Normalization Of Laboratory Values Research Articles

An unusual etiology in an 8-year-old girl with chest pain and elevated troponin-I.

Acute post-streptococcal glomerulonephritis (APSGN) is the most common glomerulonephritis of childhood, and clinical presentation can vary widely. This case report presents an atypical manifestation of APSGN in an 8-year-old female patient with pleuritic chest pain and elevated troponin-I, despite lacking classical kidney symptoms. Imaging studies showed cardiomegaly and interstitial lung opacities. Further investigations revealed hematuria and proteinuria, and the diagnosis was confirmed through elevated antistreptolysin-O (ASO) titers and low complement 3 (C3) levels. The patient was successfully managed with fluid restriction, diuretics, and antihypertensives, resulting in the resolution of symptoms and normalization of laboratory values. This case highlights the significance of recognizing atypical manifestations of APSGN for ensuring prompt diagnosis and proper management in the pediatric population.

Antibiotic-induced DRESS syndrome (drug reaction with eosinophilia and systemic symptoms) in an infant with Down syndrome

IntroductionDrug Rash with Eosinophilia and Systemic Symptoms syndrome (DRESS) is a rare but serious drug reaction. This syndrome is associated with an immune reaction triggered by the use of certain medications. Rare cases of DRESS have been reported in early infancy or in infants with chromosomal disorders, making their prognosis unknown. ObservationA 20-month-old infant with trisomy 21 was hospitalized for a febrile (39°C) rash with systemic symptoms. He had been hospitalized twenty days earlier for pneumonia, which was treated with antibiotics (cefotaxime and vancomycin). Physical examination revealed facial edema, a diffuse maculopapular rash involving the entire body and face and cervical lymphadenopathy. Laboratory tests showed leukocytosis with eosinophilia and liver damage (liver transaminases were 20 times the normal level). The diagnosis of DRESS syndrome due to antibiotics was made based on a RegiSCAR score of 7. The infant was treated with IV corticosteroids at a dose of 1mg/kg/day, along with a 2g/kg infusion of gammaglobulin. Clinical and laboratory improvement was observed with disappearance of skin lesions and normalization of laboratory values. DiscussionThe diagnosis of DRESS syndrome due to antibiotics was made based on a RegiSCAR score of 7. This class of drugs is the leading cause of DRESS syndrome described in children. The evolution of our infant patient with Down syndrome and DRESS syndrome was favorable.

(101) Ovarian Hyperthecosis as a Cause of Hypersexualization in a Post-menopausal Female

Abstract Introduction Ovarian Hyperthecosis (OH) is a rare benign virilizing ovarian syndrome characterized by androgen overproduction due to stromal and thecal hyperplasia resulting in hirsutism and virilization(1). It is believed to be similar to polycystic ovarian syndrome (PCOS) however, presents with more robust androgen overproduction and an ovarian vein ratio of androstenedione/testosterone less than one, compared with values around 20 in pre-menopausal females and patients with PCOS(2). Specifically in post-menopausal women, the phenotypical presentation is due to unmasking of the increased thecal cell testosterone release which results from lack of granulosa cell mediated aromatization of testosterone to estradiol.(3) Some have described treatment of OH with medications that inhibit ovarian production of estrogen and progesterone especially in pre-menopausal women. However, results are mixed and oophrectomy seems to be the most effective treatment modality for OH(3). Objective Here we describe a case of OH resulting in elevated androgen levels and distressing hypersexualization in a post-menopausal female who was effectively treated with bilateral oophorectomy resulting in normalization of laboratory values and resolution of hypersexualization. Methods The patient is a 75-year-old female with past medical history significant for bipolar disorder, Sjogren’s syndrome and hypothyroidism who presented to the office for evaluation of recurrent vulvovaginitis and urinary tract infections related to vigorous and frequent sexual activity. She reported symptoms of hypersexuality developing a few years prior to presentation at which time she began engaging in sexual activity 2-3 times daily and masturbating with a variety of objects. She had also noted new heavy facial hair and scalp hair loss. There were no changes in her medical history at the time. She was bothered by the increased need for sexual stimulation and orgasm, prompting further evaluation and treatment. Her exam was notable for vulvar contact dermatitis, mild vulvovaginal atrophy, and no prolapse or urinary incontinence. Vaginal infection testing was negative. Results of an endocrine work up are summarized in Table 1. Abdominal imaging showed normal adrenal glands and ovaries. An MRI of the brain showed a normal pituitary gland. She had been started on Prozac by her psychiatrist, which reduced her libido but worsened her mania and therefore was discontinued. A diagnosis of OH was suspected and bilateral oophorectomy was planned. Results The patient underwent a laparoscopic bilateral salpingo-ophrectomy with surgical pathology confirming stromal hyperplasia and hyperthecosis. Post-operatively, her labs rapidly normalized. She noted normalization of her libido, significant improvement in vulvar contact dermatitis and increased scalp hair growth. Conclusions Bilateral oophorectomy in a post-menopausal woman with OH was an effective treatment to normalize androgen levels and restore normal libido resulting in resolution of vulvar contact dermatitis from excessive masturbation. Although an uncommon diagnosis, this disease is an important consideration in the workup of excess androgen production in post-menopausal women. Disclosure No

