The intravesical phallus in patients with cloacal exstrophy: An embryologic conundrum.

Background: Phalluses present inside the extrophied bladder of cloacal exstrophy (CE) newborns have been sporadically reported in the literature; this clinical entity has largely unknown origins and may represent an extremely rare anomaly of CE.

Objective: Along with nearly doubling the number of reported intravesical phalluses in the literature, this study aims to outline the common anatomic features and discuss the implications for theories of CE embryogenesis.

Study Design: The authors retrospectively identified patients with CE and a potential intravesical phallus between 1997 and 2017 at two high-volume centers.

CDK7 inhibition suppresses super-enhancer-linked oncogenic transcription in MYCN-driven cancer.

The MYC oncoproteins are thought to stimulate tumor cell growth and proliferation through amplification of gene transcription, a mechanism that has thwarted most efforts to inhibit MYC function as potential cancer therapy. Using a covalent inhibitor of cyclin-dependent kinase 7 (CDK7) to disrupt the transcription of amplified MYCN in neuroblastoma cells, we demonstrate downregulation of the oncoprotein with consequent massive suppression of MYCN-driven global transcriptional amplification. This response translated to significant tumor regression in a mouse model of high-risk neuroblastoma, without the introduction of systemic toxicity.

A receptor tyrosine kinase network composed of fibroblast growth factor receptors, epidermal growth factor receptor, v-erb-b2 erythroblastic leukemia viral oncogene homolog 2, and hepatocyte growth factor receptor drives growth and survival of head and neck squamous carcinoma cell lines.

Our laboratory has previously shown that some gefitinib-insensitive head and neck squamous cell carcinoma (HNSCC) cell lines exhibit dominant autocrine fibroblast growth factor receptor (FGFR) signaling. Herein, we deployed a whole-genome loss-of-function screen to identify genes whose knockdown potentiated the inhibitory effect of the FGFR inhibitor, AZ8010, in HNSCC cell lines. Three HNSCC cell lines expressing a genome-wide small hairpin RNA (shRNA) library were treated with AZ8010 and the abundance of shRNA sequences was assessed by deep sequencing.

Human botulism immune globulin for the treatment of infant botulism.

Background: We created the orphan drug Human Botulism Immune Globulin Intravenous (Human) (BIG-IV), which neutralizes botulinum toxin, and evaluated its safety and efficacy in treating infant botulism, the intestinal-toxemia form of human botulism.

Methods: We performed a five-year, randomized, double-blind, placebo-controlled trial statewide, in California, of BIG-IV in 122 infants with suspected (and subsequently laboratory-confirmed) infant botulism (75 caused by type A Clostridium botulinum toxin, and 47 by type B toxin); treatment was given within three days after hospital admission. We subsequently performed a 6-year nationwide, open-label study of 382 laboratory-confirmed cases of infant botulism treated within 18 days after hospital admission.

The detection of a deletion in the type B neurotoxin gene of Clostridium botulinum A(B) strains by a two-step PCR.

Differences between the type B neurotoxin gene sequence of Clostridium botulinum type A(B) and Cl. botulinum type B, including a six nucleotide deletion, were recently proposed as a cause of the lack of expression of this gene in the type A toxigenic strains. A polymerase chain reaction (PCR) based on two sets of primers was designed to investigate the absence of the 6-nucleotide sequence in the apparently unexpressed type B toxin gene of 42 strains of Cl.

Botulism in the United States: a clinical and epidemiologic review.

Botulism is caused by a neurotoxin produced from the anaerobic, spore-forming bacterium Clostridium botulinum. Botulism in humans is usually caused by toxin types A, B, and E. Since 1973, a median of 24 cases of foodborne botulism, 3 cases of wound botulism, and 71 cases of infant botulism have been reported annually to the Centers for Disease Control and Prevention (CDC).

A large outbreak of botulism: the hazardous baked potato.

In April 1994, the largest outbreak of botulism in the United States since 1978 occurred in El Paso, Texas. Thirty persons were affected; 4 required mechanical ventilation. All ate food from a Greek restaurant.

Sensitive assay for measurement of antibodies to Clostridium botulinum neurotoxins A, B, and E: use of hapten-labeled-antibody elution to isolate specific complexes.

The measurement of chicken and human antibodies to Clostridium botulinum neurotoxins A, B, and E was accomplished by affinity isolation of complexes containing these antibodies. By this approach, a mixture of toxin with the test antibody, fluoresceinated antibody, and enzyme (Russell's viper venom factor X activator)-labeled antibody is allowed to form a complex in solution phase. This complex is then bound to a matrix containing antifluorescein antibody.

Endogenous antibody production to botulinum toxin in an adult with intestinal colonization botulism and underlying Crohn's disease.

