Repeated blood transfusions in transfusion dependent thalassemia (TDT) leads to iron overload-related endocrine complications. Hypoparathyroidism (HPT) with severe signs of hypocalcemia is a recognized complication among these patients. A 14-year-old thalassaemic boy, on regular transfusion and on anticonvulsant therapy with a presumptive diagnosis of epilepsy for the last 1 year, was admitted with high fever and severe muscle cramps with positive Trousseau’s sign. He was diagnosed as a case of primary HPT and magnesium deficiency on the basis of low serum calcium, high phosphate, normal alkaline phosphates, very low intact parathyroid hormone (iPTH), normal serum vitamin D and very low serum magnesium level. His calcium, magnesium and phosphate level normalised following treatment with intravenous magnesium and calcium. His iPTH improved but remained at low normal. He was discharged from hospital with oral calcium, calcitriol, and magnesium supplementation. The anticonvulsant (Phenobarbitone) was successfully withdrawn gradually over the next six months without any recurrence of seizure in the subsequent 3 years of follow up. Acquired HPT (apparently from hemosiderosis) is a common cause of hypocalcemia; and magnesium depletion further complicated the situation leading to severe hypocalcemia with recurrent episodes of convulsion. Magnesium replacement improved the parathyroid hormone (PTH) value proving its role in acquired HPT. Very high phosphate level on admission and poor PTH response with respect to the low serum calcium, indicates intrinsic parathyroid pathology. Metabolic abnormalities should always be evaluated in thalassaemic subject with seizure disorder and it appears that the initial convulsive episodes were due to hypocalcemia. Muscle pain, cramps or convulsion may occur from HPT and simultaneous magnesium deficiency in transfusion dependent thalassaemic subjects. Metabolic correction is more important than anticonvulsant medication. Calcium and magnesium should both be assessed routinely in transfusion dependent thalassemic patients.
Hemosiderosis ; Hypoparathyroidism ; Thalassemia

Hemosiderosis is characterized by the deposition of excess iron in body tissues. The choroid plexus is an important part of the central nervous system that can be the primary site of iron overload. T2*-weighted gradient echo (GRE) sequence provides high sensitivity for demonstrating cerebral microhemorrhagic foci and iron deposition. In the present study, we describe the case of a 15-year-old boy with acute lymphoblastic leukemia, in whom repeated transfusion led to iron accumulation in the brain. GRE sequence effectively demonstrated hemosiderin deposition in the choroid plexus.
Adolescent ; Brain ; Central Nervous System ; Choroid Plexus ; Choroid ; Hemosiderin ; Hemosiderosis ; Humans ; Iron Overload ; Iron ; Magnetic Resonance Imaging ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma

Background and Objectives: Iron is an essential element that plays a vital role in a wide variety of cellular processes. But when present in excess concentration in organs, it may increase the risk for liver disease, heart failure, and diabetes. Recently, siderophores, which are iron-chelating agents produced by microorganisms, have attracted tremendous attention because of their strong binding and high selectivity to the ferric form of iron. Thus, the use of siderophore in sequestering excess iron in the body as a form of therapy is very attractive. This study determined the effects of commercially available siderophore in sequestering excess iron in organs such as liver, heart, and pancreas under excess iron conditions. Methodology: First, iron-overload was induced by injecting iron dextran (20 mg) into male ICR mice for three consecutive days. The effects of iron to the liver, heart, and pancreas and the possible sequestration by siderophore were determined by scoring histological sections. The liver iron concentration was also assessed by atomic absorption spectroscopy (AAS). Results and Conclusion The study showed that iron-overloaded mice exhibited skin hyperpigmentation and hemosiderosis in liver, heart, and pancreas. Significant changes in the liver include hepatomegaly and development of tumor. Iron-overloaded mice had 2,935% increase in liver iron content compared to the salinetreated mice. However, when iron-overloaded mice were treated with either 100 µg or 200 µg siderophore, there was a 77% and 84% decrease in liver iron content, respectively. Moreover, the treatment of ironoverloaded mice with siderophore prevented the development of hemosiderosis, tumor, and structural changes in the tissues studied. The results showed that siderophore can effectively reduce excess iron and organ damage in iron-overloaded mice and can be potentially employed in chelation therapy of iron-overload diseases. Further studies on the possible mechanisms of siderophore aside from decreasing iron excess and lowering organ dysfunction are recommended.
Siderophores ; Iron Overload ; Iron Chelating Agents ; Hemosiderosis ; Hepatomegaly

