Characteristics and predictors of chronic kidney disease in children with myelomeningocele: a nationwide cohort study.

BACKGROUND: Myelomeningocele (MMC) is highly prevalent in developing countries, and MMC-related neurogenic bladder is an important cause of childhood chronic kidney disease (CKD). This nationwide study aimed to evaluate demographic and clinical features of pediatric patients with MMC in Turkey and risk factors associated with CKD stage 5. METHODS: Data from children aged 0-19 years old, living with MMC in 2022, were retrospectively collected from 27 pediatric nephrology centers. Patients > 1 year of age without pre-existing kidney abnormalities were divided into five groups according to eGFR; CKD stages 1-5. Patients on dialysis, kidney transplant recipients, and those with eGFR < 15 ml/min/1.73 m2 but not on kidney replacement therapy at time of study constituted the CKD stage 5 group. RESULTS: A total of 911 (57.8% female) patients were enrolled, most of whom were expectantly managed. Stages 1-4 CKD were found in 34.3%, 4.2%, 4.1%, and 2.4%, respectively. CKD stage 5 was observed in 5.3% of patients at median 13 years old (range 2-18 years). Current age, age at first abnormal DMSA scan, moderate-to-severe trabeculated bladder on US and/or VCUG, and VUR history were independent risk factors for development of CKD stage 5 (OR 0.752; 95%; CI 0.658-0.859; p < 0.001; OR 1.187; 95% CI 1.031-1.367; p = 0.017; OR 10.031; 95% CI 2.210-45.544; p = 0.003; OR 2.722; 95% CI 1.215-6.102; p = 0.015, respectively). Only eight CKD stage 5 patients underwent surgery related to a hostile bladder between 1 and 15 years old. CONCLUSION: MMC-related CKD is common in childhood in Turkey. A proactive approach to neurogenic bladder management and early protective surgery in selected cases where conservative treatment has failed should be implemented to prevent progressive kidney failure in the pediatric MMC population in our country.

Rituximab treatment for difficult-to-treat nephrotic syndrome in children: a multicenter, retrospective study

Background/aim: This study aimed to evaluate the efficacy of rituximab in children with difficult-to-treat nephrotic syndrome, considering the type of disease (steroid-sensitive or ­resistant) and the dosing regimen. Materials and methods: This multicenter retrospective study enrolled children with difficult-to-treat nephrotic syndrome on rituximab treatment from 13 centers. The patients were classified based on low (single dose of 375 mg/m2) or high (2-4 doses of 375 mg/m2) initial dose of rituximab and the steroid response. Clinical outcomes were compared. Results: Data from 42 children [20 steroid-sensitive (frequent relapsing / steroid-dependent) and 22 steroid-resistant nephrotic syndrome, aged 1.9­17.3 years] were analyzed. Eleven patients with steroid-sensitive nephrotic syndrome (55%) had a relapse following initial rituximab therapy, with the mean time to first relapse of 8.4 ± 5.2 months. Complete remission was achieved in 41% and 36% of steroid-resistant patients, with the median remission time of 3.65 months. At Year 2, eight patients in steroid-sensitive group (40%) and four in steroid-resistant group (18%) were drug-free. Total cumulative doses of rituximab were higher in steroid-resistant group (p = 001). Relapse rates and time to first relapse in steroid-sensitive group or remission rates in steroid-resistant group did not differ between the low and high initial dose groups. Conclusion: The current study reveals that rituximab therapy may provide a lower relapse rate and prolonged relapse-free survival in the steroid-sensitive group, increased remission rates in the steroid-resistant group, and a significant number of drug-free patients in both groups. The optimal regimen for initial treatment and maintenance needs to be determined.

Report of a Non-Hodgkin lymphoma case presenting with pleural effusion.

Initial presentation of Non-Hodgkin Lymphoma (NHL) as primary pleural lymphoma is extremely rare. We report a case with NHL, who was initially presented with pleural effusion and pleural thickening. Our patients at first received intensive treatment of broad spectrum antibiotics for 10 days with no change in the clinical status, and then were diagnosed with T-lymphoblastic lymphoma with the examination of pleural fluid by using cytologic and flow cytometric methods, and treated with LMT89 group B treatment protocol. Our case points out the necessity for considering the NHL within the differential diagnosis of any pediatric patient presenting with sterile pleural effusion and pleural thickening who does not respond to antimicrobial therapy.

An analysis of children with brucellosis associated with pancytopenia.

