The Quality of Life in Patients with Familial Mediterranean Fever and Their Parents Perception.

BACKGROUND: Familial Mediterranean fever (FMF) is a chronic disease characterized by recurrent episodes of fever and polyserositis. This study aimed to assess children's quality of life (QoL), as reported by children and their parents, and to compare the results according to clinical variables. MATERIAL AND METHODS: The study examined 107 children with FMF, evaluating their demographic and genetic data, utilizing the Pediatric Quality of Life Inventory (PedsQL) to assess QoL, and comparing scores based on disease severity. RESULTS: The severity of FMF is inversely correlated with QoL scores, with mild cases having the highest scores (97±4), followed by moderate (76±11) and severe cases (52±10.3) (p<0,001). Disease severity, treatment adherence, healthcare utilization, genetic mutations, family income, and maternal age at birth all significantly impact perceived quality of life in FMF patients (p<0,001). Additionally, parents reported lower QoL for children with FMF who experienced various adverse factors such as low family income, household smoking, frequent attacks, hospitalizations, irregular medication use, and low maternal education levels (p<0,001). CONCLUSION: Children's daily activities, academic performance, and family functioning are all significantly impacted by FMF. Physicians caring for patients with FMF should be aware of the QoL changes in the management of these patients. As a result, medical therapy, patient education, and indicators of psychological and social support can all be offered more effectively.

Pediatric kidney care experience after the 2023 Türkiye earthquake.

BACKGROUND: Two earthquakes on 6 February 2023 destroyed 10 cities in Türkiye. We report our experience with pediatric victims during these catastrophes, with a focus on crush syndrome related-acute kidney injury (Crush-AKI) and death. METHOD: Web-based software was prepared. Patient demographics, time under rubble (TUR), admission laboratory data, dialysis, and kidney and overall outcomes were recorded. RESULTS: A total of 903 injured children (median age 11.62 years) were evaluated. Mean TUR was 13 h (interquartile range 32.5, max 240 h). Thirty-one of 32 patients with a TUR of >120 h survived. The patient who was rescued after 10 days survived. Two-thirds of the patients were given 50 mEq/L sodium bicarbonate in 0.45% sodium chloride solution on admission day. Fifty-eight percent of patients were given intravenous fluid (IVF) at a volume of 2000-3000 mL/m2 body surface area (BSA), 40% at 3000-4000 mL/m2 BSA and only 2% at >4000 mL/m2 BSA. A total of 425 patients had surgeries, and 48 suffered from major bleeding. Amputations were recorded in 96 patients. Eighty-two and 66 patients required ventilator and inotropic support, respectively. Crush-AKI developed in 314 patients (36% of all patients). In all, 189 patients were dialyzed. Age >15 years, creatine phosphokinase (CK) ≥20 950 U/L, TUR ≥10 h and the first-day IVF volume <3000-4000 mL/m2 BSA were associated with Crush-AKI development. Twenty-two deaths were recorded, 20 of 22 occurring in patients with Crush-AKI and within the first 4 days of admission. All patients admitted after 7 days survived. CONCLUSIONS: These are the most extensive pediatric kidney disaster data obtained after an earthquake. Serum CK level was significantly associated with Crush-AKI at the levels of >20 950 U/L, but not with death. Adolescent age and initial IVF of less than 3000-4000 mL/m2 BSA were also associated with Crush-AKI. Given that mildly injured victims can survive longer periods in the disaster field, we suggest uninterrupted rescue activity for at least 10 days.

Urine soluble TLR4 levels may contribute to predict urinary tract infection in children: the UTILISE Study.

