Evaluation of early period changes in intraocular pressure, ocular biometry and anterior segment after upper eyelid blepharoplasty.

PURPOSE: To investigate the acute effects of upper eyelid blepharoplasty on intraocular pressure (IOP) and ocular biometric parameters. METHOD: This prospective cross sectional study examined the eyes of 49 patients with dermatochalasis. Following a detailed ophthalmological examination, corneal topography was used to evaluate the eyes mesopic and photopic pupil diameter, anterior chamber depth, and corneal astigmatism on the day of surgery and on days first and seventh postoperatively. Ocular biometry was used to assess axial length and intraocular lens power. Goldmann applanation tonometry was used to measure intraocular pressure. RESULTS: Forty-nine eyes (26 males and 23 females) of 49 patients aged between 44 and 76 years (mean 61 ± 7.9) were included in our study. The mean anterior chamber depth (ACD) was 2.90 ± 0.37 mm preoperatively, 3.00 ± 0.29 mm at postoperative day first, and 3.04 ± 0.29 mm at postoperative day seventh, and the increase in anterior chamber depth was statistically significant (p < 0.001). The mean astigmatism values were measured as preoperative 0.73 ± 0.69 D, postoperative first day 0.93 ± 0.81 D, and postoperative seventh day 1.26 ± 0.90 D. The increase in astigmatism values was statistically significant (p < 0.001). The mean pupil diameter measurements in the mesopic environment were 4.20 ± 0.61 mm preoperatively, 4.40 ± 0.59 mm on the first postoperative day, and 4.39 ± 0.57 mm on the seventh postoperative day, and there was a statistically significant difference between the three measurements (p = 0.03). The mean IOP measurements of the patients were 15.91 ± 3.51 mmHg preoperatively, 16.81 ± 3.36 mmHg on the first postoperative day, and 16.97 ± 3.13 mmHg on the seventh postoperative day. The increase between these three measurements was statistically significant (p = 0.013). CONCLUSION: This study includes important insights into the potential acute phase impact of blepharoplasty surgery on ocular findings. Patients undergoing upper eyelid surgery should be informed about the possible change in ocular biometric parameters, intraocular pressure, and pupil diameter.

Evaluation of Patients with COVID-19 Followed Up in Intensive Care Units in the Second Year of the Pandemic: A Multicenter Point Prevalence Study.

OBJECTIVE: A 1-day point prevalence study was planned to obtain country data by determining the clinical characteristics, follow-up and treatment methods of coronavirus disease 2019 (COVID-19) cases that required intensive care unit (ICU) treatment in the second year of the pandemic. MATERIAL AND METHODS: All patients who were hospitalized in the ICUs due to COVID-19 between March 11, 2022, 08.00 am, and March 12, 2022, 08.00 am, were included in the study. Demographic characteristics, intensive care and laboratory data, radiological characteristics, and follow-up results of the patients were recorded. RESULTS: A total of 811 patients from 59 centers were included in the study, 59% of the cases were male, and the mean age was 74 ± 14 years. At least one comorbid disease was present in 94% of the cases, and hypertension was the most common. When ICU weight scores were examined, Acute Physiology and Chronic Health Evaluation-II: 19 (15-27) and Sequential Organ Failure Assessment: 7 (4-10) were seen. Sepsis was present in 37% (n = 298) of cases. PaO2/FiO2 ratios of the patients were 190 the highest and 150 the lowest and 51% of the cases were followed via invasive mechanical ventilation. On the study day, 73% bilateral involvement was seen on chest x-ray, and ground-glass opacities (52%) were the most common on chest tomography. There was growth in culture in 40% (n = 318) of the cases, and the most common growth was in the tracheal aspirate (42%). CONCLUSION: The clinical course of COVID-19 is variable, and ICU follow-up was required due to advanced age, comorbidity, presence of respiratory symptoms, and widespread radiological involvement. The need for respiratory support and the presence of secondary infection are important issues to be considered in the follow-up. Despite the end of the second year of the pandemic and vaccination, the high severity of the disease as well as the need for follow-up in ICUs has shown that COVID-19 is an important health problem.