Brucellosis produces a variety of nonspecific hematologic abnormalities. Hematologic complications of mild anemia and leukopenia have been frequently associated with acute brucellosis, but pancytopenia is less frequently seen. In this study, records of children with brucellosis aged under or equal to 16 years, admitted to Yuzuncu Yil University Hospital between 2004 and 2010, were analyzed retrospectively. Over this time period, 187 patients with brucellosis were diagnosed. Twenty-five (13.3%) of 187 patients had pancytopenia during admission to hospital. The diagnosis of brucellosis was confirmed by standard tube agglutination test in all patients; titers were 1:320 in 1 patient and 1:1280 in 24 patients. Blood culture was positive for Brucella melitensis in 3 patients (12%). Fever was the most common manifestation, followed by malaise, anorexia, sweating, and weight loss. Fever and splenomegaly were the common signs in most patients. In addition, arthritis was observed in 5 patients, and epistaxis, headache, and abdominal pain were observed in 3 patients. The common bone marrow aspiration findings consisted of increased megakaryocytes and hyperplasia of erythroid series, with a shift to the left of the granulocytic series. Histiocytic hyperplasia was observed in the bone marrow smear of 2 patients. Mild hemophagocytosis was observed in the bone marrow of 3 patients. All patients recovered completely, and their peripheral blood counts returned to normal by 2 to 6 weeks after antibiotic treatment of brucellosis. In conclusion, the authors would like to emphasize that brucellosis should be considered in the differential diagnosis of children with pancytopenia.

Metastasis of testicular yolk sac tumor to cauda equina.

Primary germ cell tumors may spread to the lung, liver, brain, and bone hematogenously. However, spinal metastasis is extremely rare. A case with yolk sac tumor (YST) and cauda equina metastasis was presented in a 2-year-old boy. Two months prior to admisssion, he underwent left radical orchiectomy for testicular YST. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed spinal and cauda equina involvement. This is the first report of metastasis of testicular YST to cauda equina in a child.

A case of idiopathic intracranial hypertension related with vesicoureteral reflux.

Pseudotumor cerebri is idiopathic intracranial hypertension. The etiology of this syndrome has not been fully clarified. Excess cerebrospinal fluid production, scarcity of cerebrospinal fluid absorption, intracranial venous pressure elevation, increased intracranial blood volume are all thought to be responsible. The symptoms of the disease may be ordered according to prevalence as follows: headache due to increased intracranial pressure, blurred vision and diplopia. A thirteen-year-old female patient was brought in with complaints of headache, double and blurred vision. Systemic arterial hypertension (140/70 mmHg) was determined. Vesicoureteral reflux was detected as the hypertension etiology. In this article a rare pseudotumor cerebri case is presented secondary to vesicoureteral reflux which caused hypertension.

A desensitization protocol in children with L-asparaginase hypersensitivity.

BACKGROUND: L-asparaginase is effective in the treatment of malignant diseases, but it has been associated with hypersensitivity reactions in 5% to 45% of the patients. PURPOSE: To determine whether a desensitization protocol in children with native Escherichia coli L-asparaginase hypersensitivity allows subsequent safe administration of native E. coli L-asparaginase. PATIENTS AND METHODS: A desensitization protocol was used in 9 children with leukemia (n=8) or Langerhans cell histiocytosis (n=1) and previous severe (n=4) or mild/moderate (n=5) L-asparaginase hypersensitivity. Dexamethasone (2 mg/kg intravenously) and pheniramine hydrogen maleate (1 mg/kg intravenously) were administered 1 hour before each of the following L-asparaginase administrations. Subsequently, 0.1%, 1%, 5%, 10%, and the remaining 84% of the total dose of L-asparaginase (10,000 IU/m2) were each prepared in 240 mL 0.9% saline and infused over 4 hours (20 h in total). RESULTS: After desensitization, none of the patients had recurrence of hypersensitivity reactions during the subsequent 2 to 15 doses (median: 6 doses/patient; 68 doses in total) of native E. coli L-asparaginase. CONCLUSION: This desensitization protocol was safe and allows continued administration of native E. coli L-asparaginase administration.

The association of oxidant status and antioxidant capacity in children with acute and chronic ITP.