BACKGROUND: One of the most common bacterial infections in childhood is urinary tract infection (UTI). Toll-like receptors (TLRs) contribute to immune response against UTI recognizing specific pathogenic agents. Our aim was to determine whether soluble TLR4 (sTLR4), soluble TLR5 (sTLR5) and interleukin 8 (IL-8) can be used as biomarkers to diagnose UTI. We also aimed to reveal the relationship between urine Heat Shock Protein 70 (uHSP70) and those biomarkers investigated in this study. METHODS: A total of 802 children from 37 centers participated in the study. The participants (n = 282) who did not meet the inclusion criteria were excluded from the study. The remaining 520 children, including 191 patients with UTI, 178 patients with non-UTI infections, 50 children with contaminated urine samples, 26 participants with asymptomatic bacteriuria and 75 healthy controls were included in the study. Urine and serum levels of sTLR4, sTLR5 and IL-8 were measured at presentation in all patients and after antibiotic treatment in patients with UTI. RESULTS: Urine sTLR4 was higher in the UTI group than in the other groups. UTI may be predicted using 1.28 ng/mL as cut-off for urine sTLR4 with 68% sensitivity and 65% specificity (AUC = 0.682). In the UTI group, urine sTLR4 levels were significantly higher in pyelonephritis than in cystitis (p < 0.0001). Post-treatment urine sTLR4 levels in the UTI group were significantly lower than pre-treatment values (p < 0.0001). CONCLUSIONS: Urine sTLR4 may be used as a useful biomarker in predicting UTI and subsequent pyelonephritis in children with UTI. A higher resolution version of the Graphical abstract is available as Supplementary information.

The ureteral diameter ratio as a predictive factor in renal scarring associated with primary vesicoureteral reflux.

INTRODUCTION: The ureteral diameter ratio (UDR) is reported to be effective in predicting the outcomes of vesicoureteral reflux (VUR) in several studies. OBJECTIVE: The objective of the current study was to compare the risk of scarring in patients with VUR relative to UDR and the VUR grade. We also aimed to demonstrate other associated risk factors in scarring and investigate the long-term complications of VUR and their relationship with UDR. STUDY DESIGN: Patients diagnosed with primary VUR were retrospectively enrolled in the study. UDR was calculated by dividing the largest ureteral diameter (UD) by the distance between L1-L3 vertebral bodies. Demographic and clinical data, laterality, VUR grade, UDR, delayed upper tract drainage on voiding cystourethrogram, recurrent urinary tract infections (UTI), and long-term complications of VUR were compared between the patients with and without renal scars. RESULTS: A total of 127 patients and 177 renal units were included in the study. There was a significant difference between the patients with and without renal scars according to age at diagnosis, bilaterality, reflux grade, UDR, recurrent UTI, bladder bowel dysfunction, hypertension, decreased estimated glomerular filtration rate, and proteinuria. The logistic regression analysis revealed that UDR had the highest odds ratio among the factors affecting scarring in VUR. DISCUSSION: VUR grading based on the evaluation of the upper urinary tract is one of the most important predictors for treatment options and prognosis. However, it is more likely to reflect ureterovesical junctional anatomy and function, which play a crucial role in the pathogenesis of VUR. CONCLUSION: UDR measurement seems to be an objective method that can help clinicians predict renal scarring in patients with primary VUR.

Follow up of renal outcomes in children with solitary kidney.

BACKGROUND: Solitary kidney (SK) affects 1/1000 people worldwide, and there are controversies concerning renal outcomes in these patients. This study aimed to investigate clinical findings and renal outcomes in children with SK and to compare the results for congenital (CSK) and acquired SK (ASK) groups. METHODS: The study included patients that presented to our pediatric nephrology department with SK between January 2010 and January 2021. Demographic and clinical data were recorded retrospectively. RESULTS: Of the 101 patients with SK, 71 had CSK (55 had unilateral renal agenesis and 16 had a multicystic dysplastic kidney) and 30 had ASK (17 had previously undergone unilateral nephrectomy due to a renal tumor and 13 had urological structural anomalies). There were nine patients (9%) with renal injury. The serum uric acid level was significantly higher and the estimated glomerular filtration rate was significantly lower in the patients with ASK compared with those with CSK (p = 0.005 and p < 0.001, respectively). There was a positive correlation between renal injury and the uric acid level (p < 0.001, r = 0.45). CONCLUSION: In addition to the management of blood pressure and proteinuria, it is important to control uric acid levels in patients with SK, especially those with ASK, to prevent renal injury. The ASK group has a greater risk of renal injury than the CSK group. There is a need for new markers to predict early stage renal damage in SK.

