芳野 弘

AIM: In Japan, an additional system for patients with suspected or diagnosed dementia during hospitalization for physical illness began in 2016. We examined the clinical characteristics of older patients with suspected or diagnosed dementia during hospitalization. METHODS: The study participants consisted of 569 patients. Current age, sex, comorbidities, causative disease for admission, body mass index (BMI), blood tests, environment before admission, prescription history before admission and rate of delirium were examined. Simple regression analysis for high-frequency diseases was carried out with the respective risk factors as independent variables. Multiple regression analysis was then carried out. RESULTS: Infection had the highest frequency in the causative diseases for admission. A total of 48% of patients had delirium during hospitalization. The delirium group had the highest frequency of being at home. In the infection group, BMI and serum albumin were lower in the non-infection group (BMI 19.0 ± 3.7 vs 20.4 ± 4.1; P < 0.001, albumin 2.1 ± 0.7 vs 3.3 ± 0.6 g/dL; P < 0.001 respectively). White blood cell count and C-reactive protein were higher than in the non-infection group, respectively (white blood cell count 11181.4 ± 6533.3 vs 8765.8 ± 111 424.3/μL; P = 0.007, C-reactive protein 8.6 ± 8.6 vs 3.0 ± 5.2 mg/dL; P < 0.001 respectively). Using independent factors associated with infection, multiple regression analysis was performed. BMI (P = 0.013), serum albumin (P < 0.001) and nursing home care before admission (P < 0.001) had significant correlations. CONCLUSIONS: Assessment of delirium and the environment before admission of older patients with suspected or diagnosed dementia when hospitalized are necessary. Furthermore, evaluation of nutrition might also contribute to reducing deterioration due to physical illness. Geriatr Gerontol Int 2024; 24: 641-645.

Aging is known as one of the important risk factors for coronary artery disease (CAD). We explore whether an association of metabolic syndrome (Met-S) increases subclinical atherosclerosis among elderly diabetic subjects estimating the plaque score (PS) of the carotid artery. A total of 187 subjects were enrolled. Middle-aged and older groups were divided into two groups. T-test and Chi-square test were also employed. Simple regression analysis for the PS was performed with respective risk factors as independent variables. After selection of independent variables, multiple regression analysis was performed to estimated the association of PS and dependent variable of the study. There were significant differences in body mass index (BMI) (p < .001), HbA1c (p < .01), TG (p < .05), and PS (p < .001) . Multiple regression analysis in middle-aged subjects showed that the determinant of PS were age (p < .001), BMI (p = .006), Met-S (p = .004), and hs-CRP (p = .019). Multiple regression analysis in older subjects showed that neither age nor Met-S was included as significant determinant of PS. An association of Met-S is an important factor for progression of subclinical atherosclerosis, but it cannot be a significant determinant of PS if the subjects are limited within older group.

OBJECTIVE: To evaluate the effectiveness of a dyadic outpatient rehabilitation program focused on improving the real-life daily activities of patients with mild cognitive impairments or dementia and their caregivers. DESIGN: Retrospective study. SUBJECTS: Eight patients with mild cognitive impairments or dementia and their caregivers. METHODS: The rehabilitation program comprised eight 1-hour sessions by occupational therapists with patients and his/her caregivers. Patients were assessed for motor function, cognitive function, and quality of life, and their caregivers were assessed for depression and caregiver burden. Participants were assessed at pre-program and post-program, and 3-month follow-up. RESULTS: The scores of caregiver-assessed Quality of life in Alzheimer's disease scale in patients significantly improved at post-program (median [interquartile range], 30.0 [7.0]) compared with pre-program (27.0 [2.8], effect size = 0.77, p = 0.029). In caregivers, the Zarit Caregiver Burden Interview scores decreased significantly at post-program (16.5 [13.0]) compared with pre-program (22.0 [17.5], effect size = 0.72, p = 0.042). There were no significant differences in other assessments. CONCLUSIONS: The rehabilitation program focused on real daily activities and demonstrated to improve patients' quality of life and caregivers' depression and caring burden through patient-caregiver interaction. Future enhanced follow-up systems are warranted.

