Rapid drug desensitization to taxanes: a descriptive study from Turkey.

AIM: To present the characteristics of drug hypersensitivity reactions (DHRs) among taxane recipients with non-small cell lung carcinoma (NSCLC), and to describe the results of rapid drug desensitization (RDD). METHODS: A retrospective cross-sectional study included 45 patients who were treated with taxane for NSCLC and were found to be hypersensitive to taxane. All patients were administered the standard 3-bag, 12-step RDD protocol following the development of DHR. RDD success was evaluated separately for each cycle, and successful RDD was defined as the completion of the cycle with application of 12 steps of the desensitization protocol and the absence of early and/or late reactions afterwards. RESULTS: Among 45 patients hypersensitive to taxane 43 (95.6%) successfully received taxane cycles with desensitization. Failed RDD occurred in only 2 (4.4%) patients. The total number of desensitization cycles was 183, of which 181 (98.9%) were successful. The mean age of patients with successful desensitization was 59.42 ± 10.48 years and 37 (86.0%) of them were male. CONCLUSION: RDD is a reliable procedure that enables effective administration and completion of first-line taxane treatments in taxane-sensitive patients.

Platin desensitizations in thoracic malignancies and risk factors for breakthrough reactions.

Although platin desensitization is a safe and effective alternative for patients with hypersensitivity reactions (HSRs), sometimes breakthrough reactions (BTRs) can be encountered. However, data about the risk factors for BTRs are limited. The aim of this study is to define the outcomes of desensitization, the characteristics of BTRs, and to identify the risk factors for BTRs with platins in thoracic malignancies. This is a retrospective report of patients with thoracic malignancies who underwent platin desensitization. Patients' demographics, initial HSR characteristics, skin test results, desensitization outcomes, and BTR characteristics were recorded. Thirty-three lung cancer and 14 malignant pleural mesothelioma (MPM) patients were included in the study. The culprit drug was cisplatin in 29 and was carboplatin in 18 patients. Skin test positivity was 43.5% with cisplatin, 50% with carboplatin, and it was found to be higher if the interval between the initial HSR and skin testing (ST) was ˃20 days (p = 0.027). One hundred and five desensitization courses were performed. Twenty-two patients had 33 BTRs. Skin test positivity was higher in the BTR-positive group (p = 0.025). BTRs (18.2%; n = 6) were more severe than initial HSR. In the case of epinephrine administration during initial HSR, epinephrine administration during the first BTR was found to be more (p = 0.036). The target dose was achieved in 92.4% of desensitization courses. The number of previous platin infusions ≥10 was found to be an independent risk factor for BTR development (p = 0.036 OR:17.641, 95% CI: 1.211-256.971). Identification of risk factors for BTR will guide appropriate management and desensitization approaches for platin HSRs.

Successful desensitization under antihistamine suppression in a case with urticaria due to osimertinib.

INTRODUCTION: Osimertinib is an approved therapy for patients with a Thr790met (T790M) mutation diagnosed with non-small cell lung cancer (NSCLC) that progresses during epidermal growth factor receptor-tyrosine kinase inhibitor (EGFR-TKI) therapy. However, in 7-13% of patients, drug-related side effects lead to discontinuation of osimertinib treatment. In such cases, osimertinib desensitization is a treatment option that can be considered. CASE REPORT: A 59-year-old female patient, who was followed up with the diagnosis of stage 4 NSCLC, was consulted to the allergy clinic because of urticaria. The patient developed urticaria plaques 20 h after the third dose of osimertinib tablet. MANAGEMENT & OUTCOME: With the diagnosis of osimertinib-induced urticaria, desensitization was planned for the patient. Treatment was started with a dose of 0.1 mg/day osimertinib. The procedure was completed in approximately 50 days, and a dose of 80 mg/day was reached with antihistamine suppression. DISCUSSION: Here, a successful osimertinib desensitization in a patient with a history of osimertinib-related type 1 allergic reaction is reported. Osimertinib desensitization is a treatment option that should be considered in cases where treatment has to be ceased due to drug-related side effects.

