Ismail Islek

Intravenous Immunoglobulin Treatment in Children with Guillain-Barre Syndrome

Guillain-Barre syndrome is an acquired demyelinating polyneuropathy that is presumed to be immune-mediated. On the basis of this assumption, intravenous immunoglobulin (IVIG) has been used in the treatment of Guillain-Barre syndrome in recent years and found to be effective. To test this we performed a randomized study in patients with Guillain-Barre syndrome by giving IVIG (1 g/kg body weight per day over 2 consecutive days) in 9 children who were compared with 9 patients who were observed but not given specific therapy. We concluded that intravenous immunoglobulin is a safe and effective treatment for childhood Guillain-Barre syndrome which shortens the time to recovery.

Acute immune thrombocytopenic purpura: A comparative study of very high oral doses of methylprednisolone and intravenously administered immune globulin☆☆☆★

We compared very high doses of methylprednisolone with intravenously administered immune globulin for treatment of acute idiopathic thrombocytopenic purpura. Fifty-seven children were randomly assigned to receive the immune globulin preparation, 0.5 gm/kg per day for 5 consecutive days (n = 19), orally administered methylprednisolone, 30 mg/kg per day for 7 days (n = 19), or orally administered methylprednisolone, 50 mg/kg per day for 7 days (n = 19). There were no differences in the response of the platelet counts among the groups. We conclude that these two therapies were equally effective; choice between them may be made according to cost and therapy-related risks.

Reversible nephrotoxicity after overdose of colloidal bismuth subcitrate.

Abstract Although toxicity due to acute and chronic use of bismuth salts is well known, nephrotoxicity after ingestion of colloidal bismuth has been reported in few cases so far. Here we report the first case of acute renal failure (ARF) due to colloidal bismuth subcitrate overdosage in childhood. A 2-year-old boy was admitted to the hospital 6 h after ingestion of 28 De-Nol tablets (colloidal bismuth subcitrate 8.4 g). On admission, physical examination was unremarkable and he showed no signs of encephalopathy. Initially gastric lavage was performed then appropriate fluid therapy was started. ARF associated with uremia and oliguria developed on day 2 and peritoneal dialysis therapy was prescribed on day 4 for 10 days. Blood and urine bismuth levels were 739 µg/l and 693 µg/l, respectively, 10 days after the pills had been taken. His urine volume gradually increased and plasma BUN and creatinine levels decreased during peritoneal dialysis. On day 20 post-admission, plasma BUN and creatinine were 14 mg/dl and 0.7 mg/dl, respectively. Blood bismuth levels were 96 µg/l on day 60 and 12 µg/l on day 105. Now the patient is well and has no problem. This case suggests that ARF may develop in children following colloidal bismuth subcitrate overdosage; the prognosis is good, and peritoneal dialysis may be useful in these cases.

Endothelin levels in Henoch-Schonlein purpura

Abstract. Henoch-Schonlein purpura (HSP) is one of the most common types of vasculitis disorders seen in childhood and is characterized by a rash, arthritis, abdominal pain, and renal involvement. Although HSP is an immunoglobulin A (IgA) related immune complex disease, the pathogenesis has not been fully elucidated. Cytokines have been implicated in the pathogenesis, but endothelins (ET) – vasoconstrictor hormones produced by endothelial cells – have not been studied in patients with HSP. In a controlled study, we measured ET-1 levels in children with HSP during the acute and remission phases. ET-1 levels were significantly higher in the HSP patients during the acute phase compared with the control group and the HSP patients in the remission phase. There was no correlation between ET-1 levels and disease severity, acute phase reactant response, or morbidity. The role of endothelins and other cytokines in the pathogenesis of HSP needs to be further explored.

Adrenomedullin and nitrite levels in children with Bartter syndrome

Abstract Children with Bartter syndrome have lower than normal vascular reactivity with normotension in spite of biochemical and hormonal abnormalities which are typical of hypertension. Nitric oxide (NO) is a potent endogenous vasodilator, and plays an important role in the control of vascular tone. Adrenomedullin (AM) is a novel hypotensive peptide originally isolated from human pheochromocytoma. The possible role of NO and AM in maintaining this reduced vascular reactivity was examined by studying plasma and urinary nitrite, a stable metabolite of NO, and AM levels in ten children with Bartter syndrome, ten healthy controls, and five children with hypokalemia of causes other than Bartter syndrome (pseudo-Bartter). Urinary excretion of nitrite (µmol/mg urinary creatinine) was 8.9.±1.2 in children with Bartter syndrome, 4.7.±0.9 in healthy controls, and 2.9.±0.8 in pseudo-Bartter (P<0.05). Plasma nitrite levels (µmol/l) were 101.9±23.4, 59.9±14.7, and 65.0±29.7, respectively (P>0.05), in the three groups. Urinary excretion of AM (pmol/mg urinary creatinine) was 187±40, 65±10, and 160±50, respectively (P<0.05), in the three groups. Plasma AM levels were 47.4±1.8, 39.9±5.9, and 42.4±3.9, respectively (P>0.05), in the three groups. The same parameters were repeated in the two groups of controls and in the Bartter patients in the 6th month of therapy. Urinary nitrite and AM levels were still higher in the Bartter patients than in the other groups. We conclude that in Bartter syndrome the increased NO production may be responsible for the reduced vascular response of the disease. Initially, increased levels of AM in Bartter syndrome and pseudo-Bartter may be a compensatory response to acute hypokalemia; however, continuation of a high level of urinary excretion of AM in children with Bartter syndrome may suggest also the possible role of AM in the reduced vascular response of the disease.