BACKGROUND: Dementia is a disease that is frequently associated with significant caregiving burden. The present study was conducted on patients with dementia in order to investigate the causes of caregiver burden using the Dementia Assessment Sheet for Community-based Integrated Care System-21 items (DASC-21) and Zarit Caregiver Burden Interview (ZBI). METHODS: The study subjects consisted of 206 elderly patients (mean age: 80.9 ± 5.9 years) with dementia in an outpatient clinic. Current age, gender, type of dementia, Mini-Mental State Examination, Hasegawa Dementia Scale-Revised, Clinical Dementia Rating (CDR), Geriatric Depression Scale (GDS) and the DASC-21 were investigated. Caregiver burden was assessed by using the ZBI. Behavioural and Psychological Symptom of Dementia (BPSD) were evaluated by using the Neuropsychiatric Inventory (NPI). Simple regression analysis for the ZBI was performed with respective risk factors as independent variables. Thereafter, multiple regression analysis was performed. RESULTS: The mean ZBI score was 28.0 ± 18.1, whereas mean DASC-21 score was 44.4 ± 13.2. Simple regression analysis for the ZBI was performed with respective risk factors as independent variables. The DASC-21 (P < 0.001), CDR (P < 0.001), NPI (P < 0.001), and GDS (P = 0.034) were significantly associated with the ZBI. For each item of the DASC-21, Memory (P < 0.001), Orientation (P < 0.001), Solving issues/Common sense (P < 0.001), Instrumental activities of daily living (IADL) outside the home (P < 0.001), IADL inside the home (P < 0.001), Physical ADL① (P < 0.001) and Physical ADL② (P = 0.014) were also significantly associated with the ZBI. To find the independent association of the ZBI, multiple regression analysis was performed. The results showed that DASC-21 (P < 0.001) and NPI (P < 0.001) had significant correlation. For each item of the DASC-21, Memory (P = 0.014) and Solving issues/Common sense (P < 0.001) were also shown to have significant correlation. CONCLUSIONS: Both BPSD, cognition and IADL have affected caregiver burden. The DASC-21 is useful for determining the causes of caregiver burden of dementia patients.

BACKGROUND: In this study, we investigated subjective geriatric complaints (SGCs) as conditions regarding health concerns in community-dwelling older people and analyzed their frequencies with aging and relationships with other factors. METHODS: This cross-sectional study enrolled 10,434 older people living in a community with a representative aging population in Japan. A questionnaire was sent by mail to those who had not applied for formal care needs certification. The presence of and concern for symptoms common in old age were asked as SGCs, as were physical function levels, multimorbidity, and depression. Categorical principal component analysis (CATPCA) of the symptoms was performed, and the association between the obtained components and other factors was analyzed. RESULTS: The mean age of the participants was 73.7 ± 6.1 years, and 52.5% were women. On average, they had 1.72 ± 1.57 SGCs, which showed a gradual increase with age. The results of the CATPCA revealed four components: SGC1, excretory/circulatory/swallowing complaints; SGC2, audiovisual complaints; SGC3, neurological complaints; and SGC4, musculoskeletal complaints. All SGC components were independently associated with physical function, multimorbidity, and depression. CONCLUSIONS: Each SGC showed various frequencies and differences along with aging, and SGCs were classified into four components that were thought to share a common background. These findings could contribute to the planning of better health management strategies for older people.

BACKGROUND: Dementia cafés have been attracting attention as a new approach to dementia care, but the effects of the participation of medical professionals remain unclear. OBJECTIVE: To clarify the significance of collaboration between medical professionals and dementia cafés. METHODS: Questionnaires regarding the numbers of staff and guests, whether medical professionals introduced guests, whether cafés announced their activities to medical institutions, and whether people with dementia played a role were sent to dementia cafés throughout Japan. The responding dementia cafés were then divided into two groups according to the presence or involvement of medical professionals and institutions and compared. RESULTS: Responses were received from 148 dementia cafés, among which, medical professionals participated in 96 (64.9%). Significantly more people with dementia living at home attended cafés run or staffed with medical professionals (p = 0.021 and p = 0.017, respectively), as well as when medical professionals introduced guests to the café or when the café announced their activities to medical institutions (p = 0.001 and p = 0.002, respectively). Significantly more people with dementia played a role in cafés where medical professionals were administrators or staff (p = 0.008 and p = 0.018, respectively). Similar effects were observed for family caregivers. CONCLUSION: The participation and involvement of medical professionals and institutions in dementia cafés increased the attendance of people with dementia, especially those living at home. These results suggest that dementia cafés are an effective hub for connecting care for dementia with medical care, and thus help avoid fragmentation in dementia care.

