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1.
Yonsei Medical Journal ; : 25-34, 2023.
Article in English | WPRIM | ID: wpr-968885

ABSTRACT

Purpose@#Hypoxaemia is a significant adverse event during endoscopic retrograde cholangiopancreatography (ERCP) under monitored anaesthesia care (MAC); however, no model has been developed to predict hypoxaemia. We aimed to develop and compare logistic regression (LR) and machine learning (ML) models to predict hypoxaemia during ERCP under MAC. @*Materials and Methods@#We collected patient data from our institutional ERCP database. The study population was randomly divided into training and test sets (7:3). Models were fit to training data and evaluated on unseen test data. The training set was further split into k-fold (k=5) for tuning hyperparameters, such as feature selection and early stopping. Models were trained over k loops; the i-th fold was set aside as a validation set in the i-th loop. Model performance was measured using area under the curve (AUC). @*Results@#We identified 6114 cases of ERCP under MAC, with a total hypoxaemia rate of 5.9%. The LR model was established by combining eight variables and had a test AUC of 0.693. The ML and LR models were evaluated on 30 independent data splits. The average test AUC for LR was 0.7230, which improved to 0.7336 by adding eight more variables with an l 1 regularisation-based selection technique and ensembling the LRs and gradient boosting algorithm (GBM). The high-risk group was discriminated using the GBM ensemble model, with a sensitivity and specificity of 63.6% and 72.2%, respectively. @*Conclusion@#We established GBM ensemble model and LR model for risk prediction, which demonstrated good potential for preventing hypoxaemia during ERCP under MAC.

2.
The Korean Journal of Orthodontics ; : 317-327, 2023.
Article in English | WPRIM | ID: wpr-1003098

ABSTRACT

Objective@#This study aimed to evaluate the association between low tongue position (LTP) and the volume and dimensions of the nasopharyngeal, retropalatal, retroglossal, and hypopharyngeal segments of the upper airway. @*Methods@#A total of 194 subjects, including 91 males and 103 females were divided into a resting tongue position (RTP) group and a LTP group according to their tongue position. Subjects in the LTP group were divided into four subgroups (Q1, Q2, Q3, and Q4) according to the intraoral space volume. The 3D slicer software was used to measure the volume and minimum and average cross-sectional areas of each group. Airway differences between the RTP and LTP groups were analyzed to explore the association between tongue position and the upper airway. @*Results@#No significant differences were found in the airway dimensions between the RTP and LTP groups. For both retropalatal and retroglossal segments, the volume and average cross-sectional area were significantly greater in the patients with extremely low tongue position. Regression analysis showed that the retroglossal airway dimensions were positively correlated with the intraoral space volume and negatively correlated with A point-nasion-B point and palatal plane to mandibular plane. Males generally had larger retroglossal and hypopharyngeal airways than females. @*Conclusions@#Tongue position did not significantly influence upper airway volume or dimensions, except in the extremely LTP subgroup.

3.
Journal of the Korean Association of Oral and Maxillofacial Surgeons ; : 208-213, 2023.
Article in English | WPRIM | ID: wpr-1001624

ABSTRACT

Objectives@#Orthognathic surgery is a corrective intervention for maxillofacial deformities. Bleeding is a major concern for oral and maxillofacial surgeons. Various agents, such as hemocoagulase, tranexamic acid, and aprotinin have been developed to reduce intraoperative bleeding and transfusion requirements. Therefore, in this study we aimed to investigate the effects of hemocoagulase and tranexamic acid, as well as their simultaneous use, to reduce bleeding during orthognathic surgery. @*Patients and Methods@#This retrospective study included patients who had undergone simultaneous orthognathic surgery of the maxilla and mandible between January 2013 and September 2022 and were classified into three groups based on drugs administered: hemocoagulase (Botropase), tranexamic acid, and a combination of both drugs. We recorded patient age, sex, weight, blood loss, and duration of surgery. Red blood cell (RBC), hemoglobin, hematocrit, and platelet levels were measured before, immediately after, and one day after surgery. @*Results@#No statistically significant differences were found in blood loss, RBC, hemoglobin, hematocrit, or platelet levels between any of the groups.There were no differences in the drug effects between Le Fort I and bilateral mandibular sagittal split osteotomies, with or without double genioplasty.However, there were significant reductions in RBC, hemoglobin, hematocrit, and platelet levels during genioplasty. @*Conclusion@#Tranexamic acid, hemocoagulase, and their combination had similar efficacy in patients who underwent Le Fort Ⅰ and bilateral mandibu-lar sagittal split osteotomies with and without genioplasty.

