Mucopolysaccharidosis type III (subtype IIIB) diagnosis as a spectrum disorder: A case report from Kosovo.

Mucopolysaccharidosis type IIIB (MPS IIIB), also known as Sanfilippo syndrome type B, is a metabolic disease caused by mutations in both alleles of the NAGLU gene encoding for the enzyme α-N-acetylglucosaminidase. A malfunction of this enzyme causes  inability to degrade heparan sulfate, which leads to accumulation of glycosaminoglycans in the cells. MPS IIIB is associated with different symptoms such as neurodegeneration, extreme hyperactivity, sleeping problems, aggressive behavior, reduced fear, and cognitive  deterioration. The condition is by now not curable. Here we describe a patient with MPS IIIB diagnosed at the age of 5 presenting with communication problems, motor dysfunctions, and speech and sleeping problems.Standard biochemical tests for neurodegenerative disorders and DNA analyses including NAGLU mutation screening were performed. We also did some psychological tests assessing the patient's communication skills and behavior. The patient was heterozygote for two mutations in the gene NAGLU (Y140C and Ser169fs). Thus, he suffered from MPS IIIB due to two mutations in the disease-causing gene.The patient presented with clear signs and symptoms of MPS IIIB with at least one of the two mutations affecting the α-N-acetylglucosaminidase protein function severely. Here we report the combination of a well-known and previously unreported mutation in the NAGLU gene; this could be dependent on geographical origin of the patient, which needs to be clarified by molecular studies of more MPS IIIB patients from Southeast Europe.

Targeted Treatment and Immunotherapy in High-risk and Relapsed/ Refractory Pediatric Acute Lymphoblastic Leukemia.

Acute lymphoblastic leukemia is the most frequent pediatric malignancy in children, comprising 30% of all pediatric malignancies; adult ALL comprises 5% of all ALL cases, which have a 186.6 per 1 million incidence. In pediatric ALL (pALL), on which this review focuses, approximately 1 in 285 children are diagnosed with cancer before the age of 20, and approximately 1 in 530 young adults between the ages of 20 and 39 years old is a childhood cancer survivor. The survival probability in pALL is now very high, approximately 80-90%. Thus, the most important is to improve supportive care and treatment based on relapse risk, optimally being based on the genetic feature of malignant cells. Improvements made by now are mainly the classifying of subgroups based on genetic characteristics such as aneuploidy or translocation and aligning them with treatment response. Relevant genetic changes in ALL pathogenesis are transcription regulators of lymphoid development (PAX5, IKZF1, EBF1, and LEF1) and/or coactivators (TBL1XR1 and ERG), lymphoid signaling (BTLA, and CD200 TOX), and tumor suppressor genes (CDKN2A, CDKN2B, RB1, and TP53). This review aims to summarize treatment strategies inhibiting tyrosine kinases, influencing different signaling pathways, BCL inhibitors, and anti-CD therapy (anti-cluster differentiation therapy) in pALL. CAR T-cell therapy (chimeric antigen receptors T-cell therapy) is under research and requires further development.

Correlation between level of vitamin D in serum and value of lung function in children diagnosed with bronchial asthma.

INTRODUCTION: Many authors in their research have suggested an association between vitamin D and asthma, but the results from these publications are sometimes confusing. AIM: Our aim was to assess the relationship between serum vitamin D and lung function in patients previously diagnosed with asthma. MATERIALS AND METHODS: The present study started in September 2019 and was completed in May 2020. All patients were diagnosed at the University Clinical Center-Prishtina, Kosovo. Spirometry was performed on children of ages 6-16 years old with a spirometer according to the recommendations of the American Thoracic Society. RESULTS: Of the 57 children who visited the University Clinical Center of Kosovo-Department of Pediatrics, 29 were diagnosed with asthma. The Spearman coefficient correlation showed statistical significance between vitamin D and body weight, and vitamin D and FEF75% at level 0.05. Other parameters did not show statistical significance with vitamin D, but such statistical significance was found in other parameters between asthma and healthy groups. CONCLUSIONS: Our data suggested that serum vitamin D level was insignificant for FVC%, FEV1%, Tiffeneau Index values, and PEF. Statistical significance was observed between vitamin D and body weight; vitamin D and FEF75% (p=0.05).

Case Report: Identification of likely recurrent CEP290 mutation in a child with Joubert syndrome and cerebello-retinal-renal features.

