Pyridoxine Deficiency and Neurologic Dysfunction: An Unlikely Association.

Pyridoxine deficiency is a prevalent condition in the United States that primarily affects patients with alcohol use disorder. The presentation of this condition is very nonspecific and commonly presents with a constellation of symptoms including peripheral neuropathy, stomatitis, dermatitis, confusion, depression, encephalopathy, and seizures. Over half of these patients have associated alcohol use disorder, which causes pyridoxine deficiency due to the breakdown of pyridoxal phosphate during ethanol metabolism in the liver. As an important cofactor in the synthesis of γ-aminobutyric acid (GABA), deficient levels of pyridoxine may lower the seizure threshold due to reduced GABA-mediated inhibition. This case details a 57-year-old male with chronic alcoholism and a history of seizures who developed episodes of myoclonic jerks, tremors, anxiety, and neuropathy whose symptoms persisted even while on anti-epileptic medication. He was found to have pyridoxine deficiency and had full resolution of symptoms shortly after the administration of vitamin B6 supplementation. Pyridoxine deficiency may lead to severe neurologic disorders such as encephalopathy and seizures. Hence, it is important to consider pyridoxine deficiency in the workup of neurologic complaints, especially in high-risk patients.

Preventing Vitamin B6-Related Neurotoxicity.

BACKGROUND: Vitamin B6 is essential for life and plays a critical role in many biochemical and physiological processes in the human body. The term B6 collectively refers to 6 water-soluble vitamers, and only the pyridoxal 5'-phosphate (PLP) serves as the biologically active form. A plasma PLP concentration above 30 nmol/L (7.4 µg/L) is indicative of an adequate vitamin B6 status for all age and sex groups. The currently recommended daily allowance of B6 (1.5-2 mg/d) from dietary sources frequently results in inadequate B6 status (<20 nmol/L or 5 µg/L) in many elderly patients and patients with comorbid conditions. PLP-based supplements are preferred and should be administered weekly in low doses (50-100 mg) to maintain a stable serum PLP level between 30 and 60 nmol/L or 7.4 and 15 µg/L. AREAS OF UNCERTAINTY: It is challenging for physicians to prescribe a safe dose of B6 supplements because of the narrow therapeutic index. The association between elevated levels of pyridoxine and neuropathy is not well established. PLP-based supplements are shown to be least neurotoxic, but further clinical trials are needed to establish the long-term safety in high doses. DATA SOURCES: PubMed search of randomized control trials and meta-analyses. THERAPEUTIC OPINION: Plasma B6 levels should be ordered as a part of workup of any unexplained anemia before labeling as "anemia of chronic disease." B6 supplementation is also crucial in the management of chronic Mg deficiency resistant to therapy. When B6 is administered daily in supraphysiologic doses, there is a potential for the development of neurotoxicity (typically at levels >100 nmol/L or 25 µg/L). PLP-based supplements are preferred over pyridoxine supplements because of minimal neurotoxicity observed in neuronal cell viability tests. Since B6 metabolites have a long half-life, weekly administration is preferred over daily use to prevent toxicity.

Magnesium Supplementation in Vitamin D Deficiency.

BACKGROUND: Vitamin D and magnesium (Mg) are some of the most studied topics in medicine with enormous implications for human health and disease. Majority of the adults are deficient in both vitamin D and magnesium but continue to go unrecognized by many health care professionals. AREAS OF UNCERTAINTY: Mg and vitamin D are used by all the organs in the body, and their deficiency states may lead to several chronic medical conditions. Studies described in the literature regarding these disease associations are contradictory, and reversal of any of these conditions may not occur for several years after adequate replacement. One should consider the supplementation therapy to be preventative rather than curative at this time. DATA SOURCES: PubMed search of several reported associations between vitamin D and Mg with diseases. RESULTS: Vitamin D and Mg replacement therapy in elderly patients is known to reduce the nonvertebral fractures, overall mortality, and the incidence of Alzheimer dementia. CONCLUSIONS: Vitamin D screening assay is readily available, but the reported lower limit of the normal range is totally inadequate for disease prevention. Based on the epidemiologic studies, ∼75% of all adults worldwide have serum 25(OH)D levels of <30 ng/mL. Because of the recent increase in global awareness, vitamin D supplementation has become a common practice, but Mg deficiency still remains unaddressed. Screening for chronic magnesium deficiency is difficult because a normal serum level may still be associated with moderate to severe deficiency. To date, there is no simple and accurate laboratory test to determine the total body magnesium status in humans. Mg is essential in the metabolism of vitamin D, and taking large doses of vitamin D can induce severe depletion of Mg. Adequate magnesium supplementation should be considered as an important aspect of vitamin D therapy.