This article reports 4 girls with clinical manifestations of recurrent cough and anemia. The age of onset was less than 4 years, and three of them had shortness of breath. None of them had acute hemoptysis. All the girls had positive results of hemosiderin test for bronchoalveolar lavage fluid. As for imaging examination, 3 patients had ground-glass opacity, and 1 had interstitial change. Three girls were given the treatment for idiopathic pulmonary hemosiderosis and had no response. Selective bronchial arteriography was performed for the 4 girls and found bronchial artery to pulmonary circulation shunt (BPS). After they were diagnosed with BPS, they were given transcatheter embolization. The girls were followed up for half a year after surgery, and none of them was readmitted due to "cough and anemia". BPS manifests as abnormal shunt between the bronchial artery and the pulmonary artery/vein and has unknown causes. It is rare in children and should be considered for children who were thought to have idiopathic pulmonary hemosiderosis and had poor response to corticosteroid therapy.
Anemia ; etiology ; Bronchial Arteries ; Child ; Child, Preschool ; Embolization, Therapeutic ; Female ; Hemorrhage ; complications ; Hemosiderosis ; complications ; Humans ; Lung Diseases ; complications ; Pulmonary Alveoli ; Pulmonary Circulation

OBJECTIVETo investigate the etiology and clinical manifestation of hemoptysis in children.

METHODA retrospective analysis was performed for 106 cases of hemoptysis who were admitted to The Second Affiliated Hospital & Yuying Children's Hospital of Wenzhou Medical University from January 2005 to December 2014.The clinical information including laboratory tests and image data were collected and analyzed.

RESULTA total of 106 patients (50 males and 56 females) were identified. The median age was 9.1 years (range 2 months to 18 years). Pneumonia (35, 31.1%) was the most common etiology of hemoptysis, which included bacterial pneumonia (27 cases), mycoplasmal pneumonia(4 cases), chlamydial pneumonia (3 cases), and influenza pneumonia(1 case). Other causes included bronchitis(15, 14.2%), pulmonary tuberculosis (11, 10.4%), bronchiectasis (11, 10.4%), diffuse alveolar hemorrhage (8, 7.5%), idiopathic pulmonary hemosiderosis(6, 5.7%), cardiovascular dysplasia(6, 5.7%), pulmonary contusion (4, 3.8%), foreign body in bronchus (2, 1.9%), allergic bronchopulmonary aspergillosis (2, 1.9%). Eighty-six patients manifested mild hemoptysis; moderate and massive hemoptysis were found in nine and eleven patients, respectively. Pneumonia accounted for 33.7% of mild hemoptysis and 45.5% of massive hemoptysis were due to bronchiectasis; 80.2% were treated with antibiotics and 41.5% were given hemostatic agents; 8.5% received lobectomy. Ninety-six patients (90.6%) were cured and parents gave up treatment in 4 cases (3.8%). Six patients (5.7%) suffered from recurrent hemoptysis.

CONCLUSIONHemoptysis mainly occurred in children who were older than 6 years, the most common cause of hemoptysis was respiratory tract infection. In most cases, the amount of hemoptysis was small and the overall prognosis was good.

Adolescent ; Bronchiectasis ; complications ; Bronchitis ; complications ; Child ; Child, Preschool ; Female ; Foreign Bodies ; complications ; Hemoptysis ; diagnosis ; etiology ; therapy ; Hemosiderosis ; complications ; Humans ; Infant ; Influenza, Human ; complications ; Lung Diseases ; complications ; Lung Injury ; complications ; Male ; Pneumonia, Bacterial ; complications ; Prognosis ; Retrospective Studies ; Tuberculosis, Pulmonary ; complications

PURPOSE: Studies on hemoptysis is rare because hemoptysis is an uncommon symptom in children. The aim of this study was to identify the causes of hemoptysis in children. METHODS: Medical chart review of patients with hemoptysis was retrospectively conducted at 2 tertiary hospitals from November 2008 to December 2014. Patients were divided into 3 groups according to age. The amount of hemoptysis was categorized as mild (<20 mL/day), moderate (20–99 mL/day), and massive (≥100 mL/day). RESULTS: A total of 59 patients were identified, and their mean age was 11.0±5.6 years. Among the causes of hemoptysis, respiratory tract infection was most common. Other causes included vasculitis syndrome, neoplasm in the airway, idiopathic pulmonary hemosiderosis, cardiac disease, and bronchiectasis. According to age, a significant difference was identified between the age groups in children with pneumonia (<6 years vs. 12–18 years, P=0.001). Differences were verified between the age groups in children with tuberculosis (<6 years vs. 12–18 years and 6–11 years vs. 12–18 years, P=0.023). According to amounts of hemoptysis, no significant difference was identified regardless of the causes. CONCLUSION: This study showed that the causes of hemoptysis in children were heterogeneous and the respiratory tract infection was most common. In children with hemoptysis, the age of onset and the amount of hemoptysis are needed to be considered for more precise diagnosis and more proper management of the underlying cause of hemoptysis.
Age of Onset ; Bronchiectasis ; Child* ; Diagnosis ; Heart Diseases ; Hemoptysis* ; Hemosiderosis ; Humans ; Pneumonia ; Respiratory Tract Infections ; Retrospective Studies ; Tertiary Care Centers ; Tuberculosis ; Vasculitis