Does occupational radiation exposure affect retinal nerve fiber thickness?

PURPOSE: To evaluate the thickness of peripapillary nerve fibers in radiation-exposed healthcare workers. METHODS: The study included 60 radiation-exposed healthcare workers and 60 healthy control groups. SD-OCT was used to measure mean peripapillary RNFL thickness along with peripapillary RNFL thickness in the upper, lower, nasal, and temporal quadrants. RESULTS: The mean age of the subjects participating in the study was 37 ± 6.3 (range 25-50) in the radiation-exposed group and 38 ± 4.9 (range 26-48) in the control group. The mean duration of exposure to radiation in healthcare workers was 11 ± 5 years (range 5-27). While the mean total rim thickness was 94.25 ± 8.2 in the radiation-exposed group, it was 102.8 ± 7.4 in the control group (p < 0.001). The mean superior rim thickness was 123.93 ± 15.13 in the radiation-exposed group, while it was measured as 129.75 ± 14.64 in the control group (p = 0.34). While the mean inferior rim thickness was 110.88 ± 13.43 in the group exposed to radiation, it was 130.08 ± 13.44 in the control group (p < 0.001). The mean nasal rim thickness was 70.25 ± 9.50 in the group exposed to radiation, while it was 75.38 ± 13.77 in the control group (p = 0.46). While the mean temporal rim thickness was 71.77 ± 8.73 in the group exposed to radiation, it was measured as 75.78 ± 13.15 in the control group (p = 0.52). Significant thinning of nerve fiber thickness was found statistically significant in all and especially the inferior quadrants of the healthcare workers exposed to radiation compared to the control group. CONCLUSION: After at least 5 years of radiation exposure, the thickness of the peripapillary retinal nerve fibers may decrease in healthcare workers.

Evaluation of the effects of duloxetine treatment on anterior segment parameters by optical coherence tomography.

PURPOSE: To evaluate the effects of Duloxetine on anterior segment parameters and intraocular pressure (IOP) in open angle eyes. METHODS: 38 eyes of 38 patients with fibromyalgia who had open or wide open angles according to the Shaffer classification. Anterior segment optic coherence tomography was performed before and after (month 3) Duloxetine treatment. IOP, central corneal thickness (CCT), corneal endothelial cell density (CECD) and anterior chamber depth (ACD) were also recorded and evaluated. RESULTS: No statistically significant difference was determined in IOP, CCT and CECD (p > 0.05). However, a statistically significant decrease was determined in both the temporal and nasal anterior chamber angle, angle opening distance, nasal trabecular-iris space area and ACD values between the baseline and month 3 (p < 0.001). DISCUSSION: We think that the short term use of Duloxetine does not lead to clinically significant changes despite their statistically significant effects on the anterior chamber parameters.

Effect of Intracameral Carbachol and Epinephrine Use on Choroidal Thickness After Uncomplicated Phacoemulsification.

BACKGROUND Safety concerns about drugs used intracamerally during cataract surgery have been the subject of many studies. In this study, the effect of using intracameral carbachol and epinephrine on choroidal thickness was evaluated. MATERIAL AND METHODS This prospective interventional study included 81 eyes of 81 patients undergoing cataract surgery. During cataract surgery, intracameral carbachol was administered to 27 eyes, intracameral epinephrine was administered to 20 eyes, and 34 eyes were the control group. Macular choroidal thickness measurement was performed before, 1 day, and 1 week after phacoemulsification surgery in all patients using optical coherence tomography. RESULTS Subfoveal choroidal thickness was significantly reduced at day 1 and week 1 in the group receiving intraoperative carbachol compared with preoperative measurement (P=0.016). In addition, choroidal thickness in the 500 µm nasal fovea was significantly reduced in the carbachol group at 1st week compared to the preoperative measurement (P=0.008). There was no significant difference in postoperative subfoveal thickness in the intraoperative epinephrine group and control group (P=0.179 and P=0.953, respectively). Choroidal thickness at 1000 µm nasal fovea was significantly higher in the epinephrine group at postoperative 1st day than preoperative and postoperative 1st week values (P=0.009). CONCLUSIONS The use of intracameral epinephrine caused an increase in choroidal thickness 1000 µm nasal of the fovea, while intracameral carbachol caused thinning in the subfoveal and 500 µm nasal quadrant. Intracameral drug administration during cataract surgery may be associated with posterior segment complications.