INTRODUCTION: Diabetes mellitus (DM) is a risk factor for Alzheimer's disease (AD). It has also been pointed out that AD associated with DM may have unique characteristics. However, the characteristics of impairment in daily functioning when associated with DM have not been sufficiently investigated. METHODS: In the present study, we compared the characteristics of 261 patients with AD diagnosed in the outpatient memory clinic of a university hospital, divided into diabetic and nondiabetic groups. The MMSE was used to assess cognitive function, and the Dementia Assessment Sheet for Community-based Integrated Care System 21-items (DASC-21) was used as an observational method to assess cognitive function and activities of daily livings. The two groups were compared. Furthermore, simple and multiple regression analysis was carried out in order to find the independent association of age, sex, education, DM, and HbA1c with the DASC-21 and each individual item of the DASC-21. RESULTS: Diabetic subjects were as follows: MMSE 18.8 ± 4.0, DASC-21 46.0 ± 13.2, and HbA1c 7.07 ± 1.24%, respectively. On the other hand, nondiabetic subjects were as follows: MMSE 19.0 ± 4.5 and DASC-21 42.1 ± 12.2, respectively. In the diabetic group, total score of DASC-21 was higher (DM vs. nondiabetes mellitus [NDM]: 46.0 ± 13.2 vs. 42.1 ± 12.2; p < 0.05) and solving issues and common sense on the DASC-21 were higher than in the nondiabetic group (NDM) (DM vs. NDM: 8.58 ± 2.71 vs. 7.76 ± 2.66; p < 0.05). Multiple regression analysis showed that the presence of DM was the significant determinant of solving issues and common sense on the DASC-21 (p < 0.05). CONCLUSIONS: In AD patients, DM may be associated with impairment of solving issues and common sense.

Variant hemoglobin is often detected during the diagnosis and treatment of diabetes mellitus. We here describe a case of α2-chain variant hemoglobin (Hb Chad) that was identified as a result of differences in HbA1cs values determined by different assays. HbA1c measured by immunoassay was thus falsely high, whereas that measured by high-performance liquid chromatography (HPLC) was slightly low. Sequencing analysis revealed a heterozygous GAG (glutamic acid) → AAG (lysine) mutation at amino acid position 23 of the α2-globin gene. This residue is located at the surface of the α-chain in the crystal structure of hemoglobin. The high HbA1c value determined by immunoassay might have been the result of increased antigenicity of the variant hemoglobin, whereas the low value measured by HPLC reflected differential fractionation of the variant relative to the wild-type protein. Hb Chad has been reported in only three cases to date, and HbA1c was measured for the first time. This is the first case where falsely high HbA1c measured by immunoassay due to increased antigenicity in α-chain variant hemoglobin. This case highlights the importance of comparison with other parameters related to plasma glucose such as glycated albumin if an HbA1c abnormality is suspected. Supplementary Information: The online version contains supplementary material available at 10.1007/s13340-021-00529-y.

AIM: This study aimed to assess whether CogEvo, a computerized cognitive assessment and training tool, could distinguish patients with mild Alzheimer's disease and mild cognitive impairment from cognitively normal older people. METHODS: This cross-sectional study enrolled 166 participants with Alzheimer's disease, mild cognitive impairment and cognitively normal older people. In CogEvo, five types of cognitive tasks were carried out, and the z-scores were used as a composite score. Logistic regression and receiver operating characteristics analyses were then carried out to evaluate the usefulness of CogEvo in distinguishing between the three groups. RESULTS: CogEvo and Mini-Mental State Examination scores showed excellent correlation, and could significantly differentiate between the Alzheimer's disease, mild cognitive impairment and cognitively normal older people groups (Mini-Mental State Examination 20.4 ± 3.5, 25.5 ± 1.6 and 27.6 ± 2.0, respectively; CogEvo: -1.9 ± 0.9, -0.8 ± 0.8 and 0.0 ± 1.0, respectively; both P < 0.001 by analysis of variance). Logistic regression analysis adjusted for age, sex and years of education significantly differentiated the mild cognitive dysfunction group (mild cognitive impairment plus mild Alzheimer's disease; n = 78) from the cognitively normal group (n = 88) (P < 0.001), whereas receiver operating characteristics analysis showed moderate accuracy (area under the receiver operating characteristic curve 0.830). CONCLUSIONS: These results suggest that CogEvo, a computerized cognitive assessment tool, is useful for evaluating early-stage cognitive impairment. Further studies are required to assess its effectiveness as a combination assessment and training tool. Geriatr Gerontol Int 2021; 21: 192-196.

