We report a case of TTP in a sickle cell/β+-thalassemia heterozygote diagnosed by presence of schistocytes on peripheral smear (above). Included in this case is a discussion of the development of functional hyposplenism (evidenced by Howell-Jolly bodies, above), a rarely reported complication, limitation of ADAMTS-13 in diagnosis, and the use of platelet transfusion.

Idiopathic Systemic Capillary Leak Syndrome is a potentially fatal disorder that is under diagnosed. It commonly presents as recurrent undifferentiated shock with hypotension, hypoalbuminemia and hemoconcentration. There are three distinct phases that define the syndrome; Prodromal, Extravasation and Recovery.

The oral manifestations of EBV-positive mucocutaneous ulcers have a worrisome clinical appearance but relatively benign clinical course, responding well to conservative treatment. Elderly patients who develop an unexplained, persistent ulcer of the oral mucosa should have the lesion examined for EBV.

Infantile pyknocytosis is a rare cause of neonatal jaundice and hemolytic anemia. We report on two cases in twin girls that were diagnosed on peripheral blood smear reading. Pyknocytosis should be considered in cases of early unexplained severe hemolytic anemia, and systematic peripheral smear review performed. Its management consists of phototherapy and RBC transfusion.

Necrobiosis lipoidica (NL) is a rare idiopathic cutaneous condition exceptionally associated with autoimmune thyroiditis. We describe the first case of NL, Hashimoto's thyroiditis and positive detection of autoantibodies. Appropriate screening for NL in patients with autoimmune thyroiditis may clarify its real incidence and the existence of a common pathogenetic pathway.

Unstable 4 part pertrochanteric fractures without lateral cortical support presents considerable technical problem in treatment with high risk of failure with any implant. Anatomical or slight valgus reduction and subchondral central position of the lag screw (TAD <25 mm) reduces the chance of screw cut out and other complications.

Traumatic CSDH enlarged in two cases with VP or LP shunt system although the shunt valve pressure was increased to 200 mmH₂O. In surgery, the hematoma cavity pressure was found to be 130 and 140 mmH₂O, suggesting that to raise the shunt valve pressure is not effective for decreasing CSDH volume.

Lenalidomide is an immunomodulatory drug which is used to treat patients with MDS with deletion 5q chromosomal abnormality. In 2008, WHO introduced a new disease entity called MDS/MPN. No specific treatment for MDS/MPN subtype has yet been identified. We report a patient with MDS/MPN who responded well to lenalidomide therapy.

Hypertension with Chronic kidney disease is often difficult to control medically. In such patients, nephrectomy can help to control blood pressure (BP). We describe a case of a 6-year-old boy with autosomal recessive polycystic kidney disease who showed a paradoxical increase in BP following bilateral nephrectomy.

We present a pediatric case of medically unmanageable juvenile colonic polyposis, initially treated with subtotal colectomy and an ileostomy followed by a proctectomy, ileal-J-pouch and serial transverse enteroplasties (STEP) of the distal ileum. The STEP procedure in an adequate length was able to control stooling of our patient.

Upper gastrointestinal bleeding causes significant morbidity and mortality worldwide. We report a rare case of hematemesis secondary to a gastric schwannoma in a Tanzanian female. Gastric schwannomas should be considered in the differential diagnosis of gastric masses and distinguished from other etiologies, given their excellent postresection prognosis.

Calcium channel blocker toxicity can be devastating. Initial therapy with fluid, calcium, and adrenoreceptor agonists should be prompt and novel therapies can be added if no response. Determining cardiogenic shock versus vasoplegia with echocardiogram or other hemodynamic monitoring may guide treatment options.

We present a patient with trisomy 8p11.21q11.21 associated with language, gross motor, fine motor, and cognitive delay. Furthermore, using array-based comparative genomic hybridization, we identify the specific genes duplicated in our patient.

In this article, we report a case of two synchronous ileal adenomyomas leading to intussusception. This rare occurrence has never been reported in the literature. Our case is noteworthy, because the lesion is rare and should be considered in the differential diagnosis of intussusception in adults.

Lower-limb bone metastases are uncommon in low-grade endometrial carcinoma, and are mostly located on the axial skeleton. Here, we present a rare case of bilateral femur metastases in low-grade endometrial carcinoma and performed a review of the current literature.

Prolonged clotting times were observed in a patient with spontaneous hemorrhage. Analysis showed severe factor X deficiency due to clearance by a noninhibitory antibody. Lymphadenopathy identified on imaging led to diagnosis of marginal B-cell lymphoma. Treatment of lymphoma with rituximab and chlorambucil resulted in complete disappearance of the bleeding disorder.

The electrocardiogram (ECG) of a 73-year-old, asymptomatic woman showed deep T-wave inversion. The complete workup was negative. Ten years later, she developed takotsubo cardiomyopathy with abnormal ECG again. Isolated deep T-wave inversion might be an aftereffect of takotsubo cardiomyopathy that does not warrant an invasive workup.

Extensive full-thickness skin wounds are quite common in domestic animals. In these report, following the failure of reconstructive surgery, adipose tissue-derived mesenchymal stem cells and platelet-rich plasma were successfully used in a dog to improve speed and quality of skin tissue healing, avoiding suffering, and debilitating effects.

