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Allergology International : Official... Apr 2024Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE mediated food allergy presenting with delayed onset of projectile vomiting in the absence of cutaneous... (Review)
Review
Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE mediated food allergy presenting with delayed onset of projectile vomiting in the absence of cutaneous and respiratory symptoms. The pathophysiology of FPIES remains poorly characterized. The first international consensus guidelines for FPIES were published in 2017 and provided clinicians with parameters on the diagnosis and treatment of FPIES. The guidelines have served as a resource in the recognition and management of FPIES, contributing to an increased awareness of FPIES. Since then, new evidence has emerged, shedding light on adult-onset FPIES, the different phenotypes of FPIES, the recognition of new food triggers, center-specific food challenge protocols and management of acute FPIES. Emerging evidence indicates that FPIES impacts both pediatric and adult population. As a result, there is growing need to tailor the consensus guidelines to capture diagnoses in both patient groups. Furthermore, it is crucial to provide food challenge protocols that meet the needs of both pediatric and adult FPIES patients, as well as the subset of patients with atypical FPIES. This review highlights the evolving clinical evidence relating to FPIES diagnosis and management published since the 2017 International FPIES Guidelines. We will focus on areas where recent published evidence may support evolution or revision of the guidelines.
Topics: Adult; Child; Humans; Infant; Food Hypersensitivity; Vomiting; Enterocolitis; Allergens; Administration, Cutaneous; Dietary Proteins
PubMed: 38326194
DOI: 10.1016/j.alit.2024.01.006 -
Indian Journal of Otolaryngology and... Dec 2023Spontaneous cerebrospinal fluid (CSF) oto-rhinorrhoea is rare and may develop secondary to inner ear malformation. Any child discharging watery fluid through nose or ear...
Spontaneous cerebrospinal fluid (CSF) oto-rhinorrhoea is rare and may develop secondary to inner ear malformation. Any child discharging watery fluid through nose or ear spontaneously in a head dependent position should be a high index of suspicion of CSF leak. If watery rhinorrhoea is present then apart from biochemical analysis of fluid discharge to confirm it to be CSF, computed tomography of paranasal sinuses and temporal bone should be carried out to differentiate between CSF oto-rhinorrhoea and CSF rhinorrhoea. Congenital deformities of the inner ear can be associated with meningitis and varying degrees of hearing loss. Here we describe two cases, one of CSF oto-rhinorrhea in a 1 year old child who presented with spontaneous watery rhinorrhoea following violent projectile vomiting of 1 month duration. On evaluation, child was found to have CSF oto-rhinorrhoea with right Mondini deformity and profound hearing loss on right side. 2nd case was of 12 years old male with profound hearing loss right ear and recurrent episodes of meningitis diagnosed as common cavity malformation of inner ear. Both children underwent closure of CSF leak from oval window successfully. There was no recurrence after more than 1 year of follow up. Congenital inner ear malformations are an important cause of recurrent meningitis in children and require a high index of suspicion for diagnosis. Thorough clinical evaluation and radiological study is suggested in all cases of CSF oto-rhinorrhoea for the identification inner ear malformation and management of CSF oto-rhinorrhoea. Patients who receive an accurate and early diagnosis can avoid severe complications and have a good prognosis.
PubMed: 37974848
DOI: 10.1007/s12070-023-04098-1 -
The Journal of the Association of... Apr 2024A 21-year-old female patient presented to us with severe low back pain for 4 months. On examination, patient was afebrile, with severe pallor, and tenderness in both...
HISTORY AND EXAMINATION
A 21-year-old female patient presented to us with severe low back pain for 4 months. On examination, patient was afebrile, with severe pallor, and tenderness in both sacroiliac (SI) joints. Patient was being admitted and evaluated, and during the course of evaluation, developed severe headache, which was severe in intensity and associated with nausea and projectile vomiting. Initial investigations: An X-ray of the bilateral SI joints revealed inflammation, and the antinuclear antibody (ANA) turned out to be 4+ with pancytopenia and raised lactate dehydrogenase (LDH), but the liver function tests were normal. Rest of the rheumatological profile was unremarkable. During the course of the evaluation, she developed a severe headache, which, on imaging, showed presence of cerebral edema with chronic subdural hematoma, and a concomitant coagulopathy workup revealed evidence of disseminated intravascular coagulation (DIC).