Background. Joubert syndrome (JS) is a rare autosomal recessive ciliopathy with an estimated prevalence of 1 in 100,000. JS is characterized by hyperpnoea, hypotonia, ataxia, developmental delay and various neuropathological abnormalities in the brain including cerebellar hypoplasia and cerebellar vermis aplasia. JS can also have variable multi-organ involvement, including the retina, kidneys, liver, and musculoskeletal system. Methods and Results. Here we report a clinical description of two-year-old girl presenting with breathing difficulties, hyperechoic kidneys with loss of corticomedullary differentiation. Brain magnetic resonance imaging revealed the typical molar tooth sign consistent with a clinical diagnosis of JS and retinal examination showed severe retinal dystrophy leading to blindness. Molecular genetic analysis using whole exome sequencing and Sanger sequence confirmation demonstrated a homozygous mutation (c.5493delA, p.(A1832fs*19) in CEP290 which segregated from either parent and was consistent with the multisystem ciliopathy phenotype. This precise variant has been described previously in 2 families from the Kosovar-Albanian region suggesting this allele is a recurrent mutation in this population. Conclusions. Mutations in CEP290 lead to multisystem ciliopathy syndromes and molecular genetic diagnostics of such cases allows precise diagnosis, screening of at risk relatives and appropriate management.

Joubert syndrome: Molecular basis and treatment.

Joubert syndrome (JS; MIM PS213300) is a rare genetic autosomal recessive disease characterized by cerebellar vermis hypoplasia, a distinctive malformation of the cerebellum and the so-called "molar tooth sign." Other characteristic features are hypotonia with lateral ataxia, intellectual disability/mental retardation, oculomotor apraxia, retinal dystrophy, abnormalities in the respiratory system, renal cysts, hepatic fibrosis, and skeletal changes. Such pleiotropic characteristics are typical of many disorders involving primary cilium aberrations, providing a significant overlap between JS and other ciliopathies such as nephronophthisis, Meckel syndrome, and Bardet-Biedl syndrome. This review will describe some characteristics of JS associated with changes in 35 genes, and will also address subtypes of JS, clinical diagnosis, and the future of therapeutic developments.

Mucopolysaccharidosis III: Molecular basis and treatment.

Mucopolysaccharidoses (MPSs) are known as rare genetic diseases which are caused by mutation in the enzyme heparin sulfate, which normally leads to degradation and accumulation of glycosaminoglycans in the cells. There are 11 types of MPSs, whereby neuropathy may occur in seven of them (MPS I, II, IIIA, IIIB, IIIC, IIID and VII). Accumulation of degraded heparin sulfate in lysosomes causes cellular dysfunction and malfunction of several organs. However, the exact molecular mechanism how protein degradation and storage leads to cellular dysfunction is not understood, yet. Nonetheless, several genetic and biochemical methods for diagnosis of MPSs are available nowadays. Here we provide an overview on known molecular basis of MPS in general, including enzyme defects and symptoms of MPS; however, the main focus is on MPS type III together with potential and perspective therapy-options.

A Case of Successfully Implanted Dual Chamber Pacemaker in a Young Patient with Dextrocardia and Sick Sinus Syndrome.

Dextrocardia is a rare congenital anomaly, whereas its association with sick sinus syndrome in young adults without accompanying heart abnormalities is exceptional. We report a case of a 38 year old female patient who was admitted to our hospital due to syncope as a consequence of sinus pauses up to 4.9 seconds. She was also diagnosed with situs inversus totalis, with mirror image dextrocardia. Pacemaker implantation was indicated. Under local anesthesia, from the left subclavian vein, the guide wire was passed through superior vena cava to the right atrium and ventricle without any obstacles. In conclusion, situs inversus totalis with mirror image dextrocardia may present in combination with sick sinus syndrome as early as the fourth decade of life. Implantation of pacemaker leads from the left subclavian vein appears accessible and safe in patients with dextrocardia with situs inversus (mirror image).

Type II Pleuropulmonary Blastoma in a 4 Month Old Infant with Negative Dicer1 Mutation on Next Generation Sequencing.