A patient with obstruction of the terminal ileum from Crohn's disease developed complete paralysis in week 1 of hospitalization. Features initially suggested Guillain-Barre syndrome, but botulinum toxin was identified in serum and stool specimens from week 1 and type A toxin-producing Clostridium botulinum in stool specimens from weeks 3 to 19, confirming botulism due to intestinal colonization. In week 19, the inflamed small bowel was resected, and C.

An outbreak of type A botulism associated with a commercial cheese sauce.

Background: Although botulism is rare, recognition of a possible case of this illness represents a public health emergency. To prevent more cases, prompt investigation must be done to determine whether illness is linked to commercial product or restaurant. Botulism can masquerade as other illnesses, and seemingly unlikely foods can harbor botulinum toxin.

Genetic characterization of Clostridium botulinum type A containing silent type B neurotoxin gene sequences.

A recent study detected genes encoding type B botulinum neurotoxin in some type A strains of Clostridium botulinum that exhibit no type B toxin activity. In this study, we investigated the presence, structure, linkage, and organization of genes encoding botulinum neurotoxin (BoNT) and other components of the progenitor complex. Sequence analysis showed that the silent BoNT/B gene is highly related to that from authentic proteolytic type B C.

Botulism: the present status of the disease.

The main form of human botulism throughout the world is the classic foodborne intoxication. Would botulism is very rare, and most of the documented cases have been found in the United States. While infant botulism remains rare throughout the world, it has become the most frequent form of the disease in the United States in recent years.

Detection of type A, B, and E botulism neurotoxin genes in Clostridium botulinum and other Clostridium species by PCR: evidence of unexpressed type B toxin genes in type A toxigenic organisms.

We studied the effectiveness of the PCR in detecting the type A, B, and E botulism neurotoxin genes in 209 strains of Clostridium botulinum and 29 strains of other Clostridium spp. All 79 strains that produced type A toxin, 77 strains that produced type B toxin, and 51 organisms that produced type E toxin (46 C. botulinum and 5 C.

Wound botulism in a patient with a tooth abscess: case report and review.

We describe a case of wound botulism associated with a tooth abscess in a 5-year-old boy. We reviewed the literature and reports to the Centers for Disease Control and Prevention (Atlanta) of laboratory-confirmed cases of wound botulism. From 1943 through 1990, 47 cases were reported.

A massive outbreak of type E botulism associated with traditional salted fish in Cairo.

In April 1991, 91 hospitalized patients in Cairo were reported to the Egyptian Ministry of Health with botulism intoxication. To define the spectrum of illness and identify a food vehicle, 45 patients were interviewed and a case-control investigation was conducted among families of 5 hospitalized patients. Clinical specimens and specimens of implicated food were tested for toxin and cultured for Clostridium botulinum.

Clinical and laboratory comparison of botulism from toxin types A, B, and E in the United States, 1975-1988.

Cases of adult botulism (n = 309) were studied to identify clinical differences between toxin types and to evaluate the sensitivity of diagnostic laboratory testing. Patients with illness from type E toxin had the shortest incubation periods. Sporadic case-patients were more severely ill: 85% required intubation compared with only 42% in multiperson outbreaks.

Type F botulism due to neurotoxigenic Clostridium baratii from an unknown source in an adult.

Type F botulism was confirmed in a 54-year-old male with signs compatible with botulism who reported to the emergency unit of a hospital. Botulinal neurotoxin was detected in the patient's serum and fecal specimens, and a neurotoxigenic organism whose physiologic characteristics correspond to those of Clostridium baratii was isolated. The toxin produced by the isolate was neutralized by type F botulinal antitoxin and cross-neutralized with lower efficiency by type E antitoxin.

Toxigenic clostridia.

Toxigenic clostridia belonging to 13 recognized species are discussed in this review. Each species or group of organisms is, in general, introduced by presenting the historical aspects of its discovery by early investigators of human and animal diseases. The diseases caused by each species or group are described and usually discussed in relation to the toxins involved in the pathology.

Clostridium perfringens food poisoning: use of serotyping in an outbreak setting.

An outbreak of Clostridium perfringens food poisoning occurred among attendees of a firehouse luncheon. The predominant symptoms of diarrhea (100%) and abdominal pain (81%) among case-patients, the mean incubation period (13.4 h), and the mean duration of illness (21.

Multilocus enzyme electrophoresis of Clostridium argentinense (Clostridium botulinum toxin type G) and phenotypically similar asaccharolytic clostridia.

Twenty-three strains of Clostridium argentinense, C. subterminale, C. hastiforme, and other phenotypically similar asaccharolytic clostridia recently placed in seven DNA hybridization groups were compared by multilocus enzyme electrophoresis.

Genetic confirmation of identities of neurotoxigenic Clostridium baratii and Clostridium butyricum implicated as agents of infant botulism.

Two unusual neurotoxigenic clostridia isolated from fecal specimens from patients with type F and type E infant botulism were phenotypically identical to the existing species Clostridium baratii and C. butyricum, respectively. DNA hybridization experiments confirmed that one strain was C.