4.
Korean Journal of Anesthesiology ; : 227-234, 2023.
Article in English | WPRIM | ID: wpr-977181

ABSTRACT

Background@#Double-lumen endobronchial tube (DLT) displacement, defined as migration of the DLT > 15 mm from optimal position, may threaten patient safety. Lateral decubitus positioning (LDP) of the patient can induce DLT displacement; however, little is known regarding the predictors for DLT displacement after this maneuver. Obesity may further aggravate DLT displacement by distorting mediastinal anatomy, but no comprehensive data exist concerning the impact of obesity on DLT displacement after LDP. Therefore, we evaluated the predictive value of preoperative risk factors, including obesity, for DLT displacement after LDP in patients who underwent pulmonary resection. @*Methods@#Data of patients who underwent pulmonary resection between July 2020 and July 2021 were retrospectively reviewed. Age, sex, height, weight, body mass index, left main bronchus diameter, DLT size, lateral decubitus direction, DLT depth, patient height divided by the DLT depths in supine and lateral decubitus positions (H/Dsupine and H/Dlateral, respectively), and extent of DLT dislocation were assessed. Logistic regression analysis identified risk factors, and the optimal cutoff values for continuous variables were determined using receiver operating characteristic curve analysis. @*Results@#Data from 428 patients were analyzed. DLT displacement was observed in 52 patients (12.1%). Obesity and H/Dsupine were independent predictors for DLT displacement after LDP (odds ratio [OR]: 5.69, 95% CI [2.89, 11.23], P < 0.001 and OR: 8.28, 95% CI [2.92, 23.48], P < 0.001, respectively). @*Conclusions@#Obesity was significantly associated with DLT displacement after LDP. Pre-emptively advancing the DLT from its optimal position before LDP may be advantageous in patients with obesity.

5.
Annals of Dermatology ; : S97-S99, 2023.
Article in English | WPRIM | ID: wpr-976636

ABSTRACT

Cytomegalovirus (CMV) infection is common among immunocompromised hosts; however, its cutaneous manifestation is considered rare in comparison to internal organ involvement. Clinical manifestations of cutaneous CMV infection generally include perioral or perianal ulcerations. On the other hand, autoimmune bullous dermatosis can have bullae and ulcerations similar to those caused by cutaneous CMV infection. Autoimmune bullous dermatosis requires treatment with immunosuppressive agents for relatively long periods, which may cause reduction of immunocompetence. Because of this iatrogenic immunosuppression, patients with autoimmune bullous dermatosis subsequently acquire increased risk for opportunistic infections. However, cases of bullous pemphigoid (BP) complicated by cutaneous CMV infection are rarely reported. Herein, we report the case of an 88-year-old male who had BP and subsequently recalcitrant perianal skin lesions, which were eventually diagnosed as cutaneous CMV infections.

6.
Journal of Neurogastroenterology and Motility ; : 104-110, 2022.
Article in English | WPRIM | ID: wpr-915751

ABSTRACT

Background/Aims@#Chronic intestinal pseudo-obstruction (CIPO) is a clinically heterogeneous syndrome characterized by compromised peristalsis and intestinal obstruction. Variants of actin gamma 2 (ACTG2), a protein crucial for correct enteric muscle contraction, have been found in CIPO patients. The aim of this study is to examine the clinical features and ACTG2 variants in Korean patients with CIPO. @*Methods@#From January 1995 to August 2020, 12 patients diagnosed with CIPO were included and genetic analysis testing of ACTG2 was performed. @*Results@#Heterozygous ACTG2 missense variants were found in 6 patients (50.0%). The p.Arg257Cys variant was found in 3 patients, and p.Arg63Gln and p.Arg178His variants were found in 1 patient each. A novel variant, p.Ile193Phe, was found in 1 patient. Three patients were diagnosed at birth, 2 at the age of 1 year, and 1 at 3 years of age. Abnormal prenatal genitourinary ultrasonographic findings were found in all 6 patients; microcolon was found in 4 patients (66.7%), and megacystis in all 6 patients. The pathology showed abnormal ganglion cells as well as myopathic findings. All patients are dependent on total parenteral nutrition and are to date alive. @*Conclusions@#ACTG2 variants are commonly found in Korean patients with CIPO. In CIPO patients with megacystis and abnormal prenatal ultrasonography, genetic testing of ACTG2 should be considered. Molecular diagnosis of CIPO is more important than pathologic diagnosis.

