Pediatric kidney care experience after the 2023 Turkey/Syria earthquake.

BACKGROUND/AIMS: Two earthquakes on February 6th, 2023 destroyed ten 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: A web-based software was prepared. Patient demographics, time under rubble (TUR), admission laboratory data, dialysis, and kidney and overall outcomes were asked. RESULTS: 903 injured children (median age: 11.62 years) were evaluated. Mean TUR was 13 h (Interquartile range-IQR: 32.5), max 240 h). 31 of 32 patients with a TUR of >120 h survived. The patient who rescued after ten days survived.Two-thirds of the patients were given 50 mEq/L sodium-bicarbonate in 0.45% sodium-chloride solution on admission day. 58% of patients were given intravenous fluid (IVF) at a volume of 2000-3000 mL/m2 body surface area (BSA), 40% of 3000-4000 mL/m2 BSA, and only 2% of >4000 mL/m2 BSA. 425 patients had surgeries, 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). 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. 22 deaths were recorded, 20 of 22 occurred in patients with Crush-AKI and within the first 4 days of admission. All patients admitted after 7 days survived. CONCLUSIONS: This is the most extensive pediatric kidney disaster data 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 asscoiated 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.

Can microalbuminuria be an indicator of renal involvement in pediatric Covid 19 patients?

BACKGROUND: Proteinuria (both tubular and glomerular in origin) and its implications are well-known features of adult patients with COVID19. However currently studies addressing proteinuria and its role in the outcome of kidney and patients of pediatric COVID 19 is scarce. We aimed to evaluate the presence of microalbuminuria in order to detect early renal involvement in pediatric COVID 19 patients. METHODS: We prospectively evaluated 100 pediatric patients hospitalized with COVID 19 between April and July 2020. Clinical presentations, laboratory findings and outcomes were investigated. Microalbuminuria was compared with the age, gender, disease severity, and hemoglobin, platelet, leukocyte count and serum CRP levels of the patients. RESULTS: Twenty seven out of 100 patients had microalbuminuria. Fourteen patients had mild and fourteen had moderate disease. There was not any significant relation according to age and gender. Microalbuminuria was not related to the severity of the disease. Also the mean microalbuminuria level did not differ according to the disease course. Hemoglobin, platelet, leukocyte counts and serum CRP levels were also were not correlated with microalbuminuria levels. CONCLUSION: Although there was no difference between the groups with different disease course; microalbuminuria is detected in an important ratio of pediatric patients with COVID 19 in this study. In the highlight of our findings we suggest that urinary findings of pediatric COVID patients should be carefully evaluated.

Endocan levels in children with renal hypodysplasia.

AIMS: Severe reduction in nephron numbers that are characteristic of renal hypodysplasia (RHD) are one of the cause of childhood chronic kidney disease (CKD). Glomerular hyperfiltration, glomerular hypertrophy, progressive glomerular scarring, and interstitial fibrosis due to reduced nephron number are risk factors for CKD. In recent years, studies on specific markers for early diagnosis of renal failure and mortality have been carried out. The objectives of this study were to identify serum and urinary endocan levels that are expressed in glomerular endothelial cells and tubular epithelial cells in RHD. MATERIALS AND METHODS: 29 children with RHD were compared to 26 healthy controls in terms of serum and urinary endocan levels. RESULTS: The mean serum endocan level in the RHD group and the control group was 700.72 ± 323.19 and 426.86 ± 233.14 pg/mL, respectively. The mean serum endocan level was significantly higher (p = 0.003) in the RHD group. The mean urinary endocan level in the RHD group was 63.62 ± 92.46 pg/mL, and in the control group it was 80.26 ± 142.49 pg/mL. The mean urinary endocan level did not change between groups (p = 0.95). There was also a significant correlation between serum endocan level and uric acid level in the study group (r = 0.325, p = 0.028). CONCLUSION: To our knowledge, this was the first study that evaluated serum and urinary endocan levels in children with RHD. Although serum endocan level was found to be significantly higher in patients with RHD, further studies are needed to validate whether endocan could serve as a marker of poor renal prognosis in RHD.

A mysterious case with abdominal pain and syndrome of inappropriate anti-diuretic hormone secretion.

