Granulocyte transfusion therapy in chronic granulomatous disease. Report of a patient and review of the literature

A l6-year-old boy with chronic granulomatous disease (CGD) and multiple Staphylococcus aureus hepatic abscesses failed to respond to intense antimicrobial therapy and surgical drainage procedures. He was started on a l3-day course of daily granulocyte transfusions obtained by intermittent-flow leukapheresis (haemonetics Model 30 [Braintree, Massachusetts]) utilizing hydroxyethyl starch. Each transfusion contained an average of 9 × l0(9) granulocytes. He defervesced on the seventh transfusion day, and the sedimentation rate returned to normal on the thirteenth transfusion day. Radionuclide scan showed decrease in size of the liver abscesses. No other new therapeutic modalities were attempted during the period of granulocyte transfusion. Although the use of granulocyte transfusion has been suggested to treat infections in CGD, we report the first successful use of granulocyte transfusion therapy in S. aureus abscess disease in CGD. Granulocyte transfusions may be helpful in treating infections refractory to standard medical- surgical management in patients with CGD and other disorders of neutrophil function.     

Management of Adult Onset Seizures

Epilepsy is a common yet heterogeneous disease. As a result, management often requires complex decision making. The ultimate goal of seizure management is for the patient to have no seizures and no considerable adverse effects from the treatment. Antiepileptic drugs are the mainstay of therapy, with more than 20 medications currently approved in the United States. Antiepileptic drug selection requires an understanding of the patient's epilepsy, along with consideration of comorbidities and potential for adverse events. After a patient has failed at least 2 appropriate antiepileptic drugs, they are determined to be medically refractory. At this time, additional therapy, including dietary, device, or surgical treatments, need to be considered, typically at a certified epilepsy center. All these treatments require consideration of the potential for seizure freedom, balanced against potential adverse effects, and can have a positive effect on seizure control and quality of life. This review article discussed the treatment options available for adults with epilepsy, including medical, surgical, dietary, and device therapies.     

A case report of pyogenic liver abscess caused by hypervirulent Klebsiella pneumoniae diagnosed by metagenomic next-generation sequencing

A 56-year-old woman with a history of diabetes mellitus presented with high fever but no abdominal pain. An abdominal computed tomography scan showed a large liver abscess. Hypervirulent, string test-positive, rmpA/ampA2-, and iutA-positive Klebsiella pneumoniae was rapidly identified from drainage fluid of the liver abscess using metagenomic next-generation sequencing (mNGS). After intravenous antibiotic therapy and drainage of the abscess, the patient’s condition resolved. This case report highlights the value of mNGS in rapidly and accurately identifying a pathogenic microorganism, which helps reduce the incidence of antimicrobial resistance and enables the targeted use of antibiotics.     

A rare case of a prostatic abscess,bacteremia and chronic granulomatous disease associated with Klebsiella pneumoniae

Chronic granulomatous disease (CGD) is a primary immunodeficiency disease characterized by severe recurrent infections such as pneumonia, liver and skin infections. However, prostatic abscesses are rare as only two cases have been reported thus far. We present the case of a 41-year-old patient with CGD who was admitted to the hospital with fever and subsequently, Klebsiella pneumoniae was identified on blood culture. Abdominal computed tomography revealed a prostatic abscess. He improved with intravenous antibiotics and drainage of the abscess. After he was taken off the intravenous antibiotics and started on an oral agent, he was discharged from the hospital. We confirmed a reduction in the prostatic abscess size and continued the antibiotic therapy for 52 days. A prostatic abscess is an uncommon disease being diagnosed at a median age of 49 years. Sometimes it is discovered in patients with fever of unknown origin and might be considered as an infection site of CGD patients.     

A Review of Chronic Granulomatous Disease

Chronic granulomatous disease (CGD) is a primary immunodeficiency caused by defects in any of the five subunits of the NADPH oxidase complex responsible for the respiratory burst in phagocytic leukocytes. Patients with CGD are at increased risk of life-threatening infections with catalase-positive bacteria and fungi and inflammatory complications such as CGD colitis. The implementation of routine antimicrobial prophylaxis and the advent of azole antifungals has considerably improved overall survival. Nevertheless, life expectancy remains decreased compared to the general population. Inflammatory complications are a significant contributor to morbidity in CGD, and they are often refractory to standard therapies. At present, hematopoietic stem cell transplantation (HCT) is the only curative treatment, and transplantation outcomes have improved over the last few decades with overall survival rates now > 90% in children less than 14 years of age. However, there remains debate as to the optimal conditioning regimen, and there is question as to how to manage adolescent and adult patients. The current evidence suggests that myeloablative conditioning results is more durable myeloid engraftment but with increased toxicity and high rates of graft-versus-host disease. In recent years, gene therapy has been proposed as an alternative to HCT for patients without an HLA-matched donor. However, results to date have not been encouraging. with negligible long-term engraftment of gene-corrected hematopoietic stem cells and reports of myelodysplastic syndrome due to insertional mutagenesis. Multicenter trials are currently underway in the United States and Europe using a SIN-lentiviral vector under the control of a myeloid-specific promoter, and, should the trials be successful, gene therapy may be a viable option for patients with CGD in the future.     