Fibrinogen-to-Albumin Ratio in Familial Mediterranean Fever: Association with Subclinical Inflammation.

BACKGROUND: Familial Mediterranean fever (FMF) is the most seen monogenic periodic fever syndrome characterised by bouts of fever and serositis. It is known that subclinical inflammation (SI) can persist in the symptom-free period and lead to amyloidosis even under colchicine treatment. This study aimed to evaluate the role of the fibrinogen-to-albumin ratio (FAR) in FMF and its correlation with SI. MATERIAL AND METHODS: A total of 112 patients with FMF and 78 controls were enrolled in this retrospective study. Demographic, laboratory and genetic data were obtained from the hospital records. RESULTS: The FAR values of the FMF cases were significantly higher than the control group (p<0.001). In the FMF group, the patients with SI had higher FAR values than those without SI (p<0.001). FAR was positively correlated with SI (r=0.413, p<0.001). The receiver operating characteristic curve analysis showed that FAR had a higher area under the curve value than albumin and fibrinogen. CONCLUSION: Detecting SI in patients with FMF is crucial in preventing amyloidosis, the most devastating complication of FMF. FAR is a simple, inexpensive, easily obtained indicator which can be used for reflecting SI in FMF. HINTERGRUND: Familiäres Mittelmeerfieber (FMF) ist das am häufigsten auftretende monogene periodische Fiebersyndrom, das durch Fieberschübe und Serositis gekennzeichnet wird. Die subklinische Entzündung (SI) kann bekanntlich auch in der symptomfreien Phase fortbestehen und zu Amyloidose führen, selbst unter Behandlung mit Colchicin. Ziel dieser Studie ist es, die Beziehung zwischen Fibrinogen und Albumin (AFR) bei FMF und deren Korrelation mit SI zu untersuchen. MATERIAL UND METHODEN: Insgesamt wurden für diese retrospektive Studie 112 Patienten mit FMF und 78 Kontrollpatienten eingeschlossen. Die demographischen, labortechnischen und genetischen Daten wurden aus den Krankenhausunterlagen entnommen. ERGEBNISSE: Die Werte von AFR bei Patienten mit FMF waren signifikant höher als die der Kontrollgruppe (<0,001). Patienten mit FMF mit SI zeigten höhere AFR-Werte als FMF-Patienten ohne SI (<0,001). AFR korrelierte positiv mit SI (r=0,413, p<0,001). Die Analyse der ROC-Kurve (Receiver Operating Characteristic) zeigte, dass AFR einen höheren Wert der Fläche unter der Kurve (AUC) hatte als Albumin und Fibrinogen. DISKUSSION: Die Erkennung von SI bei FMF-Patienten ist entscheidend für die Vermeidung von Amyloidose, der schlimmsten Komplikation von FMF. AFR ist ein einfacher, kostengünstiger, leicht durchzuführender Indikator, der in FMF zur Spiegelung von SI verwendet werden kann.