INTRODUCTION: Pleuropulmonary blastoma (PPB) is a rare, but aggressive tumor in the pediatric population. PPB is a dysontogenetic neoplasm of childhood that involves the lungs and/or pleura. Young relatives of children with PPB have an increased incidence of neoplasias and dysplasias. According to tumor tissue histopathology, PPB evolves from a cystic to solid state over time. PPBs can be sub-classified as type I (purely cystic), type II (having both cystic and solid elements), and type III (completely solid). Type II and type III tumors may be associated with metastasis, with the brain being the most common metastatic site. Due to the primitive nature of cells in the tumor mass, PPBs are very aggressive tumors that are resistant to therapy. The prognosis depends on the histopathology content and tumor type. Respiratory problems are the main complaint and diagnosis can be made only after additional examinations. Genetic relations through family members are associated with mutations in the DICER1 gene; between 60-80% of patients with PPBs are positive for DICER1 mutations. Mosaicism has also been reported. AIM: The aim was to present a case of a 4 month-old infant with type II PPB, who had a negative result for DICER1 mutation in next generation sequencing. To detail the clinical presentation of this patient, we present radiographic and ultrasound findings and results of histopathological analysis, as well as genetic and scintigraphic findings and chemotherapy treatment. CASE REPORT: Here we describe the genetic analysis of a patient with PPB who was negative for mutations in DICER1 and who had no relatives with disease. This patient underwent radical resection of the tumor and began therapy, but subsequently died after developing leukopenia and sepsis. CONCLUSION: This case provides an example of a patient with PPB who was negative for DICER1 mutation upon genetic analysis and emphasizes the potential for disease that does not involve mutation of this gene.

Celiac Disease in Kosovar Albanian Children: Evaluation of Clinical Features and Diagnosis.

BACKGROUND: Celiac disease is an immune-mediated disorder characterized by variable clinical manifestations, specific antibodies, HLA-DQ2/DQ8 haplotypes, and enteropathy. OBJECTIVES: The aim of this study was to present the clinical spectrum and patterns of celiac disease in Kosovar Albanian children. METHODS: A cross-sectional retrospective study was performed with Albanian children aged 0-18 years, treated for celiac disease in the Pediatric Clinic, University Clinical Center of Kosovo from 2005 to 2016. RESULTS: During the study period, 63 children were treated for celiac disease. The mean age at diagnosis was 5.5 years (SD ± 3.31). The mean age at celiac disease onset was 3.3 years (SD ± 2.02), while the mean delay from the first symptoms indicative of celiac disease to diagnosis was 2.2 years (SD ± 2.09). More than 70% of the patients were diagnosed in the first 7 years of life, mainly presented with gastrointestinal symptoms, while primary school children and adolescents mostly showed atypical symptoms (p<0.001). The classical form of celiac disease occurred in 78% of the cases. Sixty (95%) patients carried HLA-DQ2.5, DQ2.2 and/or HLA-DQ8 heterodimers, and only three of them tested negative. CONCLUSION: Kosovo, as the majority of developing countries, is still facing the classical form of celiac disease as the dominant mode of presentation; as a result, most children with other forms of the celiac disease remain undiagnosed. Physicians should be aware of the wide range of clinical presentations and utilize low testing thresholds in order to prevent potential long-term problems associated with untreated celiac disease.

HLA Haplotype Association with Celiac Disease in Albanian Pediatric Patients from Kosovo.

Genetic predisposition to celiac disease (CD) is strongly associated with the presence of HLA alleles in the individual genotype encoding HLA-DQ2 and/or HLA-DQ8 heterodimers. The main aim of this study was to analyze the HLA-A, -B, -DRB1, and -DQ allele and five-locus haplotype frequencies in 60 Albanian pediatric CD patients and 124 non-CD children from Kosovo. The most prevalent haplotype in patients was the ancestral AH 8.1 haplotype present in 22.5% of the cases compared to 2.8% of the controls (P < 0.0001). Additionally, two other haplotypes were also overrepresented in patients (HLA-A∗02~B∗50~DRB1∗07~DQA1∗02:01~DQB1∗02:02 and HLA-A∗68~B∗44~DRB1∗07~DQA1∗02:01~DQB1∗02:02). Analysis showed that 95.0% of CD patients and 43.3% of controls were carriers of HLA-DQ2 and/or HLA-DQ8 heterodimers. The most frequent CD-predisposing HLA-DQ haplotypes in patients were HLA-DQ2.5 (46.7%) and HLA-DQ2.2 (11.6%), while the most prevalent genotypes were HLA-DQ2.5/DQX (58.3%) and HLA-DQ2.5/DQ2.2 (20.0%). The frequency of the HLA-DQ8 heterodimer among CD patients (4.2%) compared to the control group (8.1%) was without statistical significance. The given data demonstrate differences in the distribution of HLA haplotypes among Albanian CD patients from Kosovo in comparison to other European and non-European populations, as well as provide additional population data to supplement the thus far undisputed importance of the role of HLA-DQ2 and HLA-DQ8 heterodimers in the development of CD.