A New Perspective for Infantile Hepatic Hemangioma in the Age of Propranolol: Experience at Baskent University.

Propranolol was first used in 2008 to treat hemangioma; its efficacy and safety have since changed the classical treatment indications. Infantile hepatic hemangioma presents as a spectrum of clinical conditions varying from simple asymptomatic lesions to lethal complications. Tufted hemangioma and Kaposiform hemangioendothelioma are congenital vascular tumors that lead to Kasabach-Merritt syndrome. Hemangiomas, like pure arteriovenous malformations, can cause hyperdynamic heart failure, and diffuse nodular-type hemangiomas can present with hypothyroidism. Respiratory problems and hepatic failure can be associated with diffuse nodular-type liver hemangiomas. There is a spectrum of approaches to management, varying from "watchful waiting" to liver transplant. In the age of propranolol, there has been a prominent change in the infantile hepatic hemangioma treatment algorithm. Our suggestion is early treatment with 3 mg/kg/day propranolol plus 1.0 to1.5 mg/kg/day prednisolone in all patients. This protocol is the most effective strategy for type 3 infantile hepatic hemangioma. Approximately one-third of patients with abdominal compartment syndrome in the era before propranolol treatment required liver transplant; this new treatment obviates transplant for many of these patients.

A different cause for respiratory disorder in children: cases with pulmonary Langerhans cell histiocytosis.

BACKGROUND AND AIMS: In children, complaints of a respiratory disorder are very frequent. Etiology of respiratory illness is a broad spectrum that varies from a simple viral infection to a malignant disorder. Pulmonary Langerhans cell histiocytosis (PLCH) is one of these entities and it is truly rare in children. The aim of this study is to evaluate our patients with PLCH. METHODS: Patients who had been diagnosed with PLCH were retrospectively evaluated. Features of medical history, onset of the complaints, date of the diagnosis, chest X-Ray and computed tomography (CT) findings, histopathology and other laboratory investigations were considered. RESULTS: There were four cases with PLCH. All of them were male, ages were between 5 months and 16 years. In three cases, major complaints were chronic respiratory problems whereas in one of them there was acute respiratory distress beginning with cough and leading to pneumothorax. In all of the cases, multisystemic involvement was prominent. The diagnosis was proven by histopathology in all of the cases. In two children with smaller age, skin involvement was detected. Time from complaint to diagnosis was minimum 3 months and maximum 3 years. CONCLUSION: PLCH is a rare disorder in children. Pulmonary involvement is generally a component of systemic involvement but in many cases it might have been detected with early respiratory complaints. So, children with chronic respiratory problems should be carefully evaluated and should be followed up for rare entities like PLCH.

Spleen Salvaging Treatment Approaches in Non-parasitic Splenic Cysts in Childhood.

The aim of this study was to evaluate our experience with primary non-parasitic splenic cysts (NPSC) which are relatively rare in children and consist almost exclusively of single case reports or small case series in the literature. The medical records of all patients who presented to our clinic with NPSC between 2005 and 2015 were evaluated retrospectively. There were 22 children whose ages ranged from 2 months to 14 years (mean 9.2 ± 4.7 years). The size of the cysts was in the range of 5 to 200 mm (mean 55.4 ± 48.2 mm). Ten patients underwent surgery for splenic cysts. Partial splenectomy (n = 2), total cyst excision (either open n = 4 or laparoscopically n = 1), and total splenectomy (n = 3) were performed. The non-operated patients were asymptomatic and followed with ultrasound (US). The follow-up period in non-operated patients ranged from 6 months to 5 years (mean 2.27 ± 1.29 years). Complete regression was observed in four (33 %) non-operated patients. The regressed cyst measurements were 10, 16, 30, and 40 mm, respectively. Approximately half of the NPSC is diagnosed incidentally. Small (<5 cm) asymptomatic cysts should be under regular follow-up with US/physical examination for regression. If surgery is required, we prefer open cyst excision as it gives excellent results and preserves splenic immune function.