BACKGROUND: Acute intermittent porphyria (AIP) is a rare, hereditary, metabolic disease caused by a defect in heme biosynthesis. Hormonal changes may trigger porphyria attacks. CASE: Here we present a 17 -year- old adolescent refugee mother who applied to the pediatric emergency department with the complaint of diffuse abdominal pain at puerperium. The patient was hypertensive, and had convulsions after admission. Hyponatremia (serum sodium; 121 meq/L) was detected, and syndrome of inappropriate anti-diuretic hormone secretion (SIADH) was found to be the cause of hyponatremia which responded well to fluid restriction. Infectious, autoimmune and toxicologic laboratory work-up did not reveal any specific pathologies. Despite prompt utilization of analgesic treatment, the patient continued to have unbearable abdominal pain. The preference of prone position to relieve the pain and the family history of a mother who had died with similar symptoms, led us to the diagnosis of AIP. Genetic analysis showed a heterozygous mutation in hydroxymethylbilane synthase (HMBS) gene (c160+6T > A) which confirmed our diagnosis. CONCLUSION: Acute porphyrias should be considered in differential diagnosis of abdominal pain, especially when there are accompanying symptoms like hyponatremia, seizures, mental changes and hypertension.

Hypokalemia and hearing loss in a 3-year-old boy: Questions.

Bartter syndrome with sensorineural deafness (Bartter syndrome type 4) is an autosomal recessive disorder characterized with polyhydramniosis, premature birth, massive polyuria, renal salt-wasting, hypokalemic metabolic alkalosis, normotensive hyperreninemic hyperaldosteronism, and hearing loss. Homozygous mutations in BSND, CLCNKA, and CLCNKB mutations cause the disorder. Here we report a 3-year-old boy who had not been evaluated and investigated before cochlear implantation. Hypokalemia was detected during the routine laboratory workup before surgery. Further analyses revealed metabolic alkalosis with high renin and aldosterone levels. Hypokalemia improved with oral potassium chloride supplementation. Genetic tests revealed a homozygous c.139G>A (pG47R) mutation in BSND gene, and both parents were heterozygous for the same mutation. We want to emphasize the importance of evaluating hearing loss in children, since some of the genetic syndromes may cause life threatening abnormalities.

Tolvaptan Use to Treat SIADH in a Child.

Hyponatremia is one of the most common electrolyte abnormalities encountered in the clinical setting in hospitalized patients. The syndrome of inappropriate antidiuretic hormone secretion (SIADH) is the leading cause of hyponatremia in most of these cases. While fluid restriction, hypertonic saline infusion, diuretics, and the treatment of underlying conditions constitute the first line of treatment of SIADH, in refractory cases, and especially for pediatric patients, there seems not to be any other choice for treatment. Tolvaptan, although its use in pediatric patients is still very limited, might be an attractive treatment option for correction of hyponatremia due to SIADH. Here we present a pediatric case of SIADH that was resistant to treatment with fluid restriction and hypertonic saline infusion and was treated successfully with tolvaptan. Tolvaptan could be a good, safe, and effective treatment option in pediatric SIADH cases that are resistant to treatment. However, the dosage should be titrated carefully.

Management of stone disease in infants.

Evaluating and treating renal stone disease in infants are technically challenging. In this study, we evaluated the surgical treatment of renal stones in children under 1 year of age. We retrospectively reviewed the records of patients under 1 year old who were treated with ESWL, endourological or open surgical procedures for renal stone disease between January, 2009 and December, 2012. The patients' age, gender, stone size, stone location and number, complications, stone-free status, and postoperative complications were recorded. 19 of 121 infants with a mean age of 10.2 ± 3.07 months were treated with surgical procedures. Six (75%) of eight cystinuria patients required a surgical intervention. Retrograde endoscopic management was performed in thirteen patients (63.4%) as an initial surgical approach. There were three major (15.7%) complications. The rate of open surgical procedures was 31.6% (6 of 19 infants). The cutoff value of stone size for open surgery was 10 mm. There was a significant relationship between the conversion to open procedures and stone size, stone location, and symptom presentation especially the presence of obstruction (p < 0.05). After repeated treatments, the stone clearance rate of RIRS reached 84.6%. Retrograde intrarenal surgery is an effective and safe treatment method for renal stones in infants and can be used as a first-line therapy in most patients under 1 year old. This is especially important if an associated ureteral stone or lower pole stone that requires treatment is present and for patients with cystinuria, which does not respond favorably to ESWL.

Hemophagocytic lymphohistiocytosis secondary to Varicella zoster infection in a child with Henoch-Schönlein purpura.

Hemophagocytic lymphohistiocytosis (HLH) is a fatal, hyper-inflammatory syndrome that is characterized by untimely activation of macrophages, and manifests as cytopenia, organ dysfunction, and coagulopathy. Secondary HLH can be associated with infection, drugs, malignancy, and transplantation, and is mostly triggered by infection. Herein, we report the case of a patient with Henoch-Schönlein purpura (HSP) who developed severe HLH secondary to Varicella zoster infection.