An Unlikely Cause of Hypokalemia

Background

Hypokalemia is a common clinical disorder caused by a variety of different mechanisms. Although the most common causes are diuretic use and gastrointestinal losses, elevated cortisol levels can also cause hypokalemia through its effects on the renin–angiotensin–aldosterone system. Cushing's syndrome refers to this general state of hypercortisolemia, which often manifests with symptoms of generalized weakness, high blood pressure, diabetes mellitus, menstrual disorders, and psychiatric changes. This syndrome is most commonly caused by exogenous steroid use, but other etiologies have also been reported in the literature. Ectopic adrenocorticotropic hormone production by small-cell lung cancer is one rare cause of Cushing's syndrome, and may be associated with significant hypokalemia.

Recurrent pyogenic liver abscess after pancreatoduodenectomy caused by common hepatic artery injury: A case report

BACKGROUNDPancreaticoduodenectomy (PD) has been increasingly performed as a safe treatment option for periampullary malignant and benign disorders. However, the operation may result in significant postoperative complications. Here, we present a case that recurrent pyogenic liver abscess after PD is caused by common hepatic artery injury in atypical celiac axis anatomy.CASE SUMMARYA 56-year-old man with a 1-d history of fever and shivering was diagnosed with hepatic abscess. One year and five months ago, he underwent PD at a local hospital to treat chronic pancreatitis. After the operation, the patient had recurrent intrahepatic abscesses for 4 times, and the symptoms were relieved after percutaneous transhepatic cholangial drainage combining with anti-inflammatory therapy in the local hospital. Further examination showed that the recurrent liver abscess after PD was caused by common hepatic artery injury due to abnormal abdominal vascular anatomy. The patient underwent percutaneous drainage but continued to have recurrent episodes. His condition was eventually cured by right hepatectomy. In this case, preoperative examination of the patient’s anatomical variations with computed tomography would have played a pivotal role in avoiding arterial injuries.CONCLUSIONA careful computed tomography analysis should be considered mandatory not only to define the operability (with radical intent) of PD candidates but also to identify atypical arterial patterns and plan the optimal surgical strategy.     

Filifactor alocis brain abscess identified by 16S ribosomal RNA gene sequencing: A case report

We report a clinical case of Filifactor alocis brain abscess in an 85-year-old man who had decayed teeth 1 week prior. In this case, the abscess was surgically drained after empirical antibiotics had been initiated. Although the causative organism could not be identified by culture, F. alocis was detected via 16S ribosomal RNA (16S rRNA) gene sequencing of the pus isolated from the abscess. The patient recovered without serious sequelae after surgical drainage and prolonged antibiotic treatment, including metronidazole, ceftriaxone and meropenem for 8 weeks. The findings in this case emphasize that 16S rRNA gene sequencing allows bacterial diagnosis of brain abscess when phenotypic identification fails, such as in cases where patients are undergoing antimicrobial treatment at the time of sampling or where patients are infected with fastidious organisms.     

A cool side effect of valproic acid administration: Single dose-induced hypothermia

Thermoregulatory derangements secondary to valproic acid (VPA) administration, specifically hypothermia, have been reported throughout the literature, but a handful of times. This case report describes a 28-year-old male presenting status-post multiple tonic-clonic seizures, treated for persistent seizure activity refractory to benzodiazepines with valproic acid (VPA), levetiracetam, and fosphenytoin. After just over an hour, the patient's core temperature fell from 36.8?°C to 34.9?°C. Temperatures were repeated for confirmation, no further doses of VPA were administered, and the patient's temperature returned to normal over the next 7?h with the use of warming blankets. Levetiracetam and fosphenytoin were continued with no further reported development of hypothermia during the patient's admission. After reviewing other potential causes, a thorough drug database review was performed that found VPA to be the only medication administered with published reports of inducing hypothermia. The mechanism of thermoregulatory derangement associated with VPA is not clearly defined and much of the evidence surrounds alterations in gamma-aminobutyric acid (GABA) activity in animal studies. To our knowledge, this case report is the first reported case of VPA-induced hypothermia following a single dose in the emergency department and offers the potential that prompt return to normothermia is likely following discontinuation of the offending agent.     

