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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 -
Cureus Jun 2023We present a 22-day-old male born full term who presented with worsening non-projectile, non-bilious vomiting and failure to thrive (FTT) and was admitted to the...
We present a 22-day-old male born full term who presented with worsening non-projectile, non-bilious vomiting and failure to thrive (FTT) and was admitted to the pediatric intensive care unit (PICU) for severe metabolic acidosis with an elevated anion gap. Despite changing the formula, the patient continued to have spit-ups after feeds since birth. Before this admission, his vomiting worsened with every feed, which was now forceful along with two days of loose stools. Obstructive causes of emesis were ruled out with an upper gastrointestinal series, and a decision was made to evaluate for organic causes of FTT. Transient resolution of symptoms was noticed when the patient was placed NPO (nothing by os/mouth) briefly. His symptoms returned on resuming cow milk-based formula feeds. At this time, a presumptive diagnosis of cow milk protein allergy (CMPA) was made. Positive fecal occult blood supported the diagnosis, and his formula was changed to an extensively hydrolyzed formula (eHF). This is a case of severe CMPA with prolonged vomiting and FTT presenting with severe metabolic acidosis with an elevated anion gap. This case report highlights how CMPA can lead to severe dehydration with metabolic acidosis and increased anion gap.
PubMed: 37503473
DOI: 10.7759/cureus.40973 -
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 -
BMC Pediatrics Apr 2024Thyroglossal Duct Cyst (TDC) is a common lesion of the midline neck, originating from an incomplete involution of the thyroglossal duct. It is typically observed in...
BACKGROUND
Thyroglossal Duct Cyst (TDC) is a common lesion of the midline neck, originating from an incomplete involution of the thyroglossal duct. It is typically observed in pre-scholar patients and surgery is the treatment of choice to prevent infections. Here reported a case of incidental diagnosis in a newborn patient.
CASE PRESENTATION
a 3-week-old male baby was admitted to our hospital for weight loss and projectile vomits after breastfeeding. After a diagnosis of hypertrophic pyloric stenosis, the baby underwent pyloromyotomy. During the endotracheal tube placement, the anesthetist noticed the presence of a midline neck mass. The suspect of TDC was confirmed by an intraoperative ultrasound, so, despite the age of the patient, we proceeded with the excision of the lesion according to Sistrunk's procedure to avoid future complications and anesthesia.
CONCLUSIONS
even if TDC is a common lesion of pediatric patients, anecdotical neonatal cases were described in the literature, all of them symptomatic. An accurate physical examination and ultrasound are essential diagnostic tools to distinguish TDC from other middle neck lesions, particularly ectopic thyroidal tissue. Sistrunk's procedure is the most effective surgical approach. When diagnosis is made in a newborn, we suggest postponing surgery, unless the baby requires general anesthesia for other surgical procedures, such as in our case.
Topics: Humans; Thyroglossal Cyst; Male; Incidental Findings; Intubation, Intratracheal; Infant, Newborn; Ultrasonography
PubMed: 38654283
DOI: 10.1186/s12887-024-04742-x