Salbutamol Delivered by Jet Nebulizer: Closed System Design and Impact of a Model Biofilm.

BACKGROUND: Aerosol therapy is commonly used by intensivists during invasive mechanical ventilation. More information is needed to optimize outcomes. The first aim of this study was to assess the deposition of salbutamol on components of a closed mechanical ventilation system, both in the presence and absence of biofilm generated by Acinetobacter baumannii. The second aim was to evaluate the deposition of salbutamol, using a single dose and a double dose, delivered via a jet nebulizer placed between the flexible tube and the heat and moisture exchanger. METHODS: A mechanical ventilator was connected to a standard system, and a jet nebulizer was placed between the heat and moisture exchanger and the flexible tube. Clinical isolates of A. baumanii were used to generate a biofilm layer on the endotracheal tube. Two amounts of salbutamol were delivered via the jet nebulizer. An analytical liquid chromatography tandem mass spectrometry method was developed to evaluate salbutamol deposition. RESULTS: The presence of a biofilm on the endotracheal tube had no impact on salbutamol deposition (P = .83). There was no difference in surface deposition of salbutamol on component parts of the closed system in a comparison of a single dose and a double dose delivered via a jet nebulizer. CONCLUSIONS: Our findings indicate that an A. baumannii biofilm had no impact on the extent of salbutamol deposition. Salbutamol deposition was comparatively low and could be delivered without removal of the heat and moisture exchanger.

Evaluation of the effects of selective laser trabeculoplasty on anterior segment parameters by anterior segment optical coherence tomography.

To prospectively examine the effects of selective laser trabeculoplasty (SLT) on the anterior chamber angle (ACA) and its related parameters using anterior segment-optic coherence tomography (AS-OCT). Fifty eyes of 50 patients with primary open angle glaucoma (POAG) and ocular hypertension were included in the study. AS-OCT was performed before SLT application, immediately after and at 1 day and 1 month. Intraocular pressure (IOP), central corneal thickness (CCT) and anterior chamber depth (ACD) were also recorded and evaluated. No statistically significant difference was determined in ACA and other AS-OCT parameters (AOD, angle opening distance at 500 and 750 mm; TISA, trabecular-iris space area at 500 and 750 mm) before and 1 day after SLT application (p > 0.05). However, a statistically significant increase was determined in both the temporal and nasal ACA, AOD and TISA values between the baseline and day 30 (p < 0.001). No statistically significant change was observed in the CCT or ACD values (p > 0.05). SLT resulted in an increase in ACA, AOD and TISA when evaluated using AS-OCT. We think that this study provides a different perspective concerning the effects of SLT in the angle region and the involved mechanism.

The effects of intravitreal H2 S application on apoptosis in the retina and cornea in experimental glaucoma model.