Severe beta blocker and calcium channel blocker overdose: Role of high dose insulin.

A 54-year-old female presented after taking an overdose of an unknown amount of hydrochlorothiazide, doxazocin, atenolol and amlodipine. She was initially refractory to treatment with conventional therapy (intravenous fluids, activated charcoal, glucagon 5 mg followed with glucagon drip, calcium gluconate 10%, and atropine). Furthermore, insulin at 4 U/kg was not effective in improving her hemodynamics. Shortly after high dose insulin was achieved with 10 U/kg, there was dramatic improvement in hemodynamics resulting in three of five vasopressors being weaned off in 8 h. She was subsequently off all vasopressors after six additional hours. The role of high dose insulin has been documented in prior cases, however it is generally recommended after other conventional therapies have failed. However, there are other reports that suggest it as initial therapy. Our patient failed conventional therapies and responded well only with maximum dose of insulin. Physicians should consider high dose insulin early in severe beta blocker or calcium channel blocker overdose for improvement in hemodynamics. This leads to early discontinuation of vasopressors. It is important that emergency physicians be aware of the beneficial effects of high dose insulin when initiated early as opposed to waiting for conventional therapy to fail; as these patients often present first to the emergency department. Early initiation in the emergency department can be beneficial in these patients.

Hidden incision endoscopic surgery (HIdES) trocar placement for pediatric robotic pyeloplasty: comparison to traditional port placement.

Robotic assisted laparoscopy pyeloplasty (RALP) has been associated with shorter recovery, less pain and improved cosmesis. To minimize visible scars, the hidden incision endoscopic surgery (HIdES) trocar placement has been previously developed. Our aim was to compare outcomes between the HIdES and traditional port placement (TPP) for pediatric RALP. A retrospective study was performed on patients under 15 years of age who underwent RALP at a single institution between August 2011 and November 2013. Patient demographics, intraoperative details, narcotic administration, and complications were reviewed. A total of 49 patients were identified (29 in HIdES, 20 in TPP). There was no difference in median age (p = 0.77) or median height (p = 0.88) between the two groups. Median operative time was 180 min for HIdES and 194 min for TPP (p = 0.27). Eleven patients (11/29, 37.9%) in the HIdES group and fourteen patients (14/20, 70%) in the TPP group received postoperative narcotics (p < 0.05). Median follow-up was 42 months for HIdES and 41 months for TPP (p = 0.96). There were two complications (2/29, 6.9%) with HIdES, and one complication (1/20, 5.0%) with TPP (p = 1.00). The success rates were 96.6% (28/29) for HIdES and 100% (20/20) for TPP (p = 1.00). HIdES trocar placement for pediatric robotic pyeloplasty is a safe and viable alternative to TPP. HIdES is comparable to TPP regarding operative time, narcotic administration, hospital stay, and complication rate, without compromising success.

Empiric Antibiotic Therapy of Nosocomial Bacterial Infections.

