Sebnem Yilmaz

Disseminated intravascular coagulation in pediatric patients: clinical and laboratory features and prognostic factors influencing the survival.

Although disseminated intravascular coagulation (DIC) has been a well-known disorder for many years, there is lack of sufficient number of clinical trials about incidence, frequency of underlying disorders, and prognosis of DIC in children. The aim of this study was to evaluate the frequency, etiologic factors, and clinical and laboratory findings of DIC and to determine the prognostic factors influencing the mortality in hospitalized pediatric patients. Medical records of 5535 children who were hospitalized were investigated. Sixty-two patients who were diagnosed as acute DIC were enrolled. The frequency of DIC was 1.12%. The underlying etiologic factors were infection in 59 patients (95.2%) and major trauma in 3 patients (4.8%). The frequency of bleeding and thrombosis was 48.8 and 4.8%. Respiratory, cardiovascular, hepatic, renal, neurologic, and gastrointestinal dysfunction was present in 71, 67.7, 35.5, 16.1, 16.1 and 11.3% of patients, respectively. Respiratory and cardiovascular dysfunctions were significantly associated with mortality. Multiorgan dysfunction syndrome (MODS) was present in 85.5% of the patients, and 54.8% of the patients had developed acute respiratory distress syndrome (ARDS). Mortality rate was significantly high in patients with MODS and ARDS. In multivariete logistic regression analysis, only ARDS and cardiovascular dysfunction had predictive and prognostic value on mortality. None of the diagnostic laboratory tests had predictive or prognostic value and the degree of abnormality of these tests did not show any correlation with mortality. In conclusion, DIC is not a rare disorder in hospitalized children, especially in patients with sepsis, and MODS, ARDS, and respiratory and cardiovascular system dysfunctions are poor prognostic factors.

Acrodermatitis enteropathica with Pseudomonas aeruginosa sepsis

Abstract: Acrodermatitis enteropathica is characterized by eczematous and scaly plaques on the face, scalp, acral, and anogenital regions. In addition to typical lesions, unusual prominent vesiculobullous lesions are also described. We report a full‐term, 9‐month‐old boy who has acrodermatitis enteropathica and Pseudomonas sepsis. In this patient there were clinical findings of sepsis and eczematous vesiculobullous lesions on the periorificial and acral areas. Serum zinc level was extremely low. Pseudomonas aeruginosa was identified in cultures of blood and fluid which was aspirated from the bullous lesions. After oral zinc sulfate and intravenous antibiotic treatment his condition improved within 2 weeks.

Serum leptin concentrations in children with mild protein-energy malnutrition and catch-up growth.

Abstract Background : The aim of the present study was to clarify the relationship between changing nutritional anthropometric data and serum leptin concentrations during the catch‐up growth process in children.

Serum leptin concentrations in children with mild-to-moderate protein-energy malnutrition

Background : The aim of the present study was to clarify the relationship between nutritional anthropometric parameters and serum leptin concentrations in otherwise healthy children with mild‐to‐moderate protein‐energy malnutrition (PEM) secondary to inadequate energy intake.

The changing face of celiac disease: a girl with obesity and celiac disease.