Idiopathic pulmonary hemosiderosis (IPH) is a rare respiratory disease with an unknown etiology, and is diagnosed with laboratory, radiology, and pathology tests. Chief complaints of IPH include hemoptysis, cough, and dyspnea. Since it is considered an immune-mediated disease, the first line of treatment is systemic corticosteroid therapy. The three cases reported here showed a decrease in ferritin level and improvement in the hemoglobin level with prednisolone treatment. However, long-term corticosteroid therapy may cause several side effects, particularly growth retardation and obesity, which can affect growing children. In the present study, all patients had cushingoid symptoms and obesity. Therefore, we switched to deflazacort (DFZ), which has lesser side-effects of weight gain. This report describes clinical courses of the disease and comparison of body mass index of three patients with IPH who took DFZ instead of prednisolone. DFZ was effective for IPH, and is useful for weight gain reduction.
Body Mass Index ; Child ; Cough ; Dyspnea ; Ferritins ; Hemoptysis ; Hemosiderosis ; Humans ; Obesity ; Pathology ; Prednisolone ; Weight Gain

Pulmonary siderosis, or Welder's lung disease is an occupational lung disease caused by iron-inhalation. Diagnosis of pulmonary siderosis is based on occupational history of the patient, radiologic findings, and pathologic findings of iron-laden macrophages within lung tissue or broncho-alveolar lavage fluid. We observed a case of a 43 years-old welder diagnosed with pulmonary siderosis via thoracoscopic lung biopsy. Sputum culture along with pathology also identified a non-tuberculotic mycobacterial infection with a sputum culture and the pathologic findings. The patient was treated with anti-tubercular medication and cessation of iron-exposure. And his condition improved within a few months.
Biopsy ; Hemosiderosis ; Humans ; Lung ; Lung Diseases ; Macrophages ; Mycobacterium Infections, Nontuberculous ; Pneumoconiosis ; Siderosis ; Sputum ; Therapeutic Irrigation ; Thoracic Surgery, Video-Assisted

A 29-month-old boy presented with fever, dyspnea, and paleness. He was initially diagnosed with pneumonia and severe sepsis. Although he was treated with intravenous antibiotics and high dose methylprednisolone, dyspnea and paleness recurred two times. Under suspicion of pulmonary hemosiderosis, we performed video-assisted thoracoscopic lung biopsy and bronchoalveolar lavage on him and found hemosiderin-laden macrophages in both specimens. Despite thorough history and laboratory examination, we could not find any pathologic or serologic evidence for primary and secondary causes of pulmonary hemosiderosis except for one that indicating Heiner's syndrome. After taking oral prednisolone he showed improvement of anemia and dyspnea, which was maintained by milk avoidance. Based on the history and the existence of immunoglobulin G antibodies against milk components, we are considering it as the case of Heiner's syndrome.
Anemia ; Anti-Bacterial Agents ; Antibodies ; Biopsy ; Bronchoalveolar Lavage ; Dyspnea ; Fever ; Hemosiderosis ; Immunoglobulin G ; Lung ; Lung Diseases ; Macrophages ; Methylprednisolone ; Milk ; Milk Hypersensitivity ; Pneumonia ; Prednisolone ; Sepsis

Adult ; Arsenic Poisoning ; pathology ; Chronic Disease ; Hemosiderin ; metabolism ; Hemosiderosis ; metabolism ; pathology ; Humans ; Hypertension, Portal ; chemically induced ; metabolism ; pathology ; Liver Cirrhosis ; chemically induced ; metabolism ; pathology ; Male ; Pancytopenia ; chemically induced ; metabolism ; pathology ; Splenomegaly ; chemically induced ; metabolism ; pathology