PURPOSE: This study was undertaken to investigate oxidant and antioxidant systems in patients with immune thrombocytopenic purpura (ITP). With this purpose, we measured the levels of serum malondialdehyde (MDA), total antioxidant capacity (TAC), total oxidant status (TOS), and other oxidative stress parameters. PATIENTS AND METHODS: Fifty-two pediatric patients with ITP (25 acute, 27 chronic) and 21 healthy children were included in the study. Patients with acute ITP were studied, before and after, methylprednisolone treatment. RESULTS: Hemoglobin, hematocrit, platelet count, and TAC were statistically significantly lower in patients with acute ITP before treatment than those in the control group (P<0.05). In addition, in this group, MDA, TOS levels, and OSI (oxidative stress index) were found to be higher than those in the control group. In chronic ITP group, although hemoglobin hematocrit, platelet counts, and TAC levels were statistically significantly lower than those in the control groups,the mean platelet volume, MDA, TOS, and OSI were found to be statistically significantly higher (P<0.05). Platelet count and mean platelet volume values were statistically significantly lower in patients with acute ITP before treatment than after treatment (P<0.05). We also found a positive correlation between thrombocyte count and TAC, in patients with acute ITP before treatment (r: 0.601, P<0.001) and acute ITP after treatment (r: 0.601, P<0.001) and chronic ITP (r: 0.601, P<0.001). A negative correlation was found between thrombocyte count and serum MDA levels, in patients with acute ITP before treatment (r: -0.356, P<0.001) and acute ITP after treatment (r: -0.356, P<0.001) and chronic ITP (r: -0.356, P<0.001). We also found a negative correlation between thrombocyte count and serum OSI, in patients with acute ITP before treatment (r: -0.494, P<0.001) and acute ITP after treatment (r: -0.494, P<0.001) and chronic ITP (r: -0.494, P<0.001). A negative correlation was found between thrombocyte count and TOS, in patients with acute ITP before treatment (r: -0.470, P<0.001) and acute ITP after treatment (r: -0.470, P<0.001) and chronic ITP (r: -0.470, P<0.001). In conclusion, increased MDA, TOS and OSI, and decreased TAC levels were found in patients with acute and chronic ITP. CONCLUSIONS: On the basis of these findings, we suggest that free oxygen radicals may have an effect on the structural and functional damage of platelets, and on the mechanism of thrombocytopenia in both, acute and chronic ITP.

A 3-day-old boy with a right preauricular swelling.

A 3-day-old boy was admitted for right preauricular swelling. Examination showed a toxic looking neonate with poor neonatal reflexes. There was erythema and swelling of 4 × 3 cm at the level of the right cheek. Laboratory investigation pointed to a bacterial infection. With parenteral antibiotics, the lesion resolved completely.

Clinical findings in children with cutaneous anthrax in eastern Turkey.

Anthrax is a zoonosis produced by Bacillus anthracis. The aim of this study was to evaluate the clinical findings, therapy, and outcome in children with cutaneous anthrax (CA). Data on age, gender, occupation, clinical symptoms and findings, location and type of lesions, clinical history, laboratory findings, treatment, and outcome were recorded from patients' medical records, retrospectively. The study included 65 patients between 1 month and 18 years old (9.0±4.0 years), 37 patients (56.9%) were male and 28 (43.1%) were female. Most of the patients (89.1%) were admitted in summer and autumn (p<0.001). Of the 65 patients, 20 patients (30.8%) had received antibiotherapy before admission to our hospital. All patients except one had a contact history with infected animals. A 1-month-old patient had a contact history with his mother who had CA. On clinical examination, anthrax edema was noted in 36 (55.3%) patients, anthrax pustule in 27 (41.5%), and anthrax edema and anthrax pustule in two (3%) patients. Gram staining and culture was positive for B. anthracis in 35 (53.8%) patients, and only Gram staining was positive in 10 (15.4%) patients. In the remaining 20 (30.8%) patients, the diagnosis was made by clinical findings. Because the anthrax outbreak in Turkey was associated with slaughtering or milking of ill cows, sheep, or goats, and handling raw meat without taking any protective measures, persons in the community must be educated about using personal protective equipment during slaughtering of animals and handling of meat and skins.

The incidence of asymptomatic hypertension in school children.

BACKGROUND: Although hypertension is a well-documented risk factor for cardiovascular diseases, including myocardial infarction and stroke, in adults, only recently has knowledge about childhood hypertension increased significantly. AIM: To determine the incidence of asymptomatic hypertension in school-age children. SUBJECTS AND METHODS: Children in primary school were chosen with a randomized sampling method. During measurement of blood pressure, a calm, comfortable setting was provided. Blood pressure measurements were performed by only 1 researcher. For accurate measurement of blood pressure, recommended standards were followed. RESULTS: A total of 1,963 children were included in the study. The incidence of systolic hypertension and diastolic hypertension were 7% and 2%, respectively. Obesity was present in 10.5% girls with hypertension and 13.9% of boys with hypertension. CONCLUSION: Our findings indicate that hypertension is an important health problem in children, and its prevalence is quite high. Blood pressure measurements must be a part of routine clinical examinations. Further studies should be performed in high-risk populations to prevent hypertension and to establish methods of early diagnosis and treatment in children.

The prevalence of urinary lithiasis in children in Van Region, Turkey.

