone). Supplemental sodium chloride (salt) is also recommended for neonates with classic CAH who could have salt-wasting.