One of the most important causes of visual loss (blindness) is glaucoma, which occurs due to the degeneration of the ganglion cells in retina. It has been shown that hydrogen sulphide (H2 S) acts an antioxidant, neuroprotective and neuromodulator and provides protection against oxidative stress and apoptosis. This study aims to examine through which apoptotic pathway H2 S acts in experimental glaucoma model. Twenty-two male wistar albino rats were used in this study. Group 1 (n = 6, control group): Intravitreal saline was given in the third week without inducing ocular hypertension (OHT) with laser photocoagulation. Group 2 (n = 8): After the induction of OHT with laser photocoagulation, intravitreal saline was given in the third week. Group 3 (n = 8): After the induction of OHT with laser photocoagulation, intravitreal H2 S's donor sodium hydrosulphide (NaSH) 100 nmol/L was given in the third week. At the end of the 6th week, the eyes of the rats were sacrified under anaesthesia and extracted and then routine tissue follow-up was undertaken. Besides haematoxylin & eosin (H&E) staining, Bax, Bcl-2, p53 and caspase-3 activation were examined immunohistochemically in the retina and the cornea. This showed that ocular hypertension caused apoptosis through the intrinsic pathway, due to Bax and caspase-3 activation, in both retina and cornea, and that this led to DNA damage due to p53 activation. Also, we found that H2 S exposure in glaucoma distinctly suppressed Bax, caspase-3 and p53 activations in retina but that it has a limited effect on the cornea. According to these results, glaucoma caused apoptosis in the retina through intrinsic pathway, and the damage to the retina could be compensated partially by H2 S but would have limited on the cornea.

Evaluation of the optic nerve and scleral-choroidal-retinal layer with ultrasound elastography in glaucoma and physiological optic nerve head cupping.

AIM: To evaluate the strain ratio of the optic nerve and retina-choroid-sclera (RCS) layers in individuals with physiological optic disc cupping (PC) and glaucoma patients using strain elastography. MATERIAL AND METHODS: We evaluated 56 eyes of 56 subjects (20 eyes with glaucoma, 19 eyes with PC, and 17 normal eyes). The strain ratio of orbital fat to optic nerve (SROFON) was calculated as the ratio of the optic nerve to intraconal fat tissue and the strain ratio of orbital fat to retina-choroid-sclera (SROFRCS) was calculated as the ratio of RCS layers to intraconal fat tissue. RESULTS: SROFON was 0.92 in the control group, 1.07 in the PC group and 1.6 in the glaucoma group and a statistically significant difference was present between the three groups (p<0.05). SROFRCS had no statistically significant difference between the three groups. CONCLUSIONS:  SROFON values could contribute to the differentiation of the patients with glaucoma and PC.

The evaluation of retinal and choroidal structural changes by optical coherence tomography in patients with chronic obstructive pulmonary disease.

PURPOSE: To evaluate the retinal nerve fiber layer (RNFL) thickness, ganglion cell-inner plexiform layer (GCL+) thickness, and macular choroidal thickness (mCT) in patients with chronic obstructive pulmonary disease (COPD) using spectral domain optical coherence tomography (SD-OCT). METHODS: A total of 79 COPD patients and 71 age- and sex-matched healthy individuals were enrolled in this prospective cross-sectional study. The patients were divided into two subgroups (with mild-to-moderate COPD and severe COPD) using spirometric data suggested by the Global Initiative for Chronic Obstructive Lung Disease guideline. The RNFL, GCL+, and mCT were compared between groups. RESULTS: The average and nasal RNFL thicknesses in the COPD group were significantly lower than those in control group (p = 0.023 and 0.027 respectively). Statistically significant reductions in average thickness and in those of all six wedge-shaped GCL+ sectors were evident in the COPD group compared with control group and were more marked in patients with severe COPD. The other RNFL data did not differ significantly between COPD and control groups. The mCT was somewhat thinner at all the measured locations in COPD group compared with control group, but statistically significance was not attained. CONCLUSIONS: The study results revealed significant average, nasal RNFL, global GCL+ loss, and a nonsignificant choroidal thinning in patients with COPD compared to healthy subjects. The eye seems to be one of the affected tissues during the natural course of the COPD.

Vitamin B12 deficiency evaluation and treatment in severe dry eye disease with neuropathic ocular pain.