DISCUSSION
Taking the whole picture into consideration, a malignant process in the body was suspected, and serum tumor markers carcinoembryonic antigen (CEA), carbohydrate antigen 19-9 (CA19-9), and cancer antigen 125 (CA-125) were sent, all of which were raised. Validating the clinical clue was the bone marrow biopsy done for pancytopenia, which revealed malignant epithelial infiltration. A contrast-enhanced computed tomography (CECT) thorax and whole abdomen were done to find out the primary, which showed a neoplastic mass at the gastroesophageal junction along with bony metastases in the vertebrae and left adrenal. Tissue from the primary lesion was taken for histopathological examination (HPE) through upper gastrointestinal endoscopy. Although HPE revealed grade III poorly differentiated stomach adenocarcinoma, the patient had succumbed to the disease process by the time the diagnosis came to light.
CONCLUSION
In short, this case perfectly illustrates how solid organ malignancies might be a mimicker of multisystem disorders, thereby delaying diagnosis and worsening the prognosis even further.
Topics: Humans; Female; Pancytopenia; Disseminated Intravascular Coagulation; Young Adult; Autoimmunity
PubMed: 38881089
DOI: 10.59556/japi.72.0497 -
Cureus Jan 2024One-and-a-half syndrome (OHS) is a horizontal gaze palsy in one direction with internuclear ophthalmoplegia (INO) in the other. The only eye movement possible is the...
One-and-a-half syndrome (OHS) is a horizontal gaze palsy in one direction with internuclear ophthalmoplegia (INO) in the other. The only eye movement possible is the abduction of the contralateral eye with nystagmus. The usual structures affected are the medial longitudinal fasciculus and paramedian pontine reticular formation or the abducens nucleus. Most commonly, the OHS is caused by ischemia and demyelinating lesions. The other causes include infectious, neoplastic, and rarely traumatic. We report a case of a 42-year-old non-compliant hypertensive female who presented with giddiness, projectile vomiting, and right-sided hemiparesis and was found to have OHS on cranial nerve examination in the emergency department (ED). In the ED, the presence of complete horizontal gaze palsy in one direction with INO in the other direction should raise suspicion of a brainstem pathology.
PubMed: 38425582
DOI: 10.7759/cureus.53193 -
JPGN Reports Nov 2023Infantile hypertrophic pyloric stenosis is a common surgical disease in infants, with an incidence of 2 to 5 cases per 1000 live births. It often presents with...
Infantile hypertrophic pyloric stenosis is a common surgical disease in infants, with an incidence of 2 to 5 cases per 1000 live births. It often presents with nonbilious projectile vomiting after feeding and a mid-epigastric mass in infants between the third and eighth weeks of life. Ramstedt pyloromyotomy remains the gold standard of treatment. Postoperative emesis is common; however, further evaluation for incomplete pyloromyotomy and recurrent pyloric stenosis should be conducted with prolonged, or new-onset postoperative emesis. While repeat pyloromyotomy is the standard of care for infants presenting with incomplete pyloric stenosis, treatment for the rare development of recurrent pyloric stenosis is not clearly outlined. Here, we report a successful balloon dilation procedure in an 8-week-old female with recurrent pyloric stenosis three and a half weeks after the initial laparoscopic pyloromyotomy.
PubMed: 38045639
DOI: 10.1097/PG9.0000000000000364 -
Qatar Medical Journal 2024Cerebral venous sinus thrombosis (CVST) is a rare and life-threatening condition that may be encountered during pregnancy and puerperium. The diagnosis of CVST is a...
INTRODUCTION
Cerebral venous sinus thrombosis (CVST) is a rare and life-threatening condition that may be encountered during pregnancy and puerperium. The diagnosis of CVST is a challenge because of its varied presentation.
CASE REPORT
A 28-year-old woman presented with headache, projectile vomiting, and generalized tonic-clonic seizures 10 days after delivery by cesarean section. She had an uneventful antenatal period of 38 weeks of gestation. High clinical suspicion and the availability of magnetic resonance venography helped in making a diagnosis of CVST. She was successfully managed with a low-molecular-weight heparin (LMWH) and anti-epileptic therapy with no residual complications.
DISCUSSION
Pregnancy induces several prothrombotic changes in the coagulation system that predispose to CVST. These changes persist for six to eight weeks after birth. Infection and cesarean section are the additional risk factors for CVST during puerperium. The symptoms of CVST depend on the sinuses and veins involved, raised intracranial pressure, and the extent of brain parenchymal injury.
CONCLUSION
Greater awareness of the disease and the availability of imaging modalities have contributed to the early diagnosis and favorable outcomes in these cases. LMWH is the main stay of treatment in this disease.
PubMed: 38567103
DOI: 10.5339/qmj.2024.13 -
Journal of Clinical Medicine Sep 2023Taliglucerase alfa is an enzyme replacement therapy approved for Gaucher disease. We assessed the duration/compliance/safety of such home infusions in commercial use in...