A Rare Complication Developing After Hematopoietic Stem Cell Transplantation: Wernicke's Encephalopathy.

Thiamine is a water-soluble vitamin. Thiamine deficiency can present as a central nervous system disorder known as Wernicke's encephalopathy, which classically manifests as confusion, ataxia, and ophthalmoplegia. Wernicke's encephalopathy has rarely been reported following hematopoietic stem cell transplantation. Herein, we report Wernicke's encephalopathy in a patient with acute myeloid leukemia who had been receiving prolonged total parenteral nutrition after haploidentical allogeneic hematopoietic stem cell transplantation. To the best of our knowledge, this is the first case reported from Turkey in the literature.

Value of sonographic anterior-posterior renal pelvis measurements before and after voiding for predicting vesicoureteral reflux in children.

PURPOSE: Voiding cystourethrography (VCUG) is the gold standard for diagnosing vesicoureteral reflux (VUR), but it is important to minimize the use of VCUG because of the urinary catheterization and radiation exposure required. Ultrasound (US) observations suggest that pelvicalyceal dilatation varies according to the degree of bladder fullness in children with urinary tract infection. The aim of this study was to assess whether anterior-posterior (AP) measurements of the renal pelvis on US before and after voiding can be used as a screening tool while predicting the presence of VUR in children. METHODS: The subjects were toilet-trained children older than 4 years who required VCUG. Two groups were established based on the VCUG results: a VUR group of 40 kidney units (each unit defined as calyces and ureter) that exhibited different severities of reflux, and a control group of 68 kidney units unaffected by VUR. Prior to VCUG, US AP measurements of the renal pelvis of each kidney unit were recorded when the urinary bladder was full and again after bladder emptying. The change in AP measurement from before to after voiding was compared between the two groups. RESULTS: The mean change in AP measurements from before to after voiding in the VUR group was significantly greater than that in the control group (p = 0.003). CONCLUSIONS: Comparing US AP measurements of the renal pelvis before and after voiding is useful for identifying children who are suspected to have VUR and thus require immediate VCUG.

Variations in apparent diffusion coefficient values following chemotherapy in pediatric neuroblastoma.

PURPOSE: In children the assessment of solid tumors' response to chemotherapy is based primarily on size reduction, which can be unreliable and a late marker, in the presence of necrosis. We aimed to establish whether apparent diffusion coefficient (ADC) values of childhood neuroblastomas show proportional changes in relation to chemotherapy response. METHODS: We evaluated 15 pediatric patients with abdominopelvic neuroblastomas, who had undergone MRI before and after chemotherapy. Two radiologists retrospectively analyzed all images by drawing a round uniform region-of-interest in the solid/contrast-enhancing portion of the lesions in consensus. The ADC values from pre- and postchemotherapy images were compared. RESULTS: Postchemotherapy ADC values were significantly higher than those obtained before treatment (P < 0.05, for minimum, maximum, and median ADC values). CONCLUSION: Our results support diffusion-weighted MRI as a promising noninvasive biomarker of therapeutic responses. To the best of our knowledge, this is the first report to compare diffusion- weighted imaging findings before and after chemotherapy in childhood neuroblastic tumors.

Solid variant of aneurysmal bone cyst of the rib presenting as a left intrathoracic mass without radiological bone destruction.

An aneurysmal bone cyst (ABC) is a benign but often rapidly expanding osteolytic multi-cystic osseous lesion that occurs as a primary, secondary, intra-osseous, extra-osseous, solid, or conventional lesion. A 15-year-old boy presented with a left-sided intrathoracic mass displacing the lung without bone destruction. The mass was totally resected without rib resection, and the pathological diagnosis was ABC. The clinical manifestations, etiology, management, and pathology are discussed, with a brief discussion regarding the difficulty in the preoperative differential diagnosis.