PURPOSE: This study aims to understand the effect of vitamin B12 deficiency on neuropathic ocular pain (NOP) and symptoms in patients with dry eye disease (DED). METHODS: Patients with severe DED (without receiving topical artificial tears treatment) and ocular pain were enrolled (n = 90). Patients with severe DED and vitamin B12 deficiency (group 1, n = 45) received parenteral vitamin B12 supplement + topical treatment (artificial tears treatment + cyclosporine), and patients with severe DED and normal serum vitamin B12 level (group 2, n = 45) received only topical treatment (artificial tears treatment + cyclosporine). Patients were evaluated by the ocular surface disease index (OSDI) questionnaire, 3rd question (have you experienced painful or sore eyes during last week?) score of OSDI as a pain determiner and pain frequency measure), tear break up time (TBUT), and Schirmer's type 1 test. We compared the groups' OSDI, TBUT, and Schirmer's test recordings at the first visit and after 12 weeks retrospectively. RESULTS: The OSDI score, 3rd OSDI question score, TBUT, and Schirmer's test results improved after 12 weeks (p < 0.001 for each group). The mean vitamin B12 level at enrollment was 144.24 ±43.36 pg/ml in group 1 and 417.53 ±87.22 pg/ml in group 2. The mean vitamin B12 level in group 1 reached to 450 ±60.563 pg/ml after 12 weeks of treatment. The mean score changes between the groups were not statistically significant; however, the decrease in the OSDI questionnaire score (-30.80 ±5.24) and 3rd OSDI question score (-2.82 ±0.53) were remarkable in group 1 (Table 2). The mean TBUT increase was +7.98 ±2.90 s and Schirmer's test result increase was +12.16 ±2.01 mm in group 1. The mean TBUT increase was +6.18 ±1.49 s and Schirmer's test result increase was +6.71 ±1.47 mm in group 2. CONCLUSIONS: These findings indicate that vitamin B12 deficiency is related with NOP. It may be important to consider measuring the serum vitamin B12 level in patients with severe DED presenting with resistant ocular pain despite taking topical treatment.

Effects of Molsidomine on Retinopathy and Oxidative Stress Induced by Radiotheraphy in Rat Eyes.

PURPOSE: To determine the role of Molsidomine in preventing radiation-induced retinopathy after head and neck region irradiation of rats with a single radiation dose of 15 Gy. MATERIALS AND METHODS: Male Wistar albino rats were randomly grouped into five as follows: (1) control group rats, which were applied through an intraperitoneal (i.p.) vehicle without radiotherapy (RT); (2) RT group rats received a single dose of 15 Gy irradiation and after daily 0.1 ml vehicle i.p. for 5 consecutive days; (3) molsidomine (MOL) group rats were treated for 5 consecutive days by i.p. with 4 mg/kg/day MOL; (4) irradiation plus MOL group (RT+MOL) rats received irradiation and after 10 days single daily i.p. dose of MOL for 5 consecutive days; and (5) MOL+RT group rats were treated for 5 consecutive days by i.p. with MOL before RT. At the end of the work the rats were sacrificed under high-dose anesthesia on the 16th day and then eye tissues were taken for histopathological, immunohistochemical (caspase-3), and biochemical analyses (superoxide dismutase [SOD], glutathione peroxidase [GSH], and malondialdehyde [MDA]). RESULTS: RT significantly decreased both the content of GSH and the activity of SOD, and significantly increased the production of MDA level in the rat eyes. MOL treatment significantly increased the SOD and GSH levels and significantly decreased the MDA production (p < 0.0001). In addition, RT significantly increased the number of ganglion cells (GCs; p = 0.001), whereas especially pretreatment with MOL improved (p = 0.013). RT led to significant retinopathy formation, and MOL therapy protected the retina from radiation-induced retinopathy (p < 0.0001). CONCLUSIONS: We suggest that MOL is a powerful antioxidant and free radical scavenger that prevents the rat eyes from radiation-induced retinopathy and oxidative stress.