Urinary NGAL is a Potential Biomarker for Early Renal Injury in Insulin Resistant Obese Non-diabetic Children

Objective: Neutrophil gelatinase-associated lipocalin (NGAL) is one of the new biomarkers for detecting acute renal injury. There are studies showing the relationship between NGAL and renal injury in obese children. The aim of this study was to investigate whether urinary levels of NGAL, kidney injury molecule-1, and serum cystatin C are increased in insulin resistance (IR) patients before the development of diabetes. Methods: Cross-sectional, case-controlled study that included non-diabetic obese children and adolescent patients with IR and a non-diabetic obese control group with no IR, who attended a tertiary center pediatric endocrinology outpatient clinic between 2016-2018. Those with diabetes mellitus and/or known renal disease were excluded. NGAL and creatinine (Cr) levels were evaluated in the morning spot urine from all participants. Serum renal function was evaluated. Results: Thirty-six control and 63 IR patients were included in the study, of whom 68 (68.7%) were girls. The mean age of all participants was 13.12±2.64 years and no statistically significant difference was found between the two groups in terms of age or gender distribution. Median (range) spot urinary NGAL (u-NGAL) values in the IR group were significantly higher at 26.35 (7.01-108.7) ng/mL than in the control group at 19.5 (3.45-88.14) ng/mL (p=0.018). NGAL/Cr ratio was also significantly higher in the IR group compared to the control group (p=0.018). Conclusion: Obese pediatric patients with IR were shown to have elevated levels of u-NGAL, a marker of renal injury. u-NGAL examination may show early renal injury before development of diabetes.

Indoxyl sulfate associates with cardiovascular phenotype in children with chronic kidney disease.

BACKGROUND: Cardiovascular disease is the leading cause of death in children with chronic kidney disease (CKD). Serum levels of gut-derived uremic toxins increase with deterioration of kidney function and are associated with cardiac comorbidities in adult CKD patients. METHODS: Indoxyl sulfate (IS) and p-cresyl sulfate (pCS) were measured by high-performance liquid chromatography in serum of children participating in the Cardiovascular Comorbidity in Children with CKD (4C) Study. Results were correlated with measurements of the carotid intima-media thickness (cIMT), central pulse wave velocity (PWV), and left ventricular mass index (LVMI) in children aged 6-17 years with initial eGFR of 10-60 ml/min per 1.73 m2. RESULTS: The median serum levels of total IS and of pCS, measured in 609 patients, were 5.3 µmol/l (8.7) and 17.0 µmol/l (21.6), respectively. In a multivariable regression model, IS and pCS showed significant positive associations with urea and negative associations with eGFR and uric acid. Furthermore, positive associations of pCS with age, serum albumin, and non-Mediterranean residency and a negative association with glomerular disease were observed. By multivariable regression analysis, only IS was significantly associated with a higher cIMT SDS at baseline and progression of PWV SDS within 12 months, independent of other risk factors. CONCLUSIONS: Serum levels of gut-derived uremic toxins IS and pCS correlated inversely with eGFR in children. Only IS was significantly associated with surrogate markers of cardiovascular disease in this large pediatric CKD cohort.

Henoch-Schonlein purpura, post-streptococcal glomerulonephritis and acute rheumatic carditis after Group A ß-haemolytic streptococcal infection.

Besides association with acute rheumatic fever (ARF) and acute glomerulonephritis (APSGN), in up to 40% of cases, Group A ß-haemolytic streptococcal (GABHS) infections are also implicated as a trigger for Henoch-Schonlein purpura (HSP). A 7-year-old girl with GABHS throat infection who developed HSP, APSGN and rheumatic carditis is reported. She presented with palpable purpura and arthritis in both ankles and later developed carditis characterised by mitral/aortic regurgitation and glomerulonephritis characterised by mixed nephritic/nephrotic syndrome. She had a raised anti-streptolysin titre (ASOT), blood urea nitrogen and creatinine and hypocomplementaemia (C3), and renal biopsy demonstrated endocapillary and extracapillary proliferative glomerulonephritis with crescents. Immunofluorescence microscopy demonstrated a 'full house' of immunoglobulin and complement, viz. IgA + 2, IgG + 3, IgM + 2, C3c + 1, Clq + 2 with predominantly IgG deposition. One week earlier, her 4-year-old sister had presented to another hospital with HSP complicated by microscopic haematuria, nephrotic-range proteinuria and gastro-intestinal involvement, and with raised ASOT and low C3 levels. Although HSP has been associated with either ARF or APSGN, this is the first case of a child with HSP, ARF and APSGN in combination.