Broad-spectrum antibiotics are commonly used by physicians to treat various infections. The source of infection and causative organisms are not always apparent during the initial evaluation of the patient, and antibiotics are often given empirically to patients with suspected sepsis. Fear of attempting cephalosporins and carbapenems in penicillin-allergic septic patients may result in significant decrease in the spectrum of antimicrobial coverage. Empiric antibiotic therapy should sufficiently cover all the suspected pathogens, guided by the bacteriologic susceptibilities of the medical center. It is important to understand the major pharmacokinetic properties of antibacterial agents for proper use and to minimize the development of resistance. In several septic patients, negative cultures do not exclude active infection and positive cultures may not represent the actual infection. This article will review the important differences in the spectrum of commonly used antibiotics for nosocomial bacterial infections with a particular emphasis on culture-negative sepsis and colonization.

Use of rivaroxaban in a patient with history of nephrotic syndrome and hypercoagulability.

OBJECTIVE: Hypercoagulability, resulting in thromboembolic events, can be a life-threatening complication of nephrotic syndrome (NS). Conventional anticoagulants, such as warfarin, have been the standard of care for more than 50 years; however, the availability of target-specific oral anticoagulants (TSOACs) have provided additional options for the treatment and prevention of thromboembolic events. Documented use of the TSOACs in patients with NS and hypercoagulability is currently limited. CASE SUMMARY: We present the case of an 18-year-old young woman with NS and renal vein thrombosis who was readmitted with bilateral pulmonary emboli on therapeutic doses of warfarin, with a goal international normalized ratio of 2.0 to 3.0. The decision was made to transition the patient from warfarin to rivaroxaban, an oral factor Xa inhibitor. DISCUSSION: Rivaroxaban was the first of the emerging TSOACs to be FDA approved for both prevention and treatment of venous thromboembolism. With favorable safety and efficacy data compared with warfarin in addition to a predictable pharmacokinetic profile and the lack of requirement of routine monitoring, rivaroxaban provides a useful alternative in this patient population. SUMMARY: While on therapeutic anticoagulation, a patient previously diagnosed with NS and renal vein thrombosis experienced pulmonary emboli on a conventional anticoagulant and was switched to a target-specific oral anticoagulant with documented completion of 6 months of therapy without recurrent thromboembolism.

Predictors of significant jaundice in late preterm infants.

OBJECTIVES: To study (i) the incidence and course of jaundice, and (ii) the predictors of significant jaundice in late preterm infants. DESIGN: Prospective analytical study. SETTING: Urban perinatal center. PATIENTS: Inborn late preterm infants (post menstrual age of 34 0/7 to 36 6/7 weeks). METHODS: Infants were followed till day 14 of life or till onset of significant jaundice. Relevant maternal, perinatal and neonatal variables were prospectively recorded. Transcutaneous bilirubin (TcB) was measured in each infant twice daily for the first 48 hours of life. OUTCOMES: Significant jaundice defined as requirement of phototherapy/exchange transfusion as per hour specific total serum bilirubin (TSB) nomogram of AAP guidelines. RESULTS: 216 infants were enrolled, of which 123 (57%) had significant jaundice. 36% of the jaundiced infants had TSB greater than 15 mg/dL. The mean duration of onset of significant jaundice was 61 ± 32 hours. The mean duration of phototherapy was 49 ± 26 hours. Large for gestation, lower gestational age, birth trauma and previous sibling with jaundice predicted severe jaundice. TcB measured at 24-48 hrs was a better predictor of significant jaundice with onset after 48 hrs than clinical risk factors. CONCLUSION: There is a high incidence of significant jaundice in late preterm infants. TcB measured at 24-48 hrs of life better predicts significant jaundice after 48 hours of life, in comparison with clinical risk factors.

The impact of the alexander technique on improving posture and surgical ergonomics during minimally invasive surgery: pilot study.

