• Clinical and Experimental Pediatrics
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• v.50 no.8
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• pp.794-798
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• 2007

Hepatopulmonary syndrome is a triad that includes: hepatic dysfunction, intrapulmonary vascular dilatation and abnormal arterial oxygenation. The incidence of intrapulmonary vascular dilatations, in adults with end-stage liver disease, has been reported to be 13% to 47%, however the incidence in children is unclear and the cases in Korean children have never been reported. The hepatopulmonary syndrome may occur as a result of chronic liver disease following nonalcoholic steatohepatitis in children with hypothalamic or pituitary dysfunction. We report a case of hepatopulmonary syndrome in a 13-year-old child who had rapidly progressive liver dysfunction secondary to panhypopituitarism after craniopharyngioma resection. Careful monitoring and treatment of endocrine abnormalities and metabolic status, as well as liver function, are required in all children undergoing pituitary tumor resection.

• Journal of Korean Neurosurgical Society
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• v.38 no.6
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• pp.450-455
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• 2005

Objective : Pituitary apoplexy is a rare clinical syndrome caused by pituitary hemorrhage, hemorrhagic infarction, or ischemic infarction within a pituitary tumor or surrounding structure. We analyzed surgical outcomes of pituitary apoplexy. Methods : From 1995 to 2004, we reviewed our experience of 29 cases with pituitary apoplexy. In all patients, pre- and postoperative clinical presentation were checked and endocrine study were performed. Results : The most frequent symptoms were visual disturbance [24 cases, 82.8%] and headache [22 cases, 75.9%]. After surgery, headache improved in 86.4%, 88.9% among 18cases who had preoperative reduction in visual acuity and 75.0% among 12 cases who had preoperative reduction in visual field improved. In endocrine study, long-term steroid and thyroid hormone replacement therapy was necessary in 42.9% of 14 cases presenting preoperative hypopituitarism. Postoperative transient hypopituitarism developed in 5 cases [33.3%] and they all recovered in follow up study. Postoperative endocrinological recovery were in 77.9% of 9 cases with preoperative prolactinoma, 1 case in 2 cases with acromegaly and one case with Cushing disease. Postoperative complications were diabetes inspidus[DI] in 1 case [3.4%]. cerebrospinal fluid[CSF] leakage in 2 cases [6.8%] and death in 1 case [3.4%] due to sepsis, Conclusion : We report good results through surgery of pituitary apoplexy in a clinical and endocrine outcomes. The surgery should be performed as soon as possible to be a suitable method for treating pituitary apoplexy.

• Journal of Genetic Medicine
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• v.18 no.1
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• pp.64-69
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• 2021

Primary cilium has a signal transduction function that is essential for brain development, and also determines cell polarity and acts as a mediator for important signaling systems, especially the Sonic Hedgehog (SHH) pathway. TBC1D32 is a ciliary protein, implicated in SHH signaling. Biallelic mutations in the TBC1D32 gene causes a kind of ciliopathy, heterogeneous developmental or degenerative disorders that affect multiple organs, including the brain. Here we report a boy who carried compound heterozygous variants in TBC1D32. The patient showed hypotonia, respiratory difficulty, and multiple anomalies at his birth. He was diagnosed with congenital hypopituitarism and treated with T4, hydrocortisone, and growth hormone. Despite the hormonal replacement, the patient needed long-term respiratory support with tracheostomy and nutritional support with a feeding tube. His developmental milestones were severely retarded. Hydrocephalus and strabismus developed and both required surgery, during the outpatient follow-up. Whole-exome sequencing indicated compound heterozygous variants, c.2200C>T (p.Arg734^*) and c.156-1G>T, in TBC1D32 gene. This is the first Korean case of TBC1D32-related ciliopathy and we reported detailed and sequential clinical features. This case demonstrated the utility of whole-exome sequencing and provided valuable clinical data on ultra-rare disease.