Evaluation of abnormal radiological findings in children aged 2 to 36 months followed by recurrent urinary tract infection: a retrospective study.

Our aim is to determine the rational usage of imaging techniques in order to prevent or minimize permanent renal damage in recurrent urinary tract infections (UTIs). This study was enrolled children aged between 2 and 36 months, following-up with the diagnosis of recurrent UTI. All children had ultrasonography (USG) and dimercaptosuccinic acid scanning, 39 of them had underwent on voiding cystourethrography. There were 133 children (87 girls, 46 boys) with the mean age of 32.82 ± 38.10 months included into the study. Forty-three kidney units were normal in ultrasonogram of which seven units had reflux whereas among 35 units with hydronephrosis 22 units had reflux. Sensitivity and specificity presence of hydronephrosis in ultrasonogram for prediction of reflux was 75.9% and 73.5%, respectively. There were 19 dilated ureters in ultrasonogram, and among them 14 had reflux. Sensitivity and specificity of presence with ureteral dilatation in ultrasonogram for prediction of reflux was found as 48.3% and 89.8%, respectively. The sensitivity of parenchymal thinning seen in ultrasonogram for the evaluation of renal parenchyma was 15.9%, whereas specificity was 98.2% .Sensitivity and specificity of dimercaptosuccinic acid for prediction of reflux was 51.6% and 72.3%, respectively. The normal ultrasonogram findings cannot rule out neither possibility of reflux presence nor development of renal scarring. Therefore, DMSA scanning has major role both in determination of parenchymal damage and prevention of scarring. Also we get an important result as ureteral dilatation seen in USG, related to presence of reflux.

Risk factors for renal scarring in children with primary vesicoureteral reflux disease.

To determine the incidence of renal scarring among patients with primary vesicoureteral reflux (VUR) and the possible risk factor(s), we studied 90 children (60 girls and 30 boys) with VUR followed in the Pediatric Nephrology Unit at the Ege University Hospital from 1998 to 2003. All the patients were assessed for VUR grade by voiding cystoureterography and for presence of renal scarring by (99 m) technetium dimercapto-succinic acid scintigraphy. All infants with VUR were given low-dose prophylactic antibiotics and followed-up until resolution of the reflux. Grade of reflux and number of urinary tract infection (UTI) episodes (≥3) were found to be statistically significant risk factors for renal scarring (P <0.05). However, gender, familial history and laterality of the disease were not found to be statistically significant risk factors (P >0.05). Similarly, there was no statistically significant difference of frequency of renal scarring among the different age groups (P >0.05). We conclude that recurrences of UTI and VUR severity are significant risk factors for renal scarring in children with VUR. Therefore, identification of VUR at an early age may offer the opportunity to prevent episodes of UTI and possible formation of renal scars that may result in end-stage renal failure.

A patient with cystinosis presenting like bartter syndrome and review of literature.

BACKGROUND: Nephropathic cystinosis is an autosomal recessively inherited metabolic disorder presenting with metabolic acidosis, Fanconi syndrome and renal failure. CASE PRESENTATION: We present a 6-year-old girl with severe growth failure, hyponatremia and hypokalemia. Her parents were 4(th) degree relatives. Two relatives were diagnosed as end stage renal failure. She also had persistant hypokalemic hypochloremic metabolic alkalosis. Her renal function was normal at presentation. She was thought to have Bartter syndrome with supporting findings of elevated levels of renin and aldosterone with normal blood pressure, and hyperplasia of juxtaglomerular apparatus. Her metabolic alkalosis did not resolve despite supportive treatment. At 6(th) month of follow-up proteinuria, glucosuria and deterioration of renal function developed. Diagnosis of cystinosis was made with slit lamp examination and leukocyte cystine levels. At 12(th) month of follow-up her metabolic alkalosis has converted to metabolic acidosis. CONCLUSION: In children presenting with persistant metabolic alkalosis, with family history of renal failure, and parental consanguinity, cystinosis should always be kept in mind as this disease is an important cause of end stage renal failure which may have features mimmicking Bartter syndrome.