An analysis of the levels of the soluble form of the endothelial protein C receptor in children with Henoch-Schönlein Purpura.

The pathogenesis of Henoch-Schönlein Purpura (HSP) has not been clearly defined. Inflammatory cytokines have been associated with HSP but there are only a few reports that have focused on coagulation. The endothelial protein C receptor (EPCR), which has anticoagulant and antiinflammatory activity, is the key component of the protein C pathway. Recent studies have implicated the soluble form of EPCR (sEPCR) in Wegener's granulomatosis, Behçet's disease, and systemic lupus erythematosus. The aim of this study was to evaluate the levels of sEPCR in HSP children. Twenty-two children with HSP and 17 healthy children were included. We found no significant differences (P > .05) between patient and control groups in the levels of von Willebrand factor and thrombomodulin. The median sEPCR values in the HSP group were lower than the control group (79 vs. 102 ng/mL, respectively) (P > .05). The mean sEPCR value in HSP patients with severe abdominal pain was lower than without (88.8 ± 54.9 vs. 108.2 ± 66.3 ng/mL, respectively) (P > .05). In addition, the mean IL-6 serum levels were significantly elevated in HSP patients during the acute stage of HSP (2.1 ± 1.7 vs. 1.5 ± 1.2 pg/mL, P = .038). We also observed a slight negative correlation between the levels of sEPCR and IL-6 (R = -.135, P > .05). To our knowledge, this was the first study to analyze sEPCR levels in HSP. Our results did not conclusively identify a direct role of sEPCR in HSP, but our findings warrant further investigations, especially in severe HSP cases characterized by gastrointestinal bleeding or renal involvement.

Clinical characteristics of paediatric neuro-Behçet's disease: a single tertiary centre experience.

OBJECTIVES: To investigate the demographic and neurological features and treatment modalities of neuro-Behçet's disease (NBD) in children, to share our experiences and to summarise the literature. METHODS: We retrospectively reviewed the medical records of Behçet's disease (BD) patients who attended our paediatric rheumatology department between December 2005 and October 2013. Five patients had the diagnosis of NBD. Initial neurological presentation, clinical BD presentation, magnetic resonance imaging pictures of those five patients was recorded. RESULTS: A total of 18 patients were diagnosed with BD. Among BD patients five of them were identified with NBD (27.8%). The mean age of NBD patients at the time of diagnosis was 12.4 years (range 5.5-15 years). The mean follow-up time after the neurological involvement was 5.2 years (range 0.5-14). In two cases neurological involvement occurred at the same time with the onset of other clinical findings of BD (40%). Both of these patients had parenchymal involvement. Three patients were admitted with headache as the initial neurological symptom. They revealed benign intracranial hypertension. One of them had cerebral venous sinus thrombosis (CVST). The other two had normal cranial magnetic resonance imagines. All patients received colchicine and steroid, two of them who had parenchymal involvement received also cytotoxic drugs. CONCLUSIONS: This study has shown that neurological symptoms can be the first manifestations of BD in children. Clinicians should be aware of this possibility and when a patient presents with neurological manifestations, it would be valuable to query the patient for the clinical features of Behçet's disease.

Juvenile polyarteritis nodosa associated with toxoplasmosis presenting as Kawasaki disease.

Polyarteritis nodosa (PAN) is a vasculitis characterized by inflammatory necrosis of medium-sized arteries. Juvenile PAN and Kawasaki disease (KD) both cause vasculitis of the medium-sized arteries, and share common features. They have overlapping clinical features. Treatment should be managed according to the severity of symptoms and persistence of clinical manifestations. Herein is described the case of a 14-year-old boy first diagnosed with KD, who then fulfilled the criteria for juvenile PAN due to the development of severe myalgia, persistent fever, polyneuropathy and coronary arterial dilatation. He also had acute toxoplasmosis at the onset of vasculitis symptoms. The final diagnosis was of juvenile PAN associated with toxoplasmosis infection. Toxoplasma infection can be considered as an etiological agent for PAN and other vasculitis syndromes. Awareness of toxoplasmosis-related PAN facilitates early diagnosis, and instigation of appropriate treatment.

Right nutcracker syndrome associated with left-sided inferior vena cava, hemiazygos continuation and persistant left superior vena cava: a rare combination.

The term nutcracker syndrome refers to compression of left renal vein between aorta and superior mesenteric artery causing renal venous hypertension. Right nutcracker syndrome associated with a left-sided inferior vena cava is an extremely rare anomaly. Reported two cases in English literature were diagnosed by ultrasonography and computed tomography angiography in adulthood. Herein, we present a case of right nutcracker syndrome with left-sided inferior vena cava and hemiazygos continuation in a 12-year-old girl.