• Journal of Korean Neurosurgical Society
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• v.59 no.2
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• pp.161-164
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• 2016

Few preoperative extrapontine myelinolysis (EPM) cases with pituitary adenoma have been reported. No such case had long follow-up to see the outcome of EPM. We reported a 38-year-old man complaining of nausea, malaise and transient loss of consciousness who was found to have severe hyponatremia. Neurologic deficits including altered mental status, behavioral disturbances, dysarthria and dysphagia developed despite slow correction of hyponatremia. Endocrine and imaging studies revealed hypopituitarism, nonfunctional pituitary macroadenoma and extrapontine myelinolysis. Transsphenoidal surgery was performed after three weeks of supportive therapy, when neurological symptoms improved significantly. The patient recovered function completely 3 months after surgery. Our case indicates that outcome of EPM can be good even with prolonged periods of severe neurologic impairment.

• Journal of Korean Neurosurgical Society
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• v.29 no.8
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• pp.1098-1102
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• 2000

Pituitary abscess is a very rare disorder, since antibiotics therapy has become widely available. Clinically and radiologically, the preoperative diagnosis of pituitary abscess is difficult because of its features which may be identical to those of any space-occupying lesions of the sella turcica. A combination of clinical features, such as meningitis, paranasal sinusitis and panhypopituitarism with intrasellar cystic lesion with homogeneous ring enhancement on computed tomography or magnetic resonance image should raise the suspicion of a pituitary abscess. Drainage of the abscess through trans-sphenoidal approach, appropriate antibiotics therapy, endocrine assessment and hormonal replacement should bring complete recovery to the patient. The authors review the literature and report two cases of primary pituitary abscess.

• Journal of Korean Neurosurgical Society
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• v.41 no.1
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• pp.50-52
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• 2007

Lymphocytic hypophysitis is a rare inflammatory disease of the pituitary gland that is one of the cause of hypopituitarism. The majority of cases occur in women during the peripartum period, and it is rare in men. Testicular feminization syndrome is a genetic disorder presenting a 46XY karyotype, but a normal female phenotype. We report a case of lymphocytic hypophysitis in a 23-year-old woman with testicular feminization syndrome.

• Journal of mucopolysaccharidosis and rare diseases
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• v.5 no.1
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• pp.34-38
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• 2021

Prader-Willi syndrome is a complicated genetic disorder caused by a mutation on chromosome 15q11-13. The disease results in morbid obesity due to hyperphagia, growth disturbance, multiple endocrine problems from hypopituitarism, developmental delay, and cognitive or behavioral problems. Recombinant human growth hormone has been used to improve body composition and muscle mass, which plays a main role in treating patients with Prader-Willi syndrome. We describe previous studies showing the efficacy and safety of growth hormone treatment in children with Prader-Willi syndrome and provide treatment guidelines. Growth hormone therapy could be beneficial for children with Prader-Willi syndrome and improve their quality of life.

• The Korean Journal of Nuclear Medicine
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• v.6 no.1
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• pp.25-32
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• 1972

The plasma HGH concentrations were assayed in total 138 cases by the radioimmunoassay. The groups of control, typhoid fever, epidemic hemorrhagic fever, tuberculous meningitis and other febrile diseases were studied, also were the groups of hyperthyroidism, acromegaly and hypopitutarism. Insulin stimulation test was performed in control, typhoid fever and hypopituitarism. In the control group, the plasma HGH concentration in fasting (early morning) was $2.06{\pm}1.183m{\mu}g/ml$ and its upper limit was $4.5m{\mu}g/ml$. No sexual difference was observed. By the insulin stimulation, plasma HGH concentration had rised to the peak level of $24.1{\pm}15.71m{\mu}g/ml$, 60 min. after the intravenous insulin injection, then decreased to the normal level progressively. In typhoid fever, fasting HGH concentrations in febrile state and in defeverence were $2.5{\pm}1.35m{\mu}g/ml\;and\;2.2{\pm}3.32m{\mu}g/ml$ respectively, showing no significant difference with the control group. However, the levels of individual cases ranged widely, conpared with the control group. The response to the insulin stimulation test was similar to the control group. In epidemic hemorrhagic fever the HGH concentrations in oliguric phase, in diuretic phase and in convalescence were $4.2{\pm}3.71m{\mu}g/ml,\;2.2{\pm}1.30m{\mu}g/ml\;and\;3.4{\pm}3.01m{\mu}g/ml$ respectively. No significant differences were observe compared to the control, but they showed wide range of plasma HGH levels. In tuberculous meningitis, the fasting HGH concentration was $2.9{\pm}1.42m{\mu}g/ml$. In the other febrile diseases, the value was $2.5{\pm}2.23m{\mu}g/ml$. In 4 cases of hypopituitarism, the fasting HGH concentration was $2.3{\pm}0.42m{\mu}g/ml$ and ranged normally. However, the response to the insulin stimulation test was not observed. Very high plasma HGH concentrations were observed in acromegalic patients.