Hypohidrotic ectodermal dysplasia associated with glucose-6-phosphate dehydrogenase deficiency.

Hypohidrotic ectodermal dysplasia (HED) is a syndrome characterized by hypodontia, hypotrichosis, and partial or total ecrine sweat gland deficiency. The most prevalent form of HED is inherited as an X linked pattern. Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency is an X-linked recessive disease, which leads to hemolytic anemia and jaundice. It is expressed in males, while heterozygous females are usually clinically normal. A 12-year-old boy with the complaints of hair and eyebrow disturbances, teeth disfigurement, decreased sweating, and xerosis presented to the outpatient clinic. Dermatological examination revealed sparse hair and eyebrows, conical-shaped teeth, xerosis, syndactylia, transverse grooves, and discoloration of nails. Laboratory findings indicated anemia. His 3-year-old sister also had sparse hair and eyebrows, xerosis, and syndactylia. We learned that the patient had a previous history of neonatal jaundice and a diagnosis of G-6-PD deficiency. Although it has been shown that loci of ectodermal dysplasia and G-6-PD deficiency genes are near to one another, we did not find any case study reporting on occurrence of these two genetic diseases together. With the aspect of this rare and interesting case, the relationship between HED and G-6-PD deficiency was defined.

A Case of Henoch-Schönlein Purpura with P369S Mutation in MEFV Gene.

BACKGROUND: Henoch-Schönlein purpura (HSP) is the most common vasculitis of childhood. HSP can affect multiple organs presenting with a characteristic rash in most of the patients. Familial Mediterranean Fever (FMF) is an inherited inflammatory disease common in mediterranean populations. HSP is the most common vasculitis seen in children with FMF. CASE PRESENTATION: A 16 year old boy was referred with history of abdominal pain lasting for 20 days. He was hospitalized and had appendectomy. Due to the persistence of his abdominal pain after surgery he was admitted to our hospital. His physical examination showed palpable purpuric rashes symmetrically distributed on lower extremities. Abdominal examination revealed periumbilical tenderness. Laboratory tests showed elevated erythrocyte sedimentation rate, Creactive protein and fibrinogen. Urinalysis revealed microscopic hematuria and severe proteinuria. The fecal occult blood testing was positive. Based on these clinic findings, the patient was diagnosed as HSP with renal, gastrointestinal tract and skin involvement. We performed DNA analysis in our patient because he had diagnosis of vasculitis with severe symptoms and found that he was carrying heterozygote P369S mutation. CONCLUSION: Our case is noteworthy as it indicates that it may be important not to overlook presence of FMF mutations in patients with a diagnosis of severe vasculitis.

Childhood Pityriasis rosea inversa without Herald Patch Mimicking Cutaneous Mastocytosis.

BACKGROUND: Pityriasis rosea is a self-limited inflammatory condition of the skin that mostly affects young adults. Several less common atypical presentations have been reported. CASE PRESENTATION: A 6-year old girl with red-brown maculopapular eruption sized 0, 5-1 cm in diameter localized on neck, trunk and popliteal region visited our general pediatric outpatient clinic. The eruption was wide spread especially on flexural areas. After consulting dermatologist skin biopsy was performed. According to clinical and histopathological findings as inverse (flexural) pityriasis rosea was diagnosed. For treatment, systemic antihistamine, topical corticosteroid cream and emollient were applied. The lesions healed in two months. Spontaneous healing of the eruption also confirmed the diagnosis of pityriasis rosea. CONCLUSION: We present this interesting pediatric case to show and discuss pityriasis rosea, atypical presentations, differential diagnosis and the importance of dermatological examination and importance of dermatologic consultation for pediatric patients with skin eruption.

