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Biomedical subjects

A Koren

Publications and source records attributed to A Koren.

At least 55 records · Page 3Linked to original sources

Acute chest syndrome in children with sickle cell anemia.

Vaso-occlusive crisis is the most common cause of morbidity in patients with sickle cell anemia (SCA). Those patients are frequently admitted with chest signs and symptoms suggesting an infective process. The term acute chest syndrome (ACS) is used to describe those chest episodes in SCA patients, and it is postulated that a primary bone vaso-occlusive crisis may be the cause of the acute chest syndrome in SCA patients. In this study we report 52 episodes of ACS in a group of 22 children with SCA. Chest pain, fever, and leukocytosis were a constant clinical finding. The hematological, radiological, and bacteriological studies are reported. There is a constant and significant fall in hemoglobin levels from 88 +/- 10 g/L (8.8 +/- 1.0 g/dl) to 68 +/- 15 g/L (6.8 +/- 5 g/dl). Unilateral or bilateral pulmonary basal infiltrations were found in 50 episodes. Pleural effusion was noticed in 60 episodes, and it was bilateral in three. No significant bacteriological findings were present.

Adolescent↗

[Toxocariasis in Emek Israel].

Toxocariasis is a rare zoonotic disease in Israel. It usually affects children under the age of 10. Toxocara canis and Toxocara catis are common parasites among dogs and cats which affect man when he ingests the eggs of these helminths. We describe 3 children with different clinical presentations of the infestation. A 6-year-old boy had pain in the muscles of the limbs and diffuse patches in the right lower lobes on X-ray; a 7-year-old girl presented with a limp; and a 3-year-old boy had abdominal pain and a maculopapular rash covering the whole body. All 3 had eosinophilia. They illustrate the importance of toxocariasis in the differential diagnosis of eosinophilia.

Child↗

[Hepatitis A and pleural effusion in children].

Pleural effusion associated with hepatitis A is rare and has not previously been reported in children. A 5-year-old girl who recovered from this association of diseases is presented. An immunological mechanism is suggested.

Child, Preschool↗

Congenital dysgranulopoietic neutropenia in two siblings: clinical, ultrastructural, and in vitro bone marrow culture studies.

Two siblings with congenital neutropenia are reported. The first patient, female, died after Pseudomonas sepsis. The second patient male, suffered from recurrent pyogenic infections, with a more benign course. Bone Marrow (BM) and Peripheral Blood (PB) analysis in the second patient revealed a reduced number of granules and myelin bodies in the PB neutrophils, suggesting a developmental defect of primary and secondary granules. BM promyelocytes were almost normal, but the myelocytes and metamyelocytes showed defective granulogenesis. The BM in vitro granulocyte-macrophage-colony-forming cell (GM-CFC) growth and the PB white blood cells (WBC) granulocyte-macrophage-colony-stimulating factor (GM-CSF) production, which were analyzed in the second patient, showed normal numbers of GM-CFC, with differentiation mostly toward monocytes and a defect in the GM-CSF production capacity. The second patient's PB mononuclear cells or serum did not inhibit normal GM-CFC when added to control BM cells. We suggest that in this specific form of congenital neutropenia, which is probably an autosomal recessive disorder, the abnormal neutrophil granule production and the defective provision of GM-CSF by PB WBC are unique pathognomonic characteristics, possibly associated with the overt neutropenia.

Agranulocytosis↗

Diffuse abdominal gallium-67 citrate uptake in salmonella infections.

Two pediatric patients with salmonella infections (one with typhoid fever and the second with salmonella C2 gastroenteritis), had a diffuse abdominal uptake of Ga-67 citrate. The possible explanation for this finding is discussed. Salmonella infection should be included as a cause in the differential diagnosis of diffuse accumulation of Ga-67 citrate.

Abscess↗

Right ventricular cardiac dysfunction in beta-thalassemia major.

In patients with iron overload associated with severe, transfusion-dependent beta-thalassemia, congestive heart failure develops during the second decade of life. Biventricular heart function was studied by multigated radionuclide angiography in 22 patients with beta-thalassemia major. Six patients were symptomatic. Congestive heart failure developed in five patients at the time of blood transfusions, and one other patient had been treated for multiple ventricular extrasystole. The mean (+/- SD) left ventricular ejection fraction was normal (63.0% +/- 7.6%). Only one patient had a left ventricular ejection fraction under the normal level (less than 50%). The mean (+/- SD) right ventricular fraction (RVEF) was 33.3% +/- 9.4%. In only three patients was the RVEF normal (greater than or equal to 40%); an RVEF under 30% was registered in six patients. We suggest that the early right ventricular dysfunction in patients with beta-thalassemia may be due to pulmonary hypertension secondary to iron overload and iron deposits in the ventricles.