The Influence of Type 1 Diabetes Mellitus on Dental Caries and Salivary Composition.

Diabetes mellitus is the most common chronic disease that affects the oral health. The aim of the study is to evaluate the dental caries, salivary flow rate, buffer capacity, and Lactobacilli in saliva in children with type 1 diabetes mellitus compared to the control group. Methods. The sample consisted of 160 children of 10 to 15 years divided into two groups: 80 children with type 1 diabetes mellitus and 80 children as a control group. Dental caries was assessed using the DMFT index for permanent dentition. Stimulated saliva was collected among all children. Salivary flow rate and buffer capacity were measured, and the colonies of Lactobacillus in saliva were determined. The observed children have answered a number of questions related to their dental visits and parents' education. The data obtained from each group were compared statistically using the chi-square test and Mann-Whitney U-test. The significant level was set at p < 0.05. Results. DMFT in children with type 1 diabetes was significantly higher than that in the control group (p < 0.001). Diabetic children have a low level of stimulated salivary flow rate compared to control children (0.86 ± 0.16 and 1.10 ± 0.14). The buffer capacity showed statistically significant differences between children with type 1 diabetes and control group (p < 0.001). Also, children with type 1 diabetes had a higher count and a higher risk of Lactobacillus compared to the control group (p < 0.05 and p < 0.001). Conclusion. The findings we obtained showed that type 1 diabetes mellitus has an important part in children's oral health. It appears that children with type 1 diabetes are exposed to a higher risk for caries and oral health than nondiabetic children.

Gallbladder ascariasis in Kosovo - focus on ultrasound and conservative therapy: a case series.

BACKGROUND: Ascaris lumbricoides is one of the most common intestinal infections in developing countries, including Kosovo. In contrast to migration to the bile duct, migration of the worm to the gallbladder, due to the narrow and tortuous nature of the cystic duct, is rare. When it does occur, it incites acalculous cholecystitis. CASE PRESENTATIONS: This case series describes a 16-month-old Albanian girl, a 22-month-old Albanian girl, a 4-year-old Albanian girl, and a 10-year-old Albanian boy. Here we report our experience with gallbladder ascariasis including clinical manifestations, diagnostic procedures, and treatment. Fever, diarrhea and vomiting, dehydration, pale appearance, and weakness were the manifestations of the primary disease. In all patients, a physical examination revealed reduced turgor and elasticity of the skin. Abdomen was at the level of the chest, soft, with minimal palpatory pain. The liver and spleen were not palpable. A laboratory examination was not specific except for eosinophilia. There were no pathogenic bacteria in coproculture but Ascaris was found in all patients. At an ultrasound examination in all cases we found single, long, linear echogenic structure without acoustic shadowing containing a central, longitudinal anechoic tube with characteristic movement within the gallbladder. Edema of the gallbladder wall was suggestive of associated inflammation. There were no other findings on adjacent structures and organs. All patients received mebendazole 100 mg twice a day for 3 days. They also received symptomatic therapy for gastroenteritis. Because of elevated markers of inflammation all patients were treated with antibiotics, assuming acute cholecystitis, although ultrasound was able to confirm cholecystitis in only two of our four patients. Since the length of stay was dependent on the primary pathology it was 7 to 10 days. At control ultrasounds on 14th day, third and sixth month, all patients were free of ascariasis. CONCLUSIONS: Gallbladder ascariasis should be considered in all patients presenting with abdominal pain, distension, colic, nausea, anorexia, and intermittent diarrhea associated with jaundice, nausea, vomiting, fever, and severe radiating pain. Eosinophilia, ova, and parasites on stool examination as well as an anechogenic tube with characteristic movement within the bile duct found on abdominal ultrasound are conclusive for diagnosis. Mebendazole is an effective drug for the treatment. Surgical treatment is rarely needed.