Pediatric ureteroceles: diagnosis, management and treatment options.

OBJECTIVE: The aim of the study was to evaluate clinical characteristics of ureteroceles particularly for diagnostic and treatment challenges. METHODS: Data about patients treated for ureterocele in the two hospital clinics during 1996- 2009 are retrospectively evaluated. FINDINGS: There were 12 girls and 7 boys. Symptomatic urinary tract infection was found in twelve cases. Ureterocele was associated with duplex systems in eleven cases. Vesicoureteral reflux was detected in 4 patients. Bladder diverticulum complicated with ureterocele in 1 patient. Ultrasonography diagnosed ureterocele in 12 patients. Renal scarring was detected in 6 patients at the side of ureterocele. Fifteen patients showed varying degrees of hydro-ureteronephrosis. Surgical therapy included upper pole nephrectomy in 3 cases. Bladder level reconstruction was performed in 11 cases. Five patients were treated only by endoscopic incision. In the follow up period 4 patients showed long term urinary tract infections whereas 3 of them were treated endoscopically. Postoperative reflux was still present in two patients who were treated by endoscopic incision. CONCLUSION: Ureterocele diagnosis and treatment show challenges. Urinary tract infection is important marker for urinary system evaluation. Preoperative management generally depends on a combination of diagnostic methods. Endoscopic incision needs serious follow up for postoperative problems.

Rapidly progressive glomerulonephritis in a child with Henoch-Schönlein Vasculitis and familial Mediterranean fever.

Henoch-Schonlein Vasculitis (HSV) is systemic small vessel vasculitis involving the skin, kidney, joints, and gastrointestinal tract. The proportion of patients reported to have renal involvement varies between 20% and 80%. Rapidly progressive glomerulonephritis (RPGN)is rare syndrome in children, characterized by clinical features of glomerulonephritis (GN) and rapid loss of renal function. We present a severe kidney involvement in a 14 year old boy with HSV in who is carring MEFV mutation. A 14 year old boy had developed sudden onset of palpable purpuric rash on his extensor surfaces of lower extremities. He had elevated an erythrocyte sedimentation rate (ESR) (45 mm/h), C-reactive protein (3.74 mg/dl), serum urea 66 mg/dl, serum creatinine 1.8 mg/dl. Also, he had hypocomplementemia. Antinuclear antibody, anti ds DNA, antineutrophil cytoplasmic antibody, anticardiolipine antibodies were negative. Urinalysis revealed macroscopic hematuria and proteinuria with a 24-h urinary protein excretion of 55 mg/m2/h. The renal biopsy specimen showed crescentic and necrotizing glomerulonephritis. He had also M694V/E148Q compound heterozygote mutation. Clinical symptoms and renal failure resolved with intermittant hemodialysis and medical therapy.

[Common viral etiologies of community acquired lower respiratory tract infections in young children and their relationship with long term complications]. / Küçük çocuklarda toplum kökenli viral alt solunum yolu enfeksiyonu etkenlerinin sikligi ve uzun dönem komplikasyonu ile iliskileri.