X-linked intellectual deficiency (XLID) is a large group of genetic disorders. MED12 gene causes syndromic and nonsyndromic forms of XLID. Only seven pathological mutations have been identified in this gene. Here, we report a novel mutation segregating with XLID phenotype. This mutation could be in favor of genotype–phenotype correlations.

The continuing resorption of the alveolar ridge will eventually result in insufficient bone height superior to the IAN, making dental implant placement impossible. The augmentation procedure above the IAN in terms of height provides sufficient bone for implant placement and allows long-term successful restoration of missing teeth with implant-supported prosthesis.

We report a 53-year-old man diagnosed with Richter syndrome. He was heavily pretreated and was refractory to prior therapy. He received rituximab and ibrutinib, and achieved a significant response after 1 month of therapy. Our case illustrates the importance of investigation of rituximab and ibrutinib in Richter's syndrome.

Despite decrease in morbidity and mortality from invasive pneumococcal disease (IPD), individuals with asplenia remain at risk for IPD compared to the general population. This report describes a young adult with hemoglobin SD and documented splenic auto-infarction with pneumococcal sepsis, meningitis, and pneumonia within seven months of immunization with PPSV-23.

A 76-year-old Japanese woman contracted a Mycobacterium tuberculosis (TB, Manila type) infection in Japan, despite never having traveled. However, her son was treated for TB in the Philippines 3 years before he stayed at her house. Spoligotyping allows us to identify the TB genotype and identify the route of infection.

Localized nodular pulmonary amyloidosis is rare. However, the disease should be considered in the differential diagnosis of multiple lung nodules.

Organ dysfunctions caused by intraabdominal hypertension is named as abdominal compartment syndrome (ACS). A patient with prostate cancer admitted with dyspnea and oliguria. After decompression his health status improved. For patients with malignant disorders presented with oliguria and respiratory problems who have abdominal distension, ACS should be in mind.

Emphysema results in narrowing of the small airways due to inhaling of cigarette smoke and other noxious particles. Oxygen therapy, corticosteroids, and bronchodilators increase the risk of pneumonia, arrhythmia, and fractures in long term. Therapy with human embryonic stem cells resulted in improved symptoms of a patient with emphysema.

A 65-year-old male, Parkinson's disease patient, was evaluated for GPR109A expression, niacin index, UPDRS scale, handwriting test, and quality of sleep with and without niacin treatment. The evaluation was repeated 3 months after niacin was stopped. Niacin modulated the abovementioned parameters and showed the overall improvement without side effects.

Iliopsoas abscesses have been reported in adult diabetic patients, but only one case has been so far reported in the pediatric diabetic literature. We report three cases of iliopsoas abscesses in three adolescents with type 1 diabetes mellitus, suggesting that an increased awareness of this condition is required for its early recognition and prompt treatment.

This is the first reported case of fetal pericardial effusion in association with X-linked adrenoleukodystrophy and hypocortisolism from a nonautoimmune cause. Our hypothesis is that in experienced hands and after accurate genetic counseling, isolated pericardial effusion can constitute an indication for a severe metabolic disease.

Treatment of patients with refractory Hodgkin lymphoma is a significant issue. We report a patient with Proteus syndrome and relapsed Hodgkin lymphoma, whose remission was finally achieved after brentuximab vedotin therapy, allowing her to receive a haploidentical stem cell transplant. The possible relationship between both disorders was discussed.

We present a case of severe, irreversible neurotoxicity in a 55-year-old-patient with myelofibrosis undergoing hematopoietic stem cell transplantation following a reduced intensity conditioning including fludarabine. The patient developed progressive sensory-motor, visual and consciousness disturbances, eventually leading to death. MRI imaging pattern was unique and attributable to fludarabine neurotoxicity.

Convulsion in diabetics is often considered as a result from fluctuation of blood glucose level. However, if a diabetic patient also presents abnormal neurological signs, mitochondrial diseases need to be considered in the differential diagnosis.

We report the rare case of delayed hemothorax (DHX) with an inferior phrenic artery (IPA) injury due to blunt thoracic trauma. Our case suggests that DHX almost always occurs early after injury, and endovascular treatment is an effective procedure for traumatic hemothorax including DHX.

Haematopoietic abnormalities associated with tacrolimus are relatively rare with reversible pure red cell aplasia being the most common. We report for the first time, to our best knowledge, tacrolimus therapy associated with bone marrow fibrosis, abnormal megakaryocytosis, and dyserythopoiesis in a 17-year-old male treated with tacrolimus for nephrotic syndrome.

We present a 12-year-old immunocompetent girl with hepato splenic cat-scratch disease (CSD). Her sole inaugural complaint was isolated epigastric pain. She fully recovered, with normalized abdominal CT scan following 2 weeks course of Azythromycin^®. CSD should be included in differential diagnosis in children with epigastric pain, especially in those with domestic pets.

We present the successful application of C1 esterase inhibitor (C1INH) concentrate to a patient with clinical amniotic fluid embolism (AFE).

In patients with foveal hypoplasia, anterior segment dysgenesis and an absence of systemic findings, consider a recently described syndrome of foveal hypoplasia, optic nerve decussation defects and anterior segment dysgenesis (FHONDA) in the differential diagnosis.

An ink marker at the descending part of the gastrojejunostomy or duodenojejunostomy after a pancreaticoduodenectomy or pylorus-preserving pancreaticoduodenectomy prevents a misplacing of a postoperative endoscopic intervention.