We aimed to determine the prevalence and etiology of urinary lithiasis in childhood in our region, Van, Turkey. A total of 1120 children were included in the study. Urinary ultrasonography was performed in all the children between April 2003 and June 2003. During the study, the kidneys were examined longitudinally and transversally using a 3.75 MHz convex probe in a Hitachi EUB -315 ultrasonography machine. The children's ages ranged from 7 years to 14 years (10.16 +/- 1.92 years) and 572 (51.1%) were males and 548 (48.9%) females. Urinary ultrasonography showed that 19 (1.7%) children had urinary lithiasis, which was in the right kidney in 15 children and in the left kidney in four children. Urinary lithiasis was in the upper urinary tract in all children. The etiological studies showed metabolic disorder in 14 children, and congenital renal anomaly in one child, but no underlying cause was diagnosed in four children. In conclusion, we found that was the prevalence of urinary lithiasis was 1.7% in school-aged children in our region. It was also noted that all urinary lithiasis was in the upper urinary system and its most common cause was metabolic disorder.

Acute amitriptyline intoxication: an analysis of 44 children.

The tricyclic antidepressant agents, particularly amitriptyline and dothiepin, are recognized for their potentially lethal cardiovascular and neurological effects in poisoned patients. In this article, the clinical and laboratory findings of 44 children with amitriptyline intoxication are reviewed. Our purpose was to investigate amitriptyline intoxication in childhood. Of 44 patients, 21 (47.7%) were boys, 23 (52.3%) were girls, and the ages ranged from 12 months to 14 years (mean +/- SD; 4.09 +/- 2.9 years). All children except one who took an overdose of amitriptyline to decrease his pain accidentally ingested an overdose of amitriptyline. The amount of amitriptyline ingested was between 2 mg/kg and 97.5 mg/kg (mean +/- SD; 13.6 +/- 17.7 mg/kg per dose) (the drug dosage was not known in 13 children). The most commonly observed clinical and laboratory findings were lethargy, tachycardia, convulsion, hyperglycemia and leukocytosis. In all patients except for two children who died the abnormal clinical and laboratory findings returned to normal within a few days after admission and they were discharged from the hospital in good health within the fourth day of admission. One of the children ingested 97.5 mg/kg amitriptyline and probably died due to status epilepticus and another child who died ingested 36 mg/ kg amitriptyline and died due to cardiopulmonary arrest. In conclusion, our findings showed that initial symptoms and signs of acute amitriptyline intoxication appeared severe, but they disappeared with only supportive care required in most children except for cases that ingested high doses of drug within a few days. In contrast to adults, we infrequently noted respiratory insufficiency, arrhythmia and hypotension in children with acute amitriptyline intoxication.

Does maternal diet affect infantile colic?

OBJECTIVE: The aim of this study was to investigate the effect of maternal diet on infantile colic without any interventions or food restrictions. METHODS: Thirty colicky and 29 non-colicky infants were included in this prospective study. Mother's diet and baby crying time were recorded for 1 week by mothers; nutritionist classified contents of mother's diet and compared the diet of mother in colicky and non-colicky infants. RESULTS: It was found that mothers of non-colicky infants consumed significantly more grapes and lemons than mothers of colicky infants (p = 0.044). The crying time was moderately negatively correlated with the percentage of protein in the maternal diet (R = -0.45, p = 0.01) and the presence of potatoes in the maternal diet (R = -0.38, p = 0.034) and positively correlated with the maternal consumption of walnut (R = 0.38, p = 0.034), banana (R = 0.44, p = 0.01). CONCLUSIONS: Removing bananas from the maternal diet may reduce colic. The consumption of a protein-rich maternal diet, grapes, lemons and potatoes by breastfeeding mothers may protect infants from colic.

Report of eight children with amitraz intoxication.

Many pesticides are formulated in organic solvents. An example is amitraz, one of the formamidine groups of pesticidal chemicals. It is commonly used for the treatment of generalized demodicosis in dogs and for the control of ticks and mites in cattle and sheep. In this article, the clinical and laboratory findings of eight children with amitraz intoxication are reviewed. The purpose was to enlighten the findings of amitraz intoxication in children. Of the eight patients, five (62.5%) were boys, three (37.5%) were girls, and the ages ranged from 1 to 4 years. All children accidentally ingested amitraz orally, with no dermal exposure. The most common observed signs were decreased consciousness and bradycardia. Leukocytosis, hyperglycemia, hypernatremia, increased serum aspartate transaminase level, and prolonged partial prothrombin time were diagnosed in children. None of the children had hypothermia, hypotension, or convulsion and none of the patients died. The findings show that the initial signs and symptoms of acute amitraz intoxication appeared severe but they disappeared, with only supportive care needed in most cases within a few days.