The widespread use of serological tests has changed the clinical spectrum of recently diagnosed celiac patients. In this paper, we present an obese patient with Hashimoto’s thyroiditis who was investigated for hypochromic microcytic anaemia that is unresponsive to iron replacement therapy and finally had the diagnosis of celiac disease. A 7-year-old girl was admitted to our hospital with the complaint of being overweight for 3 years. Her medical history was unremarkable except for consuming high energy-containing foods and sedentary life-style. Her mother was taking treatment for Hashimoto’s disease. On physical examination, her weight was 42.5 kg (>95 percentile), height 126.8 cm (75–90 percentile), weight for height 167%. She had an obese appearance but no signs of stria, hepatomegaly or acanthosis nigricans. Laboratory results showed: haemoglobin: 11.2 g/dL, hematocrit: 34.8%, MCV: Mean corpuscular volume 69 fL, RDW: Red cell distribution width 18, leukocyte: 9800/mm, thrombocyte: 361 000/ mm, fasting blood glucose: 85 mg/dL, aspartate aminotransferase: 18 U/L, alanine aminotransferase: 30 U/L, triglyceride: 150 mg/ dL, total cholesterol: 233 mg/dL, LDL-cholesterol: Low density lipoprotein 158 mg/dL, free T3: 4.11 (2–4.1 pg/mL), free T4: 0.91 (0.8–2.2 ng/dL), thyroid stimulating hormone: 56.3 (0.6– 5.5 U/mL), anti-T: 621 IU/mL, anti-G: 92.9 IU/mL, in abdominal ultrasonography there was no sign of fatty liver. Thyroid ultrasonography revealed fibrous septa in both thyroid glands consistent with autoimmune thyroiditis. She was started thyroid replacement therapy for Hashimoto’s disease and also diet and exercise for obesity. At the first year of follow-up, her weight for height was 152%. She was found to have a persistent iron-deficiency anaemia that is not responsive to iron supplements (Table 1). She did not have bleeding history and stool examination was negative for occult blood. Haemoglobin electrophoresis was normal. An antibody screening test resulted positive for anti-endomysium IgA and anti-gliadin IgG and IgA antibodies. Serum IgA level was within the normal ranges (212 mg/dL (N: 33–226)). Her parent’s screening tests for celiac disease were negative. Endoscopic duodenal biopsy showed villous atrophy, crypt hyperplasia and lymphocyte proliferation consistent with the celiac disease. She was started a gluten-free diet and encouraged to consume iron-rich foods. After 4 months of treatment with gluten-free diet, all her haematological parameters returned to normal values. There are few published data in the literature about the co-existence of obesity and celiac disease. Classically in a disease where there is a disorder of absorption, the expected clinical condition is malnutrition rather than obesity. Co-existence of obesity and celiac disease is explained with a compensation theory. According to this theory, nutrients that cannot be absorbed because of villous atrophy in proximal small bowel is taken into body with increased absorption in distal small bowel and as a result, these patients do not lose weight. The patients reported with obesity and celiac disease presented with different clinical signs in the literature. Franzese et al. determined hepatosteatosis in an obese patient. This patient was evaluated for persistent liver disease despite the hypocaloric-diet and celiac disease was diagnosed. Oso and Fraser detected celiac disease in an obese adolescent patient who had recurrent diarrhoea attacks especially after eating spaghetti. Another published obese celiac case is a 5-year-old girl with short stature and recurrent abdominal pain. Our patient was presented with obesity and refractory irondeficiency anaemia. Anaemia can be seen in obese patients. Bekri et al. reported that 24% of morbid obese patients presented with anaemia. Similarly, in childhood series, anaemia frequency was found to be higher in obese children than normal children. The possible causes of this situation are early menarche in girls and on the contrary of assumed; the fact that obese patients consume inadequate iron in their diet. Our patient was taking adequate iron through her diet and supplementation. In celiac disease patients who have obesity, anaemia is a frequent finding. Four adult morbid obese patients who had the diagnosis of celiac disease were reported. Two of these patients had diarrhoea and one of them had gastroesophageal reflux complaints. At admission, iron deficiency was determined in two patients while vitamin B12 deficiency was determined in one. The prevalence of celiac disease in adults with Hashimoto’s disease was 5%. Similarly, the frequency of autoimmune thyroiditis is increased in celiac patients compared with normal population. But in childhood period, this togetherness of two diseases was reported very rarely. Our patient was admitted to our clinic for obesity and found to have autoimmune thyroiditis after investigations. She was screened for celiac disease in the follow-up period because of iron-deficiency anaemia. In conclusion, patients who have symptoms and signs suggesting celiac disease should be screened for celiac disease without considering their body weight.

Asymptomatic intracranial sewing needle: An unsuccessful infanticide attempt?

