Notes

[Treatment involving advanced epithelial ovarian growth. Hospital Italiano p Buenos Aires experience].
Açarı C, Ünsal E, Hakgüder G, Soylu A, Özer E. Pediatric mesenteric panniculitis three cases and a review of the literature. Turk J Pediatr 2019; 61 798-803. Mesenteric panniculitis is an inflammatory and fibrotic process in the mesenteric adipose tissue with unknown etiology. It is rarely seen in general, particularly in children. Etiology is unknown, and pathophysiology is not clear. Factors that trigger the disease are malignancy, tuberculosis, trauma, medications and past surgical interventions. There is no pediatric case series in the literature except single case reports. This paper consists of 3 cases The first case is a 5-month-old girl, the youngest patient in the literature, who was referred to a pediatric surgeon with vomiting and abdominal distention. She had diffused intraabdominal fluid and mesenteric panniculitis documented by perioperative biopsy. The second case had acute abdominal pain with perforated appendicitis, who eventually had mesenteric panniculitis in the evaluation of the pathological specimen. The last case had a diagnosis of polyarticular juvenile idiopathic arthritis (JIA), successfully treated with etanercept, and has been in remission for 2 years. Interestingly, in one of her routine visits, she had pallor, anemia and renal failure. Bilateral hydronephrosis was detected. Magnetic resonance imaging (MRI) of the abdomen revealed retroperitoneal fibrosis, and mesenteric panniculitis was the histopathological diagnosis.Sakarya Güneş A, Maraş Genç H, Uyur Yalçın E, Yılmaz V, Saruhan Direskeneli G, Kara B. Acute ophthalmoparesis and persistent mydriasis expanding the clinical spectrum of anti-GQ1b positive cranial neuropathy in a 5.5-year-old girl. Turk J Pediatr 2019; 61 794-797. Acute ophthalmoparesis without ataxia (AO) is an atypical form of Miller- Fisher syndrome (MFS) and is rare in children. Anti-GQ1b antibodies can be detected in patients with AO, as in MFS. A 5.5-year-old girl had total ophthalmoparesis, blurred vision, ptosis, diplopia and mydriasis non-reactive to light or near stimuli with preserved consciousness and deep tendon reflexes. She had no ataxia. Cerebrospinal fluid (CSF) examination and cranial MRI were normal. Serum antiGQ1b antibodies were positive. She was diagnosed with AO and intravenous Immunoglobulin (IVIG) was ordered, 400 mg/ kg/day, for 5 days. Ophthalmoparesis and blurred vision improved in a few weeks. https://www.selleckchem.com/products/bms-986365.html At the end of the first year, mydriasis still persisted, but improved and became responsive to near stimuli. Pupillary involvement may be seen in approximately 50% of MFS patients, and improvement in a few weeks or months has been reported in adults. Our case shows the expanding clinical spectrum of anti-GQ1b positive cranial neuropathy as early-onset AO and prolonged mydriasis more than one year.Kurnaz E, Savaş Erdeve Ş, Özgür S, Keskin M, Özbudak P, Çetinkaya S, Aycan Z. Congenital long-QT syndrome in type 1 diabetes a unique association. Turk J Pediatr 2019; 61 791-793. In contrast to acquired long QT syndrome (LQTS), congenital LQTS is a relatively rare channelopathy with an incidence of 1/2,500. We describe a patient found to have a prolonged QTc in the setting of newly diagnosed Type 1 DM. To the best of our knowledge, this unique association has not been previously reported. Currently, it is shown that glucose ingestion aggravated cardiac repolarization disturbances in LQT2 patients and prolonged the cardiac repolarization phase in healthy controls. Our case presented to the hospital with syncope after increased glucose level. Therefore, it seems that increased glucose level may have prolonged QTc interval and aggravated cardiac repolarization disturbances in the presented case. By this report, we want to emphasize the importance of hyperglycaemia in congenital LQTS.Paç Kısaarslan A, Sözeri B, Baştuğ F, Gündüz Z, Yel S, Nalçacıoğlu H, Şahin N, Özdemir Çiçek S, Poyrazoğlu H, Düşünsel R. Elemental mercury intoxication in 7 patients admitted to a pediatric rheumatology clinic. Turk J Pediatr 2019; 61 786-790. Mercury (Hg) is a toxic heavy metal that can be classified into three groups; organic (methyl), inorganic (mercuric), and elemental (metallic) mercury(Hg0). Mercury intoxication occurs mostly with the elemental form which can potentially damage the function of any organ, or any subcellular structure. The target organ of mercury is the brain, but peripheral nerve function, renal function, immune function, endocrine and muscle function, and several types of dermatitis have been described. We present 7 patients admitted to a pediatric rheumatology clinic with severe extremity pain. One of the patients had acrodynia, two of them had hypertension, two of them had tubulopathy, and three of them had neuropathy. The treatments were Dimercaptosuccinic acid and metalcaptase. In this report, we emphasize that mercury intoxication should be kept in mind with unexplained extremity pain. Timely diagnosis and treatment may prevent severe morbidity and mortality.Nazmy N, Elhady G, Refaat E, Kholeif S. Familial reciprocal non robertsonian translocation t(14;22) resulting in 22q11.2 deletion syndrome. Turk J Pediatr 2019; 61 780-785. We report the clinical and genetic characterization of 2 cousins sharing the same chromosomal anomaly; a 22pter-q11.2 deletion and a 14pter-q13 duplication due to an unusual familial reciprocal non robertsonian translocation between 2 acrocentric chromosomes t(14;22)(q13;q11.2), the mother of patient 1 was the first cousin of the father of patient 2. Fluorescent in situ hybridization confirmed the cytogenetic results. The patients showed dysmorphic features and developmental delay with evident intrafamilial phenotypic variability. Reciprocal non robertsonian translocation is a rare event, and has not been reported in patients with 22q11.2 deletions. The mechanism responsible for this rare type of translocation is discussed herein.Gentile F, Martino M, Santangelo L, Giordano P, Torres DD, Carbone V, Di Palma AM, Rossini M, Gesualdo L, Giordano P, Giordano M. From Uganda to Italy a case of nephrotic syndrome secondary to Plasmodium infection, Quartan malarial nephropathy and kidney failure. Turk J Pediatr 2019; 61 776-779. Malaria (M), the first parasitic infection, is sometimes associated with nephrotic syndrome (NS) in tropical areas. Kidney involvement during quartan malaria is represented by immune-complex mediated glomerulonephritis (GN). Generally, NS develops several weeks after onset of quartan fever and its clinical course proceeds slowly to end-stage kidney disease (ESKD) even after eradication of the infection. We describe a case of Plasmodium malariaeassociated nephrotic syndrome and chronic proliferative glomerulopathy in a boy from Uganda. Renal biopsy revealed chronic proliferative GN with capillary wall thickening producing a double contour, segmental sclerosis and tubular atrophy. Blood Giemsa smear contained rare ring-form trophozoites and gametocytes of Plasmodium spp.
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