Taliglucerase alfa is an enzyme replacement therapy approved for Gaucher disease. We assessed the duration/compliance/safety of such home infusions in commercial use in four countries where home infusion programs are available. The treatment duration/compliance study included 173 patients (Israel, 58; US, 61; Brazil, 48; Australia, 6) who received ≥1 taliglucerase alfa home infusion through 6/2021. The median age at home therapy initiation was 38 (range, 2-87) years; 58% were females. The median treatment duration (at home) was 2.7 (range, 0.04-9.0) years. The annual compliance rate was stable (≥95%) throughout the study period. A search of the Pfizer global safety database (through 6/2021), identified 19 adverse events (AEs) as related to "definite home use" and 14 to "possible home use" of taliglucerase alfa; 42.4% of these AEs were serious; none were fatal. Twelve serious AEs in five separate case reports were considered treatment related: one case of chest discomfort/pain and hypertension and one case of erythema associated with a toe blister, for which causality could not be excluded; pain in extremity; projectile vomiting and chills, alongside excessive eye blinking; and an infusion-related AE (pruritus). In conclusion, this real-life global study demonstrated that taliglucerase alfa home infusions are safe with high compliance rates.
PubMed: 37762854
DOI: 10.3390/jcm12185913 -
Journal of Medical Case Reports Aug 2023Cryptococcal meningitis (CM) is an inflammatory mycosis of the central nervous system caused by meninge infection or brain parenchyma with Cryptococcus species. It is... (Review)
Review
BACKGROUND
Cryptococcal meningitis (CM) is an inflammatory mycosis of the central nervous system caused by meninge infection or brain parenchyma with Cryptococcus species. It is associated with high morbidity and mortality, and patients with acquired immune deficiency syndrome are particularly susceptible. There have been increasing reports of CM in HIV-negative patients in China over the last few years.
CASE PRESENTATION
A 31-year-old healthy Chinese male presented with fever and gradually developed headache, projectile vomiting, and other manifestations that were later confirmed as Cryptococcus gattii meningoencephalitis. However, multiple disease changes occurred during the course of treatment, and the regimen was accordingly modified after the diagnosis of post-infectious inflammatory response syndrome (PIIRS). The patient eventually recovered.
CONCLUSION
There has been a growing trend in the incidence of C. gattii meningoencephalitis in HIV-negative patients. It shows rapid onset and severe prognosis. This case report can provide a reference to treat PIIRS following CM in HIV-negative patients.
Topics: Humans; Male; Adult; Meningoencephalitis; HIV Infections; Inflammation; Magnetic Resonance Imaging; Cryptococcus gattii; Meningitis, Cryptococcal
PubMed: 37542340
DOI: 10.1186/s13256-023-04066-x -
International Journal of Surgery Case... Nov 2023A volvulus is a serious surgical emergency caused by torsion or hyper-flexion of the bowel loop and its mesentery on a fixed point. Cecal volvulus is an uncommon cause...
INTRODUCTION
A volvulus is a serious surgical emergency caused by torsion or hyper-flexion of the bowel loop and its mesentery on a fixed point. Cecal volvulus is an uncommon cause of intestinal obstruction, accounting for just 1-1.5 % of all cases of bowel obstruction.
CASE PRESENTATION
A 33-year-old intellectually disabled male presented to the emergency department with complaints of generalized abdominal pain, absolute constipation, and non-projectile vomiting. He had a grossly distended and rigid abdomen with generalized tenderness and guarding. The abdomen was hyper-resonant. Bowel sounds were hypoactive. Digital rectal examination revealed an empty and collapsed rectum with no stool staining of the finger. Laboratory reports showed leukocytosis and neutrophilia. Radiographic imaging was consistent with acute intestinal obstruction. Laparotomy was performed, and cecal volvulus with viable bowel and acute appendicitis was diagnosed intra-operatively. Manual detorsion, cecopexy, and appendectomy were performed. The patient had an uneventful postoperative course. The patient is still on follow-up since June 2023, and no complication has occurred.
CLINICAL DISCUSSION
Cecal volvulus is an uncommon cause of intestinal obstruction with multiple etiologies. The annual incidence of cecal volvulus is estimated to be between 2.8 and 7.1 cases per million. Cecal volvulus may lead to life-threatening complications such as bowel ischemia and perforation.
CONCLUSION
Diagnosis of cecal volvulus must be made promptly to prevent bowel gangrene and perforation. In this case, manual detorsion and cecopexy were performed as the bowel was viable per-operatively due to early surgical intervention.
PubMed: 37844385
DOI: 10.1016/j.ijscr.2023.108904