Acute transverse myelitis in a child with Lyme disease and a review of literature.

Acute transverse myelitis is a rare Borellia burgdorferi-related neurologic complication in childhood. We present a 12-year-old girl who was diagnosed with acute transverse myelitis associated with a borreliosis infection. We also review clinical features in all five cases of Borellia burgdorferi-related transverse myelitis in children. We describe here the sixth child with borreliosis-related transverse myelitis.

Multidetector computed tomography diagnosis of ileal and antropyloric gallstone ileus.

Gallstone-induced ileus is a rare complication of cholelithiasis, and gastric outlet obstruction is even rarer. We describe the multidetector computed tomographic diagnosis of small bowel obstruction resulting from a gallstone impacted in the distal ileum and of gastric outlet obstruction from a gallstone impacted in the pyloric antrum (Bouveret syndrome).

Propranolol for infantile hemangiomas: a preliminary report on efficacy and safety in very low birth weight infants.

Despite the relatively recent introduction of propranolol in the treatment of infantile hemangiomas, there can be little doubt of its efficacy. With regard to safety issues, there are no prior data for very low weight infants. In this study, we used propranolol in preterm and very low weight infants. We used clinical criteria to assess the response to the therapy. We noted all side effects expected from beta-adrenergic blocking drugs, and followed the patients' weight gain during propranolol treatment. Objective, clinical evidence of hemangioma regression was seen after two months in all patients. None of the patients required treatment discontinuation due to adverse side effects. During the propranolol treatment, weight gain was normal in all patients. To the best of our knowledge, this is the first report on the use of propranolol in preterm and very low weight infants, and also the first report from Turkey on the use of propranolol in infantile hemangiomas.

Primary vaginal calculus secondary to urethrovaginal fistula with imperforate hymen in a 6-year-old girl.

Primary vaginal stones are extremely rare in children and may be mistaken for bladder calculi on plain radiography. We present a case of a large vaginal calculus in a 6-year-old girl who had an imperforate hymen and urethrovaginal fistula. Hymenotomy and urethrovaginal fistula repair were performed, and the vaginal stone was extracted. It was postulated that the vaginal calculus originated from stasis of urine through the urethrovaginal fistula in the obstructed vagina. This is a unique case of a vaginal calculus with a congenital urethrovaginal fistula associated with an imperforate hymen.

Prenatal diagnosis of a mass in the adrenal region that proved to be a teratoma.

A prenatally detected suprarenal cystic mass measuring 2 cm was found to have enlarged upon postnatal ultrasonography at 6 weeks of age. Magnetic resonance imaging showed a 57 x 50 mm mass in the left adrenal region displacing the kidney inferiorly. The infant underwent an adrenalectomy with total resection of the tumor, which proved on histologic examination to be a mature teratoma. Prenatally detected suprarenal masses are likely to be neuroblastoma or adrenal hemorrhage, but may be rare benign lesions such as extralobar pulmonary sequestration, bronchogenic cyst, or renal dysplasia. Although teratoma in the adrenal region is extremely rare, it should be included in the clinical and radiologic differential diagnosis of prenatally detected suprarenal masses. Total excision of the mass for histologic diagnosis is indicated.

Metastatic pulmonary calcification in a patient with chronic renal failure.

Metastatic pulmonary calcification characterized by diffuse calcium deposition in the lungs is known to occur in patients with chronic renal failure. We present a case of a 47-year-old man with chronic renal failure presented with dyspnea, high-resolution computed tomography of the chest revealed multiple, centrilobular, calcified nodules and patchy areas of ground-glass opacity throughout both lungs, consistent with metastatic pulmonary calcification. Calcification was also seen in the bronchi and trachea.