Sulfasalazinom indukovan DRESS (drug reaction with eosinophilia and systemic symptoms) sindrom sa teškim akutnim hepatitisom - prikaz slučaja

Introduction: Drug Reaction with Eosinophilia and Systemic Symptoms Syndrome is a rare and severe form of drug induced delayed-type hypersensitivity reaction with mortality rate up to 10%. It usually manifests with skin rash, fever, lymphadenopathy, hematological abnormalities and involvement of one or more internal organs. Establishing the diagnosis is sometimes late due to variable clinical presentation. Current recommendations for therapy rely mainly on expert opinions, retrospective studies, case reports and series. Sulfasalazine was firstly synthesized in 1930 and is currently being prescribed for various inflammatory and rheumatic diseases. Case report: We present a 45-year-old patient who was prescribed sulfasalazine tablets by a rheumatologist due to reactive arthritis. In the fourth week of therapy, he developed skin rash and fever up to 39.5°C. On admission, generalized maculopapular exanthema covering over 60% of the body surface area, edema of the lower eyelids and bilateral cervical and inguinal lymphadenomegaly were registered. Laboratory findings showed leukocytosis with significant eosinophilia, lymphocytosis, elevated bilirubin values, ALT <100 U/L, while ultrasonography of the upper abdomen confirmed hepatosplenomegaly. The patient was diagnosed with Drug Reaction with Eosinophilia and Systemic Symptoms Syndrome using RegiSCAR and J-SCAR diagnostic criteria and systemic therapy with methylprednisolone at a dose of 1.5 mg/kg and other supportive therapy was applied, which resulted in complete regression of the skin changes and normalization of laboratory values. Conclusion: The authors would like to recall the occurrence of Drug Reaction with Eosinophilia and Systemic Symptoms Syndrome as a rare and potentially fatal drug reaction in which awareness of this disease is of key importance for early recognition. Identification of the offending drug followed by a prompt withdrawal and subsequent treatment is essential for decreasing disease related morbidity and mortality and thus we wish to familiarize the sulfasalazine prescribers with this syndrome.

Surgical Site Infection After Posterior Stabilization of the Spine - When do we Have to Remove the Implants?

Retrospective data analysis. The primary objective of this investigation was to analyze if treatment of Postoperative surgical site infections (PSSI) after posterior stabilization of the spine (PS) without radiological signs of screw loosening (RSL) shows a sufficient success rate without implant removal and if there was any difference between early and late PSSI. PSSI after PS are usually treated by implant removal and reinstrumentation if loosening of one of more screws is detected. There is presently no conclusive data that shows the success rate of the treatment of PSSI after PS without implant removal if no RSL are perceived. All patients who were treated for a PSSI after PS without RSL in a single spine center from 12/2009 to 03/2020 were enrolled in a retrospective analysis. Patients were treated by revision surgery with debridement and irrigation and subsequent antibiotic therapy. Implant removal was performed if the initial treatment did not lead to an improvement in wound healing and normalization of laboratory values. Statistical analysis was performed by Statistical Package for the Social Sciences 25. Descriptive data are given as mean and standard error of mean, a χ 2 test was performed. Of the 32 enrolled patients, 17 had an early PSSI, 15 a late PSSI. In 71.9% (23/32), the PSSI was treated without implant removal: 12/17 in early PSSI, 11/15 in late PSSI. The difference was not significant ( P >0.05). One patient died, all other patients were discharged from the hospital with no remaining laboratory signs of the infection and with closed soft tissues. In our group of patients, the success rate of irrigation and debridement without implant removal was 71.9%. In the light of this data, performing at least two irrigations and debridement before implant removal seems to be a valid treatment option in PSSI after PS if there are no RSL in early and late PSSI.

Identifying encephalopathies from acute metabolic derangements.

