Banu Acar

Possible effect of subclinical inflammation on daily life in familial Mediterranean fever

This study was performed to investigate the attack-free complaints of patients with familial Mediterranean fever (FMF) and the impact of colchicine on these symptoms and on subclinical inflammation. A questionnaire that includes information about the disease course and symptoms during the attack-free period was administered to the parents of 50 FMF patients. For evaluation of the attack-free period, questions were asked about four items concerning daily activities of the children—weakness, lack of appetite, sleep problems, and decreased activity. The respondents rated the items and the total score was taken as the sum of all of the specific items. The laboratory values were noted from the patients’ files. During the attack-free period, patients with mild disease had higher total scores, higher weakness, and decreased activity scores than patients with moderate disease. When we compared the daily activity scores before and after colchicine therapy, a statistically significant increase was observed in the total scores and in all of the specific item scores. Also a significant decrease was seen in the erythrocyte sedimentation rate and white blood cell counts, and a significant increase was seen in the hemoglobin levels during the attack-free period after colchicine usage. Regression of inflammation together with improvement in daily activities were observed. FMF patients seem to have complaints during the attack-free period that may be related to subclinical inflammation. Moreover, colchicine besides preventing the FMF attacks and the dangerous complication ol amyloidosis also seems to hinder the symptoms of the attack-free period in children with FMF.

Hypertension induced reversible posterior leukoencephalopathy syndrome: a report of two cases.

Reversible posterior leukoencephalopathy syndrome (RPLS) is a recently described disorder with typical radiological findings of bilateral grey and white matter abnormalities in the posterior regions of the cerebral hemispheres. The majority of patients with RPLS are adults and it is rare in children. In this report, two patients with RPLS are presented. In the first patient the primary diagnosis was acute post-streptococcal glomerulonephritis, a known cause of RPLS both in adults and in children. The second patient had Henoch Schönlein purpura. Conclusion:These patients are presented to highlight the importance of reversible posterior leukoencephalopathy syndrome. As the spectrum of associated diseases is diverse, paediatricians must be aware of this syndrome in order to initiate appropriate management.

The value of the levels of acute phase reactants for the prediction of familial Mediterranean fever associated amyloidosis: a case control study

In order to determine the role of levels of acute phase proteins (APPs) for the development of amyloidosis in familial Mediterranean fever (FMF) patients, the levels of serum amyloid A (SAA), C reactive protein (CRP), fibrinogen and erythrocyte sedimentation rate were measured in paired sera of 36 FMF patients during and in between acute attacks, 39 of their healthy parents (obligate heterozgotes), and 15 patients with FMF associated amyloidosis. To compare the levels of APPs, 39 patients with chronic infections or inflammatory diseases who may develop secondary amyloidosis, 20 patients with acute infections who are known to have elevated acute phase response but will never develop amyloidosis and 19 healthy controls were included. The median levels of all APPs are increased in the patients with FMF during attacks and a significant decrease was observed after the attack was over. The level of SAA was above reference range in all FMF patients during the attack free period and the level of at least one other APP was also above normal in 64% of the patients. Both CRP and SAA levels were found to be higher in obligate heterozygotes compared to controls. The levels of SAA in patients with FMF during the attack-free period, obligate heterozygotes and patients with FMF-amyloidosis were found to be similar. The levels in each group were found to be higher than SAA levels found in healthy controls yet lower than the levels measured in the patients with acute infections and patients with chronic inflammation or chronic infections. In conclusion, our results show that SAA level reflects subclinical inflammation with high sensitivity but its value for the prediction of amyloid formation process seems to be low.

Polyarteritis nodosa: successful diagnostic imaging utilizing pulsed and color Doppler ultrasonography and computed tomography angiography

The diagnosis of polyarteritis nodosa (PAN) is often delayed because patients present with diverse clinical symptoms, but with less disease-specific signs. However, early diagnosis and treatment of PAN is necessary to prevent serious organ damage. Herein, we present a boy in whom the diagnosis of classical PAN was established rapidly with ultrasound and computed tomography (CT) angiography. Moreover, complete disappearance of the aneurysms after one month of therapy was documented. This case is presented in order to underline the role of new imaging techniques in the diagnosis and follow-up of patients with PAN.

Renal replacement therapies in pediatric intensive care patients: Experiences of one center in Turkey

Background: Despite constant improvements in caring for critically ill neonates and infants with congenital cardiac disease, sepsis, bone marrow and solid organ transplantation, acute renal failure (ARF) is an important problem in these children. ARF, severe fluid overload and inborn errors of metabolism are some of the indications for acute dialysis in infants and children.