Infanticide attempts may appear in various clinical scenarios. Although very rarely reported, insertion of foreign objects through the cranial sutures may sometimes be used as a method of infanticide. In the present report, a 10-year-old orphaned girl who was brought to the emergency department because of minor head trauma and diagnosed to have a sewing needle in her brain parenchyma is investigated.

A pediatric case of lymphomatoid granulomatosis with onset after completion of chemotherapy for acute myeloid leukemia

In this case report, we present a pediatric case of lymphomatoid granulomatosis (LG) with onset just after the completion of chemotherapy for childhood acute myeloid leukemia (AML). After the completion of maintenance therapy, the patient was admitted to our clinic with a complaint of cough. Radiologic examinations revealed nodular lesions in lungs, liver, and kidney. His bone marrow was in remission. The histopathologic examination of the open lung biopsy was consistent with LG. He received only one cycle of cyclophosphamide and high-dose methyl prednisolone treatment and continued to receive interferon (IFN) alpha-2b therapy for 18 months. This treatment regimen resulted in an excellent response. In conclusion, LG may occur after the treatment of pediatric AML as a rare complication and IFN alpha-2b may be an effective treatment choice in these patients.

Chorioangiomatosis Presenting with Severe Anemia and Heart Failure in a Newborn

Although chorioangiomas are the most common placental tumors, multiple chorioangiomas (or chorioangiomatosis) are extremely rare. We report a female newborn at 37 weeks of gestation presenting with severe anemia, thrombocytopenia, heart failure, and intrauterine growth retardation. The pathological examination of the placenta revealed chorioangiomatosis explaining the pathophysiology of the symptoms presented. The patient was treated successfully with erythrocyte and thrombocyte transfusions and was discharged after full recovery. We conclude that evaluating placental lesions may be of great value in explaining severe problems during the fetal and the neonatal period and thus prevents performing unnecessary clinical investigations.

Sigmoid sinus thrombosis following mastoiditis: early diagnosis enhances good prognosis.

Abstract: Sigmoid sinus thrombosis following mastoiditis is a rare, but potentially life-threatening, condition. Its treatment usually consists of systemic antibiotics and mastoidectomy. In this report, we describe a pediatric case of sigmoid sinus thrombosis following mastoiditis, presenting with nonspecific symptoms such as fever, otalgia, and headache. Diagnosis was based on magnetic resonance imaging. The patient responded very well to intravenous antibiotics with a rapid clinical improvement and complete recanalization of the thrombosed sigmoid sinus. In conclusion, mastoiditis may present few clinical symptoms. In case of treatment failure or new-onset neurologic deficit in children with acute otitis media, life-threatening complications associated with mastoiditis should be considered. Early diagnosis is important, as favorable prognosis can be achieved with conservative management without performing any surgical intervention.

Neutrophil Apoptosis in Patients with β-Thalassemia Major

Increased susceptibility to infection is reported in patients with g -thalassemia major due to toxic effect of iron on neutrophil functions and reticuloendothelial system dysfunction. This study investigated the association between the neutrophil apoptosis and frequency of infection episodes, desferrioxamine treatment, and serum ferritin levels in patients with g -thalassemia major. A total of 35 children diagnosed with g -thalassemia major were enrolled. Group 1 consisted patients who were receiving desferrioxamine (DFO) and group 2 consisted of patients who did not start to receive DFO. A total of 15 healthy children were enrolled to serve as a control group. Frequency of infection episodes within a year was noted from hospital records. In all patients, the same method based on flow cytometry (annexin V labeled with FITC) was used to assess neutrophil apoptosis. Neutrophil count and percentage of apoptotic neutrophils did not differ significantly between the groups. When frequency of infection episodes among groups was evaluated, frequency of infection episodes of the patients who were receiving DFO was significantly higher than in the other groups. When correlation between neutrophil apoptosis and frequency of infection episodes, serum ferritin levels, and neutrophil count of the patients was analyzed according to groups, no significant correlation was found. The results indicate that high serum ferritin level and DFO use in patients with g -thalassemia major do not enhance neutrophil apotosis in vivo and enhanced neutrophil apoptosis cannot be a possible cause for increased susceptibility to infections in these patients.