Spontaneously shrinking lung mass due to Mycobacterium avium mimicking lung cancer

NTM‐SPN is often indistinguishable from malignancy. Although surgical resection is sometimes chosen for the diagnosis and treatment, the mass in this case shrank spontaneously. Careful observation is required to avoid unnecessary interventions.     

Lung abscess following bronchoscopy due to multidrug-resistant Capnocytophaga sputigena adjacent to lung cancer with high PD-L1 expression

Lung abscess following flexible bronchoscopy is a rare and sometimes fatal iatrogenic complication. Here, we report the first case of a lung abscess caused by multidrug-resistant Capnocytophaga sputigena following bronchoscopy. A 67-year-old man underwent bronchoscopy to evaluate a lung mass. Seven days after transbronchial lung biopsy, he presented with an abscess formation in a lung mass. Empirical antibiotic therapy, including with garenoxacin, ampicillin/sulbactam, clindamycin and cefepime, was ineffective. Percutaneous needle aspiration of lung abscess yielded C. sputigena resistant to multiple antibiotics but remained susceptible to carbapenem. He was successfully treated by the combination therapy with surgery and with approximately 6 weeks of intravenous carbapenem. Finally he was diagnosed with a lung abscess with adenocarcinoma expressing high levels of programmed cell death ligand 1. The emergence of multidrug-resistant Capnocytophaga species is a serious concern for effective antimicrobial therapy. Clinicians should consider multidrug-resistant C. sputigena as a causative pathogen of lung abscess when it is refractory to antimicrobial treatment.     

Fournier’s gangrene progressing from the buttocks to the scrotum following a perianal abscess

We describe a case of gas-producing infection following a perianal abscess. A 61-year-old man was admitted to our hospital complaining of perineal pain and was found to have a perianal abscess. He was diabetic but had not received treatment for the disease. Although the perianal abscess was drained and antibiotic treatment started, severe swelling of the scrotum, with crepitation, redness, and partial necrosis progressed rapidly. Computed tomography revealed subcutaneous gas formation in the scrotum. A culture study revealed Clostridium, Enterococcus, and numerous other types of bacteria. The patient was diagnosed with Fournier’s gangrene caused by infection with Clostridium in combination with other species of bacteria. The infection was refractory to drainage and antibiotic therapy. Thus, repeated extensive debridement of all necrotic tissue in the scrotum was required until healthy granulation was present in the wound. Our case shows that, in patients with Fournier’s gangrene caused by infection with Clostridium in combination with other species of bacteria, the mainstay of treatment should be open drainage and aggressive surgical debridement of all necrotic tissue, followed by broadspectrum antibiotic therapy.     

Spontaneous internal hemorrhage of a giant hepatic hemangioma with infection: a case report and literature review

Spontaneous internal hemorrhage from a hepatic hemangioma is rare. This case describes a 59-year-old woman who was hospitalized with recurrent cough and fever for 6 months. The doctor thought that she had pneumonia, but other infectious diseases could not be ruled out. Therefore, related tests were performed and strong antibiotics were used, but the symptom of fever was persistent and recurred. Enhanced computed tomography (CT) findings showed a right hepatic giant hemangioma with hemorrhage, while tuberculosis, liver abscess, and immune disease were excluded by the physician. Because the patient’s fever was associated with a large hepatic hemangioma, the patient was transferred to surgery. During treatment, the amount of bleeding increased, so she underwent a right hepatic hemangioma resection in the emergency department. Her postoperative fever symptoms subsequently resolved. Pathological examination confirmed hemorrhagic necrosis with infection in hepatic hemangioma. Follow-up showed that the patient was afebrile.     

Pott's Disease in a Patient with Subtle Red Flags

Background

Tuberculosis (TB) is now rare in developed countries; however, it is an important diagnosis for the Emergency Physician to be able to make. Classically thought of as a respiratory disease, TB can present in other ways, making it more challenging to recognize.

Surgery and diagnostic imaging in abdominal Crohn’s disease

Surgery is well-established option for the treatment of Crohn’s disease that is refractory to medical therapy and for complications of the disease, including strictures, fistulas, abscesses, bleeding that cannot be controlled endoscopically, and neoplastic degeneration. For a condition like Crohn’s disease, where medical management is the rule, other indications for surgery are considered controversial, because the therapeutic effects of surgery are limited to the resolution of complications and the rate of recurrence is high, especially at sites of the surgical anastomosis. In the authors’ opinion, however, surgery should not be considered a last-resort treatment: in a variety of situations, it should be regarded as an appropriate solution for managing this disease. Based on a review of the literature and their own experience, the authors examine some of the possibilities for surgical interventions in Crohn’s disease and the roles played in these cases by diagnostic imaging modalities.     