PURPOSE: One of the main ergonomic challenges during surgical procedures is surgeon posture. There have been reports of a high number of work related injuries in laparoscopic surgeons. The Alexander technique is a process of psychophysical reeducation of the body to improve postural balance and coordination, permitting movement with minimal strain and maximum ease. We evaluated the efficacy of the Alexander technique in improving posture and surgical ergonomics during minimally invasive surgery. MATERIALS AND METHODS: We performed a prospective cohort study in which subjects served as their own controls. Informed consent was obtained. Before Alexander technique instruction/intervention subjects underwent assessment of postural coordination and basic laparoscopic skills. All subjects were educated about the Alexander technique and underwent post-instruction/intervention assessment of posture and laparoscopic skills. Subjective and objective data obtained before and after instruction/intervention were tabulated and analyzed for statistical significance. RESULTS: All 7 subjects completed the study. Subjects showed improved ergonomics and improved ability to complete FLS™ as well as subjective improvement in overall posture. CONCLUSIONS: The Alexander technique training program resulted in a significant improvement in posture. Improved surgical ergonomics, endurance and posture decrease surgical fatigue and the incidence of repetitive stress injuries to laparoscopic surgeons. Further studies of the influence of the Alexander technique on surgical posture, minimally invasive surgery ergonomics and open surgical techniques are warranted to explore and validate the benefits for surgeons.

Use of customized MIC-KEY gastrostomy button for management of MACE stomal complications.

OBJECTIVES: Severe bowel dysfunction often accompanies neurogenic bladder, and Malone antegrade continence enema procedures can lead to improvement in the quality of life for these patients. The most commonly reported complication has been stomal stenosis of the conduit. We report our experience with the use of the MIC-KEY gastrostomy button for the management of this complication. METHODS: A retrospective cohort study of 53 consecutive children who had undergone the Malone antegrade continence enema (ACE) procedure from 2000 to 2006 was performed. The records of patients with stomal complications were assessed for presentation, management, and outcomes. RESULTS: We identified 20 patients (38%) with stomal stenosis. Stomal dilation and stenting alone was successful in 4 patients, and stomal revision was necessary in 16 (30%). Six children required placement of the MIC-KEY gastrostomy button into the ACE stoma for recurrent stomal stenosis (n = 5) or a buried stoma (n = 1). All patients were successfully treated with placement of a MIC-KEY button to continue their bowel irrigation. The size of the button was determined by the endoscopic assessment of the length and width of the ACE stoma and was custom made by the manufacturer. The average time that the button was in place was 26 months, and it was generally changed every 3 months. CONCLUSIONS: In the rare subset of patients with recurrent stomal stenosis or a buried stoma, the MIC-KEY gastrostomy button was found to be a practical and easy method for the management of these conditions and to preserve the ACE conduit.

Perforation of Malone antegrade continence enema: diagnosis and management.

PURPOSE: Severe bowel dysfunction often accompanies neurogenic bladder, and Malone antegrade continence enema (MACE) procedures can lead to improvement in the quality of life of these patients. However, complications such as catheter false passage with subsequent intraperitoneal instillation of irrigation can lead to significant morbidity. We present our experience with the diagnosis and management of this condition. MATERIALS AND METHODS: The records of all patients undergoing MACE procedures at pediatric institutions from 1989 to 2002 were retrospectively reviewed. The records of patients diagnosed with a perforation were assessed for presentation, initial imaging studies, management and outcomes. RESULTS: Of 187 consecutive patients treated with MACE procedures we identified 6 females and 1 male (3.7%). Mean patient age at initial surgery was 11.3 years. Of the 7 patients presented within 3 months of the initial surgery, 6 presented with abdominal pain after irrigation and 4 reported traumatic catheterization. Six patients had extravasation of contrast material on imaging studies. Two patients presented with peritonitis and underwent immediate laparotomy. In 5 patients endoscopy was performed with catheter placement which was then maintained for 6 weeks. After a mean followup of 4.7 years 4 patients have complete continence, 2 have a MACE button in place and 1 has mild fecal leakage. CONCLUSIONS: MACE procedures have a low incidence of conduit false passage and perforation. Prompt diagnosis and early intervention are crucial to management. Endoscopic evaluation with catheter placement can be helpful in preserving continence and decreasing morbidity.