Viral lower respiratory tract infections (LRTIs) and their late complications are important causes of morbidity and mortality in childhood. The aims of this study were the detection of viral agents that cause community-acquired LRTIs in young children and investigation of the relationship between viral etiology and bronchiolitis obliterans (BO) which is one of the late complications of LRTIs. A total of 151 children (86 male, 65 female; mean age: 2.9 +/- 1.9 years) who were diagnosed to have LRTIs between the period of 2002-2004, at Pediatric Allergy and Pulmonology Department of a University Hospital in Manisa (located in the Aegean region of Turkey) were included to the study. The presence of respiratory viruses [respiratory syncytial virus (RSV), influenza virus type A and B, parainfluenza virus types 1, 2 and 3, adenovirus] in the nasopharyngeal aspirate specimens collected from children have been searched by direct fluorescence antibody test (Biotrin, Ireland). Respiratory viruses were detected in 25.2% (38/151) of the patients with LRTIs, while this rate was 46.8% (22/47) for 2002 period, 13.3% (8/60) for 2003 period and 18.2% (8/44) for 2004 period. RSV and adenoviruses both detected with a frequency of 31.5% (n= 12/38); were the most common agents encountered, and followed by parainfluenza (10/38, 26.3%) and influenza (9/38, 23.6%) viruses. Postinfectious BO have been diagnosed in 7.3% (11/151) of the patients; seven in 2002, one in 2003 and three in 2004 periods. Viral etiology were present in all of the patients who developed BO in 2002, while viral infection was detected in one of the patients who developed BO in 2003-2004 periods. Adenoviruses were the most frequently detected agents (n= 5) in BO cases with viral etology (n= 8). Viral agents were found positive in 72.7% (8/11) and 21.4% (30/140) of the patients with and without BO development, respectively, and this difference was found statistically significant (p= 0.02). Besides, BO development was detected in 21.1% (8/38) and 2.6% (3/113) of LRTI patients with and without viral etiology, respectively, and this difference was also significant (p< 0.05). In conclusion, the long term follow-up is important in young children with viral LRTIs for the early diagnosis of complications. Thus the identification of viruses might aid in estimation of prognosis.

Sleep quality and depression-anxiety in mothers of children with two chronic respiratory diseases: asthma and cystic fibrosis.

BACKGROUND: Sleep quality and psychological well being of parents are expected to be influenced by the child's health and disease status. The aim of this study was to compare sleep quality and depression-anxiety parameters in mothers of children with cystic fibrosis (CF) asthma and healthy controls. METHODS: The study included mothers of 62 children with asthma, 21 children with CF and 35 healthy children. All mothers filled in the Pittsburgh Sleep Quality Index (PSQI) questionnaire and hospital anxiety depression scale (HADS). RESULTS: Comparison of the three groups with Kruskall Wallis analysis demonstrated that subjective sleep, sleep efficiency and total PSQI scores were significantly different between the groups (p=0.02, p=0.01 and p=0.04 respectively). Comparisons of the groups in pairs with Mann Whitney U test with Bonferroni correction revealed that subjective sleep quality scores in mothers of children with asthma were significantly higher than the ones in the control group (1.0+/-0.9 vs 0.6+/-0.7, p=0.015). The other PSQI scores as well as the anxiety and depression scores were higher in CF and asthma groups when compared to the control group but did not reach statistical significance. Anxiety and depression scores were significantly correlated with PSQI total score in CF (rho=0.54 and 0.49 respectively) and asthma groups (rho=0.45 and 0.60 respectively) but not in the control group. CONCLUSION: In conclusion, presence of a chronic respiratory disease in a child may be associated with disturbed sleep quality and increased depression and anxiety in mothers.

Incidence of giardiasis among siblings in Turkey.

BACKGROUND: Giardiasis, a common infection among children, is caused by a flagellated protozoan called Giardia lamblia. It is well known to be contagious in common living places. This is an epidemiologic study investigating the incidence of giardiasis among the siblings of patients with giardiasis living in the same household. METHODS: Stool samples of 50 patients with a G. lamblia positive sibling in the same household, and 50 patients with a G. lamblia negative sibling in the same household, were examined for giardiasis by saline-Lugol, formalin-ethyl acetate concentration and trichrome staining methods. Other causes of diarrhea were excluded by microbiologic laboratory tests. RESULTS: Thirty-eight per cent of siblings of G. lamblia positive patients and 8% of siblings of G. lamblia negative children were found to be positive for G. lamblia cysts and/or trophozoites. The differences between the groups were statistically significant (P < 0.001). CONCLUSION: The results suggested that the examination of G. lamblia among the siblings of patients with giardiasis both in the same age group and living in the same household should be considered.