Metabolic derangements, when acute and severe, affect brain function. This presents mostly with a marked decline in the level of consciousness, resulting in impaired responsiveness, abnormal receptivity, impaired content, and loss of memory retention. The term metabolic encephalopathy has been used but is conjecture that can be challenged in the age of modern neuroimaging. We now recognize that many metabolic encephalopathies may involve structural lesions and at an early stage. Common clinical conundrums are the evaluation of the degree of brain injury and its recoverability. This review discusses the appropriate terminology for these conditions, the diagnostic approach, therapy recommendations, and prediction of recovery potential. In evaluating a presumed metabolic cause for encephalopathy, we must (1) search for and rule out structural injury to the brain despite an obvious explanatory metabolic derangement, (2) recognize that several confounding conditions often co-exist, and (3) acknowledge that resolution of brain dysfunction may be protracted despite normalization of laboratory values.

Taurolidine lock in the treatment of colonization and infection of totally implanted venous access devices in cancer patients.

Taurolidine lock is known to be effective in preventing catheter-related infections in a variety of venous access devices, including long term venous access devices for chemotherapy. Though, literature about the use of taurolidine for treating catheter colonization or catheter-related blood stream infection is scarce. We have retrospectively reviewed the safety and efficacy of 2% taurolidine lock for treatment of catheter-colonization and of catheter-related bloodstream infection in cancer patients with totally implanted venous access devices. Diagnosis of colonization or catheter-related infection was based on paired peripheral and central blood cultures, according to the method of Delayed Time to Positivity. We recorded 24 cases of catheter-related infection and two cases of colonization. Taurolidine lock-associated with systemic antibiotic therapy-was successful in treating all cases of catheter-related infection, with disappearance of clinical symptoms, normalization of laboratory values, and eventually negative blood cultures. Taurolidine lock was also safe and effective in treating device colonization. No adverse effect was reported. In our retrospective analysis, 2% taurolidine lock was completely safe and highly effective in the treatment of both catheter-colonization and catheter-related bloodstream infection in cancer patients with totally implanted venous access devices.

Pancreatic head resections in the setting of celiac axis stenosis: Case report and review of literature

Introduction: Ischemic complications are a notable cause of morbidity in patients after pancreatic head resections. Stenosis of celiac axis in patients undergoing pancreatoduodenectomy requires further perioperative attention. Case report: We present a patient with pancreatic head malignancy scheduled for Whipple procedure in the setting of hemodynamically significant celiac axis stenosis. Despite release of the artery from compression by median arcuate ligament, elevation of liver function tests on the first postoperative day was noted. Endovascular stenting was performed on the same day with significant radiological improvement and subsequent normalization of laboratory values. The patient had no further postoperative complications. Conclusion: Fast recognition of ischemic complications after pancreatic head resection is crucial. Even postoperatively, endovascular intervention might be a feasible treatment modality of celiac axis stenosis in selected patients who undergo pancreatoduodenectomy.

Convalescent plasma to treat coronavirus disease 2019 (COVID-19): considerations for clinical trial design.

KEY IDEAS Case series studying convalescent plasma use in the treatment of COVID‐19 have been promising, but additional, high‐quality studies are needed to determine the efficacy of the treatment when applied for prophylaxis, for early phases of illness, and for severe illness.Previous studies of convalescent plasma in treating other viral diseases have identified factors to consider when designing treatment protocols, including timing of administration relative to onset of illness, timing of donation relative to resolution of symptoms, severity of illness of the donor, pretransfusion serology of the recipient, and antibody titers of the donor.There are many clinical trials studying treatment of, and prophylaxis against, COVID‐19 using convalescent plasma. In addition to clinical trials, the FDA also allows treatment through two other pathways: the “Expanded Access to Convalescent Plasma for the Treatment of Patients with COVID‐19” protocol, and emergency investigational new drug applications. The FDA also provides criteria for donation of convalescent plasma.

A Retrospective Review of Mercaptopurine Metabolism Reveals High Rate of Patients with Suboptimal Metabolites Successfully Corrected with Allopurinol