7.
Korean Journal of Dermatology ; : 687-691, 2022.
Article in English | WPRIM | ID: wpr-968043

ABSTRACT

Prurigo nodularis (PN) is a chronic inflammatory skin disease characterized by multiple nodules and papules, which are accompanied by intense pruritus. Treatment is challenging and requires a diverse approach. Recently, a few reports have described successful treatment of PN with dupilumab. We report a case of a patient with treatment-resistant PN who was successfully treated with dupilumab. Dupilumab was administered at the standard dose: an initial induction dose of 600 mg, followed by 300 mg every 14 days. Pruritus reduced after 2 weeks and almost completely disappeared after 4 weeks of treatment.

8.
Annals of Dermatology ; : 225-227, 2022.
Article in English | WPRIM | ID: wpr-925461

ABSTRACT

Intralymphatic histiocytosis (ILH) is a rare cutaneous condition with uncertain pathogenesis. It is characterized by dilated lymphatic vessels that contain histiocytes within their lumina. Although the etiology of ILH remains unknown, it has been associated with various inflammatory and neoplastic diseases, such as rheumatoid arthritis (RA), reaction to metal joint implants and Merkel cell carcinoma, breast cancer and colon cancer. An 83-year-old female presented with an erythematous patch on the left forearm that had appeared six months previous. She had suffered from osteoarthritis (OA) and the cutaneous lesion was located in the vicinity of the affected joint. Skin biopsy from the lesion showed dilated dermal vessels and some ectatic vessels that contained many mononuclear histiocytes. Based on the clinical and histopathological findings, we diagnosed her with ILH with OA. Two sessions of intralesional triamcinolone acetonide injection (5 mg/ml) were administered to treat the skin lesion, which gradually improved over a period of a few months. We here report a rare case of ILH associated with degenerative OA.

9.
International Journal of Stem Cells ; : 85-94, 2022.
Article in English | WPRIM | ID: wpr-925071

ABSTRACT

Background and Objectives@#Brain organoids have the potential to improve our understanding of brain development and neurological disease. Despite the importance of brain organoids, the effect of vascularization on brain organoids is largely unknown. The objective of this study is to develop vascularized organoids by assembling vascular spheroids with cerebral organoids. @*Methods@#and Results: In this study, vascularized spheroids were generated from non-adherent microwell culture system of human umbilical vein endothelial cells, human dermal fibroblasts and human umbilical cord blood derived mesenchymal stem cells. These vascular spheroids were used for fusion with iPSCs induced cerebral organoids. Immunostaining studies of vascularized organoids demonstrated well organized vascular structures and reduced apoptosis. We showed that the vascularization in cerebral organoids up-regulated the Wnt/β-catenin signaling. @*Conclusions@#We developed vascularized cerebral organoids through assembly of brain organoids with vascular spheroids. This method could not only provide a model to study human cortical development but also represent an opportunity to explore neurological disease.

10.
Neonatal Medicine ; : 41-47, 2021.
Article in English | WPRIM | ID: wpr-902822

ABSTRACT

Neonatal diabetes mellitus can be categorized as transient, permanent, or syndromic, and approximately half of the cases are transient. We present a case involving a term newborn who showed overt progression of transient neonatal diabetes mellitus, with complete remission within 6 months. On the second day of life, the patient presented with tachypnea, hyperglycemia, and decreased serum levels of C-peptide and insulin. Continuous subcutaneous infusion of insulin and continuous glucose monitoring were well tolerated. The patient showed a normal growth pattern, with no hyperglycemic or hypoglycemic episodes at 6 months of age. As it is rare and often asymptomatic, hyperglycemia may be attributed to various factors, including intrauterine environment, perinatal stress, and diverse genetic background. Therefore, consistent blood glucose monitoring and prompt early insulin therapy are crucial for any term newborns with persistent hyperglycemia, to prevent further diabetic complications. Moreover, continuous subcutaneous insulin infusion and the utilization of continuous glucose monitoring devices are the most effective and practical management strategies.