Successful treatment of gastrointestinal involvement in Henoch-Schönlein purpura with plasmapheresis

Sir, We report on severe gastrointestinal bleeding that was successfully treated with four sessions of plasmapheresis. In a 13-year-old girl, Henoch-Schonlein purpura (HSP) was diagnosed 5 year earlier following a vaccination against hepatitis B [1]. This time, 2 days after hepatitis B revaccination, necrotizing purpura started on lower extremities and spread throughout the body, including the face. Urinary protein excretion was 4.2 mg/m/h, but serum creatinine was not elevated. A skin biopsy showed perivascular deposits of immunoglobulin A (IgA) when using direct immunofluorescence. After three pulses of methylprednisolone (30 mg/kg), 2 mg/kg prednisolone treatment was given because of necrotizing purpuric lesions. Although she was using steroids, in next 15 days, she twice had severe gastrointestinal bleeding. Her hemoglobin dropped from 14.3 to 10.2 g/dl. As she had repeated gastrointestinal bleedings 800 mg/m intravenous immunoglobulin was infused, but necrotizing purpura at the lower limbs and gastrointestinal hemorrhage recurred after 5 days of wellbeing. Treatment with 500 mg/m cyclophosphamide at day 25 of the disease did not stop gastrointestinal bleeding. Therefore, we started four sessions of plasmapheresis. A total of 800 ml of human plasma was exchanged per cycle on alternate days. Cutaneous lesions were resolved 48 h after the start of plasmapheresis, and no gastrointestinal bleeding was noted thereafter. After the plasmapheresis, low-dose steroid treatment was given in alternate-day cycle during the second month of the disease. Two months after discharge, she had no complaints, physical examination was normal, and laboratory parameters were in the normal range. In conclusion, we feel that plasma exchange might be an effective therapy in patients with HSP presenting severe symptoms, including severe gastrointestinal symptoms [2–4].

Wegener granulomatosis as an uncommon cause of panhypopituitarism in childhood.

Abstract Wegener granulomatosis (WG) is a cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA)-associated, multi-system, necrotizing granulomatous vasculitis. Inflammation of the nasal or oral mucosa, and lung and kidney involvements are typical in the course of the disease. In rare cases, pituitary involvement may occur and cause panhypopituitarism. Pituitary involvement is very rare, and only two pediatric case reports have been published to date out of a total of 24 cases. This is a case report of an adolescent patient who presented with panhypopituitarism symptoms and was later diagnosed with WG. A 16-year-old female patient complained of fever, headache, purulent nasal discharge and severe muscle and joint pain. Additionally, she had polyuria and polydipsia. Investigations revealed a pituitary mass and panhypopituitarism. Positivity of c-ANCA and renal biopsy result compatible with WG confirmed the diagnosis.

The association of cystic nephroma with pulmonary sequestration: is it a coincidence or not?

Cystic nephroma (CN) is a rare, presumably benign, multilocular cystic renal tumor. Pulmonary sequestration (PS) also presents as cystic masses of non-functioning primitive lung tissue. We describe a 15-month-old girl with CN and PS. Although some rare associations of renal and pulmonary lesions have been reported, this is the first case report in the English literature that shows the association of CN with PS.

Plasma exchange therapy for severe gastrointestinal involvement of Henoch Schonlein purpura in children

OBJECTIVES The aim of this report is to evaluate the plasma exchange as a choice for the management of life-threating gastrointestinal system (GIS) involvement in Henoch-Schönlein purpura (HSP) when refractory to conventional therapies. METHODS We retrospectively reviewed the medical records of HSP patients who had plasma exchange therapy due to massive GIS involvement. We reported age, gender, initial HSP presentation, etiological or triggering factors and disease course. Treatment modalities, side effects and their outcomes were noted. RESULTS We reported 7 cases of childhood HSP with severe gastrointestinal involvement refractory to common immunosuppression with systemic steroid and cytotoxic therapy. All patients gave inadequate response to pulse methyl prednisolone or oral prednisolone therapy with ongoing GIS bleeding and severe abdominal pain. Therefore, pulse cyclophosphamide was added to the treatment. Two patients received additional intravenous immunoglobulin (IVIG) therapy. Gastrointestinal manifestations continued and plasma exchange was performed. All patients improved after plasma exchange treatment. CONCLUSIONS Treatment of GI involvement in HSP with plasma exchange has been mainly based on case reports. According to our data, we propose that, plasma exchange may be a safe and efficient management choice in paediatric HSP patients with massive GIS involvement that are refractory to other therapies.

Multicystic dysplastic kidney and caliceal diverticulum in a child a coincidence or an association

Multicystic dysplastic kidney (MCDK) is a common developmental anomaly that has an incidence of approximately 1 in 4000 live births [1,2]. Caliceal diverticulum is a rare disorder in which a urine-filled cavity is connected to the renal calix by a narrow isthmus. This rare abnormality is usually asymptomatic and detected incidentally on routine intravenous pyelography (IVP), with an incidence of 4.5 per 1000 IVP [3]. This case is presented in order to take attention to the association of two cystic renal diseases, MCDK and caliceal diverticulum in a child and to emphasize a possible common pathogenetic mechanism.