Introduction: 6-Mercaptopurine (6-MP) is the most frequently used chemotherapy agent in the management of acute lymphoblastic leukemia (ALL) and lymphoblastic lymphoma (LL). Skewed drug metabolism can decrease the effectiveness of 6-MP while also resulting in unnecessary toxicities. Each individual's ability to metabolize 6-MP into the desired therapeutic product, 6-thioguanine nucleotide (6-TGN), is directly opposed by the development of the hepatotoxic byproduct, 6-methyl-mercaptopurine (6-MMPN). Certain individuals, referred to as 'shunters,' preferentially generate high levels of 6-MMPN resulting in decreased production of 6-TGN. Current guidelines suggest holding or lowering 6-MP doses in the setting of toxicity. However this approach results in decreased intensity of 6-MP treatment, potentially risking an increase in disease relapse. Allopurinol, a drug used to manage hyperuricemia, can alter 6-MP metabolism to maximize 6-TGN production while reducing the hepatotoxic metabolite, 6-MMPN. Methods: We performed a single institution, IRB approved, retrospective cohort study to quantify and characterize the rate of shunters treated in our center. Chart review was conducted of ALL and LL patients treated for at least one year of maintenance therapy at our center over a 10 year period from January 1, 2009 to June 1, 2019. Incidents of hypoglycemia (glucose <74), hepatic inflammation (ALT >123), and hypogammaglobulinemia (IgG <400) were recorded. If available, metabolite levels were collected, noting levels of 6-MMPN from 6,000 -10,000 and greater than 10,000 pmol/8 x 108 RBC. If the patient was started on allopurinol, we noted the effects of the intervention on the aforementioned lab values and their clinical course. Stata was utilized for all statistical analysis. Results: We performed a chart review on 42 eligible patients and included demographic information (Table 1). Using lab data, we documented potential markers of toxicity (Table 2). Seventy four percent of patients had a least one episode of documented hypoglycemia, and 88% had at least one episode of elevated ALT > 3 times the upper limit of normal. Metabolites were checked in 66% (28/42) of our patients. 6-MMPN levels were > 10,000 in 82.1% (23/28). Shunting was observed in 54% of the patients; meaning one half of the patients were eligible for allopurinol combination therapy. Allopurinol was initiated by the primary team for metabolite and laboratory derangements in 12 of 23 patients. All patients who received allopurinol had normalization of laboratory values with combined treatment. Discussion: In our population of pediatric and young adults treated for leukemia and lymphoblastic lymphoma, over half of the patients were shunters with metabolite derangements and associated signs of toxicity. Current guidelines in the Children's Oncology Group (COG) leukemia protocols recommend checking metabolites in the context of increased sensitivity to 6-MP, as in the case of TPMT and NUDT15 deficient patients, but do not specifically address the more common problem of skewed metabolism. Previous gastroenterology publications suggest there may be as many as 15% of shunters in their population of patients being prescribed 6MP. We found a much higher incidence of shunters among our leukemia population, many of whom had concurrent toxicities. The patients who received allopurinol during their treatment period showed adequate count suppression with reversal of undesired toxicities, suggesting that combination therapy may be beneficial for many more patients in the future. All of these patients remain in remission through 1 Jun 2019, the longest being 6 years post-treatment. Based on our institutional experience, we propose an algorithm for the use of allopurinol in conjunction with chemotherapy for patients with ALL or LL who have inappropriate 6-MP metabolism (Figure 1) to optimize their treatment while decreasing 6-MP associated toxicities. Disclosures No relevant conflicts of interest to declare. OffLabel Disclosure: Allopurinol inhibits the enzyme, xanthine oxidase, and is commonly used for prevention of tumor lysis syndrome and gout. However, it is understood that allopurinol also affects the metabolism of 6-Mercaptopurine. Allopurinol directs 6MP metabolism towards 6-TGN, the desired product, and away from 6-MMPN. 6-MMPN is associated with increased toxicity in patients. We describe in our abstract the incorporation of allopurinol therapy in many of our patients with abnormal 6-MP metabolism

Dolutegravir-based anti-retroviral therapy is effective and safe in HIV\u2013infected paediatric patients

BackgroundTreatment of HIV infection in adolescents is challenging due to long duration of therapy and poor adherence. Recently, the integrase strand transfer inhibitor dolutegravir (DTG) has been approved for the use in adolescents with HIV, but evidence in clinical practice is very limited.MethodsWe describe six cases of HIV-infected children/adolescents successfully treated with DTG-based regimen. Data relative to children/adolescents managed at the Referral Center for Pediatric HIV/AIDS of the University of Naples were reviewed. Patients were tested before introduction of DTG, after 1 month and every 3 months in the first 2 years to assess virologic and immunological response, tolerance and development of side effects. Families were asked to report any suspected adverse events.ResultsSix patients (2 male, median age 17 years, range 12–18) were started on DTG-based anti-retroviral regimen due to low adherence to anti-retroviral treatment (ART), multiple drug resistance mutations, or development of ART-related side effects. Within 4–8 weeks after DTG treatment onset, a complete viral suppression and a concomitant increase of CD4+ cell count was observed. Four patients showed a persistent suppression after 2 years of follow-up, and 2 patients at about 1 year. One month after the introduction of DTG, the patient enrolled because of severe dyslipidaemia and hyper-transaminasemia showed a complete normalization of laboratory values. During follow-up (median 24 months, range 9–24) no adverse events were reported and most patients demonstrated a good adherence to treatment.ConclusionsDTG-based treatments demonstrated efficacy and good safety profile in adolescents. All patients demonstrated a rapid virologic and immunological response within 4–8 weeks, with good adherence and absence of side effects.