Introduction: Insulin is synthesized in the β-cells from preproinsulin. Preproinsulin becomes proinsulin after leaving the signal peptide. Proinsulin is separated into C-peptide and insulin by 2 enzymes. Hyperproinsulinemia is suspected to be a pancreatic β-cell defect that is augmented by the increased demand placed on the β-cell by hyperglycemia. Case presentation: A 39-year-old Japanese man visited to Shin-suma hospital in May 2013. Liver dysfunction, dyslipidemia, and hyperuricemia had been found in medical checkups in his workplace. Therefore, he visited Shin-suma hospital in order to receive intensive examination. Diet and exercise therapy were initiated. In November 2013, intact proinsulin and proinsulin per insulin (PI/I) ratio were evaluated as part of an ongoing study. His intact proinsulin level and PI/I ratio were markedly elevated. A 75 g oral OGTT revealed that his glucose tolerance was impaired. His glycosylated hemoglobin was 6.9%. He was diagnosed as having type 2 diabetes mellitus. Although, diet and exercise therapy continued, his hyperproinslinemia and diabetes mellitus remained. Therefore, aloguliptin was started in order to recover insulin secretion in November 2014. Thereafter, pioglitazone was added to improve insulin resistance. Finally, luseogliflozin was commenced to expect glucose-lowering effects. His HbA1c was stabilized. To the best of our knowledge, there have been few reports of patients with hyperproinsulinemia. Conclusion: When the physicians face treatment resistance in diabetes mellitus, we emphasize that evaluation of proinsulin should be considered as one of the methods.

An 84-year-old man was admitted to our hospital. His blood glucose level was 20 mg/dL. Since laboratory tests showed high titers of insulin antibodies, insulin autoimmune syndrome (IAS) was diagnosed. In order to avoid hypoglycemia, steroids can be effective in the long-term management of IAS in elderly patients.

A patient with underlying Hashimoto's thyroiditis developed amiodarone-induced thyrotoxicosis type 1 that was successfully treated using methimazole in combination with potassium iodide. A 35-year-old woman admitted for perinatal care of twin-to-twin transfusion syndrome was given amiodarone for 7 days for paroxysmal ventricular contraction following pulseless ventricular tachycardia 1 day after delivery. She developed thyrotoxicosis one month after the discontinuation of amiodarone therapy and was negative for thyroid-stimulating hormone receptor antibody. An increased peak velocity of the superior thyroid artery suggested amiodarone-induced thyrotoxicosis type 1. Her thyroid function recovered after combination therapy with methimazole and potassium iodide.

AIMS: To explore the effects of intermittent use of empagliflozin, a sodium-glucose co-transporter-2 inhibitor, on dietary self-management and glycaemic control in patients with inadequately controlled type 2 diabetes. MATERIALS AND METHODS: We conducted a prospective, randomized, open-label, blinded-endpoint, parallel-group, comparative clinical trial of 50 patients with type 2 diabetes, treated with no more than three oral antidiabetic drugs (glycated haemoglobin [HbA1c] ≥52 mmol/mol but <86 mmol/mol). The participants were randomized to take 10 mg/d empagliflozin either every day (regular group, n = 25) or on the day on which they considered they had overeaten (intermittent group, n = 25) for 24 weeks. We limited empagliflozin prescription to half of the required period in the intermittent group. The primary endpoint was change in HbA1c at the end of the 24-week treatment period relative to baseline. The secondary outcomes included changes in body weight, daily energy intake and diabetes treatment-related quality of life (QoL). Energy intake was assessed using a diet-specific validated questionnaire rather than actual assessments of food intake. RESULTS: The intake rate of empagliflozin was 96.7 ± 7.2% for the regular group and 45.7 ± 7.0% for the intermittent group. Interestingly, ΔHbA1c was identical in the two groups (-0.64 ± 0.19% and - 0.65 ± 0.17%, respectively). Body weight decreased (-2.72 ± 0.52 and - 1.50 ± 0.45 kg, respectively) and diabetes treatment-related QoL increased significantly from baseline in both groups. Energy intake, however, decreased significantly only in the intermittent group (-221.0 ± 108.3 kcal/d). CONCLUSIONS: Intermittent empagliflozin supplementation is a useful therapeutic option that empowers dietary self-management, improves glycaemic control and is accompanied by body weight loss and an increase in diabetes treatment-related QoL in patients with inadequately controlled type 2 diabetes.