肝癌化疗栓塞并发肝脓肿的临床特点及易感因素分析

目的 探讨肝癌患者化疗栓塞(TACE)后并发肝脓肿的临床特点及易感因素.方法 回顾性分析523 例肝癌患者共1167 次TACE 术后并发肝脓肿的发病率、临床表现、治疗转归等临床特点,并分析肝脓肿形成与糖尿病、Child-Pugh B级肝硬化、门静脉阻塞、胆道梗阻及追加颗粒性栓塞剂栓塞等易感因素的关系.结果 8 例患者TACE 术后共发生9 次肝脓肿.肝脓肿并发症的发病率为0.77%(共1167 次操作).肝脓肿临床表现有肝大、肝区疼痛、发热、白细胞增多,发病时间为术后9 ～29 d,平均(18 ±9)d.除1 例患者由于诊断治疗不及时死亡外,余7 例肝脓肿患者经及时诊断和积极经皮肝穿置管引流及抗生素治疗后均痊愈.χ2 检验结果显示,具有胆道阻塞易感因素的患者术后肝脓肿的发生率明显高于控制组(χ2 ＝143.848,P ＜0.001),而糖尿病、门静脉阻塞及追加颗粒性栓塞剂栓塞等易感因素的组间差异无统计学意义(P ＞0.05).结论 肝癌TACE 后肝脓肿并发症具有一定的临床特点,术前胆道梗阻是TACE 并发肝脓肿的易感因素.     

Liver atrophy after transcatheter arterial embolization and percutaneous ethanol injection therapy for a minute hepatocellular carcinoma

A 63-year-old male patient with compensated cirrhosis underwent transcatheter arterial embolization (TAE) and percutaneous ethanol injection therapy (PEIT) for a minute hepatocellular carcinoma (HCC). Although the HCC was successfully treated, esophageal varices worsened and refractory ascites developed 3 months after the TAE and PEIT. Liver atrophy progressed rapidly compared to the natural course of liver cirrhosis.     

Brain abscess: recent experience at a community hospital

Brain abscess is a formidable diagnostic and therapeutic problem with mortality ranging from 35% to 65%. It may occur at any age, and there is a male:female ratio of 2:1. Brain abscess arises from a contiguous focus of infection, direct implantation due to trauma, or hematogenous spread from a remote site. The commonest organisms isolated from brain abscess include streptococci, Staphylococcus aureus, Bacteroides species, and Enterobacteriaceae. Brain abscess frequently produces headache, vomiting, focal neurologic signs, and depressed level of consciousness. Fever and leukocytosis often are absent. Diagnosis is suggested by computerized tomography, but most cases require surgical confirmation. Optimal management consists of intensive antibiotic therapy. Aggressive surgical treatment is required in cases not responding to antimicrobial therapy. Long-term neurologic deficit occurs in up to 60% of cases.     

Chemotherapy for alpha-fetoprotein producing gastric cancers expressing human epidermal growth factor receptor 2

Although, gastric cancer is one of the most common cancers worldwide, alpha-fetoprotein (AFP) producing human epidermal growth factor receptor 2 (HER2) positive gastric cancers are rare. AFP producing gastric cancer has a poor prognosis and an appropriate treatment option has not been established to date. A 75-year-old woman with AFP- producing gastric cancer was treated with S-1, an oral fluoropyrimidine derivative, chemotherapy after distal gastrectomy. Recurrence of gastric cancer was observed after 18 months and immunohistochemistry analysis showed AFP and HER2 positive gastric cancer. The patient received combination therapy containing capecitabine, cisplatin, and trastuzumab. Computed tomography scans showed regression of the lymph node metastasis. The patient's quality of life substantially improved after the treatment. Thus, the present case suggests that AFP and HER2 positive gastric cancer can be effectively treated with, capecitabine, cisplatin, and trastuzumab combination therapy.     

Life-threatening massive empyema: A novel complication of intrathoracic omental herniation

Intrathoracic omental herniation (ITOH) is the herniation of the omentum through the esophageal hiatus without herniation of the stomach. It is a rare disease and serious complications due to ITOH have not been reported in the literature. Here, we reported the case of 47-year old man who presented to the emergency department with dyspnea and chest pain. Enhanced computed tomography (CT) demonstrated a large retrocardiac mass and ITOH was suspected. During the observation period in the emergency department, the patient's condition rapidly deteriorated. Follow-up CT showed large parapneumonic effusion and empyema. Emergency surgery was performed and the omental sac was removed. The patient's vital signs were restored and his symptoms were relieved. He was discharged on hospital day 15 without complications. Emergency physicians should be aware that severe complications of ITOH could develop and that if the patient's symptoms and vital signs worsen, emergency surgery should be considered.