Henoch–Schönlein purpura associated with hepatitis A infection

common vasculitis conditions of childhood, and is characterized by nonthrombocytopenic purpura, arthritis and/or arthralgia, abdominal pain, gastrointestinal hemorrhage and renal involvement. Although the etiology of HSP is still unknown, it is considered to be an immune-mediated vasculitic disorder resulting from an immune complex reaction to various antigenic stimuli. Many bacterial and viral organisms such as streptococci, adenovirus, parvovirus, hepatitis B virus (HBV), Epstein–Barr virus, varicella and mycoplasma have been reported as predisposing factors for HSP.1 So far, HSP associated with hepatitis A virus (HAV) infection has only been reported in one other patient, as a letter to the editor.2 In the present report we describe two patients with HSP associated with acute hepatitis A.

Henoch-Schonlein purpura and pulmonary tuberculosis

Henoch–Schonlein purpura (HSP) is one of the most common vasculitic disorders of childhood and is characterized by non-thrombocytopenic purpura, arthritis and/or arthralgia, abdominal pain, gastrointestinal hemorrhage and renal involvement. The cause of the disease still remains obscure, although there is often an antecedent respiratory infection. Many bacterial and viral organisms including streptococci, adenovirus, parvovirus, Epstein–Barr virus (EBV), varicella and mycoplasma have been reported as preceding factors for HSP.1,2 A review of published reports found that HSP associated with tuberculosis has been rarely reported.3–9 Here we present an unusual child patient with HSP associated with pulmonary tuberculosis. Such an association in childhood has not been reported so far.

Adrenomedullin and total nitrite levels in children with Henoch-Schönlein purpura

Nitric oxide (NO) is synthesized from endothelium and has an important role in the control of vascular tonus. Adrenomedullin (AM) is a potent vasodilator, and cytoprotective peptide is produced not only in adrenal medulla, but also in the vascular smooth muscle and endothelial cells. To investigate the endothelial synthesis of AM and NO, and endothelial injury in Henoch-Schönlein purpura (HSP), we measured their levels in 16 children with HSP, who were evaluated during the acute and remission phases, and compared with 12 healthy controls. Plasma AM levels (pmol/ml) were significantly higher in acute phase children (46.87±11.49) than in those in remission (35.59±12.39, p<0.01) and controls (30.70±9.12, p<0.001). Similarly, plasma total nitrite levels (μmol/l) were higher in acute phase patients (47.50±12.30) than in those in remission (35.94±10.08, p<0.005) and controls (34.56±11.51, p<0.05). Urinary excretion of AM (pmol/mg creatinine) was higher in acute phase patients (53.85±23.22) than in remission patients (29.97±9.33, p<0.01) and controls (37.43±15.78, p<0.05). Patients had increased urinary nitrite excretion (μmol/mg creatinine) in acute phase (2.39±1.18) compared to those in remission (1.53±0.90, p<0.05) and controls (1.05±0.61, p<0.005). There was no significant difference between remission phase and controls in AM and nitrite levels (p>0.05). This study concluded that AM and NO may have a role in the immunoinflammatory process of HSP, especially in the active stage, although whether this perpetuates, or protects against, further vascular injury is not clear. Further studies are needed to clearly establish the roles of AM and NO in the pathogenesis of HSP.

Hypersensitivity vasculitis induced by cefoperazone/sulbactam

BackgroundCefoperazone has not been reported to cause vasculitic complications before. Here, we report a case of hypersensitivity vasculitis associated with cefoperazone/sulbactam.Case presentationA 13-year-old girl with appendicitis developed hypersensitivity vasculitis on the fifth day of cefoperazone/sulbactam therapy. Hypersensitivity vasculitis resolved gradually after removal of the agent on the seventh day and did not recur. Although hypersensitivity vasculitis has multiple causes, coexistence of hypersensitivity vasculitis and cefoperazone treatment, and the quite resolution of the disease after removal of the drug, strongly favours a causative relationship.ConclusionTo our knowledge, this is the first report of a hypersensitivity vasculitis associated with cefoperazone.

Adrenomedullin and total nitrite levels in children with familial Mediterranean fever

Aim:  Familial Mediterranean fever (FMF) is the most frequent periodic syndrome characterised by recurrent attacks of polyserositis. However, recent studies revealed that there might be an ongoing subclinical inflammation between the attacks. As nitric oxide (NO) and adrenomedullin (AM) are both synthesised in the endothelium, and mediates many functions within immune system, we considered them to be an interesting target of investigation in FMF.