Mo1209 Assessing Performance and Perceived Fatigue in Adolescents With Crohn's Disease: Is There an Association With Disease Activity?

normal albumin levels (75%), platelet counts (53%), and CRP levels (64%) at wk 52 (Table). HD ADA also improved mean hemoglobin levels at wk 52 (mean change from BL LD -0.96 v. HD 3.43 g/L, p<0.001). CONCLUSION. ADA led to normalization of laboratory values in clinically meaningful proportions of pts who had abnormal albumin levels, platelet counts, and CRP levels at BL in IMAgINE 1. Improvements in hemoglobin levels were also observed at wk 52. Reference: 1. Hyams et al Gastroenterol 2012;143:365 Normalization of laboratory values in pts with abnormal values at BL (LOCF)

Pneumonia in childbirth as a mask of postpartum cardiomyopathy in the 28 -year-old patient in the early postpartum period

The article described a rare clinical case of perinatal cardiomyopathy as a form of dilated cardiomyopathy of unknown etiology. Perinatal cardiomyopathy was diagnosed on the eighth day after delivery in a patient with fever and diagnosis of generalized infection (pneumonia) that masked the symptoms of heart disease. Signs of cardiomyopathy were confirmed by the ehocardiographic study on the ninth day after the delivery. The patient was hospitalized, perinatal cardiomyopathy and pneumonia were treated, resulting in the patient’s condition improved, and there was complete resolution of pneumonia and normalization of laboratory values.

Self-Reported Activities and Outcomes of Ambulatory Care Staff Registered Nurses: An Exploration

Ambulatory care is a growing field of nursing practice. As ambulatory registered nurse (RN) practice grows, there has been an ongoing effort to identify the desired role of the staff RN in outpatient care and to provide linkages to preferred outcomes. This study sought to describe the perceived impact of components of the staff RN role on specific activities and outcomes, as guided by the structures, processes, and outcomes of the Nursing Role Effectiveness Model. This exploratory research study used a descriptive, self-report survey design. Survey respondents were ambulatory care staff RNs from various primary and specialty care clinics (n = 187) in an integrated health care organization in Southern California. The most frequently reported activities included patient assessment, nurse advice during message management, and completion of patient triage. Reported patient outcomes most frequently affected by RN activities were patient satisfaction, normalization of laboratory values, receiving the correct level of medical treatment, and prevention of complications. Respondents expressed that "emergency situations" periodically occur in the ambulatory setting. This research study supports what ambulatory care RNs say they are doing: daily, diverse, and complex patient care activities that influence multiple relevant patient outcomes. Future research studies could reveal best practices related to message management, in addition to activities and outcomes unique to specialty care populations.