11.
Korean Journal of Dermatology ; : 618-623, 2021.
Article in English | WPRIM | ID: wpr-902024

ABSTRACT

Background@#A direct potassium hydroxide (KOH) smear is used to diagnose onychomycosis despite its broad sensitivity range. For a more accurate diagnosis, histopathologic examination can be used and consistently show high sensitivity. @*Objective@#We investigated the value of histopathologic examination of the nail plate as a diagnostic tool for onychomycosis. We proposed effective routine diagnostic staining to compare sensitivity between periodic acid-Schiff (PAS) and Gomori’s methenamine silver (GMS) staining. @*Methods@#This retrospective study was conducted from January 1, 2019 to May 31, 2020, and included 97 patients who showed negative results on direct KOH smear but had clinical manifestations that implied onychomycosis. We performed nail plate biopsy and PAS or GMS staining to identify fungal hyphae missed in the direct KOH smear. Sensitivity comparison between PAS and GMS was performed in co-stained samples. @*Results@#Among 97 patients with 102 cases, 55 cases (53.9%) of onychomycosis were confirmed by histopathologic examination. A total of 68 patients (70.1%) had a previous medical history of antifungal agents within previous six months. PAS and GMS staining were concurrently performed in 73 cases, and onychomycosis was confirmed in 41 cases. The sensitivity of PAS was 100% (41/41), while that of GMS was 87.8% (36/41); this difference was not significant. @*Conclusion@#This study suggests that histologic examination of the nail plate is an effective tool to diagnose onychomycosis and can be performed with a direct KOH smear. Two staining methods, PAS and GMS, are recommended for concurrent performance to enhance the identification of fungal hyphae.

12.
Neonatal Medicine ; : 41-47, 2021.
Article in English | WPRIM | ID: wpr-895118

ABSTRACT

Neonatal diabetes mellitus can be categorized as transient, permanent, or syndromic, and approximately half of the cases are transient. We present a case involving a term newborn who showed overt progression of transient neonatal diabetes mellitus, with complete remission within 6 months. On the second day of life, the patient presented with tachypnea, hyperglycemia, and decreased serum levels of C-peptide and insulin. Continuous subcutaneous infusion of insulin and continuous glucose monitoring were well tolerated. The patient showed a normal growth pattern, with no hyperglycemic or hypoglycemic episodes at 6 months of age. As it is rare and often asymptomatic, hyperglycemia may be attributed to various factors, including intrauterine environment, perinatal stress, and diverse genetic background. Therefore, consistent blood glucose monitoring and prompt early insulin therapy are crucial for any term newborns with persistent hyperglycemia, to prevent further diabetic complications. Moreover, continuous subcutaneous insulin infusion and the utilization of continuous glucose monitoring devices are the most effective and practical management strategies.

13.
Korean Journal of Dermatology ; : 618-623, 2021.
Article in English | WPRIM | ID: wpr-894320

ABSTRACT

Background@#A direct potassium hydroxide (KOH) smear is used to diagnose onychomycosis despite its broad sensitivity range. For a more accurate diagnosis, histopathologic examination can be used and consistently show high sensitivity. @*Objective@#We investigated the value of histopathologic examination of the nail plate as a diagnostic tool for onychomycosis. We proposed effective routine diagnostic staining to compare sensitivity between periodic acid-Schiff (PAS) and Gomori’s methenamine silver (GMS) staining. @*Methods@#This retrospective study was conducted from January 1, 2019 to May 31, 2020, and included 97 patients who showed negative results on direct KOH smear but had clinical manifestations that implied onychomycosis. We performed nail plate biopsy and PAS or GMS staining to identify fungal hyphae missed in the direct KOH smear. Sensitivity comparison between PAS and GMS was performed in co-stained samples. @*Results@#Among 97 patients with 102 cases, 55 cases (53.9%) of onychomycosis were confirmed by histopathologic examination. A total of 68 patients (70.1%) had a previous medical history of antifungal agents within previous six months. PAS and GMS staining were concurrently performed in 73 cases, and onychomycosis was confirmed in 41 cases. The sensitivity of PAS was 100% (41/41), while that of GMS was 87.8% (36/41); this difference was not significant. @*Conclusion@#This study suggests that histologic examination of the nail plate is an effective tool to diagnose onychomycosis and can be performed with a direct KOH smear. Two staining methods, PAS and GMS, are recommended for concurrent performance to enhance the identification of fungal hyphae.