• Journal of Korean Neurosurgical Society
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• v.66 no.1
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• pp.72-81
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• 2023

Objective : Ischemia and hemorrhage of pituitary adenomas (PA) caused important clinical syndrome. However, the differences on clinical characteristics and surgical outcomes between these two kinds apoplexy were less reported. Methods : A retrospective analysis was made of patients with pituitary apoplexy between January 2013 and June 2018. Baseline and clinical characteristics before surgery were reviewed. All patients underwent transsphenoidal surgery and were followed up at least 1 year. Results : Total 67 cases (5.8%) among 1147 pituitary tumor patients were enrolled, which consisted of 28 (~2.4%) ischemic PA and 39 (~3.4%) hemorrhagic PA. There were more male patients in the ischemic group compared with hemorrhagic group (78.6% vs 53.8%, p=0.043). However, the mean age, tumor size and functional tumor ratio were significant higher in the hemorrhagic group. Headache was more common in ischemic PA (82.1%) than that of hemorrhagic PA (51.3%, p=0.011). Magnetic resonance imaging findings found that mucosal thickening and enhancement of the sphenoid sinus was observed in 15 ischemic PA patients (n=27, 55.6%), but none in patients with hemorrhagic PA (n=38, p<0.0001). It was worth noting that the rate of pre-surgical hypopituitarism in ischemic PA patients were seemed higher than that in hemorrhagic PA patients, but not significant. The two groups got a total tumor resection rate at 94.1% and 92.9%, independently. No significant difference on the operative time, blood loss in operation and complications in perioperative period was observed in two groups. After operation, cranial nerve symptoms recovered to normal at 81.8% of ischemic PA patients and 82.6% of hemorrhagic PA patients. Importantly, the incidence of postoperative hypopituitarism partially decreased in both groups, among which the rate of hypothyroidism in ischemic PA patients significantly decreased from 46.4% to 18.5% (p=0.044). Conclusion : Patients with ischemic PA presented different clinical characteristics to the hemorrhagic ones. Transsphenoidal surgery should be considered for the patients with neuro-ophthalmic deficits and might benefit for pituitary function recovery of the apoplectic adenoma patients, especially pituitary thyroid axis in ischemic PA patients.

• Journal of Korean Neurosurgical Society
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• v.65 no.4
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• pp.591-597
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• 2022

Objective : Pituitary adenomas frequently extend into the suprasellar space. After a suprasellar tumor is removed, the superiorly extended arachnoid becomes redundant and sinks down into the intrasellar space which often hiders visualization and accessibility to the hidden space behind the evaginated arachnoid. We introduced arachnoid remodeling by clipping technique, and evaluated its usefulness and safety during TSS. Methods : Total 223 patients who underwent arachnoid remodeling with our new clipping technique were included. Redundant arachnoid was clipped along the dural edge with multiple 2.6-mm titanium clips until the redundant arachnoid membrane no longer blocked the surgical route. To check for possible deterioration of hormonal function by this technique, we assessed anterior pituitary function of 166 patients who underwent arachnoid remodeling by clipping and compared this with those of other 429 control patients. Results : Our technique greatly enhanced the accessibility and visualization of intrasellar and parasellar spaces, both of which are generally hindered by redundant arachnoid during transsphenoidal surgery (TSS). We found no difference in anterior pituitary function between a clip-assisted arachnoid remodeling group and the control group, implying that this technique does not result in hypopituitarism. Conclusion : During TSS for pituitary adenomas with suprasellar extension, arachnoid remodeling by clipping technique is very useful and convenient for the management of the redundant arachnoid membrane to enhance visualization and surgical accessibility.