Hypereosinophilic Syndrome Presenting As Acute Pancreatitis

RationaleHypereosinophilic syndrome (HES) is characterized by heterogeneous clinical presentations depending on the specific organ involvement. Few cases with pancreatic involvement have been reported in the adult literature. We present a case of pediatric HES presenting with acute pancreatitis.MethodsA 17-year-old male presented with acute abdominal pain and elevated lipase (3000 u/L), amylase (237 u/L), and ALP (1371 u/L). His absolute eosinophilic count (AEC) was 1400/mm3. He was diagnosed with autoimmune pancreatitis and was started on 30 mg of prednisone daily with excellent clinical response and normalization of laboratory values. After tapering to 5 mg every other day, the patient experienced a relapse of epigastric pain. Laboratory tests revealed AEC of 30,000/mm3, normal lipase and amylase, elevated sedimentation rate (47 mm/hr), ALP (707 u/L), AST (93 u/L) and ALT (66 u/L).ResultsBone marrow biopsy showed marked eosinophilia without evidence of B and T cell lymphoproliferative disease. Testing for parasitic infections was negative; mutation analysis for JAK2V617F mutation was negative and is pending for FIP1LI PDGFRA mutation. ECG showed sinus bradycardia (42/min). 2D cardiac Echo and troponin levels were normal as was a serum tryptase level. He was treated with high dose prednisolone at 60mg daily with rapid resolution of clinical symptoms as well as peripheral eosinophilia.ConclusionsA pubmed search revealed only 3 cases of HES with pancreatic involvement reported in adult patients. To the best of our knowledge, this is the first reported case of HES presenting with pancreatitis in the pediatric age group. RationaleHypereosinophilic syndrome (HES) is characterized by heterogeneous clinical presentations depending on the specific organ involvement. Few cases with pancreatic involvement have been reported in the adult literature. We present a case of pediatric HES presenting with acute pancreatitis. Hypereosinophilic syndrome (HES) is characterized by heterogeneous clinical presentations depending on the specific organ involvement. Few cases with pancreatic involvement have been reported in the adult literature. We present a case of pediatric HES presenting with acute pancreatitis. MethodsA 17-year-old male presented with acute abdominal pain and elevated lipase (3000 u/L), amylase (237 u/L), and ALP (1371 u/L). His absolute eosinophilic count (AEC) was 1400/mm3. He was diagnosed with autoimmune pancreatitis and was started on 30 mg of prednisone daily with excellent clinical response and normalization of laboratory values. After tapering to 5 mg every other day, the patient experienced a relapse of epigastric pain. Laboratory tests revealed AEC of 30,000/mm3, normal lipase and amylase, elevated sedimentation rate (47 mm/hr), ALP (707 u/L), AST (93 u/L) and ALT (66 u/L). A 17-year-old male presented with acute abdominal pain and elevated lipase (3000 u/L), amylase (237 u/L), and ALP (1371 u/L). His absolute eosinophilic count (AEC) was 1400/mm3. He was diagnosed with autoimmune pancreatitis and was started on 30 mg of prednisone daily with excellent clinical response and normalization of laboratory values. After tapering to 5 mg every other day, the patient experienced a relapse of epigastric pain. Laboratory tests revealed AEC of 30,000/mm3, normal lipase and amylase, elevated sedimentation rate (47 mm/hr), ALP (707 u/L), AST (93 u/L) and ALT (66 u/L). ResultsBone marrow biopsy showed marked eosinophilia without evidence of B and T cell lymphoproliferative disease. Testing for parasitic infections was negative; mutation analysis for JAK2V617F mutation was negative and is pending for FIP1LI PDGFRA mutation. ECG showed sinus bradycardia (42/min). 2D cardiac Echo and troponin levels were normal as was a serum tryptase level. He was treated with high dose prednisolone at 60mg daily with rapid resolution of clinical symptoms as well as peripheral eosinophilia. Bone marrow biopsy showed marked eosinophilia without evidence of B and T cell lymphoproliferative disease. Testing for parasitic infections was negative; mutation analysis for JAK2V617F mutation was negative and is pending for FIP1LI PDGFRA mutation. ECG showed sinus bradycardia (42/min). 2D cardiac Echo and troponin levels were normal as was a serum tryptase level. He was treated with high dose prednisolone at 60mg daily with rapid resolution of clinical symptoms as well as peripheral eosinophilia. ConclusionsA pubmed search revealed only 3 cases of HES with pancreatic involvement reported in adult patients. To the best of our knowledge, this is the first reported case of HES presenting with pancreatitis in the pediatric age group. A pubmed search revealed only 3 cases of HES with pancreatic involvement reported in adult patients. To the best of our knowledge, this is the first reported case of HES presenting with pancreatitis in the pediatric age group.

Early Laparoscopic Cholecystectomy for Mild Gallstone Pancreatitis

HYPOTHESIS Patients with mild gallstone pancreatitis may undergo an early laparoscopic cholecystectomy (LC) within 48 hours of hospital admission without awaiting the normalization of pancreatic and liver enzyme levels. This may decrease the hospital stay without increasing morbidity or mortality and may minimize the unnecessary use of endoscopic retrograde cholangiopancreatography. DESIGN A retrospective review. SETTING Two university-affiliated urban medical centers. PATIENTS A total of 303 patients with mild gallstone pancreatitis, of whom 117 underwent an early LC and 186 underwent a delayed LC. MAIN OUTCOME MEASURES Hospital length of stay, morbidity and mortality rates, and the use of endoscopic retrograde cholangiopancreatography. RESULTS Similar hospital admission variables were observed in the early and delayed LC groups, although the delayed group was older (P=.006). The median hospital length of stay was significantly less for the early group than for the delayed group (3 vs 6 days; P&lt;.001). There were no patients who died, and the complication rates were similar for both groups. However, the patients who underwent an early LC were less likely than patients who underwent a delayed LC to undergo endoscopic retrograde cholangiopancreatography (P=.02). CONCLUSIONS An early LC may be safely performed for patients with mild gallstone pancreatitis, without concern for increased morbidity and mortality, resulting in shortened hospital stays and a decrease in the use of endoscopic retrograde cholangiopancreatography. The practice of delaying an LC until normalization of laboratory values appears to be unnecessary.