14.
Korean Journal of Head and Neck Oncology ; (2): 61-65, 2021.
Article in Korean | WPRIM | ID: wpr-917692

ABSTRACT

Epithelial-myoepithelial carcinoma is rare, low-grade malignant neoplasm that compromises approximately 1% of all salivary gland neoplasms. We reported a 68-year-old woman with epithelial-myoepithelial carcinoma in the parotid gland. We analyzed demographic and clinicopathological characteristics of salivary gland epithelial-myoepithelial carcinoma which has been reported in 33 cases in Korea from 1992 to 2017, and compared this result to overseas retrospective cohort studies. There was no significance in terms of mean age at diagnosis, the predominance of females, low frequency in regional lymph nodes, and distant metastasis between the two groups. However, T classification and AJCC stage at diagnosis in domestic cases are more advanced than those of overseas cases. Although the recurrence rate in domestic cases has been reported lower than that of overseas, further study may be needed considering that the follow-up period after treatment is short in domestic cases.

15.
Gut and Liver ; : 142-145, 2021.
Article in English | WPRIM | ID: wpr-874568

ABSTRACT

Hereditary fructose intolerance (HFI) is an autosomal recessive disorder caused by a mutation in the aldolase B gene. HFI patients exhibit nausea, vomiting, abdominal pain, hypoglycemia, and elevated liver enzymes after dietary fructose exposure. Chronic exposure might lead to failure to thrive, liver failure, renal failure, and, eventually, death. HFI usually manifests in infants when they are being weaned off of breastmilk. Because HFI has an excellent prognosis when patients maintain a strict restrictive diet, some patients remain undiagnosed due to the voluntary avoidance of sweet foods. In the past, HFI was diagnosed using a fructose tolerance test, liver enzyme assays or intestinal biopsy specimens. Currently, HFI is diagnosed through the analysis of aldolase B mutations. Here, HFI was diagnosed in a 41-year-old woman who complained of sweating, nausea, and vomiting after consuming sweets. She had a compound heterozygous mutation in the aldolase B gene; gene analysis revealed pathogenic nonsense (c.178C>T, p.Arg60Ter) and frameshift (c.360_363delCAAA, p.Asn120LysfsTer32) variants. This is the first report of a Korean HFI patient diagnosed in adulthood.

16.
Korean Journal of Dermatology ; : 674-680, 2020.
Article in English | WPRIM | ID: wpr-901928

ABSTRACT

Background@#Chronic actinic dermatitis is a rare, acquired, persistent eczematous eruption of photo-distributed areas.There are limited data available on the clinical characteristics of Korean patients diagnosed with chronic actinic dermatitis. @*Objective@#To evaluate the clinical features and prognosis of chronic actinic dermatitis patients in Korea. @*Methods@#Sixty-two Korean patients diagnosed with chronic actinic dermatitis through clinical findings, phototesting, and skin biopsy from six hospitals were included in this study, and their clinical characteristics were evaluated. @*Results@#Among the 62 patients, 51 were men, and the mean age at diagnosis was 60.3±12.8 years. Phototesting was performed for 27 patients, with results available for 18 patients. Patch tests were performed for only 4.8% of the patients. Skin pathology tests were performed for 47 patients and showed the following: spongiosis, acanthosis, actinic elastosis, and pseudo-lymphomatous change. Twelve patients were clinically diagnosed without using diagnostic tools such as phototesting, patch testing, and skin biopsy. The most commonly used systemic treatments were antihistamine, cyclosporine, steroid, and azathioprine. Although avoiding ultraviolet irradiation and outdoor activities are critical, only 22.6% of patients used sunscreen. @*Conclusion@#Although phototesting is highly recommended for diagnosing chronic actinic dermatitis, phototesting was performed for less than half of the patients to diagnose chronic actinic dermatitis. Patch tests and photopatch tests are also recommended to disclose a causative agent.

17.
Korean Journal of Dermatology ; : 344-346, 2020.
Article | WPRIM | ID: wpr-832705

ABSTRACT

Pilomatricoma, also known as calcifying epithelioma of Malherbe, is a benign adnexal skin tumor arising from hair matrix cells. Clinically, pilomatricoma typically presents as a firm, bluish or red colored, deep-seated nodule with overlying normal skin. Several variants of pilomatricoma have been described including perforating, extruding, bullous, melanocytic, giant, keratoacanthoma-like, exophytic, lymphangiectatic, familial, anetodermic, and multiple. Among these, an extruding variant has been very rarely reported. A 10-year-old girl presented with a 2-month history of a rapid-growing, solitary, firm, skin-colored to pinkish, pedunculated papule with a central crusted perforation on the left cheek. Based on the clinical and histological findings, we made a diagnosis of exophytic pilomatricoma with perforating figure. She had no signs of recurrence of the lesion for 8 months after removal.