Dental Caries Among Kosovar Children with Type 1 Diabetes Mellitus

Objective: To evaluate the oral health status in children with type 1 diabetes mellitus. Material and Methods: Dental examinations, based on World Health Organization caries diagnostic criteria for DMFT index for permanent dentition and survey were performed among 160 children, aged 10-15-year-old, divided into two groups. The first group consisted of 80 children with type 1 diabetes mellitus (41 females, 39 males), and in the second group, consisted 80 healthy children (49 females, 31 males). Frequency, odds ratio and Mann-Whitney U test were used in the statistical analyses. The level of significance was set at 5%. Results: The higher mean of the DMFT index was found among children with type 1 diabetes compared to the healthy group. The mean DMFT index for diabetic children was 6.56 ± 3.56 and for the healthy group was 4.21 ± 2.63. Moreover, the frequency of decayed teeth was higher in children with type 1 diabetes than in the healthy group. The higher risk of caries was found in diabetic children compared with healthy for 1.35 times. A higher proportion of children, 61.25% with type 1 diabetes mellitus, reported that they brush their teeth once per day, 22.50% twice per day, and 16.25% rarely. From the healthy group, 46.25% of children brush their teeth once per day, and 42.50% twice per day and 11.25% rarely brush their teeth per day. Conclusion: Diabetic children are at higher risk for caries than are healthy children.

Congenital Nephrotic Syndrome - Finish Type.

INTRODUCTION: Identification of the NPHS1 gene, which encodes nephrin, was followed by many studies demonstrating its mutation as a frequent cause of congenital nephrotic syndrome (CNS). While this gene is found in 98% of Finnish children with this syndrome, non-Finnish cases have lower level of incidence ranging from 39 to 80%. CASE REPORT: This report describes the clinical presentation of a two-week-old neonate who presented with periorbital and lower extremities edema, abdominal distention, heavy proteinuria, serum hypoproteinemia and failure to thrive. Genetic analysis revealed NHPS1 gene mutation leading to CNS-Finnish type diagnosis. CONCLUSION: Through this case we want to create awareness about diagnosis and treatment challenges in developing countries for rare congenital diseases.

Hashimoto Thyroiditis and Nephrocalcinosis in a Child with Down Syndrome.

INTRODUCTION: Hypothyroidism has been reported to affect renal function and structure. However, the association of hypothyroidism with distal renal tubular acidosis (dRTA) is rarely reported in children. CASE PRESENTATION: We present a 6-year-boy with Down syndrome admitted in our department due to vomiting, weakness, polyuria, polydipsia, irritability and weight loss in the last few weeks. Investigations revealed features of hypokalemia, metabolic acidosis and alkaline urine consistent with dTRA. Abdominal ultrasound found nephrocalcinosis. In addition, Antithyroid peroxidase antibodies were positive, suggesting an autoimmune background for the pathogenesis of the tubular dysfunction. Treatment for dRTA and hypothyroidism was started and symptomatic improve was noticed. CONCLUSION: dRTA should be excluded in children with autoimmune disorders who develop weakness, polyuria, polydipsia or growth failure. Early diagnosis would reduce long-term complications.

Sensitivity and specificity of procalcitonin to determine etiology of diarrhea in children younger than 5 years.

AIM: The aim of this study is to assess the sensitivity and specificity of procalcitonin to determine bacterial etiology of diarrhea. THE EXAMINEES AND METHODS: For this purpose we conducted the study comprising 115 children aged 1 to 60 months admitted at the Department of Pediatric Gastroenterology, Pediatric Clinic, divided in three groups based on etiology of the diarrhea that has been confirmed with respective tests during the hospitalization. Each group has equal number of patients - 35. The first group was confirmed to have bacterial diarrhea, the second viral diarrhea and the third extra intestinal diarrhea. The determination of procalcitonin has been established with the ELFA methods of producer B.R.A.H.M.S Diagnostica GmbH, Berlin, (Germany). RESULTS: From the total number of 1130 patient with acute diarrhea procalcitonin was assessed in 105. 67 (63.8%) of these patient were male. More than one third (38.14%) of the children in our study were younger then 12 months. Approximately the same was the number of children 13-24 months (33 patients or 31.43%) and 25-60 months (32 patients or 30.43%). The mean value of PRC in children with viral diarrhea was 0.13±0.5 ng/mL in children with bacterial diarrhea was 5.3±4.9 ng/m Land in children with extra intestinal diarrhea was 1.7±2.8 ng/mL. When measured using ANOVA and Turkey HSD tests, results have shown the statistical significance when comparing viral with bacterial and extra intestinal diarrhea but were statistically insignificant when comparing bacterial and extra intestinal diarrhea. CONCLUSION: Procalcitonin is an important but not conclusive marker of bacterial etiology of acute diarrhea in children younger than 5 years.