Ganciclovir ophthalmic gel treatment shortens the recovery time and prevents complications in the adenoviral eye infection.

The purpose of this study was to determine the effectiveness of ganciclovir ophthalmic gel (GOG) in the treatment of adenoviral eye infection (AEI) by looking at the effect of the drug on shortening recovery time, preventing transmission, reducing sequelae, and on complications such as corneal infiltrates and conjunctival pseudomembranes. 200 patients' examination records were evaluated retrospectively. Patients who were within the first 3 days of AEI were divided into two groups: Group 1 with 100 patients who used artificial tears as treatment, and Group 2 with 100 patients who used GOG plus artificial tears (GAT). All patients underwent an eye examination by the same ophthalmologist on the 1st, 5th, 10th, and 15th day after treatment. Using the examination records, variables were compared using SPSS 22.0. There was a statistically significant difference between Groups 1 and 2. Group 2 showed better and faster response to treatment. There was less transmission to the contralateral eye and environment, and less formation of corneal subepithelial infiltrate and conjunctival pseudomembrane in Group 2. Only three patients in Group 2 had corneal involvement. A comparison of each group pre-treatment and during treatment revealed improved signs and symptoms in Group 2 (p < 0.005). The study showed a trend toward more rapid improvement, less corneal and conjunctival involvement, and less transmission to the contralateral eye and environment in the GAT group. These results need to be confirmed by additional studies.

Histopathological and ophthalmoscopic evaluation of apocynin on experimental proliferative vitreoretinopathy in rabbit eyes.

The aim of the current study was to evaluate the effect of apocynin (APO) on the development of proliferative vitreoretinopathy (PVR). New Zealand-type male rabbits were randomly grouped into three as follows: (1) Sham group rabbits which were applied intraperitoneal (i.p.) vehicle without PVR; (2) PVR group rabbits where PVR was created and an i.p. vehicle was administered for 21 successive days; (3) PVR + APO group rabbits where PVR was created and i.p. APO was administered for 21 successive days. Fundus examination was conducted with an indirect ophthalmoscope before starting the experiments and at each visit afterwards. At the end of the work, the rabbits were sacrificed under high-dose anesthesia and then eye tissues were taken for histopathological analyses. In the PVR + APO group, histopathologic and ophthalmoscopic examination revealed significant decrease in PVR formation. As the result, it has been observed that APO at least partially inhibits PVR formation.

Assessment of pulmonary artery pressure and right ventricular function in children with adenotonsillar hypertrophy using different parameters.

OBJECTIVE: Our aim was comparison of preoperative and postoperative right ventricular functions of children with adenotonsillar hypertrophy (ATH) who have findings of upper airway obstruction, using new echocardiographic parameters. METHODS: Forty-one children who have admitted to our hospital with symptoms suggestive of upper airway obstruction, whose history and physical examination findings suggest upper airway obstruction and who have undergone adenoidectomy/adenotonsillectomy and 40 healthy children, all of whom between 2 and 12 years of age, were included in the study. Patient group was evaluated by pulsed wave tissue Doppler echocardiography as well as with conventional echocardiography before the operation and 6 months after the operation. RESULTS: Of 41 children in study group, 26 (63.4%) had adenotonsillectomy and 15 (36.6%) had adenoidectomy. Tricuspid annular plane systolic excursion (TAPSE) was significantly lower in preoperative group compared to control group (18.46±1.67, 19.77±1.62; p=0.000, respectively). Myocardial performance index (MPI) was significantly higher in preoperative group than postoperative and control group (0.40±0.07, 0.36±0.06, 0.35±0.07; p=0.032, respectively). Tricuspid isovolumic acceleration (TIVA) was significantly lower in preoperative group than preoperative and control group (2.97±0.8, 3.43±0.7, 3.43±0.9; p=0.020, respectively). Disappearance of this difference was found between postoperative and control groups (p=0.984). Pulmonary acceleration time (PAcT) was found to be significantly lower in preoperative group compared to postoperative and control group (109.68±18.03, 118.93±17.46, 120.0±14.07; p=0.010, respectively). Mean pulmonary artery pressure (mPAP) was significantly higher in preoperative group than control group (29.64±8.11, 24.95±6.33; p=0.010, respectively). In postoperative group mPAP was found to be similar to control group (25.48±7.85, 24.95±6.33; p=0.740, respectively). CONCLUSIONS: TAPSE, PAcT, MPI and TIVA are useful markers for evaluation of preoperative and postoperative ventricular function in children with ATH who have findings of upper airway obstruction. We think that using these practical and easy-to perform parameters may be relevant for evaluation and postoperative follow-up of patients with ATH who have findings of upper airway obstruction. Besides adenotonsillectomy is a beneficial treatment option for these patients.