INTRODUCTION: Dementia cafés have recently been attracting attention. The increased involvement of citizen volunteers and the competence of dementia café staff could enhance the potential of dementia cafés. The aim of the present study was to examine enhancement of the competence of citizen volunteers using a new assessment tool. METHODS: This cross-sectional analysis included 433 dementia café staff members, including medical and care professionals and citizen volunteers. A 20-item dementia café staff self-assessment (DCSA) instrument was newly developed. After confirmation of the reliability and validity of the instrument, DCSA scores among citizen volunteers were evaluated. RESULTS: DCSA showed very good psychometric properties. The mean (±SD) DCSA score was significantly higher for café staff with a medical and care professional background (n = 267) than for citizen volunteers (n = 166) (2.2±0.5 vs. 1.7±0.7, respectively; p < 0.001). The DCSA scores of citizen volunteers became significantly higher with increasing attendance (minimum: n = 24; 1.3±0.7; intermediate: n = 65; 1.6±0.6; and frequent: n = 77; 1.8±0.7; p < 0.01). CONCLUSION: Assessment of the competence of dementia café staff using the DCSA revealed the potential of citizen volunteers. This tool could also enhance the potential of dementia cafés.

INTRODUCTION: We comprehensively evaluated the effects of combination therapy with insulin glargine and the incretin-based drugs lixisenatide or vildagliptin in Japanese patients with type 2 diabetes. METHODS: In this 12-week, randomized, open-label, parallel-group, multicenter study (GLP-ONE Kobe), the incretin-based drug sitagliptin was randomly switched to lixisenatide (20 μg/day, n = 18) or vildagliptin (100 mg/day, n = 20) in patients with inadequate glycemic control despite combination therapy with insulin glargine and sitagliptin. The dose of insulin glargine was titrated after the switch to maintain fasting blood glucose at approximately 110 mg/dL. The primary end points of the study were the change in glycosylated hemoglobin (HbA1c) level between before and 12 weeks after the treatment switch, the proportion of patients achieving an HbA1c level below 7.0%, and the postprandial increase in glucose concentration as assessed by self-monitoring of blood glucose. RESULTS: The change in HbA1c level from baseline to 12 weeks did not differ significantly between the lixisenatide and vildagliptin groups (- 0.6 ± 0.7% and - 0.6 ± 1.2%, respectively, P = 0.920). Neither the proportion of patients achieving an HbA1c level below 7.0% nor the postprandial increase in glucose concentration was different between two groups. Body weight and serum low density lipoprotein (LDL) cholesterol level decreased significantly in the lixisenatide and vildagliptin groups, respectively. Both drugs were associated with mild gastrointestinal symptoms but not with severe hypoglycemia. Vildagliptin was associated with elevation of serum aspartate transaminase. Treatment satisfaction as assessed with the Diabetes Treatment Satisfaction Questionnaire did not differ significantly between the two groups. CONCLUSION: The combinations of basal insulin and either lixisenatide or vildagliptin have similar efficacies with regard to improvement of glycemic control. TRIAL REGISTRATION: This trial has been registered with UMIN (No. 000010769).

Cavernous hemangioma is a rare, non-functional, benign adrenal tumor. Adrenal cavernous hemangioma is often diagnosed after surgery with a histologic examination. A 70-year-old man complaining of appetite loss was admitted to our hospital. An incidental large left adrenal mass was found by computed tomography (CT). There were no clinical signs of adrenogenital syndrome, Cushing's syndrome or primary aldosteronism. Total resection was carried out. The pathological diagnosis was cavernous hemangioma. The differentiation of adrenal tumor is necessary in cases of large tumors, and resection is desirable given the risks of hemorrhaging and rupture.