Eosinophilic Fasciitis Associated with Mycoplasma arginini Infection

Eosinophilic fasciitis (EF) with generalized sclerodermiform skin lesions developed over a 19-month period in a previously healthy 23-year-old man. Although we confirmed EF by skin histology and laboratory tests, the recurrent fevers and the clinical observation of sclerotic prepuce with urethritis indicated further bacteriological analysis by conventional microbiological and DNA-based tests. Urethra cultures were positive for an arginine-hydrolyzing mycoplasma and Ureaplasma urealyticum. The patient also had serum IgM antibodies to Mycoplasma pneumoniae using enzyme-linked immunosorbent assay (ELISA)-based qualitative detection. Mycoplasma arginini was isolated from two independent venous blood serum samples and was identified by conventional microbiological tests and sequencing of the 16S rRNA and rpoB genes (GenBank sequence accession numbers HM179555 and HM179556, respectively). M. arginini genomic DNA also was detected by species-specific PCR in the skin lesion biopsy sample. Treatment with corticosteroids and long-term courses of selected antibiotics led to remission of skin symptoms and normalization of laboratory values. This report provides the first evidence of EF associated with mycoplasma infection and the second report of human infection with M. arginini and therefore suggests that this mycoplasma infection might have contributed to the pathogenesis of the disease.

Thrombotic Microangiopathy with Tacrolimus/Sirolimus-Based GVHD Prophylaxis Regimen in Patients Undergoing Hematopoietic Stem Cell Transplant from a Matched Unrelated Donor

Thrombotic microangiopathy (TMA) is a multifactorial complication of related and unrelated allogeneic hematopoietic stem cell transplant (allo-HSCT). The true incidence of TMA is difficult to estimate due to lack of agreement on a single definition. Diagnosis is often complicated by multiple potential etiologies for the clinical findings. Sirolimus (SIR), an inhibitor of mammalian target of Rapamycin (mTOR), is a novel immunosuppressive agent that works synergistically with calcineurin inhibitors (CNI) to prevent graft-versus-host disease (GVHD) in allo-HSCT. Recently, the addition of SIR to CNIs was reported to result in a higher than expected incidence (10.8%) of TMA (Cutler et al. BBMT 2005; 11:551–7). We evaluated the incidence and risk factors for TMA in a cohort of patients undergoing matched unrelated (MUD) HSCT using SIR combined with tacrolimus (TAC) and mini-methotrexate for GVHD prophylaxis at City of Hope. TMA was defined as SCr increase of ≥ 50% above baseline, LDH twice the institutional upper normal limit, presence of schistocytes or persistent presence of nucleated red blood cells, and prolonged or progressive thrombocytopenia (platelets <50 × 109/L or ≥ 50% decrease from previous count). A case series of 47 MUD-HSCT patients were included in this retrospective chart review study. The median age was 50 years (range: 19–67); (male/female: 28/19). Conditioning regimens consisted of fludarabine/melphalan (65%) and FTBI combined with cyclophosphamide or etoposide (35%). Diagnoses included ALL (32%), AML (25%), NHL (15%), MDS (15%), MPD (9%), CML (2%), CLL (2%). Twenty-six patients (55%) had a 10/10 matched (HLA-A, B, C, DRB1, and DQB1) donor by high-resolution on typing. The median follow up for the 30 surviving patients is 14.5 months (2.8–26). The one-year probabilities of overall survival and non-relapse mortality (NRM) were 61% and 19%, respectively. Grade II-IV acute GVHD (aGVHD) was reported in 60% of all patients (grade III-IV: 25%). Thirteen (28%) patients met the above diagnostic criteria for TMA. In addition, we included two patients who did not meet the criteria due to missing tests but were clinically diagnosed with TMA by independent attending physicians, resulting in the total incidence of 32% (15/47). Four of the 15 patients met the criteria for TMA as a result of ongoing multi-organ failure secondary to other causes. The median time to TMA onset was five weeks (2–20 weeks). Most cases (93%) occurred within the first 100 days post-HSCT. Thirteen patients developed both TMA and aGVHD, in which the majority of patients (70%) developed TMA after a diagnosis of aGVHD had been made. Initial treatments for TMA included holding TAC (33%), holding SIR (20%), holding or adjusting doses (27% and 20%, respectively) for both drugs. One patient underwent plasma exchange. Sixty percent of patients subsequently recovered from TMA as defined by normalization of laboratory values. Of the 17 expired patients, ten were diagnosed with TMA. Causes of death were as follows: for TMA cases, relapse mortality=3, NRM=7; for Non-TMA patients, relapse mortality=6, NRM=1. At the time of TMA diagnosis, the median TAC and SIR levels were 11.3 (0–18.8) and 7 (0–23.9) ng/ml, respectively, in contrast to the median TAC and SIR levels for non-TMA patients at 6.1 (p= 0.02) and 5.5 (p=0.13) ng/ml, respectively. To identify other possible risk factors for TMA, the following patient and treatment-related characteristics were examined: age, conditioning regimen, disease type, degree of HLA match, and exposure to triazole antifungals. Only higher tacrolimus levels (HR: 6.9, p<0.01) and aGVHD grades III-IV (HR: 3.5, p=0.02) were associated with an increased risk for TMA. In conclusion, TMA is common after MUD allo-HSCT using SIR-containing GVHD prophylaxis. The risk factors for TMA suggest that careful monitoring and adjustment of TAC/SIR dosages to avoid super-therapeutic levels is critical, particularly during ongoing GVHD.