18.
Korean Journal of Dermatology ; : 472-475, 2020.
Article | WPRIM | ID: wpr-832630

ABSTRACT

Fibrofolliculoma is a benign perifollicular connective tissue tumor that usually occurs in the face. Most fibrofolliculoma present as multiple lesions and typically occur with trichodiscomas and acrochordons, constituting the Birt-Hogg-Dube syndrome. Solitary fibrofolliculoma is nonhereditary and unassociated with other cutaneous abnormalities. A 72-year-old man visited our clinic complaining of a solitary umbilicated nodule on the antitragus of the left ear. Skin biopsy from the lesion showed a central dilated follicular infundibulum with anastomosing bands of follicular epithelium extending into the fibrous stroma. The diagnosis of a solitary fibrofolliculoma was made based on the clinical and histopathological findings. No signs of lesion recurrence for 1 year after shave removal were noted. We report a rare case of solitary fibrofolliculoma that occurred on the antitragus of the ear.

19.
Journal of the Korean Society of Emergency Medicine ; : 23-38, 2020.
Article | WPRIM | ID: wpr-834914

ABSTRACT

Objective@#This study examined the characteristics of the patients taking poison intentionally at the pre-hospital stage to prevent it at the community level. @*Methods@#We retrospectively reviewed the data that had been reported to fire stations from January 2017 to December 2018. This data included sex, age, occupation, the season of the year, time, place, methods, alcohol ingestion, transferred to the hospital or not, and we examined how taking poison had an effect on the suicide success rate. @*Results@#The subjects were a total of 1,356 patients who had been reported to fire stations due to intentionally taking poison. Forty-five point five percent of them were male, and 54.5% were female. The most common method of intentional taking poison was sedatives (58.3%), followed by pesticides (24.6%), antidepressants (19.0%), and other methods (12.6%). The home place was preferred more than any other places. The suicide success rate was 2.1% in males and 1.4% in females. For the age groups, those patients 40-64 years old tried taking poison much more than the other age groups. In the aspect of the season of the year, summer was the highest season for taking poison, at 30.3%. The daytime was more preferred than the night time. @*Conclusion@#In this study, we analyzed the characteristics of the pre-hospital intentional poisoning cases according to gender, age, occupation, season of the year, time, and between the transferred and the untransferred groups. Efforts should be made in cooperation with the community to prevent suicide attempts by intentionally taking poison.

20.
Allergy, Asthma & Respiratory Disease ; : 206-212, 2020.
Article in Korean | WPRIM | ID: wpr-913291

ABSTRACT

Purpose@#Pulmonary underdevelopment is one of the pulmonary causes of neonatal mortality, which is categorized into pulmonary agenesis, pulmonary aplasia, and pulmonary hypoplasia. Congenital diaphragmatic hernia is a well-known cause of pulmonary hypoplasia; however, further studies are needed for other etiologies. Patients with pulmonary underdevelopment show varying degrees of clinical severity. Most patients with pulmonary underdevelopment have respiratory distress at birth, while a few have only mild symptoms. Our study investigated the natural course and prognosis of pulmonary underdevelopment by analyzing multiple cases from a single tertiary medical center. @*Methods@#A retrospective review was conducted on patients diagnosed with pulmonary hypoplasia and agenesis from January 2000 to August 2019. Cases were extracted from the clinical database of Seoul National University Children’s Hospital by searching for keywords related to pulmonary underdevelopment. @*Results@#A total of 28 patients were identified. Four patients had pulmonary agenesis, and all were idiopathic. Twenty-four patients had evidence of pulmonary hypoplasia with diverse causes. The median age at diagnosis was 11.5 days (range, 1–240 days) and the mean gestational age was 35 weeks 2 days± 3 weeks 6 days. Twenty patients received ventilator care and 11 had various degrees of pulmonary hypertension. The long-term survival rate was 50%. The median age of death among pulmonary hypoplasia patients were 11 days (range, 1–730 days). All patients without ventilator care survived. The incidence of ventilator care, pneumothorax, and pulmonary hypertension were significantly higher among patients who died. @*Conclusion@#The prognosis of pulmonary underdevelopment depends on the severity of respiratory distress and pulmonary hypertension in earlier life. Patients with severe distress appear to have severe degree of pulmonary hypoplasia. Patients without ventilator support in the neonatal period survived without significant morbidities.

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