Adolescent↗

The complementary role of sequential 99mTc-MDP and 67Ga-citrate scanning in the diagnosis and follow-up of neuroblastoma.

Fourteen children with histopathologically confirmed neuroblastoma underwent 38 studies using 99mTc-methylene-diphosphonate (MDP) and galliumcitrate Ga67 whole-body scintigraphy during various stages of the disease. Ten patients (71%) showed 99mTc-MDP accumulation in the primary tumoral site, whereas 11 patients (78.6%) showed 67Ga concentration. In 12 patients (86%), at least one of these two radiopharmaceuticals concentrated in the primary tumor. Nine patients had osseous or extraosseous metastases. All of these metastases (100%) were positive on 99mTc-MDP scintigraphy. No 67Ga-citrate uptake was demonstrable in osseous metastases; only one extraosseous lung metastasis concentrated this radiopharmaceutical. 67Ga-citrate was superior to 99mTc-MDP with regard to accurately demonstrating the extent of primary tumors. Only 99mTc-MDP indicated the relationship of the tumor to the kidneys and neighbouring osseous structures, providing early screening of kidney compression and possible damage caused by the tumor. From these results, we found these two methods to be complementary for the diagnosis and follow-up of neuroblastoma; their combined use resulted in high diagnostic accuracy and a considerable gain of information. We therefore recommend sequential 99mTc-MDP and 67Ga-citrate scans for the diagnosis and evaluation of the primary tumor; periodic 99mTc-MDP whole-body scans should be used in the follow-up of treatment, and for discovering disease exacerbations and metastases.

Bone Neoplasms↗

Nearly total absence of pulmonary perfusion with corresponding technetium-99m MDP and gallium-67 uptake in a patient with mediastinal neuroblastoma.

A case of unilateral nearly total hypoperfusion of the left lung in a 13-month-old girl is presented. The combination of the lung hypoperfusion and accumulation of the Tc-99m MDP and Ga-67 citrate in the same area suggested the preoperative diagnosis of mediastinal neuroblastoma. Explorative thoracotomy revealed the presence of a neuroblastoma compressing the left lung pedicle. The described scintigraphic appearance in the pediatric age group is suggested as typical of mediastinal neuroblastoma. This pathology should be included in the following gamuts in nuclear medicine: unilateral decrease or absent lung perfusion, unilateral diffuse chest uptake of Ga-67 citrate, and unilateral pulmonary uptake in bone scintigraphy.

Female↗

Frontal and orbital bone infarctions causing periorbital swelling in patients with sickle cell anemia.

Two cases of unilateral and bilateral periorbital hematomas occurred in patients with sickle cell anemia. The cause of periorbital swelling in these cases was found to be orbital and frontal bone infarctions, respectively, diagnosed by technetium Tc 99m medronate bone scintigraphy. To our knowledge, periorbital bone infarction, as a part of the differential diagnosis of periorbital hematoma and as part of the possible ocular manifestations in patients with sickle cell anemia, has not previously been described.

Anemia, Sickle Cell↗

Bone infarction in children with sickle cell disease: early diagnosis and differentiation from osteomyelitis.

An early differential diagnosis between bone infarction and osteomyelitis in sickle cell patients is practically impossible using routine laboratory methods. Twenty radioisotope studies in sickle cell patients during vaso-occlusive crises, were analyzed. A three stage process can be described. In the first stage a decreased uptake can be demonstrated by Tc 99m methylene diphosphonate (MDP) bone scanning. In osteomyelitis, an increased uptake area is usually seen at this early stage, corresponding to increased uptake in Ga-67 citrate scanning. At the second stage, approximately a week later, normal uptake can be seen. Two to four weeks later an area of increased uptake is recorded that corresponds to the healing process, stage three. We recommend therefore Tc 99m MDP bone scanning in the early stages if clinical signs and symptoms suggest a vaso occlusive crisis or osteomyelitis in a sickle cell patient. This study can be followed by a Ga-67 citrate scintigraphy in doubtful cases. Later studies should be used for the assessment of the healing process. Two illustrative case reports are included.

Anemia, Sickle Cell↗