INTRODUCTION: Optimal adjustment of basal insulin to overcome hypoglycemia and glycemic variability (GV) depends on its duration of action and peak-less profile. Owing to the ability of long-acting basal insulin to avoid hypoglycemia, we titrated pre-meal glucose to normal fasting blood glucose, 80-110 mg/dL (4.5-6.1 mmol/L), and post-meal glucose to 80-140 mg/dL (4.5-7.8 mmol/L). The purpose of this study was to evaluate two basal insulin analogues degludec (IDeg) and glargine (IGlar), injected in the morning, for GV using continuous glucose monitoring (CGM) in type 1 diabetes (T1DM). METHODS: In this crossover study, 20 Japanese patients with T1DM (age 54 ± 16 years, disease duration 16 ± 8 years, BMI 24 ± 4 kg/m2, HbA1c 7.4 ± 0.8%) were randomized into one of two different starting regimens, and CGM was conducted on three consecutive days during the last week of each 12-week titration period. Treatment satisfaction was assessed at the end of each treatment period using the Diabetes Therapy-Related Quality of Life Questionnaire (DTR-QOL). RESULTS: There were no differences in HbA1c, total insulin dosage, body weight changes, and basal to bolus ratio between the IDeg and IGlar arms. The day-to-day variability in fasting interstitial GV on the CGM curves was significantly less in the IDeg than IGlar treatment period (25.9 ± 22.0 vs. 43.8 ± 30.1 mg/dl, p = 0.04). Other markers of GV, calculated by the EasyGV software, including mean amplitude of glycemic excursions (MAGE), J-index, total and nocturnal hypoglycemia were not different between the two treatment periods. The score of "satisfaction with treatment", a subdomain of the DTR-QOL system, was higher in the IDeg period. CONCLUSION: Thus, the morning injection of the two long-acting insulin analogues seemed similar with regard to the magnitude of hypoglycemia in T1DM, but treatment with IDeg was associated with lower day-to-day variation in glucose level. These results suggest that IDeg is safe with minimal morning GV in patients with T1DM. CLINICAL TRIAL REGISTRATION: Japanese Clinical Trials Registry, UMIN000012358.

Acute suppurative thyroiditis (AST) accompanied by an abscess is a rare clinical case. Hemodialysis patients are at risk for infections. Sepsis mortality was from 100 to 300 times higher for chronic dialysis patients than that for the general public. Thus, special care should be taken against infection in patients under hemodialysis.

The clinical entity idiopathic normal pressure hydrocephalus (iNPH) is characterized by dementia, urinary incontinence, gait ataxia. An 80-year old man with a past history of Type 2 diabetes mellitus admitted to our hospital. Combination of twice Aspart and Aspart premixed30/70 insulin were used. Although, he was unable to inject insulin by himself recently. On physical examination, he walked in a mildly wide based manner. According to his family, urinary incontinence was existed. Laboratory data were as follows: Postrandial blood glucose 243 mg/dl and glycated hemoglobin 8.0% (NGSP). Brain magnetic resonance imaging (MRI) scans showed thinning of the corpus callosum with enlargement of the lateral ventricles on a colonal image. Evan's ratio was 0.29. The revised version of Hasegawa's Dementia scale (HDS-R) was 10. The patient showed no evidence a related antecedent event, such as head trauma, intracerebral hemorrhage and meningitis. Thus, he was diagnosed as having possible Idiopathic normal pressure hydrocephalus (iNPH). The following several psychological tests and walking test were applied. Before and after the tap, he was evaluated using the HDS-R, Mini mental state examination (MMSE), Timed Up and Go test (TUG). Insulin was replaced by glargine, and Sitagliptin was added. On the 31 day, the patient underwent Ventriculo-perioneal shunt. Laboratoly data and memory impairment were also improved. 8 month's later, HbA1c was 7.5%. iNPH occurs in the elderly and is characterized by a clinical triad of gait disturbance, urinary incontinence and dementia. In the present case, thinning of the corpus callosum with enlargement of the lateral ventricles was detected by MRI. 49% of iNPH patients had Diabetes mellitus. However, we were unable to detect a relationship iNPH and Diabetes mellitus. Cognitive impairment may interfere with the insulin therapy. In the present case, failure of insulin self-injection was the first clinical sign to appear. We were able to reduce dose of insulin. We conclude that iNPH is a treatable disorder, especially when treatment is started early in the course of the disease.