Thrombocytopenia absent radius (TAR) syndrome.

AIM: The aim of the work was a presentation of one case with Thrombocytopenia absent radius (TAR) syndrome. METHODS: Diagnosis of TAR syndrome has been established on the basis of pedigree, laboratory findings (hemogram, platelet count, peripheral smear), bone marrow biopsy, radiological examination and karyotype. RESULTS: A patient was a two months old female child, hospitalized due petechial bleeding, upper limb anomalies and diarrhea. LABORATORY FINDINGS: red blood cell count was 2.1 x 1012/L, hemoglobin value was 62 g/L, white blood cell count indicated the existence of leukemoid reaction (40.0 x 109/L), the eosinophyle count at the leukocyte formula was increased (3%), bleeding time was prolonged (10'). The platelets at the peripheral blood smear were rarely present, whereas the megacaryocytes appeared in the bone marrow aspiration in the decreased number, or did not appear at all. At the radiological examination of the upper limbs, radius was absent in both shoulders. CONCLUSION: TAR syndrome is a rare hereditary disease. Obligatory clinical manifestations are: thrombocytopenia and bilateral absence of the radius. Prenatal diagnosis can be established during the 16th week of gestation by ultrasound and if it is continued with the pregnancy it is preferred that the platelet transfusion be given intrauterine. The mortality rate depends on the age of the patient and the platelet count.

Most frequent causes of urinary tract infections in children.

AIM: to determine the most frequent causes of urinary tract infection (UTI) amongst children according to gender and age. METHODS: 299 children with urinary tract infections were evaluated. All of the cases were clinically evaluated and quantitative and qualitative examinations of the urine (culture with antibiogram) were done. RESULTS: There were 54.58% males and 45.15% females in the research group. Similar distribution was present in age groups: 1-28 days (67.86% male, 32.14% female), 1 month up to 1 year (64.56% male, 35.44% female), 2-6 years (55.29% male, 44.71% female). The only difference was found in the age group of 7-16 years where there were more female patients (56.07% female, 43.93% male). Based on microbiological findings, in 91.3o% of the cases only one species was isolated, and more than one species was isolated in 8.70% of cases. Escherichia coli was the most common cause of urinary tract infection (62.88%), the second most common was Klebsiella (23.080/%), followed by Proteus mirabilis (7.02%), Citrobacter(5.35%), Staphylococcus saprophyticus (1.34%) and Candida albicans (0.33%). E. coli was the most frequent causative of UTI amongst children of all ages. In the age group of newborns, E. coli was isolated as the etiological causative of UTI in 57.14% of cases. In the age group ofi month-1 year, E.coli was the most frequent cause of urinary tract infection (60.76%). In the age group of 2-6 years, E.coli was the most frequent cause of urinary tract infection (74.12%). In the age group of 7-16 years, E. coli was isolated in 59.81% of cases. Klebsiella was the second most frequent causative (23.08%) and with a higher incidence in the age group of newborns (42.86%). CONCLUSION: UTI was generally more present in male patients. E. coli was the most frequent cause of UTI in children of all age, while the second most frequent cause of UTI in children was Klebsiella.

Choice of surgical procedure for ptosis correction.

PURPOSE: the ideal procedure for ptosis management is not available until now. In many ptosis cases there is a doubt which procedure will give the best results and patient satisfaction. The aim of this paper was to present our point of view in this topic. METHOD: retrospective review and analysis of 350 ptosis cases operated by a single surgeon was done. Primary outcome measure was cosmetic appearance, secondary outcome measures includes the need for reoperation and specific complications for each operation: central picking of operated eyelid for Fasanella-Servat operation, presence and degree of lid-lag phenomenon and lagophthalmos for levator muscle resection and frontalis muscle suspension. RESULTS: 350 cases of ptosis are operated by a single consultant surgeon. Among them, Fasanella-Servat procedure was done in 119 cases (reoperation rate was 8.4%), levator muscle resection in 112 (reoperation rate was 10.7%), frontalis muscle suspension in 116 (reoperation rate was 10.3%) and Mc. Cord procedure in 3 cases. CONCLUSION: levator muscle function is a key determining factor in choosing the appropriate operating procedure for ptosis correction regardless of the degree and etiology of ptosis.