External ophthalmomyiasis caused by Oestrus ovis misdiagnosed as bacterial conjunctivitis.

Ophthalmomyiasis is the infestation of the eye by maggots or bots of certain flies which is mostly caused by Oestrus ovis. The most common form of ophthalmomyiasis is external that only the external surface of the eye is involved. Symptoms of external myiasis are nonspecific so it can be misdiagnosed as any other conjunctivitis if the doctors do not take myiasis into consideration. We report a case series of external ophthalmomyiasis. All the patients complained of mild foreign body sensation, redness, watery eyes and some of them had lid swelling. Larvae on the external surface of the patients must be removed under dim light. Larvae can hide in the fornices of the patients as happened in our two cases. Because of our lack of experience about ophthalmomyiasis, we misdiagnosed the first patient as bacterial conjunctivitis. Ophthalmomyiasis may be difficult to detect so ophthalmologists must keep in mind the possibility of ophthalmic myiasis especially in rural regions.

Clarithromycin-Induced Long QT Syndrome: A Case Report.

Long QT syndrome develops for a number of reasons. The number of non-antiarrhythmic drugs reported to induce QT interval prolongation with or without torsade de pointes continues to increase. Clarithromycin is a macrolide antibiotic being increasingly used for the treatment of atypical pneumonia. In this paper, we describe a patient who developed long QT prolongation syndrome after receiving clarithromycin for the treatment of atypical pneumonia.

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.

A patient with Bartter syndrome accompanying severe growth hormone deficiency and focal segmental glomerulosclerosis.

Bartter syndrome is a rare autosomal recessive, salt-losing disorder characterized by hypokalemic hypochloremic metabolic alkalosis. A 10-year-old boy had severe growth retardation (height standard deviation score -8.15). He had a thin, triangular face, prominent ears and forehead, and big eyes. Megacystis, bilateral hydroureteronephrosis, and residual urine were detected in ultrasonography, but there was no vesicoureteral reflux. Lumbosacral magnetic resonance (MR) showed posterior disc bulging at L4-5. Serum sodium and chloride levels were normal, but mild hypokalemia was overlooked initially. During follow-up, hypokalemic hypochloremic metabolic alkalosis developed, with high urinary chloride and potassium excretion (52 and 43 mEq/L, respectively). The patient, with renal salt loss, was thought to have classic Bartter syndrome due to absence of nephrocalcinosis, presence of persistent hypercalciuria and sensorineural deafness, and presence of relatively mild clinical and laboratory findings, except polyuria initially. The child was treated with indomethacin, spironolactone, and oral potassium in addition to growth hormone (GH). During treatment, he had considerable increase in weight and height compared with the period of GH therapy only. We present this case because, although growth retardation is a major feature of Bartter syndrome, associated GH deficiency is rarely reported in the literature. Diagnosis of Bartter syndrome was made later, as our patient was followed for megacystis and megaureter secondary to the neurogenic bladder and GH deficiency initially; and proteinuria associated with focal segmental glomerulosclerosis responded to treatment for Bartter syndrome.