Gastric banding for the treatment of type 2 diabetes mellitus in morbidly obese

To assess the efficacy of the Swedish adjustable gastric band in the treatment of type 2 diabetes mellitus (T2DM), impaired glucose tolerance (IGT), and the metabolic syndrome (MS) in the morbidly obese. We identified all patients with T2DM, IGT, or the MS at surgery from our database of 905 consecutive patients who had undergone placement of the Swedish adjustable gastric band between January 2001 and April 2007. The patients were followed up by our multidisciplinary team, and their T2DM was managed by their treating primary care physician and/or endocrinologist. A total of 682 patients had >6 months of follow-up. Of these, 78 patients had T2DM, 64 had IGT, and 100 had the MS. At a median follow-up of 12.5 months, patients with T2DM had a mean +/- SD excess weight loss of 38% +/- 15%. This was associated with hemoglobin A1c and fasting blood sugar levels decreasing from 8.0% +/- 1.7% to 6.1% +/- 1.0% (P <.0001) and from 9.6 +/- 3.4 mmol/L to 5.7 +/- 1.5 mmol/L (P <.0001), respectively. Remission and/or improvement in patients with T2DM was judged by the complete cessation and/or reduction in medication and normalization of laboratory values. This occurred in 81% of those taking oral hypoglycemic agents. Of the patients taking a combination of oral hypoglycemic agents and insulin, 43% ceased and/or reduced their oral hypoglycemic agents, and 93% ceased and/or reduced their insulin requirements. Of those on insulin only, 75% ceased and/or reduced their insulin. No patient with IGT developed diabetes or progressed to require medications. Remission and/or improvement in the MS occurred in 88% of patients. Remission of T2DM was dependent on both the magnitude of excess weight loss (P = .008) and the duration of the pre-existing T2DM. Using binary logistic regression analysis, a duration of T2DM of <5 years before surgery was 6.5 times more likely to lead to resolution of T2DM after the weight loss (P = .004). Weight loss after Swedish adjustable gastric band placement is an effective treatment of T2DM in morbidly obese patients, with early intervention offering the greatest chance of remission. It might even prevent the occurrence of T2DM in patients with IGT.

Biliary atresia in infants with prolonged cholestatic jaundice: diagnostic accuracy of hepatobiliary scintigraphy

Cholestatic jaundice during infancy is one of the most problematic challenges for pediatricians. Biliary atresia (BA) and neonatal hepatitis syndrome (NHS) are major causes of cholestatic jaundices. Our aim was to compare the diagnostic accuracy of hepatobiliary scintigraphy with liver biopsy and ultrasonography in excluding BA. Seventy consecutive patients, all suffering from prolonged cholestatic jaundice (>1 month), were included. Laparotomy with surgical cholangiography was considered as the gold standard; however, in nine patients, based on the patient's recovery from jaundice and the normalization of laboratory values during the clinical follow-up period (=6-12 months), the diagnosis of NHS was verified and performing laparotomy was unnecessary. All patients underwent hepatobiliary scintigraphy, liver biopsy and ultrasonography and their results were compared. Based on the gold standards mentioned above, 46 patients (46/70 = 65.7%) had BA. The sensitivity, specificity, PPV, NPV, and accuracy of the hepatobiliary scintigraphy in diagnosis of BA were 90%, 80%, 91.8%, 76.2%, and 84.5%, respectively. The respective values for liver biopsy were 92.5%, 88.9%, 94.9%, 84.2%, and 90.1% and for ultrasonographic analysis were 41.7%, 90.9%, 90.9%, 41.7%, and 66.3%. Hepatobiliary scintigraphy is an important imaging technique in the diagnostic evaluation of infants with prolonged cholestatic jaundice. It is a convenient and reliable method of differentiating BA from NHS, with a diagnostic accuracy superior to that of US but slightly inferior to that of liver biopsy. Ultrasonography is the least sensitive and specific available modality and its findings should be confirmed by scintigraphy or liver biopsy.